Frequency of congenital heart defects
Out of 1,000 live births, 8 to 10 children have a congenital heart defect or a large vessel defect that leads to the heart . Congenital heart defects are thus the most common congenital diseases. Without treatment , 25 percent of children die in infancy and around 60 percent die in their first year of life. Only 15 percent reach adulthood without treatment.
25 percent of children still suffer from other illnesses , such as a number of chromosomal defects (genetic diseases). As an example, the Down syndrome is mentioned, which is associated to 30 - 40 percent with narrowing of the main artery ( Aortenisthmusstenose ).
Classification and presentation of possible congenital heart defects
Congenital heart defects can be divided into the following groups:
- Cardiac and vascular defect without short-circuit connection, with pressure load of the ventricle (eg aortic coarctation, aortic stenosis, pulmonary stenosis)
- Malformation with pulmonary circulation or volume load of the small circulation: Left, right shunt (eg ductus botalli, ventricular septal defect, atrial septal defect)
- Malformation with pulmonary blood flow: right, left shunt (eg, Fallot's tetralogy, ventricular septal defect)
- Rotational malformations, which are often combined with atrial or ventricular septal defects and pulmonary or sub-circulation (eg, transposition of the large arteries, double-outlet left / right ventricles)
Below are explanations of some of the known congenital heart defects.
aortic stenosis
In the case of aortic stenosis, an adhesion of the pocket flaps usually leads to a reduced opening area . Only by increasing left ventricular muscle mass can the increased resistance at the heart valve be overcome and the heart defect compensated.
If the children develop discomfort such as breathlessness or paleness on exertion, the operation serves to restore normal valve function.
pulmonary stenosis
A narrowing in the area of the pulmonary valve leads to an increased load on the right ventricle. Often there is a fusion of the pockets which prevents complete opening of the pulmonary valve.
In addition to the expansion by means of a cardiac catheter, various surgical procedures for correcting the stenosis can be used, depending on the anatomical findings. The age of surgery depends on the severity of pulmonary stenosis.
Tetralogy of Fallot
Fallot tetralogy is a cyanotic heart defect that involves the combination of four related cardiac abnormalities : pulmonary valve stenosis, with concomitant increase in right ventricular muscle mass, aortic root displacement, ventricular septal defect.
The resulting reduced pulmonary blood flow characterizes the clinical picture with heavy breathing and blue-bloodedness.
In the foreground of the always operational procedure is the lung perfusion to be improved. The aim is a complete correction with elimination of pulmonary valve stenosis and closure of the ventricular septal defect in infancy. However, this is not always possible due to a complex change in heart anatomy or heavy blue crops. For improved pulmonary blood flow, a short circuit of the aorta with the pulmonary circulation is initially created. A correction will be made later.
Open ductus arteriosus botalli (ductus botalli)
The Ductus Botalli connects the Pulmonalarterienstrombahn with the main artery and leads before birth, the blood coming from the right ventricle to the systemic circulation. This bypass of the pulmonary circulation is, among other things, important for the oxygen and nutrient supply of the unborn child.
Normally, the ductus botalli closes spontaneously in the first days after birth. However, if this occlusion fails, more blood enters the pulmonary circulation in the opposite direction. Left ventricular strain and pulmonary hypertension are the result. The focus of treatment is the occlusion of the ductus botalli. An operative ligation occurs occasionally already in the newborn age .
Transposition of the Great Arteries (TGA)
In this severe cyanotic heart defect, the aorta originates from the right ventricle and the pulmonary artery from the left ventricle.
Survival is only possible if there is a sufficient connection between the pulmonary circulation and the systemic circulation. Due to the pronounced cyanosis after birth, an operation to transpose these two large arteries and the coronary arteries is required within the first few weeks of life .
Ventricular septal defect (VSD)
Ventricular septal defect (VSD) is the most common congenital heart defect . Due to an opening in the ventricular septum, arterial blood increasingly passes from the left ventricle into the right ventricle. Depending on the size of the ventricular septal defect, hypertension in the pulmonary circulation develops in addition to the left heart strain.
If spontaneous occlusion does not occur in the first years of life, surgical correction of the ventricular septal defect is required. The closure of the opening in the septum by means of seam or art patches.
Atrial septal defect (atrial septal defect, ASD)
In atrial septal defect, a defect in the atrial septum redirects some of the blood from the left atrium back to the right atrium . This results depending on the size of the atrial septal defect an increasingly circulating pulmonary circulation with concomitant right heart strain.
If the pendulum volume exceeds a critical value, closure of the defect is required. In large atrial septal defects and unfavorable position, the opening is surgically closed mostly in preschool age.
Causes of congenital heart disease
If there is growth disturbance in the area of the heart and / or the large supply and / or discharge vessels during cardiac development in the womb, congenital heart defects result. In more than 90 percent, the cause is unknown.
One cause may be infectious diseases , for example, redness, herpes or cytomegalovirus infection, in the first trimester of pregnancy. In addition to alcohol consumption , drug intake can also influence embryonic heart development. Genetic radiation damage can also lead to congenital heart defects.
Symptoms vary
Symptoms may vary depending on the heart defect. The most noticeable is a heavy cyanosis (Blauchucht). With incipient heart failure, the body can no longer be sufficiently supplied with oxygen. Children and adults with congenital heart disease are constantly tired and find every activity strenuous. They breathe fast and short.
Cardiac arrhythmias can also be the result of congenital heart disease. In adulthood, surgically corrected or not, damage is most likely to be noticeable with typical signs of heart failure. In addition to tiredness and fatigue, these are shortness of breath and cardiac arrhythmia.
Diagnosis of a congenital heart defect
As a drug of choice (gold standard), the ultrasound of the heart ( echocardiography ) has proven itself. In children, echocardiography through the chest wall is usually sufficient from the outside. Increasing chest circumference and rib strength in adults with complex heart defects is indicated by " swallowing ultrasound" (TEE), where a small ultrasound head is inserted into the esophagus via a tube and the doctor has an unobstructed view of the heart from behind the esophagus allowed.
Furthermore, examinations using computer and magnetic resonance tomography are available. As an invasive method, with puncture of the vessels in the groin, both the small and the large systemic circulation can be visualized and assessed by means of contrast media (angiography).
Protection against inflammation of the inner lining of the vessel
Both children and adults with congenital heart defects have to be saved for life from developing inflammation of the endocrine (endocarditis). Most commonly, the heart valves are affected. In particular with interventions at the dentist, in the mouth and throat area, gastrointestinal tract as well as at the urinary tract a suitable "Endokarditisprophylaxe" must be operated.
Treatment of malformation
Corrective surgery has been developed over the past 25 to 30 years for almost all cardiac and vascular malformations . In a few cases, the operation will create a completely healthy heart. In most cases these are "repair conditions" with critical points in the area of the seams. The development of pediatric surgery has made it possible, especially in the last 10 to 15 years, to successfully operate on complex congenital heart defects.
Due to the short surgical history, long-term results will not be available in the next few years. Furthermore, in recent years, catheter techniques have been developed which allow to treat heart septum defects and valve narrowings.
Patients with corrected congenital heart disease that have outgrown adolescence are being treated by pediatric and adult cardiologists.
Prognosis for children with congenital heart disease
The prospects of children with congenital heart disease are very different, depending on the severity of the heart failure and the time of diagnosis. With medical advances, especially cardiac surgery, 90 percent of children with congenital heart disease reach adulthood.
In specialist centers, pediatric and adult cardiologists have joined forces to ensure optimal care for patients with congenital heart disease. Although adolescents and adults with congenital heart disease are chronically ill, they can achieve a good quality of life through targeted care and counseling.