Top Things to Know: Evaluation and Management of Chronic Heart Failure in Children and Adolescents with Congenital Heart Disease
Published: May 29, 2024
- As of 2023, a significant proportion of children diagnosed with congenital heart disease (CHD) are expected to survive into adulthood. The emergence of heart failure (HF) as a leading cause of morbidity and mortality in aging CHD patients is increasingly acknowledged.
- Both HF with reduced ejection fraction and HF with preserved ejection fraction manifest in congenital HD patients. The origins of HF in this demographic differ from those in adults, often stemming from chronic myocardial stress due to underlying congenital HD-related physiological factors (such as chronic pressure or volume overload, cyanosis, impaired coronary blood flow) or myocardial injury (resulting from exposure to cardiopulmonary bypass or perioperative low cardiac output). Additionally, genetic predisposition in congenital HD patients may elevate the risk of developing cardiomyopathy leading to HF.
- Concurrent conditions like arrhythmias, pulmonary hypertension, and cyanosis frequently co-occur with congenital HD, potentially exacerbating HF. Furthermore, common comorbidities in congenital HD patients—renal, hepatic, pulmonary, and neurological—may complicate the administration of medical and surgical HF treatments.
- Evaluating HF severity in children and adolescents with congenital HD necessitates a comprehensive array of assessment tools, encompassing the Ross/New York Heart Association functional class, frequency of acute decompensated heart failure episodes, growth percentiles, natriuretic peptides, and both noninvasive and invasive measurements of cardiac structure and physiology.
- Data evaluating the safety and efficacy of adult HF therapies in children and adolescents with congenital HD is sparse. The only two randomized controlled trials of HF therapies in children with subaortic right or single ventricles failed to demonstrate a treatment benefit of enalapril or carvedilol over a placebo.
- Managing HF in congenital HD patients encompasses more than addressing ventricular dysfunction; it also involves managing conditions like anemia and malnourishment and mitigating risk factors for acquired cardiovascular diseases such as diabetes, and hypertension.
- Tachy- and bradyarrhythmias are commonplace in congenital HD patients and necessitate prompt intervention due to their potential to exacerbate or trigger HF development. Collaborative efforts between HF specialists and electrophysiologists are crucial for evaluating the suitability of permanent pacing or cardiac resynchronization therapy in preventing or ameliorating HF in congenital HD patients.
- The unique nature of congenital HD often requires customized ventricular assist device configurations and poses surgical and perioperative challenges during device implantation or transplantation. Timely referral of children and adolescents with congenital HD and HF to specialized teams of pediatric advanced HF and transplant clinicians is pivotal for optimizing outcomes.
- Establishing uniform definitions, staging criteria, follow-up protocols, and treatment approaches among health care professionals is essential for fostering multi-institutional collaborations that can generate evidence to enhance HF outcomes in children and adolescents with congenital HD.
- A robust collaborative partnership involving researchers, health care professionals, patients, caregivers, and public and private funding agencies is imperative for generating high-quality data.
Citation
Amdani S, Conway J, George K, Martinez HR, Asante-Korang A, Goldberg CS, Davies RR, Miyamoto SD, Hsu DT; on behalf of the American Heart Association Council on Lifelong Congenital Heart Disease and Heart Health in the Young; Council on Arteriosclerosis, Thrombosis and Vascular Biology; Council on Cardiovascular Surgery and Anesthesia; and Council on Cardiovascular and Stroke Nursing. Evaluation and management of chronic heartfailure in children and adolescents with congenital heart disease: a scientific statement from the AmericanHeart Association. Circulation. Published online May 29, 2024. doi: 10.1161/CIR.0000000000001245