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OBJECTIVES: Pulmonary arterial hypertension (PAH) is a devastating complication of pediatric congenital heart disease (CHD). A recent study has identified the protein high mobility group box-1 (HMGB1) as a diagnostic tool in adults with CHD-associated PAH. HMGB1 levels in adults with CHD-associated PAH correlated with mean pulmonary artery pressure and pulmonary vascular resistance, and HGMB1 levels fell in response to sildenafil therapy. We wanted to assess if HGMB1 was a biomarker of pediatric CHD-PAH. DESIGN: Prospective cohort study. SETTING: Quaternary pediatric academic hospital PARTICIPANTS: Children ≤18 years with CHD with and without known pulmonary hypertension. Controls were children undergoing dental or urologic surgery with no known heart disease. INTERVENTIONS: Pulmonary hemodynamics, echocardiographic assessment, and biomarker measurement. Controls had biomarker measurement only. MEASUREMENTS AND MAIN RESULTS: Patients with CHD-PAH had mean pulmonary vascular resistance index of 10 Wood units/m2. Neither HGMB1 nor N-terminal pro-brain-type natriuretic peptide levels were significantly different between the groups. Neither marker correlated with pulmonary hypertension. CONCLUSIONS: Unlike in adults, HGMB1 is not a biomarker of PAH in pediatric CHD. Further work will continue to explore for biomarkers for this high-risk population.
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BACKGROUND: The Melody valve (Medtronic, Minneapolis, MN) for mitral valve replacement (MVR) (MelodyMVR) has been an effective strategy to treat unrepairable mitral valve disease in small children. This study analyzed survival, durability, and complications of the MelodyMVR strategy. METHODS: Patients who underwent MelodyMVR between 2014 and 2023 were included. Transplant-free survival was analyzed with Kaplan-Meier analysis. The Fine and Gray subdistribution method was applied to quantify the cumulative incidence. RESULTS: Twenty-five patients underwent MelodyMVR. Median age and weight were 6.3 months (interquartile range, 4.4-15.2 months) and 6.36 kg (interquartile range, 4.41-7.57 kg). Fifteen patients (60%) had congenital mitral valve disease and 13 (52%) had dominant mitral regurgitation. The median diameter of the implanted Melody was 16 mm (interquartile range, 14-18 mm). Mortality at 6 months, 1 year, and 5 years was 8.3% (95% CI, 2.2%-29.4%), 12.5% (95% CI, 4.2%-33.9%), and 17.6% (95% CI, 7.0%-40.7%), respectively. Two hospital survivors (8%) required early Melody replacement. Competing risk analysis showed that â¼50% of patients underwent mechanical MVR by 3.5 years after MelodyMVR. Freedom from bleeding and thrombosis at 4 years was 87.5% (95% CI, 74.2%-100%). Eleven patients underwent subsequent mechanical MVR with no deaths. One (9%) required pacemaker implantation after mechanical MVR. CONCLUSIONS: MelodyMVR provides reasonable early and medium-term survival in small children and a high rate of successful bridge to mechanical MVR. MelodyMVR is associated with minimal pacemaker requirement, bleeding, and thrombosis. Early Melody functional deterioration necessitates early repeat MVR, which can be achieved with minimal morbidity and mortality.
Subject(s)
Heart Valve Prosthesis Implantation , Heart Valve Prosthesis , Mitral Valve , Humans , Male , Female , Infant , Mitral Valve/surgery , Retrospective Studies , Heart Valve Prosthesis Implantation/methods , Prosthesis Design , Mitral Valve Insufficiency/surgery , Treatment Outcome , Postoperative Complications/epidemiology , Heart Valve Diseases/surgery , Survival Rate/trends , Follow-Up StudiesABSTRACT
Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease. Palliative procedures, either surgical or transcatheter, aim to improve oxygen saturation, affording definitive procedures at a later stage. Transcatheter interventions have been used before and after surgical palliative or definitive repair in children and adults. This review aims to provide an overview of the different catheter-based interventions for TOF across all age groups, with an emphasis on palliative interventions, such as patent arterial duct stenting, right ventricular outflow tract stenting, or balloon pulmonary valvuloplasty in infants and children and transcatheter pulmonary valve replacement in adults with repaired TOF, including the available options for a large, dilated native right ventricular outflow tract.
