Bicuspid Aortic Valves in the Young, a Descriptive, Retrospective Study

Background: Limited information about bicuspid aortic valves in the young (BAV) is available. BAV give risk of development of valve pathology, and dilatation of the ascending aorta. It can be seen in conjunction with cardiac malformations and syndromes. BAV know several morphologic phenotypes, which influences course and prognosis.

Aim: To gain more knowledge about bicuspid aortic valves in the young and to give an overview of prevalence and morphology of BAV in children.

Method: Retrospective study, in the Radboud University Medical Center, Nijmegen, Netherlands. Patients with BAV in 2005–2013, aged between 1 and 18 years, were identified by use of the echocardiography database. Medical records and most recent echocardiography were reviewed. Classification of valve morphology was performed by use of a modified Sievers classification system.

Results: BAV was reported in 286 patients. In 251 patients (2.1% of 11792 patients) BAV was actually present. Mean age at inclusion: 5.8 (±4.8) years, body surface area 0.81 (±0.43). The majority was male (N = 161, 64.1%). Minority 29 (11.6%) had a syndrome. Valve morphology was most often classified as type 0, AP (42.4%). Within type 1, RN had more valve pathology (p < 0.001). Valve pathology was present in 136 patients, a minority (N = 37) patients received valve intervention, mean age at first valve intervention was 1.99 years (±3.36 SD).

Conclusion: Importance of enhancing knowledge concerning BAV in pediatric population is clearly demonstrated by this study. Valve pathology was seen in the majority of patients and importance of the determination of valve morphology is underlined. Interventions due to valve pathology were seen in the minority and were mostly conducted at young age. Morphology type was related to occurrence of valve pathology and intervention. Additionally, the application of the modified Sievers classification system seemed to be of added value in clinical practice.