The Ross-Konno Procedure With or Without Mitral Valve Surgery: A Systematic Review With Individual Data Pooling
Published: March 08, 2024.
Volume 15, Number 4
Pages 411-418
Contributors
Nabil Dib; Thierry Ducruet; Nancy Poirier; Paul Khairy
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 411-418, July 2024.
Background: The Ross-Konno procedure is a technically demanding surgical option to treat multilevel left ventricular outflow tract obstruction. Methods: A systematic review with pooled analyses was conducted according to PRISMA criteria on studies published between January 2000 and May 2022 that assessed outcomes following the Ross-Konno intervention in children. Individual patient data were extracted from published Kaplan-Meier curves using digitalization software. Overall survival and freedom from reintervention were assessed by time-to-event approaches. Determinants of one-year survival were investigated by meta-regression analyses. Results: Ten studies with a total population of 274 patients were included. The overall pooled early (≤30 days) survival rate was 86.9% (95% CI [87.6%-78.4%]). Five-year survival rates in patients without and with (N = 50 [18.2%] of 274 total patients) concomitant mitral valve surgery were 82.5% (95% CI [87.6%-77.4%]) versus 56.1% (95% CI [74.1%-38.1%]), hazard ratio 2.67, 95% CI (1.44-4.93), P < .0001. Five- and ten-year freedom from pulmonary autograft reoperation rates were 93.5% and 90.9%, respectively. Five- and ten-year freedom from right ventricular outflow tract reoperation rates were 74.3% and 57.3%, respectively. By meta-regression analysis, resection of endocardial fibroelastosis (N = 32 [11.7%] of 274 total patients) was associated with superior one-year survival (P = .027). Conclusion: The Ross-Konno procedure is associated with substantial early mortality and gradual attrition thereafter. Mortality is higher in patients with concomitant mitral valve surgery. Resection of endocardial fibroelastosis is associated with superior survival. Right ventricular outflow tract reinterventions are common.
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Invited Commentary: What Is the Significance of the Konno Incision in Pediatric Ross Procedures?
Published: July 26, 2024.
Volume 15, Number 4
Pages 419-420
Contributors
Yishay Orr
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 419-420, July 2024.
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Excellent Survival With a Focus on Pulmonary Artery Reinterventions After the Arterial Switch Operation
Published: May 16, 2024.
Volume 15, Number 4
Pages 421-429
Contributors
Arif Selcuk; Mahmut Ozturk; Aybala Tongut; Vanessa Sterzbecher; In Hye Park; Rittal Mehta; Manan Desai; Can Yerebakan; Yves d’Udekem
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 421-429, July 2024.
Background: We sought to evaluate the outcomes in patients who underwent the arterial switch operation (ASO) over a 20-year period at a single institution. Methods: The current study is a retrospective review of 180 consecutive patients who underwent the ASO for biventricular surgical correction of dextro-transposition of the great arteries (d-TGA) between 2002 and 2022. Results: Among 180 patients, 121 had TGA-intact ventricular septum, 47 had TGA-ventricular septal defect and 12 had Taussig-Bing Anomaly (TBA). The median follow-up time was 6.7 years (interquartile range: 3.9-8.7 years). There were five early (2.8%) and one late (0.6%) mortality. Survival was 96.6% at one year and beyond. Reoperations were performed in 31 patients (17%). Taussig Bing Anomaly was found to increase the risk of reoperation by 17 times (P < .0001). A total of 37 (21%) patients underwent 53 reinterventions (14 surgical procedures, 39 catheter interventions) specifically addressing pulmonary artery (PA) stenosis. Freedom from PA reintervention was 97%, 87%, 70%, and 55% at 1, 5, 10, and 15 years, respectively. By bivariable analysis, TBA (P = .003, odds ratio [OR]: 6.4, 95% confidence interval [CI]: 1.9-21.7), mild PA stenosis at discharge (P ≤ .001, OR: 6.1, 95% CI: 2.7-13.6), and moderate or severe PA stenosis at discharge (P ≤ .001, OR: 12.7, 95% CI: 5-32.2) were identified as predictors of reintervention on PA. In the last follow-up of 174 survivors, 24 patients (14%) had moderate or greater PA stenosis, two (1%) had moderate neoaortic valve regurgitation, and 168 were New York Heart Association status I. Conclusions: Our results demonstrated excellent survival and functional status following the ASO for d-TGA; however, patients remain subject to frequent reinterventions especially on the pulmonary arteries.
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Invited Commentary: Pulmonary Artery Stenosis After the Arterial Switch Operation: Ounces of Prevention Better Than Pounds of Cure
Published: July 26, 2024.
Volume 15, Number 4
Pages 430-431
Contributors
Joshua L. Hermenson; Petros V. Anagnostopoulos
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 430-431, July 2024.
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Long-Term Outcomes of Ascending Sliding Arch Aortoplasty
Published: March 11, 2024.
Volume 15, Number 4
Pages 432-438
Contributors
Fumiya Yoneyama; Alyssa B. Kalustian; E. Dean McKenzie; Jeffrey S. Heinle; Tam T. Doan; Ziyad Binsalamah
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 432-438, July 2024.
Background: Coarctation of the aorta can be associated with significant hypoplasia of the aortic arch. In contrast to patch aortoplasty, ascending sliding arch aortoplasty uses viable autologous tissue for potential growth in children. We reviewed the mid- to long-term outcomes of this technique. Methods: Between 2002 and 2023, 28 patients underwent ascending sliding arch aortoplasty for the patients with coarctation of the aorta (n = 22) and interrupted aortic arch (n = 2). Four patients underwent previous surgical coarctation repair at other institutions. The median patient age and body weight were 28.5 months (3 weeks to 15.6 years) and 13.4 kg (3.7-70 kg), respectively. Results: Although one patient had a recurrent nerve injury postoperatively, there were no other major morbidities or mortalities. The last follow-up echocardiography demonstrated that the mean peak velocity improved from 3.9 ± 0.6 to 0.9 ± 0.8 m/s, and the pressure gradient improved from 63.6 ± 21.5 to 7.1 ± 7.7 mm Hg. The postoperative diameters of the ascending aorta, proximal arch, distal arch, and isthmus all increased significantly. The mean postoperative length of stay was 5.9 ± 2.1 days, and the median follow-up time was 7.3 years (10 days to 20.5 years). No reoperation or catheterization-based intervention was performed for residual coarctation. Conclusions: Ascending sliding arch aortoplasty is safe and effective for treating coarctation of the aorta with aortic arch hypoplasia. This technique is applicable for children ranging in size from neonates to older children (or adolescents), recurrent coarctation cases, and provides complete relief of narrowing by utilizing viable native aortic tissue.
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Public–Private Partnership for Treatment of Congenital Heart Diseases: Experiences From an Indian State
Published: January 24, 2024.
Volume 15, Number 4
Pages 439-445
Contributors
Debasis Das; Nilanjan Dutta; Shubhadeep Das; Manish Kumar Sharma; Amitabha Chattopadhyay; Sanjiban Ghosh; Jayita Nandy Das
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 439-445, July 2024.
Background: Treatment of congenital heart disease (CHD), being the most common congenital anomaly, puts immense financial burden in low- and middle-income countries (LMICs) and contributes significantly to infant mortality. We report experiences of treatment of CHD in the Indian state of West Bengal by a public–private partnership (PPP) model. Methods: Under the Rashtriya Bal Swasthya Karyakram, the government of the state of West Bengal in India launched a program called the “Sishu Sathi Scheme” to provide free treatment to children who need heart surgeries, irrespective of economic status. Treatment was provided in selected private hospitals and some public hospitals in a reimbursement model where government compensated the hospitals. Data were collected on such procedures from 2013 to 2022 and analyzed. Results: A total of 27,844 patients with CHD received treatment under the Sishu Sathi Scheme from August 2013 to December 2022. The average number of patients per year was 3,093. Detailed data of procedures from January 2016 to December 2022 showed a total of 22,572 procedures (6,249 device interventions, 4,840 cardiac catheterizations, and 11,483 surgical interventions). The in-hospital mortality of surgical procedures and catheterization lab procedures were 5.2% and 0.9%, respectively. Conclusions: A large number of patients with CHD were successfully treated under a PPP in the state of West Bengal in India. In spite of its inherent challenges, this model is of special relevance in LMICs where access and affordability for treatment of CHD always remain a challenge.
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Real-Time Detection of Circulating Thrombi in an Extracorporeal Circuit Using Doppler Ultrasound: In-Vitro Proof of Concept Study
Published: January 31, 2024.
