- Emergency
- Prenatal information.
- Congenital heart defects.
- Atrial septal defect (ASD).
- Atrioventricular septal defect (AVSD).
- Ventricular septal defect (VSD).
- Aortopulmonary window.
- Aortic arch anomalies.
- Coarctation of the aorta.
- Interrupted aortic arch.
- Persistent ductus arteriosus (PDA).
- Cor triatriatrum.
- Ebstein’s anomaly.
- Pulmonary stenosis.
- Fallot’s tetralogy.
- Coronary artery anomalies.
- D-transposition of the great arteries (d-TGA).
- Aortic stenosis (AS).
- Total anomalous pulmonary venous connection (TAPVC).
- Pulmonary atresia with intact ventricular septum (PA + IVS).
- Double Outlet Right Ventricle (DORV).
- Truncus Arteriosus (TA).
- Hypoplastic left heart syndrome (HLHS).
- Univentricular heart (UVH).
- Treatment principles.
- Heart transplantation in infancy and childhood.
- Children's ward.
Hypoplastic left heart syndrome (HLHS)
The term “hypoplastic left heart syndrome" is characterised by the following changes:
- Hypoplastic left ventricle
- Mitral valve stenosis or mitral valve atresia
- Aortic valve stenosis or aortic valve atresia
- Hypoplastic aortic arch
In addition, there is often an intra-atrial gap that allows outflow of pulmonary venous blood. Otherwise, the defect is not compatible with life. The systemic circulation is supplied through a patent ductus arteriosus. The life expectancy of the patients depends on the size of the atrial septal defect and on the calibre of the ductus arteriosus. Without treatment, the average life expectancy is 4 to 7 days.
From the surgical aspect, two options are offered today:
- Heart transplantation
- Norwood's operation (this operation is usually performed in three stages).
In the first operation, performed shortly after birth, the ascending aorta is extended using the pulmonary artery, which is divided before the bifurcation, and the aortic arch is widened as far as the back of the isthmus of the aorta using a homograft patch. Lung perfusion is ensured by creating a modified Blalock-Taussig shunt or recently by creating a RV-PA shunt.
The second step is performed at the age of about four to six months and consists of removal of the shunt along with a Glenn or hemi-Fontan operation.
The third step at the age of about 18-24 months, finally, is completion of the Fontan circulation, either by implantation of an intra-atrial tunnel or by creation of an extracardiac conduit from the inferior vena cava to the pulmonary artery.