CHDFNigeria
Total Anomalous Pulmonary Venous Return (TAPVR) is a cyanotic congenital heart defect in which all the pulmonary veins fail to connect to the left atrium as they normally do. Instead, they drain into the right atrium leading to a mixing of oxygenated and deoxygenated blood. TAPVR is a type of Anomalous Pulmonary Venous Return (APVR), with its mild form been Partial Anomalous Pulmonary Venous Return. TAPVC implies that all the pulmonary veins connect to the systemic veins or right atrium, either by a common vertical vein or individually.
Anatomically TAPVC can be divided into four subtypes based on the level of the anomalous connection as supracardiac, cardiac, infracardiac, and mixed.
- Supracardiac: Pulmonary veins anomalously connect, most commonly to the left innominate vein. Other locations for connections include SVC and azygos vein.
- Cardiac: Pulmonary veins drain into the coronary sinus or right atrium directly.
- Infracardiac: Pulmonary veins form a vertical vein and drain into the portal vein, hepatic vein, or IVC below the diaphragm
- Mixed: In mixed-type, there is a combination of connections at different levels.
The supracardiac variety is the most common of the TAPVC.
In Partial anomalous pulmonary venous connections (PAPVC), one or more but not all pulmonary veins abnormally drain into the right atrium either directly to the right atrium or through draining into systemic veins.
Signs and symptoms
Symptoms of TAPVR often appear immediately after birth, though in some cases they may develop later.
- Difficulty in breathing or rapid breathing
- Poor feeding and weight gain
- Cyanosis (bluish skin color)
- Extreme sweat especially during feeds
- Low oxygen saturations
- Lethargy (Unusual tiredness)
- Heart murmur
Serious congenital heart defects like TAPVR are usually identified before or shortly after birth. See our previous webinars to learn about pre-natal diagnosis of CHDs.
Diagnosis
Diagnosis of TAPVR can be suspected or made before child is born (prenatally) via foetal echocardiography. After childbirth, the following diagnostic tools are useful to establish diagnosis.
- Chest X-rays
- Echocardiogram.
- Electrocardiogram
- Pulse oximetry
- Cardiac MRI or CT scan
- Cardiac catheterization
Management of TAPVR
Stabilization: The first step to management is to stabilize the child, while planning for the surgical correction through OHS. Oxygen is given to help the baby breathe better. The child may be intubated and ventilated if they suffer from breathing difficulty. Prostaglandin E1, a medicine to keep a small blood vessel (ductus arteriosus) open so some oxygenated blood can still circulate especially important in the blocked (obstructed) type of TAPVR. If necessary, Inotropes may be prescribed to help the heart pump better.
Surgical correction
Open heart surgery is necessary as soon as possible to connect the lung veins to the correct place, the left atrium and close any holes in the heart. It is usually urgent, especially if blood flow is blocked and there is no inter-atrial communication.
What to expect
If the baby is diagnosed and treated early, under the best cardiac specialist care, the chances of recovery are good. However, for the Nigeria healthcare systems, kindly visit Nigerian open-heart registry at https://www.nigeriaheartregistry.com/paediatric-case-distribution for information on where to access surgical treatments.
