Structural changes in pulmonary vessels and coronary arteries in hypoplastic left heart syndrome.

A Macedo, E Pinto, S Ramos, M J Brito, M M Costa, I Serrano, I Torrezão, M Lima, M E Silva, M L Roriz


In order to correlate gross morphological features with pulmonary and coronary vasculature changes in hypoplastic left heart syndrome (HLHS) 15 heart-lung necropsy specimens from neonates were analysed. Histology of left atrium wall, lung vessels with pulmonary arteriolar disease morphometric grading, study of intra and extra pulmonary wall veins and coronary arteries were recorded. Classic HLHS was observed in 11, and DORV with mitral atresia in 4, the foramen ovale (FO) being the only left atrium outflow tract in all. All specimens had increased % of thickness and muscular extension of arterioles and increased wall thickness of pulmonary veins, but severer arteriolar and venous changes with left atrium fibrosis were noticed in 8 specimens with either closed FO (3 cases) or mean FO diameter (d) of 5 mm when compared with specimens with mean FOd of 9 mm. Coronary arteries histology disclosed in 4 of 8 specimens with opened but hypoplastic mitral valve, intima proliferative changes with lumen narrowing, not observed in 7 specimens with mitral valve atresia. We concluded that in HLHS closed or restrictive FO and permeable mitral valve may predispose neonates to respiratory complications, right ventricle abnormal function and arrhythmias, specially after cardiac surgery.

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