Abstract
Congenital heart disease is one of the major diagnoses in pediatric heart transplantation recipients of all age groups. Assessment of pulmonary vascular resistance in these patients prior to transplantation is crucial to determine their candidacy, however, it is frequently inaccurate because of their abnormal anatomy and physiology. This problem places them at significant risk for pulmonary hypertension and right ventricular failure post transplantation. The pathophysiology of pulmonary vascular disease in children with congenital heart disease depends on their pulmonary blood flow patterns, systemic ventricle function, as well as semilunar valves and atrioventricular valves structure and function. In our review we analyze the pathophysiology of pulmonary vascular disease in children with congenital heart disease and end-stage heart failure, and outline the state of the art pre-transplantation medical and surgical management to achieve reverse remodeling of the pulmonary vasculature by using pulmonary vasodilators and mechanical circulatory support.
Highlights
Pulmonary Hemodynamic ProfileExtension of muscle into peripheral arteries normally no striking evidence of medial Increased pulmonary nonmuscular, either as a solitary finding or associated with hypertrophy, same as in the blood flow without a mild increase in the medial wall thickness of the normorphometric grade A evidence of increased mally muscular arteries (1.5 normal)
Heart transplantation (HT) is increasingly considered a treatment option for patients with congenital heart disease (CHD) and end-stage heart failure
The assumption that elevated pulmonary vascular resistance (PVR) may contribute to late Fontan failure even in patients with “normal” pre-HT PVR was studied by Mitchell and colleagues [6]
Summary
Extension of muscle into peripheral arteries normally no striking evidence of medial Increased pulmonary nonmuscular, either as a solitary finding or associated with hypertrophy, same as in the blood flow without a mild increase in the medial wall thickness of the normorphometric grade A evidence of increased mally muscular arteries (1.5 normal). Grade A findings with greater medial hypertrophy pressure. Medial wall thickness is greater than 1.5 but less than 2 no striking evidence of medial times normal hypertrophy, same as in the morphometric grade B(mild). Medial hypertrophy can be associated with pulappreciated subjectively as in monary arterial hymorphometric grade B (severe) pertension. Grade B (severe) findings with a reduced number of peripheral arteries relative to alveoli and usually decreased arterial size more than half the normal number of arteries is present. Moderate-to-severe elevation in pulmonary vascular resistance when half the normal number of arteries or less is present
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