A 30-year experience with mixed-type total anomalous pulmonary venous connection: a word of caution. (20th September 2016)

• Record Type: Journal Article
• Authors: Kogon, Brian; Fernandez, Jan; Shashidharan, Subhadra; Kanter, Kirk; Alsoufi, Bahaaldin
• Is Part Of: Cardiology in the young. Volume 27:Number 5(2017)
• Page Start: 870
• Page End: 876
• Publication Date: 2016-09-20
• Subjects: Pulmonary vein, -- mixed type, -- mortality; Pediatric cardiology -- Periodicals; 618.9212
• DOI: 10.1017/S1047951116001414
• Languages: English
• ISSNs: 1047-9511

Abstract

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). Conclusion: 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.