Clinical profile of patients with Total Anomalous Pulmonary Venous Return and their short term outcome in pediatric cardiac centre at Dhaka Shishu Hospital

Abstract

Background: Total anomalous pulmonary venous return (TAPVR) is an uncommon congenital cardiovascular anomaly with poor natural prognosis without proper intervention. It has been detected more frequently in recent year due to the advent of echocardiography. The aim of this study is to evaluate the clinical manifestations, age at diagnosis and short term outcomes in TAPVR patients. Methods: From 1st January 2013 to 31st December 2013, a total of 34 cases with TAPVR were admitted in pediatric cardiac centre at Dhaka Shishu Hospital, Dhaka, Bangladesh. All of them were evaluated with 2-dimensional (2-D) and color Doppler echocardiography examination. CXR and ECG were also done. Patient's sex, age at diagnosis, types of TAPVR, clinical manifestations, radiological finding, ECG findings and outcomes were compiled and analyzed. Results: In 34 patients with TAPVR, 23 (67.6%) were male and 11 (32.4%) were female with male to female ratio of 2.09:1. Most of the patients were diagnosed between 0-6 months of age that is 13 (38.2%) cases were in 0-2 month's age group, 14 (41.2%) cases were in more than 2 month's to 6 month's age group. Tachypnea and cyanosis were more common symptoms. The types of TAPVR was supra-cardiac 18 (52.9%), cardiac 11 (32.4%), infra-cardiac 3 (8.8%) and mixed in 2 (5.9%) cases. Pulmonary hypertension was present in 31 (91.2%) of 34 cases. Among them, 20 (58.8%) patients had severe pulmonary hypertension. The most common associated intra-cardiac lesions of TAPVR patients were ASD 13 (38.2%) and PFO 13 (38.2%). ECG findings of TAPVR, 18 (52.9%) patient had right axis deviation (RAD), right ventricular hypertrophy (RVH) and 14 (41.2%) had right axis deviation (RAD), right ventricular hypertrophy (RVH), right atrial enlargement (RAE). X-ray findings of TAPVR patients, 32 (94.1%) patients had Cardiomegaly and increased pulmonary vascularity. Among admitted patient, 3 (8.8%) patients died due to pneumonia and intractable heart failure, 31 (91.2%) patients referred to advanced cardiac centre for operative treatment. Conclusions: Tachypnea and Cyanosis were an obvious clinical symptom of TAPVR. 2-D and color Doppler echocardiography can provide quick and accurate diagnostic information of TAPVR. Death rate is high in TAPVR patient in spite of adequate medical management. So, early detection and definitive surgical treatment of TAPVR is much needed.

Late-onset total anomalous pulmonary venous connection in a 70-year-old woman

“…3 4 TAPVC is classified into four types: supracardiac, cardiac, infracardiac and mixed connection. 6 In general, patients with supracardiac or cardiac connection survive longer than those with infracardiac or mixed connection. 7 The major factors that determine the survival of these patients are the size of the ASD and pulmonary arterial pressure.…”

Perioperative Anesthetic Management for Surgical Repair of an Adult with Supracardiac Total Anomalous Pulmonary Venous Communication and Pulmonary Hypertension: A case report and Mini Review

“…Neonates with severe obstructive TAPVC present with marked cyanosis, tachypnoea, dyspnoea, metabolic acidosis, low cardiac output, and vulnerable to death, and require an emergent intervention [11]. In addition neonates with obstructive TAPVC have a tendency to develop extraordinarily vulnerable PAH due to Pulmonary vascular changes in form of increased arterial medial thickness, intimal proliferation in preacinar veins, and abnormally small and thick-walled extrapulmonary veins [12].…”

Infracardiac type total anomalous pulmonary venous return with obstruction and dilatation of portal vein

“…This type of congenital cardiovascular malformation is more frequently seen in patients with heterotaxy syndromes, especially asplenia or polysplenia [3] , [4] , [8] . The malformation usually presents itself accompanied by neonatal cyanosis and tachypnea [9] and if a systemic-pulmonary shut (right to left heart shunt) is not performed, it may be a cause of considerably mortality [1] , [4] ; typical shunts occur by an atrial septal defect (patent foramen ovale) or patent ductus arteriosus (less common), with these communications being almost obligatory for survival [1] , [2] , [4] , [8] . TAPVR is a consequence of the persistence of the primitive connections of the pulmonary veins with the cardinal systemic veins, which results in a failure of common pulmonary veins to connect to the left atrium [10] , [11] .…”