Hypoplastic right heart (HRH)
In HRHS there is also no way to stimulate the underdeveloped right side of the heart to grow. The surgical options depend on whether all sections of the right heart are underdeveloped or only individual parts are affected. In tricuspid atresia, the right ventricle is so small that the left side of the heart has to take over the entire work of the heart in the long term. In pulmonary atresia, the situation can be somewhat more favourable and the opening of the closed pulmonary valve (pulmonary artery valve) by surgery or cardiac catheter intervention allows the small right ventricle to catch up.
1. Aortopulmonary shunt:
The first operation is usually to create a connection between the aorta and the pulmonary artery. For this purpose, a small plastic tube is sewn between the brachial artery and the pulmonary artery. The aortopulmonary shunt replaces the ductus arteriosus, which is then also surgically closed. This ensures the child's oxygen supply, but the patient remains cyanotic (cyanosis). The operation is performed in the first few days of life. In some paediatric heart centres, including ours, the ductus arteriosus can be fitted with a vascular support (stent) as an alternative to the aortopulmonary shunt. This is done in a cardiac catheter intervention, so that surgery is not initially necessary. In combination with an opening of the pulmonary valve, also in the cardiac catheter laboratory (radiofrequency perforation) Link, the situation may develop so favourably that the right ventricle takes over more and more of its intended work and no further operations are necessary.
2. Glenn operation:
If the conditions are not so favourable, the large vein of the upper half of the body (superior vena cava) is connected to the pulmonary artery (Glenn operation) at the age of 4-6 months and the aortopulmonary shunt or stented duct is surgically closed at the same time.
3. Total cavapulmonary anastomosis (TCPC):
As with hypoplastic left heart syndrome, in all those patients in whom the right ventricle remains too small at the age of 3 years (2-4 years), the large vein of the lower half of the body (inferior vena cava) is also connected to the pulmonary artery. For this purpose, a plastic tube is sewn between the two blood vessels. This idea of supplying venous blood to the lungs was first invented by the Frenchman "Francis Fontan" in the 1980s, and the circulation that is achieved with it is also known as the Fontan circulation. From this point onwards, patients no longer have cyanosis (cyanosis). Translated with www.DeepL.com/Translator (free version)