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Pulmonary atresia with intact ventricular septum: management of, and outcomes for, a cohort of 210 consecutive patients

Published online by Cambridge University Press:  21 January 2005

Umesh Dyamenahalli ,
Brian W. McCrindle ,
Cathy McDonald ,
Kalyani R. Trivedi ,
Jeffrey F. Smallhorn ,
Lee N. Benson ,
John Coles ,
William G. Williams  and
Robert M. Freedom
Umesh Dyamenahalli
Affiliation:
Departments of Pediatrics, Cardiovascular Surgery, Pathology and Diagnostic Imaging, Divisions of Cardiology and Cardiovascular Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Canada
Brian W. McCrindle
Affiliation:
Departments of Pediatrics, Cardiovascular Surgery, Pathology and Diagnostic Imaging, Divisions of Cardiology and Cardiovascular Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Canada
Cathy McDonald
Affiliation:
Departments of Pediatrics, Cardiovascular Surgery, Pathology and Diagnostic Imaging, Divisions of Cardiology and Cardiovascular Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Canada
Kalyani R. Trivedi
Affiliation:
Departments of Pediatrics, Cardiovascular Surgery, Pathology and Diagnostic Imaging, Divisions of Cardiology and Cardiovascular Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Canada
Jeffrey F. Smallhorn
Affiliation:
Departments of Pediatrics, Cardiovascular Surgery, Pathology and Diagnostic Imaging, Divisions of Cardiology and Cardiovascular Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Canada
Lee N. Benson
Affiliation:
Departments of Pediatrics, Cardiovascular Surgery, Pathology and Diagnostic Imaging, Divisions of Cardiology and Cardiovascular Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Canada
John Coles
Affiliation:
Departments of Pediatrics, Cardiovascular Surgery, Pathology and Diagnostic Imaging, Divisions of Cardiology and Cardiovascular Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Canada
William G. Williams
Affiliation:
Departments of Pediatrics, Cardiovascular Surgery, Pathology and Diagnostic Imaging, Divisions of Cardiology and Cardiovascular Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Canada
Robert M. Freedom
Affiliation:
Departments of Pediatrics, Cardiovascular Surgery, Pathology and Diagnostic Imaging, Divisions of Cardiology and Cardiovascular Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Canada
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Abstract

Objectives: We sought to determine trends, and outcomes, for a cohort of patients with pulmonary atresia with intact ventricular septum born between 1965 and 1998. Background: Pulmonary atresia with intact ventricular septum is a complex lesion that remains a therapeutic challenge, particularly regarding the suitability for biventricular repair. Methods: We identified 210 consecutive patients, and reviewed their medical records, initial angiograms, and echocardiograms, along with the relevant surgical and pathology reports. Results: The mean initial Z-score for the diameter of the tricuspid valve was −0.99 ± 1.95, with Ebstein's malformation in 8%. A right ventricular dependent coronary arterial circulation was found in 23%. The proportion of patients who received treatment increased over time, although placement of an arterial shunt was the predominant initial procedure throughout the experience. At the last follow-up, 107 patients had not reached the planned final stage of their repair, and 79% of these had died. Of the 103 reaching the final stage of planned repair, 58 had undergone attempted biventricular repair, with 34% dying; 14 had undergone attempted one and a half ventricular repair, with 7% dying, and 31 had undergone attempted functionally univentricular repair, with 10% dying. Overall, survival was 57% at the age of 1 year, 48% at 5 years, and 43% at 10 years. Survival improved over time, with survival of 75% at 1 year, and 67% at 5 years, for patients born between 1992 and 1998. An earlier date of birth, the presence of Ebstein's malformation, and prematurity were all significant independent factors associated with decreased survival. A greater severity of coronary arterial abnormalities was significantly associated with a greater likelihood of left ventricular dysfunction during follow-up. Conclusions: The outcomes for patients born with pulmonary atresia with intact ventricular septum have improved over time, albeit that careful initial management, and better selection, is still indicated for those planned to undergo biventricular repair.

