Cited 3 times since 2018 (0.5 per year) source: EuropePMC Heart (British Cardiac Society), Volume 105, Issue 9, 27 4 2018, Pages 701-707 Coronary anatomy in Turner syndrome versus patients with isolated bicuspid aortic valves. Koenraadt WMC, Siebelink HJ, Bartelings MM, Schalij MJ, van der Vlugt MJ, van den Bosch AE, Budde RPJ, Roos-Hesselink JW, Duijnhouwer AL, van den Hoven AT, DeRuiter MC, Jongbloed MRM
Objective
Variations in coronary anatomy, like absent left main stem and left dominant coronary system, have been described in patients with Turner syndrome (TS) and in patients with bicuspid aortic valves (BAV). It is unknown whether coronary variations in TS are related to BAV and to specific BAV subtypes.
Aim
To compare coronary anatomy in patients with TS with/without BAV versus isolated BAV and to study BAV morphology subtypes in these groups.
Methods
Coronary anatomy and BAV morphology were studied in 86 patients with TS (20 TS-BAV, 66 TS-tricuspid aortic valve) and 86 patients with isolated BAV (37±13 years vs 42±15 years, respectively) by CT.
Results
There was no significant difference in coronary dominance between patients with TS with and without BAV (25% vs 21%, p=0.933). BAVs with fusion of right and left coronary leaflets (RL BAV) without raphe showed a high prevalence of left coronary dominance in both TS-BAV and isolated BAV (both 38%). Absent left main stem was more often seen in TS-BAV as compared with isolated BAV (10% vs 0%). All patients with TS-BAV with absent left main stem had RL BAV without raphe.
Conclusion
The equal distribution of left dominance in RL BAV without raphe in TS-BAV and isolated BAV suggests that presence of left dominance is a feature of BAVs without raphe, independent of TS. Both TS and RL BAV without raphe seem independently associated with absent left main stems. Awareness of the higher incidence of particularly absent left main stems is important to avoid complications during hypothermic perfusion.
