Systematic Evaluation of the Proximal Cardiac Veins for Echocardiographic Diagnosis of Total Anomalous Pulmonary Venous Connection

Purpose: Total anomalous pulmonary venous connection (TAPVC) is a rare congenital cardiovascular malformation that is characterized by an absent connection of the pulmonary veins to the left atrium. Echocardiographic differentiation of TAPVC and persistent Pulmonary hypertension of the newborn (PPHN) can be a great diagnostic challenge. The aim of our Study was the assessment of a systematic echocardiographic approach to evaluate the feasibility and reliability of noninvasive diagnosis of TAPVC. Methods: Between January 1995 and January 2005, 15 consecutive patients with isolated TAPVC were diagnosed in our institution. 5 patients had supracardiac type, 5 cardiac type, 3 infracardiac and 2 had mixed type of TAPVC. Since 2001 preoperative diagnosis was performed exclusively by echocardiography (8/15 patients). Results: TAPVC could be diagnosed correctly in all cases using a systematic echocardiographic approach with careful investigation of the proximal cardiac veins. Infracardiac type of TAPVC was diagnosed using systematic evaluation of the liver with visualization of a pathological vessel with venous flow directed away from the heart. In all cases the echocardiographic findings were confirmed during surgery. Conclusion: Echocardiography with Doppler and color Doppler sonography is an effective method for noninvasive diagnosis of TAPVC The presented systematic echocardiographic approach permits reliable noninvasive differentiation of TAPVC and PPHN.