Publications by authors named "Sunil J Ghelani"

Objective: For neonatal repair of coarctation of the aorta, patients may either undergo thoracotomy with extended end-to-end anastomosis or sternotomy for aortic arch reconstruction with cardiopulmonary bypass. The objective of this study was to evaluate the comparative effectiveness of the 2 approaches in patients with arch hypoplasia.

Methods: This is a single-center retrospective cohort study from July 2005 through May 2022 of patients who underwent neonatal repair for isolated coarctation of the aorta with additional arch hypoplasia.

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Patients with congenitally corrected transposition of the great arteries (ccTGA) can be treated with a double switch operation (DSO) to restore the normal anatomical connection of the left ventricle (LV) to the systemic circulation and the right ventricle (RV) to the pulmonary circulation. The subpulmonary LV progressively deconditions over time due to its connection to the low pressure pulmonary circulation and needs to be retrained using a surgical pulmonary artery band (PAB) for 6-12 months prior to the DSO. The subsequent clinical follow-up, consisting of invasive cardiac pressure and non-invasive imaging data, evaluates LV preparedness for the DSO.

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Background: Patients with hypoplastic left ventricles (LV) who undergo volume-loading procedures (recruitment, biventricular (BIV) repair) are at risk for adverse outcomes including heart failure and death. We investigated pre-BIV LV shape as a predictor of outcome after BIV repair in patients with hypoplastic LVs.

Methods: Baseline and post-recruitment cardiac MRI and CT data were analyzed in patients with hypoplastic LV (< 50 ml/m).

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Article Synopsis
  • The study seeks to improve risk stratification for patients with congenital heart disease (CHD) by using an AI-enhanced electrocardiogram (ECG) tool, addressing a significant gap in current medical practice.* -
  • A convolutional neural network was developed and tested on a large set of ECGs from patients at Boston Children's Hospital to predict 5-year mortality, showing good performance compared to traditional indicators like age and heart function metrics.* -
  • The model displays potential for timely risk assessment across various ages and types of CHD, helping to guide future monitoring and therapeutic interventions for patients.*
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Objectives: Although conduction location can be reliably predicted in double inlet ventricle, ventricular septation continues to carry a significant risk of complete heart block. This study describes our experience using intraoperative conduction mapping during ventricular septation.

Methods: Patients undergoing ventricular septation from 2017 to 2023 were identified.

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Background: Artificial intelligence-enhanced electrocardiogram (AI-ECG) analysis shows promise to predict mortality in adults with acquired cardiovascular diseases. However, its application to the growing repaired tetralogy of Fallot (rTOF) population remains unexplored.

Objectives: This study aimed to develop and externally validate an AI-ECG model to predict 5-year mortality in rTOF.

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Background: Artificial intelligence-enhanced electrocardiogram (AI-ECG) analysis shows promise to detect biventricular pathophysiology. However, AI-ECG analysis remains underexplored in congenital heart disease (CHD).

Objectives: The purpose of this study was to develop and externally validate an AI-ECG model to predict cardiovascular magnetic resonance (CMR)-defined biventricular dysfunction/dilation in patients with CHD.

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Mean dP/dt is a quantitative measurement of ventricular function that can be obtained noninvasively by echocardiography. In adults with mitral regurgitation (MR), it has been shown to be a more sensitive predictor of postoperative left ventricular ejection fraction (EF). The utility of dP/dt in pediatric congenital heart diseases with MR has been underexplored.

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Article Synopsis
  • This study aimed to evaluate if unsupervised machine learning can differentiate patients with Fontan circulation from healthy individuals based on heart function metrics derived from cardiovascular magnetic resonance (CMR) imaging.
  • By analyzing 503 CMR studies from Fontan patients and 42 from controls, researchers identified four distinct patient clusters with different risks for adverse outcomes like heart failure or transplantation.
  • The highest-risk group, characterized by specific heart shapes and poor functioning, showed significantly greater health risks compared to the lowest-risk group, suggesting that machine learning can help target distinct patient subgroups for better clinical management.
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Secundum atrial septal defect (ASD2) detection is often delayed, with the potential for late diagnosis complications. Recent work demonstrated artificial intelligence-enhanced ECG analysis shows promise to detect ASD2 in adults. However, its application to pediatric populations remains underexplored.

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Background: Abnormal left ventricular (LV) rotational mechanics in biventricular hearts are associated with adverse outcomes; however, these are less well characterized for hearts with functionally single ventricles.

Objectives: The purpose of this study was to characterize ventricular rotational mechanics in the Fontan circulation and their relationship to outcomes.

Methods: Single-center, retrospective analysis of magnetic resonance examinations for 329 Fontan patients (15 [IQR: 10-21] years) and 42 controls.

