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Review

Acquired heart conditions in adults with congenital heart disease: a growing problem Oktay Tutarel Correspondence to Dr Oktay Tutarel, Department of Cardiology & Angiology, Hannover Medical School, CarlNeuberg-Str. 1, Hannover 30625, Germany; [email protected] Received 22 April 2014 Revised 3 July 2014 Accepted 9 July 2014

ABSTRACT The number of adults with congenital heart disease is increasing due to the great achievements in the field of paediatric cardiology, congenital heart surgery and intensive care medicine over the last decades. Mortality has shifted away from the infant and childhood period towards adulthood. As congenital heart disease patients get older, a high prevalence of cardiovascular risk factors is encountered similar to the general population. Consequently, the contribution of acquired morbidities, especially acquired heart conditions to patient outcome, is becoming increasingly important. Therefore, to continue the success story of the last decades in the treatment of congenital heart disease and to further improve the outcome of these patients, more attention has to be given to the prevention, detection and adequate therapy of acquired heart conditions. The aim of this review is to give an overview about acquired heart conditions that may be encountered in adults with congenital heart disease.

patients with Eisenmenger syndrome is an extrinsic compression of the left coronary ostium by a dilated pulmonary artery.6 Furthermore, manipulation of the coronary arteries can be an unavoidable part of the surgical repair of the congenital heart defect, for example, reimplantation of the coronary arteries during the arterial switch procedure in transposition of the great arteries or during aortic root replacement in Marfan patients. And finally, atherosclerotic disease similar to that found in patients without congenital heart disease can develop in ACHD patients.7 While a great deal of effort was put into improving surgical techniques to limit the effect of surgical manipulation on the coronary arteries, prevention and treatment of atherosclerotic disease is often not a priority during the care of ACHD patients since the focus is on the treatment of the congenital heart defect and its sequelae itself.

Cardiovascular risk factors The number of adults with congenital heart disease (ACHD) is increasing due to the great achievements in the treatment of congenital heart defects over the last decades. Mortality has shifted away from the infant and childhood period towards adulthood, with a steady increase of age at death. Even the population of ACHD patients above the age of 60 is increasing dramatically.1 As ACHD patients are getting older, acquired morbidities, for example, heart conditions like coronary artery disease (CAD) or non-cardiac conditions like chronic renal failure, are becoming more and more important for the outcome of this population in conjunction with the underlying congenital heart defect.1 2 Accordingly, the percentage of patients without any comorbidity is decreasing while the number of patients with multiple comorbidities is increasing.3 Especially, acquired heart diseases are frequently encountered.4 Therefore, early prevention, detection and therapy of acquired comorbidities are of utmost importance and should be in our focus as much as the treatment of the congenital heart defect and its sequelae itself. This review aims to give an overview about acquired heart conditions that may be encountered in ACHD patients.

CORONARY ARTERY DISEASE

To cite: Tutarel O. Heart 2014;100:1317–1321.

A number of factors can foster the development of CAD in ACHD patients. First, congenital coronary artery abnormalities (anomalous origin and/or course) have been described in a variety of congenital heart defects, for example, congenitally corrected transposition of the great arteries.5 Another rare cause of angina and myocardial ischaemia in

The important contribution of traditional cardiovascular risk factors (CVRF) like arterial hypertension and hyperlipidaemia to the development of atherosclerotic disease is well established in patients without congenital heart defects.8 The prevalence of these CVRF was studied in 141 ACHD patients with concomitant CAD.7 The majority of patients (82%) had one or more CVRF with systemic arterial hypertension and hyperlipidaemia being the most prevalent (53% and 25%, respectively).7 A study from Belgium that included 1976 patients with ACHD reported that only around 20% of the patients had a fully heart-healthy lifestyle without any CVRF.4 Considering that the median age of these patients was only 26 years, these findings are especially alarming. Worryingly, data on cholesterol levels were not available in this study.4 Therefore, the full picture may be even worse with a much smaller number of patients presenting without any CVRF. Arterial hypertension is associated with stroke, myocardial infarction, sudden death, heart failure, peripheral artery disease and end-stage renal disease in the general population.9 Its prevalence in ACHD patients was found to be somewhere between 30% and 50%.4 10 This is in a similar range as reported for the general population.9 Furthermore, some ACHD patients with selected congenital heart defects are even more prone to have a high blood pressure than the general population or patients with other congenital heart defects. This holds especially true for patients with coarctation of the aorta, in whom the prevalence of arterial hypertension was reported to be much higher than in patients with a ventricular septal defect in a

