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| City | Cost (USD) | |
|---|---|---|
| Jeddah | $35,000 – $65,000 | Explore More |
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Total Anomalous Pulmonary Venous Connection (TAPVC) or Total Anomalous Pulmonary Venous Return (TAPVR) is a rare congenital disorder characterized by a malformation in all the four pulmonary veins that carry oxygenated blood from the lungs to the atrium. The malformation is such that the veins do not connect to the left atrium perfectly.
In case of TAPVC, the four pulmonary veins drain into the right atrium because of an anomalous connection. There are different TAPVC types, depending on where and how the pulmonary veins drain into the right atrium. The common TAPVC types include supracariac, cardiac, and infracardiac TAPVC.
All types of TAPVC are corrected with the help of a surgery, which is conducted during infancy itself. Additionally, all types of TAPVC almost always have an atrial septal defect (ASD), which requires a separated surgery for its closure. An ASD is a hole between the upper two chambers of the heart. As a result, some amount of oxygenated blood from the right atrium is transferred to the left atrium and out from the body.
TAPVC is detected as soon as the baby is born or even before. The following are some of the main TAPVC symptoms:
Treatment for total anomalous pulmonary venous connection (TAPVC) is given to treat the abnormal return of pulmonary veins to the right atrium or the systemic circulation, which can lead to insufficient oxygen levels in the body. The aim is to normally return blood from the lungs to the left atrium, improve oxygenation, and prevent heart failure or death.
You need to go for medical treatment as soon as possible if a newborn experiences increased breathing, cyanosis (bluish discolouration), poor feeding, lethargy, or failure to thrive. TAPVC is usually diagnosed within the first few days of life because the symptoms are generally severe.
Preparation involves diagnostic testing such as echocardiography, chest X-ray, cardiac MRI or CT, and cardiac catheterisation. Stabilisation with oxygen, mechanical ventilation, or medications to sustain heart function may be needed in infants before surgery.
Rerouting the pulmonary veins to the left atrium with open heart surgery is the only effective treatment.
The operation typically lasts 4–6 hours. Hospital stay can be 2–3 weeks, depending on the baby's status and recovery.
Surgical repair corrects normal circulation, enhances oxygenation, alleviates symptoms, and is potentially curative. The majority of children continue to have normal or near-normal lives.
Recovery entails close intensive care monitoring, ventilator assistance, and gradual return to normal feeding and activity. Long-term follow-up consists of echocardiograms to observe for complications like narrowing of veins or rhythm disturbances. Complete recovery may take weeks.
Success rates for surgery are high, particularly for non-obstructed TAPVC. Obstructed TAPVC is more severe but has increasingly better outcomes with early treatment and modern surgical methods.
After TAPVC repair, the 5-year survival rate was 92.6%, with no gradient across the anastomosis. Patients less than one year had a much lower survival rate (78.8%) than those older than one year.
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