New Techniques Assist Patients with Congenital Heart Disease

With new surgical techniques, the outlook for pediatric patients with congenital heart disease is more promising than ever. These “touch-free” techniques can be applied to all forms of congenital heart disease and, because of limited exposure between the surgeon’s hand and heart tissue, have extremely low infection rates (less than 1%). Additionally, the use of robotic platforms, such as robotic telemanipulation, result in an extremely small incision on the patient’s chest and short hospital stays.

With neonates excluded, almost all patients (children and adults) undergoing touch-free congenital heart surgery at California Pacific have had their breathing tubes removed in the operating room prior to intensive care transfer. Children with vascular rings and patent ductus arteriosus (PDA) (Case 1) are frequently discharged within 24 hours of surgery. Patients with lesions requiring the heart-lung machine are frequently discharged home on the second postoperative day. Additionally, by using new adjuvant technologies, repair strategies can be optimized (Case 2).

Case 1: Vascular Ring

Upper respiratory symptoms associated with progressive dysphagia frequently indicate the presence of a vascular ring. Since respiratory symptoms are common in pediatric patients, a thorough work-up including radiological examination and even bronchoscopy may be required when stridor is present to rule out such a congenital anomaly. When dysphagia is the dominant symptom, delayed diagnosis is common.

Our child had been followed for ongoing eating difficulties. Respiratory symptoms were less evident. At 6 years old, the child’s diet was limited to puddings, gelatin and fluids. A chest radiograph was suspicious for a vascular ring and a barium swallow confirmed a right aortic arch, a right descending aorta and an aberrant left subclavian artery. Vascular compression remained secondary to a Kommerell’s diverticulum and a persistent left ductus arteriosus.

Using “touch-free” techniques including a total thorascopic approach via the left chest, we successfully dissected the ductus arteriosus region, the left subclavian artery, the diverticulum and a portion of the right descending aorta. Division of the ductus arteriosus and a partial diverticulectomy allowed for an immediate improvement in ventilation and visualization of the thoracic esophagus. This child was extubated in the operating room and began drinking fluids within hours following the procedure. For the first time in months, coughing and sputtering were absent! Soon after, she successfully ingested solid foods (after much reassurance), also for the first time in months. She was discharged from the hospital less than 24 hours after admission. As evidenced in Figure 1, the child has only a Band-aid®-sized incisions as a reminder of the hospitalization.

Case 2: Congenital Mitral Valve Stenosis

Frequently associated with anatomical aberrations of the heart’s left side, congenital mitral valve stenosis is difficult to treat and, in children, frequently requires a prosthetic valve. The most common associated malformations include: coarctation of the aorta, aortic valve stenosis and subvalvular aortic stenosis. The association of multiple levels of left-sided inflow and outflow tract obstruction is termed the Shone complex.

Our child was diagnosed with a cardiac ailment at approximately 3 years of age. She had evidence of labored breathing which had progressively increased, a decreased appetite and exercise tolerance, and unusual muscle cramping. Attending school was difficult. A physical exam and echocardiogram showed an enlarged left atrium, a small left ventricle, mitral valve stenosis, a bicuspid aortic valve and a grossly enlarged main pulmonary artery with pulmonary hypertension. Medical therapy was initiated with a temporizing effect.

During the initial surgical consultation, the patient was extremely pale and lethargic. She showed signs of weight loss and had a poor appetite. The patient also lacked the energy to talk, play or interact with our team. An urgent operative strategy was planned to address the mitral valve. Review of the valve demonstrated what appeared to be restrictive motion possibly due to a supra-mitral valve ring. In addition, we suspected a single papillary muscle (a parachute mitral valve). Surgery was planned on an urgent basis.

Using “touch-free” techniques including the use of a limited incision and cardioscopy, we were able to successfully remove the supra-mitral valve ring that encased both the anterior and posterior mitral valve leaflets. The use of intra-cardiac imaging, including increased magnification and illumination, was extremely helpful as the mitral valve orifice is limited in size. Following excision, the leaflet mobility appeared normal. To our surprise, there was no real communication between the left atrium and ventricle other than a small fibrotic orifice of 3mm (i.e. a mitral valve arcade was found.) Extremely low cardiac output explained the symptoms noted above, including the muscle cramps. The repair strategy included fenestration of the non-perforated diaphragm and repair of the subsequent mitral valve regurgitation. Following the procedure, the patient was discharged home with an improvement in her color, appetite and energy. The left ventricle now appears to be “filled” and she has increased cardiac output. Although this young child will likely require repeat cardiac surgery, a prosthetic mitral valve can now be delayed. If necessary, a larger prosthetic valve can be inserted.


This information provided by California Pacific Medical Center's Division of Pediatric Cardiovascular Surgery
Michael Black, M.D., chief, pediatric heart surgery
Tel. (415) 600-1KID (1543)
Fax. (415) 600-1245;
Email. blackm@sutterhealth.org