2025 ICD-10-CM code Q22.0
Pulmonary valve atresia.
Medical necessity for treatment of pulmonary valve atresia is established by the life-threatening nature of the condition. Without intervention, the lack of oxygenated blood circulating to the body will lead to severe complications and death.Treatment aims to restore blood flow to the lungs and improve oxygenation.
Diagnosis and management of pulmonary valve atresia typically falls under the purview of pediatric cardiologists and cardiothoracic surgeons.They are responsible for confirming the diagnosis, determining the best course of treatment (which may involve surgery or other interventions), and providing long-term follow-up care.Other specialists may be involved depending on the presence of other associated conditions.
- Congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
- Congenital malformations of the circulatory system (Q20-Q28)
In simple words: Pulmonary valve atresia is a birth defect where the heart's pulmonary valve, which controls blood flow to the lungs, doesn't form. This prevents blood from getting oxygen.Babies with this condition often appear blueish shortly after birth and require urgent treatment.
Pulmonary valve atresia is a congenital heart defect where the pulmonary valve does not form at all, completely blocking blood flow from the right ventricle to the pulmonary artery. This blockage prevents blood from reaching the lungs to receive oxygen.The right ventricle and tricuspid valve may be underdeveloped, and a ventricular septal defect may be present.Blood finds alternate routes to the lungs, often through a patent foramen ovale or ductus arteriosus.Infants with this condition typically present with cyanosis shortly after birth as the ductus arteriosus closes.
Example 1: A newborn infant presents with cyanosis shortly after birth.Echocardiography reveals the absence of a pulmonary valve and a diagnosis of pulmonary valve atresia is made., A fetus is diagnosed with pulmonary valve atresia during a prenatal ultrasound.The infant is delivered in a hospital equipped to handle the condition and immediate treatment is initiated after birth., A child with known pulmonary valve atresia presents for a follow-up appointment with their cardiologist to monitor for any complications or changes in their condition.
Documentation for pulmonary valve atresia should include imaging studies (such as echocardiography) confirming the diagnosis, details about the anatomy of the heart defect (including presence or absence of a VSD and the size of the right ventricle), and clinical findings such as cyanosis and oxygen saturation levels.
- Specialties:Pediatric cardiology, cardiothoracic surgery
- Place of Service:Inpatient Hospital, Outpatient Hospital