Stenosis of the individual pulmonary veins (a rare cause of pulmonary hypertension?).

Abstract

In the evaluation of patients with pulmonary hypertension, the etiology is usually apparent: in most instances, it is acquired or congenital heart disease, pulmonary disease, etc. A small group of cases in which the cause is not readily apparent, however, are usually termed “idiopathic.” Such would have been the diagnosis in a 6-year-old boy seen by the authors if pulmonary arteriography had not been performed. This case, one of stenosis of the pulmonary veins, is therefore reported as an example of the importance of pulmonary arteriography in diagnosing rare and obscure causes of pulmonary hypertension. Moreover, as no good example of the angiocardiographic findings exists in previously reported cases, we were of the opinion that our angiocardiograms would be of further educational value. This 6-year-old boy was admitted to the St. Louis Childrens Hospital, Washington University, St. Louis, Mo., for evaluation of a heart murmur discovered at the time of preschool examination. Except for a Grade 2/6 ejection type systolic murmur heard loudest at the left base of the heart, physical examination was negative. Other clinical data, which included an electrocardiogram and cardiac roentgen series, were also normal. Because of the questionable character of the murmur and the nature of the referral, the patient was subjected to cardiac catheterization. The only positive finding was mild elevation of the pulmonary arterial pressure (35/10 mm Hg). The left atrium was not entered after several attempts at passage across the atrial septum. A pulmonary wedge pressure was not obtained, as this would have necessitated replacing a closed-end with an open-end catheter. We are probably open to criticism for not performing a more aggressive and complete hemodynamic study, but the prevailing tendency during the procedure was to regard the pulmonary artery pressure as instrumentation error. This preconceived attitude was reinforced by normal oxygen saturations, plus the essentially normal clinical findings. It was decided, however, to undertake pulmonary arteriography to rule out peripheral pulmonary artery stenosis or some mild obstructive lesion on the left side of the heart. Subsequent analysis of the pulmonary venous phase of the angiocardiogram revealed focal areas of stenosis in the major intrapulmonary branches of the right upper and left upper pulmonary veins (Fig. 1). Relatively severe stenosis of the major trunk of the left lower pulmonary vein at its junction with the left atrium was shown by moderately prolonged opacification of the left lower lobe veins (Fig. 1, D). No definite areas of stenosis were observed in the right lower lobe pulmonary veins, although there was some delay in flow. Thus, mild stenosis of the right lower lobe pulmonary vein at its junction with the left atrium could not be ruled out. Stenosis of the individual pulmonary veins is an extremely rare condition.