Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: Experience with 85 patients

V. Mohan Reddy, Doff B. McElhinney, Zahid Amin, Phillip Moore, Andrew J. Parry, David F. Teitel, Frank L. Hanley

Research output: Contribution to journalArticle

145 Citations (Scopus)

Abstract

Background - Pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCAs) is a complex lesion with marked heterogeneity of pulmonary blood supply. Traditional management has involved staged unifocalization of pulmonary blood supply. Our approach has been to perform early 1-stage complete unifocalization in almost all patients. Methods and Results - Since 1992, 85 patients with pulmonary atresia, VSD, and MAPCAs have undergone unifocalization (median age, 7 months). Complete 1- stage unifocalization and intracardiac repair were performed through a midline approach in 56 patients, whereas 23 underwent unifocalization in a single stage with the VSD left open, and 6 underwent staged unifocalization through sequential thoracotomies. There were 9 early deaths. During follow-up (1 to 69 months), there were 7 late deaths. Actuarial survival was 80% at 3 years. Among early survivors, actuarial survival with complete repair was 88% at 2 years. Reintervention on the neo-pulmonary arteries was performed in 24 patients. Conclusions - Early 1-stage complete unifocalization can be performed in >90% of patients with pulmonary atresia and MAPCAs, even those with absent true pulmonary arteries, and yields good functional results. Complete repair during the same operation is achieved in two thirds of patients. There remains room for improvement; actuarial survival 3 years after surgery is 80%, and there is a significant rate of reintervention. These results must be appreciated within the context of the natural history of this lesion: 65% of patients survive to 1 year of age and slightly >50% survive to 2 years even with surgical intervention.

Original languageEnglish (US)
Pages (from-to)1826-1832
Number of pages7
JournalCirculation
Volume101
Issue number15
DOIs
StatePublished - Apr 18 2000
Externally publishedYes

Fingerprint

Arteries
Pulmonary Artery
Survival
Pulmonary Atresia
Lung
Ventricular Heart Septal Defects
Thoracotomy
Pulmonary Atresia With Ventricular Septal Defect
Natural History
Survivors

Keywords

  • Heart defects, congenital
  • Surgery
  • Survival

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Cite this

Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries : Experience with 85 patients. / Reddy, V. Mohan; McElhinney, Doff B.; Amin, Zahid; Moore, Phillip; Parry, Andrew J.; Teitel, David F.; Hanley, Frank L.

In: Circulation, Vol. 101, No. 15, 18.04.2000, p. 1826-1832.

Research output: Contribution to journalArticle

Reddy, V. Mohan ; McElhinney, Doff B. ; Amin, Zahid ; Moore, Phillip ; Parry, Andrew J. ; Teitel, David F. ; Hanley, Frank L. / Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries : Experience with 85 patients. In: Circulation. 2000 ; Vol. 101, No. 15. pp. 1826-1832.
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abstract = "Background - Pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCAs) is a complex lesion with marked heterogeneity of pulmonary blood supply. Traditional management has involved staged unifocalization of pulmonary blood supply. Our approach has been to perform early 1-stage complete unifocalization in almost all patients. Methods and Results - Since 1992, 85 patients with pulmonary atresia, VSD, and MAPCAs have undergone unifocalization (median age, 7 months). Complete 1- stage unifocalization and intracardiac repair were performed through a midline approach in 56 patients, whereas 23 underwent unifocalization in a single stage with the VSD left open, and 6 underwent staged unifocalization through sequential thoracotomies. There were 9 early deaths. During follow-up (1 to 69 months), there were 7 late deaths. Actuarial survival was 80{\%} at 3 years. Among early survivors, actuarial survival with complete repair was 88{\%} at 2 years. Reintervention on the neo-pulmonary arteries was performed in 24 patients. Conclusions - Early 1-stage complete unifocalization can be performed in >90{\%} of patients with pulmonary atresia and MAPCAs, even those with absent true pulmonary arteries, and yields good functional results. Complete repair during the same operation is achieved in two thirds of patients. There remains room for improvement; actuarial survival 3 years after surgery is 80{\%}, and there is a significant rate of reintervention. These results must be appreciated within the context of the natural history of this lesion: 65{\%} of patients survive to 1 year of age and slightly >50{\%} survive to 2 years even with surgical intervention.",
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T2 - Experience with 85 patients

AU - Reddy, V. Mohan

AU - McElhinney, Doff B.

AU - Amin, Zahid

AU - Moore, Phillip

AU - Parry, Andrew J.

AU - Teitel, David F.

AU - Hanley, Frank L.

PY - 2000/4/18

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N2 - Background - Pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCAs) is a complex lesion with marked heterogeneity of pulmonary blood supply. Traditional management has involved staged unifocalization of pulmonary blood supply. Our approach has been to perform early 1-stage complete unifocalization in almost all patients. Methods and Results - Since 1992, 85 patients with pulmonary atresia, VSD, and MAPCAs have undergone unifocalization (median age, 7 months). Complete 1- stage unifocalization and intracardiac repair were performed through a midline approach in 56 patients, whereas 23 underwent unifocalization in a single stage with the VSD left open, and 6 underwent staged unifocalization through sequential thoracotomies. There were 9 early deaths. During follow-up (1 to 69 months), there were 7 late deaths. Actuarial survival was 80% at 3 years. Among early survivors, actuarial survival with complete repair was 88% at 2 years. Reintervention on the neo-pulmonary arteries was performed in 24 patients. Conclusions - Early 1-stage complete unifocalization can be performed in >90% of patients with pulmonary atresia and MAPCAs, even those with absent true pulmonary arteries, and yields good functional results. Complete repair during the same operation is achieved in two thirds of patients. There remains room for improvement; actuarial survival 3 years after surgery is 80%, and there is a significant rate of reintervention. These results must be appreciated within the context of the natural history of this lesion: 65% of patients survive to 1 year of age and slightly >50% survive to 2 years even with surgical intervention.

AB - Background - Pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCAs) is a complex lesion with marked heterogeneity of pulmonary blood supply. Traditional management has involved staged unifocalization of pulmonary blood supply. Our approach has been to perform early 1-stage complete unifocalization in almost all patients. Methods and Results - Since 1992, 85 patients with pulmonary atresia, VSD, and MAPCAs have undergone unifocalization (median age, 7 months). Complete 1- stage unifocalization and intracardiac repair were performed through a midline approach in 56 patients, whereas 23 underwent unifocalization in a single stage with the VSD left open, and 6 underwent staged unifocalization through sequential thoracotomies. There were 9 early deaths. During follow-up (1 to 69 months), there were 7 late deaths. Actuarial survival was 80% at 3 years. Among early survivors, actuarial survival with complete repair was 88% at 2 years. Reintervention on the neo-pulmonary arteries was performed in 24 patients. Conclusions - Early 1-stage complete unifocalization can be performed in >90% of patients with pulmonary atresia and MAPCAs, even those with absent true pulmonary arteries, and yields good functional results. Complete repair during the same operation is achieved in two thirds of patients. There remains room for improvement; actuarial survival 3 years after surgery is 80%, and there is a significant rate of reintervention. These results must be appreciated within the context of the natural history of this lesion: 65% of patients survive to 1 year of age and slightly >50% survive to 2 years even with surgical intervention.

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KW - Surgery

KW - Survival

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