Ectopic pulmonary veins: What you need to know

The article was professionally consulted with Master, Doctor Cao Thanh Tam - Cardiologist - Cardiovascular Center - Vinmec Central Park International General Hospital.

Ectopic pulmonary venous drainage is a congenital heart disease, when the pulmonary veins do not empty into the left atrium but into the right atrium or into the accessory vein.

Anatomically, a complete ectopic pulmonary venous drainage can be classified into four types:
Pulmonary veins drain into a common superior vena cava in the majority (55%) of patients such as the left superior vena cava or rarely than the right superior vena cava. The pulmonary veins drain directly into the right atrium or into the coronary sinus in 30% of patients Pulmonary veins drain into the inferior vena cava or portal trunk, sometimes red into the duct of Arantius in 12% of patients Other sites accounted for 3%. Partially ectopic pulmonary vein is one or several pulmonary veins (2/3 of these veins are right pulmonary veins) with abnormal emptying locations such as draining into superior vena cava, left unnamed venous trunk , inferior vena cava, left superior vena cava and in rare cases empty into the coronary sinus, azygos vein, portal vein.
Some of the common combined malformations are atrial septal defect, monoventricular heart, transposition of the great arteries, Eisenmenger complex, tricuspid atrophy...
In the case of pulmonary venous outflow, it is completely ectopic. Systemic venous blood and pulmonary venous blood both rush to the right atrium, dilating the right chambers of the heart, dilating the pulmonary artery.
In case the pulmonary vein empties partially, part of the venous blood flows into the right atrium and returns to the urinary circulation. The long-term increase in the burden of the right chambers of the heart will lead to heart failure in patients.

The condition of ectopic pulmonary veins is detected when children are young, it can also be detected in older children and adults in the process of diagnosing atrial septal defect.
The symptomatology depends on the pathology and the magnitude of the left and right shunt. Patients present with varying degrees of shortness of breath and are prone to lung infections.
Children with ectopic pulmonary veins often grow slowly. Patients with partially ectopic pulmonary veins may not have cyanosis. The degree of cyanosis is greater if the pulmonary artery pressure is greater and the heart failure is more severe.

Cardiac doppler echocardiography is definitive in the majority of cases. If echocardiography is in doubt, the results of cardiac catheterization and chest imaging can be relied upon.

4.1 Treatment of ectopic pulmonary veins Surgical repair of anatomical abnormalities (pairing to return pulmonary venous blood flow to the left atrium, closure of the atrial septal defect) with the help of an external circulatory system body is the treatment of ectopic pulmonary veins. Depending on the prognosis and indications of the specialist, the patient will be treated in the following cases:
The patient has a confirmed diagnosis of an ectopic pulmonary vein The patient has symptoms: shortness of breath, cyanosis, recurrent infections, growth retardation, pulmonary hypertension Pulmonary hypertension, pulmonary vascular resistance <8 Wood units. Relative contraindications to ectopic pulmonary vein surgery in the following cases:
Fixed pulmonary hypertension Heart failure, severe hepatic and renal failure Complicated congenital heart disease Progressive infection Fixed pulmonary hypertension in Eisenmenger syndrome. Patients are prepared before surgery to treat ectopic pulmonary veins by bathing twice with water mixed with betadine and changing all clean clothes. In addition, brush your breasts with betadine soap before applying an antiseptic solution to the surgical area. The patient is also clearly explained by the doctor and signed an undertaking about the surgical treatment before the procedure.
4.2 Performing surgery on the ectopic pulmonary vein The patient is under endotracheal anesthesia, systemic Heparin anticoagulation, invasive monitoring lines. The patient was placed in a supine position with a padded pillow under the shoulder. Open midsternal line. Install a system of arterial and venous tubes, connecting the heart to the artificial heart-lung system. Inject cardiac paralysis solution to stop the heart. Open left heart chambers – must accurately assess lesions. Treatment of anatomical lesions Close the chambers of the heart. Hemostasis, placement of drainage system, electrodes. Closes the pericardium, closes the sternum and chest wall.

4.3 Monitoring and managing complications after surgery Patients after surgery to treat ectopic pulmonary veins need to be cared for and closely monitored for circulatory indicators such as pulse, electrocardiogram, arterial blood pressure. max, min, mean, central venous pressure, temperature; oxygen saturation, drainage tubes, urine output, ventilator parameters, mental status of the patient 1 hourly for 24 hours after surgery.
In addition, it is necessary to monitor the drainage tube, and the nature of the fluid 1 time / hour. If there are signs of bleeding, re-operate early to stop the bleeding. Patients also need to use pulmonary antihypertensive drugs if there are symptoms of pulmonary hypertension before surgery, chest x-ray, blood gas, electrolytes, hematocrit. In particular, patients need to maintain regular clinical examination and ultrasound every 6 months. If the condition is stable for 3 years, the disease is considered cured.
Ectopic pulmonary veins need to be treated early and in time to avoid life-threatening complications.
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