Pulmonary vein malocclusion

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Classification according to ICD-10
Q26.2 Total misjunction of the pulmonary veins
Q26.3 Partial misjunction of the pulmonary veins
ICD-10 online (WHO version 2019)

In an anomalous pulmonary venous transporting pulmonary vein oxygen-rich blood to the right side of the heart - circulatory system's and not, as usually, in the left atrium.

Total anomalous pulmonary venous connection (TAPVC)

All four pulmonary veins are connected to the right circulation. So there is a complete pulmonary vein malfunction . They can lead to different parts:

An operation is performed as soon as possible after the diagnosis using a heart-lung machine . If the foramen ovale closes too quickly after birth , a Rashkind maneuver (tearing of the atrial septum) is carried out to bridge the gap until the operation .

Partial pulmonary vein malocclusion

Only some of the pulmonary veins open incorrectly and only affect one lung at a time, with the right pulmonary vein malfunctioning twice as often as the left pulmonary vein. They flow into

  • the superior vena cava
  • in the right atrium
  • rarely into the inferior vena cava

In most cases, there is also an atrial septal defect (ASD).

Due to the sometimes wrong opening of the pulmonary veins, more oxygen-rich blood reaches the right and in terms of volume loads the right heart (atrium and ventricle) and leads to increased blood flow to the lungs.

The children are usually fully resilient and symptom-free. The heart defect is often discovered by chance during a preventive examination or an infection .

If the right heart and lungs are clearly stressed, the operation using the heart-lung machine is indicated. The misdirected lung blood is diverted into the left atrium using a patch in a tunnel shape.

The Scimitar Syndrome

A very rare form of partial pulmonary vein malfunction is the Scimitar syndrome (also called "Turkish saber"). All or part of the pulmonary veins from the right lung - mostly the pulmonary veins of the right middle and lower lobes - open into a collecting vessel, which leads to the inferior vena cava in the area of ​​the diaphragm and opens there. This collection vessel can be seen in the X-ray image . It has a winding course that is reminiscent of the shape of a Turkish sword - a scimitar . This name was first used in 1956. However, there are descriptions of this clinical picture from the years 1836 and 1912. Blood vessels can also move from the aorta (body artery) to these lung lobes and thereby form lung areas that are flooded with blood. These proportions are called lung sequesters . Often with this clinical picture the right lung and / or the bronchi are underdeveloped and the heart is shifted to the right side.
The high blood supply to the right heart (left-right shunt ) puts stress on the entire cardiac output and the lungs and can lead to increased lung infections and bronchitis . There is an increased risk of pulmonary hypertension (high pulmonary pressure), especially with an additional blood supply from the aorta .
A distinction is made between an "adult form" of this clinical picture, which manifests itself in the form of a shunt, as in the case of atrial septal defect , from the "infant form", in which there is severe underdevelopment of the lungs and pulmonary hypertension has developed.

Treatment for Scimitar syndrome is surgical correction. The blood flow from the scimitar vein is diverted to the left atrium using patch techniques. The procedure depends on the patient's anatomical findings. The long-term results in the adult form after the operation are to be regarded as favorable. In the infant form, the prognosis depends on the extent of the pulmonary hypoplasia .

Diagnosis

forecast

The long-term successes after the operation of a pulmonary vein malocclusion are usually very good. With the exception of (possibly) the “infant form” in Scimitar syndrome, no impairment of resilience is to be expected. Lifelong cardiological check- ups at longer intervals are indicated. Lifelong endocarditis prophylaxis must also be taken into account.