Volume 15, Number 4
Pages 446-452
Contributors
Syed Murfad Peer; Manan Desai; Syed Bukhari; Yunchuan Delores; Richard Jonas; Pranava Sinha
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 446-452, July 2024.
BackgroundThromboembolic stroke continues to be by far the most common severe adverse event in patients supported with mechanical circulatory assist devices. Feasibility of using Doppler ultrasound to detect circulating thrombi in an extracorporeal circuit was investigated.MethodsA mock extracorporeal circulatory loop of uncoated cardiopulmonary bypass tubing and a roller pump was setup. A Doppler bubble counter was used to monitor the mean ultrasound backscatter signal (MUBS). The study involved two sets of experiments. In Scenario 1, the circuit was sequentially primed with human blood components, and the MUBS was measured. In Scenario 2, the circuit was primed with heparinized fresh porcine blood, and the MUBS was measured. Fresh blood clots (diameter <1,000 microns, 1,000-5,000 microns, >5,000 microns) were injected into the circuit followed by protamine administration.ResultsIn Scenario 1 (n = 3), human platelets produced a baseline MUBS of 1.5 to 3.5 volts/s. Addition of packed human red blood cells increased the baseline backscatter to 17 to 21 volts/s. Addition of fresh frozen plasma did not change the baseline backscatter. In Scenario 2 (n = 5), the blood-primed circuit produced a steady baseline MUBS. Injection of the clots resulted in abrupt and transient increase (range: 3-30 volts/s) of the baseline MUBS. Protamine administration resulted in a sustained increase of MUBS followed by circuit thrombosis.ConclusionsDoppler ultrasound may be used for real-time detection of circulating solid microemboli in the extracorporeal circuit. This technology could potentially be used to design safety systems that can reduce the risk of thromboembolic stroke associated with mechanical circulatory support therapy.
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Handmade Bicuspid Valved Polytetrafluoroethylene Conduit for Right Ventricular Outflow Tract Reconstruction
Published: March 08, 2024.
Volume 15, Number 4
Pages 453-458
Contributors
Dhananjay P. Malankar; Shyam Dhake; Amit Mhatre; Bharat Soni; Dinesh Kandavel; Sujit Bamne; Swati Garekar
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 453-458, July 2024.
Background: Excellent outcomes of right ventricle to pulmonary artery conduits with polytetrafluoroethylene (PTFE) valves have been reported. The purpose of this study was to analyze the short-term results of our handmade PTFE bicuspid valved conduit (VC) for right ventricular outflow tract reconstruction. Methods: Between September 2019 and May 2023, bicuspid PTFE-VC was implanted in 17 patients at a median age of 2.5 years (range, 3 months to 13.6 years). The PTFE-VC was fashioned from a commercially available PTFE tube graft (14 mm in three patients, 16 mm in three patients, 18 mm in one patient, 20 mm in three patients, and 22 mm in seven patients) and 0.1 mm thick PTFE membrane for the leaflet material. Valve function was assessed by echocardiogram after the implantation. The conduit reoperation and the conduit dysfunction were analyzed. There were no early deaths, but there was one late death. Results: There were no postoperative in-hospital deaths. Follow-up echocardiograms were available for 14 of 17 patients. The median follow-up was 21 months (range, 7-49 months). Conduit stenosis was none or trivial in 11 patients while it was mild in two and moderate in one patient and severe in 0 patients. Conduit insufficiency was mild or trivial in all 14 patients. By the end of the study period, freedom from reoperation/reintervention was 100%. There were no episodes of aneurysmal dilatation of the conduit or endocarditis. Conclusions: Handmade bicuspid PTFE VC shows good short-term outcome, with no significant valve dysfunction and no reintervention. A longer follow-up is necessary to evaluate the long-term advantages of using the handmade bicuspid PTFE VC.
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Early Impairment of Cerebral Bioenergetics After Cardiopulmonary Bypass in Neonatal Swine
Published: April 22, 2024.
Volume 15, Number 4
Pages 459-466
Contributors
Danielle I. Aronowitz; Tracy R. Geoffrion; Sarah Piel; Emilie J. Benson; Sarah R. Morton; Jonathan Starr; Richard W. Melchior; Hunter A. Gaudio; Rinat E. Degani; Nicholas J. Widmann; M. Katie Weeks; Tiffany S. Ko; Daniel J. Licht; Marco Hefti; J. William Gaynor; Todd J. Kilbaugh; Constantine D. Mavroudis
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 459-466, July 2024.
Objectives: We previously demonstrated cerebral mitochondrial dysfunction in neonatal swine immediately following a period of full-flow cardiopulmonary bypass (CPB). The extent to which this dysfunction persists in the postoperative period and its correlation with other markers of cerebral bioenergetic failure and injury is unknown. We utilized a neonatal swine model to investigate the early evolution of mitochondrial function and cerebral bioenergetic failure after CPB. Methods: Twenty piglets (mean weight 4.4 ± 0.5 kg) underwent 3 h of CPB at 34 °C via cervical cannulation and were followed for 8, 12, 18, or 24 h (n = 5 per group). Markers of brain tissue damage (glycerol) and bioenergetic dysfunction (lactate to pyruvate ratio) were continuously measured in cerebral microdialysate samples. Control animals (n = 3, mean weight 4.1 ± 1.2 kg) did not undergo cannulation or CPB. Brain tissue was extracted immediately after euthanasia to obtain ex-vivo cortical mitochondrial respiration and frequency of cortical microglial nodules (indicative of cerebral microinfarctions) via neuropathology. Results: Both the lactate to pyruvate ratio (P < .0001) and glycerol levels (P = .01) increased in cerebral microdialysate within 8 h after CPB. At 24 h post-CPB, cortical mitochondrial respiration was significantly decreased compared with controls (P = .046). The presence of microglial nodules increased throughout the study period (24 h) (P = .01, R2 = 0.9). Conclusion: CPB results in impaired cerebral bioenergetics that persist for at least 24 h. During this period of bioenergetic impairment, there may be increased susceptibility to secondary injury related to alterations in metabolic delivery or demand, such as hypoglycemia, seizures, and decreased cerebral blood flow.
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Successful Late Repair of Truncus Arteriosus: A Single Center Experience
Published: March 22, 2024.
Volume 15, Number 4
Pages 467-471
Contributors
Mohamed M. Elgayar; Mohamed M. Mostafa; Mohamed Adel; Hatem Hosny
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 467-471, July 2024.
Background: Truncus arteriosus is a rare congenital heart defect resulting from the failure of the truncus arteriosus to divide during fetal development. It leads to a single outflow tract from the heart and, if left untreated, can be fatal. Late presentation and repair can also increase the risk of pulmonary hypertensive crises, which can lead to morbidity and mortality after repair. Methods: We performed a retrospective study examining outcomes of late-presenting patients who were repaired for this anomaly at our institution. Results: We identified seven patients who underwent late repair of truncus arteriosus who were 3 to 11 years of age. There were six females and one male. Postoperatively, all patients showed improvement in symptoms and hemodynamic parameters, with no reported mortality. The median duration of stay in the intensive care unit was nine days and with a range from 3 to 18 days, while the median hospital stay was 29 days with a range from 21 to 60 days. Conclusion: These findings highlight the potential for successful outcomes even in cases of delayed diagnosis.
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Operative Repair of Aortopulmonary Window: A 25-Year Experience
Published: April 22, 2024.
Volume 15, Number 4
Pages 472-480
Contributors
Alyssa B. Kalustian; Richard C. Tang; Michiaki Imamura
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 472-480, July 2024.
Background: Aortopulmonary window (APW) is a rare anomaly with variable morphology and associated cardiac anomalies. We evaluated impact of patient and operative factors on mid-term outcomes following APW repair. Methods: Twenty-nine patients underwent surgical APW repair at our institution from 1996 to 2022. Eight (28%) had simple APW, accompanied by only atrial septal defect or patent ductus arteriosus; 21 (72%) had complex APW with additional cardiovascular lesions, including nine with interrupted aortic arch. Median operative age was 19 days (range 2 days-1.5 years) via single-patch (n = 12, 41%), double-patch (n = 15, 52%), or ligation and division (n = 2, 7%). Results: The only mortality occurred in-hospital 1.4 years postoperatively following remote myocardial infarction. Factors associated with longer postoperative length of stay were complex APW (P = .003), genetic syndrome (P = .003), noncardiovascular comorbidities (P = .002), lower birth weight (P = .03), and lower operative weight (P = .03). Six patients (21%) with complex APW underwent unplanned cardiothoracic reintervention(s), including two with arch reintervention following arch advancement for interruption. Reintervention-free survival was similar for simple versus complex APW, operative age categories, and repair techniques. At median follow-up 5.5 years postoperatively, no patients had residual APW or persistent pulmonary hypertension, 1 (3%) had greater than mild ventricular dysfunction, and 25 (89% survivors) had NYHA class I functional status. Conclusions: Operative APW repair has excellent mid-term survival, durability, and functional status, regardless of operative age, cardiovascular comorbidities, or repair technique. Cardiac and noncardiac comorbidities may be associated with prolonged length of stay.
