Alexander Opotowsky

The Anatomic and Physiological (AP) classification system proposed in the 2018 American College of Cardiology/American Heart Association adult congenital heart disease (ACHD) guidelines assigns 2 dimensions to each patient: anatomic class (AnatC) and physiological stage (PhyS). This approach has not been tested in practice; we assessed interrater reliability and identified sources of disagreement. METHODS: Consensus definitions for AP categories were developed with input from 4 experts...

To assess atrial function in Fontan patients using cardiac MRI (CMR) and determine the relationships between atrial function, hemodynamics, and clinical outcomes. Two center chart review identified all Fontan patients with technically adequate CMR to assess atrial function within 1 year of cardiac catheterization and age-matched controls with CMR. Atrial reservoir, conduit, and pump global longitudinal strain and strain rate measurements were determined by CMR. Univariate and stepwise multivariable analysis were conducted to detect associations of atrial function measures with ventricular end diastolic pressure (EDP), cardiac index (CI), exercise capacity, liver stiffness and a composite outcome of heart transplantation, ventricular assist device or death...

The Fontan procedure has improved survival and quality of life for patients with single ventricle physiology. Along with extended life expectancy comes a growing population that is experiencing long-term multiorgan adverse effects. Whereas cardiorenal interactions have been extensively studied in patients with a structurally normal heart, these are less well understood in patients with a single ventricle. Several studies have investigated the prevalence of reduced glomerular filtration rate and albuminuria in the Fontan population; however, the long-term implication of renal dysfunction is not well established in this population...

Pulmonary hypertension (PH) is common in adults with congenital heart disease and carries fundamental implications for management and prognosis. A high index of suspicion, combined with knowledge of the pathogenesis and pathophysiology of PH, is required to achieve a timely, accurate diagnosis, and appropriate classification and treatment. This article provides a guide on how to approach the adult with congenital heart disease and suspected PH of different types, including current management.

INTRODUCTION: Cardiorespiratory fitness (CRF) measured by oxygen consumption (VO2) during exercise is an important marker of health. The traditional method of indexing VO2 to total body mass is suboptimal since skeletal muscle mass (SMM), rather than fat and extracellular fluid, is the main contributor to CRF. The traditional estimating equations for peak VO2 in youth do not account for this. Bioelectric impedance analysis (BIA) is a non-invasive method to accurately measure body composition...

No abstract text is available yet for this article.

The Fontan procedure has provided patients with single ventricle physiology extended survival into adulthood and in many cases has improved their quality of life. Atrioventricular valve regurgitation (AVVR) is common in single ventricle patients and is associated with increased risk of mortality. AVVR is more common in patients with a systemic tricuspid or common atrioventricular valve but is generally progressive irrespective of underlying valve morphology. AVVR can be attributable to diverse structural and functional abnormalities at multiple levels of the valvar apparatus, as well as ventricular dysfunction and dilation...

BACKGROUND AND OBJECTIVES: Endothelin-1 is a potent endothelium-derived vasoconstrictor peptide implicated in the pathogenesis of hypertension, congestive heart failure, and inflammation, all of which are critical pathophysiologic features of CKD. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: To test the hypothesis that plasma endothelin-1 levels are associated with increased risks of mortality and hospitalization in patients with chronic kidney failure, we measured plasma endothelin-1 levels in a prospective cohort of 794 individuals receiving maintenance hemodialysis...

Background In adults with acquired heart disease, depression is common and associated with adverse outcomes. Depression may also be important in adults with congenital heart disease (CHD). Methods and Results We conducted a cohort study of outpatients with CHD, aged ≥18 years, enrolled in a prospective biobank between 2012 and 2017. Clinical data were extracted from medical records. Survival analysis assessed the relationship between depression, defined by a history of clinical diagnosis of major depression, with all-cause mortality and a composite outcome of death or nonelective cardiovascular hospitalization...

Lymphopenia is common in adults who have had a Fontan operation although its aetiology and clinical implications remain unknown. Previous work suggests an association between lymphopenia and both liver disease and splenomegaly. The objective of this study was to assess the prevalence of lymphopenia in adults with a Fontan circulation and evaluate its associations with risk factors and clinical outcomes. Using a retrospective cohort study design, we studied 73 adult Fontan patients (age 25.0 ± 8.4 years) who had a complete blood count and abdominal imaging performed...

No abstract text is available yet for this article.

OBJECTIVE: Optimal donor sizing for heart transplantation (HT) in adults with congenital heart disease (CHD) remains unclear, given the propensity for pulmonary hypertension related to shunting, staged repairs, and periods of pulmonary overcirculation. We studied HT outcomes related to donor size matching in the adult CHD population. METHODS: We conducted a retrospective cohort analysis of patients with CHD undergoing HT in the United States from January 1, 2000, to December 31, 2015...

Background People with a Fontan circulation experience a range of physical, psychosocial and neurodevelopmental challenges alongside, or caused by, their cardiac condition, with significant consequences for health-related quality of life (HRQOL). We meta-analyzed HRQOL outcomes reported by people with a Fontan circulation or their proxies and evaluated predictors of poorer HRQOL. Methods and Results Six electronic databases were searched for peer-reviewed, English-language articles published before March 2019...

Background: Thromboembolism (TE) and Fontan-associated liver disease (FALD) are common and lead to significant morbidity in Fontan circulations. Risk factors for TE and the potential link between TE and FALD are not well understood. The objective of this study was to evaluate the association between TE and the severity of FALD based on radiologic liver stiffness. Methods: Using a retrospective cohort study design, 85 Fontan patients (aged 27.7 ± 8.2 years) who had liver stiffness measurement were included...

Congenital heart disease (CHD)-related heart failure is common and associated with significant morbidity, mortality, and resource utilization. In adults with CHD (ACHD), exercise limitation is often underestimated. Quantitative assessment with cardiopulmonary exercise testing (CPET) provides a comprehensive evaluation of exercise capacity and can help risk stratify patients, particularly across serial testing. CPET parameters must be interpreted within the context of the underlying anatomy, specifically for patients with either single ventricle physiology and/or cyanosis...

PURPOSE: The clinical investigation of exertional intolerance generally focuses on cardiopulmonary diseases, while peripheral factors are often overlooked. We hypothesize that a subset of patients exists whose predominant exercise limitation is due to abnormal systemic oxygen extraction (SOE). METHODS: We reviewed invasive cardiopulmonary exercise test (iCPET) results of 313 consecutive patients presenting with unexplained exertional intolerance. An exercise limit due to poor SOE was defined as peak exercise (Ca-vO2 )/[Hb] ≤ 0...

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 consisting of an atriopulmonary connection (APC) (21.4%), lateral tunnel (LT) (41.8%), or extracardiac conduit (EC) (36...

No abstract text is available yet for this article.

No abstract text is available yet for this article.

BACKGROUND: Glomerular filtration rate is a key physiologic variable with a central role in clinical decision making and a strong association with prognosis in diverse populations. Reduced estimated glomerular filtration rate (eGFR) is common among adults with congenital heart disease (ACHD). METHODS: We conducted a prospective cohort study of outpatient ACHD ≥18 years old seen in 2012-2017. Creatinine and cystatin C were measured; eGFR was calculated using either the creatinine or cystatin C Chronic Kidney Disease-Epidemiology Collaboration equation (CKD-EPICr and CKD-EPICysC , respectively)...