Spring 2010

Surgical Innovations Raise Prospects for Children with Congenital Heart Disease

Anthony Azakie, M.D."More advanced technology, more innovative techniques and deep surgical experience all contribute to many children and adults with congenital heart disease living longer and better lives than they have in the past," says Anthony Azakie, M.D., chief of Pediatric Cardiothoracic Surgery at UCSF Benioff Children's Hospital.

The program at UCSF is a good example of just how successful this combination has become. A recent, independent review of 1,000 cases ranging from neonates to adults — including those with defects associated with high risk and high mortality rates — found that the entire program has a 99 percent survival rate. Older children and adults are included in the specialized service because many people with congenital heart disease now survive into adulthood, but often need surgery, either because the problem has long gone untreated or because prior procedures are failing.

Petro Anagnostopoulos, M.D."The only way to achieve the kind of success we've had is with a multidisciplinary team — from anesthesia and nursing to critical care, cardiology and the surgeons — working in
a synchronized and knowledgeable way,"
says Petros Anagnostopoulos, M.D.,
Azakie's colleague in UCSF Pediatric Cardiothoracic Surgery.

Hybrid Procedures

Among the procedures that require multidisciplinary expertise is a hybrid stage 1 (Norwood) procedure, the first of three surgeries used for repair of hypoplastic left heart syndrome. The hybrid version avoids the cardiac and neurological risks of bypass.

After a surgeon makes an incision in the sternum, he or she places Gore-Tex bands on branches of the pulmonary artery to modulate blood flow to the lungs. The surgeon then introduces a catheter directly into the pulmonary artery, while the interventional cardiologist places a stent in the ductus arteriosus to ensure that it remains open.

"We have had greater than 90 percent stage 1 survival for hypoplastic left heart syndrome," says Azakie. "The addition of the hybrid procedure is a complementary approach, and we also are looking at an off-pump Glenn and Fontan because we can use this for palliation of any single ventricle defect."

Similarly, for the more common tetralogy of Fallot, surgeons at UCSF have created a hybrid technique that, while it still requires bypass, enables pulmonary valve insertion in high-risk patients. "We close the hole and open the right ventricle in a way that preserves or reconstructs the valve," says Anagnostopoulos. "The procedure leads to shorter postop, less pulmonary insufficiency and shorter length of stay."

Sutureless Repairs, Cerebral Monitoring

Other important advances also have improved morbidity and mortality rates for congenital heart disease.

  • Primary treatment of pulmonary vein stenosis through sutureless repair and creation of a marsupial sac in the left atrium decreases risks, compared with more traditional techniques. The same is true for use of minimally invasive techniques for atrial septum defect.
  • Improved prenatal diagnoses have helped surgeons better address low-birthweight babies with congenital heart disease. "We have better than 90 percent survival for complex heart disease in low-birthweight babies," says Azakie.
  • Constant cerebral monitoring during neonatal heart surgery, combined with selective cerebral perfusion, enables the surgical team to optimize monitoring for oxygenation protection before, during and after surgery. The improved monitoring appears to have lessened neurological complications. "We think this is a trend that will improve neuro outcomes," says Azakie.

For more information, contact Dr. Azakie or Dr. Anagnostopoulos at (415) 476–3501.


Spring 2010 Table of Contents

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