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Comparison of Right Ventricular Outflow Tract Reconstruction Techniques on Mid-Term Pulmonic Valve Fate
Published: April 27, 2024.
Volume 15, Number 4
Pages 481-487
Contributors
Noppon Taksaudom; Pradchaya Thuropathum; Thitipong Tepsuwan; Apichat Tantraworasin; Rekwan Sittiwangkul; Amarit Phothikun; Surin Woragidpoonpol
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 481-487, July 2024.
Introduction: The pulmonic valve-sparing technique (PVS) is an emerging approach of right ventricular outflow tract reconstruction in tetralogy of Fallot (TOF) correction aimed at reducing the incidence of pulmonic regurgitation (PR) and the need for subsequent reintervention. This study aims to compare the long-term occurrence of moderate to severe PR/stenosis (PR/PS) between three different approaches. Patients and Methods: We conducted a retrospective cohort study involving 173 patients who underwent TOF correction at Chiang Mai University hospital between January 2006 and December 2016. The patients were divided into three groups: transannular patch (TAP; n = 88, 50.9%), monocusp insertion (MCI; n = 40, 23.1%), and PVS (n = 45, 26%). The study assessed freedom from moderate to severe PR/PS. Results: The median overall follow-up time was 79.8 months (interquartile range: 50.7-115.5 months. The PVS exhibited larger PV Z-score (−2.6 ± 2.3 mm, P < .001), with predominantly tricuspid morphology (64.4%). The PVS had significantly shorter median ventilator time, intensive care unit stay, hospital stay, and longer median follow-up time. Postoperative moderate-severe PR was lower in the PVS group (P < .001), with no significant difference in PS (P = .356) and complications among the groups. Freedom from moderate-severe PR/PS was longer in the MCI group (2.8, 0.2-42.3 months vs 30.9, 0.2-50.9 months, respectively). Multivariable analysis showed TAP and MCI had a higher risk of developing moderate-severe PR (hazard ratio [HR] 2.51; 95% confidence interval [CI] 1.23-5.13 vs HR 1.41; 95%CI 0.59-3.38) but lower risk of moderate-severe PS (HR 0.14; 95%CI 0.02-0.9 vs HR 0.39; 95%CI 0.05-3.19). Conclusion: Pulmonic valve-sparing reconstruction showed promise in preventing late moderate-severe PR in patients with favorable PV anatomy. However, it should be noted that this technique is associated with a higher incidence of PS.
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Deep Sedation in Pediatric Patients With Single Ventricle Physiology Outside of the Operating Room
Published: January 12, 2024.
Volume 15, Number 4
Pages 488-493
Contributors
Mouhammad Yabrodi; Sarah Abdel-Mageed; Kamal Abulebda; Lee D. Murphy; Andrew Rodenbarger; Hamza Bhai; Riad Lutfi; Matthew L. Friedman
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 488-493, July 2024.
Background: Advancements in palliative surgery of patients with single ventricle physiology have led to an increase in the need for deep sedation protocols for painful procedures. However, positive pressure ventilation during anesthesia can result in unfavorable cardiopulmonary interactions. This patient population may benefit from sedation from these painful procedures. Methods: This study aims to demonstrate the safety and efficacy of deep sedation by pediatric intensivists outside the operating room for children with single ventricle physiology. This is a single-center, retrospective chart review on consecutive pediatric patients with single ventricle physiology who received deep sedation performed by pediatric intensivists between 2013 and 2020. Results: Thirty-three sedations were performed on 27 unique patients. The median age was 3.7 years (25th%-75th%: 2.1-15.6). The majority of the sedations, 88% (29/33), were done on children with Fontan physiology and 12% (4/33) were status-post superior cavopulmonary anastomosis. The primary cardiac defect was hypoplastic left heart in 63% (17/27) of all sedation procedures. There were 24 chest tube placements and 9 cardioversions. Ketamine alone [median dose 1.5 mg/kg (range 0.8-3.7)], ketamine [median dose 1 mg/kg (range 0.1-2.1)] with propofol [median dose 2.3 mg/kg (range 0.7-3.8)], and ketamine [median dose 1.5 mg/kg (range 0.4-3.0)] with morphine [median dose 0.06 mg/kg (range 0.03-0.20)] were the most common sedation regimens used. Adverse events (AEs) occurred in 4 patients (15%), three of which were transient AEs. All sedation encounters were successfully completed. Conclusion: Procedural deep sedation can be safely and effectively administered to single ventricle patients by intensivist-led sedation teams in selective case.
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Mentoring Pediatric Heart Surgeons: A Difficult Road for Both Mentors and Mentees
Published: May 23, 2024.
Volume 15, Number 4
Pages 494-497
Contributors
Emile Bacha
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 494-497, July 2024.
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Autologous Reconstruction of Anomalous Origin of a Right Pulmonary Artery From the Aorta
Published: March 22, 2024.
Volume 15, Number 4
Pages 498-502
Contributors
Sachin Talwar; Pratik Kumar Jha; Niwin George; Arindam Choudhury; Amitabh Satsangi; Shiv Kumar Choudhary
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 498-502, July 2024.
Anomalous origin of a pulmonary artery branch from the aorta is a rare congenital anomaly in which one of the pulmonary arteries arises from the aorta. These patients require early surgery to prevent development of severe irreversible pulmonary arterial hypertension. Multiple techniques have been described for repair of this condition. In this report, we describe a different technique compared with previously described procedures and discuss its advantages.
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Dual Drainage Total Anomalous Pulmonary Venous Connection With Infracardiac and Supracardiac Drainage
Published: May 22, 2024.
Volume 15, Number 4
Pages 503-505
Contributors
Nabeel Valappil Faisal; Suad Akhtar; Niraj Nirmal Pandey; Saurabh Kumar Gupta
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 503-505, July 2024.
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Gerbode Defect—Multimodality Imaging
Published: May 16, 2024.
Volume 15, Number 4
Pages 506-507
Contributors
M Kardos; J Polakova Mistinova
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 506-507, July 2024.
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Anomalous Left Coronary Artery From the Pulmonary Artery With Ventricular Septal Defect and Double Aortic Arch: A Unique Case Report
Published: May 02, 2024.
Volume 15, Number 4
Pages 508-510
Contributors
Fadi Sabateen; Matej Nosáľ; Pavel Valentík; Lucia Bobík; Vladimír Soják
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 508-510, July 2024.
Anomalous left coronary artery from the pulmonary artery (ALCAPA) is an uncommon life-threatening congenital heart defect that occurs mostly as an isolated lesion. It is very rarely associated with other cardiac abnormalities. In this report, we present a case of an infant who successfully underwent complete surgical repair of a rare triad of congenital heart defects, including ALCAPA, ventricular septal defect (VSD), and double aortic arch (DAA). To the best of our knowledge, this is only the second case of ALCAPA diagnosis associated with VSD and DAA that has been described in the literature.
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An Unusual Presentation of Double-Outlet Right Atrium
Published: April 27, 2024.
Volume 15, Number 4
Pages 510-514
Contributors
Hamood Al Kindi; Amr Abolwafa; Halima Al Hashmi; Salim Al-Maskari; Madan Mohan Maddali; Robert Henry Anderson
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 510-514, July 2024.
We describe an unusual example of double-outlet right atrium with separate atrioventricular junctions. The straddling and overriding tricuspid valve had two orifices, and the mitral valve was morphologically normal. An appropriate understanding of the morphology of the atrioventricular junctions, the valves, and the subvalvar apparatus, along with the location of the atrioventricular conduction axis, allowed for successful biventricular repair.
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Eleven Years After Scimitar Syndrome Repair With the Lugones Technique: Usefulness of Four-Dimensional Flow Magnetic Resonance Imaging
Published: May 07, 2024.
Volume 15, Number 4
Pages 515-518
Contributors
Idara Zezular; Lisa Carlson Hanse; Hyon Ju Choe; Damian Craiem; Ignacio Lugones
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 515-518, July 2024.
Anatomic and physiologic assessment of surgical repair of scimitar syndrome can be challenging. We evaluated the first patient who underwent the Lugones procedure in 2012 using 4D flow magnetic resonance imaging. With this technology, we demonstrate that the reconstructed right pulmonary venous return drains into the left atrium with laminar flow, just as normal pulmonary veins do.