Subject(s)
Balloon Valvuloplasty , Cardiac Catheterization , Heart Valve Prosthesis Implantation , Palliative Care , Stents , Tetralogy of Fallot , Humans , Tetralogy of Fallot/surgery , Tetralogy of Fallot/diagnostic imaging , Tetralogy of Fallot/physiopathology , Cardiac Catheterization/instrumentation , Cardiac Catheterization/adverse effects , Infant , Treatment Outcome , Age Factors , Child, Preschool , Child , Adult , Heart Valve Prosthesis Implantation/instrumentation , Heart Valve Prosthesis Implantation/adverse effects , Adolescent , Infant, Newborn , Young Adult , Cardiac Surgical Procedures/adverse effects , Risk Factors , Female , Pulmonary Valve/diagnostic imaging , Pulmonary Valve/surgery , Pulmonary Valve/physiopathology , Male , Hemodynamics , Middle Aged , Recovery of FunctionABSTRACT
OBJECTIVE: Machine learning (ML) can facilitate prediction of major adverse cardiovascular events (MACEs) in repaired tetralogy of Fallot (rTOF). We sought to determine the incremental value of ML above expert clinical judgement for risk prediction in rTOF. METHODS: Adult congenital heart disease (ACHD) clinicians (≥10 years of experience) participated (one cardiac surgeon and four cardiologists (two paediatric and two adult cardiology trained) with expertise in heart failure (HF), electrophysiology, imaging and intervention). Clinicians identified 10 high-yield variables for 5-year MACE prediction (defined as a composite of mortality, resuscitated sudden death, sustained ventricular tachycardia and HF). Risk for MACE (low, moderate or high) was assigned by clinicians blinded to outcome for adults with rTOF identified from an institutional database (n=25 patient reviews conducted by five independent observers). A validated ML model identified 10 variables for risk prediction in the same population. RESULTS: Prediction by ML was similar to the aggregate score of all experts (area under the curve (AUC) 0.85 (95% CI 0.58 to 0.96) vs 0.92 (0.72 to 0.98), p=0.315). Experts with ≥20 years of experience had superior discriminative capacity compared with <20 years (AUC 0.98 (95% CI 0.86 to 0.99) vs 0.80 (0.56 to 0.93), p=0.027). In those with <20 years of experience, ML provided incremental value such that the combined (clinical+ML) AUC approached ≥20 years (AUC 0.85 (95% CI 0.61 to 0.95), p=0.055). CONCLUSIONS: Robust prediction of 5-year MACE in rTOF was achieved using either ML or a multidisciplinary team of ACHD experts. Risk prediction of some clinicians was enhanced by incorporation of ML suggesting that there may be incremental value for ML in select circumstances.
Subject(s)
Heart Defects, Congenital , Tachycardia, Ventricular , Tetralogy of Fallot , Humans , Adult , Child , Tetralogy of Fallot/diagnosis , Tetralogy of Fallot/surgery , Tachycardia, Ventricular/diagnosis , Tachycardia, Ventricular/etiology , Heart , Machine LearningABSTRACT
OBJECTIVE: Outcomes after adult coarctation of the aorta (CoA) stenting is scant. We explored predictors of aortic remodeling after stent implantation and report early- and intermediate-term clinical outcomes. METHODS: Adult patients who underwent stenting between July 2003 and December 2017 were included in this single-center retrospective study. We created a novel index of aortic volumetric and diameter changes using computed tomography (CT)/magnetic resonance (MR) images measured through TeraRecon and AngioQ. Predictors of aortic remodeling were explored using univariable linear regression analysis. RESULTS: One hundred and thirty-four patients (mean age 35.2 years, 58.2% men) underwent CoA stenting. Paired aortic diameter measurements were available in 20 patients, and 40 paired patients in volumetric measurements. There was significant reduction in aortic diameter immediately proximal to the left carotid and subclavian arteries, and the aorta distal to the stenosis (P less than .05) at follow-up. There was a significant volumetric reduction in the ascending aorta, aortic arch, and the aortic segment most proximal to the top of the stent (P less than .05). Univariate predictors of aortic remodeling included sex, age, presence of previous surgical repair, aortic valve morphology, and the number of antihypertensive medications. Mean follow-up time was 4.0 ± 3.8 years, where 5% of patients underwent reintervention due to complications, 3% developed aneurysms, and 3% had stent fractures. CONCLUSIONS: This study is the first to examine the anatomical changes that occur in the aorta post stent repair through analysis of serial imaging. Patients with stent-repaired coarctation demonstrated negative remodeling in multiple areas of the aorta with regards to the aortic diameter and volumetric measurements.
