Surgical strategy for atrioventricular septal defect and tetralogy of fallot or double-outlet right ventricle

Vijayakumar Raju, Harold M. Burkhart, Natalie Rigelman Hedberg, Benjamin W. Eidem, Zhuo Li, Heidi Connolly, Hartzell V Schaff, Joseph A. Dearani

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Abstract

Background: Tetralogy of Fallot, or double-outlet right ventricle with atrioventricular (AV) septal defect (TOF/DORV-AVSD), is rare, with limited long-term data available. We report our institutional experience and outcome over a 50-year period. Methods: From January 1961 to January 2011, 73 patients (50 males [68%]), with a mean age of 6.8 ± 4.4 years (range, 1 month to 35 years), underwent surgical repair of TOF/DORV-AVSD. Symptoms included cyanosis in 50 (69%) and heart failure in 12 (17%). Down syndrome was present in 25 (34%). Rastelli type A, B, and C was seen in 12%, 7%, and 81% of patients, respectively. Moderate or more common AV valve (AVV) regurgitation was present in 40%. Forty-nine patients (67%) had previous palliation, including 36 with a systemic-to-pulmonary arterial shunt. Results: Surgical management included two-ventricle complete repair (CR) in 35 (48%) and single-ventricle (SV) palliation in 38 (52%). Overall, early mortality was 31% for CR and 34% for SV; after 1990, mortality was 6% for CR and 14% for SV. Repair before 1990 (p = 0.008) and the presence of significant common AVV regurgitation (p = 0.016) were univariate risk factors for early death in both groups. Median follow-up was 9.8 years (maximum, 32 years). Late mortality rate was 12% in CR (n = 6) and 18% (n = 9) in SV (p = 0.95). The presence of significant right AVV regurgitation was associated with late death (p = 0.02). Overall survival at 1, 5, and 15 years was 92%, 77%, and 77% in CR, and 83%, 79%, 70% in SV (p = 0.9). Freedom from reoperation at 1, 5, and 15 years was 95%, 85%, 67% in CR and 96%, 91%, 82% in SV (p = 0.1). Reoperations were most common for right ventricular outflow tract pathology, Fontan revision, and AVV intervention. Right AVV regurgitation (p = 0.018) and repair before 1990 (p = 0.041) were risk factors for late reoperation in both groups. Conclusions: Complete repair of TOF/DORV-AVSD is standard of care and associated with low early mortality rate in the current era, with reasonable long-term outcome. SV palliation continues to have significant risk. The presence of AVV regurgitation is a significant risk factor for death and reoperation.

Original languageEnglish (US)
Pages (from-to)2079-2085
Number of pages7
JournalAnnals of Thoracic Surgery
Volume95
Issue number6
DOIs
StatePublished - Jun 2013

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Double Outlet Right Ventricle
Tetralogy of Fallot
Reoperation
Mortality
Cyanosis
Standard of Care
Down Syndrome
Heart Failure
Pathology
Lung
Survival
Atrioventricular Septal Defect

ASJC Scopus subject areas

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

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Surgical strategy for atrioventricular septal defect and tetralogy of fallot or double-outlet right ventricle. / Raju, Vijayakumar; Burkhart, Harold M.; Rigelman Hedberg, Natalie; Eidem, Benjamin W.; Li, Zhuo; Connolly, Heidi; Schaff, Hartzell V; Dearani, Joseph A.

In: Annals of Thoracic Surgery, Vol. 95, No. 6, 06.2013, p. 2079-2085.

