A 3-week-old male born at term presents to the emergency department with cyanosis. The mother reports poor feeding and rapid breathing since day of life 2. Vital signs show SpO2 58% on room air, heart rate 148/min, respiratory rate 46/min, and blood pressure 64/40 mmHg. On examination, the infant is alert but cyanotic with a single loud S2 and no murmur. A hyperoxia test (FiO2 1.0) shows minimal improvement in SpO2 to 61%. Chest X-ray demonstrates a right aortic arch with a narrow mediastinum and "egg-on-string" appearance. Echocardiography shows all four pulmonary veins draining to a confluence posterior to the left atrium with no connection to the left atrium. The patient receives prostaglandin E1 and undergoes balloon atrial septostomy with minimal improvement in oxygenation. Which of the following is the most likely diagnosis?
- A)Total anomalous pulmonary venous return (supracardiac type)GABARITO
- B)Transposition of the great arteries with intact ventricular septum
- C)Tricuspid atresia with severe restrictive atrial septum
- D)Pulmonary atresia with intact ventricular septum
- E)Critical tetralogy of Fallot
Explicação
Total anomalous pulmonary venous return (TAPVR) with supracardiac type is the correct diagnosis. The clinical presentation of profound cyanosis refractory to PGE1 and balloon atrial septostomy is pathognomonic for TAPVR. All pulmonary veins drain to a confluen... Ver explicação completa e trilha adaptativa →