When a child is born with a congenital heart defect, both parents and doctors are concerned. Although congenital heart defects are by far the most common congenital disease in newborns, there are many questions.

May 5th is the day of the heart-sick child. That was why the Team Fair Imaging talked to Univ.- Professor Dr. med. Felix Berger, the director of the Clinic for Pediatric Cardiology at Charité – University Medicine Berlin and director of the clinic for congenital heart defects – pediatric cardiology at the German Heart Center Berlin.

Univ.- Professor Dr. med. Felix Berger

Felix Berger has been director of the pediatric cardiology clinic at Charité – University Medicine Berlin and director of the clinic for congenital heart defects – pediatric cardiology at the German Heart Center Berlin since 2004.

Born in the Palatinate and formerly an ice hockey player, is a specialist in the treatment of congenital heart defects and heads several international consortia dedicated to the research of therapies for congenital heart defects.

How often are children born with congenital heart defects?

Every 100th newborn is born with a congenital heart defect. With 700,000 to 800,000 live births in Germany, this means that around 7000 to 8000 children are born with a congenital heart defect each year.

When exactly does the congenital heart defect appear and how does it get recognized?

The congenital heart defect occurs in the first weeks of embryonic development at different times during the development of the heart and can be very complex.

Basically, one can expect that about 1/3 of the heart defects are simple heart defects and show a frequent tendency to heal spontaneously or require treatment only in later childhood or adolescence.

Another third are moderate heart defects that need to be treated; treatment can take place until school age.

The last third is very complex and urgent, so treatment for severe congenital heart disease must be done in the newborn or early infant period.

What about the prognosis and the importance of early detection?

The child’s prognosis depends on the timing and accuracy of the diagnosis. This is why prenatal diagnosis is crucial. In experienced hands, it enables the detection and classification of almost all heart defects at an early stage, which then allows pregnancy advice and birth planning in a congenital heart disease center.

The decisive factor is the detection and timely and specific treatment of the heart defect in order to enable survival without irreversible organ damage and a good quality of life.

The successes in the treatment of congenital heart diseases already allow more than 90% of all children with congenital heart diseases to live in good quality of life up to middle to old adult age. The prerequisite for this is permanent support from specialists for congenital heart defects in order to ensure optimal care and therapy control at all times.

How are children with cardiac diseases supported by imaging in pediatric cardiology and radiology?

The most important aspect is the timely and exact diagnosis. It defines the choice and control of therapy. This makes imaging in pediatric cardiology one of the most important tools for diagnosing. The most important working tool is echocardiography (echo) as a non-invasive imaging method, which in almost all cases allows the diagnosis and exact description of the congenital heart defects. Echo also allows control of treatment and outpatient controls visits at any time.

In order to answer specific questions, “sectional imaging” by means of an MRI (X-ray-free) or CT (X-ray-based) is advised (Fig. 1 and 2).

With these examination methods, for example, spatial relationships of vascular connections in the different parts of the heart can be defined. In addition, the positional relationships of the vessels to the chambers, but also the dimensions of the chambers, the spatial relationship of defects and the assignment to the vessel structures can be described pretty well. This additional information alone often makes it possible to decide to what extent a heart defect can be completely corrected or whether preparatory or partially corrective operations are necessary. In addition, there is invasive cardiac catheterization, which has moved away from the diagnostic to the therapeutic aspect in recent years. Cardiac catheterization can be used, for example, to measure the pressure in different heart and vessel sections. Today, however, cardiac catheterization is used to optimally carry out minimally invasive treatments for mild to moderate heart defects without surgery for the individual patient.

What are the challenges in treating these children in pediatric cardiology?

In addition to timely diagnosis, the initiation of specific therapy for heart defects is crucial. The primary goal is not only to treat the heart defect as correctively as possible, but also to achieve the best life prospects with maximum quality of life. Avoiding accompanying organ damage (lung / kidney / brain) has highest priority and the interaction of experts in a multidisciplinary team is an important prerequisite.

Here, the contribution of the respective specialist expertise in radiology, pneumology, nephrology and neurology plays a central role in addition to cardiological and cardiosurgical care. Risks such as radiation exposure are particularly important in order to avoid long-term consequences or other illnesses in later life. It is always about finding the most gentle form of therapy and diagnosis without having to accept a deficit of information.

Which family aspects are considered in the treatment in pediatric cardiology?

A congenital heart defect affects not only the individual patient, but also the whole family. Be it the clarification of a possible inheritance of the heart defect or the explicit information of the parents and later of course also of the patient him- or herself about the heart defect and the resulting consequences such as resilience or restrictions in daily life or special features relating to school or training .

All of this influences the social structure of the family. Special care by psychosocial staff and psychologists is therefore important during treatment.

The parents’ fears and insecurities about the present heart defect and the prospects often pose major problems that can only be addressed comprehensively through the transparent handling of all information and the sensitive integration of all psychosocial support services.

Fig. 1: CT diagnosis of an infant (1 month) with a complex early childhood heart defect. Precise knowledge of the anatomy is essential for precise diagnosis of the therapy. The examination was carried out on a state-of-the-art CT, which enables a quick examination with very low radiation dose. A-D shows a clearly changed anatomy of the heart, which is on the patient’s right side (B-D). In addition to other anatomical changes, it is also noticeable that the second main cavity of the heart (C *) is reduced in size. There is also a pathological connection between the two atria and the large cardiac cavities (à). The flow of blood to the heart is also different in this patient than usual, the lower vena cava is not created. Instead, the blood from the legs is passing through a continued vein from the abdominal cavity (azygos vein) to the right heart, which runs parallel to the main artery (A, B *).
© Dr. Thula Walter-Rittel

Fig. 2: Emergency CT diagnosis in a young girl after surgical replacement of the main artery.
Young patient who received a surgical replacement of the aorta due to a congenital dilation. The patient came to the clinic in an emergency with severe chest pain. The CT diagnostics were started immediately to rule out rupture of the aorta and occlusion of the coronary arteries. With modern CT technology, a quick and accurate diagnosis is possible. The CT shows the aortic replacement without rupture signs (A, B à), as well as the open coronary arteries (C *).
© Dr. Thula Walter-Rittel

Interdisciplinary Team members contributing to this blog post in addition to Prof. Berger:

Dr. Thula Walter-Rittel, Dr. Sven-Christian Weber-Bärenbrinker, Bernd Opgen-Rhein, Priv.-Doz. Thomas Elgeti, Prof. Marc Dewey