Isolated Right Superior Vena Cava Drained to Left Atrium in a Child with Waardenburg Syndrome and Neurofibromatosis Type I

Omer A Algonaid; Yahya H Almashham; Abdulrahman S Almoukirish

doi:10.37616/2212-5043.1293

Isolated Right Superior Vena Cava Drained to Left Atrium in a Child with Waardenburg Syndrome and Neurofibromatosis Type I

J Saudi Heart Assoc. 2022 Mar 10;34(1):11-14. doi: 10.37616/2212-5043.1293. eCollection 2022.

Authors

Omer A Algonaid¹, Yahya H Almashham¹, Abdulrahman S Almoukirish¹

Affiliation

¹ Pediatric Cardiology, King Salman Heart Center, King Fahad Medical City, Riyadh, Saudi Arabia.

Abstract

Isolated right superior vena cava (SVC) draining to the left atrium (RSVC-LA) is an extremely rare cyanotic congenital heart disease (CHD). Such lesion is easily missed with improper scanning or inattentive interpretation of echocardiography. This can result in potential systemic severe complications. We report a child with RSVC-LA who has two significant non-cardiac co-morbidities, including Waardenburg syndrome and n neurofibromatosis type I (NF1). This patient was referred to cardiology assessment due to unexplained low saturation and was diagnosed as RSVC-LA; however, fortunately not yet showing complications of systemic thromboembolic phenomenon (STEP).

Keywords: Cyanosis; Echocardiographic bi-caval view; Left atrium; Neurofibromatosis; Right superior vena cava; Thromboembolism; Waardenburg.

Publication types

Case Reports