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Background: Patients who underwent surgery for aortic coarctation (COA) have an increased risk of arterial hypertension. We aimed at evaluating (1) differences between hypertensive and non-hypertensive patients and (2) the value of cardiopulmonary exercise testing (CPET) to predict the development or progression of hypertension., Methods: Between 1999 and 2010, CPET was performed in 223 COA-patients of whom 122 had resting blood pressures of <140/90 mmHg without medication, and 101 were considered hypertensive. Comparative statistics were performed. Cox regression analysis was used to assess the relation between demographic, clinical and exercise variables and the development/progression of hypertension., Results: At baseline, hypertensive patients were older (p=0.007), were more often male (p=0.004) and had repair at later age (p=0.008) when compared to normotensive patients. After 3.6 ± 1.2 years, 29/120 (25%) normotensive patients developed hypertension. In normotensives, VE/VCO2-slope (p=0.0016) and peak systolic blood pressure (SBP; p=0.049) were significantly related to the development of hypertension during follow-up. Cut-off points related to higher risk for hypertension, based on best sensitivity and specificity, were defined as VE/VCO2-slope ≥ 27 and peak SBP ≥ 220 mmHg. In the hypertensive group, antihypertensive medication was started/extended in 48/101 (48%) patients. Only age was associated with the need to start/extend antihypertensive therapy in this group (p=0.042)., Conclusions: Higher VE/VCO2-slope and higher peak SBP are risk factors for the development of hypertension in adults with COA. Cardiopulmonary exercise testing may guide clinical decision making regarding close blood pressure control and preventive lifestyle recommendations., (Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.)

Background: To investigate aortic dimensions in women with Turner syndrome (TS) in relation to aortic valve morphology, blood pressure, karyotype, and clinical characteristics., Methods and Results: A cross sectional study of 102 women with TS (mean age 37.7; 18-62 years) examined by cardiovascular magnetic resonance (CMR- successful in 95), echocardiography, and 24-hour ambulatory blood pressure. Aortic diameters were measured by CMR at 8 positions along the thoracic aorta. Twenty-four healthy females were recruited as controls. In TS, aortic dilatation was present at one or more positions in 22 (23%). Aortic diameter in women with TS and bicuspid aortic valve was significantly larger than in TS with tricuspid valves in both the ascending (32.4 +/- 6.7 vs. 26.0 +/- 4.4 mm; p < 0.001) and descending (21.4 +/- 3.5 vs. 18.8 +/- 2.4 mm; p < 0.001) aorta. Aortic diameter correlated to age (R = 0.2 - 0.5; p < 0.01), blood pressure (R = 0.4; p < 0.05), a history of coarctation (R = 0.3; p = 0.01) and bicuspid aortic valve (R = 0.2-0.5; p < 0.05). Body surface area only correlated with descending aortic diameter (R = 0.23; p = 0.024)., Conclusions: Aortic dilatation was present in 23% of adult TS women, where aortic valve morphology, age and blood pressure were major determinants of the aortic diameter.

Background To investigate aortic dimensions in women with Turner syndrome (TS) in relation to aortic valve morphology, blood pressure, karyotype, and clinical characteristics. Methods and results: A cross sectional study of 102 women with TS (mean age 37.7; 18-62 years) examined by cardiovascular magnetic resonance (CMR- successful in 95), echocardiography, and 24-hour ambulatory blood pressure. Aortic diameters were measured by CMR at 8 positions along the thoracic aorta. Twenty-four healthy females were recruited as controls. In TS, aortic dilatation was present at one or more positions in 22 (23%). Aortic diameter in women with TS and bicuspid aortic valve was significantly larger than in TS with tricuspid valves in both the ascending (32.4 ± 6.7 vs. 26.0 ± 4.4 mm; p < 0.001) and descending (21.4 ± 3.5 vs. 18.8 ± 2.4 mm; p < 0.001) aorta. Aortic diameter correlated to age (R = 0.2 - 0.5; p < 0.01), blood pressure (R = 0.4; p < 0.05), a history of coarctation (R = 0.3; p = 0.01) and bicuspid aortic valve (R = 0.2-0.5; p < 0.05). Body surface area only correlated with descending aortic diameter (R = 0.23; p = 0.024). Conclusions Aortic dilatation was present in 23% of adult TS women, where aortic valve morphology, age and blood pressure were major determinants of the aortic diameter. [ABSTRACT FROM AUTHOR]

Aims The current study was designed to assess midterm results of stent implantation into the aorta for native and recurrent coarctation (CoA) in children and young adults. [ABSTRACT FROM PUBLISHER]

Objective Multiple studies have demonstrated the predictive value of arterial stiffness parameters like augmentation index (AIx) for cardiovascular events, the onset of hypertension, and the progression of heart failure. There is evidence that arterial stiffness is increased in some diagnostic subgroups of patients with congenital heart defects (CHD). This study aims to investigate AIx in a large cross-sectional cohort of patients with CHD. Patients and methods We prospectively examined 1125 consecutive patients with various congenital heart defects (27.3 ± 12.1 years, 464 female) referred for routine cardiopulmonary exercise testing (CPET) in our institution, and 322 healthy volunteers (29.4 ± 18.4 years, 165 female). AIx was estimated in supine position using the oscillometric Vicorder device (SMT medical, Würzburg, Germany). Afterward patients performed a CPET. Results In multivariable regression, presence of a CHD emerged as independent risk factor for higher AIx (p < .001). AIx was also higher in older (p < .001), smaller (p < .001) and heavier (p < .001) patients and in females (p = .008). Patients with aortic stenosis (p < .001), Tetralogy of Fallot (p < .001), transposition of the great arteries after atrial switch (p < .001) or Rastelli procedure (p = .013) and after Fontan procedure (p = .002) had higher AIx. Higher peak oxygen uptake (p < .001) and an ACE-inhibitor (p = .088) were associated with a lower AIx. Conclusions AIx is increased in patients with CHD. Several diagnostic subgroups are at risk. A better understanding of pathophysiologic mechanisms, genetic predisposition, the role of surgical aortic scars or implanted conduits/patches and medication is needed to define the value of AIx for further cardiovascular risk assessment in this cohort. [ABSTRACT FROM AUTHOR]

