Total Anomalous Pulmonary Venous Connection with Severe Pulmonary Venous Obstruction: A Clinical Entity

摘要: The clinical, physiologic, and anatomic features of a special group cases total anomalous, pulmonary venous connection are reviewed. occurrence severe obstruction in anomalous produces characteristic syndrome: 1. Very early onset dyspnea heart failure. 2. Considerably more cyanosis than the usual case without obstruction. 3. Typical x-ray showing diffuse hazy lung fields with reticulated appearance cardiac enlargement. 4. Electrocardiographic evidence right ventricular hypertrophy tall R or qR pattern lead V 1 reversal R/S ratio over precordium. 5. Physiologic findings practically normal flow, arterial pressures greater systemic, marked gradient between "wedge" atrial pressures. 6. Rapid deterioration death first weeks months life.