Patients with coarctation of the aorta (CoA) may develop hypertension and cerebrovascular accident (CVA) late after intervention, but the risk is still unclear. Therefore, we studied the epidemiologic profile from a general population aged <60 years to assess the relative risk. Our study population consisted of 126,943 patients with congenital heart disease (CHD) derived from Taiwan’s National Health Insurance database from 2000 to 2010 (population 22,765,535). There were 2,295 patients with CoA (50.3% men) (prevalence: 0.116/1000). Associated cardiac lesions in 44% of the patients with CoA included ventricular septal defect (VSD; 23.3%), patent ductus arteriosus (7.5%), atrial septal defect (9.0%), and so forth. Hypertension occurred in 190 patients (8.3%) and was related to age, without associated VSD (odds ratio [OR] 5.90, 95% confidence interval [CI] 2.12 to 16.39, p = 0.001) and male gender (OR 1.59, 95% CI 1.09 to 2.30, p = 0.015). The incidence of hypertension increased rapidly in the young adulthood and was 1.36% in pediatric age group and 28.9% and 45.7% in age group 20 to 29 and 30 to 39, respectively. CVA occurred in 29 patients (1.67%), and the coexisting hypertension was the single predictor (OR 3.19, 95% CI 1.13 to 9.00, p = 0.029). Furthermore, in all patients with CHD, CoA was an independent risk factor for hypertension (OR 15.65, 95% CI 12.44 to 19.68, p <0.001) and CVA (OR 6.55, 95% CI 4.41 to 9.74, p <0.001). Comparing with non-CoA CHD patients, patients with CoA, particularly men, adult, and patients without VSD, have high risk of hypertension, which would further increase the risk of CVA. Early recognition and timely intervention for the hypertension in patients with CoA is mandatory.
Patients with coarctation of aorta (CoA) late after the CoA intervention are at risk of systemic arteriopathy, including systemic hypertension and intracranial aneurysm or hemorrhage. From our previous institutional cohort study, the 20-year freedom from hypertension was 78.6% and 51.0% in transcatheter and surgical intervention groups, respectively. The development of hypertension was not related to levels of renin and angiotensin. Other than hypertension, patients with CoA are also associated with intracranial aneurysms. A prospective screening of 43 adult patients with CoA revealed the presence of intracranial aneurysms in 5 (11%). With co-existing hypertension, these patients might be at a greater risk of intracranial hemorrhage or cerebrovascular accident (CVA) from these aneurysms. However, the incidence and the relative risk of these complications, particularly the CVA, in patients with CoA remain still unclear.
The population of Taiwan is approximately 23 million. The national health insurance program was implemented in 1995 and covered >99% of the general population. The health care system in Taiwan is regarded as sound and the child health index is similar to that in United States. The number of patients with CoA and late cardiovascular complications in Taiwan, a country with fully covered and easily accessible medical care, would adequately reflect the late cardiovascular and intracranial risk in patients with CoA. Therefore, this study, based on our national database spanning for 11 years, explored the current epidemiologic profile of CoA, focusing on the late development of hypertension and CVA.
Methods
The old age from World Health Organization’s definition starts from age 60 to 65 years. To avoid the compounding from other diseases commonly encountered in old ages, we enrolled only those aged <60 years at the last medical visits. All the health care records logged from January 1, 2000, to December 31, 2010, were retrieved from the complete computerized database of National Health Insurance. The average general population aged <60 years was 19,725,030. We selected patients who met the criteria listed for congenital heart disease (CHD), on the basis of the International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) code, who constituted the CHD cohort. Of them, patients with CoA (code 747.10 or 747.11) were enrolled. Each health record had a scrambled identification number and contained information such as the patient’s date of birth, date of hospital visit, gender, type of visit (admission or outpatient department visit), diagnosis, and treatment codes. To avoid the error from a tentative diagnosis, only those who received OPD visits more than twice or admission under the CHD-specific diagnosis were enrolled. Those patients with CoA who had associated severe CHD, such as, atrioventricular or ventriculoarterial discordance, or double outlet ventriculoarterial connection were not included in the CoA cohort.
Identified patients were tracked for any complications until December 31, 2010. The occurrence of complication was defined when the respective disease codes were listed in the list of the diagnosis (hypertension diagnosis, ICD codes 401, 402, 404, and 405: CVA, ICD-9 codes 430 to 437). Besides, intracranial hemorrhage was further defined when the ICD-9 codes 430 (subarachnoid hemorrhage), 431 (intracerebral hemorrhage), or 432 (other intracerebral hemorrhage) was listed as the diagnosis. Patients who had received antihypertensive medications for more than 2 OPD visits (>2 months) were also identified. Because the purpose of the medications, such as diuretics, angiotensin-converting enzyme inhibitors, angiotensin receptor blocker, and β blockers, varies more widely, we limit the index antihypertensive drug to calcium channel blockers, except that used in patients with coexisting coronary arterial disease or arrhythmias. Those patients were also listed as patients with hypertension. Mortality was defined as the event of death occurring at discharge and confirmed by the status of health insurance at December 31, 2010.
We used the Statistical Package for Social Sciences statistical software (SPSS, Version 15.0; SPSS Inc., Chicago, Illinois) for analysis. The estimation of CoA prevalence was driven from the population from 2000 to 2010, which were adopted from the Statistical Yearbook of Interior, Department of Statistics, Ministry of Interior. We used the chi-square test to analyze the associations between categorical variables. Multivariate logistic regression was then applied to identify the predictors. All p values were 2 sided, and the statistical significance was defined while a p value was <0.05. Kaplan–Meier analysis was used for the estimate for overall event-free rate of hypertension.
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