Anesthetic implications of total anomalous systemic venous connection to left atrium with left isomerism

Abstract

Total anomalous systemic venous connection (TASVC) to the left atrium (LA) is a rare congenital anomaly. An 11-year-old girl presented with complaints of palpitations and cyanosis. TASVC with left isomerism and noncompaction of LV was diagnosed after contrast echocardiogram and computed tomography angiogram. The knowledge of anatomy and pathophysiology is essential for the successful management of these cases. Anesthetic concerns in this case were polycythemia, paradoxical embolism and rhythm abnormalities. The patient was successfully operated by rerouting the systemic venous connection to the right atrium.

Total anomalous systemic with partial anomalous pulmonary venous connections

“…TASVC is usually associated with atrioventricular septal defect, common atrium, both primum as well as secundum types of ASD, and heterotaxia. 1,[6][7][8] Cyanosis and paradoxical embolic events are often the presenting features. 1-8 Two-dimensional transthoracic and transesophageal echocardiography when combined with different bolus injections of contrast from the left and right peripheral arm and femoral veins, provides a reliable method of assessment of systemic venous connections.…”

“…1,[6][7][8] Cyanosis and paradoxical embolic events are often the presenting features. [1][2][3][4][5][6][7][8] Two-dimensional transthoracic and transesophageal echocardiography when combined with different bolus injections of contrast from the left and right peripheral arm and femoral veins, provides a reliable method of assessment of systemic venous connections. 2,3 Angiocardiography is required only for confirmation of the diagnosis.…”

“…Such cases require preanaesthetic management of polycythemia and altered coagulation profile along with prevention of arrhythmia and air embolism. 7,8 The surgical challenges include the institution of CPB. [4][5][6] Zhang and colleagues 6 classified TASVC into 2 types according to the type of vena cava cannulation.…”

“…All systemic venous flow, including the right superior vena cava (RSVC), persistent left superior vena cava (LSVC), inferior vena cava (IVC), and coronary sinus, drains abnormally into the heart. [1][2][3][4][5][6][7][8] This disorder is usually associated with an atrioventricular canal, common atrium, atrial septal defect (ASD), ventricular septal defect, and heterotaxia. [3][4][5][6] Approximately 20 such cases have been reported in the English literature hitherto.…”

“…[3][4][5][6] Approximately 20 such cases have been reported in the English literature hitherto. [6][7][8] The association of TASVC with partial anomalous pulmonary venous connection (PAPVC) has not been reported. We discuss the successful surgical treatment of a case of TASVC with PAPVC.…”