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Total Aortic Arch and Neoaortic Root Replacement Following Stage III Palliation for Hypoplastic Left Heart Syndrome
Published: March 11, 2024.
Volume 15, Number 4
Pages 518-520
Contributors
Sayar Kumar Munshi; Tommaso Generali; Arul Narayanan; Raul Jose Correia; Ramesh Kutty; Ram Dhannapuneni
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 518-520, July 2024.
Progressive dilatation of the neoaortic root and reconstructed aortic arch is a serious complication after the Norwood procedure. There are no clear guidelines on the management of this complex anatomy in the setting of single ventricle physiology, and the surgical treatment of such an entity remains anecdotal. We describe a successful surgical repair in a 15-year-old girl presenting with a severely dilated neoaortic root and aortic arch causing compression and narrowing of the left pulmonary artery after successful three-stage palliation for hypoplastic left heart syndrome.
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A Rare Cause of a Continuous Murmur in a Newborn
Published: March 08, 2024.
Volume 15, Number 4
Pages 521-523
Contributors
Alyson R. Pierick; Hillary Liken; Michael R. Joynt
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 521-523, July 2024.
We describe what we believe to be the first reported case of a neonate with right coronary ostial atresia with the right coronary artery originating from the left circumflex coronary artery, in conjunction with a right coronary artery to right ventricle fistula in a patient with otherwise normal cardiac anatomy. This was found following an evaluation for a continuous murmur at 2 weeks of life with elevated troponin and abnormal electrocardiogram. Thus far the child has required no intervention and is asymptomatic at 17 months of age, but he will require long-term follow-up to monitor the size of the fistula and potential for myocardial insufficiency.
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Uhl’s Anomaly in Adulthood
Published: May 02, 2024.
Volume 15, Number 4
Pages 523-525
Contributors
Omar A. M. Mohamed; Marwa EL-Dardeery; Kareem Zayed; Eleia Mosaad; Muhammad M. Abdulwahhab; Soha Romeih
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 523-525, July 2024.
Uhl’s anomaly is a rare congenital syndrome characterized by the absence of right ventricular myocardium. The widely accepted pathological mechanism is intrauterine myocardial apoptosis. Uhl’s syndrome carries a poor prognosis. In rare situations, patients with Uhl’s anomaly reach adulthood. We will present a case of a 29-year-old patient with Uhl’s syndrome treated at our center, highlighting the diagnostic, surgical, and postoperative challenges in management.
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A Case of Persistent Junctional Reciprocating Tachycardia Coincident With Cor Triatriatum Sinister
Published: April 22, 2024.
Volume 15, Number 4
Pages 525-527
Contributors
Molly C. Carney; Brock A. Karolcik; Aditi Gupta; Gaurav Arora; Lee B. Beerman; Christopher W. Follansbee
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 525-527, July 2024.
Persistent junctional reciprocating tachycardia is a rare form of refractory atrioventricular reentrant tachycardia that accounts for <1% of supraventricular tachycardia in pediatrics. The accessory pathways are generally isolated with few reported underlying structural heart defects. We present a case of a five-month-old male with refractory tachyarrhythmia found to have cor triatriatum sinister, which to our knowledge, is the first reported case of these two rare anomalies coexisting.
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Hypoplastic Circumflex Aorta With Anomalous Left Pulmonary Artery: Uncrossing Operation in a Neonate
Published: April 24, 2024.
Volume 15, Number 4
Pages 527-530
Contributors
Qais Alloah; Nilanjana Misra; Elena Kwon; Simone Jhaveri; Hari Rajagopal; Christopher Gasparis; David B. Meyer
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 527-530, July 2024.
Circumflex aortic arch with coarctation and anomalous origin of the left pulmonary artery from the aorta are rare cardiovascular anomalies. These conditions can lead to early pulmonary hypertension and challenging management. Early diagnosis and surgical intervention are beneficial for optimal outcome. We present a case where both anomalies coexisted and were repaired with aortic uncrossing, arch augmentation, and reimplantation of the left pulmonary artery. To our knowledge, this is the first documented instance of these anomalies coexisting and being repaired in the neonatal period.
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Complex Atrial Baffling Procedures in Left Isomerism With Right- and Left-Hand Topology
Published: May 08, 2024.
Volume 15, Number 4
Pages 530-533
Contributors
Hamood Al Kindi; Pranav Kandachar; Abdullah Mohsen; Abdullah Al Balushi; Ismail Al Abri; Madan Maddali; Robert H. Anderson
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 530-533, July 2024.
We describe complex atrial baffling procedures in the setting of left isomerism with right-hand as opposed to left-handed ventricular topology. An appropriate understanding of the connections of the systemic and pulmonary veins, along with the internal atrial anatomy, as revealed using 3D printing, allowed for successful biventricular repair.
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Atrial Septal Defect Closure: Not Always Straightforward
Published: May 06, 2024.
Volume 15, Number 4
Pages 534-535
Contributors
W. Jack Wallen; Carl L. Backer
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 534-535, July 2024.
A nine-year old boy presented following a chest x-ray for COVID-19 infection that showed cardiomegaly. Transthoracic echocardiogram revealed a large atrial septal defect, dilated right heart, and normal function. Surgical repair was performed eight weeks after his COVID diagnosis. After weaning from cardiopulmonary bypass, pulmonary artery pressures were 2/3 systemic and ectopy was seen, requiring inotropes, nitric oxide, lidocaine, and amiodarone. He was discharged on postoperative day (POD) 5. On POD 6, he presented with acute right foot ischemia. Computed tomography showed a large aortic thrombus, requiring emergent thrombectomy. Coagulopathy workup was negative. Cardiac magnetic resonance imaging (CMR) and catheterization showed reduced biventricular function and diastolic dysfunction. Diuretics and β blockers were started, with gradual improvement in left ventricular systolic function.
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Current Models for Congenital Heart Center Structure and Health System Integration
Published: April 22, 2024.
Volume 15, Number 4
Pages 536-537
Contributors
Sara K. Pasquali
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 4, Page 536-537, July 2024.
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A Rare Variant of Aorto-Atrial Fistula; Left Sinus of Valsalva Fistula to Superior Vena Cava and Right Atrium in a Neonate
Published: August 20, 2024.
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Contributors
Naveen Srinivasan
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
Aorta-atrial fistula (AAF) is defined as the communication of blood flow between the aorta and the atrium. Most of the fistula occurs between the ascending aorta and the right atrium. Aorta-atrial fistula occurs mostly due to acquired causes. Congenital causes remain a minority. We report a very rare variant of AAF in a two-day-old neonate in whom the fistula was arising from the left sinus of Valsalva of the aortic root and draining into the superior vena cava and the right atrium. The child underwent successful surgical ligation of the fistula on a beating heart and is doing well on a two-year follow-up.
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Establishment of a Global Platform for the Treatment of Congenital Heart Disease by “Creation and Unification of National Congenital Heart Surgery Databases and Registries”: It’s Time!
Published: August 09, 2024.
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Contributors
James D. St Louis; Jeffrey P. Jacobs; Brian P. Bateson; Christo I. Tchervenkov; Erle H. Austin; James K. Kirklin
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
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Revisiting the Role of Balloon Atrial Septostomy Prior to the Arterial Switch Operation
Published: August 09, 2024.
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Sujata Subramanian; Sagar Jani; Andrew Well; Matthew F. Mikulski; Hitesh Agrawal; D Byron Holt; Neil Venardos; Carlos M. Mery; Charles D. Fraser
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
ObjectivesPatients with dextro-transposition of the great arteries (d-TGA) frequently undergo balloon atrial septostomy (BAS) prior to the arterial switch operation (ASO) to promote atrial-level mixing. Balloon atrial septostomy has inherent risks as an invasive procedure and may not always be necessary. This study revisits the routine utilization of BAS prior to ASO.MethodsSingle-center, retrospective review of d-TGA patients undergoing the ASO from July 2018 to March 2023. Preoperative patient characteristics, pulse oximetry oxygen saturations (SpO2), cerebral/renal near-infrared spectroscopy (NIRS) readings along with prostaglandin status at the time of the ASO were analyzed with descriptive and univariate statistics.ResultsThirty patients underwent the ASO. Of these, 7 (23%) were female, 25 (83%) were white, and median weight at ASO was 3.2 kg (range 0.8-4.2). Twenty-two (73%) patients underwent BAS. There were no demographic differences between BAS and no-BAS patients. Of those who underwent BAS, there was a significant increase in SpO2 (median 83% [range 54-92] to median 87% [range 72-95], P = .007); however, there was no change in NIRS from pre-to-post BAS. Six (27%) patients in the BAS group were prostaglandin-free at ASO. Balloon atrial septostomy patients underwent the ASO later compared with no-BAS patients (median 8 [range 3-32] vs 4 [range 2-10] days old, P = .016) and had a longer hospital length of stay (median 13 [range 7-43] vs 10 [range 8-131] days, P = .108).ConclusionsWhile BAS is an accepted preoperative procedure in d-TGA patients to improve oxygen saturations, it is also an additional invasive procedure, does not guarantee prostaglandin-free status at the time of ASO, and may increase the interval to ASO. Birth to direct early ASO, with prostaglandin support, should be revisited as an alternative, potentially more expeditious strategy.
