At Shaare Zedek, there is a unit dedicated to treating adults with congenital heart defects. The unit is led by Professor Amiram Nir, the head of the Department of Adult Congenital Heart Disease at the Israeli Cardiology Association. Shaare Zedek Medical Center has the resources to care for patients requiring a wide range of specialized expertise in cardiology, imaging, catheterization, and more. Collaboration between cardiologists, surgeons, and pediatricians ensures the best possible care for pregnant women with heart disease.
Congenital Heart Defects in Adults
Congenital heart defects are not uncommon and occur in slightly less than one percent of the population. Some infants and children born with heart defects require surgery or catheterization to correct the problem. Thanks to successful treatments in infants and children born with congenital heart defects over the last few decades, most of them survive and reach adulthood. As a result, the number of adults born with heart defects is increasing. According to research conducted at the Gertner Institute, there are approximately 20,000 adults in Israel with congenital heart defects, which equates to about 4 patients per 1,000 adults. As this population ages, they may develop cardiovascular problems associated with aging.
What Are Congenital Heart Defects?
A congenital heart defect is a structural abnormality present in the heart from birth. The heart is a muscular pump that circulates blood. It is divided into two sides or chambers: the right side pumps blood to the lungs, and the left side receives oxygen-rich blood from the lungs and pumps it to the body. Common and simple congenital heart defects involve defects or "holes" in the heart walls. In such cases, there is mixing of blood between the two sides. Infants with significant defects may suffer from heart failure, and in such cases, the defect needs to be closed either with a catheter or through surgery. Small defects may not affect health and do not require treatment. More complex congenital heart defects, while rarer, can cause severe health problems and even be life-threatening. There are obstructive defects, where there is a blockage or narrowing of blood flow. There are also defects that affect the heart's function. In some cases, the heart chambers are not formed correctly, and blood flow is "reversed." In very rare instances, only one of the heart's chambers develops, and there is only one functioning heart system instead of two. In most cases, these defects can be corrected through surgery at a young age.
Despite significant improvements in surgical techniques to correct congenital heart defects, many surgeries do not lead to complete recovery, and adults with these conditions often experience late complications. Common problems in adults born with congenital heart defects include heart rhythm disturbances with a risk of sudden death, heart failure, endocarditis (heart valve infection), residual defects, pulmonary hypertension, and damaged blood vessels resulting from previous surgeries or catheterizations. A small subset of patients may experience cyanosis, a condition where the oxygen level in the blood is reduced due to mixing.
Pregnancy and childbirth present significant risks for women with certain congenital heart defects. Approximately 11% of women with congenital heart disease experience complications during pregnancy, primarily involving rhythm disturbances and heart failure. The highest risk is observed in women with pulmonary hypertension and women with complex heart defects. Heart failure most commonly occurs in the second half of pregnancy, during labor, and in the weeks following childbirth. Israel has the highest birthrate among OECD countries. Many women with congenital heart disease have a high number of children and, as a result, are exposed to more complications during pregnancy and childbirth. Women with heart defects should undergo a thorough evaluation before becoming pregnant, and their pregnancies should be carefully monitored. Women with congenital heart disease have a higher likelihood of giving birth to children with heart defects, so it is recommended that they undergo fetal echocardiography during pregnancy. Shaare Zedek has a team of specialists who provide care for these women.
According to recommendations from European and American professional organizations, there is a need for specialized centers or units to follow and treat adults with complex congenital heart defects. These centers should include experts in congenital heart disease, including cardiologists, cardiac surgeons, catheterization specialists, electrophysiologists, and nurses. One challenge in treating adults with congenital heart defects is the level of awareness among healthcare professionals who do not specialize in this field. At Shaare Zedek, there is a system in place to address the needs of adult patients with congenital heart defects, especially those who have undergone closure procedures in the past. Patients who have undergone closure procedures for simple defects such as ASD, VSD, or PDA may only require a one-time evaluation. However, patients with more complex defects, including transposition of the great arteries, tetralogy of Fallot, coarctation of the aorta, or atrioventricular canal defects, as well as those with unrepaired or residual defects, should be followed up in a specialized adult congenital heart disease unit. These patients are at risk for life-threatening arrhythmias, heart failure, valvular problems, pulmonary hypertension, thromboembolic events, and other issues.