Exercise Capacity in Asymptomatic Adult Patients Treated for Coarctation of the Aorta.

A reduced exercise capacity is a common finding in adult congenital heart disease and is associated with cardiovascular morbidity and mortality. However, data on exercise capacity in patients after repair of coarctation of the aorta (CoA) are scarce. Furthermore, a high rate of exercise-induced hypertension has been described in CoA patients. This study sought to assess exercise capacity and blood pressure response in asymptomatic patients long-term after CoA repair in relation to left ventricular and vascular function. Twenty-two CoA patients (age 30 ± 10.6 years) with successful surgical repair (n = 12) or balloon angioplasty (n = 10) between 3 months and 16 years of age with a follow-up of > 10 years underwent cardiopulmonary exercise testing at a mean follow-up of 23.9 years. Exercise capacity (peak oxygen uptake; VO2peak) and blood pressure response were compared to age- and gender-matched reference values. Left ventricular function and volumetric analysis was performed using cardiovascular magnetic resonance imaging. CoA patients showed preserved exercise capacity compared to the healthy reference group, with a VO2peak of 41.7 ± 12.0 ml/kg/min versus 44.9 ± 6.7 ml/kg/min. VO2peak/kg showed a significant association with age (p < 0.001) and male gender (p ≤ 0.001). Exercise-induced hypertension occurred in 82% of CoA patients, and was strongly related to left ventricular mass (p = 0.04). Of the 41% of patients who were normotensive at rest, 78% showed exercise-induced hypertension. No significant correlation was found between peak exercise blood pressure and age, BMI, age at time of repair, LVEF, or LV dimensions. Exercise capacity is well preserved in patients long-term after successful repair of coarctation of the aorta. Nevertheless, a high number of patients develop exercise hypertension, which is strongly related to systemic hypertension. Regular follow-up, including cardiopulmonary exercise testing, and aggressive treatment of hypertension after CoA repair is strongly advised.

Arterioventricular interaction after coarctation repair.

BACKGROUND: Hypertension is common in patients with coarctation of the aorta (CoA), even after successful repair. Increased aortic stiffness has been implicated in the pathology of CoA-associated hypertension. This study aimed to investigate aortic vascular function and its relationship with hypertension in well-repaired CoA-patients at long-term follow-up. Furthermore, we assessed the additive effect of hypertension to adverse arterioventricular coupling associated with increased aortic stiffness. METHODS: Twenty-two CoA-patients (age 30 ±â€¯10.6 years) with successful surgical repair (n = 12) or balloon angioplasty (BA) (n = 10) between 3 months and 16 years of age with a follow-up of >10 years and 22 healthy controls underwent cardiac magnetic resonance imaging (CMR), at mean follow-up of 29.3 years, to study aortic pulse wave velocity (PWV), aortic distensibility, global left ventricular (LV) function, LV dimensions, and LV myocardial deformation. RESULTS: CoA-patients had significantly increased aortic arch PWV (5.6 ±â€¯1.9 m/s vs. 4.5 ±â€¯1.0 m/s, P = .02) and decreased distensibility (4.5 ±â€¯1.8 × 10-3 mmHg-1 vs. 5.8 ±â€¯1.8 × 10-3 mmHg-1, P = .04) compared to controls. Significant differences in aortic arch PWV were found between hypertensive patients, normotensive patients and controls (6.1 ±â€¯1.8 m/s vs. 4.9 ±â€¯1.9 m/s and 4.5 ±â€¯1.0 m/s, respectively, P = .03). Aortic arch PWV and distensibility were correlated with systolic blood pressure (R = 0.37 and R = -0.37, respectively, P = .03 for both). Global LV function, LV mass, LV dimensions and myocardial deformation were similar in CoA-patients when compared to controls. CONCLUSIONS: Central aortic stiffness is significantly increased in well-repaired CoA-patients long-term after repair, and is associated with hypertension. Global LV function, myocardial deformation indices and LV dimensions are however preserved.

Long-term results of balloon angioplasty for native coarctation of the aorta in childhood in comparison with surgery.

OBJECTIVES: Coarctation of the aorta (CoA) can be treated either surgically or with balloon angioplasty (BA). Long-term follow-up for either treatment has been limited. Our objective was to compare long-term results of BA and surgery for treatment of native CoA in childhood. METHODS: Retrospective cohort study of patients with native CoA treated with BA or surgery between 3 months and 16 years of age. Forty-eight patients filled out questionnaires and approved review of their medical records. Twenty-four patients underwent additional testing, including 24-h ambulatory blood pressure measurement, cardiopulmonary exercise testing and cardiac magnetic resonance imaging. Results were analysed cross-sectionally and longitudinally. RESULTS: Nineteen and 29 patients received BA and surgery, respectively. Prevalence of hypertension and aneurysms was similar in both groups. Fifty percent of patients were hypertensive. Two-thirds of patients demonstrating hypertension were not receiving antihypertensive medication. Aneurysm formation occurred in 1 BA (5%) and 1 surgery (3%) patient. The BA group had a significantly higher risk of recoarctation (47% vs 24%) and reintervention (hazard ratio 2.95, 95% confidence interval 1.04-8.32). Exercise capacity and global left ventricular function were preserved in both groups and not significantly different after correction for age. Quality of life was good to excellent in the majority of the patients. CONCLUSIONS: After CoA repair in childhood, most patients perform well in daily life. However, on the long term, more than half of the patients develop hypertension and many develop re-CoA, especially in those who underwent BA. Therefore, we do not recommend BA for the treatment of native CoA in children.

