Proceedings of the 8th Scientific Meeting of the World Society for Pediatric and Congenital Heart Surgery (WSPCHS) at the 8th World Congress of Pediatric Cardiology and Cardiac Surgery (WCPCCS) 
Published: May 16, 2024.
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Contributors
Carl L. Backer; Jeffrey P. Jacobs
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 3, Page 263-264, May 2024.
Invited Commentary: One Home Run Is Much Better Than Two Doubles
Published: May 16, 2024.
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Contributors
Bahaaldin Alsoufi
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 3, Page 277-278, May 2024.
Total Anomalous Pulmonary Venous Connection in Adults: Should We Offer Surgery?
Published: April 24, 2024.
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Contributors
Vivek Bagaria; Badragiri Lahari; B. R. Jagannath; C. S. Hiremath
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 3, Page 319-324, May 2024.
Background: We aim to determine the surgical outcomes of adult patients with total anomalous pulmonary venous connection (TAPVC) and examine the regression of pulmonary artery (PA) pressures after the procedure. Methods: We reviewed the hospital records from 2003 to 2022 and identified 49 adult patients with TAPVC. We assessed their surgical outcomes and the trend of PA pressures after the procedure. Continuous data are presented as mean ± SD or median (interquartile range) and categorical variables are presented as percentages. Results: The median age of the patients was 23 years (range 18-42) and 31 (63.3%) were male. Thirty-six patients (73.5%) had supracardiac TAPVC. The mean systolic PA pressure was 65.8 ± 16.4 mm Hg and it decreased by 47.9% (34%, 61.8%) after surgery. Moderate or more tricuspid regurgitation was seen in 27 (55.1%) patients before surgery; however, it was present in only 3 (6.1%) patients during early follow-up. There was no intraoperative or 30-day mortality, and the median hospital length of stay was six days. Long-term follow-up data were available for 29 patients with the average duration of follow-up being 5.6 years (range 6 months to 15 years) and the mean systolic PA pressures of this cohort was 29.8±7.9 mm Hg. Forty-six (93.1%) patients were asymptomatic; four women had uneventful pregnancies and delivered healthy children. Conclusion: Surgical repair of the naturally selected group of adult TAPVC patients can be performed safely with good results. Regression in flow-related pulmonary hypertension and an improvement in functional quality of life are seen in nearly all patients.
Longitudinal Evaluation of Congenital Cardiovascular Surgical Performance and Skills Retention Using Silicone-Molded Heart Models
Published: April 22, 2024.
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Contributors
Matteo Ponzoni; Rawan Alamri; Brandon Peel; Christoph Haller; John Coles; Rachel D. Vanderlaan; Osami Honjo; David J. Barron; Shi-Joon Yoo
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 3, Page 332-339, May 2024.
Objective: Hands-on surgical training (HOST) for congenital heart surgery (CHS), utilizing silicone-molded models created from 3D-printing of patients’ imaging data, was shown to improve surgical skills. However, the impact of repetition and frequency of repetition in retaining skills has not been previously investigated. We aimed to longitudinally evaluate the outcome for HOST on two example procedures of different technical difficulties with repeated attempts over a 15-week period. Methods: Five CHS trainees were prospectively recruited. Repair of coarctation of the aorta (CoA) and arterial switch operation (ASO) were selected as example procedures of relatively low and high technical difficulty. Procedural time and technical performance (using procedure-specific assessment tools by the participant, a peer-reviewer, and the proctor) were measured. Results: Coarctation repair performance scores improved after the first repetition but remained unchanged at the follow-up session. Likewise, CoA procedural time showed an early reduction but then remained stable (mean [standard deviation]: 29[14] vs 25[15] vs 23[9] min at 0, 1, and 4 weeks). Conversely, ASO performance scores improved during the first repetitions, but decreased after a longer time delay (>9 weeks). Arterial switch operation procedural time showed modest improvements across simulations but significantly reduced from the first to the last attempt: 119[20] versus 106[28] min at 0 and 15 weeks, P = .049. Conclusions: Complex procedures require multiple HOST repetitions, without excessive time delay to maintain long-term skills improvement. Conversely, a single session may be planned for simple procedures to achieve satisfactory medium-term results. Importantly, a consistent reduction in procedural times was recorded, supporting increased surgical efficiency.
Aneurysms of Aortic Sinus of Valsalva Dissecting through the Interventricular Septum With Rupture into the Left Ventricle: Case Series and Literature Review
Published: April 22, 2024.
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Contributors
Mrinal Patel; Kartik Patel; Trushar Gajjar; Amit Mishra
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 3, Page 340-348, May 2024.
Aneurysm of Aortic sinus of Valsalva (ASOV) dissecting into the interventricular septum (IVS) and rupturing into the left ventricle (LV) is a rare clinical diagnosis. Systemic inflammatory diseases like tuberculosis can aggravate this condition. We describe three cases of ASOV dissecting into the IVS and rupturing into the LV. All three patients underwent surgical intervention; two had a successful outcome. A literature review was conducted and19 previously reported cases were studied. The extent and direction of septal dissection determined the associated cardiac valvular and rhythm problems. Patch closure of the mouth of the aneurysm is the surgical method of choice. In the presence of multiple sinus tracts or if there is recurrence after surgical closure, aortic sinus or root replacement techniques have better outcomes.
