[Combination of ventricular septal defect with unilateral pulmonary artery stenoses and contralateral pulmonary...

The clinical features and hemodynamics of the triad ventricular septal defect, unilateral stenoses of the pulmonary artery and contralateral pulmonary hypertension are illustrated by means of 2 case histories. At the time of cardiac catheterization the discovery of severe pulmonary vascular disease of the left lung with inoperable multiple peripheral stenoses of the right pulmonary artery in the first patient, a 4-year old girl, meant that corrective surgery could no longer be undertaken. The second patient, a 10-month old infant with embryofetal alcohol syndrome, suffered from peripheral and two central stenoses of the right pulmonary artery with a total systolic pressure gradient of 62 mm Hg. In our opinion, if the contralateral pulmonary vascular bed has to cope largely single-handed with the increased cardiac output of the right ventricle as a result of additional unilateral pulmonary artery stenosis, the hemodynamic impact of a ventricular septal defect is considerably increased and the development of pulmonary vascular disease accelerated. Since there are strong indications that unilateral pulmonary artery stenosis has more than a mere mechanical effect causing regulative (humorally?, neurogenically?) pulmonary hypertension due also to increased resistance, we are in favour of early closure of the ventricular septal defect and additionally, if technically possible, of an angioplasty or a bypass operation of the stenosis. Since, to the best of our knowledge, an association between proved pulmonary artery stenoses and embryofetal alcohol syndrome has not yet been reported, we feel that our findings are worth sharing.