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Use of Hybrid Stage I to Stratify Between Single Ventricle Palliation and Biventricular Repair
Published: August 09, 2024.
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Justin Smith; Jeffrey D. Zampi; Sowmya Balasubramanian; Bryan Mosher; Karen Uzark; Ray Lowery; Sunkyung Yu; Jennifer C. Romano
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
Background: Hybrid stage I palliation (HS1P) has been utilized for patients with single ventricle (SV) congenital heart disease (CHD). To date, reports on the use of HS1P for other indications including biventricular (BiV) CHD have been limited. Methods: We performed a single-center retrospective cohort study of patients who underwent HS1P with an anticipated physiologic outcome of BiV repair, or with an undetermined SV versus BiV outcome. Patient characteristics and outcomes from birth through definitive repair or palliation were collected and reported with descriptive statistics. Results: Nineteen patients underwent HS1P with anticipated BiV repair. Extracardiac and intracardiac risk factors (ICRF) were common. Ultimately, 13 (68%) patients underwent BiV repair, 1 (5%) underwent SV palliation, and 5 (26%) died prior to further palliation or repair. Resolution of ICRF tracked with BiV outcome (6/6, 100%), persistence of ICRF tracked with SV outcome or death (3/3, 100%). Twenty patients underwent HS1P with an undetermined outcome. Ultimately, 13 (65%) underwent BiV repair, 6 (30%) underwent SV palliation, and 1 (5%) underwent transplant. There were no deaths. Intracardiac risk factors were present in 15 of 20 patients (75%); BiV repair only occurred when all ICRF resolved (67%). Post-HS1P complications and reinterventions occurred frequently in both groups, through all phases of care. Conclusions: Hybrid stage 1 palliation can be used to defer BiV repair and to delay decision between SV palliation and BiV repair. Resolution of ICRF was associated with ultimate outcome. In this high-risk group, complications are common, and mortality especially in the marginal BiV patient is high.
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Pediatric Quadricuspid Aortic Valve: Morphology, Characteristics, Clinical Outcomes, and Literature Review
Published: July 25, 2024.
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Jordan Holthe; Rabia Javed; Frank Cetta; Elizabeth H. Stephens; Jonathan N. Johnson; Donald J. Hagler; Talha Niaz
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
BackgroundQuadricuspid aortic valve (QAV) is an exceedingly rare congenital heart defect (CHD) which has not been well-defined in a pediatric population.MethodsThe Mayo Clinic echocardiography database was retrospectively analyzed to identify patients ≤18 years diagnosed with QAV from January 1990 to December 2023. Patients with truncus arteriosus were excluded. All images were independently reviewed to define morphology of the QAV by using the Hurwitz and Roberts classification.ResultsFourteen patients with QAV were identified with a median age at time of diagnosis being 10.5 years (interquartile range [IQR] 6-14 years). Male-to-female ratio was 3:1. Associated CHDs were present in 50% (n = 7) patients. The most common morphological subtypes of QAV were Type D in 43% (n = 6) and Type B in 29% (n = 4). Aortic regurgitation was the most frequently associated valvular abnormality affecting 86% (n = 12) cases, with greater than moderate regurgitation in only two patients. Aortic valve stenosis was observed in 14% (n = 2) patients. Ascending aortic dilatation was present in 21% (3/14) of the cohort, but only 14% (1/7) of isolated QAV patients. At a mean follow up of 11 ± 6.6 years and a median follow-up age of 22 years (IQR 14-27 years), survival was 100% with no primary interventions on the aortic valve or aorta. However, four patients required surgical interventions for associated CHDs.ConclusionAmong children with QAV, almost half of the patients had additional CHD. Aortic regurgitation was the predominant hemodynamic abnormality. Long-term survival was excellent with minimal progression during childhood and adolescence.
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The Fate of the Left Ventricular Outflow Tract Following Interrupted Aortic Arch Repair
Published: July 25, 2024.
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Shuhua Luo; Paul H. Schoof; Edward Hickey; Conall Morgan; Nina A. Korsuize; Heynric B. Grotenhuis; Luc Mertens; Jaymie Varenbut; Mimi Xiaoming Deng; Maruti Haranal; William Border; Brian Schlosser; Glen Van Arsdell; Bahaaldin Alsoufi
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
Objectives: To examine the probability of left ventricular outflow tract (LVOT) reintervention following interrupted aortic arch (IAA) repair in neonates with LVOT obstruction (LVOTO) risk. Methods: This retrospective multicenter study included 150 neonates who underwent IAA repair (2003-2017); 100 of 150 (67%) had isolated IAA repair (with ventricular septal defect closure) and 50 of 150 (33%) had concomitant LVOT intervention: conal muscle resection (n = 16), Ross-Konno (n = 7), and Yasui operation (n = 27: single-stage n = 8, staged n = 19). Demographic and morphologic characteristics were reviewed. Factors associated with LVOT reoperation were explored using multivariable analysis. Results: Concomitant LVOT intervention was more likely in neonates with type B IAA, bicuspid aortic valve, aberrant right subclavian artery, smaller aortic valve annulus, and ascending aorta dimensions. On follow-up, five-year freedom from LVOT reoperation was highest following Ross-Konno (100%), 77% following Yasui (mainly for neo-aortic regurgitation), 77% following isolated IAA repair (mainly for LVOTO), and 47% following IAA repair with concomitant conal resection, P = .033. While all patients had low peak LVOT gradient at time of discharge, those who had conal resection developed higher gradients on follow-up (P = .007). Ross-Konno and Yasui procedures were associated with higher right ventricular outflow tract (RVOT) reoperation. In the cohort following isolated IAA repair, aortic sinus Z score was associated with LVOT reoperation. Conclusions: Both Yasui and Ross-Konno operations effectively mitigate late LVOTO risk. The highest risk of reintervention for LVOTO was associated with conal muscle resection while the lowest risk is associated with Ross-Konno. The RVOT reoperation risk in patients who had Ross-Konno or Yasui does not seem to affect survival.
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Common Arterial Trunk Repair at the Red Cross War Memorial Hospital, Cape Town: A 20-Year Review of Surgical Practice and Outcomes
Published: July 23, 2024.
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Contributors
A Moodley; HM Meyer; S Salie; P Human; L J Zühlke; A Brooks
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
BackgroundThis study describes the 20-year experience of managing common arterial trunk (CAT) in a low-and-middle-income country and compares the early and medium-term outcomes following the transition from conduit to nonconduit repair at the Red Cross War Memorial Children’s Hospital.MethodsSingle-center retrospective study of consecutive patients aged less than 18 years who underwent repair of CAT from January 1999 to December 2018 at the Red Cross War Memorial Children’s Hospital. Patients with interrupted aortic arch or previous pulmonary artery banding were excluded.ResultsFifty-four patients had CAT repair during the study period. Thirty-four (63.0%) patients had a conduit repair, and 20 (37.0%) patients had a nonconduit repair. There were two intraoperative deaths. Thirty-day in-hospital mortality was 22.2% (12/54). Overall, in-hospital mortality was 29.6% (16/54). Eight (21.1%) late mortalities were observed. The actuarial survival for the conduit group was 77.5%, 53.4%, and 44.5% at 6, 12, and 27 months, respectively, and the nonconduit group was 58.6% at six months. The overall freedom from reoperation between the conduit group and nonconduit group was 66.2% versus 86.5%, 66.2% versus 76.9%, and 29.8% versus 64.1% at 1, 2, and 8 years, respectively.ConclusionsThe outcomes following the transition to nonconduit repair for CAT in a low- and middle-income setting appear to be encouraging. There was no difference in mortality between conduit and nonconduit repairs, and importantly the results suggest a trend toward lower reintervention rates.
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Outcomes of Patients Undergoing the Kawashima Procedure at an Early Age: A Single Center Experience
Published: July 23, 2024.
