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.