Invited Commentary: 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.
Volume 15, Number 6
Pages 691-692
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, Volume 15, Issue 6, Page 691-692, November 2024.
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Rationale and Design of the Randomized COmparison of Methods for Pulmonary Blood Flow Augmentation: Shunt Versus Stent (COMPASS) Trial: A Pediatric Heart Network Study
Published: September 23, 2024.
Volume 15, Number 6
Pages 693-702
Contributors
Christopher J. Petit; Jennifer C. Romano; Jeffrey D. Zampi; Sara K. Pasquali; Courtney E. McCracken; Nikhil K. Chanani; Andrea S. Les; Kristin M. Burns; Allison Crosby-Thompson; Mario Stylianou; Bernet Kato; Andrew C. Glatz
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 6, Page 693-702, November 2024.
Neonates with congenital heart disease and ductal-dependent pulmonary blood flow (DD-PBF) require early intervention. Historically, this intervention was most often a surgical systemic-to-pulmonary shunt (SPS; eg, Blalock-Thomas-Taussig shunt). However, over the past two decades, an alternative to SPS has emerged in the form of transcatheter ductal artery stenting (DAS). While many reports have indicated safety and durability of the DAS approach, few studies compare outcomes between DAS and SPS. The reports that do exist are comprised primarily of small-cohort single-center reviews. Two multicenter retrospective studies suggest that DAS is associated with similar or superior survival compared with SPS. These studies offer the best evidence to-date, and yet both have important limitations. The authors describe herein the rationale and design of the COMPASS (COmparison of Methods for Pulmonary blood flow Augmentation: Shunt vs Stent [COMPASS]) Trial (NCT05268094, IDE G210212). The COMPASS Trial aims to randomize 236 neonates with DD-PBF to either DAS or SPS across approximately 27 pediatric centers in North America. The goal of this trial is to compare important clinical outcomes between DAS and SPS over the first year of life in a cohort of neonates balanced by randomization in order to assess whether one method of palliation demonstrates therapeutic superiority.
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Hybrid Interventions for Pulmonary Vein Stenosis: Leveraging Intraoperative Endovascular Adjuncts in Challenging Clinical Scenarios
Published: May 23, 2024.
Volume 15, Number 6
Pages 703-713
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, Volume 15, Issue 6, Page 703-713, November 2024.
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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Birth in the Operating Room for Immediate Cardiac Surgery: A Rare but Effective Strategy
Published: September 10, 2024.
Volume 15, Number 6
Pages 714-721
Contributors
Spencer J. Hogue; Amir Mehdizadeh-Shrifi; Kevin Kulshrestha; James F. Cnota; Allison Divanovic; Marco Ricci; Awais Ashfaq; David G. Lehenbauer; David S. Cooper; David L. S. Morales
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 6, Page 714-721, November 2024.
Background: With significant advancements in fetal cardiac imaging, patients with complex congenital heart disease (CHD) carrying a high risk for postnatal demise are now being diagnosed earlier. We sought to assess an interdisciplinary strategy for delivering these children in an operating room (OR) adjacent to a cardiac OR for immediate surgery or stabilization. Methods: All children prenatally diagnosed with CHD at risk for immediate postnatal hemodynamic instability and cardiogenic shock who were delivered in the operating room (OR) between 2012 and 2023 in which the senior author was consulted were included. Results: Eight patients were identified. Six (75%) patients were operated on day-of-life zero, all requiring obstructed total anomalous pulmonary venous return (TAPVR) repair. Of these six patients, 2 (33%) required a simultaneous Norwood procedure, 2 (33%) required pulmonary artery unifocalization and modified Blalock-Taussig-Thomas shunt, and 2 (33%) patients had repair of obstructed mixed TAPVR. The remaining 2 patients potentially planned for immediate surgery had nonimmune hydrops fetalis and went into cardiogenic shock at 12 and 72 hours postnatally, requiring a novel Norwood procedure with left-ventricular exclusion for severe aortic/mitral valve insufficiency. The median ventilation and inpatient durations were 19 [IQR: 11-26] days and 41 [IQR: 32-128] days, respectively. Three(38%) patients required one or more in-hospital reoperations. Subsequent staged procedures included Glenn (n = 5), Fontan (n = 3), biventricular repair (n = 2), ventricular assist device placement (n = 1), and heart transplant (n = 1). Median follow-up was 5.7 [IQR:1.3-7.8] years. The five-year postoperative survival was 88% (n = 7/8). Conclusion: While children with these diagnoses have historically had poor survival, the strategy of birth in the OR adjacent to a cardiac OR where emergent surgery is planned is a potentially promising strategy with excellent clinical outcomes. However, this is a high-resource strategy whose feasibility in any program requires thoughtful assessment.
