Age at demise ranged from foetal to 24 years. Of the 83 patients, 24 died of cardiac causes, for example, heart failure or arrhythmia. Twenty-five patients died perioperative or immediately postoperative.
Twenty-four patients died of non-cardiac causes, such as chromosomal abnormalities, intracerebral bleeding or pulmonary problems. For ten patients, cause of death was unknown. Characteristics of the specimens are listed in Table 1. The majority of the 83 included specimens had associated congenital anomalies Table 1. Examples are shown in Fig. Of these, 1 pulmonary valve was also strictly bicuspid, the other 4 did have a raphe. Given the common developmental background of the aortic and pulmonary valves both derived from outflow tract cushions with contributions of endocardium-derived cells, neural crest cells [ 10 ] and, possibly, epicardial-derived cells [ 14 ], one could expect that in case of BAV also the pulmonary valve is affected.
Almost two-third of the hearts with an abnormal pulmonary valve had a strictly bicuspid aortic valve, indicating a potential predisposition of maldevelopment of the pulmonary valve in a subgroup of patients with BAV Type B. Although the mechanism is unclear at this point, it seems that in cases where tripartition of the valve is severely disturbed to the extent that an anatomical third leaflet and a raphe cannot be recognized, the development of the pulmonary valve that evolves from the same endocardial cushions that contribute to the aorta during development, Fig.
The association with chromosomal abnormalities, especially a higher incidence of Trisomy 18, is in accordance with previous studies [ 9 , 15 , 16 ].
In addition, over one-third of specimens with abnormal pulmonary valve morphology had a chromosomal abnormality, and all of these had a BAV Type B. We conclude that bilateral semilunar valve disease is associated with Type B BAVs, in many cases related to chromosomal abnormalities. This study was performed in post-mortem specimens with high frequency of associated CHD and therefore cannot be applied to the general BAV population.
All authors take responsibility for all aspects of the reliability and freedom from bias of the data presented and their discussed interpretation.
This study was performed in accordance with the local ethics committee and Dutch regulations for proper use of human tissue for medical research purposes as noted in the Methods section. National Center for Biotechnology Information , U.
Pediatric Cardiology. Pediatr Cardiol. Published online Jan Wilke M. Koenraadt , 1 Margot M. Bartelings , 2 Adriana C. DeRuiter , 2 Martin J. Schalij , 1 and Monique R. Jongbloed 1, 2. Margot M. Adriana C. Gittenberger-de Groot. Marco C. Martin J. Monique R. Author information Article notes Copyright and License information Disclaimer. Corresponding author. For patients with BAVD and regurgitation, our cardiac surgeons repair the aortic valve. Valve replacement is recommended for bicuspid aortic valve with stenosis.
Our team of specialists treats more people with aortic disease than any other center in the area. The Center for Bicuspid Aortic Valve Diseases offers innovative treatments and techniques as well as a personalized care plan for patients with bicuspid aortic valve disease. Read More. Jerry lives a very active lifestyle and a veteran of many marathons and triathlons. After an EKG for a life insurance policy, Jerry was told he had a heart problem, which came as a surprise since he is so active.
Access myPennMedicine For Patients and Visitors. Patient Information. Conditions Treated A-Z. Bicuspid Aortic Valve Disease. Bicuspid Aortic Valve Symptoms A bicuspid aortic valve is often not symptomatic. Those symptoms may include: Chest pain: Discomfort and tightness are often associated with aortic stenosis. Fatigue: Increased activity may leave you feeling tired and weak. Biological valve replacement In biological valve replacement, a valve made from cow, pig or human heart tissue replaces the damaged valve.
Mechanical valve replacement Open pop-up dialog box Close. Mechanical valve replacement In mechanical valve replacement, a mechanical valve replaces the damaged valve. Share on: Facebook Twitter. Show references Bonow RO, et al. Aortic valve disease. Elsevier; Accessed March 18, Heart valve disease. National Heart, Lung, and Blood Institute.
Aortic valve stenosis AVS and congenital defects. American Heart Association. Braverman AC. Clinical manifestations and diagnosis of bicuspid aortic valve in adults. Bicuspid aortic valve: General management in adults.
What is an aneurysm? Burke CR, et al. Management of thoracic aortic aneurysm in adults. Bicuspid aortic valve. Mayo Clinic; Related Adult Congenital Heart Defects Adult congenital heart disease: What patients and families should know Infographic: Congenital Heart Disease and Lifelong Care Kinser's story Robotic or minimally invasive cardiac surgery for adult-adolescent congenital heart disease Saved from Transplant Show more related content.
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