Coronary microvascular endothelial dysfunction in transplanted children.

AIMS: To assess the response of the coronary microcirculation to acetylcholine (endothelium-dependent vasodilator) and of adenosine (endothelium-independent vasodilator) in children after heart transplantation and to verify whether endothelial dysfunction is time-dependent. METHODS AND RESULTS: We studied the endothelial function of 26 asymptomatic children previously submitted to heart transplantation, with normal transplanted hearts and epicardial coronary arteries. Ten untransplanted children served as controls. The response of coronary blood flow velocity to intracoronary infusion of acetylcholine (1.8 microg x min(-1)) and adenosine (270 microg x min(-1)) was assessed using a Doppler wire positioned in an epicardial coronary branch. In the study group, coronary blood flow velocity increased slightly during acetylcholine infusion (peak/baseline ratio=1.17+/-0.22). The ratio was inversely correlated with the length of follow-up (r=-0.50;P=0.0078). The peak/baseline ratio in control children was 1.76+/-0.73 (P<0.0002 vs. study group). After adenosine infusion, the coronary blood flow velocity peak/baseline ratio was 3.75+/-1.54 in transplanted children and 3.72+/-1.34 in controls (P=ns). CONCLUSIONS: Endothelial dysfunction in paediatric transplanted patients becomes more evident in patients with longer follow-up. This finding could prove useful in the prevention of accelerated arteriosclerosis.

Noonan syndrome: structural abnormalities of the mitral valve causing subaortic obstruction.

UNLABELLED: Among 41 consecutive children with classic Noonan syndrome, 27 patients (66%) presented cardiac anomalies. Eight patients (19.5%) had a congenital anomaly of the mitral valve consisting of 5 with partial atrioventricular canal defect and 3 with anomalous insertion of the mitral valve on the ventricular septum. Five patients (12%) presented with a significant left ventricular outflow tract obstruction caused by the anterior leaflet of the mitral valve: two cases with atrioventricular canal and three cases with isolated anomalous insertion of the mitral valve. Echocardiography is the best tool for the diagnosis. Cardiac defects of patients with Noonan syndrome may be explained on the basis of anomalies of the extracellular matrix involving cardiac valves including the mitral valve. CONCLUSION: In children with Noonan syndrome and left ventricular hypertrophy a careful echocardiographic assessment of the mitral valve should reveal those in whom the left ventricular outflow tract obstruction is anatomical in nature.