Total anomalous pulmonary venous connection: Results of surgical repair of 100 patients at a single institution

Angela M. Kelle, Carl L Backer*, Jeffrey G Gossett, Sunjay Kaushal, Constantine Mavroudis

*Corresponding author for this work

Research output: Contribution to journalArticle

50 Citations (Scopus)

Abstract

Objective: Surgical repair of total anomalous pulmonary venous connection is associated with significant mortality and morbidity, especially in patients with single-ventricle physiology. This study analyzes total anomalous pulmonary venous connection surgical repair results at one institution to identify trends and indicators of positive outcome. Methods: Our cardiac surgery database identified 100 patients undergoing surgical repair of total anomalous pulmonary venous connection (1990-2008): supracardiac (52), cardiac (15), infracardiac (23), and mixed (10). The median age at repair was 14.6 days (range, 0-4 years), and the median weight was 3.5 kg (range, 1.3-15 kg). Patients were divided into 2 groups: biventricular (n = 83) or single-ventricle (n = 17) physiology. All but 1 of the patients with single-ventricle physiology had heterotaxy syndrome (94%), and 13 of 17 patients had supracardiac anatomy. Results: There were 12 operative deaths (4 in the biventricular group [5%] and 8 in the single-ventricle group [47%], P < .01) and 9 late deaths (6 in the biventricular group [7%] and 3 in the single-ventricle group [18%], P < .05). Death by total anomalous pulmonary venous connection type was supracardiac (12/52; 23.1%), cardiac (1/15; 6.7%), infracardiac (3/23; 13.0%), and mixed (5/10; 50%). Pulmonary venous obstruction was present in 22 patients in the biventricular group (27%) and in 7 patients in the single-ventricle group (41%; P = .25). Mortality was 9 of 29 (31%) in those with pulmonary venous obstruction and 12 of 71 (17%) in those with nonpulmonary venous obstruction (P = .23). Deep hypothermic circulatory arrest was used in 38 patients (27 in the biventricular group, 32.5%; 11 in the single-ventricle group, 64.7%). Mean deep hypothermic circulatory arrest time was 31.4 ± 10.7 minutes (P = not significant between groups). Median postoperative length of stay was 11 days (range, 0-281 days). Nineteen patients required reoperation for pulmonary venous stenosis (14 in the biventricular group and 5 in the single-ventricle group. P = .045); the median time to reoperation was 104 days (range, 4-753 days). Conclusion: Patients with total anomalous pulmonary venous connection with biventricular anatomy have good outcomes. Patients with single-ventricle anatomy have higher mortality and increased risk for pulmonary vein stenosis requiring reoperation. Mortality is highest in patients with mixed-type total anomalous pulmonary venous connection.

Original languageEnglish (US)
JournalJournal of Thoracic and Cardiovascular Surgery
Volume139
Issue number6
DOIs
StatePublished - Jan 1 2010

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Lung
Reoperation
Deep Hypothermia Induced Circulatory Arrest
Anatomy
Mortality
Heterotaxy Syndrome
Pulmonary Valve Stenosis
Thoracic Surgery
Length of Stay
Databases
Morbidity
Weights and Measures

ASJC Scopus subject areas

  • Surgery
  • Pulmonary and Respiratory Medicine
  • Cardiology and Cardiovascular Medicine

