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Physical Health

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Heart

Heart and Blood Vessels

Cardiovascular abnormalities are one of the most common complications in girls and women with Turner Syndrome (TS)

As the mother of a daughter with TS heart related conditions, I know how important it is to get your first cardiac screening. Cardiac complications are the main cause for early death in those with TS, so please lower your heart risk by seeing a cardiologist. Cindy Scurlock, TSSUS President and CEO.

It is important to know that people with TS may be more likely to develop the following heart conditions.

It is important to know that people with TS may be more likely to develop the following heart conditions.

Common congenital heart abnormalities in TS:

  • Up to 30% are born with a heart valve that has 2 cusps instead of 3 cusps, called a bicuspid aortic valve.

  • About 1 in 3 have a bulge in the beginning part of the aorta, the largest blood vessel in the body, right after it leaves the heart, called a thoracic aortic aneurysm (TAA).

  •  Up to 20% are born with a narrow area within the aorta, called a coarctation of the aorta.

  • 10-20% have abnormal blood flow from the lungs to the heart, called partial anomalous pulmonary venous connection (PAPVC)

  • Some have an extra main vein traveling to the left side of the heart, called left superior vena cava (LSVC).

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Less frequent congenital heart abnormalities in TS:

  • The left side of the heart may be underdeveloped, affecting blood flow through the mitral valve, aortic valve, and main pumping chamber, or left ventricle, called hypoplastic left heart syndrome (HLHS).

  • The mitral valve, which opens to let blood from the top part of the heart flow into the left ventricle, may have floppy or thickened leaflets, the wrong number of leaflets, or other problems.

  • The coronary arteries may come off a different part of the heart than is typical, called coronary anomalies.

  • The heart may be on the right side instead of the left side of the chest, called dextrocardia.

  •  There may be a hole in the heart between heart chambers, called a ventricular septal defect (VSD) or an atrial septal defect (ASD).

  • blood pressure- associated disorders of pregnancy, including pre-eclampsia.

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High blood pressure

High blood pressure, or hypertension, occurs in 20-40% of children with TS and up to 60% of adults with TS. It means the blood going through your arteries is pushing on the artery walls too hard. The top number of your blood pressure reading is called the systolic blood pressure. If your systolic blood pressure is more than 130 mmHg, it may be too high. It is important to get your blood pressure checked regularly. Living with high blood pressure for too long can cause damage to your eyes, heart, and kidneys. Many medications are available to treat high blood pressure.

​Stroke

Some people with TS may be at risk for stroke. The main risk factors are high blood pressure and high cholesterol. These can both be treated effectively with medications and regular checkups.

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Tearing of the aorta or aortic dissection

Aortic dissections occur in less than 1% of people with TS (about 40 people out of 100,000). People with TS who also have a thoracic aortic aneurysm, bicuspid aortic valve, coarctation of the aorta and/or high blood pressure may be at higher risk to have an aortic dissection. While a dissection is life threatening, medications, regular checkups, and in some cases, surgical replacement of the aorta, can prevent dissections. 

 

Heart attack (myocardial infarction, ischemic coronary heart disease)

Many people with TS are at increased risk for heart attacks. The main cause of heart attacks is hardening and blockage of the heart arteries due to cholesterol deposits, called atherosclerosis. To prevent heart attacks, treat the causes by checking and controlling high cholesterol, high blood sugar (diabetes), and high blood pressure, the three main causes of atherosclerosis. If you smoke or vape, please try your best to quit. Smoking makes heart attacks more likely to occur.

 

Heart risks during pregnancy

If you are considering becoming pregnant, please get a thorough cardiac evaluation and include the cardiologist as a member of your pregnancy health team. There is an increased risk for tearing of the aorta and high blood pressure- associated disorders of pregnancy, including pre-eclampsia.

Growth in Turner Syndrom Turner Topic TSSUS

Turner Topic on the Heart and Turner Syndrome 

 

For convenience, the Turner Topic offers a one-sheet overview of the Heart and TS based on the most requested information, including:  

 

  • Bicuspid aortic valve

  • Hypoplastic left heart syndrome

  • Coarctation of the aorta

  • Ascending aorta dilatation and dissection

  • Hypertension

  • Atherosclerosis

  • Pregnancy and TS

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Reviewed by Siddharth Prakash, MD, Adult Cardiologist, Chairman of TSSUS Scientific Advisory Board. Dr. Prakash obtained his Ph.D. in Molecular and Human Genetics and subspecialty training in Cardiovascular Disease at Baylor College of Medicine. His research focuses on genetic causes of bicuspid aortic valves and related congenital abnormalities involving the left ventricular outflow tract and aorta. This work is supported in part by grants from the National Institutes of Health. His clinical specialty is cardiovascular medicine and aortic imaging. He co-directs the Turner Syndrome Adult Comprehensive Care Center and the Multidisciplinary Aortic and Vascular Disease Clinic in the UT Professional Building.

Reviewed by Aaron T. Dorfman, MD, is the Medical Director of the South New Jersey Section of CHOP Cardiology, and Director of New Jersey Cardiology Satellite Operations. He is an attending cardiologist in the Cardiac Center at Children’s Hospital of Philadelphia.

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