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Invited Commentary: WOW! But You Have to Ask Yourself One Question (or a Few)
Published: September 12, 2024.
Volume 15, Number 6
Pages 722-723
Contributors
Scott M. Bradley
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 6, Page 722-723, November 2024.
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Impact of Atrioventricular Valve Intervention at Each Stage of Single Ventricle Palliation
Published: September 06, 2024.
Volume 15, Number 6
Pages 724-730
Contributors
John D. Vossler; Aaron W. Eckhauser; Eric R. Griffiths; Reilly D. Hobbs; Linda M. Lambert; Lloyd Y. Tani; Niharika Parsons; Robert H. Habib; Jeffrey P. Jacobs; Marshall L. Jacobs; S. Adil Husain
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 6, Page 724-730, November 2024.
Background: Significant atrioventricular valve dysfunction can be associated with mortality or need for transplant in functionally univentricular heart patients undergoing staged palliation. The purposes of this study are to characterize the impact of concomitant atrioventricular valve intervention on outcomes at each stage of single ventricle palliation and to identify risk factors associated with poor outcomes in these patients. Methods: The Society of Thoracic Surgeons Congenital Heart Surgery Database was queried for functionally univentricular heart patients undergoing single ventricle palliation from 2013 through 2022. Separate analyses were performed on cohorts corresponding to each stage of palliation (1: initial palliation; 2: superior cavopulmonary anastomosis; 3: Fontan procedure). Bivariate analysis of demographics, diagnoses, comorbidities, preoperative risk factors, operative characteristics, and outcomes with and without concomitant atrioventricular valve intervention was performed. Multiple logistic regression was used to identify predictors associated with operative mortality or major morbidity. Results: Concomitant atrioventricular valve intervention was associated with an increased risk of operative mortality or major morbidity for each cohort (cohort 1: 62% vs 46%, P < .001; cohort 2: 37% vs 19%, P < .001; cohort 3: 22% vs 14%, P < .001). Black race in cohort 1 (odds ratio [OR] 3.151, 95% CI 1.181-9.649, P = .03) and preterm birth in cohort 2 (OR 1.776, 95% CI 1.049-3.005, P = .032) were notable predictors of worse morbidity or mortality. Conclusions: Concomitant atrioventricular valve intervention is a risk factor for operative mortality or major morbidity at each stage of single ventricle palliation. Several risk factors are associated with these outcomes and may be useful in guiding decision-making.
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Breast Milk and Necrotizing Enterocolitis in Congenital Heart Disease: A Case–Control Study
Published: July 23, 2024.
Volume 15, Number 6
Pages 731-737
Contributors
Margaret R. Christian; David Bateman; Marianne Garland; Usha S. Krishnan; Emile A. Bacha; Ganga Krishnamurthy
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 6, Page 731-737, November 2024.
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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Standardization of the Norwood Procedure Improves Outcomes in a Medium-Sized Volume Center
Published: June 10, 2024.
Volume 15, Number 6
Pages 738-745
Contributors
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, Volume 15, Issue 6, Page 738-745, November 2024.
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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Revisiting the Role of Balloon Atrial Septostomy Prior to the Arterial Switch Operation
Published: August 09, 2024.
Volume 15, Number 6
Pages 746-752
Contributors
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, Volume 15, Issue 6, Page 746-752, November 2024.
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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Invited Commentary: Don’t Blame Balloon Atrial Septostomy!
Published: October 14, 2024.
Volume 15, Number 6
Pages 753-754
Contributors
Doff B. McElhinney
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 6, Page 753-754, November 2024.
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Psychological Distress and Its Associated Factors Among Parents of Children With Congenital Heart Disease: A Cross-Sectional Mixed Method Study at the National Cardiothoracic Center, Ghana
Published: August 21, 2024.