Research output: Contribution to journalArticle

Raju, Vijayakumar ; Burkhart, Harold M. ; Rigelman Hedberg, Natalie ; Eidem, Benjamin W. ; Li, Zhuo ; Connolly, Heidi ; Schaff, Hartzell V ; Dearani, Joseph A. / Surgical strategy for atrioventricular septal defect and tetralogy of fallot or double-outlet right ventricle. In: Annals of Thoracic Surgery. 2013 ; Vol. 95, No. 6. pp. 2079-2085.
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title = "Surgical strategy for atrioventricular septal defect and tetralogy of fallot or double-outlet right ventricle",
abstract = "Background: Tetralogy of Fallot, or double-outlet right ventricle with atrioventricular (AV) septal defect (TOF/DORV-AVSD), is rare, with limited long-term data available. We report our institutional experience and outcome over a 50-year period. Methods: From January 1961 to January 2011, 73 patients (50 males [68{\%}]), with a mean age of 6.8 ± 4.4 years (range, 1 month to 35 years), underwent surgical repair of TOF/DORV-AVSD. Symptoms included cyanosis in 50 (69{\%}) and heart failure in 12 (17{\%}). Down syndrome was present in 25 (34{\%}). Rastelli type A, B, and C was seen in 12{\%}, 7{\%}, and 81{\%} of patients, respectively. Moderate or more common AV valve (AVV) regurgitation was present in 40{\%}. Forty-nine patients (67{\%}) had previous palliation, including 36 with a systemic-to-pulmonary arterial shunt. Results: Surgical management included two-ventricle complete repair (CR) in 35 (48{\%}) and single-ventricle (SV) palliation in 38 (52{\%}). Overall, early mortality was 31{\%} for CR and 34{\%} for SV; after 1990, mortality was 6{\%} for CR and 14{\%} for SV. Repair before 1990 (p = 0.008) and the presence of significant common AVV regurgitation (p = 0.016) were univariate risk factors for early death in both groups. Median follow-up was 9.8 years (maximum, 32 years). Late mortality rate was 12{\%} in CR (n = 6) and 18{\%} (n = 9) in SV (p = 0.95). The presence of significant right AVV regurgitation was associated with late death (p = 0.02). Overall survival at 1, 5, and 15 years was 92{\%}, 77{\%}, and 77{\%} in CR, and 83{\%}, 79{\%}, 70{\%} in SV (p = 0.9). Freedom from reoperation at 1, 5, and 15 years was 95{\%}, 85{\%}, 67{\%} in CR and 96{\%}, 91{\%}, 82{\%} in SV (p = 0.1). Reoperations were most common for right ventricular outflow tract pathology, Fontan revision, and AVV intervention. Right AVV regurgitation (p = 0.018) and repair before 1990 (p = 0.041) were risk factors for late reoperation in both groups. Conclusions: Complete repair of TOF/DORV-AVSD is standard of care and associated with low early mortality rate in the current era, with reasonable long-term outcome. SV palliation continues to have significant risk. The presence of AVV regurgitation is a significant risk factor for death and reoperation.",
author = "Vijayakumar Raju and Burkhart, {Harold M.} and {Rigelman Hedberg}, Natalie and Eidem, {Benjamin W.} and Zhuo Li and Heidi Connolly and Schaff, {Hartzell V} and Dearani, {Joseph A.}",
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T1 - Surgical strategy for atrioventricular septal defect and tetralogy of fallot or double-outlet right ventricle

AU - Raju, Vijayakumar

AU - Burkhart, Harold M.

AU - Rigelman Hedberg, Natalie

AU - Eidem, Benjamin W.

AU - Li, Zhuo

AU - Connolly, Heidi

AU - Schaff, Hartzell V

AU - Dearani, Joseph A.