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Brian P. Bateson; Matthew Files; Lyubomyr Bohuta
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
BackgroundPrevious reports have demonstrated the safety and efficacy of performing early Kawashima procedure (KP). Despite this, more recent studies have shown that the average age at the time of operation remains greater than one year of age. We report our experience with performing KP at an earlier age than previously reported.MethodsA retrospective review was completed of patients undergoing KP at a single center (January 2000 to June 2020). Clinical outcomes were examined.ResultsInitial palliation was performed in 11 out of 12 patients. Age and weight at time of KP were 8.25 months (6.4-9.7) and 7.7 kg (6.5-8.6). Intensive care unit stay was 3.16 days (1-12), overall length of stay was 9.5 days (3-22). There was one unplanned reoperation, and no mortality in the cohort. Discharge oxygen saturation was 88% (80%-98%). Seven patients developed pulmonary arteriovenous malformations (PAVMs) with six proceeding to hepatic vein incorporation (HVI). Interval time to development of PAVMs was 42.3 months (16-121). Four of the 12 patients were left with antegrade pulmonary blood flow (PBF) and three (75%) remain without PAVMs. Median follow-up was 10 years (1.5-22) with 11 of 12 (91.67%) survival for the cohort.ConclusionsThe KP can be done at a younger age than previously reported with adequate early and late results. Most patients will go on to develop PAVMs and require HVI but leaving patients with some antegrade PBF is likely protective but will need further investigation to show definitive benefit.
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PA-VSD Without MAPCA(s): Review of Long-Term Outcomes and Reinterventions
Published: July 23, 2024.
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Elizabeth H. Stephens; Joseph A. Dearani; William R. Miranda; Jason H. Anderson
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
BackgroundPulmonary atresia with ventricular septal defect without major aortopulmonary collateral arteries (MAPCAs) is an uncommon form of congenital heart disease. As more patients with congenital heart disease live to adulthood, the objective of this article was to review the long-term results of this specific population.MethodsA review of the PubMed database was performed using pertinent key words (pulmonary atresia, tetralogy of Fallot, conduit, right ventricle-pulmonary artery) concentrating on studies from 1990-present and published in English. Most studies of pulmonary atresia-ventricular septal defect patients included those with and without MAPCAs. Analysis included examination of the entire cohort, consideration of the proportion of patients with MAPCAs, and any subgroup analysis of the patients without MAPCAs.ResultsSurvival is approximately 80% at ten years and is improved with complete repair and larger pulmonary arteries. Some studies have found genetic syndromes and extracardiac anomalies to impact survival, while others have not. Incomplete repair has been shown to be associated with worse survival. Independent of initial management strategy, patients with pulmonary atresia and ventricular septal defects without MAPCAs require repeat intervention on the right ventricular outflow tract. Hypoplastic pulmonary arteries have been shown to be a risk factor for reintervention, and decreased conduit durability has been shown with younger age at implantation of conduit.ConclusionsLong-term outcomes have improved for patients with pulmonary atresia-ventricular septal defect without MAPCAs, with complete repair and adequate pulmonary arteries favorable for survival. Long-term outcomes include reinterventions, both catheter-based and surgical, predominantly on the right ventricular outflow tract.
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Breast Milk and Necrotizing Enterocolitis in Congenital Heart Disease: A Case–Control Study
Published: July 23, 2024.
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Contributors
Margaret R. Christian; David Bateman; Marianne Garland; Usha S. Krishnan; Emile A. Bacha; Ganga Krishnamurthy
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
Background: Necrotizing enterocolitis (NEC) is a complication that can affect infants with congenital heart disease (CHD). The objective of this study is to determine whether breast milk, which is associated with decreased incidence of NEC in preterm infants, is protective in infants with CHD. Methods: Retrospective case–control study of infants ≥ 33 weeks gestational age with CHD who underwent cardiac surgery during their admission to the Infant Cardiac Unit from 2008 to 2017. Cases were defined as infants with modified Bell’s stage ≥ II NEC. Controls were matched by date of birth, gestational age, and pre- or postcardiac surgery feed initiation. Results: A total of 926 infants with gestational age ≥ 33 weeks and CHD were admitted; 18 cases of NEC were identified and compared with 84 controls. Breast milk intake was higher in controls, but this difference was not statistically significant. Single ventricle (SV) physiology was identified as an independent risk factor for NEC by multivariable analysis. Analysis of infants with SV physiology demonstrated that median age at time of surgery was 9 days (interquartile range [IQR], 7-12) in NEC cases and 5 days (IQR, 4-9) in controls (P = .02). Conclusions: While this study is inconclusive with regard to feeding composition and risk of NEC in infants with CHD, the trend toward greater intake of breast milk in the control group suggests that breast milk may be protective for these infants. Infants with SV physiology are at high risk for NEC. Earlier time to stage I palliation may be a modifiable risk factor for NEC.
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Comparison of Blalock-Taussig-Thomas Shunts With Patent Ductus Arteriosus Ligated Versus Left Open
Published: July 23, 2024.
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Mandy W. Nasworthy; Mark Roeser; Deborah Frank; Jeffrey Vergales
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
BackgroundThe optimal approach to patent ductus arteriosus management during systemic-to-pulmonary artery shunt placement is currently unknown. The purpose of this study is to examine the outcomes of variable strategies for patent ductus arteriosus management during Blalock-Taussig-Thomas shunt surgery.MethodsA retrospective cohort study of infants who underwent shunt placement was performed, comparing those who had the ductus ligated with those who had the ductus left open. Indicators of low cardiac output syndrome, development of necrotizing enterocolitis, and secondary outcomes such as resuscitation events were examined.ResultsThirty-six infants were included all of whom had their shunt placed via median sternotomy. Twenty infants had their ductus ligated at the time of the shunt, and they were compared with 16 infants whose ductus was left open. There was no statistical difference in preoperative baseline characteristics, including corrected gestational age, age in days, weight, mechanical ventilation, vasoactive use, heterotaxy, and gastrointestinal anomalies. There was also no statistical difference in postoperative indicators of low cardiac output, including urine production, total fluids given, renal injury, maximum lactate, and vasoactive-inotropic score. Three patients had postoperative renal injury, and all were in the ligated duct group. There was also no statistical difference in any secondary outcomes, including the development of necrotizing eneterocolitis, resuscitation events, reinterventions, length of intubation, total length of stay, and mortality.ConclusionsThis study provides evidence that variable management of the ductus during shunt placement does not significantly impact outcomes. Leaving the ductus open allows for a potential rescue source of pulmonary blood flow and does not appear to increase the risk of postoperative low cardiac output.
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Atrial Septal Defect Stent Compression During Resuscitation of an Infant With Hypoplastic Left Heart Syndrome
Published: July 23, 2024.
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Chawki Elzein; Emily Pena; Narutoshi Hibino; Dhaval Patel; Luca Vricella
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
A newborn with hypoplastic left heart syndrome underwent a Norwood procedure with a Sano shunt. A month later, he had an atrial septal defect (ASD) stent placed due to ASD size and flow restriction. Three weeks later, he had sudden bradycardia and cardiac arrest in the intensive care unit. He was resuscitated and cannulated for extracorporeal membrane oxygenation (ECMO) through a right neck approach. An attempt to wean him from ECMO a week later failed due to hypoxia. Echocardiography showed ASD flow restriction and a chest x-ray showed flattening of the left atrial side of the ASD stent from chest compression resulting in a restrictive ASD. The patient underwent surgical removal of the ASD stent with ASD enlargement and was successfully weaned from ECMO after surgery.
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Truncus Arteriosus With Cardiac Type of Total Anomalous Pulmonary Venous Connection
Published: July 23, 2024.
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Neeraj Kumar; Niraj Nirmal Pandey; Sivasubramanian Ramakrishnan; Priya Jagia
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
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Neonatal Diagnosis of Berry Syndrome: Interrupted Aortic Arch, Aortopulmonary Window, Intact Ventricular Septum, and Ascending Aortic Origin of the Right Pulmonary Artery
Published: July 23, 2024.
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Nina Morgan; Arun Chandran
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
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Ebstein Anomaly: Insights on Isolated Atrial Septal Defect Device Closure
Published: July 23, 2024.
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Luciana Da Fonseca Da Silva; Mario Castro Medina; Melita Viegas
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
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Surgical Repair of Common Arterial Trunk With Ventriculoarterial Septal Defect and Dual Orifice Truncal Valve
Published: June 11, 2024.
