Total anomalous pulmonary venous drainage (TAPVD) is a congenital heart defect characterized by the absence of a normal connection between the pulmonary veins and the left atrium. Instead, the pulmonary veins drain either directly into the right atrium or into venous structures that subsequently empty into it, such as the coronary sinus, the superior or inferior vena cava and their tributaries, or, in rare cases, the portal vein. Communication between the systemic (large) and pulmonary (small) circulations usually occurs at the atrial level.
What happens in total anomalous pulmonary venous drainage?
Under normal cardiac physiology, oxygenated blood from the lungs is transported through the pulmonary veins into the left atrium. From the left atrium, blood flows into the left ventricle and then into the aorta, from where it is distributed via arteries to all organs and tissues of the body.
In total anomalous pulmonary venous drainage, this normal pathway is disrupted. Oxygen-rich blood does not enter the left atrium, leading to systemic hypoxia. Due to excessive volume and pressure load, the right-sided chambers of the heart become enlarged. As a result, progressive heart failure and pulmonary hypertension develop.
TAPVD is classified into total and partial forms. In the total form, none of the four pulmonary veins connect to the left atrium. In the partial form, one or more pulmonary veins retain a normal connection to the left atrium.
Clinical manifestations
Infants with total anomalous pulmonary venous drainage are usually born in a critically severe condition. In most cases, the first sign of the defect is marked tachypnea (rapid breathing). This is followed by symptoms of heart failure, including edema, dyspnea, hepatomegaly, and cough, as well as cyanosis of the skin and mucous membranes.
These children gain weight poorly, have feeding difficulties, and lag behind in physical development. If the child survives the early period, recurrent pneumonias and frequent respiratory infections are commonly observed later in life.
Diagnosis of TAPVD
The diagnosis of total anomalous pulmonary venous drainage is based on:
- assessment of external clinical signs, including the presence of cyanosis;
- findings on echocardiography;
- results of chest radiography;
- cardiac catheterization with angiocardiography.
The child is placed in special conditions aimed at reducing oxygen demand, including maintaining a comfortable ambient temperature and ensuring physical and emotional rest. If necessary, medical treatment for heart failure is initiated.
In cases of severe cyanosis and critical clinical condition, a palliative procedure (the Rashkind procedure) may be performed at the initial stage. This intervention enlarges the interatrial communication, thereby improving oxygen saturation of the blood.
Definitive surgical correction of total anomalous pulmonary venous drainage must be performed within the first months, and in some cases even the first days, of life. The essence of the operation is the creation of an anastomosis between the left atrium and the pulmonary veins (or a common venous collector), along with reconstruction of the atrial septum. These procedures are carried out using cardiopulmonary bypass and cold cardioplegia.
Why choose Belarus?
Belarus has accumulated extensive experience in the treatment of children with total anomalous pulmonary venous drainage. Despite the severity of this defect and the complexity of the required surgical intervention, Belarusian specialists perform the full range of necessary diagnostic and therapeutic measures at a very high professional level. This allows young patients to recover, lead normal lives, and develop on par with their peers.
