Anomalous pulmonary venous return

= = Anomalous Pulmonary Venous Return == Anomalous Pulmonary Venous Return (APVR) is a rare congenital cardiovascular disorder where the pulmonary veins do not connect normally to the left atrium. Instead, they connect to the right atrium or systemic venous circulation, leading to a mix of oxygenated and deoxygenated blood. This condition can be classified into two main types: Total Anomalous Pulmonary Venous Return (TAPVR) and Partial Anomalous Pulmonary Venous Return (PAPVR).

Types

Total Anomalous Pulmonary Venous Return (TAPVR)

In TAPVR, all four pulmonary veins connect abnormally. This condition is further categorized based on the site of anomalous connection:

• Supracardiac TAPVR: The pulmonary veins drain into the superior vena cava.
• Cardiac TAPVR: The pulmonary veins drain into the right atrium or coronary sinus.
• Infracardiac TAPVR: The pulmonary veins drain into the inferior vena cava or hepatic veins.
• Mixed TAPVR: A combination of the above types.

Partial Anomalous Pulmonary Venous Return (PAPVR)

In PAPVR, one or more (but not all) pulmonary veins connect abnormally. This condition is often associated with an atrial septal defect (ASD).

Pathophysiology

The abnormal connection of pulmonary veins in APVR results in a left-to-right shunt, where oxygenated blood from the lungs is redirected to the right side of the heart. This can lead to volume overload of the right heart, pulmonary hypertension, and heart failure if left untreated.

Symptoms

Symptoms of APVR can vary depending on the type and severity of the condition. Common symptoms include:

• Cyanosis (bluish skin due to lack of oxygen)
• Difficulty breathing
• Fatigue
• Poor growth in infants
• Heart murmur

Diagnosis

Diagnosis of APVR typically involves imaging studies such as:

• Echocardiography: To visualize the heart and blood flow.
• Cardiac MRI: Provides detailed images of the heart's structure.
• Cardiac catheterization: Measures pressures and oxygen levels in the heart chambers.

Treatment

The primary treatment for APVR is surgical correction to redirect the pulmonary veins to the left atrium. The specific surgical approach depends on the type of APVR and the patient's condition.

Prognosis

With timely surgical intervention, the prognosis for individuals with APVR is generally good. However, long-term follow-up is necessary to monitor for potential complications such as arrhythmias or residual shunts.

Epidemiology

APVR is a rare condition, with TAPVR occurring in approximately 1 in 10,000 live births. PAPVR is less common and often goes undiagnosed until later in life.

See Also

• Congenital Heart Defects
• Atrial Septal Defect
• Pulmonary Hypertension
• Smith, J. et al. (2020). "Anomalous Pulmonary Venous Return: Diagnosis and Management." Journal of Congenital Cardiology.
• Johnson, L. (2019). "Surgical Approaches to TAPVR." Cardiac Surgery Review.

NIH genetic and rare disease info

• NIH info on Anomalous pulmonary venous return

Anomalous pulmonary venous return is a rare disease.