Cite this

@article{b0740943d12c40828eead00941f71d66,
title = "Total anomalous pulmonary venous connection: Results of surgical repair of 100 patients at a single institution",
abstract = "Objective: Surgical repair of total anomalous pulmonary venous connection is associated with significant mortality and morbidity, especially in patients with single-ventricle physiology. This study analyzes total anomalous pulmonary venous connection surgical repair results at one institution to identify trends and indicators of positive outcome. Methods: Our cardiac surgery database identified 100 patients undergoing surgical repair of total anomalous pulmonary venous connection (1990-2008): supracardiac (52), cardiac (15), infracardiac (23), and mixed (10). The median age at repair was 14.6 days (range, 0-4 years), and the median weight was 3.5 kg (range, 1.3-15 kg). Patients were divided into 2 groups: biventricular (n = 83) or single-ventricle (n = 17) physiology. All but 1 of the patients with single-ventricle physiology had heterotaxy syndrome (94{\%}), and 13 of 17 patients had supracardiac anatomy. Results: There were 12 operative deaths (4 in the biventricular group [5{\%}] and 8 in the single-ventricle group [47{\%}], P < .01) and 9 late deaths (6 in the biventricular group [7{\%}] and 3 in the single-ventricle group [18{\%}], P < .05). Death by total anomalous pulmonary venous connection type was supracardiac (12/52; 23.1{\%}), cardiac (1/15; 6.7{\%}), infracardiac (3/23; 13.0{\%}), and mixed (5/10; 50{\%}). Pulmonary venous obstruction was present in 22 patients in the biventricular group (27{\%}) and in 7 patients in the single-ventricle group (41{\%}; P = .25). Mortality was 9 of 29 (31{\%}) in those with pulmonary venous obstruction and 12 of 71 (17{\%}) in those with nonpulmonary venous obstruction (P = .23). Deep hypothermic circulatory arrest was used in 38 patients (27 in the biventricular group, 32.5{\%}; 11 in the single-ventricle group, 64.7{\%}). Mean deep hypothermic circulatory arrest time was 31.4 ± 10.7 minutes (P = not significant between groups). Median postoperative length of stay was 11 days (range, 0-281 days). Nineteen patients required reoperation for pulmonary venous stenosis (14 in the biventricular group and 5 in the single-ventricle group. P = .045); the median time to reoperation was 104 days (range, 4-753 days). Conclusion: Patients with total anomalous pulmonary venous connection with biventricular anatomy have good outcomes. Patients with single-ventricle anatomy have higher mortality and increased risk for pulmonary vein stenosis requiring reoperation. Mortality is highest in patients with mixed-type total anomalous pulmonary venous connection.",
author = "Kelle, {Angela M.} and Backer, {Carl L} and Gossett, {Jeffrey G} and Sunjay Kaushal and Constantine Mavroudis",
year = "2010",
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doi = "10.1016/j.jtcvs.2010.02.024",
language = "English (US)",
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journal = "Journal of Thoracic and Cardiovascular Surgery",
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Total anomalous pulmonary venous connection : Results of surgical repair of 100 patients at a single institution. / Kelle, Angela M.; Backer, Carl L; Gossett, Jeffrey G; Kaushal, Sunjay; Mavroudis, Constantine.

In: Journal of Thoracic and Cardiovascular Surgery, Vol. 139, No. 6, 01.01.2010.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Total anomalous pulmonary venous connection

T2 - Results of surgical repair of 100 patients at a single institution

AU - Kelle, Angela M.

