Pulmonary valve atresia
ICD-10 Q22.0 is a billable code used to indicate a diagnosis of pulmonary valve atresia.
Pulmonary valve atresia is a congenital heart defect characterized by the absence or severe narrowing of the pulmonary valve, which obstructs blood flow from the right ventricle to the pulmonary artery. This condition results in inadequate oxygenation of blood, leading to cyanosis and other complications. It is often associated with other congenital heart defects, such as ventricular septal defects (VSD) and right ventricular hypertrophy. Diagnosis typically occurs through echocardiography, which reveals the structural abnormalities. Management may involve surgical interventions, such as creating a shunt to improve blood flow or performing a valve replacement. Long-term follow-up is essential to monitor for complications, including arrhythmias and heart failure. Pulmonary valve atresia can occur as an isolated defect or as part of complex syndromes, necessitating a multidisciplinary approach to care, including cardiology, genetics, and pediatric specialties.
Complete clinical history, including prenatal and postnatal assessments, echocardiographic findings, and surgical interventions.
Management of neonates with cyanosis, follow-up care post-surgery, and assessment of growth and development.
Consideration of developmental milestones and potential comorbidities in pediatric patients.
Family history of congenital heart defects, genetic testing results, and syndromic associations.
Genetic counseling for families with a history of congenital heart disease and assessment for syndromic conditions.
Awareness of chromosomal abnormalities that may be associated with pulmonary valve atresia, such as 22q11.2 deletion syndrome.
Used during surgical intervention for pulmonary valve atresia.
Operative report detailing the procedure and any associated findings.
Pediatric cardiology should document the specifics of the surgical approach.
Common associated conditions include ventricular septal defects, right ventricular hypertrophy, and other congenital heart defects. Genetic syndromes such as 22q11.2 deletion syndrome may also be associated.