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Hamood Al Kindi; Abdullah Mohsen; Pranav Kandachar; Abdullah Al Balushi; Alaa M. Mohamed; Madan Maddali; Justin T. Tretter; Robert H. Anderson
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
We present a case of a rare example of a ventriculo-arterial septal defect found in a patient with a common arterial trunk, with balanced aortic and pulmonary components, but with separate valvar orifices within the common truncal valve. We managed the lesion using a two-patch approach. Performing a palliative procedure to relieve the elevated right ventricular pressure aided in the preservation of the pulmonary component of the common valve. We validated the success of the technique using postoperative computerized tomography and four-dimensional flow magnetic resonance imaging.
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Standardization of the Norwood Procedure Improves Outcomes in a Medium-Sized Volume Center
Published: June 10, 2024.
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W. Hampton Gray; Robert A. Sorabella; Ashely B. Moellinger; Hayden Zaccagni; Luz A. Padilla; Borasino Santiago; Melissa Sindelar; Robert J. Dabal
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
The Norwood operation has become common practice to palliate patients with hypoplastic left heart structures. Surgical technique and postoperative care have improved; yet, there remains significant attrition prior to stage II palliation. The objective of this study is to report outcomes before and after standardizing our approach to the Norwood operation. Patients who underwent the Norwood operation at Children’s of Alabama were identified, those who underwent hybrid palliation operations were excluded. Pre- (2015-2020) and post- (2020-January 2023) standardization groups were compared and outcomes analyzed. Ninety-one patients were included (pre-standardization 44 (48.3%) and 47 (51.7%) post-standardization). There were no differences in baseline and intraoperative characteristics at Norwood between the pre- and post-standardization groups. Compared with pre-standardization, post-standardization was associated with decreased time to extubation (OR 0.87, 95%CI 0.79-0.96), inotrope duration (OR 0.92, 95%CI 0.86-0.98) and hospital length of stay (OR 0.98, 95%CI 0.96-0.99). There was a trend toward decreased cardiac arrest, reintervention rates, and interstage mortality for the post-standardization group. A standardized approach to complex neonatal cardiac operations such as the Norwood procedure may improve morbidity and decrease hospital resource utilization. We recommend establishing protocols at an institutional level to optimize outcomes in such high-risk patient populations.
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Pediatric Monitoring Technologies and Congenital Heart Disease: A Systematic Review
Published: May 29, 2024.
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Summer S. Duffy; Sharon Lee; Danielle Gottlieb Sen
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
Outpatient monitoring of infants with congenital heart disease has been shown to significantly reduce rates of mortality in the single ventricle population. Despite the accelerating development of miniaturized biosensors and electronics, and a growing market demand for at-home monitoring devices, the application of these technologies to infants and children is significantly delayed compared with the development of devices for adults. This article aims to review the current landscape of available monitoring technologies and devices for pediatric patients to describe the gap between technologies and clinical needs with the goal of progressing development of clinically and scientifically validated pediatric monitoring devices.
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Surgical Protocol for Partial Heart Transplantation in Growing Piglets
Published: May 23, 2024.
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Cathlyn K. Medina; Berk Aykut; Lillian Kang; Deani McVadon; Douglas M. Overbey; Kristi L. Helke; Carolyn L. Taylor; David C. Fitzgerald; Marc Hassid; Alicia M. Braxton; Stephen G. Miller; Corey Mealer; Chak-Sum Ho; Kristin M. Whitworth; Randall S. Prather; Mary E. Moya-Mendez; Sydney Jeffs; Lauren E. Parker; Joseph W. Turek; Taufiek Konrad Rajab
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
Partial heart transplantation is a new approach to deliver growing heart valve implants. Partial heart transplants differ from heart transplants because only the part of the heart containing the necessary heart valve is transplanted. This allows partial heart transplants to grow, similar to the valves in heart transplants. However, the transplant biology of partial heart transplantation remains unexplored. This is a critical barrier to progress of the field. Without knowledge about the specific transplant biology of partial heart transplantation, children with partial heart transplants are empirically treated like children with heart transplants because the valves in heart transplants are known to grow. In order to progress the field, an animal model for partial heart transplantation is necessary. Here, we contribute our surgical protocol for partial heart transplantation in growing piglets. All aspects of partial heart transplantation, including the donor procedure, the recipient procedure, and recipient perioperative care are described in detail. There are important nuances in the conduct of virtually all aspects of open heart surgery that differs in piglets from humans. Our surgical protocol, which is based on our experience with 34 piglets, will allow other investigators to leverage our experience to seek fundamental knowledge about the nature of partial heart transplants. This is significant because the partial heart transplant model in piglets is complex and very resource intensive.
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Hybrid Interventions for Pulmonary Vein Stenosis: Leveraging Intraoperative Endovascular Adjuncts in Challenging Clinical Scenarios
Published: May 23, 2024.
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Alyssa B. Kalustian; Paige E. Brlecic; Srinath T. Gowda; Gary E. Stapleton; Asra Khan; Lindsay F. Eilers; Ravi Birla; Michiaki Imamura; Athar M. Qureshi; Christopher A. Caldarone; Manish Bansal
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
Background: Pediatric pulmonary vein stenosis (PVS) is often progressive and treatment-refractory, requiring multiple interventions. Hybrid pulmonary vein interventions (HPVIs), involving intraoperative balloon angioplasty or stent placement, leverage surgical access and customization to optimize patency while facilitating future transcatheter procedures. We review our experience with HPVI and explore potential applications of this collaborative approach. Methods: Retrospective chart review of all HPVI cases between 2009 to 2023. Results: Ten patients with primary (n = 5) or post-repair (n = 5) PVS underwent HPVI at median age of 12.7 months (range 6.6 months-9.5 years). Concurrent surgical PVS repair was performed in 7/10 cases. Hybrid pulmonary vein intervention was performed on 17 veins, 13 (76%) with prior surgical or transcatheter intervention(s). One patient underwent intraoperative balloon angioplasty of an existing stent. In total, 18 stents (9 bare metal [5-10 mm diameter], 9 drug eluting [3.5-5 mm diameter]) were placed in 16 veins. At first angiography (median 48 days [range 7 days-2.8 years] postoperatively), 8 of 16 (50%) HPVI-stented veins developed in-stent stenosis. Two patients died from progressive PVS early in the study, one prior to planned reintervention. Median time to first pulmonary vein reintervention was 86 days (10 days-2.8 years; 8/10 patients, 13/17 veins). At median survivor follow-up of 2.2 years (2.3 months-13.1 years), 1 of 11 surviving HPVI veins were completely occluded. Conclusions: Hybrid pulmonary vein intervention represents a viable adjunct to existing PVS therapies, with promising flexibility to address limitations of surgical and transcatheter modalities. Reintervention is anticipated, necessitating evaluation of long-term benefits and durability as utilization increases.
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Scimitar Syndrome in a Pediatric Cohort
Published: May 22, 2024.
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Laia Brunet-Garcia; Flavio Zuccarino; Fredy Hermógenes Prada Martínez; Juan Manuel Carretero Bellon
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
BackgroundScimitar syndrome is a rare form of congenital heart disease (CHD) characterized by anomalous pulmonary venous drainage of the right lung to the inferior vena cava. We describe the presentation, diagnosis, therapeutic management and long-term follow-up of 10 pediatric patients with Scimitar Syndrome.MethodsWe performed a retrospective observational study of all pediatric patients from our institution with scimitar syndrome (March 1996-July 2023). Patients underwent systematic evaluation including medical and family history, chest x-ray, 12-lead electrocardiogram, echocardiogram, angiography and/or computed tomography; or magnetic resonance angiography.ResultsTen patients with scimitar syndrome were included. The median age at diagnosis was 10.4 [0.1-150.2] months and the median follow-up time was 7.7 [1.3-15.3] years. Eight patients presented with aortopulmonary collateral arteries which were embolized. Two patients had dual connections to the inferior vena cava and left atrium; embolization of the inferior vena cava connection was only feasible in one of them. No patients underwent surgery of the scimitar vein. Three patients had surgical correction of CHDs. There were no deaths related to scimitar syndrome during follow-up.ConclusionsAll patients with scimitar syndrome need prompt cardiovascular evaluation and follow-up. Our study demonstrates that a conservative approach with aortopulmonary collateral artery embolization, scimitar vein embolization when dual drainage to the left atrium is identified, along with correction of concomitant CHDs might have good results in patients with scimitar syndrome in order to postpone surgical correction of the anomalous pulmonary venous return to an older age when clinically or hemodynamically indicated. Further studies with longer-term follow-up and a larger sample size are needed to more effectively determine treatment strategy.
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Comparison of Levosimendan Versus Milrinone After the Arterial Switch Operation for Infants ≤3 kg
Published: May 20, 2024.
