Ali Zaidi

• Albert Einstein College of Medicine

Background Some unrestrictive congenital shunts put patients at high risk for developing irreversible pulmonary vascular disease if not closed in the first year of life. Living at high altitude also places patients at risk for developing pulmonary hypertension. There are anecdotal data that living at high altitude may be protective against the deve...

Right ventricular (RV) end-diastolic volume (RVEDV) and ejection fraction (RVEF) by cardiac MRI (cMRI) guide management in chronic pulmonary regurgitation (PR). Two-dimensional echocardiography suboptimally correlate with RV volumes. This study tested whether combination of guideline-directed RV measures in a machine learning (ML) framework improve...

Background The Fontan operation is a surgical procedure to palliate single ventricle congenital heart disease. Hepatocellular carcinoma (HCC) is a rare complication of Fontan-associated liver disease (FALD). Objectives The authors aim to examine characteristics of individuals with Fontan circulation diagnosed with HCC and to describe tumor charact...

Pulmonary vein stenosis is challenging to treat due to high rate of recurrence. Multiple interventions exist but are limited by high rates of restenosis. One theory for the high rate of recurrence is vascular inflammation. Drug-coated balloon angioplasty has been used to limit restenosis caused by inhibiting inflammation in pulmonary vein stenosis.

Sinus venosus atrial septal defect (SVASD) with partial anomalous pulmonary venous return is a rare congenital heart defect that may present in adulthood when right heart enlargement has already occurred. We describe a case of unrepaired sinus venosus atrial septal defect and partial anomalous pulmonary venous return with massive right heart enlarg...

Background: The Congenital Heart Initiative (CHI) is the first patient-powered digital registry for adults with congenital heart disease (ACHD). The CHI-Redefining Outcomes and Navigation to Adult-Centered Care (CHI-RON) study is built upon the CHI as a unique collaboration with PCORnet. Goal: Recruitment of populations that are racially diverse, y...

Transition of care refers to the continuity of health care during the movement from one healthcare setting to another as care needs change during a chronic illness. We sought to describe social, demographic, and clinical factors related to successful transition in a tertiary urban care facility in patients with CHD. Patients were identified utilisi...

Background Controversy regarding the optimal pulmonary valve substitute remains, with no approved surgical valve for pulmonary valve replacement (PVR). Furthermore, unfavorable anatomy often precludes transcatheter PVR in patients with congenital heart disease. We therefore sought to evaluate the feasibility of the Edwards Inspiris pericardial aort...

This poster explains how a registry was developed to collect patient-reported outcomes (PROs) from individuals with congenital heart disease which is a relatively unstudied, heterogeneous group of rare cardiac anomalies.

The Fontan operation has resulted in significant improvement in survival of patients with single ventricle physiology. As a result, there is a growing population of individuals with Fontan physiology reaching adolescence and adulthood. Despite the improved survival, there are long-term morbidities associated with the Fontan operation. Pulmonary com...

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a congenital malformation that classically presents within the first year of life. Few patients survive into adulthood, and initial presentation after the fourth decade of life is rare. We describe a 55-year-old woman who presented after cardiac arrest. She initially refused surge...

Together, heart failure and arrhythmia represent the most important cardiovascular sources of morbidity and mortality among adults with congenital heart disease (ACHDs). Although traditionally conceptualized as operating within 2 distinct clinical silos, these scenarios frequently coexist within the same individual; consequently the mechanistic, th...

Background We sought to characterize health care usage for adolescents with congenital heart defects (CHDs) using population‐based multisite surveillance data. Methods and Results Adolescents aged 11 to 18 years with ≥1 CHD‐related diagnosis code and residing in 5 US sites were identified in clinical and administrative data sources for the years 2...

Purpose of Review Bicuspid aortic valve (BAV) is the most common congenital cardiac abnormality. It has a wide spectrum of clinical manifestations including aortic regurgitation (AR), aortic stenosis, and an associated aortopathy with a small but increased risk of aortic dissection. This review describes current knowledge of BAV, from anatomy and g...

Introduction: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare coronary artery anomaly that carries 90% mortality in the first year of life when left untreated. The diagnosis of ALCAPA is rare in adulthood, and it includes a broad spectrum of clinical manifestations, including sudden cardiac death (SCD). C...

Eisenmenger syndrome refers to any untreated congenital cardiac defect with an intracardiac communication that leads to pulmonary arterial hypertension, reversal of intracardiac shunting, and cyanosis. We describe a 40-year-old cyanotic patient with congenital heart disease with presumed Eisenmenger syndrome who was considered inoperable. Testing r...

Background Individuals with congenital heart defects (CHDs) are recommended to receive all inpatient cardiac and non-cardiac care at facilities that can offer specialized care. We describe geographic accessibility to such centers in New York State and determine several factors associated with receiving care there. Methods We used inpatient hospita...

