A 2 week old is brought in severe distress and shock. You suspect congenital heart disease with a ductal-dependent lesion. Should you start prostaglandin E1?
The anatomy of congenital heart disease can be complex, but as one authority put it, “specific anatomical knowledge of the many complex lesions is not required for good early management.” (Brooks 2008)
The ductus arteriosus in the fetus bypasses the lungs, shunting blood from the pulmonary artery to the aorta. After birth, the pulmonary resistance drops and it closes by smooth muscle contraction. This happens within the first two weeks, and that is when these patients present.
There are two types of ductal-dependent lesions. Those that depend on the ductus for pulmonary flow and those that depend on the ductus for aortic flow. They present with cyanosis or shock, respectively. Prostaglandin E1 is indicated when the patient is unstable, and the examination suggests congenital heart disease.
Cyanosis: Apply the hyperoxia test. If the hypoxemia does not resolve with 100% oxygen, then it is assumed there is congenital heart disease.
Shock: Easily confused with sepsis and other systemic diseases. Specific signs of congenital heart disease should be sought, including pathological murmurs, abnormal pulses, rales, hepatomegaly, and cardiomegaly. If any of these are found, prostaglandin E1 is indicated.
-Cyanosis: Failing the hyperoxia test diagnoses congenital heart disease
-Shock: Asymmetric pulses, pathological murmurs, cardiomegaly, and signs of congestive heart failure diagnose congenital heart disease
-Prostaglandin E1 is indicated in the crashing neonate with any physical signs of congenital heart disease.