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Patient outcomes and decision making in the treatment of hypoplastic left heart syndrome.

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Call No.	RC687 .J46 1999
Author	Jenkins, Pamela Cullie
Title	Patient outcomes and decision making in the treatment of hypoplastic left heart syndrome.
Date	1999
Abstract	Hypoplastic left heart syndrome (HLHS) is a heart malformation that requires surgical intervention for survival. Without surgery, 95% of neonates with HLHS die in the first month of life. Two surgical approaches are available, a series of palliative surgeries and heart transplantation, but their outcomes have not been compared thoroughly. The objective of the research was to identify survival rates, risk factors for mortality, and optimal choices for treatment in patients with HLHS. This research analyzed 221 cases from 1989-1994 at four pediatric cardiac surgery centers. In Study 1, five-year survival outcomes for staged surgery and transplantation were explored. The highest mortality occurred in the first year for both approaches, and longer-term mortality was minimal by comparison. Transplantation produced higher survival at all times studied. In Study 2, a multiple logistic regression model for mortality risk in the first year was developed. Risk factors for all babies with HLHS were birth weight <3 kg, creatinine level ≥2 mg/dL, and atrial septostomy. Infants with atresia of one or both left-sided valves were at a higher mortality risk in staged surgery than in transplantation. In Study 3, decision analysis was used to compare one-year survival outcomes of specific treatment paths. The optimal choice varied with probabilities of obtaining a transplantation in three months and of stage 1 mortality. Waiting indefinitely for transplantation and performing stage 1 surgery as an interim to transplantation were not optimal paths under realistic assumptions. Under baseline assumptions, waiting two months for transplantation before proceeding to stage 1 surgery optimized survival. These results can inform decision-making for patients with HLHS. Parents and clinicians may estimate the individual mortality risk, and with center-specific infant organ donation rates, determine the optimal treatment path. Pediatric cardiac surgeons may more securely compare surtival outcomes by adjusting for known risk factors. These results imply that patients with valve atresia should receive transplants in preference to patients with mitral and aortic stenosis. Public policy and ethical issues on organ availability influence the treatment decision; xenografts, comfort care, and the regionalization of complex pediatric surgical care are issues which need attention.
Description	1 online resource (xiii, 130 leaves) : illustrations (some color)
Format	application/pdf
Advisor	'OConnor, Gerald T.'
Subject	Congenital heart disease in children -- Surgery -- Evaluation. ; Heart -- Abnormalities -- Surgery -- Evaluation. ; Outcome assessment (Medical care).
College	Dartmouth Medical School -- Center for the Evaluative Clinical Sciences.
Issuing Body	Dartmouth Dissertations are made available through the Dartmouth Digital Library.
Degree	Thesis (Ph.D)--Dartmouth College.
Copyright	In Copyright. This material is protected by copyright and/or related rights. You are free to use this material in any way that is permitted by copyright law that applies to your use. For other uses, you need to obtain permission from the rights-holder(s). http://rightsstatements.org/page/InC/1.0/
DOI	https://doi.org/10.1349/ddlp.2768

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