Some congenital heart defects cause cyanosis, or low oxygen levels in the blood, which can give children a bluish appearance. In many cases, the cyanotic heart defect is repaired in childhood, and oxygen levels return to normal. Sometimes, a complete repair isn’t possible and the cyanosis is present for life.
Cyanotic Heart Disease is a heart defect, or group of heart defects that are present at birth. Under normal circumstances, an infant’s blood contains ample oxygen that flows throughout the body. When cyanosis is present, blood flows abnormally (called right-to-left shunt), resulting in too little oxygen in the blood flow and causing the child’s skin to take on a bluish appearance. This bluish discoloration is most often seen on the fingers, lips, and toes. Several types of congenital heart disease may cause cyanosis, including:
- Pulmonary Valve Atresia
- Tricuspid Atresia
- Hypoplatic Left Heart Syndrome
- Truncus Arteriosus
- Total Anomalous Pulmonary Venous Return
Treatment and management of Cyanotic Heart Disease depends largely on the specific type of congenital heart defect that is present. After birth, infants may receive medication to assist the heart in pumping, keep the blood vessels open, and treat any heartbeat abnormalities. Typically, one or more surgeries are performed during childhood to redirect the blood flow and alleviate the cyanosis. If these surgeries are successful, the child is no longer cyanotic.
However, some older adults were born in a time period when these cyanotic heart defects could not be repaired. They may have had a shunt placed—communication between the aorta or its branches and the pulmonary artery to increase blood flow to the lungs—or they may have had no surgery at all. Some older adults were born with a hole in their heart (this is known as a ventricular septal defect) but were born before surgery was available to close the hole. Generally, if the hole was large enough, they didn’t have cyanosis as a young child, but would go on to develop it later in their childhood as the pulmonary pressures increased, causing a reversal of the shunt. This is known as Eisenmenger syndrome. Adults with Eisenmenger syndrome may benefit from medical therapy with pulmonary vasodilators, and should discuss these options with an adult congenital heart specialist.
Adults who have cyanotic heart disease require special care. Phlebotomy, or removal of blood should be avoided in general, as this leads to iron deficiency and worsening symptoms. High blood counts are the body’s way of compensating for the low oxygen levels.
Individuals with cyanotic heart must exercise caution when traveling to high altitudes, and most should avoid pregnancy. If a patient is hospitalized, an air filter should be placed in the IV lines to prevent small bubbles from reaching the brain—otherwise, the mixing of venous blood and arterial blood can occur.
All patients born with cyanotic heart disease have moderate to complex heart defects. These patients benefit from a multi-disciplinary approach at a Center specializing in the care of adult with congenital heart defects
Regardless of the chosen course of treatment, we believe that when managing this disease, it’s important to adopt a team approach in which primary care physicians, surgeons, cardiologists, nurses, OB/GYNs, hematologists, and anesthesiologists all communicate and work in conjunction to achieve the best results for the patient.
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