Surgery for congenital
heart defects in
children and adults

 
Dr. med. Eugen Sandica - Oberarzt
Our experienced team of congenital heart surgeons, anesthesiologists and perfusionists working together with cardiologists is able to manage all the congenital heart defects in newborns, infants, children as well as in adults. In our statistic the majority of the procedures (more than 75%) are open heart surgeries involving the use of heart-lung machine. About 52% represent children with congenital heart defects younger than 1 year of age and 23 % are neonates. The adults with congenital heart defects represent about 5% from our surgical population. The mean Aristotel score is 7,26.

Congenital heart defects are encountered in about 1% from the newborns. These can be simple defects that are tolerable quit a long time until they become clinically evident or contrary, can be extremely severe leading to death soon after birth (about 25% during the first month) or during the first year of life (about 60%). Late correction in the survival group has also consequences for the children in terms of heart failure, organs dysfunction and neurological sequels from long lasting cyanosis. That is why our policy is to correct rather to palliate the congenital heart diseases as soon as possible early in life, so most of the children with congenital heart defects will reach the adult age.

Neonatal cardiac surgery

Here there are some of congenital heart defects that we operate on soon after birth:

  • transposition of the great vessels
  • hypoplastic left heart syndrome
  • total anomalous pulmonary venous connection
  • aortopulmonary window
  • interrupted aortic arch with/without VSD
  • critical valvular aortic stenosis nonamendable by interventional procedures
  • truncus arteriosus
  • pulmonary atresia/critical pulmonary valve stenosis with intact ventricular septum
  • tricuspid atresia
  • congenital heart defects with univentricular physiology
  • anomalous origin of the pulmonary artery branches from the ascending aorta
  • severe Ebstein disease
  • critical aortic coarctation
  • persistent ductus arteriosus in pre-term neonates

Pediatric cardiac surgery

In the first months of life we performed elective surgery for

  • ventricular septal defects
  • atrio-ventricular septal defects
  • cor triatriatum
  • anomalous origin of the coronary arteries from the pulmonary artery
  • vascular rings and slings
  • aorta - left ventricle tunnel
  • tetralogy of Fallot
  • double oulet right ventricle
  • cavo-pulmonary anastomosis as an interim stage for Fontan circulation
  • unifocalisation for pulmonary atresia with VSD and major aortopulmonary collateral arteries

At the right time, patient with functionally univentricular heart will get the final surgery to complete the Fontan circulation either with an extracardiac conduit or with an intracardiac lateral tunnel.

Most of the children with atrial septal defect, persistent ductus arteriosus, aortic coarctation, aortic valve stenosis, pulmonary valve stenosis can be successfully treated in our center by interventional cardiology. Only those patients that are not good candidates for such an alternative will become candidates for surgery.

Improved strategies for extracorporeal circulation and myocardial protection

Taking into consideration the deleterious effect of heart-lung machine especially in small neonates that are subject of complex congenital heart defects repair soon after birth, we use special strategies to provide a good chance for recovery after open heart surgery. We avoid every unnecessary deep hypotermic circulatory arrest using instead “low flow” of selected cerebral perfusion for surgery for hypoplastic left heart syndrome as well as for aortic arch reconstruction. This provide a very good chance for no neurological complication after open heart surgery. A good myocardial protection with blood cardioplegia allows for a quick recovery of the heart function after intracradiac defects repair.

Right ventricular outflow tract reconstruction

We have a very good experience with outflow-tract reconstruction of the right ventricle using Contegra Conduit as well as homografts. The rate of reoperation is very low and is depending mostly on the growth-rate of the child. The function of the right ventricle is also a concern for us, so we studied in the animal laboratory the impact of stamm cells injection in the free wall of the right ventricle in an experimental model that reproduced the right ventricular disfunction after transanular patch implantation similar to post-repair in a Fallot patient. The results are incouraging but need futher researches.

Heart valve repair

Congenital anomalies of the heart valve are of special interest for us. Our first choice is to perform valve repair that will assure a normal childhood and adolescence with a later need in life for valve replacement. Autologous pericardial patch is our first choice in valve reconstruction concerning the valve leaflets. We have successfully replaced the leaflets of aortic valve destroyed from primary acute endocarditis in very small children. For children with aortic valve disease non-amendable by valve reconstruction or for those with associated left ventricular outflow tract obstruction we offer the Ross procedure. This allows the child to grow without the need for anticoagulation that is mandatory in case of valve replacement with mechanical prosthesis.

Hybrid therapy

We developed a very successful hybrid approach for complex congenital heart defects (hypoplastic left heart syndrome, unifocalisation, outflow tract reconstruction of the right ventricle combined with stenosis of pulmonary arteries, etc). This means that the patient will receive a combined therapy, part interventional cardiology, part heart surgery in an effort to optimize the result by decreasing the risk of the procedure.

Open heart surgery in very-low weight pre-term neonates

We have performed open heart surgery in very small pre-term neonates. We successfully operated on a 660 g pre-term baby with a calcified thrombus inside the right and left atrium. The thrombus was removed without heart-lung machine, using the in-flow occlusion technique.

Pace-maker implantation

Depending on patient size and underlying disease we offer trans-venous as well as epimyocardial pace-maker implantation for patients with heart rhythm disturbances.

Mechanical circulatory support

As mechanical circulatory support we can provide ECMO for patients with or without congenital heart disease that experience acute myocardial failure.

Adult congenital heart disease (GUCH )

The adults with congenital heart defects represent a distinct population. Some of them present with noncorected defects like atrial septal defects, small ventricular septal defects, mild form of tetralogy of Fallot, coarctation of the aorta, aortic valve stenosis due to a bicuspid aortic valve. Others have been operated in infancy or in early childhood for tetralogy of Fallot, transposition of the great vessels (arterial switch, Mustard / Senning technique), single ventricle physiology (Fontan operation) or have received a prosthetic valve that became too small regarding the patient’s size. For those with primary defects not repaired early in life it is important to assess if the defects can be still corrected. For these purpose the patient will be very well investigated so surgery can proceed with a very low risk. Most of the patient that have been operated early in life for complex heart defects and have reached the adult age, are coming for replacement of the biological conduits, stenosis of pulmonary arteries, need for prosthetic valve replacement, failing Fontan circulation, failing Mustard or Senning correction for transposition of the great arteries. We are able to provide for all these patients surgical as well as hybrid therapy alternatives to improve their quality of life:

  • closure of atrial septal defect
  • closure of ventricular septal defect
  • tetralogy of Fallot correction
  • surgery for aortic coarctation
  • aortic valve replacement with / without aortic root enlargement
  • replacement of biological conduits used for right ventricular outflow tract reconstruction
  • hybrid therapy for right ventricular outflow tract reconstruction after Fallot repair in childhood
  • conversion of the failing Fontan (atriopulmonary connection) to a total cavopulmonary connection (lateral tunnel)

 

 

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