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Outcome in infants with pulmonary atresia, intact ventricular septum, and right ventricle–dependent coronary circulation

      Patients with pulmonary atresia and intact ventricular septum (PA/IVS) have right ventricle-to-coronary artery fistulous communications in 30% to 60% of cases,
      • Calder A.L.
      • Co E.E.
      • Sage M.D.
      Coronary arterial abnormalities in pulmonary atresia with intact ventricular septum.
      ,
      • Coles J.G.
      • Freedom R.M.
      • Lightfoot N.E.
      • Dasmahapatra H.K.
      • Williams W.G.
      • Trusler G.A.
      • Burrows P.E.
      Long-term results in neonates with pulmonary atresia and intact ventricular septum.
      ,
      • Hanley F.L.
      • Sade R.M.
      • Blackstone E.H.
      • Kirklin J.W.
      • Freedom R.M.
      • Nanda N.C.
      Outcomes in neonatal pulmonary atresia with intact ventricular septum. A multiinstitutional study.
      ,
      • Fyfe D.A.
      • Edwards W.D.
      • Driscoll D.J.
      Myocardial ischemia in patients with pulmonary atresia and intact ventricular septum.
      and a subset of these patients develop coronary artery obstructive lesions.
      • Freedom R.M.
      • Wilson G.
      • Trusler G.A.
      • Williams W.G.
      • Rowe R.D.
      Pulmonary atresia and intact ventricular septum.
      In such cases, a portion of left ventricular myocardium may have a right ventricle–dependent coronary circulation (RVDCC) via the coronary artery fistulas.
      • Coles J.G.
      • Freedom R.M.
      • Lightfoot N.E.
      • Dasmahapatra H.K.
      • Williams W.G.
      • Trusler G.A.
      • Burrows P.E.
      Long-term results in neonates with pulmonary atresia and intact ventricular septum.
      Surgical repair by establishment of right ventricle-to-pulmonary artery continuity can be fatal when the resulting reduction in right ventricular pressure significantly compromises myocardial perfusion.
      • Coles J.G.
      • Freedom R.M.
      • Lightfoot N.E.
      • Dasmahapatra H.K.
      • Williams W.G.
      • Trusler G.A.
      • Burrows P.E.
      Long-term results in neonates with pulmonary atresia and intact ventricular septum.
      ,
      • O’Connor W.N.
      • Cottrill C.M.
      • Johnson G.L.
      • Noonan J.A.
      • Todd E.P.
      Pulmonary atresia with intact ventricular septum and ventriculocoronary communications surgical significance.
      ,
      • Giglia T.M.
      • Mandell V.S.
      • Connor A.R.
      • Mayer J.E.J.
      • Lock J.E.
      Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum.
      Management options for infants with RVDCC include aortopulmonary shunts, staged surgery for single ventricle palliation, aorta-to-right ventricle shunts, and cardiac transplantation, although outcome data in this rare condition are limited.
      • Hanley F.L.
      • Sade R.M.
      • Blackstone E.H.
      • Kirklin J.W.
      • Freedom R.M.
      • Nanda N.C.
      Outcomes in neonatal pulmonary atresia with intact ventricular septum. A multiinstitutional study.
      ,
      • Rychik J.
      • Levy H.
      • Gaynor J.W.
      • DeCampli W.M.
      • Spray T.L.
      Outcome after operations for pulmonary atresia with intact ventricular septum.
      ,
      • Pearl J.M.
      • Laks H.
      • Stein D.G.
      • Drinkwater D.C.
      • George B.L.
      • Williams R.G.
      Total cavopulmonary anastomosis versus conventional modified Fontan procedure.
      ,
      • Laks H.
      • Gates R.N.
      • Grant P.W.
      • Drant S.
      • Allada V.
      • Harake B.
      Aortic to right ventricular shunt for pulmonary atresia and intact ventricular septum [see comments].
      ,
      • Freeman J.E.
      • DeLeon S.Y.
      • Lai S.
      • Fisher E.A.
      • Ow E.P.
      • Pifarre R.
      Right ventricle-to-aorta conduit in pulmonary atresia with intact ventricular septum and coronary sinusoids.
      ,
      • Hawkins J.A.
      • Thorne J.K.
      • Boucek M.M.
      • Orsmond G.S.
      • Ruttenberg H.D.
      • Veasy L.G.
      • McGough E.C.
      Early and late results in pulmonary atresia and intact ventricular septum.
      Since 1986, we have pursued staged surgical palliation directed toward a Fontan procedure for all patients with PA/IVS and RVDCC. We now report intermediate-term follow-up data for this cohort.
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