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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

Fricke, Katrin LU ; Mellander, Mats ; Hanséus, Katarina LU ; Tran, Phan Kiet LU ; Synnergren, Mats ; Johansson Ramgren, Jens LU ; Rydberg, Annika ; Sunnegårdh, Jan ; Dalén, Magnus and Sjöberg, Gunnar , et al. (2022) In Journal of the American Heart Association 11. p.1-13
Abstract
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... (More)
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. (Less)
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organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Journal of the American Heart Association
volume
11
article number
e022929
pages
1 - 13
publisher
Wiley-Blackwell
external identifiers
  • scopus:85128245385
  • pmid:35348003
ISSN
2047-9980
DOI
10.1161/JAHA.121.022929
language
English
LU publication?
yes
id
3f71c4bf-87d0-4df0-bc79-669b4d8be8c1
date added to LUP
2022-03-30 19:01:01
date last changed
2024-01-31 00:56:53
@article{3f71c4bf-87d0-4df0-bc79-669b4d8be8c1,
  abstract     = {{Background<br/>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.<br/><br/>Methods and Results<br/>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&gt;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).<br/><br/>Conclusions<br/>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.}},
  author       = {{Fricke, Katrin and Mellander, Mats and Hanséus, Katarina and Tran, Phan Kiet and Synnergren, Mats and Johansson Ramgren, Jens and Rydberg, Annika and Sunnegårdh, Jan and Dalén, Magnus and Sjöberg, Gunnar and Weismann, Constance Gesina and Liuba, Petru}},
  issn         = {{2047-9980}},
  language     = {{eng}},
  pages        = {{1--13}},
  publisher    = {{Wiley-Blackwell}},
  series       = {{Journal of the American Heart Association}},
  title        = {{Impact of Left Ventricular Morphology on Adverse Outcomes Following Stage 1 Palliation for Hypoplastic Left Heart Syndrome : 20 Years of National Data From Sweden}},
  url          = {{http://dx.doi.org/10.1161/JAHA.121.022929}},
  doi          = {{10.1161/JAHA.121.022929}},
  volume       = {{11}},
  year         = {{2022}},
}