The Right Heart in Congenital Heart Disease, Mechanisms and Recent Advances

Abstract

In patients with congenital heart disease, the right heart may support the pulmonary or the systemic circulation. Several congenital heart diseases primarily affect the right heart including Tetralogy of Fallot, transposition of great arteries, septal defects leading to pulmonary vascular disease, Ebstein anomaly and arrhythmogenic right ventricular cardiomyopathy. In these patients, right ventricular dysfunction leads to considerable morbidity and mortality. In this paper, our objective is to review the mechanisms and management of right heart failure associated with congenital heart disease. We will outline pearls and pitfalls in the management of congenital heart disease affecting the right heart and highlight recent advances in the field.

Signalment, clinical features, echocardiographic findings, and outcome of dogs and cats with ventricular septal defects: 109 cases (1992–2013)

“…7,9,27,34,53 These imaging data provide crucial information for cardiac surgeons before performing VSD repair, both to choose the optimal closure technique and to assess potential VSD-associated risk factors. These combined ultrasonographic techniques are considered the most sensitive tools for noninvasive diagnosis of human VSDs and for determining the size and location of the defects as well as identifying concomitant valvular regurgitation.…”

“…Treatment status at the time of diagnosis was known for all 53 Conventional and standard Doppler echocardiographic findings at diagnosis-Some quantitative data were not available for all animals. Data regarding VSD diameter, assessed by color flow Doppler mode, were available for 46 of 53 (87%) animals (21 dogs and 25 cats) with a diagnosis of isolated VSD.…”

Electrical Stimulation of pediatric cardiac-derived c-kit+ progenitor cells improves retention and cardiac function in right ventricular heart failure

“…CHDs are the most common birth defect and are the number one cause of death from birth defects during the first year of life . CHDs are the leading cause of right ventricular heart failure (RVHF) in the pediatric population, especially in patients with defects like pulmonary stenosis, Hypoplastic Left Heart Syndrome and Tetrology of Fallot . There is much evidence on the differences between the right and left ventricle in both structure, function, and pathologies .…”

Endogenous cardiac stem cells for the treatment of heart failure

“…We have recently identified several individual cellular phenotypes within the endogenous cardiovascular stem cell population in sheep and have compared these results to our findings in humans. Four distinct subpopulations of cardiac progenitors exist in the heart of neonatal sheep, some of which lack the expression of c-kit 69. These populations were defined as CD105 + stage-specific embryonic antigen 4 (SSEA4) + c-kit + Isl1 + GATA4 + cells, CD105 + SSEA4 + c-kit + Isl1 + GATA4 − cells, CD105 + SSEA4 − c-kit − Isl1 + GATA4 − cells, and CD105 + SSEA4 − c-kit + Isl1 + GATA4 − cells.…”