Volume 15, Number 6
Pages 755-765
Contributors
Kow Entsua-Mensah; John Tetteh; George Ekem-Ferguson; Swithin M. Swaray; Innocent I. Adzamli; Nana-Akyaa Yao; Mark M. Tettey
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 6, Page 755-765, November 2024.
BackgroundThe aim of this study was to examine the prevalence and factors associated with psychological distress among parents of children with congenital heart disease (PCCHD) receiving treatment at the National Cardiothoracic Centre (NCTC), Accra.MethodsA cross-sectional mixed method study involving 248 parents of children with congenital heart disese receiving treatment at the NCTC was employed. Systematic sampling was used to select participants. The outcome variables were depression, anxiety, and somatization, assessed using the brief symptom inventory. A semistructured interview guide was used to understand the contextual factors that contribute to parents’ psychological distress. Poisson regression and inductive thematic analyses were employed for quantitative and qualitative data, respectively.ResultsThe prevalence of depression, anxiety, and somatization was 42.9% [95%CI = 36.7-49.3], 42.4% [95%CI = 36.3-48.9], and 41.2% [95%CI = 35.1-47.6] respectively. Parents who were divorced [aPR (95%CI) = 1.60(1.02-2.51)], hadonly basic level education [aPR (95%CI) = 2.13(1.34-3.39)], were without a religious affiliation [aPR (95%CI)= 2.24(1.58-3.19)], and in the low asset quintile significantly increased the likelihood of psychological distress. Delay/postponement of scheduled surgery, worsening state of child’s condition, feeling a sense of helplessness, societal stigma, and lack of funding or health insurance for the treatment of the condition were contextual factors that contributed to increased psychological distress among parents.ConclusionThe prevalence of psychological distress was quite high among parents of children with congenital heart disease in Ghana and was associated with both parent and patient factors. Psychosocial care should be integrated into the clinical health services of this group of patients and their parents to improve psychological health outcomes.
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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.
Volume 15, Number 6
Pages 766-773
Contributors
A Moodley; HM Meyer; S Salie; P Human; L J Zühlke; A Brooks
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 6, Page 766-773, November 2024.
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 Following Neonatal Cardiac Surgery in Cape Town, South Africa
Published: August 29, 2024.
Volume 15, Number 6
Pages 774-782
Contributors
Derrik du Toit; Lenise C. Swanson; Shamiel Salie; Susan Perkins; Wisdom Basera; John B. Lawrenson; Thomas Aldersley; Andre Brooks; Liesl J. Zühlke
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 6, Page 774-782, November 2024.
BackgroundNeonatal Cardiac Surgery has developed significantly since its advent, with improved outcomes, survival, and physiological repair. Limited programs offer neonatal cardiac surgery in emerging economies. We report our experience with neonates undergoing cardiac surgery in our cardiac surgery program.MethodsWe performed a secondary data analysis on all neonates aged ≤ 30 days undergoing congenital cardiac surgery from April 1, 2017 to March 31, 2020, including outcomes up to 30-days post-surgery.ResultsA total of 859 patients underwent cardiac surgery at our center, of these 81 (9.4%) were neonates. The proportion of neonates increased annually (8.7%, 9.6%, and 10.2%). There were 49 (60%) male patients, and 32 (40%) had surgery in the second week of life. Fourteen (17%) were premature, four (5%) had a major chromosomal abnormality, five (6%) a major medical illness, and eight (10%) a major noncardiac structural anomaly. The Risk Adjustment for Congenital Heart Surgery (RACHS) categorization of surgery was predominantly RACHS 3; n = 28 (35%) and 4; n = 23 (29%). Hours in the intensive care unit (ICU) were extensive; median 189 [interquartile range (IQR): 114-286] as were hours of ventilation; median 95 [IQR 45-163]. Almost 60% (n = 48) of procedures were complicated by sepsis, as defined in our database. The in-hospital mortality rate was 16% (n = 13); the 30-day mortality rate was 19.8% (n = 16).ConclusionThe proportion of neonates in our service increased over the period. Focused strategies to shorten prolonged ICU stay and decrease rates of bacterial sepsis in neonates are needed. A multidisciplinary, collaborative heart-team approach is crucial for best outcomes.
