Survival after reconstructive surgery for hypoplastic left heart syndrome: A 15-year experience from a single institution. Mahle, W., Spray, T., Wernovsky, G, Gaynor, J., & Clark, B. Circulation, 102(19 Suppl 3):III136–III141, November, 2000.
Survival after reconstructive surgery for hypoplastic left heart syndrome: A 15-year experience from a single institution. [link]Paper  abstract   bibtex   
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:\textless0.001). 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 \textgreater14 days at stage I and weight \textless2.5 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.
@article{mahle_survival_2000,
	title = {Survival after reconstructive surgery for hypoplastic left heart syndrome: {A} 15-year experience from a single institution.},
	volume = {102},
	url = {https://www.ncbi.nlm.nih.gov/pubmed/11082376},
	abstract = {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:{\textless}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 {\textgreater}14 days at stage I and weight {\textless}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.},
	language = {eng},
	number = {19 Suppl 3},
	journal = {Circulation},
	author = {Mahle, WT and Spray, TL and Wernovsky, G and Gaynor, JW and Clark, BJ},
	month = nov,
	year = {2000},
	keywords = {Treatment Outcome},
	pages = {III136--III141}
}

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