Coronary artery dilation associated with bicuspid and unicuspid aortic valve disease in children: a series of 17 patients.

INTRODUCTION Coronary artery dilation associated with bicuspid/unicuspid aortic valves is described in adults with limited data in children. We aimed to describe the clinical course of children with bicuspid/unicuspid aortic valves and coronary dilation including coronary Z-score changes over time, association of coronary changes with aortic valve anatomy/function, and complications. MATERIALS AND METHODS Institutional databases were searched for children ≤18 years with both bicuspid/unicuspid aortic valves and coronary dilation (1/2006-6/2021). Kawasaki disease and isolated supra-/subvalvar aortic stenosis were excluded. Statistics were descriptive with associations measured by Fisher's exact test and overlapping 83.7% confidence intervals. RESULTS Of 17 children, bicuspid/unicuspid aortic valve was diagnosed at birth in 14 (82%). Median age at coronary dilation diagnosis was 6.4 years (range: 0-17.0). Aortic stenosis was present in 14 (82%) [2 (14%) moderate, 8 (57%) severe]; 10 (59%) had aortic regurgitation; 8 (47%) had aortic dilation. The right coronary was dilated in 15 (88%), left main in 6 (35%), and left anterior descending in 1 (6%) with no relationship between leaflet fusion pattern or severity of aortic regurgitation/stenosis on coronary Z-score. Follow-up evaluations were available for 11 (mean 9.3 years, range 1.1-14.8) with coronary Z-scores increasing in 9/11 (82%). Aspirin was used in 10 (59%). There were no deaths or coronary artery thrombosis. DISCUSSION In children with bicuspid/unicuspid aortic valves and coronary dilation, the right coronary artery was most frequently involved. Coronary dilation was observed in early childhood and frequently progressed. Antiplatelet medication use was inconsistent, but no child died nor developed thrombosis.

• Publication year

  2023

• Venue

  Cardiology in the Young

• Publication date

  2023-04-20

• Fields of study

  Medicine

• Identifiers
  DOI 10.1017/S104795112300077XPMID 37078183
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Summary: International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional, and research purposes.

  2021cited by this paper
• Unicuspid aortic valve: Case series and review

  2020cited by this paper
• Predictors of Bicuspid Aortic Valve–Associated Aortopathy in Childhood

  2020cited by this paper
• 2020 ACC/AHA Guideline for the Management of Patients With Valvular Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines.

  2020cited by this paper
• Disease progression and variation in clinical practice for isolated bicuspid aortic valve in children

  2018cited by this paper
• Clinical and pathophysiological aspects of bicuspid aortic valve disease

  2018cited by this paper
• Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association

  2017cited by this paper
• Structural Measurements and Adjustments for Growth

  2016cited by this paper
• Coronary Artery Ectasia Are Frequently Observed in Patients With Bicuspid Aortic Valves With and Without Dilatation of the Ascending Aorta

  2016cited by this paper
• Histopathology of aortic complications in bicuspid aortic valve versus Marfan syndrome: relevance for therapy?

  2015cited by this paper
• Aortopathy is prevalent in relatives of bicuspid aortic valve patients.

  2009cited by this paper
• Coronary Artery Dilatation and Aortic Outflow Tract Enlargement in Children with Unicommissural Aortic Valves

  2005cited by this paper
• Congenital coronary artery dilatation

  2003cited by this paper
• Abnormal Extracellular Matrix Protein Transport Associated With Increased Apoptosis of Vascular Smooth Muscle Cells in Marfan Syndrome and Bicuspid Aortic Valve Thoracic Aortic Aneurysm

  2003cited by this paper
• The incidence of congenital heart disease.

  2002cited by this paper
• Exercise-induced myocardial ischemia in patients with coronary artery ectasia without obstructive coronary artery disease.

  2001cited by this paper
• Doppler evaluation of aortic regurgitation in children.

  1997cited by this paper
• The natural history of aneurysmal coronary artery disease.

  1997cited by this paper
• The Graphical Presentation of a Collection of Means

  1995cited by this paper
• Coronary artery ectasia--a variant of occlusive coronary arteriosclerosis.

  1978cited by this paper
• Coronary artery aneurysms: study of the etiology, clinical course and effect on left ventricular function and prognosis.

  1977cited by this paper

• No citing papers are available for this paper.