Pulmonary Valve Morphology in Patients with Bicuspid Aortic Valves

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.

Early Outcomes of Patients Undergoing Neoaortic Valve Repair Incorporating Geometric Ring Annuloplasty

“…The pulmonary valve is morphologically similar to the aortic valve, [32][33][34] but also has differences, such as lack of a defined fibrous annulus. In addition, it is wedged between the aortic semilunar valve and the ventricular myocardium, all of which lend support.…”

New Concepts in the Development and Malformation of the Arterial Valves

“…For example, Koenraadt et al [ 120 ] suggested that absence of raphe was represented in 25% of Dutch patients with BAV. Moreover, in cases of BPV, the majority also had BAV without raphe, suggesting that whatever process causes bicuspid valves without raphe, it affects both valves at the same time [ 139 ]. However, it should be remembered that a raphe might be present initially and then disappear later, particularly if the fusion event occurred very early in development [ 125 ].…”