The Front Line

Congenital heart surgery:
New challenges for an evolving specialty

by Charles D. Fraser, Jr., MD
Professor of Congenital Heart Surgery

Dramatic advances in technology and outcomes were seen in the field of congenital cardiac surgery over the last decade. Conditions with previously dismal prognoses are now handled with great success in large-volume centers committed to the care of patients with congenital heart anomalies. At Texas Children’s Hospital, a combination of extensive experience, institutional commitment, and dedicated personnel affords our patients the opportunity of optimum care and every possibility of a quality outcome.

Expectations for children born with complex cardiac malformations continue to improve. Unfortunately, tremendous interinstitutional differences in survival rates remain. The general medical community does not largely recognize this fact and parents can face difficulty obtaining accurate information about an institution’s performance. Peer reviewed medical publications remain the gold standard by which centers should be measured, as well as state-specific outcomes data such as are available in Texas. Parents and practitioners making decisions about patient referrals should be familiar with current performance information.

Many of the straightforward conditions, including patent ductus arteriosus and atrial septal defect, frequently are handled now through catheter-based, interventional approaches. Although they are surgically challenging, even small, premature neonates with complex cardiac malformations often are best treated with prompt, aggressive repair early in life. While these patients have additional physiologic derangements, surgery may be mandatory to avoid an unsalvageable situation. At the other extreme, it is becoming increasingly clear that adults with congenital heart disease requiring surgery often should be treated at a children’s hospital.

Prenatal diagnosis and maternal referral allows early consultation with the surgical, pediatric cardiology, and neonatology teams at Texas Children’s Hospital. This team approach greatly benefits the patient, in many cases avoiding serious perinatal complications. This, in turn, translates into a preoperative patient with the optimum physiologic state at the time of surgery.

At Texas Children’s, surgical survival rates now approach 100% for many previously fatal cardiac malformations, including transposition of the great arteries, interrupted aortic arch, total anomalous pulmonary venous return, and truncus arteriosus. Other lesions, historically associated with nearly uniform mortality, are now treated with ever improving success rates. Hypoplastic left heart syndrome (HLHS) remains the most common condition in newborns with congenital heart disease involving a single ventricle. Using a multidisciplinary approach, the operative mortality for surgical palliation of this difficult and common problem has steadily declined at Texas Children’s, where the operative survival for children undergoing first stage surgical palliation for HLHS during the last three years has been greater than 90%.

For a small subset of patients, no therapy exists except cardiac transplantation. The world’s first pediatric cardiac transplant procedure was done at Texas Children’s, and we have a very large body of experience with this therapy.

Neurodevelopment is an area of intense research in children with congenital heart disease. Patients with cyanotic congenital heart disease palliated with the Fontan operation have been noted historically to have elevated risk of delayed development and decreased ultimate neurocognitive function. The precise etiology of this remains uncertain. The need for heart-lung bypass has been the suspected source of neurologic dysfunction in some. Using novel approaches, including a dedicated pediatric cardiac perfusion team, we monitor cerebral blood flow and brain oxygenation during bypass to precisely regulate perfusion during the entire operation.

Dedicated congenital heart centers, such as ours, will continue to lead the way in advances in therapy and patient outcomes.