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Outcomes of Patients Undergoing the Kawashima Procedure at an Early Age: A Single Center Experience
Published: July 23, 2024.
Volume 15, Number 6
Pages 783-788
Contributors
Brian P. Bateson; Matthew Files; Lyubomyr Bohuta
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 6, Page 783-788, November 2024.
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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Durability of Aortic Homografts in Pulmonary Atresia and Major Aortopulmonary Collateral Arteries
Published: August 21, 2024.
Volume 15, Number 6
Pages 789-800
Contributors
Daniel K. Ragheb; Elisabeth Martin; Yulin Zhang; Ayush Jaggi; Ritu Asija; Lynn F. Peng; Michael Ma; Frank L. Hanley; Doff B. McElhinney
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 6, Page 789-800, November 2024.
Objectives: It is well-known that right ventricle-to-pulmonary artery homograft conduit durability is worse for smaller conduits and smaller/younger patients. However, there is limited literature on age and conduit-size specific outcomes, or on the role of conduit oversizing. Methods: Patients diagnosed with tetralogy of Fallot and major aortopulmonary collateral arteries undergoing right ventricular outflow tract (RVOT) reconstruction with a valved aortic homograft conduit from November 2001 through March 2023, at our institution were included. Conduits were grouped and evaluated by diameter, diameter Z-score, and patient age at implant. The primary time-related outcome was freedom from RVOT reintervention. Factors associated with freedom from time-related outcomes were assessed with univariable Cox regression analysis. Results: A total of 863 RVOT conduits were implanted in 722 patients. On multivariable analysis, younger age, male sex, Alagille syndrome, smaller diameter of the conduit, and smaller Z-score were associated with shorter freedom from reintervention. Among patients with smaller diameter conduits, larger Z-scores were associated with longer freedom from conduit reintervention (P < .001). Transcatheter interventions were commonly used to extend conduit lifespan across ages and conduit sizes. Conclusions: Larger conduit diameter, older age, and higher conduit Z-score were associated with longer freedom from reintervention in patients undergoing RVOT reconstruction in this cohort. Oversizing of conduits, even beyond a Z-score of 4, is generally appropriate.
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Ross Confers More Favorable Left Ventricular Remodeling Compared With Mechanical Aortic Valve Replacement
Published: August 28, 2024.
Volume 15, Number 6
Pages 801-805
Contributors
Garrett H. Markham; John W. Brown; Chelsea D. Wenos; Morten O. Jensen; Hanna K. Jensen; Larry W. Markham; Jeremy L. Herrmann
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 6, Page 801-805, November 2024.
Background: Aortic valve disease results in left ventricular (LV) dilation and/or hypertrophy. Valve intervention may improve, but not normalize flow dynamics. We hypothesized that LV remodeling would be more favorable following the Ross procedure versus mechanical aortic valve replacement (mAVR). Methods: Patients who were 18 to 50 years of age and underwent Ross or mAVR from 2000 to 2016 at a single institution were retrospectively reviewed. Propensity score matching was performed and yielded 27 well-matched pairs. Demographics and echocardiographic variables of LV morphology and wall thickness were collected. Those with > mild residual valve disease were excluded. Primary endpoints included LV morphology. T test and Fisher exact test analysis were used for statistical comparison. Results: Average age at operation (Ross 35.3 ± 10.2 vs mAVR 37.3 ± 8.9 years) did not differ. Indication for operation was similar between groups. Preoperative echocardiographic variables did not differ. At average follow-up duration (Ross 7.9 ± 2.4 vs mAVR 7.3 ± 2.4 years), wall thickness was significantly smaller for Ross compared with mAVR (P = .00715). Only 4/27 (15%) of mAVR patients had normalized LV parameters compared with 16/27 (59%) of Ross patients (P = .000813). Residual hypertrophy was the most common long-term abnormality for mAVR. Conclusion: Following aortic valve replacement with the Ross procedure or mechanical aortic valve prosthesis, the Ross conferred more favorable LV remodeling compared with mAVR. Future directions include analyzing longer follow-up to determine if patterns persist and the impact on cardiac morbidity and mortality.
