Anomalous pulmonary venous connection

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD's weight loss doctor NYC
Philadelphia GLP-1 weight loss and GLP-1 clinic NYC

Anomalous pulmonary venous connection
Synonyms	Total anomalous pulmonary venous return (TAPVR), Partial anomalous pulmonary venous return (PAPVR)
Pronounce	N/A
Specialty	N/A
Symptoms	Cyanosis, dyspnea, tachypnea, heart murmur
Complications	Heart failure, pulmonary hypertension
Onset	Neonatal
Duration	Lifelong if untreated
Types	N/A
Causes	Congenital defect
Risks	Genetic factors, maternal diabetes
Diagnosis	Echocardiography, chest X-ray, cardiac MRI
Differential diagnosis	Atrial septal defect, ventricular septal defect, patent ductus arteriosus
Prevention	None
Treatment	Surgical repair
Medication	N/A
Prognosis	Good with treatment
Frequency	1 in 10,000 live births
Deaths	N/A

Anomalous pulmonary venous connection (APVC) is a rare congenital heart defect where the pulmonary veins do not connect normally to the left atrium. Instead, they connect to other parts of the heart or systemic veins, leading to improper oxygenation of blood.

Types

APVC can be classified into two main types:

• Total anomalous pulmonary venous connection (TAPVC): All four pulmonary veins connect abnormally.
• Partial anomalous pulmonary venous connection (PAPVC): One or more, but not all, pulmonary veins connect abnormally.

Pathophysiology

In a normal heart, the pulmonary veins carry oxygenated blood from the lungs to the left atrium. In APVC, the pulmonary veins connect to the right atrium or to veins that drain into the right atrium, such as the superior vena cava or inferior vena cava. This results in a mixing of oxygenated and deoxygenated blood, leading to reduced oxygen levels in the systemic circulation.

Symptoms

Symptoms of APVC can vary depending on the severity and type of the defect. Common symptoms include:

• Cyanosis (bluish tint to the skin)
• Shortness of breath
• Fatigue
• Heart murmur
• Failure to thrive in infants

Diagnosis

APVC is typically diagnosed using imaging techniques such as:

• Echocardiography
• Cardiac MRI
• Cardiac catheterization

Treatment

The primary treatment for APVC is surgical correction. The specific surgical approach depends on the type and severity of the defect. Common procedures include:

• Re-routing the pulmonary veins to the left atrium
• Closing any associated atrial septal defects

Prognosis

With timely surgical intervention, the prognosis for individuals with APVC is generally good. However, long-term follow-up is necessary to monitor for potential complications such as:

• Pulmonary hypertension
• Arrhythmias
• Residual or recurrent defects

Related Pages

• Congenital heart defect
• Atrial septal defect
• Ventricular septal defect
• Pulmonary hypertension