Interventions in Adults With Repaired Coarctation of the Aorta.

Background Coarctation of the aorta coexists with other cardiac anomalies and has long-term complications, including recoarctation, which may require intervention after the primary coarctation repair. This study aims to clarify the prevalence of and risk factors for interventions related to the coarctation complex as well as late mortality in a large contemporary patient population. Methods and Results The Swedish National Register of Congenital Heart Disease was used, which comprised 683 adults with repaired coarctation of the aorta. Analysis was performed on freedom from intervention thereafter at the coarctation site, aortic valve, left ventricular outflow tract, or ascending aorta. One hundred ninety-six (29%) patients had at least 1 of these interventions. Estimated freedom from either of these interventions was 60% after 50 years. The risk of undergoing such an intervention was higher among men (hazard ratio, 1.6 [95% CI, 1.2-2.2]). Estimated freedom from another intervention at the coarctation site was 75% after 50 years. In women, there was an increase in interventions at the coarctation site after 45 years. Patients who underwent one of the previously mentioned interventions after the primary coarctation repair had poorer left ventricular function. Eighteen patients (3%) died during follow-up in the register. The standardized mortality ratio was 2.9 (95% CI, 1.7-4.3). Conclusions Interventions are common after coarctation repair. The risk for and time of interventions are affected by sex. Our results have implications for planning follow-up and giving appropriate medical advice to the growing population of adults with repaired coarctation of the aorta.

High prevalence of ascending aortic dilation in adults with repaired coarctation of the aorta.

BACKGROUND: Ascending aortic dilation is a feared complication in adults with repaired coarctation of the aorta, as the condition is associated with life-threatening complications such as aortic dissection and rupture. However, the data are currently limited regarding factors associated with ascending aortic dilation in these patients. METHODS AND RESULTS: From the national register of congenital heart disease, 165 adult patients (≥ 18 years old) with repaired coarctation of the aorta, and echocardiographic data on aortic dimensions, were identified (61.2% male, mean age 35.8 ± 14.5 years). Aortic dilation (aortic diameters > 2 SD above reference mean) was found in 55 (33.3%) of the 165 included patients, and was associated with manifest aortic valve disease in univariable logistic regression analysis (OR 2.44, 95% CI [1.23, 4.83]). CONCLUSIONS: Aortic dilation is common post-repair of coarctation of the aorta, and is associated with manifest aortic valve disease and thus indirectly with the presence of a bicuspid aortic valve. However, no association was found between aortic dilation and age or blood pressure.

Implantable cardiac devices in adult patients with repaired tetralogy of Fallot.

OBJECTIVES: Implantable cardiac devices are common in patients with tetralogy of Fallot (ToF) (18.3-21.3%) according to previous reports from large centres. We conducted this study to investigate the prevalence and incidence of cardiac devices in a less selected population of patients with ToF and assess factors other than arrhythmia associated with having a device. Design: 530 adult (≥18 years) patients with repaired ToF were identified in the national registry of congenital heart disease (SWEDCON) and matched with data from the Swedish pacemaker registry. Patients with implantable cardiac devices were compared with patients without devices. Results: Seventy-five patients (14.2%) had a device; 51 (9.6%) had a pacemaker and 24 (4.5%) had an implantable cardioverter defibrillator. The incidence in adult age (≥18 years) was 5.9/1000 patient years. Estimated device free survival was 97.5% at twenty, 87.2% at forty and 63.5% at sixty years of age. Compared with previous studies, the prevalence of devices was lower, especially for ICD. In multivariate logistic regression, cardiovascular medication (odds ratio [OR] 3.5, 95% confidence interval [CI] 1.8-6.8), impaired left ventricular function, (OR 2.6, 95%CI 1.3-5.0) and age (OR 1.02, 95%CI 1.002-1.05) were associated with having a device. Conclusion: The prevalence of devices in our population, representing a multicenter register cohort, was lower than previously reported, especially regarding ICD. This can be due to differences in treatment traditions with regard to ICD in this population, but it may also be that previous studies have reported selected patients with more severe disease.

Factors associated with health-related quality of life among adults with tetralogy of Fallot.

