Treatment of Anomalous Pulmonary Venous Connection in Turkey

Anomalous pulmonary venous connection is a group of severe congenital heart defects. To save the life of the patient, in many cases pulmonary venous return has to be corrected in the first months, and even in the first weeks of a child's life. The surgical procedure aimed at the defect elimination is complex and dangerous. The doctors often have to simultaneously restore pulmonary venous drainage and correct concomitant anomalies in the development of the cardiovascular system.

Many patients prefer to go abroad for treatment. In recent years, Turkey has become one of the most popular destinations for the correction of anomalous pulmonary venous return and other heart defects. The doctors in this country perform complex cardiac surgeries. Turkey offers services of many experienced cardiac surgeons who can restore normal pulmonary venous drainage even in newborns.

What is partial anomalous pulmonary venous connection? 

Partial pulmonary venous connection is a heart defect, in which there is no connection of one or more (but not all) pulmonary veins with the left atrium. Normally, four pulmonary venous flows into it, but with anomalous pulmonary venous return, they drain into:

• Right atrium
• Coronary sinus
• Drainage systems of the superior or inferior vena cava

The anomalous pulmonary venous connection with a combination of these options is also possible. 

Partial pulmonary venous connection is not such a severe pathology as total anomalous pulmonary venous return. Even if untreated, hemodynamics will be compensated. With the isolated partial anomalous pulmonary venous connection, patients live on average 30-40 years. With scimitar syndrome (a variant of anomalous pulmonary venous connection, in which the right pulmonary veins flow into the inferior vena cava), life expectancy without treatment is 20-30 years.

What is total anomalous pulmonary venous connection?

Total anomalous pulmonary venous connection is a group of congenital heart defects, in which there are no direct connection between the pulmonary veins and the right atrium. In this case, the child will have:

• Expansion and thickening of the chambers of the right half of the heart
• Expansion of the pulmonary artery
• Underdevelopment of the left side of the heart
• Decrease in the volume of the left atrium

The forms of total anomalous pulmonary venous connection may be different. The pulmonary venous connection can be formed with the left atrium, coronary sinus, and portal vein. Pulmonary vein stenosis occurs when the abnormal trunk and venous duct are connected to one of the hepatic veins or the inferior vena cava.

Total anomalous pulmonary venous connection is a severe heart defect. In addition, it can often be combined with other defects, such as:

• Atrioventricular septal defect
• Tetralogy of Fallot
• Absent pulmonary valve
• Transposition of the great vessels
• Coarctation of the aorta
• Ventricular septal defect

Total anomalous pulmonary venous connection is more common than the partial one. It accounts for 1-2% of all congenital heart defects. Total anomalous pulmonary venous connection is characterized by a high hospital and intraoperative mortality. Depending on the type of pulmonary venous connection, the mortality rate is from 10 to 30%.

Total anomalous pulmonary venous connection can be obstructive and non-obstructive, depending on the presence or absence of pulmonary vein stenosis. The obstructive form of anomalous pulmonary venous return is more severe. This option of total anomalous pulmonary venous connection leads to the death of the patient in the neonatal period, and therefore it forces doctors to provide treatment immediately. Nonobstructive total anomalous pulmonary venous connection in the natural course has the mortality rate of 50% during the first 3 months of life and 80% within 12 months.

Causes

Anomalous pulmonary venous connection is a congenital heart defect. Pulmonary venous pathology can be triggered by many causes: infectious, toxic, medicinal, endocrine, genetic. Sometimes anomalous pulmonary venous connection is the result of a complicated pregnancy.

Diagnostics

Partial anomalous pulmonary venous connection can sometimes remain undetected. This is due to the fact that this form of pulmonary venous drainage is not very severe. According to clinical data, pulmonary venous connection accounts for 0.3% among all congenital heart defects, while according to autopsy, it is twice as much – 0.6%.

The symptoms of the impaired pulmonary venous connection in a child: 

• Palpitations
• Fatigue
• Shortness of breath
• Poor weight gain
• Hemoptysis
• Frequent infectious lung diseases

Sometimes the symptoms of impaired pulmonary venous drainage can be mild. Partial anomalous pulmonary venous connection can be manifested only by pallor, sweating, weakness. Total anomalous pulmonary venous connection can cause cyanosis, fainting.

The diagnosis of the pathological pulmonary venous connection is established using echocardiography with the color Doppler mapping mode. To clarify the peculiarities of anomalous pulmonary venous return, MRI is used.

Cardiac catheterization with angiography for pathological pulmonary venous connection can be performed in case of suspected pulmonary hypertension. It can also be performed for patients with an obstructive form of total anomalous pulmonary venous connection to determine the degree of pulmonary venous obstruction.

Indications and timing for the operation

With partial pulmonary venous connection, the operation is performed with a ratio of Qp:Qs of more than 1.5, while with isolated partial anomalous pulmonary venous – more than 1.8. It is optimal to perform it at the age of 1-2 years.

