Pediatr. praxi. 2017;18(1):52-55 | DOI: 10.36290/ped.2017.010

Progressive dyspnea and tachypnea as the major symptoms of the rare critical congenital heart defect

MUDr. Eva Klásková, Ph.D.¹, MUDr. Pavla Flodrová², MUDr. Petr Hecht³, MUDr. Patrik Flodr, Ph.D.², MUDr. Jaroslav Wiedermann, CSc.¹

1Dětská klinika FN a LF UP v Olomouci, 2Ústav klinické a molekulární patologie FN a LF UP v Olomouci, 3Dětské kardiocentrum FN Motol a 2. LF UK v Praze

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.

Keywords: pulmonary veins stenosis, congenital heart defect, heart murmur, post-capillary pulmonary hypertension

Published: April 4, 2017  Show citation