Drenajul venos pulmonar aberant total reprezintă o cardiopatie congenitală cianogenă severă, cu o incidenţă de la 0,6 la 1,2 pe 10000 nou-născuţi sau 1 - 2% din totalul MCC. Această malformaţie cardiacă frecvent se asociază cu sindroame genetice, dar foarte rar evoluează concomitent cu hipoplazia şi obstrucţia venelor pulmonare, precum a fost în cazul clinic descris mai sus. Se prezintă cazul unui copil diagosticat la vârsta de 3 luni cu MCC DVPTA, tip I, HTP suprasistemică, ICC CF III-IV Ross, operat cu succes la vârsta de 3,5 luni. Evoluţie favorabilă postoperator, însă cu apariţia de hemoptizii ultimele 6 luni. În fi nal, la vârsta de 2,1 ani a fost diagnosticată concomitent hipoplazia venelor pulmonare stângi cu obstrucţia lor totală.

The total anomalous pulmonary venous drainage (TAPVD) is the severe cardiac heart disease, with the incedence from 0,6 to 1,2 per 10000 newborns or 1-2% of all congenital heart malformations. This heart malformaion often associated with genetic syndromes, but rearely is evolving together with the hypoplasia of the pulmonary veins, as was described in ours clinical case. This report presents the case of child who was diagnosed at three month after birth with Congenital Heart Malformations, TAPVD, Pulmonary Hypertension, Congestive Herat Failure Functional Class III-IV Ross, successfully operated at 3,5 month; with favorable evolution postoperative, but appeared the hemoptysis in the last six month. Finally, at 2,1 years while the patient was diagnosed with hypoplastic left pulmonary veins witn their total obstruction.