Coronary artery anomalies in Turner Syndrome
Mette H Viuff1, Christian Trolle1, Jan Wen1
1Department of Endocrinology and Internal Medicine and Medical Research Laboratories, Aarhus University Hospital, Aarhus, Denmark.
Insights
Coronary anomalies are common in Turner Syndrome, particularly affecting the left coronary artery. This study highlights the need for careful cardiac evaluation in these patients.
Area of Science:
- Cardiology
- Genetics
- Radiology
Background:
- Turner Syndrome is associated with congenital heart disease, especially left-sided defects.
- Case reports suggest a higher prevalence of coronary anomalies in Turner Syndrome.
- Systematic investigation of coronary arterial anatomy in Turner Syndrome patients is warranted.
Purpose of the Study:
- To systematically investigate coronary arterial anatomy in Turner Syndrome patients using computed tomographic coronary angiography (coronary CTA).
- To compare the prevalence of coronary anomalies in Turner Syndrome patients with gender-matched controls.
Main Methods:
- Fifty consecutive women with Turner Syndrome underwent coronary CTA.
- Twenty-five gender-matched healthy women served as controls.
- Coronary arterial anatomy was assessed via computed tomographic coronary angiography.
Main Results:
- Coronary anomalies were significantly more frequent in Turner Syndrome patients (20%) compared to controls (4%).
- The most common anomaly involved the left coronary artery, including absent left main trunk (7 cases) and circumflex artery originating from the right aortic sinus (2 cases).
- No correlation was found between coronary anomalies and karyotype, bicuspid aortic valve, or other congenital heart defects.
Conclusions:
- Coronary anomalies are highly prevalent in Turner Syndrome.
- The left coronary artery, particularly the left main coronary artery, is predominantly affected.
- No hemodynamically significant coronary anomalies were identified in the study population.
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.
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