Management of Cardiovascular Health Issues in Turner Syndrome: Expert Insights and Expanded Recommendations From the...

Area of Science:

• Cardiology
• Genetics
• Pediatrics

Background:

• Turner syndrome (TS) is frequently associated with congenital heart disease (CHD).
• Common CHD in TS includes bicuspid aortic valve and coarctation of the aorta.
• Cardiovascular complications are the leading cause of death in TS, highlighting the need for vigilance.

Purpose of the Study:

• To emphasize the significant burden of congenital heart and vascular lesions in TS.
• To highlight the importance of lifelong cardiovascular surveillance for individuals with TS.
• To discuss the rationale for clinical management of CHD in TS, considering TS-specific features.

Main Methods:

• Expert panel statement.
• Review of current clinical practice guidelines for TS.
• Focus on rationale for clinical management and follow-up care.

Main Results:

• Individuals with TS have a threefold increased standardized mortality rate compared to the general population.
• Cardiovascular complications, including aortic dissection, are the primary cause of death.
• TS presents unique CHD features that influence clinical decision-making.

Conclusions:

• The 2024 Clinical Practice Guidelines for TS underscore the necessity of addressing CHD.
• Lifelong cardiovascular surveillance is essential for managing TS-related heart conditions.
• Understanding TS-specific CHD features is critical for effective therapeutic options and follow-up.