Erişkin bir hastada miks drenaj total anormal pulmoner venöz dönüş olgusu

Total anormal pulmoner venöz dönüş, doğuştan kalp hastalıklarının %1.5-3’ünde görülür. Hastaların çoğunda hayatın ilk günlerinden itibaren siyanoza ciddi kalp yetmezliği eşlik ettiği için, tanıyı takiben acil cerrahi girişim gerekmektedir. Bu nadir doğuştan kalp hastalığı, genellikle erişkin dönemde cerrahi ile tedavi edilir. Cerrahi girişim olmaksızın erişkin yaşa kadar ulaşan hastalarda büyük bir atriyal septal defekt mevcuttur. Otuz altı yaşındaki kadın hasta çabuk yorulma, nefes darlığı ve dudaklarda morarma yakınmaları ile başvurdu. Transtorasik ekokardiyografi ve kateter anjiyografide total anormal pulmoner venöz dönüş, sekundum tip atriyal septal defekt, ventriküler septal defekt ve pulmoner hipertansiyon saptandı. İloprost ile yapılan pulmoner vazoreaktivite testine yanıt veren hastaya tıbbi tedavi sonrasında cerrahi planlandı

A case of mixed drainage total anomalous pulmonary venous return in an adult patient

Total anomalous pulmonary venous return is observed in 1.5 to3% of congenital heart diseases. Urgent surgical intervention isrequired following the diagnosis, since severe heart failure isaccompanied by cyanosis from the first days of life in most ofthe patients. This rare congenital heart disease is often managedin adulthood with surgery. Patients who survive until adulthoodwithout a surgical intervention have a large atrial septal defect.A 36-year-old female patient was admitted with complaintsof fatigability, shortness of breath, and cyanosis of the lips.Total anomalous pulmonary venous return, secundum-typeatrial septal defect, ventricular septal defect, and pulmonaryhypertension were diagnosed by transthoracic echocardiographyand catheter angiography. After medical treatment, surgerywas planned for the patient who responded to the pulmonaryvasoreactivity test with iloprost.

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