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The Clinical Outcomes in Patients With Atrial Isomerism Undergoing Single Ventricular Palliation: Insights From A Single-Center Study in Thailand
Published: August 28, 2024.
Volume 15, Number 6
Pages 806-814
Contributors
Tanawat Wicheannarat; Paweena Chungsomprasong; Prakul Chanthong; Chodchanok Vijarnsorn; Supaluck Kanjanauthai; Thita Pacharapakornpong; Ploy Thammasate; Kritvikrom Durongpisitkul; Jarupim Soongswang; Teerapong Tocharoenchok; Ekarat Nitiyarom; Kriangkrai Tantiwongkosri; Thaworn Subtaweesin
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 6, Page 806-814, November 2024.
Background: The management of atrial isomerism across various countries may impact survival outcomes. Methods: This retrospective study involved patients diagnosed with atrial isomerism undergoing single ventricular palliation between 2000 and 2021. The objective was to evaluate survival outcomes within the right atrial isomerism (RAI) and left atrial isomerism (LAI) groups, as well as overall survival. Results: Of the 125 patients diagnosed with atrial isomerism, 105 (84%) had RAI, and 20 (16%) had LAI. The median age at presentation was 3 days (range: birth to 7.1 years), with median follow-up of 6.6 years (range: 0.59 months to 30.8 years). In the overall cohort, survival rates at one, five, and ten years were 85.6%, 72.3%, and 66.8%, respectively, with no statistically significant difference between RAI and LAI groups (log rank P value = .293). Specifically, survival rates in the RAI group at one, five, and ten years were 83.8%, 69.0%, and 62.6%, respectively, while in the LAI group, there were 95.0%, 89.7%, and 81.6%, respectively. Following the third-stage operation, overall survival rates at one and five years were 76.4% and 76.4%, respectively. Among patients in the RAI group, survival rates at one and five years post-third stage operation were 80.2% and 80.2%, respectively. Conclusions: Despite resource limitations, the survival outcomes of patients with atrial isomerism were found to be similar with those observed in high-income countries. Although an increased mortality rate was noted within the first year of life, focusing efforts on neonatal and infant care holds potential for improving overall outcomes.
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Short-Term Results With Ozaki Valved Conduit—A Simple Solution for Patients Needing Right Ventricle to Pulmonary Artery Conduit in a Low-Resource Setting
Published: September 10, 2024.
Volume 15, Number 6
Pages 815-822
Contributors
Vijayakumar Raju; Christopher W. Baird; Naveen Srinivasan; Divya Kadavanoor Sasikumar; Rajalakshmi Moorthy; Koushik Jothinath; Sreja Gangadharan; Kalyanasundaram Muthu Swamy; Aparna Vijaya Raghavan; Mani Ram Krishna; Pavithra Ram Nath
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 6, Page 815-822, November 2024.
BackgroundThe repair of certain types of complex congenital cardiac defects may require a right ventricle-pulmonary artery (RV-PA) conduit. Using the Ozaki Aortic valve neocuspidization (AVNeo)technique, a valved RV-PA conduit was constructed with an Ozaki valve inside a Dacron graft. This study aims to evaluate the short-term outcome of the Ozaki valved RV-PA conduit.Material/MethodA total of 22 patients received the Ozaki valved RV-PA conduit from November 2019 until December 2023. The median age was 12 years (interquartile range [IQR], 5.5-21), median body weight was 35 kg (IQR, 15.8-48.5). The conduit was used in 16 patients (72.7%) under 18 years of age. Indications for conduit placement included: anatomic repair of corrected transposition of the great arteries, ventricular septal defect/pulmonary stenosis, conduit replacement, pulmonary atresia with associated anomalies, pulmonary artery aneurysm with dysplastic pulmonary valve, tetralogy of Fallot with coronary artery crossing the right ventricular outflow tract, bioprosthetic pulmonary valve regurgitation, and rheumatic heart disease. Native pericardium was used for the Ozaki valve in 12 patients and bovine pericardium for 10 patients. Conduit sizes ranged from 18 mm to 30 mm.ResultThe median intensive care unit stay was 4 (IQR, 2-6) days and the median hospital stay was 9 (IQR, 5.5-13.5) days. There were two perioperative mortalities (9.1%) both unrelated to the conduit. The median follow-up was 12.3 (IQR, 4.43-21.2) months. There was no infective endocarditis of the conduit. The median peak gradient across the conduit was 22 mm Hg (range 0-44 mm), and all were competent with trivial regurgitation on follow up.ConclusionCreation of an Ozaki valved conduit is an attractive option due to low cost, reproducibility, and excellent hemodynamics. Longer-term studies are needed to confirm the durability.