Heart failure is an important cause of morbidity and mortality in adults with congenital heart disease (ACHD). Sacubitril/valsartan is an established treatment for heart failure with reduced ejection fraction due to acquired cardiovascular disease. Data in adults with complex congenital heart disease (CHD) is lacking. Retrospective study of ACHD patients with CHD of moderate/severe complexity and heart failure under treatment with sacubitril/valsartan. Clinical data was retrieved from medical records. Altogether, 23 patients (mean age 41.2 ± 11.9 years, female 17.4%) were included. A systemic right ventricle was present in 12 pat. (52.2%), a single ventricle physiology in 4 (17.4%), and a systemic left ventricle in 7 (30.4%). During a median follow-up of 221 days [IQR 79–430], systemic ventricular function (p = 0.88) and functional status according to New York Heart Association class (p = 0.38) did not improve. While NT-proBNP levels did not change significantly under treatment (2561 ± 2042 ng/l vs. 1938 ± 1524 ng/l, p = 0.20), creatinine levels increased (1.14 ± 0.52 mg/dl vs. 1.35 ± 0.74 mg/dl, p = 0.002). Systolic (110 ± 15 mm Hg vs. 103 ± 14 mm Hg, p = 0.02) and diastolic blood pressures (68 ± 10 mm Hg vs. 61 ± 12 mm Hg, p = 0.01) were reduced under therapy. Five patients discontinued therapy, four of these due to side effects. In this small group of complex ACHD patients with heart failure, treatment with sacubitril/valsartan did not improve systemic ventricular function or functional status. Renal function needs close surveillance. • The number of adults with congenital heart disease (ACHD) is increasing. • Heart failure is an important cause of morbidity and mortality in ACHD patients. • Sacubitril/valsartan did not improve heart failure symptoms in ACHD patients. • 17% of patients discontinued the therapy due to side effects. [ABSTRACT FROM AUTHOR]

Abstract: Background: In the end of the last century arterial switch operation (ASO) replaced atrial redirection according to Senning or Mustard as the surgical technique for infants with transposition of the great arteries (TGA). Better survival with the new technique has been shown. However, exercise performance and quality of life have not yet been compared directly. Methods and results: All consecutive patients aged sixteen years or older that had undergone ASO for TGA at our institution and had performed a cardiopulmonary exercise test as part of their routine follow-up were included into the study. Those twenty-eight patients (20 male, median age 18.8years) were matched for age and gender with twenty-eight patients (20 male, median age 18.9years) who underwent atrial redirection in the same surgical period at our institution. Peak oxygen uptake was higher in patients after ASO (median 38.1 [quartiles 30.3; 44.5] ml/kg/min vs. 29.8 [23.5; 33.9] ml/kg/min; p<.0001) representing 92.0% predicted and 66.1% predicted (p<.0001), respectively. In addition, patients after ASO presented a better ventilatory efficiency ( slope, p=.029), ventilatory threshold (p=.006), peak O2 pulse (p=.0001), and oxygen saturation (p=.016), as well as a superior blood pressure (p=.046) response to exercise. Self-estimated quality of life was rather good in both groups but with better results in the physical functioning and general health domains in patients after ASO. Conclusions: Adolescents and young adults with TGA still have an improved exercise performance and quality of life in the long-term follow-up after ASO than those patients after atrial redirection. [Copyright &y& Elsevier]

Patients after coarctation repair still have an increased risk of cardiovascular or cerebrovascular events. This has been explained by the persisting hypertension and alterations in the peripheral vessels. However, involvement of the central vessels such as the retinal arteries is virtually unknown. A total of 34 patients after coarctation repair (22 men and 12 women; 23 to 58 years old, age range 0 to 32 years at surgical repair) and 34 nonhypertensive controls underwent structural and functional retinal vessel analysis. Using structural analysis, the vessel diameters were measured. Using functional analysis, the endothelium-dependent vessel dilation in response to flicker light stimulation was assessed. In the patients after coarctation repair, the retinal arteriolar diameter was significantly reduced compared to that of the controls (median 182 μm, first to third quartile 171 to 197; vs 197 μm, first to third quartile 193 to 206; p <0.001). These findings were independent of the peripheral blood pressure and age at intervention. No differences were found for venules. The functional analysis findings were not different between the patients and controls (maximum dilation 3.5%, first to third quartile 2.1% to 4.5% vs 3.6%, first to third quartile 2.2% to 4.3%; p = 0.81), indicating preserved autoregulative mechanisms. In conclusion, the retinal artery diameter is reduced in patients after coarctation repair, independent of their current blood pressure level and age at intervention. As a structural marker of chronic vessel damage associated with past, current, or future hypertension, retinal arteriolar narrowing has been linked to stroke incidence. These results indicate an involvement of cerebral microcirculation in aortic coarctation, despite timely repair, and might contribute to explain the increased rate of cerebrovascular events in such patients. [Copyright &y& Elsevier]