Hybrid Management for Hypoplastic Left Heart Syndrome

Abstract

Initial surgical reconstruction for hypoplastic left heart syndrome (HLHS) is associated with satisfactory outcomes only in a few referral centers. Moreover, there is a persistent high-risk period for sudden death while the patient waits for the next surgical procedure. The development of a less invasive approach, so-called "hybrid," postponing a major surgery outside the neonatal period, might reduce the immediate and late surgical burden on these patients. This is a retrospective study of a contemporary series of patients with HLHS seen in two separate institutions. Patients with HLHS or its variants who underwent a "hybrid" management were included in the study. Data are described as the mean and standard deviation or absolute numbers and percentage, as appropriate. From January 2004 to June 2006, 15 patients (10 male; 5 +/- 3.8 days old and 2.9 +/- 0.5 kg) were included in the study. Ten had both mitral and aortic atresia; the ascending aorta and atrial septal defect measured 2.5 +/- 1.4 and 4.9 +/- 1.2 mm, respectively. There were six hospital survivors after stage I (mortality rate 60%). During the interstage period, all but one patient needed additional procedures. One patient died of bacterial meningitis 4 months after stage I. Four patients were submitted to stage II operation at 6.6 +/- 0.5 months of age and one is waiting for the operation. All four required early reinterventions for pulmonary artery stenosis. Only one was discharged home and was not yet submitted to the third stage. The hybrid approach for HLHS was associated with poor results in this early experience from two independent institutions in a developing country. This might have been related to infrastructure and technical problems, as well as our own learning curve. Institutions working under the same conditions might face similar problems during their initial experience.

Paediatric and congenital heart disease in South America: an overview

“…Thus, a hybrid approach with banding of the pulmonary arteries and ductal stenting has emerged in some centres as an alternative strategy 72. Adjustable pulmonary artery banding has been suggested as a better means of customising pulmonary blood flow for these rapidly growing infants 73.…”

“…Adjustable pulmonary artery banding has been suggested as a better means of customising pulmonary blood flow for these rapidly growing infants 73. However, initial experience with the hybrid approach has also failed to demonstrate a significant improvement in the outcomes 72. Although the mortality rate of phase I did improve, the need for additional procedures was high, especially procedures to enlarge the ASD.…”

Early Results of Neurodevelopment Following Hybrid Stage I for Hypoplastic Left Heart Syndrome

“…Hybrid Stage I as the initial palliation for HLHS has been previously reported [1,2,4,11,12,32]. Very little is known about neurodevelopment in babies born with HLHS who have undergone Hybrid Stage I palliation, as opposed to those who have undergone the traditional Stage I Norwood or Norwood-Sano procedure where neurodevelopmental outcomes have been assessed from infancy through school age and have shown delay and sub-optimal performance [14, 16, 18-26, 28, 29, 36, 37, 39, 40].…”

Anesthetic management of the hybrid stage 1 procedure for hypoplastic left heart syndrome (HLHS)

“…At our institution, the development of the hybrid procedure has been pioneered and is now our preferred approach for the management of patients presenting with HLHS (6–9). Herein, we present a retrospective review of our experience anesthetizing 77 patients undergoing a stage 1 hybrid procedure.…”