Preserved Myocardial Deformation after Successful Coarctation Repair: A CMR Feature-Tracking Study.

Arterial vasculopathy and residual aortic obstruction can lead to left ventricular (LV) dysfunction in patients with coarctation of the aorta (CoA) related to adverse ventriculo-arterial coupling. This study aimed to investigate potential differences in LV myocardial deformation indices between repaired CoA patients and healthy controls. Twenty-two CoA patients (age 30 ± 10.6 years) after surgical repair (n = 12) or balloon angioplasty (BA) (n = 10) without residual stenosis, between 3 months and 16 years of age with > 10 years follow-up were compared to 22 healthy age- and gender-matched controls (age 30 ± 3.8 years). Cardiac magnetic resonance feature tracking (CMR-FT) was used for LV longitudinal-, circumferential-, and rotational deformation indices. Global systolic LV function was preserved in CoA patients (LV ejection fraction 58 ± 4.8 vs. 60 ± 6.8%, p = 0.56) when compared to controls, with normal LV dimensions and mass (p > 0.05). Twelve CoA patients (55%) were hypertensive, of whom 4 were on anti-hypertensive medication. LV global longitudinal strain was preserved in the four-chamber (- 18 ± 4.4 vs. - 16 ± 4.7%, p = 0.06) and two-chamber (- 22 ± 5.1 vs. - 20 ± 6.0%, p = 0.22) orientations in CoA patients. Global circumferential strain was preserved at basal (- 29 ± 4.1 vs. - 28 ± 4.8%, p = 0.43), mid-ventricular (- 27 ± 4.2 vs. - 25 ± 3.0%, p = 0.09), and apical levels (- 35 ± 7.8 vs. - 32 ± 34.9%, p = 0.32). No differences were found in global torsion (2.4 ± 1.3° vs. 2.0 ± 1.4°/cm, p = 0.28), twist (14 ± 5.8° vs. 12 ± 6.3°, p = 0.34), and recoil rate (- 17 ± 9.7° vs. - 17 ± 7.1°/cm s, p = 0.97). Analysis of intra-observer variability demonstrated good reproducibility for all CMR deformation indices. Global and rotational myocardial deformation indices are preserved in CoA patients long-term after repair without residual stenosis, despite a high incidence of hypertension.

Diagnosis, imaging and clinical management of aortic coarctation.

Coarctation of the aorta (CoA ) is a well-known congenital heart disease (CHD) , which is often associated with several other cardiac and vascular anomalies, such as bicuspid aortic valve (BAV), ventricular septal defect, patent ductus arteriosus and aortic arch hypoplasia. Despite echocardiographic screening, prenatal diagnosis of C o A remains difficult. Most patients with CoA present in infancy with absent, delayed or reduced femoral pulses, a supine arm-leg blood pressure gradient (> 20 mm Hg), or a murmur due to rapid blood flow across the CoA or associated lesions (BAV). Transthoracic echocardiography is the primary imaging modality for suspected CoA. However, cardiac magnetic resonance imaging is the preferred advanced imaging modality for non-invasive diagnosis and follow-up of CoA. Adequate and timely diagnosis of CoA is crucial for good prognosis, as early treatment is associated with lower risks of long-term morbidity and mortality. Numerous surgical and transcatheter treatment strategies have been reported for CoA. Surgical resection is the treatment of choice in neonates, infants and young children. In older children (> 25 kg) and adults, transcatheter treatment is the treatment of choice. In the current era, patients with CoA continue to have a reduced life expectancy and an increased risk of cardiovascular sequelae later in life, despite adequate relief of the aortic stenosis. Intensive and adequate follow-up of the left ventricular function, valvular function, blood pressure and the anatomy of the heart and the aorta are , therefore, critical in the management of CoA. This review provides an overview of the current state-of-the-art clinical diagnosis, diagnostic imaging algori thms, treatment and follow-up of patients with CoA.

Normative Values of Aortic Arch Structures in Premature Infants.

BACKGROUND: Aortic arch abnormalities represent 5% to 8% of all congenital heart disease. Measurements of the aortic arch dimensions on two-dimensional echocardiographic images remain of critical importance in the diagnosis of aortic arch pathology. To define aortic hypoplasia or coarctation, measured dimensions must be compared with normal values. Normal values have been described for children of all ages in earlier studies. However, normative data for premature infants are not yet available. Therefore, the aim of this study was to develop normative data in a cohort of premature infants, which could be used in the diagnosis of aortic arch abnormalities. METHODS: A single-center study was conducted in a large population of premature infants with gestational ages of ≤32 weeks without hemodynamically important congenital heart disease, chromosomal abnormalities, and/or major cerebral congenital malformations. Two-dimensional echocardiographic measurements of four aortic arch structures were made on the second, fourth, and sixth days after birth. RESULTS: Three hundred eighty-five preterm patients were included. No differences in dimensions were found among days 2, 4, and 6. The dimension of the isthmus showed no significant relation to the existence of a patent ductus arteriosus. Reference intervals with mean and SD were calculated across the range of birth weight. Regression analysis was performed with multiple determinants in different models. The best predictive value was found for birth weight in a cubic model. CONCLUSIONS: This work provides regression equations for the calculation of Z scores and reference intervals for aortic arch dimensions in a cohort of preterm infants born at gestational ages of ≤32 weeks. The normative data can be used in diagnosis and decision making involving aortic arch pathology in premature infants.