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Hypoplastic left heart syndrome: factors influencing therapeutic choice
C Corrow1, S Lapuk, K Mazzarella
1University of Connecticut School of Medicine, Farmington, USA.
Insights
Parental decisions for infants with hypoplastic left heart syndrome (HLHS) are challenging. While operative reconstruction survival rates are low, more parents opted for surgery over time, influenced by physician optimism and concerns about infant suffering.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Neonatal Surgery
Background:
- Hypoplastic left heart syndrome (HLHS) presents complex therapeutic dilemmas for parents.
- Management options include surgical reconstruction, cardiac transplantation, or comfort care only.
- Parental decision-making is influenced by perceived survival rates, quality of life, and potential infant suffering.
Purpose of the Study:
- To retrospectively analyze clinical features and parental choices for infants with HLHS.
- To understand the factors influencing parental decisions between operative and comfort care management.
- To identify trends in therapeutic option selection over time.
Main Methods:
- Retrospective review of medical records for 47 HLHS patients (1989-1999).
- Database creation of clinical features for infants undergoing operation or comfort care.
- Structured interviews with 11 families regarding their experiences and decision-making rationale.
Main Results:
- Of 38 liveborn infants, 53% chose comfort care, while 47% underwent operations.
- Survival beyond five months for operated infants was 47%, with 29% alive at follow-up (1-4.5 years).
- An increasing proportion of parents chose operative reconstruction from 1989-1995 (37%) to 1996-1999 (73%).
Conclusions:
- Parental choices for HLHS management are influenced by survival expectations, quality of life concerns, and physician input.
- Supportive resources like written materials, counseling, and family support groups are recommended.
- Further research into optimizing decision-making support for HLHS families is warranted.
Abstract:
Parent's choices among therapeutic options for their infants born with hypoplastic left heart syndrome are difficult and controversial. Currently, management options include surgical reconstruction, cardiac transplantation, and comfort measures only. We retrospectively reviewed medical records of 47 patients (1989-1999) to create a database of clinical features of infants who received either an operation or comfort care only. Eleven families were interviewed by means of a structured questionnaire pertaining to their experience and reasons for their choice. Of the 47, 20 were prenatally diagnosed and nine of these (45%) aborted. The remaining 38 of the 47 were liveborns. Of the 38, 20/38 (53%) chose comfort care only. The other 18 chose operation. Although 17 were able to survive until first stage repair, only 8/17 (47%) survived beyond five months. At the time of last contact (ages one to 4.5 years), 5/17 (29%) remained alive. Over the nine years an increasing proportion of parents chose operative reconstruction; 8/11 (73%) for 1996-99 vs 10/27 (37%) for 1989-1995. Interviewed families who chose comfort care were more likely to believe the rate of survival following operation was poor, quality of life was diminished, and seemed concerned that their infant would suffer. Influence by optimistic physicians at surgical centers seemed important for an operative choice. Most suggested that provision of written materials, professional family counseling, and support groups of hypoplastic left heart syndrome families are or would be helpful.