Results of extracardiac conduit total cavopulmonary connection in 500 patients

Toshihide Nakano, Hideaki Kado, Hideki Tatewaki, Kazuhiro Hinokiyama, Shinichiro Oda, Hiroya Ushinohama, Koichi Sagawa, Makoto Nakamura, Naoki Fusazaki, Shiro Ishikawa

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Abstract

OBJECTIVES: This single-institution study aimed to evaluate the early to mid-term outcomes of extracardiac conduit total cavopulmonary connection (EC-TCPC). METHODS: Between March 1994 and March 2014, 500 patients (median age, 3.4 years) underwent EC-TCPC at our hospital. One hundred and twenty-three patients (24.6%) showed heterotaxy, and fenestration was created in 6 patients (1.2%). The standard institutional treatment policy included postoperative anticoagulation and individualized cardiovascular medication. The mortality and morbidity rates, haemodynamic status, cardiopulmonary exercise capacity and liver examination results during the follow-up period (median, 6.7 years) were retrospectively reviewed. RESULTS: There were 2 early and 17 late deaths. The Kaplan-Meier estimated survival rate was 96.2% at 10 years and 92.8% at 15 years. Bradyarrhythmia and tachyarrhythmia occurred in 19 and 13 patients, respectively. Other late-occurring morbidities included proteinlosing enteropathy in 8, thromboembolism in 5, bleeding complications in 6 and liver cirrhosis in 1 patient. The rate of freedom from lateoccurring morbidities was 82.1% at 15 years. In the multivariate analysis, heterotaxy was found to be a predictor for mortality (P = 0.02), whereas age at operation was a predictor for new-onset arrhythmias (P = 0.048). In the cardiopulmonary exercise test (n = 312), the peak VO2 was 84.9 ± 17.3% of the predicted value, which tended to decrease with age (R2 = 0.32) and elapsed time since operation (R2 = 0.21). Postoperative cardiac catheterization (n = 468; time from surgery, 3.6 ± 4.3 years) showed central venous pressure of 9.9 ± 2.4 mmHg, ventricular end-diastolic pressure of 5.2 ± 3.3 mmHg, cardiac index of 3.4 ± 0.8 l/min/m2 and arterial oxygen saturation of 94.2 ± 4.8%. In 101 patients who were followed up for =10 years, amino-terminal type III procollagen peptide and collagen type IV levels exceeded the normal ranges in 52.9 and 75.2% of patients, respectively, and liver ultrasonography revealed hyper-echoic spots in 43.3% of patients. CONCLUSIONS: The early to mid-term outcomes of post-EC-TCPC patients managed with individualized pharmacotherapy were excellent, with low mortality and morbidity rates; however, development of late-occurring morbidities specific to Fontan physiology, including exercise intolerance and liver disease, must be carefully monitored during the long-term follow-up.

Original languageEnglish
Pages (from-to)825-832
Number of pages8
JournalEuropean Journal of Cardio-thoracic Surgery
Volume48
Issue number6
DOIs
Publication statusPublished - Jan 1 2015

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Morbidity
Collagen Type IV
Mortality
Organizational Policy
Exercise
Central Venous Pressure
Collagen Type III
Liver
Thromboembolism
Bradycardia
Cardiac Catheterization
Exercise Test
Tachycardia
Liver Cirrhosis
Liver Diseases
Cardiac Arrhythmias
Ultrasonography
Reference Values
Multivariate Analysis
Survival Rate

All Science Journal Classification (ASJC) codes

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

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Results of extracardiac conduit total cavopulmonary connection in 500 patients. / Nakano, Toshihide; Kado, Hideaki; Tatewaki, Hideki; Hinokiyama, Kazuhiro; Oda, Shinichiro; Ushinohama, Hiroya; Sagawa, Koichi; Nakamura, Makoto; Fusazaki, Naoki; Ishikawa, Shiro.

In: European Journal of Cardio-thoracic Surgery, Vol. 48, No. 6, 01.01.2015, p. 825-832.

