AI Article Synopsis

  • Hypoplastic left heart syndrome (HLHS) presents serious health challenges, with a study examining outcomes of 167 patients who underwent stage 1 palliation in Sweden from 1999 to 2018.
  • The study categorized patients based on left ventricular morphology and found that aortic atresia-mitral stenosis (AA-MS) and globular left ventricle (Glob-LV) significantly increased the risk of mortality (31%) and major adverse events (41%).
  • Patients with AA-MS or Glob-LV who received a Blalock-Taussig shunt had higher mortality rates compared to those with Sano shunts, indicating the importance of shunt type in patient management and outcomes.

Article 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 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; =0.006; overall: HR, 2.2; =0.005) and MAEs (HR, 2.4; =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; =0.004; overall: HR, 3.4; =0.0007) and MAEs (HR, 2.7; =0.0007). There was no difference in mortality and MAEs between patients with AA-MS and without AA-MS with Glob-LV (>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; =0.04; Glob- LV: HR, 2.1; =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.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9075443PMC
http://dx.doi.org/10.1161/JAHA.121.022929DOI Listing

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