ArticleAmerican journal of physiology. Heart and circulatory physiology2025
Microvascular preservation and cardiomyocyte hyperplasia underlie adaptive right ventricle development in congenital heart disease-pulmonary arterial hypertension.
Article in American journal of physiology. Heart and circulatory physiology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Right ventricular (RV) failure is the primary cause of death among patients with pulmonary arterial hypertension (PAH). Patients with congenital heart disease-associated PAH (CHD-PAH) demonstrate improved outcomes compared with patients with other forms of PAH, which is related to the maintenance of an adaptively hypertrophied RV. In an ovine model of CHD-PAH, we aimed to elucidate the cellular, microvascular, and transcriptional adaptations to congenital pressure overload that support RV function. Fetal surgery was performed on late gestation lambs to insert an aortopulmonary graft, leading to a persistent congenital left-right shunt and RV pressure load. At 3 days and 4-6 wk of life, shunt RV myocardial structure, growth mechanisms, and transcriptomes were compared with age-matched control and unoperated fetal RV. At 4-6 wk of age, shunt lambs demonstrate significant RV enlargement (shunt 37.1 ± 7.6 g vs. control 15.9 ± 1.9 g,
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