Impact of Left Ventricular Morphology on Adverse Outcomes Following Stage 1 Palliation for Hypoplastic Left Heart Syndrome: 20 Years of National Data From Sweden

Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease

Constance G. Weismann¹  Katrin Fricke¹  Petru Liuba,¹  Katarina Hanséus¹  Magnus Dalén²  Annika Rydberg³  Mats Synnergren⁴  Mats Mellander⁴  Jan Sunnegårdh⁴  Gunnar Sjöberg⁵  Phan‐Kiet Tran⁶  Jens Johansson Ramgren⁶ 

[1] Cardiology Pediatric Heart Centre Skåne University Hospital Lund Sweden;Department of Cardiothoracic Surgery Karolinska University Hospital Stockholm Sweden;Department of Clinical Sciences, Pediatrics Umeå University Umeå Sweden;Department of Pediatrics Institute of Clinical SciencesSahlgrenska Academy Gothenburg Sweden;Department of Women's and Children's Health Karolinska Institute Stockholm Sweden;Pediatrics Department of Clinical Sciences Lund University Lund Sweden;

关键词: adverse outcome;    aortic atresia‐mitral stenosis;    globular left ventricle;    hypoplastic left heart;    left ventricular morphology;   

DOI  :  10.1161/JAHA.121.022929

来源: DOAJ

【 摘 要 】

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.

【 授权许可】

Unknown