Cardiac disease is a leading cause of morbidity and mortality in pregnant women. An increased prevalence of the cardiovascular disease has been found in women of childbearing age, in which the responsibility of the treating physician extends to the mother and to the unborn fetus. As a result, care of these high-risk pregnant women with cardiovascul...

The American College of Cardiology (ACC) collaborated with the American Heart Association, American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic...

Objectives: Most individuals born with congenital heart defects (CHDs) survive to adulthood, but healthcare utilization patterns for adolescents and adults with CHDs have not been well described. We sought to characterize the healthcare utilization patterns and associated costs for adolescents and young adults with CHDs. Methods: We examined 200...

Purpose of review: With advances in the diagnosis and treatment of congenital heart disease (CHD), over 90% of infants born with CHD now reach adulthood. Patients with CHD require lifelong care and are at an increased risk of psychological distress, neurocognitive deficits, social challenges, and a lower quality of life (QOL). There exists limited...

Background In the United States, >1 million adults are living with congenital heart defects (CHDs), but gaps exist in understanding the health care needs of this growing population. Objectives This study assessed the demographics, comorbidities, and health care use of adults ages 20 to 64 years with CHDs. Methods Adults with International Classif...

Background: Women with congenital heart defects (CHDs) experiencing pregnancies require specialized delivery care and extensive monitoring that may not be available at all birthing hospitals. In this study, we examined proximity to, and delivery at, a hospital with an appropriate level of perinatal care for pregnant women with CHDs and evaluated p...

Atrial septal defects are common congenital heart defects, characterized by insufficient/absent tissue at the interatrial septum. An unrepaired defect may be associated with right heart volume overload, atrial arrhythmia or pulmonary arterial hypertension. The 3 major types of atrial septal defect are: ostium secundum defect, ostium primum defect,...

December 2019, a pathogenic novel human coronavirus (HCoV), termed SARS-CoV-2, was recognized in Wuhan, China causing significant morbidity and mortality. The illness caused by SARS-CoV-2 is labelled COVID-19 by WHO. We report the first case of COVID-19 in an ACHD patient with Single Ventricle (SV) physiology S/P Fontan palliation.

Life expectancy of patients with congenital heart disease (CHD) continues to increase dramatically over the last few decades, primarily due to significant medical and surgical advancements in the field. Adult congenital heart disease (ACHD) patients are now living longer but continue to face morbidity and reduced long-term survival when compared to...

Background Most individuals born with congenital heart defects (CHDs) survive to adulthood, but healthcare utilization patterns for adolescents and adults with CHDs have not been well described. We sought to characterize the healthcare utilization patterns and associated costs for adolescents and young adults with CHDs. Methods We examined 2009-201...

Background Thromboembolic events contribute greatly to morbidity and mortality following Fontan surgery for univentricular hearts. Objectives This study sought to evaluate the effect of type of Fontan surgery on thromboembolic risk. Methods A North American multicenter retrospective cohort study enrolled 522 patients with Fontan palliation consis...

Background: Pregnant women with congenital heart defects (CHDs) may be at increased risk for adverse events during delivery. Objectives: This study sought to compare comorbidities and adverse cardiovascular, obstetric, and fetal events during delivery between pregnant women with and without CHDs in the United States. Methods: Comorbidities and...

The prevalence, co-morbidities, and healthcare utilization in adolescents with congenital heart defects (CHDs) is not well understood. Adolescents (11 to 19 years old) with a healthcare encounter between January 1, 2008 (January 1, 2009 for MA) and December 31, 2010 with a CHD diagnosis code were identified from multiple administrative data sources...

Background Adults presenting with an unrepaired atrial septal defect and pulmonary arterial hypertension (ASD-PAH) are typically classified as “correctable” or “non-correctable”. The use of directed PAH medical therapy in non-correctable ASD-PAH leading to favorable closure candidacy, repair status and long-term follow-up is not well studied. We th...

In 1971, Fontan and Baudet described a surgical technique for successful palliation of patients with tricuspid atresia. Subsequently, this technique has been applied to treat most forms of functional single ventricles and has become the current standard of care for long-term palliation of all patients with single ventricle congenital heart disease....

Background Atrial arrhythmias are the most common complication encountered in the growing and aging population with congenital heart disease (CHD). We assessed the types and patterns of atrial arrhythmias, associated factors, and age-related trends. Methods A multicenter cohort study enrolled 482 patients with CHD and atrial arrhythmias, age 32.0...

Background: Many individuals with congenital heart defects (CHDs) discontinue cardiac care in adolescence, putting them at risk of adverse health outcomes. Because geographic barriers may contribute to cessation of care, we sought to characterize geographic access to comprehensive cardiac care among adolescents with CHDs. Methods: Using a popula...