Subject(s)
Aortic Coarctation , Adult , Male , Humans , Female , Aortic Coarctation/diagnosis , Aortic Coarctation/surgery , Retrospective Studies , Aorta , Antihypertensive Agents , Constriction, PathologicABSTRACT
Importance: Valsartan has shown promise in attenuating cardiac remodeling in patients with early-stage sarcomeric hypertrophic cardiomyopathy (HCM). Genetic testing can identify individuals at risk of HCM in a subclinical stage who could benefit from therapies that prevent disease progression. Objective: To explore the potential for valsartan to modify disease development, and to characterize short-term phenotypic progression in subclinical HCM. Design, Setting, and Participants: The multicenter, double-blind, placebo-controlled Valsartan for Attenuating Disease Evolution in Early Sarcomeric Hypertrophic Cardiomyopathy (VANISH) randomized clinical trial was conducted from April 2014 to July 2019 at 17 sites in 4 countries (Brazil, Canada, Denmark, and the US), with 2 years of follow-up. The prespecified exploratory VANISH cohort studied here included sarcomere variant carriers with subclinical HCM and early phenotypic manifestations (reduced E' velocity, electrocardiographic abnormalities, or an increased left ventricular [LV] wall thickness [LVWT] to cavity diameter ratio) but no LV hypertrophy (LVH). Data were analyzed between March and December 2022. Interventions: Treatment with placebo or valsartan (80 mg/d for children weighing <35 kg, 160 mg/d for children weighing ≥35 kg, or 320 mg/d for adults aged ≥18 years). Main Outcomes and Measures: The primary outcome was a composite z score incorporating changes in 9 parameters of cardiac remodeling (LV cavity volume, LVWT, and LV mass; left atrial [LA] volume; E' velocity and S' velocity; and serum troponin and N-terminal prohormone of brain natriuretic peptide levels). Results: This study included 34 participants, with a mean (SD) age of 16 (5) years (all were White). A total of 18 participants (8 female [44%] and 10 male [56%]) were randomized to valsartan and 16 (9 female [56%] and 7 male [44%]) were randomized to placebo. No statistically significant effects of valsartan on cardiac remodeling were detected (mean change in composite z score compared with placebo: -0.01 [95% CI, -0.29 to 0.26]; P = .92). Overall, 2-year phenotypic progression was modest, with only a mild increase in LA volume detected (increased by 3.5 mL/m2 [95% CI, 1.4-6.0 mL/m2]; P = .002). Nine participants (26%) had increased LVWT, including 6 (18%) who developed clinically overt HCM. Baseline LA volume index (LAVI; 35 vs 28 mL/m2; P = .01) and average interventricular septum thickness (8.5 vs 7.0 mm; P = .009) were higher in participants who developed HCM. Conclusions and Relevance: In this exploratory cohort, valsartan was not proven to slow progression of subclinical HCM. Minimal changes in markers of cardiac remodeling were observed, although nearly one-fifth of patients developed clinically overt HCM. Transition to disease was associated with greater baseline interventricular septum thickness and LAVI. These findings highlight the importance of following sarcomere variant carriers longitudinally and the critical need to improve understanding of factors that drive disease penetrance and progression. Trial Registration: ClinicalTrials.gov Identifier: NCT01912534.
Subject(s)
Cardiomyopathy, Hypertrophic , Ventricular Remodeling , Adult , Child , Humans , Male , Female , Adolescent , Genetic Predisposition to Disease , Hypertrophy, Left Ventricular , Valsartan/therapeutic useABSTRACT
The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery (WCPCCS) will be held in Washington DC, USA, from Saturday, 26 August, 2023 to Friday, 1 September, 2023, inclusive. The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery will be the largest and most comprehensive scientific meeting dedicated to paediatric and congenital cardiac care ever held. At the time of the writing of this manuscript, The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery has 5,037 registered attendees (and rising) from 117 countries, a truly diverse and international faculty of over 925 individuals from 89 countries, over 2,000 individual abstracts and poster presenters from 101 countries, and a Best Abstract Competition featuring 153 oral abstracts from 34 countries. For information about the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery, please visit the following website: [www.WCPCCS2023.org]. The purpose of this manuscript is to review the activities related to global health and advocacy that will occur at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.Acknowledging the need for urgent change, we wanted to take the opportunity to bring a common voice to the global community and issue the Washington DC WCPCCS Call to Action on Addressing the Global Burden of Pediatric and Congenital Heart Diseases. A copy of this Washington DC WCPCCS Call to Action is provided in the Appendix of this manuscript. This Washington DC WCPCCS Call to Action is an initiative aimed at increasing awareness of the global burden, promoting the development of sustainable care systems, and improving access to high quality and equitable healthcare for children with heart disease as well as adults with congenital heart disease worldwide.
