Consultation hours for congenital heart defects

Heart valve defects are among the most common congenital heart defects.

The bicuspid aortic valve occurs in 1-2% of the population. This congenital heart defect can also be genetic. It is characterized by the fact that the valve on the aorta only has two pockets instead of three, as is the case in a healthy heart. This can lead to the valve leaking and/or narrowing. However, the opposite is also often the case, i.e. the aorta is dilated, sometimes by more than 50% (aortic aneurysm).

Mitral valve prolapse is also often congenital. This condition often occurs with connective tissue weakness and can cause the mitral valve to become increasingly permeable, i.e. leaky. Most patients with this heart defect do not require surgery. They simply need to undergo regular check-ups and have any accompanying symptoms, such as cardiac arrhythmia, treated if necessary.

Pulmonary stenosis, i.e. narrowing of the pulmonary valve, is often ballooned (or operated on) in childhood and then remains stable, i.e. practically does not change in the course of life. If the pulmonary valve is leaking, no intervention is often necessary. The pulmonary valve rarely has to be replaced by an artificial heart valve; nowadays this can occasionally be done using a catheter.

A congenital disease of the tricuspid valve is rare and occurs, for example, in Ebstein's anomaly. Ebstein's anomaly is often associated with arrhythmia and an open foramen ovale or an atrial septal defect. In the case of an open foramen ovale or atrial septal defect, there is usually a very small hole in the septum between the atria. Heart valve surgery for an Ebstein's anomaly is very rare and must be performed at a university center.

Any hole in a dividing wall of the heart can result in a more or less large overflow of blood (shunt). Depending on the pressure conditions, the blood then flows through the hole from the systemic circulation into the pulmonary circulation or vice versa, which can lead to symptoms.

At birth, everyone has a patent foramen ovale (PFO), i.e. a small connection between the right and left atrium of the heart. This connection is still present in 25% of adults, but this is not considered a heart defect. An open foramen ovale very rarely causes problems. For example, an open foramen ovale can lead to a so-called paradoxical embolism. The occurrence of a paradoxical embolism depends on the size of the PFO, the pressure in the right atrium and the mobility of the atrial wall. A paradoxical embolism is characterized by the passage of a small clot from the right-sided heart cavities to the left side into the systemic circulation, which can lead to a stroke, heart attack or occlusion of another arterial vessel. A small hole in the atrial septum can also lead to a drop in saturation in the great circulation caused by deoxygenated blood from the right side. If any of these problems occur, the open foramen ovale can be closed with a small device.

The atrial septal defect is at least as common as the ventricular septal defect. In an atrial septal defect, there is a gap in the septum between the two atria. An atrial septal defect can either be closed with an "umbrella" (e.g. an Amplatzer septal occluder) or surgically. Occasionally, family screening is also recommended to find out whether this heart defect is genetic.

A ventricular septal defect (VSD) is a hole in the ventricular septum between the right and left ventricle. A VSD is rare in adulthood; small VSDs usually close in childhood or adolescence or are closed surgically. Occasionally, however, these defects are not closed, especially if they are very small or inoperable for other reasons.

A persistent ductus arteriosus botalli is a connection between the aorta and the pulmonary artery. Normally, this open connection only exists in the womb and closes in the first few days after birth. If the connection does not close, an operation or closure with a device is necessary. If the open D. Botalli is very small, it can usually be left open.

Anomalous pulmonary veins are rare and can occur in isolation or in combination with another heart defect, such as a sinus venosus defect. A sinus venosus defect, in which one or rarely several pulmonary veins mistakenly open into the right atrium, is usually operated on depending on the age of the patient and any other diseases that may be present. If there is no other heart defect, surgery may not be necessary in some cases.

Tetralogy of Fallot is a combination of four different defects: Pulmonary stenosis (narrowing of the pulmonary valve), ventricular septal defect (hole in the cardiac septum), hypertrophy (muscle thickening) in the right ventricle (heart chamber) and a so-called riding aorta (the main artery exits from both ventricles instead of the left ventricle). Patients with tetralogy of Fallot have usually already undergone surgery in adulthood, but require lifelong monitoring. Certain residual findings, such as pulmonary valve insufficiency, are common and do not always require surgery.

Aortic isthmus stenosis is a congenital narrowing of the aorta after the aortic arch. This heart defect is usually diagnosed in childhood or in newborns and treated by surgery or by inserting a balloon/stent. As adults, patients with aortic coarctation must undergo regular check-ups. It is important to ensure optimal blood pressure and to be able to rule out the possibility that certain parts of the aorta in the area of the operated site are still narrowed.

In transposition of the great vessels (TGA), the aorta departs from the right ventricle and the pulmonary artery from the left ventricle. This can also rarely occur in the form of a so-called congenitally corrected transposition, in which the atria are also reversed. In this case, the blood flow in the child or adult is not inadequate, so there is no cyanosis. Nevertheless, heart failure and heart valve problems can occur at a later stage. In the more common normal TGA, the newborn's circulation is severely impaired, it is cyanotic and there is cardiac insufficiency. Therefore, an intervention must be carried out immediately after birth, which is nowadays performed in the form of a so-called switch operation. This involves reconnecting the arteries to the right ventricle. In the past, complex operations such as the Senning or Mustard operation were performed instead of the switch operation. This involved diverting the blood flow in the atria so that the right ventricle remained the main ventricle. Patients with this heart defect must undergo regular check-ups with a cardiologist for the rest of their lives.