Progressive dyspnea and tachypnea as the major symptoms of the rare critical congenital heart defect
Abstract
Authors report a rare case of an infant presenting with progressive dyspnea and tachypnea together with heart murmur at the age of four weeks as a result of multivessel primary stenosis of common pulmonary veins drainage into the left atrium. The patient underwent two sutureless pulmonary vein stenosis repairs which always left to the only temporary amelioration of symptoms.
At the age of seven months, the baby died of severe post-capillary pulmonary hypertension. Pulmonary vein stenosis represents a relatively rare disease typically occurring in infants.
It is related to inadequate proliferation of myofibroblastic cells in pulmonary veins wall resulting in progressive pulmonary vein obliteration, and leading to death rapidly within weeks or months after diagnosis.