Decreased Left Ventricular Coronary Artery Density in Pulmonary Atresia and Intact Ventricular Septum

Abstract

Background: The prognosis for pulmonary atresia and intact ventricular septum (PAIVS) has been poor. Our hypothesis is that intrinsic abnormal left ventricular (LV) intramyocardial circulation might be related to the poor outcomes of these patients. Methods:Neonatal heart specimens were examined microscopically in four groups of 6 cases each. Group I had PAIVS with ventriculocoronary artery connections (VCAC), group II had PAIVS without VCAC, group III had normal hearts, and group IV had LV hypertrophy. A projection microscope with grid overlay was used to count the LV intramyocardial coronary artery density (IMCAD), which was expressed as the number of profiles/mm². Results: The LV IMCAD of groups I (0.40 ± 0.14/mm²) and II (0.45 ± 0.15/mm²) were significantly lower than those of groups III (0.77 ± 0.11/mm²) and IV (0.76 ± 0.09/mm²; all with p = 0.002). There was no significant difference between either groups I and II (p = 0.394) or groups III and IV (p = 0.818). Conclusions: This study demonstrates lower LV IMCAD in a widely heterogeneous spectrum of neonatal hearts with PAIVS, which might potentially predispose these patients to myocardial ischemia and in turn contribute to the poor prognosis of this disease.

A Rare Coronary Collateral in Pulmonary Atresia and Intact Septum With Coronary Sinusoids

“…3 Patients with PAIVS typically also have a diminutive left coronary system with the risk of an ischemic event leading to sudden death correlating inversely with the LV intramyocardial coronary density. 7 The severity of this disease is illustrated in a report by Eisses et al 8 of a neonate with PAIVS, ventriculocoronary sinusoids, and bilateral ostial atresia who underwent an mBTs and died postoperatively. Another case report of a neonate with PAIVS, ventriculocoronary sinusoids, and RVDCC describes deterioration of ventricular function and clinical status following the construction of an mBTs.…”

Critical care outcomes in pulmonary atresia and intact ventricular septum undergoing single-ventricle palliation

“…7,8 Much of the suboptimal survival can be attributed to the tenuous coronary circulation to both left and right ventricles. 9,10 There is a higher early mortality associated with the primary intervention; in most cases, this is a modified Blalock-Taussig shunt, [11][12][13] however ductal stenting may also be an option. 14 The purpose of this study was to look at those infants with very small ventricles, dependant of coronary blood flow undergoing single-ventricle palliation, who have the worst survival.…”

“…Survival for these infants is disproportionately low compared with other lesions that have undergone single-ventricle palliation 7 , 8 . Much of the suboptimal survival can be attributed to the tenuous coronary circulation to both left and right ventricles 9 , 10 . There is a higher early mortality associated with the primary intervention; in most cases, this is a modified Blalock–Taussig shunt, 11 – 13 however ductal stenting may also be an option 14 .…”