Background: Due to improved care, the numbers of patients with tetralogy of Fallot (ToF) are increasing. However, long-term morbidity and need for reinterventions are concerns and also address issues of quality of life (QoL). Methods: Patients with ToF and valid EuroQol-5 dimensions questionnaire (EQ-5D) were identified in the national Swedish register on congenital heart disease. EQ-5Dindex was calculated and dichotomised into best possible health-related QoL (EQ-5Dindex=1) or differed from 1. Results: 288 patients met the criteria and were analysed. Univariate logistic regression showed a positive association between New York Heart Association (NYHA) class I (OR 8.32, 95% CI 3.80 to 18.21), physical activity >3 h/week (OR 3.34, 95% CI 1.67 to 6.66) and a better right ventricular function (OR 2.56, 95% CI 1.09 to 6.02). A negative association between symptoms (OR 0.23, 95% CI 0.13 to 0.42), cardiovascular medication (OR 0.31, 95% CI 0.18 to 0.53), age (OR 0.97, 95% CI 0.96 to 0.99) and EQ-5Dindex was observed. In multivariate logistic regression, NYHA I (OR 7.28, 95% CI 3.29 to 16.12) and physical activity >3 h/week (OR 2.27, 95% CI 1.07 to 4.84) remained associated with best possible health-related QoL. Replacing NYHA with symptoms in the model yielded similar results. Conclusion: In this registry study, self-reported physical activity, staff-reported NYHA class and absence of symptoms were strongly associated with best possible health-related QoL measured by EQ-5D. Physical activity level is a potential target for intervention to improve QoL in this population but randomised trials are needed to test such a hypothesis.

Aerobic capacity in adolescence is associated with time to intervention in adult men with atrial septal defects.

BACKGROUND: Atrial septal defect (ASD) is a congenital heart lesion that often remains undiagnosed until adulthood. The reasons for this may be multifactorial. It is, however, known that closure of a hemodynamically significant ASD improves exercise capacity. This study aimed to explore whether the aerobic capacity in late adolescence is associated with time to diagnosis and intervention in adult men with late diagnosis of an atrial shunt. METHODS: The Swedish Military Conscription Service Register contains data on exercise tests performed in late adolescence. By linking these data with the National Patient Register, 254 men with a later intervention for an ASD were identified. RESULTS: Interventions were performed at a mean of 26.5 ±â€¯7.9 years after the initial exercise tests. The mean absolute workload among those with a later diagnosed ASD was similar to those without a later diagnosed ASD (274 ±â€¯51 W vs. 276 ±â€¯52 W, p = 0.49). Men with a higher exercise capacity (≥1 SD) had their intervention earlier (21.9 ±â€¯8.6 years vs. 27.5 ±â€¯7.4 years, p < 0.001). CONCLUSIONS: The aerobic exercise capacity was similar in adolescent men with later interventions for ASD compared to the reference population. Furthermore, those with high exercise capacity appeared to be diagnosed earlier. Thus, low exercise capacity may not be a feature of ASD during adolescence, but rather develop later in life as a natural progression of the disease.

Poor blood pressure control in adults with repaired coarctation of the aorta and hypertension: a register-based study of associated factors.

BACKGROUND: Arterial hypertension is common in adults with repaired coarctation of the aorta, and is associated with several severe complications. Aims This study aimed to investigate the prevalence of poorly controlled (⩾140/90 mmHg) blood pressure among patients with diagnosed hypertension and to identify associated factors. METHODS: In the national register for CHD, adults with repaired coarctation of the aorta and diagnosed hypertension - defined as a registry diagnosis and/or use of anti-hypertensive prescription medication - were identified. Logistic regression analysis was used to identify variables associated with poorly controlled blood pressure. RESULTS: Of the 243 included patients, 27.2% were female, the mean age was 45.4±15.3 years, and 52.3% had poorly controlled blood pressure at the last registration. In a multivariable model, age (years) (OR 1.03, CI 1.01-1.06, p=0.008) was independently associated with poorly controlled blood pressure and so was systolic arm-leg blood pressure gradient in the ranges [10, 20] mmHg (OR 4.92, CI 1.76-13.79, p=0.002) to >20 mmHg (OR 9.93, CI 2.99-33.02, p<0.001), in comparison with the reference interval [0, 10] mmHg. Patients with poorly controlled blood pressure had, on average, more types of anti-hypertensive medication classes prescribed (1.9 versus 1.5, p=0.003). CONCLUSIONS: Poorly controlled blood pressure is common among patients with repaired coarctation of the aorta and diagnosed hypertension, despite what seems to be more intensive treatment. A systolic arm-leg blood pressure gradient is associated with poorly controlled blood pressure, even at low levels usually not considered for intervention, and may be an indicator of hypertension that is difficult to treat.

Hypertension in adults with repaired coarctation of the aorta.

