Hypoplastic right heart syndrome
ICD-10 Q22.6 is a billable code used to indicate a diagnosis of hypoplastic right heart syndrome.
Hypoplastic right heart syndrome (HRHS) is a complex congenital heart defect characterized by underdevelopment of the right side of the heart, including the right ventricle, right atrium, and associated structures such as the pulmonary artery and tricuspid valve. This condition leads to inadequate blood flow to the lungs, resulting in cyanosis and heart failure. Infants with HRHS often present with symptoms such as difficulty breathing, poor feeding, and lethargy shortly after birth. The severity of the condition can vary, but it typically requires surgical intervention, often in multiple stages, to improve blood flow and heart function. The etiology of HRHS is not fully understood, but it may involve genetic factors and environmental influences during fetal development. Diagnosis is usually made through echocardiography, which provides detailed images of the heart's structure and function. Management of HRHS involves a multidisciplinary approach, including pediatric cardiology, cardiothoracic surgery, and ongoing follow-up care to monitor for complications and assess the need for further interventions.
Pediatric documentation should include detailed clinical notes on the infant's symptoms, diagnostic imaging results, and any surgical interventions performed. Growth and developmental assessments are also crucial.
Common scenarios include newborns presenting with cyanosis, failure to thrive, and respiratory distress, necessitating immediate evaluation by a pediatric cardiologist.
Coders must be aware of the developmental milestones and potential long-term complications associated with HRHS, which may affect future coding.
Genetic documentation should include family history, genetic testing results, and any syndromic associations with HRHS. This information is vital for understanding potential hereditary patterns.
Scenarios may involve genetic counseling for families with a history of congenital heart defects or syndromes associated with HRHS.
Coders should consider the implications of genetic findings on the management and prognosis of HRHS, as well as the need for potential genetic testing.
Used during surgical intervention for HRHS to improve blood flow to the lungs.
Detailed operative notes describing the procedure and indications.
Pediatric cardiothoracic surgeons must provide comprehensive documentation to support the procedure.
Common surgical interventions for HRHS include the Norwood procedure, which is performed in the neonatal period to establish systemic-to-pulmonary blood flow, followed by the Glenn procedure and Fontan procedure in later stages to optimize blood flow and heart function.
