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We present two patients with thromboembolic pulmonary hypertension associated with unusual complications probably caused by disseminated fibromuscular dysplasia (FMD) or FMD-like vascular lesions. Intimal fibroplasia, which is typical of the vascular lesions associated with FMD, was observed in both patients. The presence of such intimal lesions suggests that there was a systemic factor that caused the formation of recurrent thrombi in the systemic vessels in these patients. These cases are the first ones reported in which an association between FMD and pulmonary hypertension has been observed. The pathogenesis of the thrombi in our patients was thought to be recurrent pulmonary thromboembolisms resulting from FMD.

A 51 year old woman was transferred to our hospital for surgical treatment of a paracardiac mass. The patient was free of symptoms on admission. The chest x ray and electrocardiogram were normal. Echocardiography showed a large paracardiac mass with a homogeneous acoustic pattern in the right atrioventricular groove. Magnetic resonance imaging also showed a cardiac mass of about 6 cm in diameter located between the crux cordis and the posterior interventricular groove. Right coronary angiograms showed a discrete coronary artery aneurysm originating from the posterior descending branch (figure). The aneurysm was almost completely filled with a thrombus which was subsequently opacified in a right coronary angiogram. The distal segment of the posterior descending coronary artery was severely diseased and showed areas of dilatation. The other coronary arteries were normal.

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A cylindrical valve was designed to prevent regurgitation of the semilunar valve. The valve is made of a sheet of polytetrafluoroethylene (PTFE) or porcine pericardium, and has three cusps and three commissures. The diameter of the valve is equal to the height of the cusps. We have used these valves in pulmonary stenosis after Jatene's operation and total correction of tetralogy of Fallot, and for truncal valve regurgitation. Regurgitation was trivial on color Doppler echocardiography in all cases. Advantages in comparison with the implantation of commercially available artificial valves include the ability to insert a larger size and no compression of the valve ring when closing the sternum. Outflow tract obstruction does not occur even when the valve is implanted in a small infant. In the present report, we describe this simple technique.