Research output: Contribution to journalArticle

Nakano, T, Kado, H, Tatewaki, H, Hinokiyama, K, Oda, S, Ushinohama, H, Sagawa, K, Nakamura, M, Fusazaki, N & Ishikawa, S 2015, 'Results of extracardiac conduit total cavopulmonary connection in 500 patients', European Journal of Cardio-thoracic Surgery, vol. 48, no. 6, pp. 825-832. https://doi.org/10.1093/ejcts/ezv072
Nakano, Toshihide ; Kado, Hideaki ; Tatewaki, Hideki ; Hinokiyama, Kazuhiro ; Oda, Shinichiro ; Ushinohama, Hiroya ; Sagawa, Koichi ; Nakamura, Makoto ; Fusazaki, Naoki ; Ishikawa, Shiro. / Results of extracardiac conduit total cavopulmonary connection in 500 patients. In: European Journal of Cardio-thoracic Surgery. 2015 ; Vol. 48, No. 6. pp. 825-832.
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abstract = "OBJECTIVES: This single-institution study aimed to evaluate the early to mid-term outcomes of extracardiac conduit total cavopulmonary connection (EC-TCPC). METHODS: Between March 1994 and March 2014, 500 patients (median age, 3.4 years) underwent EC-TCPC at our hospital. One hundred and twenty-three patients (24.6{\%}) showed heterotaxy, and fenestration was created in 6 patients (1.2{\%}). The standard institutional treatment policy included postoperative anticoagulation and individualized cardiovascular medication. The mortality and morbidity rates, haemodynamic status, cardiopulmonary exercise capacity and liver examination results during the follow-up period (median, 6.7 years) were retrospectively reviewed. RESULTS: There were 2 early and 17 late deaths. The Kaplan-Meier estimated survival rate was 96.2{\%} at 10 years and 92.8{\%} at 15 years. Bradyarrhythmia and tachyarrhythmia occurred in 19 and 13 patients, respectively. Other late-occurring morbidities included proteinlosing enteropathy in 8, thromboembolism in 5, bleeding complications in 6 and liver cirrhosis in 1 patient. The rate of freedom from lateoccurring morbidities was 82.1{\%} at 15 years. In the multivariate analysis, heterotaxy was found to be a predictor for mortality (P = 0.02), whereas age at operation was a predictor for new-onset arrhythmias (P = 0.048). In the cardiopulmonary exercise test (n = 312), the peak VO2 was 84.9 ± 17.3{\%} of the predicted value, which tended to decrease with age (R2 = 0.32) and elapsed time since operation (R2 = 0.21). Postoperative cardiac catheterization (n = 468; time from surgery, 3.6 ± 4.3 years) showed central venous pressure of 9.9 ± 2.4 mmHg, ventricular end-diastolic pressure of 5.2 ± 3.3 mmHg, cardiac index of 3.4 ± 0.8 l/min/m2 and arterial oxygen saturation of 94.2 ± 4.8{\%}. In 101 patients who were followed up for =10 years, amino-terminal type III procollagen peptide and collagen type IV levels exceeded the normal ranges in 52.9 and 75.2{\%} of patients, respectively, and liver ultrasonography revealed hyper-echoic spots in 43.3{\%} of patients. CONCLUSIONS: The early to mid-term outcomes of post-EC-TCPC patients managed with individualized pharmacotherapy were excellent, with low mortality and morbidity rates; however, development of late-occurring morbidities specific to Fontan physiology, including exercise intolerance and liver disease, must be carefully monitored during the long-term follow-up.",
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AU - Nakano, Toshihide

