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A Multifaceted Approach to Pulmonary Hypertension in Adults With Congenital Heart Disease
Mouhammad Fathallah1, Richard A Krasuski1
1Department of Cardiovascular Medicine, Duke University Health System, Durham, NC.
Insights
Adults with congenital heart disease (CHD) may develop pulmonary arterial hypertension (PAH-CHD), a serious complication. Early diagnosis and specialized care, including hemodynamic assessment, are crucial for managing PAH-CHD and improving patient outcomes.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Pulmonary Hypertension
Background:
- Congenital heart disease (CHD) management advances have increased adult survivors.
- Pulmonary arterial hypertension related to CHD (PAH-CHD) affects up to 10% of patients, even post-repair.
- PAH-CHD can lead to severe, fatal consequences, necessitating careful patient evaluation.
Purpose of the Study:
- To review current understanding of PAH-CHD.
- To emphasize the importance of specialized care and collaboration for PAH-CHD management.
- To highlight the expanding therapeutic options for PAH-CHD.
Main Methods:
- Review of current literature on PAH-CHD.
- Emphasis on hemodynamic assessment for therapeutic approach.
- Discussion of surgical, transcatheter, and medical management strategies.
Main Results:
- PAH-CHD is a common complication of adult congenital heart disease.
- Timely defect correction and advanced medical therapies may improve quality of life and survival.
- Management of milder degrees of PAH-CHD requires further study.
Conclusions:
- Close follow-up in specialized centers is essential for PAH-CHD patients.
- Multidisciplinary collaboration is key in managing complex PAH-CHD cases.
- Expanding treatment options offer improved prospects for PAH-CHD patients.
Abstract:
Advances in the management of congenital heart disease (CHD) in children have resulted in growing numbers of adults with CHD. Pulmonary arterial hypertension related to CHD (PAH-CHD) is a common complication, affecting up to 10% of patients; and can arise even after successful and complete defect repair, with severe and potentially fatal consequences. Careful work-up in these patients is essential, particularly hemodynamic assessment, and can help define the most appropriate therapeutic approach. Management can be challenging, but the therapeutic armamentarium is continually expanding and now includes surgical, transcatheter and medical options. Timely correction of defects along with early treatment with advanced medical therapies appears to improve quality of life and possible even improve survival. Interestingly most studies of PAH-CHD have focused on its most severely afflicted patients, those with Eisenmenger Syndrome, making it less certain how to manage PAH-CHD of milder degrees. This review summarizes our current understanding of PAH-CHD and emphasizes the need for close follow-up in specialized centers of care where close collaboration is common practice.
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