title: Total Anomalous Pulmonary Venous Connection: A 40 years' Experience Analysis.
abstract: BackgroundTotal anomalous pulmonary venous connection is a rare cardiac malformation associated with significant morbidity and mortality rates. We report a large surgical series study to evaluate mid-term and long-term results of conventional surgical techniques.Methods and ResultsWe performed a retrospective analytic study of all patients operated on for simple total anomalous pulmonary venous connection in the University Hospital of Lyon, France, between January 1973 and June 2014. A total of 180 patients were included (43% supracardiac, 27% intracardiac, 19% infracardiac, and 11% mixed types). Mean cardiopulmonary bypass and aortic cross clamp times were respectively 66 and 39 minutes. Overall mortality was 27.1%, including 38 early deaths (21.1%) and 12 late deaths (6.1%). The percentage of early death greatly decreased over the eras, from 42.1% in the seventies to 7.4% after 2010. Besides the earlier era of intervention (p< 0.0001), significant risk factors for death in multivariate analysis were preoperative pulmonary hypertension, acidosis, and cardiopulmonary bypass time. There were 24 reoperations, including 7 for pulmonary venous obstruction; 6 died. Factors directly and independently associated with late complications were the anatomic type (mixed forms,p= 0.0023), and length of aortic cross clamp time (p= 0.01). Long-term results for survivals are excellent. We report 84.7% of asymptomatic patients with a mean follow-up of 10.8 years.ConclusionsThe overall prognosis of total anomalous pulmonary venous connection repair with conventional procedures has greatly improved over the years with excellent long-term results. A thorough evaluation of all preoperative characteristics is imperative to achieve the best outcome.
authors.affiliation: Department of Cardiac Surgery, Hopital Europeen Georges Pompidou, Paris, Île-de-France, France.
authors.forename: Anaïs
authors.initials: A
authors.lastname: Lemaire
authors.affiliation: Department of Pediatric Cardiac Surgery, Hopital Cardio-vasculaire et Pneumologique Louis Pradel, Lyon, Rhône-Alpes, France.
authors.forename: Sylvie
authors.initials: S
authors.lastname: DiFilippo
authors.affiliation: Department of Research and Biostatistic, Centre Hospitalier Universitaire de Caen, Caen, Basse-Normandie, France.
authors.forename: Jean-Jacques
authors.initials: JJ
authors.lastname: Parienti
authors.affiliation: Department of Pediatric Cardiac Surgery, Hopital Cardio-vasculaire et Pneumologique Louis Pradel, Lyon, Rhône-Alpes, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Metton
authors.affiliation: Department of Pediatric Cardiac Surgery, Hopital Cardio-vasculaire et Pneumologique Louis Pradel, Lyon, Rhône-Alpes, France.
authors.forename: Julia
authors.initials: J
authors.lastname: Mitchell
authors.affiliation: Department of Pediatric Cardiac Surgery, Hopital Cardio-vasculaire et Pneumologique Louis Pradel, Lyon, Rhône-Alpes, France.
authors.forename: Roland
authors.initials: R
authors.lastname: Hénaine
authors.affiliation: Department of Pediatric Cardiac Surgery, Hopital Cardio-vasculaire et Pneumologique Louis Pradel, Lyon, Rhône-Alpes, France.
authors.forename: Jean
authors.initials: J
authors.lastname: Ninet
mesh_terms: D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000401:mortality / Q000639:trends; D016009:Chi-Square Distribution; D002648:Child; D002675:Child, Preschool; D005260:Female; D005602:France; D006785:Hospitals, University; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D016015:Logistic Models; D008297:Male; D015999:Multivariate Analysis; D016017:Odds Ratio; D011183:Postoperative Complications / Q000601:surgery; D012086:Reoperation; D012189:Retrospective Studies; D012307:Risk Factors; D012587:Scimitar Syndrome / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D017741:Survivors; D013997:Time Factors; D016896:Treatment Outcome