Takahiko Sakamoto, Mitsugi Nagashima, Kentarou Umezu, Ryogo Houki, Jin Ikarashi, Junko Katagiri, Kenji Yamazaki
The Heart Institute of Japan and Tokyo Women’s Medical University.
Japan
Interactive Cardiovascular and Thoracic Surgery
Interact Cardiovasc Thorac Surg 2018; 27: 20-26
DOI: 10.1093/icvts/ivy034
Abstract
Objectives: Isolated total anomalous pulmonary venous connection (TAPVC) is a relatively rare congenital cardiac defect, while pulmonary venous obstruction (PVO) is associated with poor prognosis. We reviewed the long-term outcome of total correction for isolated TAPVC at our institute and analysed the risk factors for mortality and morbidity.
Methods: A total of 290 isolated TAPVC patients evaluated between 1965 and 2016 were divided into 2 groups: Group Early (n = 151) underwent surgery before 1989 ; Group Late (n = 139) underwent surgery after 1990. The mean age at operation was 10.4 ± 30.2 months (range 0 day to 23 years), and the mean body weight was 5.5 ± 6.0 kg (range 1.6-48 kg). Group Late included more patients with the infracardiac type of TAPVC and preferably used the posterior approach.
Results: There were 53 hospital deaths and 16 late deaths. Postoperative PVO was recognized in 28 patients. The mean follow-up time was 18.2 ± 9.7 years (range 2 months to 42.4 years). The actuarial survival rate was improved to 87.8% at 20 years in Group Late. Multivariable analysis revealed that death rate was significantly increased in patients of Group Early, with a body weight <2 kg and with postoperative PVO (P < 0.0001, P = 0.0041, P = 0.0003, respectively). Reoperations were performed 27 times in 22 patients (PVO repair, 11; staged repair, 4 and others, 12). PVO repair was performed at a mean of 2.5 ± 1.6 months later. The actuarial freedom from reoperation rates were 88.8% and 83.2% at 20 and 30 years, respectively. Multivariable analysis revealed that the risk of reoperation was associated with mixed-type TAPVC and postoperative PVO (P = 0.0064 and P < 0.0001, respectively).
Conclusions: Long-term surgical outcomes of isolated TAPVC have improved over the past 25 years. Postoperative PVO, the mixed-type TAPVC and a body weight <2 kg might be the important factors contributing to mortality and morbidity.
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
Influence of Surgical Era on the Onset, Severity or Outcome of Disease
Length of Life Associated with Pulmonary Venous Obstruction
Year of Publication: 2018
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