title: A 30-year experience with mixed-type total anomalous pulmonary venous connection: a word of caution.
abstract: BACKGROUND
  Patients with total anomalous pulmonary venous connection can be problematic, particularly those with mixed-type pathology. We aimed to describe a cohort of patients with mixed-type anomalous drainage, highlighting the treatment challenges, and identifying risk factors for poor outcome.
  
  
  METHODS
  We reviewed the clinical records of patients who underwent repair for mixed-type total anomalous pulmonary venous connection between 1986 and 2015.
  
  
  RESULTS
  A total of 19 patients were identified. The median age and weight of patients at surgery were 18 days (with a range from 1 to 185) and 3.4 kg (with a range from 1.9 to 6.5), respectively. Venous anatomy included a combination of duplicate supracardiac (four), supracardiac and cardiac (11), and supracardiac and infracardiac (four) drainage. Out of 19 patients, six (32%) died within 30 days or the initial hospital stay; two additional patients died from progressive pulmonary vein stenosis at 72 and 201 days, respectively, resulting in 42% mortality within the 1st year. Follow-up data were available for 8/11 long-term survivors. The median follow-up period was 7.3 years (with a range from 1.8 to 15.7). Only one patient underwent re-intervention for recurrent pulmonary vein stenosis. For surgical mortality, no statistically significant risk factors were identified, although the risk trended to be higher (p⩽0.1) with lower age and weight, an infracardiac component, and prolonged cardiopulmonary bypass. For 1-year mortality, the risk became significant (p⩽0.05) with a lower weight (p=0.01), an infracardiac component (p=0.03), and prolonged cardiopulmonary bypass (p=0.04).
  
  
  CONCLUSIONS
  The surgical and 1-year mortality in patients with mixed-type total anomalous pulmonary venous connection is high. On the other hand, among patients who survive past the 1st year, most have good outcomes without subsequent sequelae.
authors.affiliation: Department of Cardiothoracic Surgery,Emory University,Atlanta,Georgia,United States of America.
authors.forename: Brian
authors.initials: B
authors.lastname: Kogon
authors.affiliation: Department of Cardiothoracic Surgery,Emory University,Atlanta,Georgia,United States of America.
authors.forename: Jan
authors.initials: J
authors.lastname: Fernandez
authors.affiliation: Department of Cardiothoracic Surgery,Emory University,Atlanta,Georgia,United States of America.
authors.forename: Subhadra
authors.initials: S
authors.lastname: Shashidharan
authors.affiliation: Department of Cardiothoracic Surgery,Emory University,Atlanta,Georgia,United States of America.
authors.forename: Kirk
authors.initials: K
authors.lastname: Kanter
authors.affiliation: Department of Cardiothoracic Surgery,Emory University,Atlanta,Georgia,United States of America.
authors.forename: Bahaaldin
authors.initials: B
authors.lastname: Alsoufi
mesh_terms: D002315:Cardiopulmonary Bypass / Q000009:adverse effects*; D005260:Female; D005845:Georgia; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007902:Length of Stay; D016015:Logistic Models; D008297:Male; D015999:Multivariate Analysis; D011183:Postoperative Complications; D011667:Pulmonary Veins / Q000002:abnormalities / Q000601:surgery*; D012086:Reoperation; D012189:Retrospective Studies; D012307:Risk Factors; D012587:Scimitar Syndrome / Q000145:classification / Q000401:mortality* / Q000601:surgery*; D016896:Treatment Outcome
keywords: Pulmonary vein; mixed type; mortality