A.C. Zomer

Heart failure admissions in adults with congenital heart disease; risk factors and prognosis

BACKGROUND Heart failure (HF) is a serious complication and often the cause of death in adults with congenital heart disease (CHD). Therefore, our aims were to determine the frequency of HF-admissions, and to assess risk factors of first HF-admission and of mortality after first HF-admission in adults with CHD. METHODS The Dutch CONCOR registry was linked to the Hospital Discharge Registry and National Mortality Registry to obtain data on HF-admissions and mortality. Risk factors for both HF-admission and mortality were assessed using Cox regression models. RESULTS Of 10,808 adult patients (49% male), 274 (2.5%) were admitted for HF during a median follow-up period of 21 years. The incidence of first HF-admission was 1.2 per 1000 patient-years, but the incidence of HF itself will be higher. Main defect, multiple defects, and surgical interventions in childhood were identified as independent risk factors of HF-admission. Patients admitted for HF had a five-fold higher risk of mortality than patients not admitted (hazard ratio (HR)=5.3; 95% confidence interval 4.2-6.9). One- and three-year mortality after first HF-admission were 24% and 35% respectively. Independent risk factors for three-year mortality after first HF-admission were male gender, pacemaker implantation, admission duration, non-cardiac medication use and high serum creatinine. CONCLUSIONS The incidence of HF-admission in adults with CHD is 1.2 per 1000 patient-years. Mortality risk is substantially increased after HF-admission, which emphasises the importance to identify patients at high risk of HF-admission. These patients might benefit from closer follow-up and earlier medical interventions. The presented risk factors may facilitate surveillance.

Adult congenital heart disease: New challenges

Improved surgical care from the last five decades, together with the advances in medical management, led to a remarkable increase of survival of patients with congenital heart disease (CHD). However, aging of the CHD population brings new challenges. Given that many patients with CHD are prone to residua and sequelae, life-long surveillance is essential. Therefore, the first of many challenges is to optimize the transition of patients with CHD from pediatric to adult cardiology to prevent lost to follow-up, and to ensure that there are enough specialized adult cardiologists to take care of this expanding patient population. Another important challenge is the expansion of knowledge on long-term complications and comorbidity in these patients. Increased efforts are needed to gain further understanding on how to prevent and treat these. Furthermore, as patients reach the reproductive age, family planning becomes more important and research on the risks and management of pregnancy should be further extended. Finally, to improve the well-being and social life of adults with CHD efforts should be made to improve employability and insurability.

Chance of surgery in adult congenital heart disease

Background Young patients with congenital heart disease reaching adulthood face mandatory transition to adult cardiology. Their new cardiologist needs to assess the chances of major future events such as surgery. Using a large national registry, we assessed if patient characteristics at the age of 18 years could predict the chance of congenital heart surgery in adulthood. Design and methods Of 10,300 patients from the CONCOR national registry, we used general patient characteristics at age 18 years, underlying congenital heart defect, history of complications, and interventions in childhood as potential predictors of congenital heart surgery occurring from age 18 years up to age 40 and 60 years. Cox regression was used to calculate hazard ratios with 95% confidence intervals. Analyses were performed separately for all congenital heart surgery and for valvular surgery alone. Results Altogether 2427 patients underwent congenital heart surgery after age 18 years, 1389 of whom underwent valvular surgery. Underlying heart defect, male sex, multiple defects, childhood endocarditis, supraventricular arrhythmia, aortic complications and paediatric cardiovascular surgery, independently predicted adult congenital heart surgery. The mean chance of congenital heart surgery was 22% up to age 40 and 43% up to age 60 years; individual chances spanned from 9–68% up to age 40 and from 19–93% up to age 60 years. Conclusion At the time of transition from paediatric to adult cardiology, an easily obtainable set of characteristics of patients with congenital heart disease can meaningfully inform cardiologists about the patients individual chance of surgery in adulthood. Our findings warrant validation in other cohorts.

EPIDEMIOLOGY OF PULMONARY ARTERIAL HYPERTENSION IN CONGENITAL HEART DISEASE; NATIONWIDE PREVALENCE AND IMPACT ON SOCIOECONOMIC STATUS

Due to advances in surgical and medical care, survival has improved in patients with congenital heart disease (CHD). This has led to an increasing number of surviving patients with systemic to pulmonary shunt, who are at risk of developing pulmonary arterial hypertension (PAH). We aimed to determine

MORTALITY IN CONGENITAL HEART DISEASE; ARE GOVERNMENTAL REGISTRIES RELIABLE FOR CAUSE OF DEATH?

Results: Of our nationwide registry (n=7277, on date of linkage) we could link 95% (n=6933) to the governmental mortality registry. In the governmental mortality registry 196 patients (2.4%) were found and recorded deceased, versus 228 deceased patients (3.1%) recorded in our nationwide registry, during a follow up of 18,100 patient years. Median age at death was 49 years (range 20 to 91 years). Of all deaths in our registry, 77% had a cardiovascular origin; nearly 50% were due to progressive heart failure and arrhythmias. The governmental mortality registry recorded death due to progressive heart failure and arrhythmias in only 8.5%. Moreover, this registry recorded death with an ‘unspecified’ cause in approximately 30%, primarily containing patients who died from progressive heart failure and arrhythmias according to their medical records.