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[Cardiovascular malformations in Turner syndrome].
Maria Korpal-Szczyrska1, Janina Aleszewicz-Baranowska, Bohdana Dorant
1Klinika Pediatrii, Hematologii, Onkologii i Endokrynologii AM w Gdańsku.
Endokrynologia, Diabetologia I Choroby Przemiany Materii Wieku Rozwojowego : Organ Polskiego Towarzystwa Endokrynologow Dzieciecych
|October 20, 2005
Summary
Cardiovascular malformations are common in Turner syndrome, affecting 32.7% of patients. Early echocardiography and regular blood pressure monitoring are crucial for managing these cardiac conditions in girls with Turner syndrome.
Area of Science:
- Pediatric Cardiology
- Genetics
- Medical Imaging
Background:
- Turner syndrome is associated with a significant prevalence of cardiovascular malformations, ranging from 17% to 47% in reported literature.
- This study focuses on evaluating the cardiovascular health of pediatric patients diagnosed with Turner syndrome.
Purpose of the Study:
- To assess the spectrum and prevalence of cardiovascular abnormalities in a cohort of female patients with Turner syndrome.
- To emphasize the importance of cardiac monitoring in this population.
Main Methods:
- A cohort of 55 females (aged 3-20 years) with Turner syndrome underwent clinical evaluation, including interviews, physical examinations, chest X-rays, and comprehensive echocardiography.
- Advanced echocardiographic techniques such as M-mode, 2D, Doppler, and color flow mapping were utilized.
- Cardiac catheterization, cineangiography, and 24-hour blood pressure monitoring were performed in select cases.
Main Results:
- Cardiovascular malformations were identified in 18 out of 55 patients (32.7%).
- The most frequent malformations included coarctation of the aorta (50% of affected patients), bicuspid aortic valve (33%), and aortic insufficiency (22%).
- Monosomy (45,X) was prevalent in 78% of Turner subjects with cardiovascular issues, particularly those with coarctation of the aorta.
Conclusions:
- The high incidence of cardiovascular malformations necessitates regular monitoring by pediatric cardiologists for all girls with Turner syndrome.
- Echocardiography should be a standard diagnostic tool for every female diagnosed with Turner syndrome.
- Close monitoring of blood pressure is essential in managing patients with Turner syndrome due to the risk of hypertension.