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These studies suggest congenital heart failure is a condition where congenital heart disease patients exhibit chronic heart failure symptoms, requiring management based on heart failure paradigms, with distinct characteristics from adult heart failure and a need for targeted medical care.
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Congenital heart failure is a condition that arises from congenital heart disease (CHD), which refers to structural defects in the heart present at birth. These defects can lead to heart failure, a complex clinical syndrome characterized by the heart's inability to pump blood efficiently, resulting in symptoms such as exercise limitation, neurohormonal activation, and cardiac abnormalities .
Congenital heart disease is the most common congenital disorder, affecting approximately 0.8% to 1% of live births . Advances in medical and surgical care have significantly improved survival rates, allowing more than 85% of children with CHD to reach adulthood . Consequently, there is a growing population of adults living with CHD, who are at risk of developing heart failure due to residual cardiac issues and complications .
Heart failure in patients with congenital heart disease can result from various factors, including surgical interventions, abnormal preoperative loading conditions, and genetic causes. Recent research indicates that congenital heart malformations and abnormal ventricular function may share underlying genetic pathways. The pathophysiological criteria of heart failure in CHD patients include cardiac abnormalities, exercise limitations, and neurohormonal activation, similar to other forms of heart failure.
The clinical manifestations of congenital heart failure vary depending on the specific type of heart defect. Common symptoms include rapid breathing, bluish skin (cyanosis), low weight, and fatigue. Complications can be severe and include arrhythmias, endocarditis, pulmonary hypertension, and the need for repeat surgeries . Heart failure is a frequent cause of death in adults with CHD, and hospital admissions for heart failure are associated with a significantly increased mortality rate.
Current management and treatment strategies for heart failure in children with congenital heart disease are often extrapolated from adult treatment protocols. These include pharmacologic neurohormonal antagonism and exercise therapy, which have shown symptomatic and prognostic benefits . However, there is a need for more tailored therapeutic approaches based on the heart failure paradigm specific to congenital heart disease.
Emerging research is focusing on the genetic and molecular characteristics of heart failure in congenital heart disease, aiming to provide a better understanding of the disease and improve treatment outcomes. High-priority research areas include heart failure in specific CHD conditions like tetralogy of Fallot, mechanical circulatory support, transplantation, and the impact of genetic and epigenetic factors.
Congenital heart failure is a significant and growing concern due to the increasing population of adults with congenital heart disease. Understanding the pathophysiology, clinical manifestations, and complications of this condition is crucial for developing effective management and treatment strategies. Ongoing research and advancements in medical care are essential to improve the quality of life and outcomes for patients with congenital heart failure.
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