What is Congenital Heart Disease?

It is when the baby’s heart does not develop normally in the womb and structural and functional disorders occur. The baby’s heart is developed in the 8th week of pregnancy. There are many types of congenital heart diseases, from simple to complex, and the function of the abnormally structured heart and its ability to pump blood to the body can be seriously affected. Babies’ health, growth, and development may be affected to varying degrees. Congenital heart disease is seen in approximately 0.8% of every 100 live-born babies in Turkey. The development of technologies used for diagnosis, monitoring of these processes, and advances in surgical interventions allow babies with congenital heart disease to be diagnosed earlier and live a longer and healthier lives. It is recommended that babies with congenital heart disease be kept under the control of a professional team.

Anomalies in the baby’s heart can be detected early with fetal echocardiography performed during pregnancy. If congenital heart disease can be detected intrauterine (in the womb), family members can be informed to gain time to understand the diagnosis, evaluate fetal anomalies, and decide on the continuation of the pregnancy based on personal values. For families who want to continue the pregnancy, it is vital that doctors and the family plan fetal monitoring, deliver safely and on time, and choose a fully equipped hospital where medical care that may be needed immediately after birth can be provided. 

What are the symptoms of Congenital Heart Disease?

Depending on the type and severity of congenital heart disease, babies may experience many different symptoms. Some patients may have few or no symptoms. In symptomatic patients, bruising on the skin, lips, and nail bases, blisters on the fingers, respiratory distress, recurrent lung infections, difficulty in sucking, inability to gain weight, excessive sweating while sucking, shortness of breath, and getting tired easily while moving or playing may be noticed.

What are the causes of Congenital Heart Diseases?

Congenital heart disease occurs due to developmental disorders that occur in the womb during the first 8 weeks of pregnancy. Although the exact cause is unknown, some maternal factors may predispose to the development of congenital heart disease. Some medications used by the mother play a role in the development of congenital heart disease. For example, isotretinoin and anticonvulsant drugs used in the treatment of acne can be listed. It is known that some viral infections during the first 8 weeks of pregnancy may also play a role in the development of congenital heart disease. However, genetic anomalies may increase the risk of developing congenital heart disease. The most common genetic pathologies are Down syndrome, Turner syndrome, Marfan syndrome, and Di-George syndrome. The probability of developing congenital heart disease in pregnancies using assisted reproductive techniques and in babies of parents with a family history of congenital heart disease increases, albeit slightly. That’s why families need to receive genetic counseling before pregnancy. 

How are Congenital Heart Diseases diagnosed?

It can generally be diagnosed by prenatal fetal echocardiography, but in our country it is mostly diagnosed by postnatal transthoracic echocardiography (TTE). Very rarely, the diagnosis can be made at a later age due to the child’s lack of signs and symptoms. Procedures performed for diagnosis include ECG (electrocardiography), echocardiography (ECHO), angiography, magnetic resonance imaging (MRI), computerized tomography (CT). Some heart anomalies may be difficult to detect at an early age and may not be diagnosed until adulthood. The diagnosis of these patients depends on the development of complications. Besides ECG and ECHO, the most commonly used device for diagnosis is pulse oximeter. Thanks to this device, the oxygen saturation in the baby’s fingers and toes is simply measured, and the performance of the heart and the amount of oxygen in the blood circulating in the body are measured.

Who are the members of the Congenital Heart Diseases team?

Early surgery is recommended for babies who need heart surgery during the neonatal period. In the operated patient, the heart grows with the child and a faster recovery process occurs during childhood. More than two-thirds of childhood cardiovascular operations are performed on patients under one year of age. A heart-lung machine is used in most of these surgeries. This method makes our surgical procedures more efficient and safe. A unique treatment program is applied to each patient. For this reason, it is crucial to perform the necessary surgery on time with the correct diagnosis, technique, and team. The diagnosis of Congenital Heart Disease is made by the Pediatric Cardiologist, who is an integral part of the team. After the necessary interventions are performed on the patient by the Pediatric Anesthesiologist in the operating room, the surgery is performed by the Pediatric Cardiac Surgeon. The heart-lung machine used during the surgery is managed by a Perfusionist experienced in pediatric heart surgeries. When the surgery is completed, the patient is taken to the intensive care unit and entrusted to an experienced team including expert cardiologists, nurses and intensive care specialist doctors. 