Background: Atrial arrhythmias are the most common complication encountered in the growing and aging population with congenital heart disease. Objectives: This study sought to assess the types and patterns of atrial arrhythmias, associated factors, and age-related trends. Methods: A multicenter cohort study enrolled 482 patients with congenita...

A 38-year-old woman with a history of recurrent deep venous thromboses (DVTs) on chronic anticoagulation presented with acute left leg swelling. The patient was diagnosed with an acute left lower extremity (LLE) DVT in the setting of May-Thurner syndrome for which treatment with unfractionated heparin was started. Her hospital course was complicate...

Obesity is increasingly prevalent, and abnormal body mass index is a risk factor for cardiovascular disease. There are limited data published regarding body mass index and CHD. We tested the hypothesis that body mass index and obesity prevalence are increasing in patients with tetralogy of Fallot over time by analysing time since surgery, age, heig...

Background: Sudden cardiac death is a major contributor to mortality for adults with congenital heart disease. The subcutaneous implantable cardioverter-defibrillator (ICD) has emerged as a novel tool for prevention of sudden cardiac death, but clinical performance data for adults with congenital heart disease are limited. Methods and results: A...

Pulmonary hypertension (PH) due to chronic respiratory disease and/or hypoxia is classified as World Health Organization (WHO) Group III pulmonary hypertension. The patients most commonly encountered in clinical practice with group III PH include those with chronic obstructive lung disease (COPD), diffuse parenchymal lung disease, and sleep-disorde...

Objectives: The growing number of women with CHD presents unique challenges, including those related to pregnancy, which can lead to significant morbidity and mortality. We sought to evaluate the perception of paediatric cardiologists towards the reproductive health of women with CHD. Methods: Paediatric cardiologists in the United States of Ame...

Background: There is a lack of objective data on the symptoms characterizing disease activity among adults with congenital heart disease (ACHD). The purpose of this study was to elicit the most important symptoms from patients across the spectrum of ACHD and to examine whether reported symptoms were similar across the spectrum of ACHD as a foundat...

Introduction: The profile of congenital heart disease (CHD) has shifted, now with more adults than children who have survived. Few studies have provided a global assessment of adult congenital heart disease (ACHD) healthcare cost in the United States. Methods: Data from the National Inpatient Sample (2002-2012) utilizing diagnostic ICD-9 codes for...

Objective Nurse practitioners (NPs) have an established role for delivering competent care to patients in the primary care setting. The aim of this study was to compare satisfaction of patients managed by NPs vs. physicians in the outpatient adult congenital heart disease (CHD) clinic.DesignA prospective study conducted in two outpatient adult CHD...

Basic knowledge of genetics is essential for understanding genetic testing and counseling. The lack of a written, English language, validated, published measure has limited our ability to evaluate genetic knowledge of patients and families. Here, we begin the psychometric analysis of a true/false genetic knowledge measure. The 18-item measure was c...

Because of the increased risks associated with unplanned pregnancy for males and females with congenital heart disease (CHD), we investigated sexual intercourse and contraception use in these adolescents and young adults (AYA) and compared the same with national and state population data. We recruited 337 AYA with structural CHD aged 15-25 years (M...

Aberrant cellular oxygen sensing (pseudo-hypoxia) is a leading theory for development of pheochromocytoma (PHEO) and paraganglioma (PGL). To test the hypothesis that chronic hypoxia in patients with cyanotic congenital heart disease (CCHD, CHD) increases the risk for PHEO-PGL. We investigated the association between CCHD and PHEO-PGL with 2 complem...

Over the past two decades, there has been an increasing body of data that has acknowledged the progressive nature of aortic dilation in adult patients with congenital heart disease. Aortic disease can have overwhelming effects on affected individuals, resulting in severe morbidities including aneurysms, tears, dissections, and aortic valve regurgit...

The univentricular heart includes a spectrum of complex cardiac defects that are managed by staged palliative surgical procedures, ultimately resulting in a Fontan procedure. Since 1971, when it was first developed, the procedure has undergone several variations. These patients require lifelong management, including a thorough knowledge of their an...

Background Adult congenital heart disease (ACHD) clinicians are hampered by the paucity of data to inform clinical decision-making. The objective of this study was to identify priorities for clinical research in ACHD. Methods A list of 45 research questions was developed by the Alliance for Adult Research in Congenital Cardiology (AARCC), compiled...

AimsThere is a fivefold increase in the frequency of intracranial aneurysm (IA) in adults with coarctation of the aorta (CoA). Current guidelines for management of adults with CoA recommend computed tomography angiography (CTA) or magnetic resonance imaging of the intracranial vessels. However, this recommendation has not been universally accepted....

