Treatment of heart failure in adult congenital heart disease: a position paper of the Working Group of Grown-Up Congenital Heart Disease and the Heart Failure Association of the European Society of Cardiology

Abstract

Improved medical care of congenital heart disease patients increased survival into adulthood from 15% in the 1960s to over 85% in the current era. As a consequence, the prevalence of adult congenital heart disease (ACHD) increased rapidly, 1 which is estimated to be .1 million ACHD patients in North America and 1.2 million in Europe. The growing number and aging of ACHD patients led to an overall increase in hospitalizations over the last decade and a substantial increase in patients presenting with heart failure (HF) ( 20%). 2 The incidence of first HF-admission was 1.2 per 1000 patientyears in the Dutch national 'CONCOR' registry. Patients admitted with HF had a five-fold higher risk of death than those not admitted. From the same registry, the mortality was 2.8% during a follow-up period of 24 865 patient-years. Chronic HF (26%) and sudden death (19%) were recorded most frequently. The median age at death from HF was 51.0 years (range: 20.3 -91.2 years). 3 In another ACHD cohort, sudden death (26%) was the most common cause of death, followed by progressive HF (21%) and perioperative death (18%). 4 Although patients with ACHD may not readily report symptoms, clinical HF is documented in 22.2% of patients with a Mustard

Platelet count and mean platelet volume predict outcome in adults with Eisenmenger syndrome

“…Moreover, the associated drop in platelet count is feared to promote bleeding, even though no relation between platelet count and bleeding events was found in this study. Iron deficiency is associated with an increase mortality in ES,30 and while the therapeutic dilemma of how aggressively to treat it remains, the risk of promoting thrombotic or bleeding events by doing so is low, supporting our current approach of being proactive in screening and treating iron deficiency in ES.…”

Appearance of QRS fragmentation late after Mustard/Senning repair is associated with adverse outcome

“…Risk stratification remains complex 7. Both a high RV afterload due to systemic pressures and a volume overload due to increasing regurgitation of the systemic atrioventricular valve (SAVV) can lead to worsening RV systolic function and development of heart failure 8. An important contributor to the decline in clinical status is myocardial fibrosis 9.…”

Outcome of congenital heart disease with modern cardiac care

“…7 Atrial septal defects are among the most common congenital heart defects. Although the lesion can be closed nowadays with interventional or surgical techniques, timing and indication for such procedures to prevent pulmonary hypertension, 8 right heart failure, 3 and premature death is still debated. Camilla Nyboe and colleagues from the Aarhus University Hospital in Denmark address this issue in their paper entitled 'Long-term mortality in patients with atrial septal defect: a nationwide cohort-study'.…”