Treatment of Congenital Heart Diseases

Interventions that can be performed for the treatment of congenital heart patients include drug therapy, interventional angiography, and heart surgery. Although some congenital heart diseases do not require treatment, intervention is often required in children with clinical complaints. For this reason, we serve all our patients every day, every hour in our hospital. We apply all treatment methods used in cardiac surgery. NIRS (Near-Infrared Spectroscopy) device, which provides close monitoring, is used to monitor and protect our patient’s brain functions during heart surgery. Except for procedures that require a sterile environment, care is taken to ensure close contact between the family and the baby.

Closure of ventricular septal defect through a minimal incision

Our goal is to diagnose and treat cardiac disease as early as possible. Our main goal is to treat the child as soon as possible and return him to normal life before he reaches school age. While some congenital heart diseases can only be treated with medications and monitored by a pediatric cardiologist, some complicated heart diseases can also be treated with cardiac catheterization (angiography) and sometimes surgery at a very early stage. Thus, children can have normal lives after surgery or angiographic treatment. However, in other complex heart diseases, combined treatment protocols may be applied and several staged heart surgeries may be required. We have a large team that treats each patient individually and determines the best treatment for the child. For some severe congenital heart diseases, babies may need surgery immediately after birth. Early treatment and follow-up are necessary as loss of time may cause the chance of surgery to be missed.

Echocardiography (ECHO)

Echocardiography is an imaging method that obtains a two-dimensional and three-dimensional real-time image of the heart using sound waves. Information and measurements about the anatomy and function of the heart are obtained. High-frequency sound waves that are too high to be heard by the ear, sent to the chest cage through the device, are collected when they return from the body and measurements that give an idea about the appearance and functions of the heart are obtained. In other words, echocardiography is a computer that converts sound waves sent to the heart into images. It can be performed at any time throughout life, including the period in the womb. Echocardiography examination is used effectively as an auxiliary imaging method during angiography in the diagnosis of heart problems, in planning the surgery before heart surgery, and in evaluating follow-ups in the post-heart surgery period.

Types of echocardiography are Transthoracic Echocardiography (TTE), Transesophageal Echocardiography (TEE), Fetal Echocardiography, 3D Echocardiography, Stress Echocardiography, Contrast Echocardiography, Intravascular Echocardiography. Transesophageal echocardiography is an echocardiography method based on imaging the heart from inside the body through a specialized transducer placed in the esophagus. In TEE, sound waves do not have to pass through the skin, muscle, and bone tissue, resulting in a much clearer image. Sedation is always used during TEE. Sedation may sometimes be required in young children due to poor patient compliance. In this way, patient compliance is increased and image quality and measurements can be made in the most accurate way. There is no pain during echocardiography because echocardiography works with sound waves, but sometimes pressure on the chest cage to increase image quality can cause mild pain and discomfort in children. In addition, the coldness of the gel applied and the tickling when touched may cause disharmony and restlessness in the child. Echocardiography is not harmful to health, it works only with sound waves and does not emit any radiation. For this reason, it can be used safely both during pregnancy to check the baby’s heart and in children. 

Angiography

The heart chambers and veins are accessed by advancing through the vein through thin wires and pipes with high-tech features. Here, pressure measurements and blood gas samples are taken and calculations are made. Again, anatomy is revealed by applying contrast substance to these areas. However, interventional procedures can also be performed for therapeutic purposes. Removing stenosis by inflating a balloon in narrow areas, closing the holes in the heart walls with special occlusive devices, or placing a stent in the vessel that needs to remain open can be done by angiography. Angiography procedures in children are performed under general anesthesia due to the need for full patient compliance. Angiography is the method used to identify or resolve complications and pathologies before or after surgery, diagnostic or interventional.

Magnetic Resonance Imaging (MRI)

Cardiac MRI is one of the fastest-growing diagnostic tools in the field of Pediatric Cardiology. It offers the opportunity to obtain 3D and high-resolution images of the anatomy of the heart and advanced pathologies. It is a non-invasive and radiation-free method. It is often performed under sedation due to the length of the shooting period and the need for patient compliance. Its main advantages over other methods are that it provides a real high-resolution image of the anatomy and the relationship with the surrounding organs with a 3-dimensional image, that flow studies can be performed, and that it is non-invasive and does not contain radiation.

Computed Tomography (CT)

Similarly, it offers the opportunity to obtain 3D and high-resolution images non-invasively. Its biggest advantage is that it has a higher resolution and a shorter acquisition time compared to MRI. Even if sedation is necessary, short-term protocols are applied. But radiation exposure is its most important disadvantage. The radiation level has been significantly reduced in the latest technology CTs developed today.