Key result
A fetus with pulmonary atresia with intact ventricular septum and severe right ventricular hypoplasia developed severe left ventricular dysfunction, hypothesized to be due to reduced coronary blood flow.
Case Report (n=1)
Highlights a potential mechanism for severe left ventricular dysfunction in fetuses with pulmonary atresia and intact ventricular septum due to right ventricular coronary arterial dependence.
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May warrant LV monitoring in fetal pulmonary atresia/intact septum; hypothesis-generating for coronary dependence and requires validation.
Pulmonary atresia with intact ventricular septum has been described extensively, and it is recognized that associated abnormalities of the coronary arteries may cause ischemia. We describe a fetus, diagnosed antenatally as having pulmonary atresia with intact ventricular septum and severe hypoplasia of the right ventricle, who developed severe left ventricular dysfunction. We hypothesize that this is due to reduced coronary blood flow because of falling right ventricular volume in the presence of complete right ventricular coronary arterial dependence.
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Khoshhal et al. (2004) conducted a case report in Pulmonary atresia with intact ventricular septum and antenatal left ventricular failure (n=1). Pulmonary atresia with intact ventricular septum and severe hypoplasia of the right ventricle was evaluated on Severe left ventricular dysfunction. A fetus with pulmonary atresia with intact ventricular septum and severe right ventricular hypoplasia developed severe left ventricular dysfunction, hypothesized to be due to reduced coronary blood flow.
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