AIMS: In adults with coarctation of the aorta (CoA), hypertension (HTN) is a common long-term complication. We investigated the prevalence of HTN and analyzed factors associated with HTN. METHODS AND RESULTS: In the national register for congenital heart disease, 653 adults with repaired CoA were identified (mean age 36.9±14.4years); 344 (52.7%) of them had HTN, defined as either an existing diagnosis or blood pressure (BP) ≥140/90mmHg at the clinical visit. In a multivariable model, age (years) (odds ratio [OR] 1.07, CI 1.05-1.10), sex (male) (OR 3.35, CI 1.98-5.68), and body mass index (kilograms per square meter) (OR 1.09, CI 1.03-1.16) were independently associated with having HTN, and so was systolic arm-leg BP gradient where an association with HTN was found at the ranges of (10, 20] and >20mmHg, in comparison to the interval ≤10mmHg (OR 3.58, CI 1.70-7.55, and OR 11.38, CI 4.03-32.11). This model remained valid when all patients who had increased BP (≥140/90mmHg) without having been diagnosed with HTN were excluded from the analyses. CONCLUSIONS: Hypertension is common in patients with previously repaired CoA and is associated with increasing age, male sex, and elevated body mass index. There is also an association with arm-leg BP gradient, starting at relatively low levels that are usually not considered for intervention.

High incidence of infective endocarditis in adults with congenital ventricular septal defect.

OBJECTIVE: Ventricular septal defects (VSDs), if haemodynamically important, are closed whereas small shunts are left without intervention. The long-term prognosis in congenital VSD is good but patients are still at risk for long-term complications. The aim of this study was to clarify the incidence of infective endocarditis (IE) in adults with VSD. METHODS: The Swedish registry for congenital heart disease (SWEDCON) was searched for adults with VSD. 779 patients were identified, 531 with small shunts and 248 who had the VSD previously closed. The National Patient Register was then searched for hospitalisations due to IE in adults during a 10-year period. RESULTS: Sixteen (2%) patients were treated for IE, 6 men and 10 women, with a mean age of 46.3±12.2 years. The incidence of IE was 1.7-2.7/1000 years in patients without previous intervention, 20-30 times the risk in the general population. Thirteen had small shunts without previous intervention. There was no mortality in these 13 cases. Two patients had undergone repair of their VSD and also aortic valve replacement before the episode of endocarditis and a third patient with repaired VSD had a bicuspid aortic valve, all of these three patients needed reoperation because of their IE and one patient died. No patient with isolated and operated VSD was diagnosed with IE. CONCLUSIONS: A small unoperated VSD in adults carries a substantially increased risk of IE but is associated with a low risk of mortality.

Left ventricular hypertrophy in adults with previous repair of coarctation of the aorta; association with systolic blood pressure in the high normal range.

BACKGROUND: Arterial hypertension is common in adults with repaired coarctation of the aorta (CoA). The associations between the diagnosis of hypertension, actual blood pressure, other factors affecting left ventricular overload, and left ventricular hypertrophy (LVH) are not yet fully explored in this population. MATERIAL AND RESULTS: From the national register for congenital heart disease, 506 adult patients (≥18years old) with previous repair of CoA were identified (37.0% female, mean age 35.7±13.8years, with an average of 26.8±12.4years post repair). Echocardiographic data were available for all patients, and showed LVH in 114 (22.5%) of these. Systolic blood pressure (SBP) (mmHg) (OR 1.02, CI 1.01-1.04), aortic valve disease, (OR 2.17, CI 1.33-3.53), age (years) (OR 1.03, CI 1.01-1.05), diagnosis of arterial hypertension (OR 3.02, CI 1.81-5.02), and sex (female) (OR 0.41, CI 0.24-0.72) were independently associated with LVH. There was an association with LVH at SBP within the upper reference limits [130, 140] mmHg (OR 2.23, CI 1.05-4.73) that further increased for SBP>140mmHg (OR 8.02, CI 3.76-17.12). CONCLUSIONS: LVH is common post repair of CoA and is associated with SBP even below the currently recommended target level. Lower target levels may therefore become justified in this population.

Left ventricular remodelling changes without concomitant loss of myocardial fat after long-term dietary intervention.

