Pulmonary arteriovenous malformations in children after the Kawashima operation

John Brown, Mark Ruzmetov, Palaniswamy Vijay, Mark Rodefeld, Mark Turrentine

Research output: Contribution to journalArticle

42 Citations (Scopus)

Abstract

Background. Pulmonary arteriovenous malformations are a major cause of progressive late cyanosis in patients treated with cavopulmonary anastomoses. Previous experience suggests that exclusion of the hepatic venous effluent from the pulmonary circulation may cause the development of pulmonary arteriovenous malformations after the Kawashima operation in children with interrupted inferior vena cava with azygous continuation. Methods. From January 1990 to November 2004, 21 children (median age, 2 years) with heterotaxy syndrome and interrupted inferior vena cava with azygous continuation underwent Kawashima operation. The average preoperative arterial oxygen saturation was 76% ± 7% (range, 64% to 90%). Results. Follow-up was complete in all survivors except 1 at a median duration of 4.5 years. Pulmonary arteriovenous malformations were diagnosed at a median of 5 years after Kawashima operation in 11 patients (58%). Completion Fontan operation has been performed in 15 (79%). Five children who underwent a completion Fontan procedure 1 to 1.5 years after Kawashima operation did not have pulmonary arteriovenous malformations. There were 2 late deaths due to chronic congestive heart failure 4 months and 7 years after Kawashima operation. Overall survival at 10 years is 90%. Univariate and multivariate analysis demonstrated presence of bilateral superior vena cavae (p = 0.002) and interval longer than 2 years between Kawashima operation and completion Fontan operation (p = 0.04) as predictors of developing pulmonary arteriovenous malformations. Conclusions. In most patients with heterotaxy and interrupted inferior vena cava with azygous continuation, clinical evidence of pulmonary arteriovenous malformations will develop after Kawashima operation if they are followed up long enough. Early redirection of the hepatic venous effluent to the pulmonary arterial circulation may prevent or lead to regression of pulmonary arteriovenous malformations, with low mortality and morbidity.

Original languageEnglish
Pages (from-to)1592-1596
Number of pages5
JournalAnnals of Thoracic Surgery
Volume80
Issue number5
DOIs
StatePublished - Nov 2005

Fingerprint

Arteriovenous Malformations
Lung
Fontan Procedure
Inferior Vena Cava
Pulmonary Circulation
Right Heart Bypass
Heterotaxy Syndrome
Cyanosis
Superior Vena Cava
Liver
Survivors
Multivariate Analysis
Heart Failure
Oxygen
Morbidity
Survival
Mortality

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Surgery

Cite this

Pulmonary arteriovenous malformations in children after the Kawashima operation. / Brown, John; Ruzmetov, Mark; Vijay, Palaniswamy; Rodefeld, Mark; Turrentine, Mark.

In: Annals of Thoracic Surgery, Vol. 80, No. 5, 11.2005, p. 1592-1596.

