title: Practices and Attitudes of Canadian Cardiologists Caring for Patients With Trisomy 18.
abstract: Trisomy 18 (T18) is a genetic disorder with cardiac lesions in up to 90% of patients. Cardiac surgery is not frequently offered because of the overall poor prognosis, although this has recently been challenged. Our study aimed to explore the practices and attitudes of Canadian pediatric cardiologists managing T18 patients. We administered a survey to pediatric cardiologists attending the Canadian Cardiovascular Congress, Canadian Pediatric Cardiology Association Business Meeting. There were 30 respondents. Most (67%) supported comfort care for affected patients with a heart lesion. None supported palliative surgery for those with complex heart lesions. Of 30 respondents, 16 (53%) counsel families prenatally, and none would present the option of a single ventricle surgical track for complex heart disease. In a hypothetical situation in which their own child was born with T18, 67% would choose comfort care with medical treatment of heart failure, and none would choose palliative surgery. Being a parent was associated with a higher likelihood of choosing termination (14 of 20 vs 6 of 9; P = 0.046) or comfort care (14 of 20 vs 6 of 9; P = 0.036). Qualitative data suggest support for comfort care, while recognizing the need for individualization and shared decision-making, within the context of institution-specific policies. Canadian pediatric cardiologists surveyed support comfort care and medical treatment but not surgical treatment for T18 patients with cardiac lesions. They place primacy on nonmaleficence, yet also recognize the emerging need for individualized shared decision-making in these cases.
authors.affiliation: IWK Health Centre, Halifax, Nova Scotia, Canada; Department of Pediatrics, Dalhousie University, Halifax, Nova Scotia, Canada. Electronic address: aaporter@dal.ca.
authors.forename: Aisling A
authors.initials: AA
authors.lastname: Young
authors.affiliation: Department of Bioethics, Dalhousie University, Halifax, Nova Scotia, Canada.
authors.forename: Christy
authors.initials: C
authors.lastname: Simpson
authors.affiliation: IWK Health Centre, Halifax, Nova Scotia, Canada; Department of Pediatrics, Dalhousie University, Halifax, Nova Scotia, Canada.
authors.forename: Andrew E
authors.initials: AE
authors.lastname: Warren
mesh_terms: D000328:Adult; D001291:Attitude of Health Personnel; D002309:Cardiology / Q000379:methods*; D002675:Child, Preschool; D025063:Chromosome Disorders / Q000235:genetics / Q000628:therapy*; D002887:Chromosomes, Human, Pair 18; D005260:Female; D006330:Heart Defects, Congenital / Q000235:genetics / Q000628:therapy*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D010818:Practice Patterns, Physicians'; D011795:Surveys and Questionnaires