In case of total anomalous pulmonary venous connection, the operation is performed as early as possible. It is always performed during the first months of life, whereas the obstructive form of the disease requires surgery during the neonatal period.

How is an operation for anomalous pulmonary venous connection performed?

During the operation, the surgeon performs many manipulations aimed primarily at directing the pulmonary venous return to the left atrium. To do this, he creates a connection between the common pulmonary vein and the left atrium, thereby interrupting the connection of the pulmonary veins with the venous circulatory system.

Once pulmonary venous return has been normalized, the doctor will close atrial septal defect. Additionally, some patients with anomalous pulmonary venous return can undergo a surgical procedure to restore vertical vein patency.

The pulmonary venous connection can also sometimes be maintained. This is possible if during the trial clamping of a vertical vein blood pressure decreases, while the left atrium has increased pressure.

In some cases, vertical vein intervention may be delayed. The correction of pulmonary venous return can result in the narrowing of the vertical vein. In the future, its endovascular occlusion will be performed (closure of the lumen during a minimally invasive procedure with percutaneous access).

Although all patients with pathological pulmonary venous return have a decrease in the size of the left atrium, during the operation it will not be increased. This is due to the fact that this procedure does not affect the survival of patients with anomalous pulmonary venous connection.

In severe cases, the immediate surgical correction of pulmonary venous return is not possible. In this case, to stabilize the child's condition, balloon atrioseptostomy will be performed. This is a minimally invasive surgical procedure, during which a doctor creates a connection between the right and left halves of the heart. It does not normalize pulmonary venous return, but allows for future surgery to correct the heart defect.

Where to undergo anomalous pulmonary venous connection treatment?

To restore normal pulmonary venous return, you can go to Turkey. This country offers low prices for medical services, but their quality is high.

The restoration of pulmonary venous connection in this country has the following benefits:

• Experienced cardiac surgeons. Some of them have already performed hundreds of operations to correct heart defects, including the restoration of pulmonary venous return.
• Cutting-edge surgical techniques. Turkish clinics use the best methods for restoring normal pulmonary venous connection, which are well established in the West.
• Well-equipped hospitals. The Turkish government creates favorable conditions for the development of medicine in this country in order to attract foreign patients here. Therefore, patients with pathological pulmonary venous return are treated in the Heart Surgery Centers equipped with state-of-the-art medical technology.
• Low mortality. The redirection of pulmonary venous return, especially in case of anomalous pulmonary venous connection, is a complex surgical procedure. However, the best Turkish clinics demonstrate a low mortality rate of patients and a low risk of complications.
• Intensive care of newborns. If a child is not yet ready for surgery to correct pulmonary venous return, he will receive treatment that helps stabilize the patient's condition and compensate for hemodynamic impairment. If necessary, doctors can perform minimally invasive operations to prepare for the main intervention to restore normal pulmonary venous connection.
• Rehabilitation. After the restoration of pulmonary venous return, a child can undergo cardiac rehabilitation. It can not only improve physical development, increase exercise tolerance, but also favorably affects the survival of patients with pathological pulmonary venous connection.
• Prices. You can get information about the cost of treatment in Turkey at various clinics on our portal. In any case, the correction of pulmonary venous return in Turkish clinics costs 30-50% cheaper than in Western Europe, the USA or developed countries of Asia.

Where to go for help?

To restore pulmonary venous return efficiently, safely and at an affordable price, you can travel to Turkey. Cooperation with Booking Health will reduce the cost of treatment in Turkey. Our service provides information about the main clinics in Turkey, the prices of medical treatment programs. It also presents the rating of clinics and patient reviews on the treatment of pathological pulmonary venous return.

You can book anomalous pulmonary venous connection treatment in Turkey via Booking Health. You will get many benefits:

• Selection of the best clinic for anomalous pulmonary venous connection treatment in Turkey

• Direct communication with a doctor who corrects pulmonary venous return
• Reduced waiting time for anomalous pulmonary venous connection treatment in Turkey
• Booking treatment in Turkey
• Reduced cost of treatment in Turkey – the prices for the correction of pulmonary venous return will be reduced due to avoiding additional coefficients for foreign patients
• Information on the expenses for medical procedures, price control and return of unspent funds
• Insurance in the amount of up to 200 thousand euros, so the cost of treatment in Turkey will not increase even in case of complications
• Preparation of a medical treatment program for anomalous pulmonary venous connection in Turkey without repeating previous examinations
• Communication with the hospital after treatment in Turkey
• Organization of additional diagnostics, treatment in Turkey or rehabilitation after surgical correction of pulmonary venous return

Booking Health specialists provide high quality services. We will book a hotel and airline tickets for you, meet you at the airport and take you to the hospital by car, where you will undergo surgery to restore normal pulmonary venous return.