Surgical management of progressive pulmonary venous obstruction after repair of total anomalous pulmonary venous connection

Francois Lacour-Gayet, Joy Zoghbi, Alain E. Serraf, Emré Belli, Dominique Piot, Christian Rey, François Marcon, Jacqueline Bruniaux, Claude Planché

Marie-Lannelongue Hospital and Paris-Sud University.
France

Journal of Thoracic and Cardiovascular Surgery
J Thorac Cardiovasc Surg 1999; 117: 679-687
DOI: 10.1016/S0022-5223(99)70287-4

Abstract
Background: The occurrence of a progressive pulmonary venous obstruction after the repair of the total anomalous pulmonary venous connection is a severe complication.
Objectives: The objectives of this study were to retrospectively review the patients with this condition and to report our experience with a new surgical technique with a sutureless in situ pericardium repair.
Methods: Of 178 patients who underwent correction of total anomalous pulmonary venous connection, 16 patients (9%) experienced the development of a progressive pulmonary venous obstruction in a median interval of 4 months (5 weeks-12 years). Three patients had isolated anastomotic stenosis, 4 patients had isolated pulmonary venous ostial stenosis, and 9 patients had both. Pulmonary venous obstruction was bilateral in 7 patients. The surgical procedures used at reoperation included 8 patch enlargements, 5 ostial endarterectomies, 1 intraoperative stenting, and 7 sutureless in situ pericardium repairs.
Results: There were 4 deaths after reoperation (4 of 15 patients; 27%). The only significant mortality risk factor was the bilateral location of the pulmonary venous obstruction (P =.045). In patients with isolated anastomotic stenosis or with only 1 pulmonary venous ostial stenosis (n = 5), there was no death, except the patient presenting with a single ventricle. In patients with 2 or more pulmonary venous ostial stenoses (n = 10), there were 3 deaths; 5 of the 7 survivors were successfully treated with the in situ pericardial technique, with normalized pulmonary artery pressure at a mean follow-up of 26 months.
Conclusion: Progressive pulmonary venous stenosis after repair of total anomalous pulmonary venous connection remains a severe complication when bilateral. The sutureless in situ pericardial repair offers a satisfactory solution, particularly on the right side.

Category
Stenosis or Obstruction of Pulmonary Veins Following Surgical Repair of Anomalous Pulmonary Venous Connections
Incidence or Prevalence of Disease
Patient Factors Influencing the Onset, Severity or Outcome of Disease
Length of Life Associated with Pulmonary Venous Obstruction
Surgical Interventions for Pulmonary Venous Obstruction After the Onset of Disease

Year of Publication: 1999

Age Focus: Pediatric

Article Type: Retrospective Observational Cohort Studies (>10 patients)

Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: Yes