Research output: Contribution to journalArticle

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abstract = "Background. Pulmonary arteriovenous malformations are a major cause of progressive late cyanosis in patients treated with cavopulmonary anastomoses. Previous experience suggests that exclusion of the hepatic venous effluent from the pulmonary circulation may cause the development of pulmonary arteriovenous malformations after the Kawashima operation in children with interrupted inferior vena cava with azygous continuation. Methods. From January 1990 to November 2004, 21 children (median age, 2 years) with heterotaxy syndrome and interrupted inferior vena cava with azygous continuation underwent Kawashima operation. The average preoperative arterial oxygen saturation was 76{\%} ± 7{\%} (range, 64{\%} to 90{\%}). Results. Follow-up was complete in all survivors except 1 at a median duration of 4.5 years. Pulmonary arteriovenous malformations were diagnosed at a median of 5 years after Kawashima operation in 11 patients (58{\%}). Completion Fontan operation has been performed in 15 (79{\%}). Five children who underwent a completion Fontan procedure 1 to 1.5 years after Kawashima operation did not have pulmonary arteriovenous malformations. There were 2 late deaths due to chronic congestive heart failure 4 months and 7 years after Kawashima operation. Overall survival at 10 years is 90{\%}. Univariate and multivariate analysis demonstrated presence of bilateral superior vena cavae (p = 0.002) and interval longer than 2 years between Kawashima operation and completion Fontan operation (p = 0.04) as predictors of developing pulmonary arteriovenous malformations. Conclusions. In most patients with heterotaxy and interrupted inferior vena cava with azygous continuation, clinical evidence of pulmonary arteriovenous malformations will develop after Kawashima operation if they are followed up long enough. Early redirection of the hepatic venous effluent to the pulmonary arterial circulation may prevent or lead to regression of pulmonary arteriovenous malformations, with low mortality and morbidity.",
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N2 - Background. Pulmonary arteriovenous malformations are a major cause of progressive late cyanosis in patients treated with cavopulmonary anastomoses. Previous experience suggests that exclusion of the hepatic venous effluent from the pulmonary circulation may cause the development of pulmonary arteriovenous malformations after the Kawashima operation in children with interrupted inferior vena cava with azygous continuation. Methods. From January 1990 to November 2004, 21 children (median age, 2 years) with heterotaxy syndrome and interrupted inferior vena cava with azygous continuation underwent Kawashima operation. The average preoperative arterial oxygen saturation was 76% ± 7% (range, 64% to 90%). Results. Follow-up was complete in all survivors except 1 at a median duration of 4.5 years. Pulmonary arteriovenous malformations were diagnosed at a median of 5 years after Kawashima operation in 11 patients (58%). Completion Fontan operation has been performed in 15 (79%). Five children who underwent a completion Fontan procedure 1 to 1.5 years after Kawashima operation did not have pulmonary arteriovenous malformations. There were 2 late deaths due to chronic congestive heart failure 4 months and 7 years after Kawashima operation. Overall survival at 10 years is 90%. Univariate and multivariate analysis demonstrated presence of bilateral superior vena cavae (p = 0.002) and interval longer than 2 years between Kawashima operation and completion Fontan operation (p = 0.04) as predictors of developing pulmonary arteriovenous malformations. Conclusions. In most patients with heterotaxy and interrupted inferior vena cava with azygous continuation, clinical evidence of pulmonary arteriovenous malformations will develop after Kawashima operation if they are followed up long enough. Early redirection of the hepatic venous effluent to the pulmonary arterial circulation may prevent or lead to regression of pulmonary arteriovenous malformations, with low mortality and morbidity.

AB - Background. Pulmonary arteriovenous malformations are a major cause of progressive late cyanosis in patients treated with cavopulmonary anastomoses. Previous experience suggests that exclusion of the hepatic venous effluent from the pulmonary circulation may cause the development of pulmonary arteriovenous malformations after the Kawashima operation in children with interrupted inferior vena cava with azygous continuation. Methods. From January 1990 to November 2004, 21 children (median age, 2 years) with heterotaxy syndrome and interrupted inferior vena cava with azygous continuation underwent Kawashima operation. The average preoperative arterial oxygen saturation was 76% ± 7% (range, 64% to 90%). Results. Follow-up was complete in all survivors except 1 at a median duration of 4.5 years. Pulmonary arteriovenous malformations were diagnosed at a median of 5 years after Kawashima operation in 11 patients (58%). Completion Fontan operation has been performed in 15 (79%). Five children who underwent a completion Fontan procedure 1 to 1.5 years after Kawashima operation did not have pulmonary arteriovenous malformations. There were 2 late deaths due to chronic congestive heart failure 4 months and 7 years after Kawashima operation. Overall survival at 10 years is 90%. Univariate and multivariate analysis demonstrated presence of bilateral superior vena cavae (p = 0.002) and interval longer than 2 years between Kawashima operation and completion Fontan operation (p = 0.04) as predictors of developing pulmonary arteriovenous malformations. Conclusions. In most patients with heterotaxy and interrupted inferior vena cava with azygous continuation, clinical evidence of pulmonary arteriovenous malformations will develop after Kawashima operation if they are followed up long enough. Early redirection of the hepatic venous effluent to the pulmonary arterial circulation may prevent or lead to regression of pulmonary arteriovenous malformations, with low mortality and morbidity.

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