[Valvular diseases of the right heart]

Recently, the development of diagnostic and operative interventional techniques has contributed to the successful treatment of congenital valve lesions of the right heart. The clinical picture, diagnostic approaches, and therapeutic outcome are presented. In pulmonic valve stenosis the fusion of leaflets is most common. Stenosis severity corresponds to the intensity of the systolic murmur. Pressure gradients can be quantitated by Dopplersonography. Balloon valvuloplasty is the method of choice for treatment. Residual gradients (24-36 mmHg) are not significantly different from surgical results. In Tetralogy of Fallot the ventricular outflow tract obstruction and a subaortic septal defect are most characteristic, both of which can be visualized by echocardiography. Cyanosis and the pulmonary systolic murmur are typical findings. Complications due to hypoxemia are reduced by corrective surgery in early childhood. Arrhythmias and ventricular dysfunction may complicate the long-term prognosis. Pulmonary insufficiency mostly results from surgical relief of stenoses. It can be quantitated by densitometry and MNR. Even severe pulmonary insufficiency is well tolerated for decades; on the long-term its prognosis remains uncertain. In Ebstein's anomaly the leaflet attachment of the tricuspid valve is displaced into the right ventricle, which is best diagnosed by echocardiography. Symptomatology ranges from heart failure (infancy) to a systolic murmur only (childhood). Plastic recontruction of the tricuspid valve has become technically feasible.