PULMONARY ATRESIA

ALTERNATE NAMES

Pulmonary valve atresia with intact ventricular septum; PA-IVS; Pulmonary valve atresia with ventricular septal defect; PA-VSD

DESCRIPTION

Pulmonary Atresia is a rare congenital heart disease in which the pulmonary valve does not form properly. The pulmonary valve is an opening on the right side of the heart that regulates blood flow from the right ventricle (right side pumping chamber) to the lungs. Because of this defect, blood from the right side of the heart cannot go to the lungs to pick up oxygen.

Pulmonary atresia may occur with or without a hole in the wall (septum) that separates the right ventricle and the left ventricle. The hole(s) in this wall is called a VSD (ventricular septal defect). If the infant does not have a VSD, the condition is called pulmonary atresia with intact ventricular septum (PA-IVS).

Infants born with this condition also tend to have multiple other cardiac conditions, such as poorly developed tricuspid valves, underdeveloped (hypoplastic) right ventricle, and abnormal blood vessels leading into the heart. Surgery is necessary shortly after birth to form a shunt from the systemic circulation to the pulmonary circulation in order to oxygenate the blood.

Infants surviving into childhood and adults may develop problems with their heart functioning later in life due to congestive heart failure, angina, arrhythmias, cyanosis, and sudden death.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING

The physical findings include infants with bluish colored skin (cyanosis), fast breathing, fatigue, poor feeding during nursing, sweating during feeding, and shortness of breath.

Imaging studies, echocardiogram, electrocardiogram, heart catheterization, and pulse oximetry are used to diagnose this condition.

ICD-9: 746.01

ONSET AND PROGRESSION

A diagnosis of pulmonary atresia is usually made shortly after birth. Disease progression is variable based on the severity of the congenital heart defect and the response to medication and surgical interventions.

TREATMENT

There are varying responses to treatment based on the severity of the defect. Infants are given medication (i.e. Prostaglandin) to help keep the blood vessel open between the pulmonary artery and the aorta (patent ductus arteriosis or PDA) until the initial shunt surgery can occur. Later, multi-staged surgeries to repair the vessel, open-heart surgery to repair or replace a pulmonary valve, reconstruction of the heart chambers, and heart transplant have been used to treat patients with this defect. Infants surviving into adulthood should be monitored by a cardiologist specializing in the care of adults with congenital heart disease to assess medication needs, surgery, and infection throughout their lifetime.

Suggested MER for Evaluation:

Clinical description of findings, operative reports, cardiology consultation reports, imaging studies, and electrocardiograms

Suggested Listings for Evaluation:

DETERMINATION

 LISTING 

REMARKS

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104.06

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