Background: In studies of adult patients, increased QRS duration and mechanical dyssynchrony have been associated with decreased ventricular function. The aim of this study was to test the hypothesis that similar findings would be present in a population of patients with hypoplastic left heart syndrome (HLHS) after the Fontan procedure. Methods:...

Coarctation of the aorta (CoA) occurs in 0.04% of the population and accounts for approximately 10% of lesions in adults with congenital heart disease (ACHD). It can occur as an isolated lesion or as a part of a complex defect and is associated with multiple complications, both with and without treatment. Since the first surgical repair in 1944, si...

Background: With advances in cardiac care, patients with congenital heart disease, including single ventricle (SV) physiology, now survive into adulthood. These patients often suffer from congestive heart failure (CHF) with overexpression of serum biomarkers. Strain and strain rate (SR) may better describe the myocardial mechanics of a failing SV....

Background: Currently >85% of children in the US with congenital heart disease (CHD) survive to adulthood. Patients with repaired Tetralogy of Fallot (TOF) and critical pulmonary stenosis (PS) frequently present with late sequelae including pulmonary insufficiency, right ventricular (RV) enlargement and systolic dysfunction. These patients may ha...

Bartonella spp. are a known cause of culture-negative endocarditis. We report a 42-year-old male with underlying surgically corrected congenital heart disease who was diagnosed with Bartonella henselae endocarditis involving the right-ventricle-to-pulmonary-artery conduit.

Purpose: Patients with systemic right ventricle (RV) after Fontan procedure are at increased risk for heart failure. In adult studies, increased mechanical and/or electrical dyssynchrony have been associated with decreased ventricular function. The goal of this study was to determine if similar correlations exist in a population of single RV patien...

Double outlet right ventricle is a heterogeneous congenital defect that encompasses a variety of anatomic aberrations and physiologic consequences. We describe the unusual cardiovascular magnetic resonance anatomic findings and sequelae of a 44-year-old man who underwent biventricular repair of double outlet right ventricle, subpulmonary type, whic...

Atrial function is increasingly being recognized as a significant factor in overall cardiac function in adults. Limited studies evaluating atrial properties exist in the pediatric congenital heart disease population. The goal of this study was to evaluate atrial properties in patients with single ventricle physiology after Fontan completion and com...

As the prevalence of obesity continues to increase, it now includes the growing number of patients with congenital heart disease (CHD). This particular obese patient population may pose additional intraoperative as well as postoperative challenges that may contribute to poor outcomes. Our aims were to determine the influence of obesity on morbidity...

Myocardial bridging is characterized by intra-myocardial course of a segment of an epicardial coronary artery, leading to systolic compression. In most cases, it is considered a benign condition but rarely has been associated with serious cardiac events like myocardial infarction and sudden death. We describe a 25-year-old man with known d-transpos...

Patients with single left ventricle (LV) physiology maybe at increased risk for myocardial dysfunction and mechanical dyssynchrony. Newer echocardiographic modalities may be able to better quantitate LV function in this unique population. Our objective was to use 2D-speckle analysis of strain and strain rate to quantify systolic function and dyssyn...

Brugada syndrome is an arrhythmogenic disease characterized by a pattern of ST segment elevation in the right precordial leads on an electrocardiogram with an increased risk of sudden cardiac death. It is primarily reported in adults with limited data about the prevalence, prognosis, and long-term management in children. We describe a 10-year-old b...

Tissue Doppler imaging measurements could be used to quantitate ventricular function in patients with systemic single ventricles. The objective of the present study was to measure the tissue doppler imaging-derived indexes of function and dyssynchrony in patients with single ventricular physiology and compare these values with those from control pa...

Patients with single-right ventricle (RV) physiology are at increased risk for myocardial dysfunction and mechanical dyssynchrony. Newer echocardiographic modalities may be better able to quantitate right ventricular function in this unique population. The aim of this study was to use two-dimensional speckle analysis of strain and strain rate to qu...

Percutaneous device closure in patients with an atrial septal defect (ASD) or patent foramen ovale (PFO) has gained popularity because of the short learning curve, cosmetic advantage and relative safety compared with surgery. Device complications may include device embolism, erosion, pericardial tamponade or thrombus formation, and most complicatio...

Complications of transcatheter closure of atrial septal defects (ASDs) include pericardial effusion, tamponade, and even death. Transthoracic echocardiography in the adult is often limited by poor acoustic windows that lead to incomplete device assessment. Advances in multislice computed tomography (MSCT) provide an alternative modality to assess t...

We report a case of a 34-year-old woman who had a left anterior wall myocardial infarction develop in the first trimester of pregnancy. Despite urgent and successful revascularization, she demonstrated persistent segmental wall motion abnormalities by transthoracic echocardiography. To manage this patient safely through pregnancy with a better defi...