High burden of drug therapy in adult congenital heart disease:polypharmacy as marker of morbidity and mortality

Aims To assess medication use in adult congenital heart disease (ACHD) patients compared to the age- and sex-matched general population, identify patterns of pharmacotherapy, and analyse associations between pharmacotherapy and adverse outcomes in ACHD.Methods and results Data of 14 138 ACHD patients from the CONCOR registry [35 (24-48) years, 49% male] and age- and sex-matched referents (1:10 ratio) were extracted from the Dutch Dispensed Drug Register for the years 2006-14. Adult congenital heart disease patients had more cardiovascular and non-cardiovascular drugs than referents (median 3 vs. 1, P= 5 dispensed drug types yearly, was present in 30% of ACHD and 15% of referents {odds ratio [OR]=2.47 [95% confidence interval (CI) 2.39-2.54]}. Polypharmacy was independently associated with female sex [OR=1.92 (95% CI 1.88-1.96)], older age [for men: OR=2.3/10years (95% CI 2.2-2.4) and for women: OR=1.6/10years (95% CI 1.5-1.6); P-interactionConclusion Both cardiovascular and non-cardiovascular medication use is high in ACHD with twice as much polypharmacy compared with the matched general population. Patients with polypharmacy had a four-fold increased risk of mortality and adverse drug events. Recognition of distinct medication patterns can help identify patients at highest risk. Drug regimens need repeating evaluation to assess the appropriateness of all prescriptions. More high-quality studies are needed to improve ACHD care with more evidence-based pharmacotherapy.</p

Secundum atrial septal defect in adults: a practical review and recent developments

Secundum atrial septal defect (ASDII) is a common congenital heart defect that causes shunting of blood between the systemic and pulmonary circulations. Patients with an isolated ASDII often remain asymptomatic during childhood and adolescence. If the defect remains untreated, however, the rates of exercise intolerance, supraventricular arrhythmias, right ventricular dysfunction and pulmonary arterial hypertension (PAH) increase with patient age, and life expectancy is reduced. Transcatheter and surgical techniques both provide valid options for ASDII closure, the former being the preferred method. With the exception of those with severe and irreversible PAH, closure is beneficial to, and thus indicated in all patients with significant shunts, regardless of age and symptoms. The symptomatic and survival benefits conferred by defect closure are inversely related to patient age and the presence of PAH, supporting timely closure after diagnosis. In this paper we review the management of adult patients with an isolated ASDII, with a focus on aspects of importance to the decision regarding defect closure and medical follow-u

Aortic dissection and prophylactic surgery in congenital heart disease

Contains fulltext : 198450.pdf (publisher's version ) (Open Access

High burden of drug therapy in adult congenital heart disease: polypharmacy as marker of morbidity and mortality

Contains fulltext : 209444.pdf (publisher's version ) (Open Access

Factors associated with long-term mortality after Fontan procedures: a systematic review

Despite an ageing Fontan population, data on late outcomes are still scarce. Reported outcome measures and determinants vary greatly between studies making comprehensive appraisal of mortality hazard challenging. We conducted a systematic review to evaluate causes and factors associated with late mortality in patients with Fontan circulation. Late mortality was defined as mortality beyond the first postoperative year. Studies were included if they had ≥90 patients or ≥20 late mortalities and/or transplants. Studies with overlapping patients were rationalised to include only the most recent studies to avoid duplication. From 28 studies, a total of 6707 patients with an average follow-up time of 8.23±5.42 years was identified. There were 1000 deaths. Causes of late death were reported in 697 cases. The five most common causes were heart/Fontan failure (22%), arrhythmia (16%), respiratory failure (15%), renal disease (12%) and thrombosis/bleeding (10%). Factors associated with late mortality were evaluated and classified into 9 categories. Causes and factors associated with late mortality after the Fontan operation are summarised in this study. The presented information will aid in identifying patients at highest risk for mortality and guide our risk stratification efforts in this patient populatio

Predicting long-term mortality after Fontan procedures: A risk score based on 6707 patients from 28 studies

Reported long-term outcome measures vary greatly between studies in Fontan patients making comprehensive appraisal of mortality hazard challenging. We sought to create a clinical risk score to assist monitoring of Fontan patients in the outpatient setting. A systematic review was conducted to evaluate risk factors for long-term (beyond the first postoperative year) mortality in Fontan patients. Studies were eligible for inclusion if ≥90 patients were included or ≥20 long-term mortalities we reported. Risk factors for long-term mortality were determined. The pooled hazard ratios were used to create components of a clinical score for long-term mortality using meta-analysis techniques. Twenty-eight studies were included. The total number of patients was 6707 with an average follow-up of 8.23 ± 5.42 years. There were 1000 deaths. Thirty-five risk factors for late mortality were identified and classified into 9 categories and their relative hazards were used to derive the initial components of a weighted, practical and clinically based Fontan risk score (ranging from 0 to 100). The final score included 8 risk factors: anatomic risk factors, elevated preoperative pulmonary artery pressure, atriopulmonary Fontan, heart failure symptoms, arrhythmia, moderate/severe ventricular dysfunction or atrioventricular valve regurgitation, protein losing enteropathy, and end organ disease (cirrhosis or renal insufficiency). In patients with Fontan circulation, the influence of readily available risk factors can be quantified in an integer score to predict long-term mortality. Prospective validation and refinement of this risk score will be undertake

Resuscitation for out-of-hospital cardiac arrest in adults with congenital heart disease

Aims: Adult congenital heart disease (ACHD) patients are at increased risk of sudden cardiac death and out-of-hospital cardiac arrest (OHCA). Currently, insufficient data exist on outcome, causes and circumstances of OHCA of ACHD patients resuscitated for OHCA. We investigate these parameters in ACHD patients in comparison to OHCA in the general population. Methods and results: We identified ACHD patients with OHCA by linking data from a Dutch nationwide registry of ACHD patients (CONCOR, n = 15,727), and ARREST, a cohort of OHCA cases (n = 17,868). 62 ACHD patients with OHCA were identified. Ventricular septal defect (n = 11), bicuspid aortic valve (n = 10) and atrial septal defect (n = 8) were the most common diagnoses. We included OHCA cases from the general population as controls. ACHD patients were younger than controls (n = 11,624) at the time of OHCA (47 (SD ± 17) years vs. 66 (SD ± 15) years, respectively, p < 0.001), and more often had a shockable initial rhythm (67% vs 40%, respectively, p < 0.001). A cardiac cause of OHCA was identified in 76% of ACHD patients, with only 7% due to myocardial infarction or ischemia. Survival was better in ACHD patients than in controls (44% vs. 19%, p < 0.001), but this difference disappeared after correction for age, gender, witnessed arrest, bystander resuscitation, public location and shockable rhythm. Conclusions: OHCA in ACHD patients occurs at young age, is rarely caused by ischemia and occurs mainly in patients with simple congenital defects. Risk stratification efforts should therefore not be restricted to ACHD patients with severe congenital defects