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

2000; Elsevier BV; Volume: 86; Issue: 11 Linguagem: Inglês

10.1016/s0002-9149(00)01220-0

1879-1913

Andrew J. Powell, John E. Mayer, Peter Lang, James E. Lock,

Cardiac Structural Anomalies and Repair

Patients with pulmonary atresia and intact ventricular septum (PA/IVS) have right ventricle-to-coronary artery fistulous communications in 30% to 60% of cases, 1 Calder A.L. Co E.E. Sage M.D. Coronary arterial abnormalities in pulmonary atresia with intact ventricular septum. Am J Cardiol. 1987; 59: 436-442 Abstract Full Text PDF PubMed Scopus (94) Google Scholar , 2 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. Ann Thorac Surg. 1989; 47: 213-217 Abstract Full Text PDF PubMed Scopus (95) Google Scholar , 3 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. J Thorac Cardiovasc Surg. 1993; 105: 406-423 PubMed Google Scholar , 4 Fyfe D.A. Edwards W.D. Driscoll D.J. Myocardial ischemia in patients with pulmonary atresia and intact ventricular septum. J Am Coll Cardiol. 1986; 8: 402-406 Abstract Full Text PDF PubMed Scopus (58) Google Scholar and a subset of these patients develop coronary artery obstructive lesions. 5 Freedom R.M. Wilson G. Trusler G.A. Williams W.G. Rowe R.D. Pulmonary atresia and intact ventricular septum. Scand J Thorac Cardiovasc Surg. 1983; 17: 1-28 Crossref PubMed Scopus (54) Google Scholar In such cases, a portion of left ventricular myocardium may have a right ventricle–dependent coronary circulation (RVDCC) via the coronary artery fistulas. 2 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. Ann Thorac Surg. 1989; 47: 213-217 Abstract Full Text PDF PubMed Scopus (95) Google Scholar 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. 2 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. Ann Thorac Surg. 1989; 47: 213-217 Abstract Full Text PDF PubMed Scopus (95) Google Scholar , 6 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. Circulation. 1982; 65: 805-809 Crossref PubMed Scopus (59) Google Scholar , 7 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. Circulation. 1992; 86: 1516-1528 Crossref PubMed Scopus (146) Google Scholar 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. 3 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. J Thorac Cardiovasc Surg. 1993; 105: 406-423 PubMed Google Scholar , 8 Rychik J. Levy H. Gaynor J.W. DeCampli W.M. Spray T.L. Outcome after operations for pulmonary atresia with intact ventricular septum. J Thorac Cardiovasc Surg. 1998; 116: 924-931 Abstract Full Text Full Text PDF PubMed Scopus (93) Google Scholar , 9 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. Ann Thorac Surg. 1991; 52: 189-196 Abstract Full Text PDF PubMed Scopus (78) Google Scholar , 10 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]. Ann Thorac Surg. 1995; 59: 342-347 Abstract Full Text PDF PubMed Scopus (29) Google Scholar , 11 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. Ann Thorac Surg. 1993; 56: 1393-1395 Abstract Full Text PDF PubMed Scopus (20) Google Scholar , 12 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. J Thorac Cardiovasc Surg. 1990; 100: 492-497 PubMed Google Scholar 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.