Cyanosis due to Pulmonary Hypertension with Congenital Heart Disease
When there is a communication between the right and left heart (see ASD, VSD and atrioventricular septal defect) or circulations (see PDA), blood goes from the low oxygen chamber or vessel to the high oxygen chamber or vessel. An example would be shunting from the right atrium to the left atrium in patients with atrial septal defect (ASD) and pulmonary arterial hypertension. The patient who was once acyanotic becomes cyanotic. One way the body compensates is to increase the ability of the blood to carry oxygen by increasing the number of red blood cells, a condition called secondary erythrocytosis, sometimes referred to as polycythemia. It is now recognized that removing blood with a procedure called phlebotomy is only rarely needed. Symptoms that may prompt a doctor to recommend it are headaches, excessive fatigue and worsening exercise tolerance. Before this is performed, it is important that the doctors tests for iron deficiency. Phlebotomy is preferably performed in a center with experience in adult congenital disease as special precautions are required.