The simultaneous presence of bronchiectasis in a patient with double-outlet right ventricle, atrial septal defect, ventricular septal defect and pulmonary stenosis

Abstract

Cift cikimli sag ventrikul (CCSV), basta ventrikuler septal defekt (VSD) olmak uzere bircok kardiyak anomali ile birlikte gorulebilen oldukca nadir bir anomalidir. Klinik bulgularin genellikle yenidogan doneminde gelismesi nedeni ile yasamin erken donemlerinde tani koyma olanagimiz vardir. Klinik bulgular patolojiye eslik eden VSD tipine ve pulmoner stenoz gibi ek kardiyak anomali olup olmamasina gore degisir. Bu hastalarda genellikle siyanoz, konjestif kalp yetmezligi bulgulari ve interstisyel pulmoner odem zemininde gelisen akciger enfeksiyonlari sik gozlenen klinik durumlardir. Tedavisi cerrahidir. Bronsektazi, sik akciger enfeksiyonu gelisen hastalarda gorulebilecek ciddi bir akciger sorunudur. Bu yazida, CCSV, VSD, pulmoner stenoz ve atriyal septal defekt birlikteligi ve sik gecirilen akciger enfeksiyonlarina bagli olarak bronsektazi gelismis bir 20 yasinda erkek olgu sunuldu. Uygulanan kardiyak ameliyatlar ve uygulanabilecek herhangi bir akciger rezeksiyonu sirasinda ortaya cikabilecek risk faktorleri de tartisildi. Anah tar soz cuk ler: Atriyal septal defekt; bronsektazi; cift cikimli sag ventrikul; pulmoner stenoz; ventrikuler septal defekt. Double-outlet right ventricle (DORV) is a very rare anomaly which can be presented with many cardiac abnormalities and ventricular septal defect (VSD) in particular. Clinical findings usually appear in the newborn period, which allows us to diagnose in the early period of life. The clinical findings vary according to the type of accompanying VSD and whether there are additional cardiac abnormalities such as pulmonary stenosis. These patients often present with cyanosis, congestive heart failure findings and pulmonary infections based on interstitial pulmonary edema. The treatment is surgical. Bronchiectasis is a serious pulmonary problem which may be encountered in some patients with frequent pulmonary infections. In this article, a 20-year-old male case with the simultaneous presence of bronchiectasis due to frequent pulmonary infections and DORV, VSD, pulmonary stenosis and atrial septal defect is presented. The risk factors of any pulmonary resection and cardiac surgery applied were also discussed.