'Isolated' pulmonary valve stenosis as part of more widespread cardiovascular disease

In 25 patients aged 6 days to 9 years presenting as 'isolated' pulmonary valve stenosis, histology of the myocardium of right and left ventricles, coronary arteries, and ascending aorta has shown abnormality in one or all these areas. Myocardial necrosis, old and recent, unrelated to coronary occlusion was frequent. Myocardial 'dysplasia' involving both ventricles, and resembling hypertrophic cardiomyopathy (HOCM, ASH) was found in 10 and a relation of this to myocardial injury in the fetus is postulated. Varying degrees of coronary occlusion were frequently seen in both right and left coronary arteries. The histology of the ascending aorta was abnormal showing 'higgledy-piggledy' disorder of smooth muscle components in 12 (48%). In a different series of 53 patients who had pulmonary valvotomy for apparent 'isolated' pulmonary valve stenosis there were 14 with clinical evidence of left ventricular abnormality consistent with the pathological changes described, 2 with the same aortic histological changes, and 2 with macroscopical left ventricular hypertrophy. Two of them developed classical hypertrophic cardiomyopathy years later. It is suggested that when pulmonary valve stenosis presents with a thick tricuspid poorly mobile valve, particularly in infants or in patients with evidence of other congenital stigmata, it may be part of a more widespread cardiovascular abnormality. This should be recognized and considered in the evaluation of surgical patients and late survivors who may show unexpected clinical features.