PY - 2013/6

Y1 - 2013/6

N2 - Background: Tetralogy of Fallot, or double-outlet right ventricle with atrioventricular (AV) septal defect (TOF/DORV-AVSD), is rare, with limited long-term data available. We report our institutional experience and outcome over a 50-year period. Methods: From January 1961 to January 2011, 73 patients (50 males [68%]), with a mean age of 6.8 ± 4.4 years (range, 1 month to 35 years), underwent surgical repair of TOF/DORV-AVSD. Symptoms included cyanosis in 50 (69%) and heart failure in 12 (17%). Down syndrome was present in 25 (34%). Rastelli type A, B, and C was seen in 12%, 7%, and 81% of patients, respectively. Moderate or more common AV valve (AVV) regurgitation was present in 40%. Forty-nine patients (67%) had previous palliation, including 36 with a systemic-to-pulmonary arterial shunt. Results: Surgical management included two-ventricle complete repair (CR) in 35 (48%) and single-ventricle (SV) palliation in 38 (52%). Overall, early mortality was 31% for CR and 34% for SV; after 1990, mortality was 6% for CR and 14% for SV. Repair before 1990 (p = 0.008) and the presence of significant common AVV regurgitation (p = 0.016) were univariate risk factors for early death in both groups. Median follow-up was 9.8 years (maximum, 32 years). Late mortality rate was 12% in CR (n = 6) and 18% (n = 9) in SV (p = 0.95). The presence of significant right AVV regurgitation was associated with late death (p = 0.02). Overall survival at 1, 5, and 15 years was 92%, 77%, and 77% in CR, and 83%, 79%, 70% in SV (p = 0.9). Freedom from reoperation at 1, 5, and 15 years was 95%, 85%, 67% in CR and 96%, 91%, 82% in SV (p = 0.1). Reoperations were most common for right ventricular outflow tract pathology, Fontan revision, and AVV intervention. Right AVV regurgitation (p = 0.018) and repair before 1990 (p = 0.041) were risk factors for late reoperation in both groups. Conclusions: Complete repair of TOF/DORV-AVSD is standard of care and associated with low early mortality rate in the current era, with reasonable long-term outcome. SV palliation continues to have significant risk. The presence of AVV regurgitation is a significant risk factor for death and reoperation.

AB - Background: Tetralogy of Fallot, or double-outlet right ventricle with atrioventricular (AV) septal defect (TOF/DORV-AVSD), is rare, with limited long-term data available. We report our institutional experience and outcome over a 50-year period. Methods: From January 1961 to January 2011, 73 patients (50 males [68%]), with a mean age of 6.8 ± 4.4 years (range, 1 month to 35 years), underwent surgical repair of TOF/DORV-AVSD. Symptoms included cyanosis in 50 (69%) and heart failure in 12 (17%). Down syndrome was present in 25 (34%). Rastelli type A, B, and C was seen in 12%, 7%, and 81% of patients, respectively. Moderate or more common AV valve (AVV) regurgitation was present in 40%. Forty-nine patients (67%) had previous palliation, including 36 with a systemic-to-pulmonary arterial shunt. Results: Surgical management included two-ventricle complete repair (CR) in 35 (48%) and single-ventricle (SV) palliation in 38 (52%). Overall, early mortality was 31% for CR and 34% for SV; after 1990, mortality was 6% for CR and 14% for SV. Repair before 1990 (p = 0.008) and the presence of significant common AVV regurgitation (p = 0.016) were univariate risk factors for early death in both groups. Median follow-up was 9.8 years (maximum, 32 years). Late mortality rate was 12% in CR (n = 6) and 18% (n = 9) in SV (p = 0.95). The presence of significant right AVV regurgitation was associated with late death (p = 0.02). Overall survival at 1, 5, and 15 years was 92%, 77%, and 77% in CR, and 83%, 79%, 70% in SV (p = 0.9). Freedom from reoperation at 1, 5, and 15 years was 95%, 85%, 67% in CR and 96%, 91%, 82% in SV (p = 0.1). Reoperations were most common for right ventricular outflow tract pathology, Fontan revision, and AVV intervention. Right AVV regurgitation (p = 0.018) and repair before 1990 (p = 0.041) were risk factors for late reoperation in both groups. Conclusions: Complete repair of TOF/DORV-AVSD is standard of care and associated with low early mortality rate in the current era, with reasonable long-term outcome. SV palliation continues to have significant risk. The presence of AVV regurgitation is a significant risk factor for death and reoperation.

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