Advances in Anatomical Repair of Congenital Heart Diseases
from Practice Update, Winter 2004
Left Ventricular Growth at
Pre-op
Already at the national forefront for pediatric heart transplantation, with the establishment of The Children’s Hospital Heart Institute, treatment options for congenital heart disease have expanded over the last year. Advance echocardiography allows non-invasive diagnosis of many cardiac anomalies during fetal life or immediately after birth. The introduction of cardiac magnetic resonance imaging (MRI) permits very precise anatomical diagnoses. In the newly expanded catheterization laboratory, advanced interventional cardiology procedures are correcting many different anomalies without need of surgery including:
- ASD device closure
- Aortic and pulmonary stenoses
- Pulmonary atresia with intact septum opening
- Pulmonary branch stenoses dilation and stenting
- Palliation of HLHS by pulmonary artery branches device banding and ductus arteriosus stenting
- Potentially in the future, completion of Fontan procedure
The Children’s Hospital offers transplantation, staged Norwood procedures, options for biventricular repairs, pulmonary autografts, minimally invasive surgical techniques and neonatal repairs in low birth weight babies. Specialized pediatric mechanical support, including ECMO, enable surgical treatment of complex congenital heart disease. Advances in anesthesia, perfusion, surgical technique, perioperative care and a dedicated cardiac intensive care unit have contributed to our ability to successfully palliate and treat complex conditions in children and young adults.
Transplantation offers a new, fully-functioning heart. However, a limited supply of transplantable hearts, a life-long regimen of immunosuppressive medications and the potential need for retransplantation can make this choice contraindicative for some young patients.
Hypoplastic Left Heart Syndrome (HLHS) and variants, the most common congenital cardiac lesions causing death within the first year after birth, is typically treated with the Norwood procedure, biventricular repair or orthotopic cardiac transplantation. With the arrival of Dr. Lacour-Gayet, The Children’s Hospital Heart Institute routinely offers the Norwood as an alternative to transplantation.
François Lacour-Gayet, MD, one of the world’s leading surgeons in the Norwood procedure, biventricular repair and arterial switch, has substantially increased the number of complex heart surgeries at Children’s from 2002 to 2003. Total procedures reached 450 in 2003. Dr. Lacour-Gayet explains advances for treating heart disease in neonates, “Outcomes, both surgical and overall, have continued to improve. Our ability to operate on smaller and smaller infants increases steadily. We can now do repairs on infants down to two kilos in weight. These low-birth weight babies, many of whom could not be saved just a few years ago, are now not only savable, but can look forward to an improved quality of life. At Children’s, we have some of the country’s leading programs for pulmonary hypertension and interventional cardiology, which contribute to our success in surgically treating complex conditions, such as hypoplastic left heart. We also have the region’s only pediatric ventricular assist device. Our cardiac staff, from surgeons and cardiologists to nurses, perfusionists and anesthesiologists, are some of the most experienced in the country.”
Left Ventricular Growth 14 Days Post
Roth
Lacour-Gayet continues, outlining some of newest trends in treating congenital heart disease, “While some procedures, such as the Norwood, necessarily are staged, we are moving more to one-time repair surgeries in which multiple defects are addressed in one operation. Many children are slow to thrive between staged procedures. A single operation can substantially promote a child’s growth and development. Another new development in biventricular repair is our ability to promote ventricular growth by redirecting flow through the hypoplastic ventricle. This increased flow promotes growth and enables us to ultimately do a biventricular repair. Since two ventricles are more efficient than a single ventricle, this development results in more favorable overall outcomes.”