Subject(s)
Cardiac Surgical Procedures , Cardiology , Heart Diseases , Adult , Child , HumansABSTRACT
The natural history of an unrepaired isolated partial anomalous pulmonary venous connection(s) (PAPVC) and the absence of other congenital anomalies remains unclear. This study aimed to expand the understanding of the clinical outcomes in this population. Isolated PAPVC with an intact atrial septum is a relatively uncommon condition. There is the perception that patients with isolated PAPVC are usually asymptomatic, that the lesion generally has a limited hemodynamic impact, and that surgical repair is rarely justified. For this retrospective study, we reviewed our institutional database to identify patients with either 1 or 2 anomalous pulmonary veins that drain a portion of but not the complete ipsilateral lung. Patients with previous surgical cardiac repair, coexistence of other congenital cardiac anomalies that would result in either pretricuspid or post-tricuspid loading of the right ventricle (RV), or scimitar syndrome were excluded. We reviewed their clinical course over the follow-up period. We identified 53 patients; 41 with a single and 12 with 2 anomalous PAPVC. A total of 30 patients (57%) were men, with a mean age at the latest clinic visit of 47 ± 19 years (18 to 84 years). Turner syndrome (6 of 53, 11.3%), bicuspid aortic valve (6 of 53, 11.3%), and coarctation of the aorta (5 of 53, 9.4%) were commonly associated anomalies. A single anomalous left upper lobe vein was the most commonly identified variation. More than half of the patients were asymptomatic. Cardiopulmonary exercise test demonstrated a maximal oxygen consumption of 73 ± 20% expected (36 to 120). Transthoracic echocardiography demonstrated a mean RV basal diameter of 4.4 ± 0.8 cm, RV systolic pressure of 38 ± 13 (16 to 84) mm Hg. A total of 8 patients (14.8%) had ≥moderate tricuspid regurgitation. Cardiac magnetic resonance in 42 patients demonstrated a mean RV end-diastolic volume index of 122 ±3 0 ml/m2 (66 to 188 ml/m2), of which in 8 (14.8%), it was >150 ml/m2. Magnetic resonance imaging-based Qp:Qs was 1.6 ± 0.3. A total of 5 patients (9.3%) had established pulmonary hypertension (mean pulmonary artery pressure ≥25 mm Hg). In conclusion, isolated single or dual anomalous pulmonary venous connection is not necessarily a benign congenital anomaly because a proportion of patients develop pulmonary hypertension and/or RV dilation. Regular follow-up and on-going patient surveillance with cardiac imaging is advised.
Subject(s)
Atrial Septum , Heart Defects, Congenital , Hypertension, Pulmonary , Pulmonary Veins , Scimitar Syndrome , Male , Humans , Adult , Middle Aged , Aged , Female , Scimitar Syndrome/diagnostic imaging , Scimitar Syndrome/surgery , Hypertension, Pulmonary/etiology , Retrospective Studies , Heart , Heart Defects, Congenital/complications , Pulmonary Veins/diagnostic imaging , Pulmonary Veins/surgeryABSTRACT
BACKGROUND: The Harmony transcatheter pulmonary valve (TPV) is the first U.S. Food and Drug Administration-approved device for severe pulmonary regurgitation (PR) in the native or surgically repaired right ventricular outflow tract (RVOT). OBJECTIVES: One-year safety and effectiveness of the Harmony TPV were evaluated in patients from the Harmony Native Outflow Tract Early Feasibility Study, Harmony TPV Pivotal Study, and Continued Access Study, representing the largest cohort to date of Harmony TPV recipients. METHODS: Eligible patients had severe PR by echocardiography or PR fraction ≥ 30% by cardiac magnetic resonance imaging and clinical indications for pulmonary valve replacement. The primary analysis included 87 patients who received a commercially available TPV22 (n = 42) or TPV25 (n = 45) device; 19 patients who received an early device iteration prior to its discontinuation were evaluated separately. RESULTS: In the primary analysis, median patient age at treatment was 26 years (IQR: 18-37 years) in the TPV22 group and 29 years (IQR: 19-42 years) in the TPV25 group. At 1 year, there were no deaths; 98% of TPV22 and 91% of TPV25 patients were free from the composite of PR, stenosis, and reintervention (moderate or worse PR, mean RVOT gradient >40 mmHg, device-related RVOT reoperation, and catheter reintervention). Nonsustained ventricular tachycardia occurred in 16% of patients. Most patients had none/trace or mild PR (98% of TPV22 patients, 97% of TPV25 patients). Outcomes with the discontinued device are reported separately. CONCLUSIONS: The Harmony TPV device demonstrated favorable clinical and hemodynamic outcomes across studies and valve types through 1 year. Further follow-up will continue to assess long-term valve performance and durability.