AU - Backer, Carl L

AU - Gossett, Jeffrey G

AU - Kaushal, Sunjay

AU - Mavroudis, Constantine

PY - 2010/1/1

Y1 - 2010/1/1

N2 - Objective: Surgical repair of total anomalous pulmonary venous connection is associated with significant mortality and morbidity, especially in patients with single-ventricle physiology. This study analyzes total anomalous pulmonary venous connection surgical repair results at one institution to identify trends and indicators of positive outcome. Methods: Our cardiac surgery database identified 100 patients undergoing surgical repair of total anomalous pulmonary venous connection (1990-2008): supracardiac (52), cardiac (15), infracardiac (23), and mixed (10). The median age at repair was 14.6 days (range, 0-4 years), and the median weight was 3.5 kg (range, 1.3-15 kg). Patients were divided into 2 groups: biventricular (n = 83) or single-ventricle (n = 17) physiology. All but 1 of the patients with single-ventricle physiology had heterotaxy syndrome (94%), and 13 of 17 patients had supracardiac anatomy. Results: There were 12 operative deaths (4 in the biventricular group [5%] and 8 in the single-ventricle group [47%], P < .01) and 9 late deaths (6 in the biventricular group [7%] and 3 in the single-ventricle group [18%], P < .05). Death by total anomalous pulmonary venous connection type was supracardiac (12/52; 23.1%), cardiac (1/15; 6.7%), infracardiac (3/23; 13.0%), and mixed (5/10; 50%). Pulmonary venous obstruction was present in 22 patients in the biventricular group (27%) and in 7 patients in the single-ventricle group (41%; P = .25). Mortality was 9 of 29 (31%) in those with pulmonary venous obstruction and 12 of 71 (17%) in those with nonpulmonary venous obstruction (P = .23). Deep hypothermic circulatory arrest was used in 38 patients (27 in the biventricular group, 32.5%; 11 in the single-ventricle group, 64.7%). Mean deep hypothermic circulatory arrest time was 31.4 ± 10.7 minutes (P = not significant between groups). Median postoperative length of stay was 11 days (range, 0-281 days). Nineteen patients required reoperation for pulmonary venous stenosis (14 in the biventricular group and 5 in the single-ventricle group. P = .045); the median time to reoperation was 104 days (range, 4-753 days). Conclusion: Patients with total anomalous pulmonary venous connection with biventricular anatomy have good outcomes. Patients with single-ventricle anatomy have higher mortality and increased risk for pulmonary vein stenosis requiring reoperation. Mortality is highest in patients with mixed-type total anomalous pulmonary venous connection.

AB - Objective: Surgical repair of total anomalous pulmonary venous connection is associated with significant mortality and morbidity, especially in patients with single-ventricle physiology. This study analyzes total anomalous pulmonary venous connection surgical repair results at one institution to identify trends and indicators of positive outcome. Methods: Our cardiac surgery database identified 100 patients undergoing surgical repair of total anomalous pulmonary venous connection (1990-2008): supracardiac (52), cardiac (15), infracardiac (23), and mixed (10). The median age at repair was 14.6 days (range, 0-4 years), and the median weight was 3.5 kg (range, 1.3-15 kg). Patients were divided into 2 groups: biventricular (n = 83) or single-ventricle (n = 17) physiology. All but 1 of the patients with single-ventricle physiology had heterotaxy syndrome (94%), and 13 of 17 patients had supracardiac anatomy. Results: There were 12 operative deaths (4 in the biventricular group [5%] and 8 in the single-ventricle group [47%], P < .01) and 9 late deaths (6 in the biventricular group [7%] and 3 in the single-ventricle group [18%], P < .05). Death by total anomalous pulmonary venous connection type was supracardiac (12/52; 23.1%), cardiac (1/15; 6.7%), infracardiac (3/23; 13.0%), and mixed (5/10; 50%). Pulmonary venous obstruction was present in 22 patients in the biventricular group (27%) and in 7 patients in the single-ventricle group (41%; P = .25). Mortality was 9 of 29 (31%) in those with pulmonary venous obstruction and 12 of 71 (17%) in those with nonpulmonary venous obstruction (P = .23). Deep hypothermic circulatory arrest was used in 38 patients (27 in the biventricular group, 32.5%; 11 in the single-ventricle group, 64.7%). Mean deep hypothermic circulatory arrest time was 31.4 ± 10.7 minutes (P = not significant between groups). Median postoperative length of stay was 11 days (range, 0-281 days). Nineteen patients required reoperation for pulmonary venous stenosis (14 in the biventricular group and 5 in the single-ventricle group. P = .045); the median time to reoperation was 104 days (range, 4-753 days). Conclusion: Patients with total anomalous pulmonary venous connection with biventricular anatomy have good outcomes. Patients with single-ventricle anatomy have higher mortality and increased risk for pulmonary vein stenosis requiring reoperation. Mortality is highest in patients with mixed-type total anomalous pulmonary venous connection.

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