Tutarel O. Heart 2014;100:1317–1321. doi:10.1136/heartjnl-2014-305575

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Review recent study (45% vs 16%).11 In contrast, some patients with complex lesions, for example, with a Fontan circulation, have low blood pressure values due to a chronic impairment in cardiac output. Data regarding the occurrence of obesity in ACHD patients are inconclusive. While in the already mentioned Belgian study patients with ACHD were more often obese than the general population,4 a study from the Netherlands reported that the proportion of ACHD patients that were overweight or obese was smaller than in the general population.12 Nonetheless, 40% of ACHD patients were overweight or obese in the later study.12 One reason for a higher prevalence of obesity in ACHD patients could be that the issue of exercise is often not raised with ACHD patients.13 And if it is finally discussed, it is more common that patients receive prohibitive advice,13 leading to a more sedentary way of living. But in most ACHD patients exercise is safe, provided that individual exercise prescriptions are based on a comprehensive assessment of the underlying cardiac condition, possible sequelae, cardiac function, arrhythmias, pulmonary hypertension and aortic dimensions as well as individual exercise capacity.14 Recently published recommendations will hopefully lead to an improvement regarding this matter.15 A further cause for obesity discussed in ACHD patients is the encouragement of adequate nutrition as an early focus in the care of infants with CHD to catch up with their peers.16 Smoking is associated with an increased risk for all types of cardiovascular disease—CAD and stroke to name a few—as well as a plethora of non-cardiovascular diseases like cancer.8 Fortunately, the proportion of ACHD patients that smoke has been reported to be smaller than in the general population.4 12 But if we consider the hazardous effects of smoking, it is worrisome that still between 13 and 23% of ACHD patients smoke.4 12 Information regarding dyslipidemia in ACHD patients is scarce. Two large studies that assessed CVRF in ACHD patients did both not include data regarding lipid levels.4 12 One smaller study reported lower levels of total cholesterol, low-density lipoprotein cholesterol and high-density lipoprotein cholesterol in ACHD patients in comparison to a general population sample group, while triglyceride levels were not different.17 In elderly patients with ACHD, the prevalence of dyslipidemia was reported to be 27%.10 In ACHD patients with established CAD, the prevalence of hyperlipidaemia is between 10 and 75% depending on the type of congenital heart defect.7 11 18 Cardiovascular disease is the leading cause of morbidity and mortality in patients with diabetes mellitus.8 Two recent studies concordantly reported that approximately 3% of ACHD patients had diabetes.12 17 The prevalence was the same for ACHD patients and a control group without congenital heart disease.17 In conclusion, all traditional CVRF associated with atherosclerotic disease are present in a substantial number of ACHD patients (table 1).

Clinical implications The clinical picture of CAD in ACHD can be similar to that seen in the population without a congenital heart defect.7 In a study of 250 patients with ACHD that underwent coronary angiography for reasons other than suspected CAD, significant CAD was present in around 10% of these patients.18 The prevalence was similar to that in the general population. By means of CVRF, systemic arterial hypertension and hyperlipidaemia were strongly associated with CAD in this ACHD cohort.18 Interestingly, no patient with an age below 40 years had significant CAD.18 In contrast, Yalonetsky et al7 reported that in their 1318

study, 14% of ACHD patients with CAD had premature CAD (age 65 years Various ACHD CoA VSD Various ACHD

3239 (56)

Range 65–80

1469 (49)

39 (IQR 29–51)

756 (48) 6481 (56) 152 (41)

31 (IQR 24–43) 30 (IQR 23–40) Median 28

Smoking history, sports participation, hypertension, BMI, diabetes Hypertension, dyslipidemia, diabetes, smoking history, family history of CAD. Diabetes, dyslipidemia, hypertension Smoking history, sports participation, BMI, diabetes Hypertension, dyslipidemia, diabetes BMI, dyslipidemia, diabetes

Zomer et al12 Roifman et al11 Martínez-Quintana et al17 Giannakoulas et al18

Various ACHD

250 (47)

56±13

51±15

Hypertension, dyslipidemia, diabetes, smoking history, family history of CAD

High prevalence of all risk factors. CAD also present in patients

Acquired heart conditions in adults with congenital heart disease: a growing problem.

The number of adults with congenital heart disease is increasing due to the great achievements in the field of paediatric cardiology, congenital heart...
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