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Impact of Left Ventricular Morphology on Adverse Outcomes Following Stage 1 Palliation for Hypoplastic Left Heart Syndrome: 20 Years of National Data From Sweden
Cardiology, Pediatric Heart Centre, Skåne University Hospital, Lund, Sweden; Pediatrics, Department of Clinical Sciences, Lund University, Lund, Sweden.
Department of Pediatrics, Institute of Clinical Sciences, Sahlgrenska Academy, Gothenburg, Sweden; Children´s Heart Centre, Sahlgrenska University Hospital, Gothenburg, Sweden.
Cardiology, Pediatric Heart Centre, Skåne University Hospital, Lund, Sweden; Pediatrics, Department of Clinical Sciences, Lund University, Lund, Sweden.
Pediatrics, Department of Clinical Sciences, Lund University, Lund, Sweden; Cardiac Surgery, Pediatric Heart Centre, Skåne University Hospital, Lund, Sweden.
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2022 (English)In: Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease, ISSN 2047-9980, E-ISSN 2047-9980, Vol. 11, no 7, article id e022929Article in journal (Refereed) Published
Abstract [en]

BACKGROUND: Hypoplastic left heart syndrome is associated with significant morbidity and mortality. We aimed to assess the influence of left ventricular morphology and choice of shunt on adverse outcome in patients with hypoplastic left heart syndrome and stage 1 palliation.

METHODS AND RESULTS: This was a retrospective analysis of patients with hypoplastic left heart syndrome with stage 1 palliation between 1999 and 2018 in Sweden. Patients (n=167) were grouped based on the anatomic subtypes aortic-mitral atresia, aortic atresia-mitral stenosis (AA-MS), and aortic-mitral stenosis. The left ventricular phenotypes including globular left ventricle (Glob-LV), miniaturized and slit-like left ventricle (LV), and the incidence of major adverse events (MAEs) including mortality were assessed. The overall mortality and MAEs were 31% and 41%, respectively. AA-MS (35%) was associated with both mortality (all other subtypes versus AA-MS: interstage-I: hazard ratio [HR], 2.7; P=0.006; overall: HR, 2.2; P=0.005) and MAEs (HR, 2.4; P=0.0009). Glob-LV (57%), noticed in all patients with AA-MS, 61% of patients with aortic stenosis-mitral stenosis, and 19% of patients with aortic atresia-mitral atresia, was associated with both mortality (all other left ventricular phenotypes versus Glob-LV: interstage-I: HR, 4.5; P=0.004; overall: HR, 3.4; P=0.0007) and MAEs (HR, 2.7; P=0.0007). There was no difference in mortality and MAEs between patients with AA-MS and without AA-MS with Glob-LV (P>0.15). Patients with AA-MS (35%) or Glob-LV (38%) palliated with a Blalock-Taussig shunt had higher overall mortality compared with those palliated with Sano shunts, irrespective of the stage 1 palliation year (AA-MS: HR, 2.6; P=0.04; Glob-LV: HR, 2.1; P=0.03).

CONCLUSIONS: Glob-LV and AA-MS are independent morphological risk factors for adverse short-and long-term outcome, especially if a Blalock-Taussig shunt is used as part of stage 1 palliation. These findings are important for the clinical management of patients with hypoplastic left heart syndrome.

Place, publisher, year, edition, pages
John Wiley & Sons, 2022. Vol. 11, no 7, article id e022929
Keywords [en]
adverse outcome, aortic atresia-mitral stenosis, globular left ventricle, hypoplastic left heart, left ventricular morphology
National Category
Cardiac and Cardiovascular Systems
Identifiers
URN: urn:nbn:se:umu:diva-194335DOI: 10.1161/JAHA.121.022929ISI: 000778262600041PubMedID: 35348003Scopus ID: 2-s2.0-85128245385OAI: oai:DiVA.org:umu-194335DiVA, id: diva2:1655928
Funder
Swedish Heart Lung FoundationAvailable from: 2022-05-04 Created: 2022-05-04 Last updated: 2022-10-03Bibliographically approved

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Rydberg, Annika

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