Heart failure in adults with congenital heart disease: a call for action

Abstract

Major advances in the management of children with congenital heart disease (CHD) have evolved over the past five decades. As a result, most of the children reach adulthood, and the population of adults with CHD is exponentially growing. This evolution is particularly marked for patients with complex CHD. Consequently, the spectrum of congenital lesions is changing over time, with more patients having complex CHD reaching older age with additional acquired comorbidities. Heart failure (HF) is the most common complication in adults with CHD (ACHD) with a life prevalence of at least 30% in most patients with complex underlying diagnoses. This comorbidity is become the leading cause of premature death in this population. As more patients with complex heart disease survive to older age and there is a general increase in the ACHD population, HF is becoming an epidemic. This evolution has important implications on healthcare organisation and resources for ACHD. The originality and strength of the paper by Burstein et al is to compare the contemporary use of hospital resources and outcomes in patients with ACHD with HF (ACHDHF) and those without ACHD with HF (HFnonACHD) using two administrative databases in the USA, the Nationwide Emergency Department Sample and Nationwide Inpatient Sample. They confirmed the dramatic augmentation of HF prevalence in ACHD with an increase in rate of HFrelated hospitalisations of 46% between 2006 and 2016 compared with 6% in patients with no ACHD. This progression was particularly high in one of the most complex CHDs which was CHD with single ventricle usually palliated by Fontan circulation. However, HFrelated hospitalisation may only be the tip of the iceberg. Indeed, the study did not point chronic HF usually followed in outpatients and indirect costs of ACHDHF such as hospitalisations related to comorbidities (ie, arrhythmia or chronic kidney disease). Several results from this study underscore the high severity of HFACHD, particularly in the CHD with single ventricle physiology. Compared with HFnonACHDs, patients with HFACHD had a higher incidence of arrhythmia and pulmonary hypertension, were more frequently hospitalised after presenting to the emergency department, were more frequently treated with advanced cardiac therapy and had the highest hospital mortality. Preventive strategy of HF in patients with ACHD should be focused on optimising medication therapy and intervening on any residual haemodynamic lesions. This may be challenged by the fact that many adults with CHD and HF do not present with typical symptoms, making it more difficult to detect HF early. For the same reason, they are at risk of presenting late in their disease with no opportunity for medical stabilisation. Few predictors of ACHDHF have been studied to help in detecting, monitoring and preventing HF in this population and could implement outpatient followup, even if they need prospective validation on large ACHD population. In the other side, advanced cardiac therapies in this heterogeneous population are complex. Although ACHDHF frequently present with more advanced disease than nonACHD, they experience longer wait time. Indeed, they are listed at lower urgency and most heart allocation systems place adults with CHD at a disadvantage due to the unique nature of their HF. Mechanical circulatory support (MCS) options are limited in ACHD for numerous factors including nonstandard anatomy, multiple prior sternotomies, persistent shunts, malnutrition from proteinlosing enteropathy or cirrhosis from chronic rightsided congestion (ie, Fontan). Burstein et al found that HF in ACHD with two ventricles was less likely treated with a ventricular assist device (VAD) compared with HFnonACHD, and that mortality was over 50% in ACHD who received VAD. Similarly, ACHD accounted for less than 1% of patients with MCS in the INTERMACS US registry and had higher mortality (28% at 1 year). The latter outcome was attributable to more prevalent use of biventricular assist devices or a total artificial heart. All these outcomes suggest that advanced cardiac therapies are considered too late during HF course in ACHD, in too severe patients. Advanced care planning should begin long before the clinical manifestation of ACHDHF and patients should be referred for consideration of advanced therapies when clinical manifestations of HF start or when specific markers change during patient monitoring. Despite improvements in patient selection and preoperative management, perioperative morbidity and mortality remain high in ACHD who undergo transplantation reaching 30% in CHD with single ventricle physiology. It is essential to monitor outcomes and to relate them to identified risks. Early death due to technical reasons is high among ACHD heart transplant recipients. These factors are modifiable with a decrease in mortality as transplant volume and surgical expertise increase, combining expertise in congenital cardiac surgery and transplant surgery. This was well demonstrated by Menachem et al who reported superior outcomes among mediumvolume and highvolume heart transplantation centres, relative to lowvolume centres (<14 heart transplants per year). Burstein et al study attested to the great complexity of HF management in patients with single ventricle physiology (Fontan). Patients with single ventricle constituted the youngest population (median age 32), but they required more frequently advanced cardiac therapy and had the highest mortality rates. Two comorbidities are specific to patients with Fontan: proteinlosing enteropathy and Fontanassociated liver disease. These comorbidities in addition to the physiological and technical challenges of VAD or transplant in Fontan population explain the high mortality. Consequently, optimal HF management in CHD with single ventricle required intense multidisciplinary and highly specialised collaboration. Finally, the study highlighted the insufficiency of current healthcare system resources to meet the needs of the growing number of patients with advanced ACHDHF. VAD support in ACHDHF remained unchanged over the study period (2006– 2016) and the proportion of patients with HF who underwent heart transplantation decreased, while centres offering ACHD heart transplant are assessing and listing increasing numbers of patients, with INSERM U970, Paris Centre de Recherche Cardiovasculaire, Paris, France Université de Paris, Paris, France Adult Congenital Heart Disease Unit, Department of Cardiology, Centre de référence M3C, Hopital Europeen Georges Pompidou, APHP, Paris, France