Bicuspid aortic valve phenotype 1-RN was most associated with aortic stenosis, while the 1-RL morphology was most associated with other congenital anomalies in pediatric and young adult patients.
Observational (n=114)
Blinded assessors
Does valvular phenotype affect functional parameters and associated congenital anomalies in pediatric and early adult patients with bicuspid aortic valve?
Cardiac MRI demonstrates that bicuspid aortic valve phenotypes correlate with specific clinical manifestations, such as aortic stenosis in 1-RN morphology and associated congenital anomalies in 1-RL morphology.
Our study is a prospective study aiming to assess congenital bicuspid aortic valve using cardiac MRI and to detect a relationship between the leaflet fusion pattern and other functional parameters including valvular regurge, stenosis and pressure gradient. This prospective study included 114 cases 104 of them had bicuspid aortic valve, diagnosed by echocardiography while the rest (10 cases) were normal control cases. All functional and morphological cardiovascular abnormalities were recorded as well as any associated congenital diseases. Two different radiologists read the MRI blindly to each other. Patients age ranged from 40 days up to 20 years with 62 patients being in the pediatric age group (<12 years). They were 78males and 26 females. We found 54 cases (53.8%) of 1-RL morphology, 34 cases (32.7%) of 1-RN morphology, 8 cases (7.7%) of 0-AP and 6 cases (5.8%) of 0-Lateral. Aortic stenosis and regurgitation were found in 80.75% of cases. Aortic stenosis was the most common valvular lesion being more evident in the 1-RN valve type. Aortic regurge was more predominant in the pediatric age group with no predilection for a specific valve phenotype. Left ventricular function was normal in most of our cases with only 10 cases showing impairment due to long standing valvular lesion. Thirty-eight cases (36.5%) had associated aortic dilatation. Twenty-six patients (25%) showed associated aortic coarctation. Intra cardiac shunts (24 patients 23%) were the second most common associated anomaly after coarctation. Most of the associated congenital anomalies were found with the 1-RL valve morphology type. Other associated congenital anomalies were detected in 44 patients (42.3%). Cardiac Magnetic resonance should be performed for patients with bicuspid aortic valve to determine the valve phenotype, function and aortopathy for risk stratification and surgical management. Patients with RL type should be properly imaged to detect associated congenital anomalies.
Behairy et al. (Fri,) conducted a observational in Congenital bicuspid aortic valve (n=114). Bicuspid aortic valve phenotype vs. Normal controls / other phenotypes was evaluated on Relationship between leaflet fusion pattern and functional parameters (valvular regurge, stenosis, pressure gradient) and associated congenital anomalies. Bicuspid aortic valve phenotype 1-RN was most associated with aortic stenosis, while the 1-RL morphology was most associated with other congenital anomalies in pediatric and young adult patients.