Congenital absence of pulmonary valve was first described by Chevers in 1847,1 and since that time, at least 150 cases have been reported. It may occur alone or in association with other congenital malformations of the heart, such as tetralogy of Fallot, ventricular septal defect, obstruction of the right ventricular outflow, and dilatation of the pulmonary arteries. The most frequent coexisting lesion is a large ventricular septal defect, almost always infracristal.
The characteristic pathological configuration is a continuity between the right ventricular infundibulum and the pulmonary trunk eventually limited by a hypoplastic pulmonary valve, and an aneurysmatic dilatation of the pulmonary trunk and its branches. This dilatation may assume huge proportions and compress the tracheobronchial tree, producing respiratory distress and obstructive pulmonary emphysema in the first year of life. The development of congestive cardiac failure may worsen the clinical picture and induce high mortality in this group of patients.
In the absence of other cardiac malformations, pulmonary insufficiency is well tolerated, but in those patients with multiple cardiac defects, cardiac failure may present difficulties in clinical management. Although surgery is a serious consideration in this condition, indications for total intracardiac repair are not well established, since the natural history of this condition is poorly understood.
We present a patient who had atrial and ventricular septal defects, obstruction to the right ventricular outflow, and absent pulmonary valve, which were surgically repaired. Embriologic, anatomic, and surgical considerationsof the pathology are discussed, and the importance of pulmonary insufficiency is emphasized.
From the Department of Cardiovascular Surgery of Instituto de Cardiologia, Fundacao Universitariade Cardiologia, Porto Alegre, Rio Grande do Sul, Brazil.
*Former Fellow in Cardiovascular Surgery of the Texas Heart Institute, Houston, Texas.
Address for reprints:
Ivo A. Nesralla, M.D., Instituto de Cardiologia, Avenida Princesa Isabel,395, 90.000 Porto Alegre, Rio Grande do Sul, Brazil.