Total Anomalous Pulmonary Venous Connection (TAPVC)

Total Anomalous Pulmonary Venous Connection (TAPVC) is a rare congenital heart defect where the pulmonary veins, which carry oxygen-rich blood from the lungs to the heart, are not connected normally to the left atrium. Instead, they connect to the right atrium or to one of its tributaries, such as the superior vena cava, inferior vena cava, or coronary sinus.

Types of TAPVC

TAPVC can be classified into three main types:

• Supracardiac type: The pulmonary veins connect to a common chamber behind the heart, which then drains into the superior vena cava or innominate vein.
• Cardiac type: The pulmonary veins connect directly to the coronary sinus or the right atrium.
• Infra-cardiac type: The pulmonary veins connect to a common chamber below the diaphragm, which then drains into the portal vein, hepatic veins, or inferior vena cava.
• Mixed type: A combination of the above types, where some pulmonary veins connect to one site and others to another.

Symptoms and Diagnosis

Infants with TAPVC often present with symptoms such as:

• Cyanosis (blue discoloration of the skin)
• Shortness of breath
• Rapid breathing
• Poor feeding
• Fatigue
• Sweating

Diagnosis is typically made using a combination of:

• Chest X-ray
• Echocardiogram (ultrasound of the heart)
• Cardiac catheterization
• CT or MRI scans

Treatment and Prognosis

Surgical repair is usually necessary to correct TAPVC. The goal of surgery is to redirect the pulmonary veins to connect normally to the left atrium.

The prognosis for infants with TAPVC has improved significantly with advances in surgical techniques and post-operative care. However, the outcome depends on various factors, including:

• Age at diagnosis and treatment
• Severity of the defect
• Presence of associated heart defects or other medical conditions
• Overall health of the child

With successful surgical repair, most children with TAPVC can lead active and normal lives. However, they may require ongoing cardiac care and monitoring to prevent potential complications.

Frequently Asked Questions (FAQs)

What is Total Anomalous Pulmonary Venous Connection (TAPVC)?
A rare congenital heart defect where pulmonary veins connect to right atrium or its tributaries instead of left atrium.

What are the symptoms of TAPVC?
Cyanosis, shortness of breath, poor feeding, and failure to thrive in infants.

How is TAPVC diagnosed?
Echocardiogram, cardiac catheterization, and MRI or CT scans.

What are the types of TAPVC?
Supracardiac, cardiac, infracardiac, and mixed types.

What is the treatment for TAPVC?
Surgical correction to redirect pulmonary veins to left atrium.

When should surgery be performed?
As soon as possible after diagnosis, usually in infancy.

What are the risks of untreated TAPVC?
Increased risk of heart failure, pulmonary hypertension, and death.

Can TAPVC be detected prenatally?
Yes, through fetal ultrasound and echocardiogram.

Is TAPVC associated with other congenital anomalies?
Yes, often associated with other cardiac and non-cardiac defects.

What is the prognosis after surgical correction of TAPVC?
Generally good, with most patients having normal heart function and lifespan.