Congenital aortic insufficiency with ascending aortic aneurysm: Dealing with a challenging case. (December 2019)

• Record Type: Journal Article
• Authors: Raissouni, K.; Henaine, R.; Ninet, J.
• Is Part Of: Progress in pediatric cardiology. Volume 55(2019)
• Publication Date: 2019-12
• Subjects: Echocardiographic prenatal diagnosis -- Congenital aortic insufficiency -- Aortic valve leaflet agenesia -- Anomalous left coronary ostium -- Ascending aortic aneurysm -- Mechanical Bentall; Pediatric cardiology -- Periodicals; Cardiovascular Diseases -- Periodicals; Infant; Child; Cardiologie pédiatrique -- Périodiques; 618.9212005
• DOI: 10.1016/j.ppedcard.2019.04.003
• Languages: English
• ISSNs: 1058-9813

Abstract

Abstract: Congenital aortic insufficiency is a challenging condition; without appropriate management from birth, it can lead to left ventricle dilation and heart failure. Aortic insufficiency due to leaflet agenesia is rarely described in literature. We report here a case of severe congenital aortic regurgitation that required surgical treatment until the boy reached five-year-old thanks to prenatal diagnosis and adequate medical care since birth. The patient underwent a mechanical Bentall procedure for associated dystrophic pulmonary valve with mild stenosis, ascending aortic aneurysm of 34 mm and anomalous position of the left coronary ostium. We safely performed a rare paediatric procedure with encouraging short-term results. Highlights: Prenatal diagnosis of aortic regurgitation helps pediatric team in providing the best medical care from birth and post pone a necessary cardiac surgery until the newborn grows up. Aortic regurgitation in children is a challenging condition; without appropriate management, it can lead to left ventricle dilation and heart failure. Depending on the patient''s age, different surgical options can be considered. The Ross procedure remains the best choice for aortic valve replacement in infants with severe aortic insufficiency when it is technically feasible.