Nursing Care for Pediatric Patients With Congenital Heart Defects

Congenital heart defects are cardiac anomalies present at birth that affect normal blood flow within the heart. They’re divided into two categories, acyanotic and cyanotic, based on the presence of cyanosis, or a bluish discoloration of the skin. Acyanotic heart defects don’t cause cyanosis, whereas cyanotic heart defects do. As the nurse, you’ll collaborate with the registered nurse, or RN, to provide care for children with congenital heart defects.

Congenital heart defects are associated with risk factors that interfere with fetal cardiovascular development, causing cardiac structures to form improperly. Risk factors include fetal chromosomal abnormalities as well as exposure to infections like rubella, or teratogens, which are substances that cause developmental abnormalities, such as certain medications, alcohol, recreational drugs, tobacco smoke, and heavy metals like mercury.

Now, with acyanotic heart defects, there’s an abnormal communication between the left- and right-sided circulation of the heart. Examples of acyanotic heart defects include ventricular septal defect, where there’s an opening in the interventricular septum that separates the left and right ventricles; atrial septal defect, where there’s an opening in the interatrial septum between the left and right atria; and patent ductus arteriosus, which is caused by continued circulation through the fetal shunt, which is a blood vessel that normally closes after birth.

For example, with a ventricular septal defect, blood takes the path of least resistance going from the left ventricle, where the pressure is higher, through the defect to the right ventricle, where the pressure is lower, and then back into the pulmonary circulation. This is called left-to-right shunting. Now, this blood is already oxygenated so there's no cyanosis, but the recirculation of blood increases pulmonary flow and leads to pulmonary hypertension, which may eventually progress to heart failure.

Children with acyanotic heart defects can be asymptomatic, but as pulmonary hypertension develops, they can develop breathing difficulties which can result in problems with eating, poor weight gain or failure to thrive, as well as activity intolerance. They may also be more prone to respiratory infections. Upon auscultation, wheezing can often be heard over the lung fields, and a systolic murmur can be auscultated as blood moves through the defect.

On the other hand, with cyanotic heart defects, deoxygenated blood bypasses the lungs, flows into the aorta, and circulates systemically, leading to tissue hypoxia and cyanosis. An example of a cyanotic heart defect is tetralogy of Fallot, which has a combination of four heart abnormalities: pulmonary stenosis, which is narrowing of the pulmonary valve; right ventricular hypertrophy, which is enlargement of the right ventricle; ventricular septal defect; and overriding aorta, were the aorta is shifted and sits above the ventricular septal defect.