Mette H Viuff1, Christian Trolle1, Jan Wen1, Jesper M Jensen2, Bjarne L Nørgaard2, Ephraim J Gutmark3, Iris Gutmark-Little4, Kristian H Mortensen5, Claus Højbjerg Gravholt6, Niels H Andersen7. 1. Department of Endocrinology and Internal Medicine and Medical Research Laboratories, Aarhus University Hospital, Aarhus, Denmark. 2. Department of Cardiology, Aarhus University Hospital, Aarhus, Denmark. 3. Department of Aerospace Engineering and Engineering Mechanics, CEAS, University of Cincinnati, Cincinnati, OH, USA; UC Department of Otolaryngology - Head and Neck Surgery, Cincinnati, OH, USA. 4. UC Department of Pediatrics, Cincinnati, OH, USA; Division of Endocrinology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA. 5. Cardiovascular Imaging Department, Cardiothoracic Unit, Great Ormond Street Hospital for Children NHS Foundation Trust, London WC1N 3JH, United Kingdom. 6. Department of Endocrinology and Internal Medicine and Medical Research Laboratories, Aarhus University Hospital, Aarhus, Denmark; Department of Molecular Medicine, Aarhus University Hospital, Aarhus, Denmark. 7. Department of Cardiology, Aarhus University Hospital, Aarhus, Denmark. Electronic address: holmark@ki.au.dk.
Abstract
BACKGROUND: Congenital heart disease, primarily involving the left-sided structures, is often seen in patients with Turner Syndrome. Moreover, a few case reports have indicated that coronary anomalies may be more prevalent in Turner Syndrome than in the normal population. We therefore set out to systematically investigate coronary arterial anatomy by computed tomographic coronary angiography (coronary CTA) in Turner Syndrome patients. METHODS: Fifty consecutive women with Turner Syndrome (mean age 47 years [17-71]) underwent coronary CTA. Patients were compared with 25 gender-matched controls. RESULTS: Coronary anomaly was more frequent in patients with Turner Syndrome than in healthy controls [20% vs. 4% (p = 0.043)]. Nine out of ten abnormal cases had an anomalous left coronary artery anatomy (absent left main trunk, n = 7; circumflex artery originating from the right aortic sinus, n = 2). One case had a tubular origin of the right coronary artery above the aortic sinus. There was no correlation between the presence of coronary arterial anomalies and karyotype, bicuspid aortic valve, or other congenital heart defects. CONCLUSION: Coronary anomalies are highly prevalent in Turner Syndrome. The left coronary artery is predominantly affected, with an absent left main coronary artery being the most common anomaly. No hemodynamically relevant coronary anomalies were found.
BACKGROUND:Congenital heart disease, primarily involving the left-sided structures, is often seen in patients with Turner Syndrome. Moreover, a few case reports have indicated that coronary anomalies may be more prevalent in Turner Syndrome than in the normal population. We therefore set out to systematically investigate coronary arterial anatomy by computed tomographic coronary angiography (coronary CTA) in Turner Syndromepatients. METHODS: Fifty consecutive women with Turner Syndrome (mean age 47 years [17-71]) underwent coronary CTA. Patients were compared with 25 gender-matched controls. RESULTS:Coronary anomaly was more frequent in patients with Turner Syndrome than in healthy controls [20% vs. 4% (p = 0.043)]. Nine out of ten abnormal cases had an anomalous left coronary artery anatomy (absent left main trunk, n = 7; circumflex artery originating from the right aortic sinus, n = 2). One case had a tubular origin of the right coronary artery above the aortic sinus. There was no correlation between the presence of coronary arterial anomalies and karyotype, bicuspid aortic valve, or other congenital heart defects. CONCLUSION:Coronary anomalies are highly prevalent in Turner Syndrome. The left coronary artery is predominantly affected, with an absent left main coronary artery being the most common anomaly. No hemodynamically relevant coronary anomalies were found.
Authors: Marissa Schoepp; Fady Hannah-Shmouni; Jatin Matta; Ahmed M Ghanem; John A Hanover; Khaled Z Abd-Elmoniem; Ahmed M Gharib Journal: Genet Med Date: 2017-10-02 Impact factor: 8.822