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  • Disordered lymphatic drainage is common in congenital heart diseases, and this study focuses on the drainage patterns of the thoracic duct (TD) in patients with heterotaxy, a condition involving abnormal arrangement of internal organs.
  • Researchers analyzed 115 patients with heterotaxy who had cardiovascular imaging, noting that the TD was visualized in nearly half, with varying sidedness: left (25), right (29), and bilateral (2).
  • The study found that while various anatomical factors were associated with TD sidedness, only the sidedness of the superior vena cava and aortic arch were significant predictors, highlighting the complexity of lymphatic anatomy in heterotaxy for future medical interventions.
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Background: The Fontan operation is a palliative technique for patients born with single ventricle heart disease. The superior vena cava (SVC), inferior vena cava (IVC), and hepatic veins are connected to the pulmonary arteries in a total cavopulmonary connection by an extracardiac conduit or a lateral tunnel connection. A balanced hepatic flow distribution (HFD) to both lungs is essential to prevent pulmonary arteriovenous malformations and cyanosis.

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While quadricuspid morphology is commonly observed in truncal valves, quadricuspid aortic valves (QAV) are rare and their natural history is not well described. This retrospective study of 37 patients describes the diagnostic associations and morphologic variability of QAVs in children (median age at diagnosis 4.3 y IQR 0-12 y).

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Background: Artificial intelligence-enhanced ECG analysis shows promise to detect ventricular dysfunction and remodeling in adult populations. However, its application to pediatric populations remains underexplored.

Methods: A convolutional neural network was trained on paired ECG-echocardiograms (≤2 days apart) from patients ≤18 years of age without major congenital heart disease to detect human expert-classified greater than mild left ventricular (LV) dysfunction, hypertrophy, and dilation (individually and as a composite outcome).

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Article Synopsis
  • The study investigates how ventricular dyssynchrony affects clinical outcomes in patients who have undergone Fontan palliation compared to healthy individuals.
  • Using cardiac magnetic resonance imaging, the research analyzes various metrics of cardiac function, finding increased dyssynchrony, longer QRS durations, and worse systolic function in Fontan patients.
  • Key findings also show that certain factors like right ventricular morphology and ventricular dilation are linked to higher risks of death or heart transplantation, suggesting these metrics could help in assessing patient risk.
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Background Patients with pulmonary atresia or critical pulmonary stenosis with intact ventricular septum (PA/IVS) and biventricular circulation may require pulmonary valve replacement (PVR). Right ventricular (RV) remodeling after PVR is well described in tetralogy of Fallot (TOF); we sought to investigate RV changes in PA/IVS using cardiac magnetic resonance imaging. Methods and Results A retrospective cohort of patients with PA/IVS who underwent PVR at Boston Children's Hospital from 1995 to 2021 with cardiac magnetic resonance imaging before and after PVR was matched 1:3 with patients with TOF by age at PVR.

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Background: Some patients with pulmonary atresia with an intact ventricular septum (PA/IVS) or a left ventricle dominant atrioventricular canal defect (LDAVC) with a hypoplastic right ventricle (RV) and univentricular (1 V) circulation may be candidates for conversion to either a complete biventricular (2 V) repair or a one-and-a-half ventricle repair (1.5 V). We sought to identify pre-operative cardiovascular magnetic resonance (CMR) findings associated with successful conversion from 1V to 1.

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Objective: Perioperative corticosteroids have been used for pediatric cardiac surgery for decades, but the underlying evidence is conflicting. We aimed to investigate the efficacy and safety of perioperative prophylactic corticosteroids in pediatric heart surgeries.

Methods: We searched electronic databases until March 2023 to retrieve all randomized controlled trials (RCTs) that administered perioperative prophylactic corticosteroids to children undergoing heart surgery.

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We conducted this meta-analysis to compare expectant management of patent ductus arteriosus (PDA) with active treatment for PDA closure in preterm infants. Data from 7 randomized controlled trials (RCTs) showed that all-cause mortality and other clinical adverse outcomes did not differ between expectant management of PDA and active treatment. Future large-scale and double-blinded RCTs with a consistent definition for hemodynamically significant PDA, and focusing on clearly delineated high-risk subgroups or later selective treatment are needed to further evaluate the role of expectant management.

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Background: Ventricular pacing can cause myocardial dysfunction, but how lead anchoring to the myocardium affects function has not been studied.

Objective: The purpose of this study was to evaluate patterns of regional and global ventricular function in patients with a ventricular lead using cine cardiac computed tomography (CCT) and histology.

Methods: This was a single-center retrospective study with 2 groups of patients with a ventricular lead: (1) those who underwent cine CCT from September 2020 to June 2021 and (2) those whose cardiac specimen was analyzed histologically.

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Background: Lymphatic complications are common in patients with Fontan circulation. Three-dimensional balanced steady-state free precession (3D bSSFP) angiography by cardiovascular magnetic resonance (CMR) is widely used for cardiovascular anatomical assessment. We sought to determine the frequency of thoracic duct (TD) visualization using 3D bSSFP images and assess whether TD characteristics are associated with clinical outcomes.

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Background: Cross-sectional studies have reported that ventricular dilation and dysfunction are associated with adverse clinical outcome in Fontan patients; however, longitudinal changes and their relationship with outcome are not known.

Methods: This was a single-center retrospective analysis of Fontan patients with at least 2 cardiovascular magnetic resonance (CMR) scans without intervening interventions. Serial measures of end-diastolic volume index (EDVI), end-systolic volume index (ESVI), ejection fraction (EF), indexed mass (mass), mass-to-volume ratio, and end-systolic wall stress (ESWS) were used to estimate within-patient change over time.

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