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Published on: November 30, 2015
A blue baby grows up
Jamil A Aboulhosn1, John S Child
1Ahmanson/UCLA Adult Congenital Heart Disease Center, Los Angeles, Calif 90095-1679, USA. jaboulhosn@mednet.ucla.edu
Insights
This case report details a 41-year-old adult with complex congenital heart disease, focusing on pulmonary arterial hypertension management. It highlights clinical challenges in caring for adults with palliated cyanotic heart conditions.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Adult Congenital Heart Disease
Background:
- Complex congenital heart disease (CCHD) requires lifelong management.
- Palliative surgical interventions in infancy can lead to long-term complications.
- Pulmonary arterial hypertension (PAH) is a significant concern in adults with CCHD.
Observation:
- A 41-year-old female with multiple congenital heart defects, including atresia of the right atrioventricular valve and malposed great arteries, presented with PAH.
- The patient had undergone pulmonary artery banding in infancy but did not receive corrective surgery due to PAH development.
Findings:
- Pulmonary arterial hypertension (PAH) can develop or progress in adults with complex, palliated congenital heart disease.
- Management of PAH in this population presents unique clinical challenges.
- This case underscores the importance of monitoring and managing PAH in adults with CCHD.
Implications:
- Adult congenital heart disease (ACHD) programs must address the complexities of PAH.
- Early recognition and tailored treatment strategies are crucial for improving outcomes in adults with CCHD and PAH.
- Further research is needed to optimize PAH management in the ACHD population.
Abstract:
This report describes the history of a 41-year-old female patient born with atresia of the right atrioventricular valve, hypoplasia of the right ventricle, an atrial septal defect, and malposed great arteries. The patient underwent surgical pulmonary artery banding in infancy but did not undergo corrective surgery due to the development of pulmonary arterial hypertension. This report touches on several important clinical issues that may arise when caring for the adult with complex palliated cyanotic congenital heart disease, including the development and treatment of pulmonary arterial hypertension.
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