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Published on: July 18, 2014
Outcomes of cardiac surgery in trisomy 18 patients
Jun Muneuchi1, Junko Yamamoto, Yasuhiko Takahashi
1Department of Pediatrics, Kyushu Koseinenkin Hospital, Kishinoura, Yahatanishi-ku, Kitakyushu, Fukuoka, Japan. mune@pediatr.med.kyushu-u.ac.jp
Insights
Cardiac surgery in trisomy 18 patients improved survival rates but did not increase the likelihood of being discharged alive. Careful individualization of surgical indications is crucial for improving quality of life in these patients.
Area of Science:
- Cardiology
- Genetics
- Pediatric Surgery
Background:
- Trisomy 18 (Edwards syndrome) is a genetic disorder associated with severe congenital heart defects.
- Outcomes for trisomy 18 patients with cardiac complications are often poor, necessitating evaluation of treatment strategies.
Purpose of the Study:
- To investigate the impact of cardiac surgery on survival and discharge rates in patients with trisomy 18.
- To identify factors influencing outcomes in this patient population.
Main Methods:
- A retrospective analysis of 34 trisomy 18 patients with cardiac complications was performed.
- Patients were categorized into surgical and conservative treatment groups.
- Survival and alive discharge rates were compared between the two groups.
Main Results:
- Cardiac surgery was performed on nine patients, with procedures including intracardiac repair, pulmonary arterial banding, and ductus ligation.
- The surgery group demonstrated significantly higher cumulative survival rates at 1, 6, 12, and 24 months compared to the conservative group (p = 0.002).
- However, there was no significant difference in the cumulative rates of discharge alive between the surgical and conservative groups.
Conclusions:
- Cardiac surgery in trisomy 18 patients is associated with improved survival but not with a higher rate of discharge alive.
- Hazardous factors for mortality included cardiac surgery itself and pre-operative assisted ventilation.
- Individualized assessment for cardiac surgery is essential to enhance the quality of life for trisomy 18 patients.
Objective:
The objective was to clarify the outcomes of cardiac surgery in trisomy 18 patients.
Patients And Methods:
We analysed 34 consecutive trisomy 18 patients, of whom 21 were males, with cardiac complications. They were divided into patients who underwent cardiac surgery and those who were conservatively treated. We compared rates of survival and discharge alive between two groups.
Results:
The surgery group included nine patients, with six males, who underwent cardiac surgery - intracardiac repair in three patients, pulmonary arterial banding in five patients, and ligation of the ductus in one patient - at median age of 2.2 months, ranging from 0.5 to 9.8, and with median weight of 2.6 kilograms, ranging from 1.5 to 3.2. Cardiac surgery and pre-operative assisted ventilation were hazardous factors leading to death. In the surgery group, cumulative survival rates at 1 month, 6 months, 12 months, and 24 months were 63%, 38%, 25%, and 22%, respectively, compared with 51%, 26%, 9%, and 9% in the conservative group. There was a significant difference (p = 0.002). The cumulative rates of discharge alive at 1 month, 3 months, and 6 months were 0%, 12%, and 65% in the surgery group, which did not differ from the conservative group (p = 0.80).
Conclusions:
Cardiac surgery contributed to increased survival rate but not the rate of discharge alive in trisomy 18 patients. Cardiac surgery could not prevent all the trisomy 18 patients from death. The indication of cardiac surgery should be carefully individualised to improve the quality of life in trisomy 18 patients and concerned surrounding people.
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