AU - Kado, Hideaki

AU - Tatewaki, Hideki

AU - Hinokiyama, Kazuhiro

AU - Oda, Shinichiro

AU - Ushinohama, Hiroya

AU - Sagawa, Koichi

AU - Nakamura, Makoto

AU - Fusazaki, Naoki

AU - Ishikawa, Shiro

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N2 - OBJECTIVES: This single-institution study aimed to evaluate the early to mid-term outcomes of extracardiac conduit total cavopulmonary connection (EC-TCPC). METHODS: Between March 1994 and March 2014, 500 patients (median age, 3.4 years) underwent EC-TCPC at our hospital. One hundred and twenty-three patients (24.6%) showed heterotaxy, and fenestration was created in 6 patients (1.2%). The standard institutional treatment policy included postoperative anticoagulation and individualized cardiovascular medication. The mortality and morbidity rates, haemodynamic status, cardiopulmonary exercise capacity and liver examination results during the follow-up period (median, 6.7 years) were retrospectively reviewed. RESULTS: There were 2 early and 17 late deaths. The Kaplan-Meier estimated survival rate was 96.2% at 10 years and 92.8% at 15 years. Bradyarrhythmia and tachyarrhythmia occurred in 19 and 13 patients, respectively. Other late-occurring morbidities included proteinlosing enteropathy in 8, thromboembolism in 5, bleeding complications in 6 and liver cirrhosis in 1 patient. The rate of freedom from lateoccurring morbidities was 82.1% at 15 years. In the multivariate analysis, heterotaxy was found to be a predictor for mortality (P = 0.02), whereas age at operation was a predictor for new-onset arrhythmias (P = 0.048). In the cardiopulmonary exercise test (n = 312), the peak VO2 was 84.9 ± 17.3% of the predicted value, which tended to decrease with age (R2 = 0.32) and elapsed time since operation (R2 = 0.21). Postoperative cardiac catheterization (n = 468; time from surgery, 3.6 ± 4.3 years) showed central venous pressure of 9.9 ± 2.4 mmHg, ventricular end-diastolic pressure of 5.2 ± 3.3 mmHg, cardiac index of 3.4 ± 0.8 l/min/m2 and arterial oxygen saturation of 94.2 ± 4.8%. In 101 patients who were followed up for =10 years, amino-terminal type III procollagen peptide and collagen type IV levels exceeded the normal ranges in 52.9 and 75.2% of patients, respectively, and liver ultrasonography revealed hyper-echoic spots in 43.3% of patients. CONCLUSIONS: The early to mid-term outcomes of post-EC-TCPC patients managed with individualized pharmacotherapy were excellent, with low mortality and morbidity rates; however, development of late-occurring morbidities specific to Fontan physiology, including exercise intolerance and liver disease, must be carefully monitored during the long-term follow-up.

AB - OBJECTIVES: This single-institution study aimed to evaluate the early to mid-term outcomes of extracardiac conduit total cavopulmonary connection (EC-TCPC). METHODS: Between March 1994 and March 2014, 500 patients (median age, 3.4 years) underwent EC-TCPC at our hospital. One hundred and twenty-three patients (24.6%) showed heterotaxy, and fenestration was created in 6 patients (1.2%). The standard institutional treatment policy included postoperative anticoagulation and individualized cardiovascular medication. The mortality and morbidity rates, haemodynamic status, cardiopulmonary exercise capacity and liver examination results during the follow-up period (median, 6.7 years) were retrospectively reviewed. RESULTS: There were 2 early and 17 late deaths. The Kaplan-Meier estimated survival rate was 96.2% at 10 years and 92.8% at 15 years. Bradyarrhythmia and tachyarrhythmia occurred in 19 and 13 patients, respectively. Other late-occurring morbidities included proteinlosing enteropathy in 8, thromboembolism in 5, bleeding complications in 6 and liver cirrhosis in 1 patient. The rate of freedom from lateoccurring morbidities was 82.1% at 15 years. In the multivariate analysis, heterotaxy was found to be a predictor for mortality (P = 0.02), whereas age at operation was a predictor for new-onset arrhythmias (P = 0.048). In the cardiopulmonary exercise test (n = 312), the peak VO2 was 84.9 ± 17.3% of the predicted value, which tended to decrease with age (R2 = 0.32) and elapsed time since operation (R2 = 0.21). Postoperative cardiac catheterization (n = 468; time from surgery, 3.6 ± 4.3 years) showed central venous pressure of 9.9 ± 2.4 mmHg, ventricular end-diastolic pressure of 5.2 ± 3.3 mmHg, cardiac index of 3.4 ± 0.8 l/min/m2 and arterial oxygen saturation of 94.2 ± 4.8%. In 101 patients who were followed up for =10 years, amino-terminal type III procollagen peptide and collagen type IV levels exceeded the normal ranges in 52.9 and 75.2% of patients, respectively, and liver ultrasonography revealed hyper-echoic spots in 43.3% of patients. CONCLUSIONS: The early to mid-term outcomes of post-EC-TCPC patients managed with individualized pharmacotherapy were excellent, with low mortality and morbidity rates; however, development of late-occurring morbidities specific to Fontan physiology, including exercise intolerance and liver disease, must be carefully monitored during the long-term follow-up.

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