Abstract
The aortic and pulmonary valve share a common developmental origin from the embryonic arterial trunk. Bicuspid aortic valve is the most common congenital anomaly and can occur isolated as well as in association with other congenital heart disease (CHD). Data on pulmonary valve morphology in these cases are scarce. In this study, we aimed to determine pulmonary valve morphology in hearts with BAV associated with CHD. In 83 post-mortem heart specimens with BAV and associated CHD, pulmonary valve morphology was studied and related to BAV morphology. In 14/83 (17%) hearts, the pulmonary valve was affected, bicuspid in 8/83 (10%), dome-shaped in 3/83 (4%) and atretic in 3/83 (4%). In specimens with a bicuspid pulmonary valve, 5/8 (63%) had a strictly bicuspid aortic valve (without raphe), 2/3 hearts (67%) with dome-shaped pulmonary valves and 2/3 hearts (67%) with atretic pulmonary valves had BAV without raphe. Six out of eight (75%) specimens with a bicuspid pulmonary valve had a perimembranous ventricular septal defect (VSD). 4/8 (50%) specimens with a bicuspid pulmonary valve were associated with chromosomal abnormalities: 3 (38%) had trisomy 18 and 1 (13%) had trisomy 13. In BAV with associated CHD, abnormal pulmonary valve morphology was observed in 17% of the hearts. The majority of hearts with abnormal pulmonary valve morphology had a Type B bicuspid aortic valve (without raphe). Bilateral semilunar valvular disease is associated with Type B BAVs and in many cases related to chromosomal abnormalities. As this study was performed in post-mortem specimens with high frequency of associated CHD, caution is warranted with application of these results to the general BAV population.
Highlights
Bicuspid aortic valve (BAV) is the most common congenital anomaly, occurring in 1–2% of the general population [1], whereas bicuspid pulmonary valves are rare [2]
The aim of the current study is to describe the incidence of abnormal pulmonary valve morphology in post-mortem hearts with BAV and associated congenital heart disease (CHD), and to correlate findings with BAV morphology
Of the 8 specimens with a bicuspid pulmonary valve, 6 (75%) had a perimembranous ventricular septal defect (VSD). 4/8 (50%) specimens with a bicuspid pulmonary valve were associated with chromosomal abnormalities: 3 (38%) had trisomy 18, 1 (13%) had trisomy 13 and these all had a Type B BAV
Summary
Bicuspid aortic valve (BAV) is the most common congenital anomaly, occurring in 1–2% of the general population [1], whereas bicuspid pulmonary valves are rare (incidence of 0.1%) [2]. One study in human post-mortem hearts reports an incidence of 12%, mostly in hearts with associated congenital heart disease (CHD) [9]. Given the common developmental origin of aorta and pulmonary valves [10], the question arises whether pulmonary valve morphology is affected in specific subgroups of BAV patients. This does not necessarily seem the case. Animal studies have pointed out that bicuspid aortic and pulmonary valves may have a
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