Related Experiment Video
Updated: Apr 17, 2026
Precision Ultrasound-guided Stem Cell Delivery for Vascular Repair in Aortic Diseases
Published on: June 20, 2025
Aortic Involvement in Pediatric Marfan syndrome: A Review
Omonigho Ekhomu1, Zahra J Naheed
1Department of Pediatrics, John H. Stroger Hospital, 1901 W. Harrison Street, Chicago, IL, USA, omy1heart@yahoo.com.
Insights
Managing pediatric Marfan syndrome, particularly cardiovascular issues like aortic disease, is complex. This review details management strategies to improve survival rates for affected children.
Area of Science:
- Cardiovascular Medicine
- Pediatric Genetics
- Syndromic Diseases
Background:
- Marfan syndrome presents significant challenges in pediatric management due to limited clinical studies.
- Cardiovascular complications are the primary cause of morbidity and mortality in Marfan syndrome.
Purpose of the Study:
- To review aortic involvement in pediatric Marfan syndrome, including dilatation, rupture, and heart failure.
- To explore the histological, morphological, and pathogenetic basis of cardiac manifestations.
- To discuss current and potential management strategies, including medical and surgical therapies, for improved survival.
Main Methods:
- Literature review focusing on pediatric Marfan syndrome and cardiovascular manifestations.
- Analysis of histological, morphological, and pathogenetic factors.
- Case study illustration of cardiovascular manifestations.
Main Results:
- Aortic root dilatation is a common finding, progressing to dissection, rupture, and heart failure.
- Understanding the underlying pathophysiology is crucial for effective management.
- Optimal management can significantly improve survival, approaching that of the general population.
Conclusions:
- Effective management protocols for pediatric Marfan syndrome are essential for improving patient outcomes.
- A multidisciplinary approach integrating medical and surgical interventions is key.
- Further research in pediatric populations is needed to refine treatment strategies.
Abstract:
Outlining specific protocols for the management of pediatric patients with Marfan syndrome has been challenging. This is mostly due to a dearth of clinical studies performed in pediatric patients. In Marfan syndrome, the major sources of morbidity and mortality relate to the cardiovascular system. In this review, we focus on aortic involvement seen in pediatric patients with Marfan syndrome, ranging from aortic dilatation to aortic rupture and heart failure. We discuss the histological, morphological, and pathogenetic basis of the cardiac manifestations seen in pediatric Marfan syndrome and use a specific case to depict our experienced range of cardiovascular manifestations. The survival for patients with Marfan syndrome may approach the expected survival for non-affected patients, with optimal management. With this potentiality in mind, we explore possible and actual management considerations for pediatric Marfan syndrome, examining both medical and surgical therapy modalities that can make the possibility of improved survival a reality.
More Related Videos
11:20A Rodent Model of The Ross Operation: Syngeneic Pulmonary Artery Graft Implantation in A Systemic Position
Published on: April 1, 2022
06:51Author Spotlight: Development of a Minimally Invasive Large-Animal Model for Reliable and Reproducible Cardiovascular Research
Published on: October 20, 2023