Cardiovascular surgery in Turner syndrome - early outcome and long-term follow-up

Table 1 Baseline characteristics and medications (n = 51)

Characteristics	Number
Age at the first surgery at Mayo years	28 (8-41)
Height, cm	154 ± 31
Weight, kg	58 ± 20
Body surface area, m2	1.56 ± 0.46
Body mass index, kg/m2	27 ± 8
Comorbidities
Hypertension	26 (51%)
Hyperlipidemia	21 (42%)
Current or prior smoker	5 (10%)
Diabetes mellitus	9 (18%)
Sleep apnea	7 (14%)
Hypothyroidism	29 (57%)
Atrial fibrillation	7 (14%)
Coronary artery disease	9 (18%)
Prior stroke Medications	4 (8%)
Medications
Diuretics	12 (15%)
Beta and/or calcium channel blockers	28 (36%)
RAAS antagonist	26 (33%)
Warfarin	6 (6%)
Aspirin	37 (47%)
Statin	21 (27%)
Levothyroxine	27 (35%)
Estrogen	43 (55%)
Growth hormone (prior or current)	12 (25%)

RAAS: Renin angiotensin aldosterone system.

Table 2 Associated congenital heart disease (n = 51)

Associated congenital heart disease	n (%)
Bicuspid aortic valve	33 (65%)
Coarctation of aorta	31 (61%)
Persistent left superior vena cava	7 (14%)
Anomalous pulmonary vein	2 (4%)
Atrial septal defect	5 (10%)
Ventricular septal defect	4 (8%)
Patent ductus arteriosus	5 (10%)
Partial atrioventricular canal defect	1 (2%)
Unroofed coronary sinus	1 (2%)
Others	6 (12%)

Table 3 Echocardiographic data including aortic measurements (n = 39)

Echocardiographic and aortic measurements	mean ± SD/n (%)
Left ventricular ejection fraction, %	54 ± 5
Left ventricular end-diastolic dimension, mm	45 ± 7
Left ventricular end-systolic dimension, mm	32 ± 4
Left ventricular mass index, g/m2	106 ± 24
Aortic valve annulus, mm	20 ± 2
Aortic valve annulus, Z score	0.6 (-0.3-1.1)
Aortic root/sinus of Valsalva, mm	33 ± 4
Aortic root/sinus of Valsalva, Turner-specific Z score	1.7 (0.7-2.9)
Aortic root/sinus of Valsalva, Turner-specific Z score > 2	21 (54%)
Aortic size index, cm/m2	2.5 ± 0.4
Aortic size index, ≥ 2.5 cm/m2	20 (51%)
Mid ascending aorta, mm	32 ± 4
Mid ascending aorta, Turner-specific Z score	1.2 (0.6-3.0)
Mid ascending aorta, Turner-specific Z score > 2	22 (56%)
Aortic size index, cm/m2	2.2 ± 0.3
Aortic size index, ≥ 2.5 cm/m2	22 (56%)

Table 4 Prior cardiovascular procedures (n = 51)

Prior cardiovascular procedures	n (%)
Coarctation of aorta repair	11 (22%)
Aortic valvuloplasty	2 (4%)
Aortic valve replacement	1 (2%)
Atrial septal defect closure	1 (2%)
Ventricular septal defect closure	1 (2%)
Pulmonary artery banding	1 (2%)
Mitral valve replacement	1 (2%)
Mitral valve repair	1 (2%)
Repair of type B aortic dissection	1 (2%)

Table 5 Data of first surgery at Mayo Clinic (n = 51)

First surgery type	n (%)
Coarctation of aorta repair	14 (27%)
End-to-end anastomosis	8 (16%)
Subclavian flap repair	2 (4%)
Patch aortoplasty	1 (2%)
Ascending-to-descending aorta bypass	1 (2%)
Interposition graft	2 (4%)
Aortic valvotomy	2
Aortic valve replacement	6 (12%)
Aortic valve/root/ascending aorta replacement	7 (14%)
Valve sparing root/ascending aorta replacement	3 (6%)
Coronary artery bypass grafting	5 (10%)
Descending aorta replacement	2 (4%)
Pulmonary valve replacement	2 (2%)
Atrial septal defect closure	5 (10%)
Warden procedure	1 (2%)
Ventricular defect closure	3 (6%)
Tricuspid valve repair	1 (2%)
Mitral valve repair	1 (2%)
Mitral valve replacement	1 (2%)
Patent ductus arteriosus ligation	5 (10%)
Closure of ostium of coronary sinus	1 (2%)
Partial atrioventricular canal defect	1 (2%)