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Heart failure treatment in adults with congenital heart disease: where do we stand in 2014?
  1. Eric V Krieger1,
  2. Anne Marie Valente2
  1. 1Division of Cardiology, Department of Medicine, Seattle Adult Congenital Heart Service, University of Washington Medical Center and Seattle Children's Hospital, University of Washington School of Medicine, Seattle, Washington, USA
  2. 2Boston Adult Congenital Heart Disease and Pulmonary Hypertension Program Department of Cardiology, Boston Children's Hospital Department of Medicine, Division of Cardiology, Brigham and Women's Hospital Harvard Medical School, Boston, MA, USA
  1. Correspondence to Dr Anne Marie Valente, Boston Adult Congenital Heart Disease and Pulmonary Hypertension Program. Department of Cardiology, Boston Children's Hospital. 300 Longwood Avenue, Boston, MA 02115, USA; amvalente{at}


Heart failure (HF) is the leading cause of death in adults with repaired congenital heart disease (CHD). However there is currently little evidence to guide treatment strategies in this growing group of patients. Unlike the majority of HF, which is usually caused by LV systolic or diastolic dysfunction, CHD-HF is more often a consequence of RV disease, valve dysfunction, shunting or pulmonary hypertension. It is therefore not appropriate to extrapolate from the acquired HF literature and apply it to this heterogeneous population of CHD patients. Additionally, patients with CHD have been excluded from most large trials of medical or device therapy of HF, which has resulted in small retrospective and underpowered studies in the CHD population. This article critically reviews the current knowledge about CHD-HF, paying particular attention to medical therapy in different CHD populations, cardiac resynchronisation therapy and implantable cardiac defibrillators, and the challenges of heart transplantation and mechanical circulatory support in CHD patients.


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