Hostname: page-component-8448b6f56d-42gr6 Total loading time: 0 Render date: 2024-04-23T07:11:43.676Z Has data issue: false hasContentIssue false
  • English
  • Français

A 30-year experience with mixed-type total anomalous pulmonary venous connection: a word of caution

Published online by Cambridge University Press:  20 September 2016

Brian Kogon ,
Jan Fernandez ,
Subhadra Shashidharan ,
Kirk Kanter  and
Bahaaldin Alsoufi
Brian Kogon*
Affiliation:
Department of Cardiothoracic Surgery, Emory University, Atlanta, Georgia, United States of America
Jan Fernandez
Affiliation:
Department of Cardiothoracic Surgery, Emory University, Atlanta, Georgia, United States of America
Subhadra Shashidharan
Affiliation:
Department of Cardiothoracic Surgery, Emory University, Atlanta, Georgia, United States of America
Kirk Kanter
Affiliation:
Department of Cardiothoracic Surgery, Emory University, Atlanta, Georgia, United States of America
Bahaaldin Alsoufi
Affiliation:
Department of Cardiothoracic Surgery, Emory University, Atlanta, Georgia, United States of America
*
Correspondence to: B. E. Kogon, MD, Emory University, Children’s Healthcare of Atlanta, Egleston, Atlanta, Georgia, United States of America. Tel: 678 372 7324; Fax: 404 785 6266; E-mail: Bkogon@emory.edu
Get access Rights & Permissions [Opens in a new window]

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.

Keywords

Pulmonary veinmixed typemortality
Type
Original Articles
Information
Cardiology in the Young , Volume 27 , Issue 5 , July 2017 , pp. 870 - 876
Check for updates
Copyright
© Cambridge University Press 2016 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1. Kanter, KR. Surgical repair of total anomalous pulmonary venous connection. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2006; 9: 40–44.CrossRefGoogle Scholar
2. Darling, RC, Rothney, WB, Craig, JM. Total anomalous pulmonary venous drainage into the right side of the heart: report of 17 autopsied cases not associated with other major cardiovascular anomalies. Lab Invest 1957; 6: 44–55.Google Scholar
3. Karaci, AR, Harmander, B, Aydemir, NA, et al. Early and intermediate term results for surgical correction of total anomalous pulmonary venous return. J Cardiothorac Surg 2012; 27: 376–380.Google Scholar
4. Padalino, MA, Cavalli, G, De Franceschi, M, et al. Surgical outcomes of total anomalous pulmonary venous connection repair: a 22-year experience. J Cardiothorac Surg 2014; 29: 678–685.Google ScholarPubMed
5. Yong, MS, d’Udekem, Y, Robertson, T, et al. Outcomes of surgery for simple total anomalous pulmonary venous drainage in infants. Ann Thorac Surg 2011; 91: 1921–1927.Google Scholar
6. Hoashi, T, Kagisaki, K, Kurosaki, K, Kitano, M, Shiraishi, I, Ichikawa, H. Intrinsic obstruction in pulmonary venous drainage pathway is associated with poor surgical outcomes in patients with total anomalous pulmonary venous connection. Pediatr Cardiol 2015; 36: 432–437.Google Scholar
7. Imoto, Y, Kado, H, Asou, T, Shiokawa, Y, Tominaga, R, Yasui, H. Mixed type of total anomalous pulmonary venous connection. Ann Thorac Surg 1998; 66: 1394–1397.CrossRefGoogle ScholarPubMed