Feray GÜVEN, Emine KAVAS, Recep KAVAS, Nilgün GÖKYAYLA, Nazan ERBİL, Aysu SAY

Pulmoner kapak yokluğu ile birlikte olan fallot tetralojili bir olgu

Pulmoner kapak yokluğu ile birlikte olan Fallot tetralojisi (TOF/APV) nadir görülen bir konjenital kalp anomalisidir. Pulmoner kapak yaprakçıkları yokluğu ve pulmoner arterlerdeki anevrizmal dilatasyon dışında tüm kardiyak yapılar Fallot tetralojisindekine benzer. Fallot tetralojisinde bulunan ventriküler septal defekt, infundibular pulmoner stenoz, ve ata binen aort mevcuttur. Bu hastalarda klinik tabloya pulmoner regürjitasyona bağlı bulgular hakimdir. Pulmoner arterlerdeki anevrizmal dilatasyona bağlı respiratuar distress gelişir. Siyanoz Fallot tetralojisindeld kadar belirgin değildir. Kesin tanı ekokardiyografi ile konulur. Tedavi cerrahi onarımdır. Biz bu yazımızda hastanemizde doğan ve yenidoğan yoğun bakım ünitemizde teşhis edilen bir TOF/APV olgusunu sunmaktayız.

Anahtar Kelimeler:

Olgu sunumu, Fallot tetralojisi, Pulmoner kapak, Süt çocuğu, yenidoğan

A case report: Tetralogy of fallot with absent pulmonary valve

Tetralogy of Fallot with absent pulmonary valve is a rare congenital heart anomaly. Pulmoner leaflets are absent and there's the aneurysmal dilatations of the tetralogy of Fallot. There's the usual malalignment ventricular septal defect, infundibular pulmonary stenosis, and overriding of the aorta. In these cases pulmonary regurgitation dominates the clinical picture. Respiratory distress is prominant due to aneurysmal dilatation. Cyanosis is slightly seen in these infants comparing with the echocardiography. Treatment is surgery. Here we report a neonate born in our hospital and diagnosed as TOF/APV in our NICU.

Keywords:

Case report, Tetralogy of Fallot, Pulmonary Valve, Infant, Newborn,

___

1. Fyler D: Tetralogy of Fallot with absent pulmonary valve: Nadas' Pediatric Cardiology, 1992; 486-491
2. Jekel L, Benatar A, Bennik GB, et al: Tetralogy of Fallot with absent pulmonary valve. A continuing challenge. Scand Cardiovasc J, 1998; 32(4): 213-217
3. Liang CC, Tsai CC, Hseuh C, et al: Prenatal diagnosis of tetralogy of Fallot with absent pulmonary valve accompanied by hydropsfetalis. Gynecol Obstet Invest, 1997; 44(1): 61-63
4. Ram SP, Lim MK, Mazeni A: Absent pulmonary valve syn¬ drome: a case report. MedJ Malaysia 1994; 49(1): 93-95
5. Kakizawa H, Ohno T, Ozawa A, et al: Tetralogy of Fallot with absent pulmonary valve: evaluation with magnetic resonance imaging. Tohoku J Exp Med,1997;182(l): 35-39
6. Gustafson RA, Murray GF, Warden HE. et al: Early repair of tetralogy of Fallot. Ann Thorac Surg, 1988; 45: 235-241
7. Fujino S, Hoshino S, Harada Y, et al: Tetralogy of Fallot with absent pulmonary valve: a successful surgical case at 40 days after birth. Nippon Kyobu Geka Gakkai Zasshi, 1993; 41(11): 2239-2244
8. Subramanian V, Anstead M, Cottrill CM, et al: Tetralogy of Fallot with absent pulmonary valve and bronchial compression: treatment with endobronchial stents. Pediatr Cardiol, 1997; 18(3): 237-239
9. McDonnell BE, Raff GW, Gaynor JW, et al: Outcome after repair of tetralogy of Fallot with absent pulmonary valve. Ann Thorac Surg, 1999; 67(5): 1391-1395