Keywords

Hypoplastic right ventriclenatural historycongenital heart disease
Type
Original Article
Information
Cardiology in the Young , Volume 14 , Issue 3 , June 2004 , pp. 299 - 308
Copyright
© 2004 Cambridge University Press

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References

Daubeney PEF, Delany DJ, Anderson RH, et al. Pulmonary atresia with intact ventricular septum range of morphology in a population-based study. J Am Coll Cardiol 2002; 39: 1670–1679.Google Scholar
Lightfoot NE, Coles JG, Dasmahapatra HK, et al. Analysis of survival in patients with pulmonary atresia and intact ventricular septum treated surgically. Int J Cardiol 1989; 24: 159–164.Google Scholar
Coles JG, Freedom RM, Lightfoot NE, et al. Long-term results in neonates with pulmonary atresia and intact ventricular septum. Ann Thorac Surg 1989; 47: 213–217.Google Scholar
Bull C, Kostelka M, Sorensen K, de Leval M. Outcome measures for the neonatal management of pulmonary atresia with intact ventricular septum. J Thorac Cardiovasc Surg 1994; 107: 359–366.Google Scholar
Jahangiri M, Zurakowski D, Bichell D, Mayer JE, del Nido PJ, Jonas RA. Improved results with selective management in pulmonary atresia with intact ventricular septum. J Thorac Cardiovasc Surg 1999; 118: 1046–1055.Google Scholar
Laks H, Plunkett M. Surgical management of pulmonary atresia with intact ventricular septum. Prog Pediat Cardiol 2001; 13: 183–197.Google Scholar
Powell AJ, Mayer JE, Lang P, Lock JE. Outcome in infants with pulmonary atresia, intact ventricular septum, and right ventricle-dependent coronary circulation. Am J Cardiol 2000; 86: 1272–1274.Google Scholar
Giglia TM, Jenkins KJ, Matitiau A, et al. Influence of right heart size on outcome in pulmonary atresia with intact ventricular septum. Circulation 1993; 88: 2248–2256.Google Scholar
Hanley FL, Sade RM, Blackstone EH, Kirklin JW, Freedom RM, Nanda NC. Outcomes in neonatal pulmonary atresia with intact ventricular septum. A multiinstitutional study. J Thorac Cardiovasc Surg 1993; 105: 406–427.Google Scholar
Rychik J, Levy H, Gaynor JW, DeCampli WM, Spray TL. Outcome after operations for pulmonary atresia with intact ventricular septum. J Thorac Cardiovasc Surg 1998; 116: 924–931.Google Scholar
Williams WG, Burrows P, Freedom RM, et al. Thromboexclusion of the right ventricle in children with pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg 1991; 101: 222–229.Google Scholar
Freedom RM. The morphologic variations of pulmonary atresia with intact ventricular septum: guidelines for surgical intervention. Pediatr Cardiol 1983; 4: 183–188.Google Scholar
Freedom R. How can something so small cause so much grief? Some thoughts about the underdeveloped right ventricle in pulmonary atresia and intact ventricular septum. J Am Coll Cardiol 1992; 19: 1038–1040.Google Scholar
Laks H, Gates RN, Grant PW, Drant S, Allada V, Harake B. Aortic to right ventricular shunt for pulmonary atresia and intact ventricular septum. Ann Thorac Surg 1995; 59: 342–347.Google Scholar
Dyamenahalli U, Hanna BD, Sharratt GP. Pulmonary atresia with intact ventricular septum: management of the coronary arterial anomalies. Cardiol Young 1997; 7: 80–87.Google Scholar
Miyaji K, Shimada M, Sekiguchi A, Ishizawa A, Isoda T, Tsunemoto M. Pulmonary atresia with intact ventricular septum: long-term results of “one and a half ventricular repair”. Ann Thorac Surg 1995; 60: 1762–1764.Google Scholar
Mair DD, Julsrud PR, Puga FJ, Danielson GK. The Fontan procedure for pulmonary atresia with intact ventricular septum: operative and late results. J Am Coll Cardiol 1997; 29: 1359–1364.Google Scholar
Najm HK, Williams WG, Coles JG, Rebeyka IM, Freedom RM. Pulmonary atresia with intact ventricular septum: results of the Fontan procedure. Ann Thorac Surg 1997; 63: 669–675.Google Scholar
Shimpo H, Hayakawa H, Miyake Y, Takabayashi S, Yada I. Strategy for pulmonary atresia and intact ventricular septum. Ann Thorac Surg 2000; 70: 287–289.Google Scholar