Subject(s)
Heart Valve Prosthesis Implantation , Heart Valve Prosthesis , Pulmonary Valve Insufficiency , Pulmonary Valve , Ventricular Outflow Obstruction , Humans , Cardiac Catheterization , Prospective Studies , Pulmonary Valve/diagnostic imaging , Pulmonary Valve/surgery , Pulmonary Valve Insufficiency/diagnostic imaging , Pulmonary Valve Insufficiency/etiology , Pulmonary Valve Insufficiency/surgery , Treatment Outcome , Ventricular Outflow Obstruction/etiologyABSTRACT
BACKGROUND: Genetic defects in the RAS/mitogen-activated protein kinase pathway are an important cause of hypertrophic cardiomyopathy (RAS-HCM). Unlike primary HCM (P-HCM), the risk of sudden cardiac death (SCD) and long-term survival in RAS-HCM are poorly understood. OBJECTIVES: The study's objective was to compare transplant-free survival, incidence of SCD, and implantable cardioverter-defibrillator (ICD) use between RAS-HCM and P-HCM patients. METHODS: In an international, 21-center cohort study, we analyzed phenotype-positive pediatric RAS-HCM (n = 188) and P-HCM (n = 567) patients. The between-group differences in cumulative incidence of all outcomes from first evaluation were compared using Gray's tests, and age-related hazard of all-cause mortality was determined. RESULTS: RAS-HCM patients had a lower median age at diagnosis compared to P-HCM (0.9 years [IQR: 0.2-5.0 years] vs 9.8 years [IQR: 2.0-13.9 years], respectively) (P < 0.001). The 10-year cumulative incidence of SCD from first evaluation was not different between RAS-HCM and P-HCM (4.7% vs 4.2%, respectively; P = 0.59). The 10-year cumulative incidence of nonarrhythmic deaths or transplant was higher in RAS-HCM compared with P-HCM (11.0% vs 5.4%, respectively; P = 0.011). The 10-year cumulative incidence of ICD insertions, however, was 5-fold lower in RAS-HCM compared with P-HCM (6.9% vs 36.6%; P < 0.001). Nonarrhythmic deaths occurred primarily in infancy and SCD primarily in adolescence. CONCLUSIONS: RAS-HCM was associated with a higher incidence of nonarrhythmic death or transplant but similar incidence of SCD as P-HCM. However, ICDs were used less frequently in RAS-HCM compared to P-HCM. In addition to monitoring for heart failure and timely consideration of advanced heart failure therapies, better risk stratification is needed to guide ICD practices in RAS-HCM.
