Conditions Treated with Septation and/or Intracardiac Baffles

Septation and/or intracardiac baffles (complex biventricular reconstruction) are used for the following conditions or combinations of conditions:

• Complex outlets, including:

  • Double outlet right ventricle (DORV). DORV is a rare congenital heart defect where the pulmonary artery and the aorta both come from the right side of the heart, or ventricle, rather than the aorta coming from the left ventricle. Some complex forms of DORV are very difficult to surgically separate into two pumps, each with its own outflow, as in the normal heart.
  • Other conotruncal defects (structural defects of the outflow tract) with remote ventricular septal defect (VSD). In VSD, a common heart defect, there is a hole in the wall that separates the left and right ventricles. However, when a VSD is remote, it is not near a major artery and can often make separating the blood flow of the right and left sides of the heart challenging.

• Complex inlets, including:

  • Straddling tricuspid valve. A rare heart defect where the underlying structure of the tricuspid valve (the valve that connects the upper and lower right chambers of the heart) straddles, or goes through, a ventricular septal defect (VSD).
  • Straddling mitral valve. A rare condition where the underlying structure of the mitral valve (the valve that connects the upper and lower left chambers of the heart) straddles, or goes through, a ventricular septal defect (VSD). While many heart centers believe that having a straddling valve automatically disqualifies a child from receiving biventricular reconstruction, we do not.
  • Supero-inferior ventricles and crisscross heart. Extremely rare heart defects where the ventricles become rotated during fetal development, which can cause certain parts of the heart to form incorrectly or to be connected to each other in an abnormal way.

• Heterotaxy syndrome. A rare, complex genetic condition that occurs while a baby is developing in the womb. The syndrome can cause organs throughout the body to form incorrectly, including the heart. Heart defects are often very complex. Sometimes the heart and blood vessels are in the wrong positions within the chest cavity, among other problems.

• Conversion from single ventricle or Fontan. Some babies are born with such complex heart disease that it is difficult to repair the heart and create the normal two pumps or ventricles. Through a series of surgeries ending with the Fontan surgery, the single pump is assigned to pump to the body, leaving no pump to the lungs. While this solution works for a while, it isn’t sustainable over a lifetime, and most single ventricle heart patients eventually need a heart transplant. By reconfiguring the heart via septation, we create two pumps and help avoid the complications of a single ventricle heart. If you’ve been told your child must have a single ventricle palliation (surgery), come see us for a second opinion. We can at times convert hearts back from single ventricle palliation/Fontan. Having two functional ventricles instead of one can dramatically change the trajectory of an individual’s life.