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The Pediatric Heart Center Melting Pot: Sharing Recipes for Success: Proceedings from the 8th World Congress of Pediatric Cardiology and Cardiac Surgery
Published: August 21, 2024.
Volume 15, Number 6
Pages 823-832
Contributors
Sarah A. Teele; Liz Crowe; Joshua Koch; Uri Pollak; Emile Bacha; Michael P Mulreany; Stephen Trice; Christine M. Riley; David S. Winlaw; Joseph W. May; Rashmin C. Savani; Gil Wernovsky
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 6, Page 823-832, November 2024.
The challenges of present-day healthcare are urgent; there is a shortage of clinicians, patient care is increasingly complex, resources are limited, clinician turnover seems ever-increasing, and the expectations of providers and patients are monumental. To transform problems into innovative opportunities, diverse perspectives and a sense of possibility are needed. The following is a collaborative manuscript authored by the speakers of the 8th World Congress of Pediatric Cardiology and Cardiac Surgery session, “Teamwork, Culture Change, and Strategy.” Although this panel was diverse in the clinical roles, nationalities, and genders represented, several consistent themes emerged which are explored in this work.
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Predictors of Neurologic Complications and Neurodevelopmental Outcome in Pediatric Cardiac Surgery With Extracorporeal Membrane Oxygenation
Published: September 05, 2024.
Volume 15, Number 6
Pages 833-843
Contributors
Peter Pastuszko; Michael G. Katz; Shana Ravvin; Tain-Yen Hsia; G. Praveen Raju; Vishal Nigam; Richard D. Mainwaring
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 6, Page 833-843, November 2024.
Extracorporeal membrane oxygenation (ECMO) has emerged as an important intervention for children both preceding and following cardiac surgery. There is a notable lack of comprehensive information regarding neurodevelopmental outcomes. The Norwood procedure and complex biventricular repairs exhibit the highest prevalence of ECMO usage. Examination of the data demonstrates that only 50% of ECMO survivors achieved normative cognitive outcomes, with 40% of those experiencing long-term neurological deficits. It is imperative to conduct robustly designed studies with extended follow-up periods to establish guidelines for neuromonitoring and neuroprotection during ECMO in the field of congenital cardiac surgery.
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Dual Decompression of the Left Atrium in Mitral Atresia With Intact Atrial Septum
Published: August 29, 2024.
Volume 15, Number 6
Pages 844-845
Contributors
Aprateem Mukherjee; Niraj Nirmal Pandey; Lamk Kadiyani; Sivasubramanian Ramakrishnan; Priya Jagia
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 6, Page 844-845, November 2024.
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A Rare Combination of Cardiac Hydatid Cyst and Pulmonary Hydatidosis in a Child
Published: March 11, 2024.
Volume 15, Number 6
Pages 846-848
Contributors
Alwaleed Al-Dairy; Eman Shhada; Nour Aldej; Islam Kraym
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 6, Page 846-848, November 2024.
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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A Simplified Staged Left Ventricular Recruitment Strategy to Achieve Biventricular Repair in a Neonate With Hypoplastic Left Heart Variant
Published: August 22, 2024.
Volume 15, Number 6
Pages 848-851
Contributors
Ranjit Philip; Jason Johnson; Ronak J. Naik; Adegbemisola A. Perkins; Shyam Sathanandam; Christopher J. Knott-Craig; Umar Boston
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 6, Page 848-851, November 2024.
Hypoplastic left heart complex presents a diverse spectrum of variants. Traditional management has been dichotomous, involving either single ventricle palliation or high-risk biventricular repair. Surgical approaches to achieve biventricular repair in children with borderline left ventricle are continually evolving. One such method is the staged left ventricular recruitment (SLVR) strategy. Here, we illustrate a unique surgical approach to SLVR, achieving biventricular repair over 22 months in a neonate with hypoplastic left heart variant.
