تترالوژی فالوت به همراه فقدان دریچه پولمونر: گزارش موردی

عنوان انگليسی

TETRALOGY OF FALLOT WITH ABSENT PULMONARY VALVE (APV): A CASE REPORT

خلاصه انگلیسی

Introduction: Tetralogy of fallot (TOF) is the most common cyanotic congenital heart disease, but TOF with absent pulmonary valve is a very rare form of TOF and despite classic form of TOF, here aneurysmal pulmonary arteries may lead to airflow obstruction. Case presentation: A term male baby was born after normal pregnancy. He presented with respiratory distress and cyanosis. On examination exhibited stridulous respiration,prominent left parasternal impulse, harsh"To-and-Fro" systolic and diastolic murmur and single second heart sound. Chest x-ray showed enlarged cardiac silhouette that had a prominent bulge at the left cardiac border, subsequenced by dilated main pulmonary artery and branches. Echocardigraphy showed large conoventricular septal defect, overriding aorta, right ventricular enlargement and aneurysmal main and branch pumonary arteries. Pumonary vavle was absent, but a ring of tissue was present at the level of pulmonary valve. Spectral doppler across the pulmonary valve revealed severe pulmonary regurgitation and stenosis. Our patient died after 24 hours because of respiratory problems. Conclusion: Tetralogy of fallot with absent pulmonary valve is a rare condition and unlike classic form of TOF presented as cardiomegaly and signs of airway obstruction. Patients who exhibit severe respiratory compromise early in life will require urgent intervention and have a worse outcome.

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