Coronary anatomy in children with bicuspid aortic valves and associated congenital heart disease

Koenraadt, Wilke M C; Bartelings, Margot M.; Bökenkamp, Regina; Groot, Adriana C. Gittenberger‐de; DeRuiter, Marco C.; Schalij, Martin J.; Jongbloed, Monique R.M.

doi:10.1136/heartjnl-2017-311178

Cited by 14 publications

(16 citation statements)

References 33 publications

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“…The spectrum of left ventricular outflow tract obstructive lesions includes a number of malformations other than a bicuspid aortic valve, including mitral valve abnormalities, subaortic stenosis, and coarctation of the aorta. 3 These congenital defects may appear in isolation or as a constellation of abnormalities in the complete Shone's complex and hypoplastic left heart syndrome, conditions that can be seen in females with Turner syndrome. 6 The fact that females with Turner syndrome have a very frequent occurrence of a bicuspid valve (15-30%), 15 particularly in the 45, X karyotype, points towards the importance of genes on the X chromosome for development of the bicuspid valve.…”

Section: Geneticsmentioning

confidence: 99%

Clinical and pathophysiological aspects of bicuspid aortic valve disease

et al. 2018

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A bicuspid aortic valve is not only a common congenital heart defect but also an enigmatic condition that can cause a large spectrum of diseases, such as aortic valve stenosis and severe heart failure in newborns whereas aortic dissection in adults. On the contrary, a bicuspid aortic valve can also occur with normal function throughout life and never need treatment. Numerous genetic mechanisms are involved in the abnormal cellular functions that may cause abnormal development of the aortic valve during early foetal life. As several chromosomal disorders are also associated with a bicuspid valve, there does not appear to be an apparent common trigger to the abnormal development of the aortic valve. The clinical care of the bicuspid aortic valve patient has been changed by a significant body of evidence that has improved the understanding of the natural history of the disease, including when to best intervene with valve replacement and when to provide prophylactic aortic root surgery. Moreover, as bicuspid valve disease is also part of various syndromes, we can identify high-risk patients in whom a bicuspid valve is much more unfavourable than in the normal population. This review provides an overview of all aspects of the bicuspid aortic valve condition and gives an updated perspective on issues from pathophysiology to clinical care of bicuspid aortic valve disease and associated aortic disease in asymptomatic, symptomatic, and pregnant patients, as well as our viewpoint on population screening.

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Section: Geneticsmentioning

confidence: 99%

Clinical and pathophysiological aspects of bicuspid aortic valve disease

et al. 2018

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“…In some cases, cardiovascular anomalies could not be assessed due to incompleteness of the specimens. Coronary anatomy in these hearts was described in a separate paper [ 12 ].…”

Section: Methodsmentioning

confidence: 99%

Pulmonary Valve Morphology in Patients with Bicuspid Aortic Valves

et al. 2018

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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.

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“…Various mechanisms are involved including cystic medial necrosis, fragmentation of elastic fibers or the loss of smooth muscle cells in the ascending aorta wall [4] . The association between BAV and coronary anomalies has been described as a rare finding in the current literature [5][6][7] . We report a case of BAV stenosis associated with an ascending aortic aneurysm and a positional anomaly of the left main coronary ostium.…”

Section: Introductionmentioning

confidence: 93%

Aortopathy and coronary anomaly in bicuspid aortic valve: an uncommon tricky association

Salsano¹,

Natali²,

Parolari³

et al. 2020

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Bicuspid aortic valves (BAV) can be associated with aortopathy and coronary anomalies. We report the case of a 60 year-old woman undergoing surgery for severe aortic stenosis due to BAV and an ascending aortic aneurysm. During the procedure, an uncommon anomalous origin of the left main coronary artery from the posterior commissure with intramural takeoff of the left coronary artery was found. Routine pre-operative coronary angiography had failed to identify this anomaly. To avoid ischemic events or left main coronary lesions, we placed the aortic bioprosthesis by respecting the commissures, not to occlude the anomalous coronary ostium. The association of BAV, aortopathy and coronary anomalies is a rare finding. Awareness of the anatomy of the coronary arteries in patients with BAV should be considered mandatory to avoid catastrophic consequences and to select the appropriate surgical procedure.

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Coronary anatomy in children with bicuspid aortic valves and associated congenital heart disease

Cited by 14 publications

References 33 publications

Clinical and pathophysiological aspects of bicuspid aortic valve disease

Clinical and pathophysiological aspects of bicuspid aortic valve disease

Pulmonary Valve Morphology in Patients with Bicuspid Aortic Valves

Aortopathy and coronary anomaly in bicuspid aortic valve: an uncommon tricky association

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