VENTRICULO‐CORONARY MICRO‐COMMUNICATIONS IN PULMONARY ATRESIA and SEQUENTIAL CHANGES OF CORONARY ARTERIES

Abstract

In the present paper, numerous micro-communications between myocardial sinusoids and small branches of coronary arteries are studied which existed in a rudimentary right ventricle of a female neonate with pulmonary atresia and an intact ventricular septum, who was born on the 40th week of gestation. Cardioangiography revealed a large fistulous subepicardial communication. At autopsy, myocardium of the right ventricle appeared spongy with extensive sinusoid slits. In addition to the large communication which was connecting a deeply extending sinusoid of the right ventricle to the main trunk of the anterior descending artery, numerous micro-communications were found between myocardial sinusoids in the rudimentary right ventricle and intramural small branches of the coronary artery. Furthermore, morphometrical analysis demonstrated that the medias of the intramural arteries in both ventricles of the present case were significantly hypertrophic, as compared with those in the control cases without communications (p <0.05). These facts indicate the significance of hemodynamic factors on the persistence of embryonic sinusoid-coronary communication. ACTA PATHOL.

The significance of ventriculo-coronary arterial connections in the setting of pulmonary atresia with an intact ventricular septum

“…There is, nonetheless, a spectrum of coronary arterial pathology, as demonstrated both at necropsy or by angiographic imaging. It is also well established that mild obstructions, with time, can progress to more severe pathology 14,15,18,21,55,83,[93][94][95]116 (Figs 9-13).…”

“…We have already made comment concerning the extensive literature devoted to the angiocardiographic imaging of coronary arterial anatomy. 14,15,18,21,55,83,[95][96][97]116 Based on our long and substantial experience with this disorder, we use the working rule that, if selective right ventriculography fails to demonstrate connections with a coronary artery, it is very unlikely that there will be significant pathology involving that artery. 55 The rare situation when a single coronary artery, or a right coronary artery, originates from the pulmonary trunk, nonetheless, must always be remembered.…”

“…Serial angiographic studies have shown progression of coronary arterial pathology. 14,15,18,21,55,83,[95][96][97]116 458…”

Prenatal diagnosis of ventriculocoronary fistula

“…In such cases ventricular hypertension and hemodynamic disturbance are believed to force abnormal connections between myocardial sinusoids and cardiac chambers. These commonly coalesce into a single vessel connecting the right ventricular apex to the left coronary system [11][12][13] . In fetal life the presence of such fistulae may alleviate progression from endocardial fibroelastosis to ventricular hypoplasia.…”

“…A large unobstructed fistula can cause a 'steal' phenomenon because aortic blood flows freely into the right ventricle during diastole. Alternatively, the abnormal development and perfusion of ventriculocoronary connections may result in tortuosity, turbulent blood flow and segmental obstruction 11,14,15 . Concurrent abnormalities of coronary venous drainage 16,18 and intracardiac hemodynamics 19 may add to the risk of ischemia by further decreasing coronary perfusion pressure.…”