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An Unusual Culprit: Syncope in an Adolescent With Congenital Left Main Coronary Artery Atresia
Published: September 19, 2024.
Volume 15, Number 6
Pages 851-855
Contributors
Charlie J. Sang; Audrey Khoury; Michael Yeung; Thomas G. Caranasos; Elman G. Frantz
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 6, Page 851-855, November 2024.
There are fewer than 100 reported cases of congenital left main coronary artery atresia. In this report, we present an adolescent male presenting with exertional syncope in the setting of this rare coronary defect, and review important diagnostic and therapeutic considerations imperative to obtain a favorable outcome.
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Surgical Repair of Total Anomalous Pulmonary Venous Connection into the Superior Vena Cava: A Case Report
Published: August 22, 2024.
Volume 15, Number 6
Pages 856-858
Contributors
Alwaleed Al-Dairy; Batoul Ali; Ayah Aldagher
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 6, Page 856-858, November 2024.
Total anomalous pulmonary venous connection is a rare congenital anomaly that has four anatomical subtypes. In the supracardiac type, the common pulmonary vein confluence usually drains into the left innominate vein via a vertical vein; however, it may drain into the superior vena cava. Herein, we present a successful surgical repair of a rare type of total anomalous pulmonary venous connection in which the common pulmonary vein confluence was draining directly into the superior vena cava without a vertical vein.
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Unintended Sequalae of Long-Term Transvenous Pacemaker Leads in a Patient With Congenital Heart Disease
Published: September 05, 2024.
Volume 15, Number 6
Pages 858-861
Contributors
Soyoung Cheon; Muhammad Faateh; Awais Ashfaq
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 6, Page 858-861, November 2024.
Long-term use of transvenous pacemakers, a common modality used for symptomatic bradyarrhythmias, can lead to rare but significant complications, especially in patients with congenital heart disease. We present a complex and challenging surgical lead extraction for a patient with long-standing superior vena cava syndrome resulting from prolonged transvenous leads.
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Surgical Repair of Tetralogy of Fallot Using Autologous Right Atrial Appendage to Reconstruct the Right Ventricular Outflow Tract: A Case Series With Four-Year Follow-up
Published: September 05, 2024.
Volume 15, Number 6
Pages 861-863
Contributors
Oleg Anatolyevich Egunov; Roman Sergeevich Kozhanov; Frank Cetta; Evgeny Vladimirovich Krivoshchekov
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 6, Page 861-863, November 2024.
This clinical case series describes the surgical management of tetralogy of Fallot in five children using autologous right atrial appendage to reconstruct the right ventricular outflow tract. Transthoracic echocardiography performed at four-year follow-up showed a mild residual gradient and moderate regurgitation of the neopulmonary valve in all cases.
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Double-Outlet Right Ventricle With Intact Ventricular Septum and Left Atrioventricular Valve Regurgitation in a Patient With Right Atrial Isomerism
Published: September 05, 2024.
Volume 15, Number 6
Pages 864-866
Contributors
Tomohiro Nakata; Maiko Tachi; Kenji Yasuda; Shigeki Nakashima; Toshiko Minamoto; Kazuhiro Yamazaki
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 6, Page 864-866, November 2024.
We present a very rare case of right atrial isomerism, double-outlet right ventricle, and incomplete atrioventricular septal defect (intact ventricular septum). In the neonatal period, the right ventricle was compressed by a “blind-ended” left ventricle with mild-to-moderate left atrioventricular valve regurgitation. The regurgitation gradually decreased from mild-to-moderate to mild with body weight gain. The patient underwent systemic-to-pulmonary shunt at three months of age and bilateral bidirectional Glenn at eight months of age. Although the echocardiogram demonstrated that the right ventricle was still compressed by the left ventricle, over time the size of the left ventricle reduced significantly and the left atrioventricular valve regurgitation became trivial.
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Book Review: Finding the Way: A Memoir
Published: October 14, 2024.
Volume 15, Number 6
Pages 867-868
Contributors
Carl L Backer
World Journal for Pediatric and Congenital Heart Surgery, Volume 15, Issue 6, Page 867-868, November 2024.
