PARTIAL ANOMALOUS PULMONARY VENOUS CONNECTION - DIAGNOSIS BY TRANSESOPHAGEAL ECHOCARDIOGRAPHY

Objectives. This study sought to demonstrate that with proper techniqu e, identification of the normal and abnormal pulmonary venous connecti on can be made with confidence using transesophageal echocardiography (TEE). Background. Partial anomalous pulmonary venous connection (PAPV C) is an uncommon congenital anomaly whose diagnosis has classically b een made using angiography. Methods. We performed a retrospective revi ew of all patients of all ages with PAPVC diagnosed at the Mayo Clinic who had undergone TEE because of either right ventricular volume over load or suspected intracardiac shunting by transthoracic echocardiogra phy or intraoperatively. Results. A total of 66 PAPVCs were detected i n 43 patients (1.5/patient); in 2 additional patients, TEE suggested, but did not diagnose, PAPVCs. Shortness of breath was the most common presenting symptom (42.2%), followed by heart murmur and supraventricu lar tachycardia, Right-sided anomalous veins were identified in 35 pat ients (81.4%), left-sided in 7 (16.3%) and bilateral in 1 (2.3%). Ther e was a single anomalous connecting vein in 23 patients (53.5%), two i n 18 (41.9%), three in 1 (2.3%) and four in 1 (2.3%). The connecting s ite was the superior vena cava (SVC) in 39 veins (59.1%), right atrial -SVC junction in 6 (9.1%), right atrium in 8 (12.1%), inferior vena ca va in 1 (1.5%) and the coronary sinus in 2 (3.0%). Ten anomalous left pulmonary veins were connected by a vertical vein to the innominate ve in (15.1%). Sinus venosus atrial septal defect (ASD) was the most comm on associated anomaly in 22 patients (49%), followed by ostium secundu m ASD in 6 and patent foramen ovale in 4. Fifteen patients had an inta ct atrial septum. Thirty one patients (68.8%) underwent surgical repai r. PAPVC was confirmed in all patients, including the two whose TEE re sults were suggestive of PAPVC. All 49 PAPVCs detected by TEE preopera tively were confirmed at the time of operation. Conclusions. TEE is hi ghly diagnostic for PAPVC and can obviate angiography. Accurate anatom ic diagnosis may influence the need for medical and surgical managemen t. TEE should be performed in patients with right ventricular volume o verload when the precordial examination is inconclusive. (C) 1997 by t he American College of Cardiology.