Early Experience With Sacubitril/Valsartan in Adult Patients With Congenital Heart Disease.

Background: Heart failure (HF) is the leading cause of hospitalizations and death in patients with adult congenital heart disease (ACHD). Sacubitril/valsartan is a new agent in the treatment of HF, but its effects have not been assessed in ACHD.

Methods: We retrospectively studied all 15 patients with ACHD at our center who were prescribed sacubitril/valsartan between June 2017 and June 2018.

Contraceptive Practices of Women With Complex Congenital Heart Disease.

Understanding the contraceptive practices of women with complex congenital heart disease (CHD) and providing them individualized contraception counseling may prevent adverse events and unplanned high-risk pregnancies. Given this, we sought to examine the contraceptive practices in women with CHD, describe adverse events associated with contraceptive use, and describe the provision of contraception counseling. Women >18 years were recruited from 2011 to 2014 from 9 adult CHD (ACHD) centers throughout North America.

Efficacy of endothelin blockade in adults with Fontan physiology.

Objective: Phosphodiesterase-5 inhibitors have shown to improve cardiac output and functional capacity in Fontan patients. We sought to test the efficacy and safety of endothelin blockade with bosentan in adult patients with Fontan physiology.

Design: Ten patients were enrolled and seven patients completed this single-center open-label clinical trial.

Usefulness of serum brain natriuretic peptide to predict adverse events in patients with the Eisenmenger syndrome.

The aim of this study was to evaluate the prognostic value of brain natriuretic peptide (BNP) in outpatients with the Eisenmenger syndrome (ES). BNP is often elevated in patients with cyanotic congenital heart disease. The clinical utility of BNP in patients with cyanotic congenital heart disease and the ES has not been clearly delineated.

Efficacy and safety of bosentan in adults with simple and complex Eisenmenger's syndrome.

Background:   Eisenmenger's syndrome (ES) is associated with decreased longevity and reduced functional capacity. Targeted pharmacologic therapies improve functional capacity and survival in these patients. We sought to compare the response of patients with simple vs.

Arrhythmia recurrence in adult patients with single ventricle physiology following surgical Fontan conversion.

Objectives: To evaluate the incidence of atrial tachy-arrhythmia (AT) recurrence following conversion from right atrial-pulmonary artery (RA-PA) Fontan to total cavopulmonary connection (TCPC) in adults.

Background: AT is a recognized sequel of Fontan palliation, especially in RA-PA Fontans, and is associated with significant morbidity. While catheter ablation achieves fairly reliable short-term success with low morbidity, conversion to TCPC with arrhythmia surgery is a highly effective treatment option for the classical Fontan patients with incessant AT.

Relation of brain natriuretic peptide to myocardial performance index in adults with congenital heart disease.

Myocardial performance index (MPI) is an echocardiographic Doppler-derived measure of ventricular function previously validated in patients with congenital heart disease. It may be preferred over conventional noninvasive measures of ventricular function in patients with complex anatomy because it is dependent on neither geometric shape nor heart rate. Brain natriuretic peptide (BNP) is a predictor of systolic and diastolic dysfunction in anatomically correct hearts.

Usefulness of post-ventriculotomy signal averaged electrocardiograms in congenital heart disease.

Signal-averaged electrocardiography is a sensitive and specific technique for detecting the slow conduction electrophysiologic substrates of reentrant monomorphic ventricular tachycardia. Although well established, the method has not been used for electrophysiologic assessment after right ventriculotomy for the intracardiac repair of congenital heart disease. This 8-year prospective study provided this assessment.

Cyanotic congenital heart disease and coronary artery atherogenesis.

Hypoxemic erythrocytotic residents of high altitudes lack coronary atherosclerosis and have low cholesterol levels. It was postulated that hypoxemic erythrocytotic adults with cyanotic congenital heart disease (CCHD) might be analogous. The incidence of coronary atherosclerosis in this patient population has not been established, and hypocholesterolemia has not previously been recognized.

Myocardial perfusion reserve in adults with cyanotic congenital heart disease.

In patients with cyanotic congenital heart disease (CCHD), a right-to-left shunt results in systemic hypoxemia. Systemic hypoxemia incites a compensatory erythrocytosis, which increases whole blood viscosity. We considered that these changes might adversely influence myocardial perfusion in CCHD patients.

Proximal pulmonary arterial and intrapulmonary radiologic features of Eisenmenger syndrome and primary pulmonary hypertension.

Extrapulmonary and intrapulmonary radiologic features of Eisenmenger syndrome and primary pulmonary hypertension (PPH) and the clinical expressions that coincide with the radiologic features of these 2 forms of pulmonary hypertension have not previously been characterized. Computed tomography (CT) and pulmonary CT angiography (CTA) utilized GE High Speed Advantage or Siemens Evolution electron beam tomographic (EBT) scanners in 31 patients with cyanotic pulmonary vascular disease (PVD) (group A: 12 men and 19 women; mean age 38 +/- 4 years) and in 13 women with PPH-acyanotic PVD (group B: mean age 30 +/- 3 years). Ten group A and 3 group B patients underwent imaging twice, yielding 57 studies.