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Reena Khantwal Joshi; Raja Joshi; Neeraj Aggarwal; Mridul Agarwal; C Rudrappa Siddartha; Jay Relan; Anil Kumar; Manoj Modi; Parul Chug
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
Background: Various inotropes and inodilators have been utilized to treat low cardiac output syndrome after the arterial switch operation. The use of levosimendan, a calcium sensitizer has been limited in this setting. This study compares the effects of levosimendan with milrinone in managing low cardiac output after the arterial switch operation. Methods: A retrospective, comparative study was conducted in a tertiary care hospital on patients weighing up to 3 kg undergoing the arterial switch operation between January 2017 and January 2022. Patients received a loading dose followed by continuous infusion of either levosimendan or milrinone. Echocardiographic, hemodynamic and biochemical parameters were compared. Results: Forty-three patients received levosimendan and 42 patients received milrinone as the primary test drug. Cardiac index of less than 2.2 L/min/m2 on postoperative day 1 and 2 was found in 9.3% and 2.3% of patients receiving levosimendan versus 26.2% and 11.9% in those receiving milrinone, respectively (P = .04 and .08, respectively). Early lactate-clearance and better central venous oxygen saturations were noted in the levosimendan group. Prevalence of acute kidney injury was higher in the milrinone group (50% vs 28%; P = .03). Use of peritoneal dialysis in the milrinone group versus levosimendan was 31% and 16.3%, respectively (P = .11). There was no difference in hospital mortality between the groups (milrinone, 3; levosimendan, 2, P = .62). Conclusions: Levosimendan is safe and as effective as milrinone to treat low cardiac output syndrome occurring in neonates after the arterial switch operation. In addition we found that levosimendan was renal protective when compared with milrinone.
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Surgical Management of Norwood Procedure for Atypical Aortic Arch
Published: May 20, 2024.
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Shota Masaki; Yusuke Ando; Toshihide Nakano
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
Background: Postoperative restenosis of the aortic arch after the Norwood procedure is still an important complication that significantly affects surgical outcomes. The rarity of the Norwood procedure for atypical aortic morphology means appropriate arch reconstruction methods and postoperative complications are still unknown. This study aimed to assess the rate of arch reintervention and clinical outcomes after the Norwood procedure for atypical aortic arch. Methods: This retrospective single-center study was conducted between 2001 and 2022. Sixteen patients were identified, eight with a right aortic arch, five with transposition of the great arteries, one with a right aortic arch and transposition of the great arteries, and two with a large tortuous patent ductus arteriosus connected to the opposite side of the descending aorta. We selected and performed four different surgical techniques depending on each aortic arch morphology. Results: Except for one case, autologous tissue-only arch reconstruction was possible. There was one operative death and four late deaths. Overall, no patients required any surgical or catheter-based reintervention for the aortic arch. On the other hand, left pulmonary artery stenosis due to a narrow subaortic space was found in two patients. Conclusions: The Norwood procedure for atypical aortic arch was performed with good results by choosing the appropriate technique for each morphology. On the other hand, pulmonary artery stenosis is likely to occur especially in the transposition of the great arteries group. Therefore, careful surgical method selection or further improvement of the technique that allows retroaortic space should be considered.
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Pediatric Cardiac Myxoma: A Rare Cause of Stroke in a Six-Year-Old Child
Published: May 16, 2024.
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Nada Alguizzani; Maryam Alomair; Shaaban Halawa; Mohammed Aljughiman
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
This is a case report of a six-year-old child who presented with signs and symptoms of a stroke, which led to the detection of a left atrial myxoma. The myxoma was successfully removed four days after the stroke (one day after hospital transfer) and the child had nearly complete neurologic recovery.
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Advanced Imaging Guidance for Ductus Venosus Stenting in Obstructed Total Anomalous Pulmonary Venous Return
Published: May 16, 2024.
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Ernesto Mejia; Gareth J. Morgan; Matthew Stone; Michael J. Shorofsky; James Jaggers; Jenny E. Zablah
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
Obstructed infracardiac total anomalous pulmonary venous return is nearly always a surgical emergency in which infants present in severe cardiopulmonary distress. Ductal venosus stenting can provide a temporizing option for premature, low birth weight infants with high risk for surgical complications. In challenging anatomic cases, virtual reality, 3D-printed models, and fusion image guidance can aid in procedural planning and provide support for successful intervention.
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Autograft Reoperation Following the Ross Procedure: Evolving Strategies and Future Perspectives
Published: May 08, 2024.
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Tommaso Generali; Iain McPherson; Alexandru C. Visan; Amir Mohamed; Mahmoud I. Salem; Katrijn Jansen; Louise Coats; Justyna Rybicka; David Crossland; Asif Hasan; Mohamed Nassar
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
Background: Pulmonary autograft failure remains a cause of reoperation following the Ross procedure. The aim of this study is to describe our evolving approach to autograft reoperations. Methods: Retrospective study of all patients who underwent a pulmonary autograft reoperation following a Ross procedure between June 1997 and July 2022. Results: Two-hundred and thirty-five Ross procedures were performed. Thirty-six patients (15%) plus one referral underwent an autograft reoperation at a median of 7.8 years (IQR 4.6-13.6). The main indication was: neoaortic root dilatation associated with mild/moderate (n = 12) or severe (n = 8) aortic regurgitation; isolated severe aortic regurgitation (n = 6); infective endocarditis (IE) (n = 8); and aortic root pseudoaneurysm with no history of IE (n = 3). The autograft was spared in 29 cases (78%): 9 patients (24%) underwent aortic valve repair or aortic root remodeling, 15 patients (40%) aortic root reimplantation, 5 patients neoaortic root stabilization with a Personalized External Aortic Root Support (PEARS) sleeve. There were no in-hospital deaths. At a median follow up of 37 months (IQR 8-105), all patients were alive, 30 (81%) were asymptomatic. Eight patients (22%) required nine further reoperations. Estimated freedom from further reoperation was 90%, 72%, and 72% at 12-, 36-, and 60-months. Conclusions: Autograft reoperations following the Ross procedure can be safely performed and do not affect overall survival in the early and mid-term. Valve-sparing autograft replacement is technically feasible but remains at risk of further interventions. Alternative strategies, such as the PEARS sleeve, are becoming increasingly available but requires validation in the long term.
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Chylothorax in Infants and Children After Congenital Heart Surgery: Approach and Review
Published: May 06, 2024.
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Anil Kumar; Sristy Agarwal; Reena Khantwal Joshi; Arun Gupta; Siddhartha C. Rudrappa; Neeraj Aggarwal; Raja Joshi
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
Chylothorax in infants after surgery for congenital heart disease is associated with significant morbidity and mortality. Numerous management modalities applied alone or in various combinations have been utilized but definitive guidelines have not yet been established. We present two infants who developed refractory chylothorax after congenital heart surgery. We also reviewed evidence for the use of available treatment modalities. In our experience, the use of lymphangiography followed by pleurodesis by povidone-iodine was safe and our impression was that it may have played a decisive role in controlling the lymph leak.
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Heart Transplantation for Ebstein Anomaly due to Late Surgical Presentation
Published: May 02, 2024.
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Tomas Leng; Joseph A. Dearani; Elizabeth H. Stephens; Jonathan N. Johnson
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
Ebstein anomaly is a myopathy of the right ventricle characterized by failure of tricuspid valve delamination with accompanying tricuspid regurgitation. Its rarity, considerable anatomic variability, and frequent absence of symptoms can make the timing of surgery challenging. Contemporary tricuspid repair techniques can be performed with low mortality and bidirectional cavopulmonary anastomosis reduces the risk of operation when right ventricular function is poor. Here, we present a patient who presented late for surgery, failed high-risk conventional surgery and required heart transplantation.
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A Rare Combination of Cardiac Hydatid Cyst and Pulmonary Hydatidosis in a Child
Published: March 11, 2024.
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Alwaleed Al-Dairy; Eman Shhada; Nour Aldej; Islam Kraym
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
Hydatid disease is a common health problem in the sheep-farming countries of the Mediterranean, caused by infection with Echinococcus granulosus. The common sites are the liver and lungs. Cardiac hydatidosis is rare. Herein, we report a case of interventricular septum hydatid cyst with lung involvement in a four-year-old child. Cardiac hydatid cysts should be resected surgically even in asymptomatic patients since delayed diagnosis and treatment can lead to fatal outcomes. In spite of its rarity, the combination of cardiac and pulmonary hydatidosis can be managed with a single operation.
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Corrigendum
Published: May 15, 2020.
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World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