Subject(s)
Cardiomyopathy, Hypertrophic , Defibrillators, Implantable , Heart Failure , Humans , Cohort Studies , Death, Sudden, Cardiac/epidemiology , Death, Sudden, Cardiac/etiology , Defibrillators, Implantable/adverse effects , Cardiomyopathy, Hypertrophic/complications , Cardiomyopathy, Hypertrophic/genetics , Cardiomyopathy, Hypertrophic/diagnosis , Heart Failure/complications , Risk Factors , Risk AssessmentABSTRACT
OBJECTIVE: Despite decades of experience, aspects of the management of tetralogy of Fallot with pulmonary stenosis (TOF) remain controversial. Practitioners must consider newer, evolving treatment strategies with limited data to guide decision making. Therefore, the TOF Clinical Practice Standards Committee was commissioned by the American Association for Thoracic Surgery to provide a framework on this topic, focused on timing and types of interventions, management of high-risk patients, technical considerations during interventions, and best practices for assessment of outcomes of the interventions. In addition, the group was tasked with identifying pertinent research questions for future investigations. It is recognized that variability in institutional experience could influence the application of this framework to clinical practice. METHODS: The TOF Clinical Practice Standards Committee is a multinational, multidisciplinary group of cardiologists and surgeons with expertise in TOF. With the assistance of a medical librarian, a citation search in PubMed, Embase, Scopus, and Web of Science was performed using key words related to TOF and its management; the search was restricted to the English language and the year 2000 or later. Articles pertaining to pulmonary atresia, absent pulmonary valve, atrioventricular septal defects, and adult patients with TOF were excluded, as well as nonprimary sources such as review articles. This yielded nearly 20,000 results, of which 163 were included. Greater consideration was given to more recent studies, larger studies, and those using comparison groups with randomization or propensity score matching. Expert consensus statements with class of recommendation and level of evidence were developed using a modified Delphi method, requiring 80% of the member votes with 75% agreement on each statement. RESULTS: In asymptomatic infants, complete surgical correction between age 3 and 6 months is reasonable to reduce the length of stay, rate of adverse events, and need for a transannular patch. In the majority of symptomatic neonates, both palliation and primary complete surgical correction are useful treatment options. It is reasonable to consider those with low birth weight or prematurity, small or discontinuous pulmonary arteries, chromosomal anomalies, other congenital anomalies, or other comorbidities such as intracranial hemorrhage, sepsis, or other end-organ compromise as high-risk patients. In these high-risk patients, palliation may be preferred; and, in patients with amenable anatomy, catheter-based procedures may prove favorable over surgical palliation. CONCLUSIONS: Ongoing research will provide further insight into the role of catheter-based interventions. For complete surgical correction, both transatrial and transventricular approaches are effective; however, the smallest possible ventriculotomy should be utilized. When possible, the pulmonary valve should be spared; and if unsalvageable, reconstruction can be considered. At the conclusion of the operation, adequate relief of the right ventricular outflow obstruction should be confirmed, and identification of a significant fixed anatomical obstruction should prompt further intervention. Given our current knowledge and the gaps identified, we propose several key questions to be answered by future research and potentially by a TOF registry: When to palliate or proceed with complete surgical correction, as well as the ideal type of palliation; the optimal surgical approach for complete repair for the best long-term preservation of right ventricular function; and the utility, efficacy, and durability of various pulmonary valve preservation and reconstruction techniques.
Subject(s)
Cardiac Surgical Procedures , Heart Septal Defects , Pulmonary Atresia , Pulmonary Valve , Tetralogy of Fallot , Thoracic Surgery , Infant, Newborn , Infant , Humans , United States , Tetralogy of Fallot/surgery , Cardiac Surgical Procedures/adverse effects , Cardiac Surgical Procedures/methods , Treatment Outcome , Retrospective StudiesABSTRACT
PURPOSE: The purpose of this study was to compare outcomes of Melody mitral valve to mechanical mitral valve replacement (MVR) for young children. DESCRIPTION: Children who underwent Melody MVR from 2014 to 2020 were case-matched to mechanical MVR patients. Transplant-free survival and cumulative incidence of reintervention were compared. A subanalysis was performed for infants aged < 1 year (9 Melody MVRs and their matches). EVALUATION: Twelve children underwent Melody MVR. Two children (17%) salvaged from mechanical support died. Five of 10 survivors (50%) had subsequent MVR. At 1 and 3 years, transplant-free survival (Melody: 83%, 83%; mechanical: 83%, 67%; P = .180) and reintervention (Melody: 9%, 39%; mechanical: 0%, 18%; P = .18) were equivalent between groups. For children < 1 year of age, Melody MVR had a modest survival benefit (Melody: 89%, 89%; mechanical: 80%, 60%; P = .046), while rate of reintervention remained equivalent (Melody: 13%, 32%; mechanical: 0%, 22%; P = .32). CONCLUSIONS: For patients < 1 year old, Melody MVR offers a promising alternative and is a reasonable bridge to mechanical MVR, which can be performed safely at an older age. Further studies are necessary to corroborate these findings.