Trivedi KR, Azakie A, Benson LN. Collaborative interventional and surgical strategies in the management of congenital heart lesions. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2001; 4: 185–207.Google Scholar
Minich LL, Tani LY, Ritter S, Williams RV, Shaddy RE, Hawkins JA. Usefulness of the preoperative tricuspid/mitral valve ratio for predicting outcome in pulmonary atresia with intact ventricular septum. Am J Cardiol 2000; 85: 1325–1328.Google Scholar
Giglia TM, Mandell VS, Connor AR, Mayer JE Jr, Lock JE. Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum. Circulation 1992; 86: 1516–1528.Google Scholar
Satou GM, Perry SB, Gauvreau K, Geva T. Echocardiographic predictors of coronary artery pathology in pulmonary atresia with intact ventricular septum. Am J Cardiol 2000; 85: 1319–1324.Google Scholar
Rowlatt JF, Rimoldi M, Lev M. The quantitative anatomy of the normal child’s heart. Pediatr Clin North Am 1963; 10: 499–588.Google Scholar
Daubeney PEF, Blackstone EH, Weintraub RG, Slavik Z, Scanlon J, Webber SA. Relationship of the dimension of cardiac structures to body size: an echocardiographic study in normal infants and children. Cardiol Young 1999; 9: 402–410.Google Scholar
Bull C, de Leval MR, Mercanti C, Macartney FJ, Anderson RH. Pulmonary atresia and intact ventricular septum: a revised classification. Circulation 1982; 66: 266–272.Google Scholar
Anderson RH, Anderson C, Zuberbuhler JR. Further morphologic studies on hearts with pulmonary atresia and intact ventricular septum. Cardiol Young 1991; 1: 105–113.Google Scholar
Gittenberger-de Groot AC, Sauer U, Bindl L, Babic R, Essed CE, Buhlmeyer K. Competition of coronary arteries and ventriculo-coronary arterial communications in pulmonary atresia with intact ventricular septum. Int J Cardiol 1988; 18: 243–258.Google Scholar
Freedom RM, Mawson JB, Yoo S-J, Benson LN. Pulmonary atresia and intact ventricular septum. In: Congenital Heart Disease: Textbook of Angiocardiography. Armonk, NY, Futura, 1997, pp 617–662.
Blackstone EH. Outcome analysis using hazard function methodology. Ann Thorac Surg 1996; 61 (Suppl 2): S2–S7.Google Scholar
Freedom RM, Lock J, Bricker JT. Pediatric cardiology and cardiovascular surgery: 1950–2000. Circulation 2000; 102 (Suppl 4): IV58–IV68.Google Scholar
Van Arsdell GS. One and a half ventricle repairs. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2000; 3: 173–178.Google Scholar
de Leval M, Bull C, Stark J, Anderson RH, Taylor JF, Macartney FJ. Pulmonary atresia and intact ventricular septum: surgical management based on a revised classification. Circulation 1982; 66: 272–280.Google Scholar
Mainwaring RD, Lamberti JJ. Pulmonary atresia with intact ventricular septum. Surgical approach based on ventricular size and coronary anatomy. J Thorac Cardiovasc Surg 1993; 106: 733–738.Google Scholar
Pawade A, Capuani A, Penny DJ, Karl TR, Mee RB. Pulmonary atresia with intact ventricular septum: surgical management based on right ventricular infundibulum. J Card Surg 1993; 8: 371–383.Google Scholar
Starnes VA, Pitlick PT, Bernstein D, Griffin ML, Choy M, Shumway NE. Ebstein’s anomaly appearing in the neonate. A new surgical approach. J Thorac Cardiovasc Surg 1991; 101: 1082–1087.Google Scholar
Maeno YV, Boutin C, Hornberger LK, et al. Prenatal diagnosis of right ventricular outflow tract obstruction with intact ventricular septum, and detection of ventriculocoronary connections. Heart 1999; 81: 661–668.Google Scholar
Daubeney PE, Sharland GK, Cook AC, Keeton BR, Anderson RH, Webber SA. Pulmonary atresia with intact ventri-cular septum: impact of fetal echocardiography on incidence at birth and postnatal outcome. UK and Eire Collaborative Study of Pulmonary Atresia with Intact Ventricular Septum. Circulation 1998; 98: 562–566.Google Scholar
Fyfe DA, Edwards WD, Driscoll DJ. Myocardial ischemia in patients with pulmonary atresia and intact ventricular septum. J Am Coll Cardiol 1986; 8: 402–406.Google Scholar
Akiba T, Becker AE. Disease of the left ventricle in pulmonary atresia with intact ventricular septum. The limiting factor for long-lasting successful surgical intervention? J Thorac Cardiovasc Surg 1994; 108: 1–8.Google Scholar