Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease in which all four pulmonary veins are mispositioned and do not connect normally to the left atrium. Instead the four pulmonary veins drain abnormally to the right atrium.
Patients with Total Anomalous Pulmonary Venous Connection (TAPVC) and obstructed pulmonary venous return are extremely ill soon after birth. These children are severely cyanotic.
If obstruction to pulmonary venous return is not present, children may be asymptomatic. There may be mild to moderate rapid or laboured breathing.
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Types of Total Anomalous Pulmonary Venous Connection (TAPVC)
Classified into different types, based on the location of the abnormal pulmonary vein return:
- Supracardiac (50%)- In supracardiac TAPVR, the pulmonary veins come together and form an abnormal connection above the heart to the superior vena cava,
- Coronary sinus- In cardiac TAPVR, the pulmonary veins meet behind the heart and connect to the right atrium.
- Infracardiac (20%)- In infracardiac TAPVR, the pulmonary veins come together and form abnormal connections below the heart
- Mixed (10%) variant.
TAPVC can occur with obstruction or without obstruction
Signs and Symptoms
Patients with obstructed TAPVR are extremely ill soon after birth. These children are severely cyanotic. In other children, symptoms may be delayed.
- Difficult and rapid breathing
- Right ventricular hypertrophy
Tests and diagnosis
- On Auscultation presence of typicalheart murmur
- Oxygen saturation monitor to see how much oxygen is getting into the blood
- Chest x-ray to see the size and position of the heart
- ECG (electrocardiogram) to check the electrical activity
- Echocardiogram – shows definitive diagnosis showing abnormal connection of the pulmonary veins.
- Cardiac catheterization will define the abnormal connection of all pulmonary veins, Cardiac catheterization can also determine accurately whether pulmonary veins are obstructed
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- Medical history noted and clinical examination of the child performed
- Advised medical tests and diagnostic procedures
- Counselled regarding the surgery its benefits and follow up care required.
- Occasionally if a restrictive atrial septal defect is present, a balloon dilation procedure may be performed at cardiac catheterization to improve the child's condition prior to surgical repair.
- This condition must be corrected with surgery.
- The timing of the surgical repair varies depending on the type of Total Anomalous Pulmonary Venous Connection (TAPVC) present, and the condition of the child.
- With obstruction in pulmonary veins, surgery should be undertaken immediately.
- In TAPVC without obstruction, surgery can be performed within the first month of life.
- The surgery is performed under general anaesthesia
- The four pulmonary veins are reconnected to the left atrium.
- Associated heart defects such as atrial septal defect, ventricular septal defect, patent foramen ovale etc are surgically closed.
- All other routes for pulmonary venous drainage, such as the abnormal vessels are tied off.
- The surgical repair, therefore, results in a normal circulation: the pulmonary veins returning normally to the left atrium
- Patient is hospitalized for almost a week
- The incision area may feel sore, painkillers are prescribed for the same
- Antibiotics are given to prevent infection-free healing of the chest incision.
- The first few days at home, should relax
- It takes about 6 weeks for a chest incision to heal and able to return to normal activities.
- Children with repaired TAPVC may be advised to limit their physical activities
- Paediatric cardiologist will help determine the proper level of activity.
- Rarely, obstruction of the pulmonary veins at the site of reconnection can occur.
- Medical Follow-up- cardiologist can monitor you with noninvasive tests if needed. These include electrocardiograms, Holter monitors, exercise stress tests and echocardiograms.
As with any surgery, there is the risk of complications:
- Infection, poor healing of incision
- Risk of injury to blood vessels and other tissues
- Fluid accumulation
- Scar tissue forming
- Rarely obstruction of pulmonary veins at site of reconnection
Prognosis of Repair
Improvement in surgical technique, early diagnosis and well post-operative patient care have led to improved results of TAPVC correction patient.
Factors Affecting the Cost of TAPVC surgery
Following are the factors which will affect the treatment cost:
- The hospital patient is opting for.
- Operating room, recovery room charges
- Fee for the team of doctors (Surgeons, Anaesthetist, etc. )
- Medicines and sterile dressings
- Standard test and diagnostic procedures
- Cost of Procedure
- Cost of follow up care