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A 32-year-old woman with pulmonary artery atresia with ventricular septal defect (VSD) was admitted because of New York Heart Association (NYHA) class worsening from II to III during the last 6 months. The patient presented resting cyanosis, short 6-minute walk test (6MWT) distance (370 m with desaturation – baseline: 84%, after 6MWT: 67%). N-terminal prohormone of brain natriuretic peptide (NT-proBNP) level was 194–262 pg/ml. She also suffered from significant kyphoscoliosis with stable chronic ventilation disturbances: forced vital capacity (FVC) = 1.38 l, forced expiratory volume in 1 s (FEV1) = 0.94 l, FEV1/FVC = 68%.

postępy w kardiologii interwencyjnej

Pulmonary artery atresia with ventricular septal defect, segmental pulmonary hypertension, major aortopulmonary collaterals (MAPCAs) and giant MAPCA aneurysm