Dima Bsat, Dalia Safi, Jad Abdul Khalek, Issam El Rassi, Mariam Arabi
American University of Beirut Medical Center. Al Jalila Children’s Hospital.
Lebanon and United Arab Emirates
Cardiology in Review
Cardiol Rev 2026;
DOI: 10.1097/CRD.0000000000001284
Abstract
Sinus venosus atrial septal defect is a rare congenital heart defect that is frequently associated with partial anomalous pulmonary venous return (PAPVR). This defect is categorized into 2 types: superior vena cava (SVC) type and inferior vena cava type, with the SVC type being more common. The condition results in abnormal left-to-right shunting of blood, leading to right heart dilation and increased pulmonary blood flow. A comprehensive literature search was performed using PubMed, MEDLINE Ovid, and Embase, focusing on studies involving patients with sinus venosus atrial septal defect, with or without PAPVR, published in English. Data were collected on patient demographics, the type of sinus venosus atrial septal defect, comorbidities, diagnostic imaging methods, and treatment outcomes. The search aimed to gather information on the clinical presentations, diagnostic challenges, and treatment options for this congenital defect. Sinus venosus atrial septal defect accounts for 5-10% of all atrial septal defects. Most cases involve the SVC type, which is often associated with PAPVR. Patients commonly present with symptoms such as dyspnea, fatigue, palpitations, and exercise intolerance. Comorbidities such as Lutembacher syndrome, cor triatriatum dexter, and pulmonary hypertension were observed. Diagnostic methods included transthoracic echocardiography, transesophageal echocardiography, cardiac magnetic resonance imaging, and computed tomography angiography, with transesophageal echocardiography being the most sensitive. Sinus venosus atrial septal defect is a clinically significant condition that can lead to serious complications like atrial arrhythmias and pulmonary hypertension. Early diagnosis using advanced imaging techniques and surgery is essential for preventing long-term morbidity.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Review Articles Concerning Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease or Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: No