Invited Commentary: Residual Regurgitation on Intraoperative Echocardiogram After Mitral Valve Repair: Could This Technique be a Useful Addition to Improve the Durability of Repair?
Published: May 16, 2024.
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Contributors
Kirsten Finucane
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 3, Page 363-364, May 2024.
Mentoring Pediatric Heart Surgeons: A Difficult Road for Both Mentors and Mentees
Published: May 23, 2024.
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Contributors
Emile Bacha
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
Surgical Protocol for Partial Heart Transplantation in Growing Piglets
Published: May 23, 2024.
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Contributors
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.
Hybrid Interventions for Pulmonary Vein Stenosis: Leveraging Intraoperative Endovascular Adjuncts in Challenging Clinical Scenarios
Published: May 23, 2024.
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Contributors
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.
Scimitar Syndrome in a Pediatric Cohort
Published: May 22, 2024.
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Contributors
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.
Dual Drainage Total Anomalous Pulmonary Venous Connection With Infracardiac and Supracardiac Drainage
Published: May 22, 2024.
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Contributors
Nabeel Valappil Faisal; Suad Akhtar; Niraj Nirmal Pandey; Saurabh Kumar Gupta
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
Comparison of Levosimendan Versus Milrinone After the Arterial Switch Operation for Infants ≤3 kg
Published: May 20, 2024.
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Contributors
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.
Surgical Management of Norwood Procedure for Atypical Aortic Arch
Published: May 20, 2024.
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Contributors
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.
Gerbode Defect—Multimodality Imaging
Published: May 16, 2024.
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Contributors
M Kardos; J Polakova Mistinova
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
Pediatric Cardiac Myxoma: A Rare Cause of Stroke in a Six-Year-Old Child
Published: May 16, 2024.
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Contributors
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.
Excellent Survival With a Focus on Pulmonary Artery Reinterventions After the Arterial Switch Operation
Published: May 16, 2024.
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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, Ahead of Print.
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.
Advanced Imaging Guidance for Ductus Venosus Stenting in Obstructed Total Anomalous Pulmonary Venous Return
Published: May 16, 2024.
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Contributors
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.
Complex Atrial Baffling Procedures in Left Isomerism With Right- and Left-Hand Topology
Published: May 08, 2024.
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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, Ahead of Print.
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.
Autograft Reoperation Following the Ross Procedure: Evolving Strategies and Future Perspectives
Published: May 08, 2024.
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Contributors
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.
Eleven Years After Scimitar Syndrome Repair With the Lugones Technique: Usefulness of Four-Dimensional Flow Magnetic Resonance Imaging
Published: May 07, 2024.
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Contributors
Idara Zezular; Lisa Carlson Hanse; Hyon Ju Choe; Damian Craiem; Ignacio Lugones
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
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.
Atrial Septal Defect Closure: Not Always Straightforward
Published: May 06, 2024.
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Contributors
W. Jack Wallen; Carl L. Backer
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
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.
Chylothorax in Infants and Children After Congenital Heart Surgery: Approach and Review
Published: May 06, 2024.
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Contributors
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.
Heart Transplantation for Ebstein Anomaly due to Late Surgical Presentation
Published: May 02, 2024.
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Contributors
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.
Uhl’s Anomaly in Adulthood
Published: May 02, 2024.
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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, Ahead of Print.
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.
Anomalous Left Coronary Artery From the Pulmonary Artery With Ventricular Septal Defect and Double Aortic Arch: A Unique Case Report
Published: May 02, 2024.
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Contributors
Fadi Sabateen; Matej Nosáľ; Pavel Valentík; Lucia Bobík; Vladimír Soják
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
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.
Comparison of Right Ventricular Outflow Tract Reconstruction Techniques on Mid-Term Pulmonic Valve Fate
Published: April 27, 2024.
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Contributors
Noppon Taksaudom; Pradchaya Thuropathum; Thitipong Tepsuwan; Apichat Tantraworasin; Rekwan Sittiwangkul; Amarit Phothikun; Surin Woragidpoonpol
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
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.
An Unusual Presentation of Double-Outlet Right Atrium
Published: April 27, 2024.
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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, Ahead of Print.
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.
Hypoplastic Circumflex Aorta With Anomalous Left Pulmonary Artery: Uncrossing Operation in a Neonate
Published: April 24, 2024.
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Contributors
Qais Alloah; Nilanjana Misra; Elena Kwon; Simone Jhaveri; Hari Rajagopal; Christopher Gasparis; David B. Meyer
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
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.
Operative Repair of Aortopulmonary Window: A 25-Year Experience
Published: April 22, 2024.
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Contributors
Alyssa B. Kalustian; Richard C. Tang; Michiaki Imamura
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
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.
Early Impairment of Cerebral Bioenergetics After Cardiopulmonary Bypass in Neonatal Swine
Published: April 22, 2024.
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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, Ahead of Print.
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.
A Case of Persistent Junctional Reciprocating Tachycardia Coincident With Cor Triatriatum Sinister
Published: April 22, 2024.
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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, Ahead of Print.
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.
Current Models for Congenital Heart Center Structure and Health System Integration
Published: April 22, 2024.
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Contributors
Sara K. Pasquali
World Journal for Pediatric and Congenital Heart Surgery, Ahead of Print.
