Unusual presentation of total anomalous systemic venous connection

Abstract

A 9-year-old girl who presented with dyspnea on exertion was diagnosed with total anomalous systemic venous connection to the left atrium (both venae cavae), no left superior vena cava, and a moderate-sized atrial septal defect with severe pulmonary arterial hypertension and ectopic atrial rhythm. She underwent septation of the common atrium using autologous pericardium, thereby rerouting the superior vena cava, inferior vena cava, and coronary sinus to the right atrium. Her postoperative course was uneventful. This case is reported for its rarity of presentation with severe pulmonary arterial hypertension and ectopic atrial rhythm.

Prenatal diagnosis of isolated total anomalous systemic venous connection to the left atrium

“…Total abnormal systemic venous return requires a left-to-right shunt through an atrial septal defect, a ventricular septal defect, or a patent arterial duct in order to ensure pulmonary circulation. A large atrial septal defect was present in all patients of studies previously published [3][4][5][6]8 except for two of them. A 7-year-old girl had a large perimembranous ventricular septal defect, 7 and a 2-year-old boy had only a small atrial septal defect.…”

“…Most cases of total anomalous systemic venous connection to the left atrium reported in the literature were associated with left isomerism. [5][6][7][8] On surgical inspection of the appendage of the right-sided atrium, we found that it exhibited the anatomical characteristics of a left-atrial appendagenamely, a long narrow chamber with a smooth-looking wall, possibly indicating heterotaxy syndrome with left-atrial isomerism. The chest X-ray, however, did not show bilateral symmetric left bronchi with widened carina as typically observed in left bronchial isomerism.…”

“…Among 100 patients with anomalous systemic venous connections undergoing surgery, only 3 patients have a total anomalous systemic venous connection to the left atrium. 2 Only a few cases have been reported thus far, [3][4][5][6][7][8] and the literature on this subject has more of a surgical perspective. Most patients presented later in life, from childhood to adulthood, mainly with cyanosis.…”

Clarifying the anatomy and physiology of totally anomalous systemic venous connection

“…[ 2 3 4 5 6 7 8 9 ] On the other hand, the presence of a large shunt allows good oxygenation, together with the growth of RV, thus permitting complete surgical repair. [ 9 10 11 12 13 14 15 16 17 18 19 ] The presence of cyanosis despite left-to-right shunt across an ASD, in the setting of a hypoplastic RV in hearts with concordant atrioventricular and ventriculoarterial connections, is almost diagnostic of totally anomalous systemic venous connection (TASVC). Unfortunately, even in the current era of modern echocardiography, despite many clues, the diagnosis is often delayed.…”

“…Unfortunately, even in the current era of modern echocardiography, despite many clues, the diagnosis is often delayed. [ 5 8 11 12 14 15 20 ] Not uncommonly, the diagnosis is missed completely,[ 10 ] and the arrangement is misinterpreted as functionally single ventricle. It is also not uncommon to find that anomalous connection of only one caval vein, generally the superior caval vein (SCV), is correctly identified mainly because saline contrast echocardiography is generally performed only from the upper limb.…”