Abstract
Myocardial sinusoids have been noted angiographically to communicate with the small LV of infants with hypoplastic left heart syndrome (HLHS). These may represent egress of blood at high pressure from the completely obstructed chamber into the coronary vasculature when aortic valve atresia is present with intact ventricular septum. In order to further characterize these channels we examined microscopically transverse subserinl sections from 12 autopsy specimens of HLHS. The mean age at death was 5.8 days. Gross anatomic defects were: Aortic valve atresia 9/12; Critical aortic stenonis 3/12; Mitral valve (MV) hypoplasia 11/12; MV atresia 1/12; VSD 2/12; Premature closure (PC) of foramen ovale (FO) 3/12; FO under 2 mm. 5/12. The mean LV chamber diameters were 13 × 9 mm. compared with 25 × 20 mm. for the RV. The mean LV wall thickness was 7 mm.
Microscopic findings included: Calcification of the myocardium 11/12; Thick walled LV with prominent endocardial fibroelastosis 9/12; Medial hypertrophy/intinal proliferation of coronary arteries 9/12; Multiple direct communications between LV cavity and coronary arteries 9/12 (absent in both VSD cases and one combined atresia case); Spongy capillary network in midzone of LV wall 7/12 (4 cases FO under 2 mm. and 3 cases PCFO). This study provides morphologic evidence of widespread developmental abnormalities of LV myocardial vascular structure in HLHS.
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O'Connor, W., Cash, J., Cottrill, C. et al. 1212 LEFT VENTRICULAR AND CORONARY ARTERY MORPHOLOGY IN THE HYPOPLASTIC LEFT HEART SYNDROME. Pediatr Res 15 (Suppl 4), 644 (1981). https://doi.org/10.1203/00006450-198104001-01238
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DOI: https://doi.org/10.1203/00006450-198104001-01238
