Transplantation is the best treatment for hypoplastic left heart syndrome

2004 ◽  
Vol 14 (S1) ◽  
pp. 109-111 ◽  
Author(s):  
Leonard L. Bailey

Thank you for asking me to make the case for cardiac transplantation in this debate. Marshall, that was a truly comprehensive and spectacular defense of the value of reconstructive surgery in babies born with hypoplastic left heart syndrome. It is clear that the potential for babies born with this complex and lethal malformation has improved dramatically over the past 2 decades. The improvement in outcome is a direct result of complementary surgical strategies. Marshall Jacobs has constructed a stellar review outlining the important issues relating to the ever-evolving staged palliative reconstruction for hypoplastic left heart syndrome. And, he appealed to those present at the Symposium with some images of extraordinary children who represent the most favorable outcomes seen today after staged reconstructive surgery. It would be inappropriate for me to criticize or condemn any feature of these favorable outcomes.

2004 ◽  
Vol 14 (S1) ◽  
pp. 105-108 ◽  
Author(s):  
Marshall L. Jacobs

I am grateful for the opportunity to participate in this debate forum, and for the honor of exchanging concepts with Len Bailey. This debate, fortunately, represents a “win-win” situation. The innovations and evolution of both replacement of the heart and reconstructive surgery for hypoplastic left heart syndrome are stories with lots of winners. The entire field of transplantation biology, the entire scope of management of the newborn with complex congenital heart disease, including both medical and supportive therapy and surgical strategies, has each been advanced tremendously as a consequence of the introduction and refinement of these two very different therapies for hypoplastic left heart syndrome. So, it is a debate that cannot be lost.


Circulation ◽  
2000 ◽  
Vol 102 (suppl_3) ◽  
Author(s):  
William T. Mahle ◽  
Thomas L. Spray ◽  
Gil Wernovsky ◽  
J. William Gaynor ◽  
Bernard J. Clark

Background —There are limited data regarding the long-term survival of patients who have undergone reconstructive surgery for hypoplastic left heart syndrome (HLHS). We reviewed the 15-year experience at our institution to examine survival in the context of continued improvements in early operative results. Methods and Results —Between 1984 and 1999, 840 patients underwent stage I surgery for HLHS. From review of medical records and direct patient contact, survival status was determined. The 1-, 2-, 5-, 10-, and 15-year survival for the entire cohort was 51%, 43%, 40%, 39%, and 39%, respectively. Late death occurred in 14 of the 291 patients discharged to home after the Fontan procedure, although only 1 patient has died beyond 5 years of age. Heart transplantation after stage I reconstruction was performed in 5 patients. Later era of stage I surgery was associated with significantly improved survival ( P <0.001). Three-year survival for patients undergoing stage I reconstruction from 1995 to 1998 was 66% versus 28% for those patients undergoing surgery from 1984 to 1988. Age >14 days at stage I and weight <2.5 kg at stage I were also associated with higher mortality ( P =0.004 and P =0.01, respectively). Other variables, including anatomic subtype, heterotaxia, and age at subsequent staging procedures, were not associated with survival. Conclusions —Over the 15-year course of this study, early- and intermediate-term survival for patients with HLHS undergoing staged palliation increased significantly. Late death and the need for cardiac transplantation were uncommon.


2011 ◽  
Vol 21 (S2) ◽  
pp. 38-46 ◽  
Author(s):  
Stephanie Fuller ◽  
Nancy Ghanayem ◽  
Paul Chai ◽  
John Nigro ◽  
Thomas Kleisli ◽  
...  

AbstractThe management of newborns with hypoplastic left heart syndrome has changed significantly over the past three decades, with an associated dramatic improvement in outcomes. The aim of this paper is to discuss current peri-operative and palliative surgical strategies. Owing to the fact that comparative outcomes for these strategies have been addressed in a limited number of prospective trials and extractions from multi-centred databases, the primary focus of this review is descriptive.


Circulation ◽  
2000 ◽  
Vol 102 (Supplement 3) ◽  
pp. III-136-III-141 ◽  
Author(s):  
W. T. Mahle ◽  
T. L. Spray ◽  
G. Wernovsky ◽  
J. W. Gaynor ◽  
B. J. Clark

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