BACKGROUND: Accumulation of myocardial triglycerides (MTG) is associated with impaired left ventricular (LV) remodelling and function in obese and diabetic subjects. The role of MTG accumulation in development of heart failure in this group of patients is unknown. Short-term studies suggest that diets that lead to weight loss could mobilize MTG, with a favourable effect on cardiac remodelling. In a 24-month, randomized, investigator-blinded study, we assessed the effect of two different diets and subsequent weight loss on cardiac function and MTG in postmenopausal women. METHODS: Sixty-eight healthy postmenopausal women with body mass index [BMI] ≥27kg/m(2) were randomized to an ad libitum Palaeolithic diet (PD) or a Nordic Nutrition Recommendation (NNR) diet for 24months. Morphology, cardiac function, and MTG levels were measured using magnetic resonance (MR) scanning, including proton spectroscopy at baseline and 6 and 24months. RESULTS: Despite mean weight losses of 4.9 (1.0) kg (NNR) and 7.8 (1.1) kg (PD), the MTG content did not change over time (p=0.98 in the NNR and p=0.11 in the PD group at 24months). Reduced left ventricular mass was observed in both diet groups over 24months. Blood pressure was reduced at 6months, but returned to baseline levels at 24months. End diastolic volume, stroke volume, and cardiac output decreased over time. No differences between diet groups were observed. CONCLUSIONS: Diet intervention and moderate weight loss over 24months improved LV remodelling but did not alter MTG levels in overweight/obese postmenopausal women.

Height, weight and body mass index in adults with congenital heart disease.

BACKGROUND: High BMI is a risk factor for cardiovascular disease and, in contrast, low BMI is associated with worse prognosis in heart failure. The knowledge on BMI and the distribution in different BMI-classes in adults with congenital heart disease (CHD) are limited. METHODS AND RESULTS: Data on 2424 adult patients was extracted from the Swedish Registry on Congenital Heart Disease and compared to a reference population (n=4605). The prevalence of overweight/obesity (BMI ≥ 25) was lower in men with variants of the Fontan procedure, pulmonary atresia (PA)/double outlet right ventricle (DORV) and aortic valve disease (AVD) (Fontan 22.0% and PA/DORV 15.1% vs. 43.0%, p=0.048 and p<0.001) (AVD 37.5% vs. 49.3%, p<0.001). Overt obesity (BMI ≥ 30) was only more common in women with AVD (12.8% vs. 9.0%, p=0.005). Underweight (BMI<18.5) was generally more common in men with CHD (complex lesions 4.9% vs. 0.9%, p<0.001 and simple lesions 3.2% vs. 0.6%, <0.001). Men with complex lesions were shorter than controls in contrast to females that in general did not differ from controls. CONCLUSION: Higher prevalence of underweight in men with CHD combined with a lower prevalence of overweight/obesity in men with some complex lesions indicates that men with CHD in general has lower BMI compared to controls. In women, only limited differences between those with CHD and the controls were found. The complexity of the CHD had larger impact on height in men. The cause of these gender differences as well as possible significance for prognosis is unknown.

Subtypes of bicuspid aortic valves in coarctation of the aorta.

Bicuspid aortic valves (BAVs) represent a wide morphologic and functional spectrum. In coarctation of the aorta, BAVs are common, but the proportion of BAV subtypes and their relation to aortic dimensions and development of late valve dysfunction are unknown. Sixty-two cardiovascular magnetic resonance investigations of patients with coarctation of the aorta were reviewed with respect to aortic valve morphology, aortic valve function, and aortic dimensions. BAVs were identified in 45 patients (72.6%), of which 13 (20.9%) were type-0 (two commissures), 28 (45.1%) type-1 (three commissures but fusion of one commissure with a raphe) and 4 (6.5%) valves were bicuspid but not possible to classify further. Patients with BAVs type-0 had larger dimensions in their sinus of Valsalva (35.5 ± 6.8 vs. 29.7 ± 2.7 mm, p = 0.002), ascending aorta (33.1 ± 6.2 vs. 26.0 ± 4.3 mm, p = 0.005) and sino-tubular junction (29.3 ± 7.4 vs. 24.2 ± 3.5 mm, p = 0.040) compared with tricuspid aortic valves (TAVs). Moderate and severe aortic valve disease was more common in BAV type-0 compared with BAV type-1 (p = 0.030) and TAV (p = 0.016). In a multivariate linear regression model BAV type-0 (p = 0.005), BAV type-1 (p = 0.011), age (p < 0.001), patient height (p = 0.009), and aortic valve disease (p = 0.035) were independently associated with increased diameter of the ascending aorta (R (2) of the model 0.54, p < 0.001). BAV type-0 is relatively common in coarctation of the aorta. Both BAV type-0 and type-1 are associated with increased diameter of the ascending aorta but this association is stronger for BAV type-0. Development of aortic valve disease is more common in BAV type-0 than in BAV type-1. Discrimination between BAV subtypes may potentially provide clinical and prognostic information in patients with coarctation of the aorta.