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The Journal of Thoracic and Cardiovascular Surgery, Vol 105, 406-423, Copyright © 1993 by The American Association for Thoracic Surgery and The Western Thoracic Surgical Association
FL Hanley, RM Sade, EH Blackstone, JW Kirklin, RM Freedom and NC Nanda
A total of 171 neonates with pulmonary atresia and intact ventricular
septum were entered into a prospective multiinstitutional study between
January 1, 1987, and January 1, 1991. Treatment was not assigned randomly
but was selected by the responsible physicians. The Z-value (standard
deviation units) of the diameter of the tricuspid valve was less than -2 in
52% of patients and less than -4 in 26%; it was highly correlated with
right ventricular cavity size (which was small in 90% of patients and was
severely reduced in 54%). Coronary artery-right ventricular fistulas were
present in 45% of patients, and right ventricular dependency was severe in
9%; diameter (Z-value) of the tricuspid valve was negatively correlated (P
< 0.0001) with the prevalence of both. Survival was 81% at 1 month after
the first intervention and 64% at 4 years; the hazard function
(instantaneous risk of death) declined rapidly after the initial procedure
but remained appreciable for 24 months. Multivariable analysis showed small
diameter of the tricuspid valve, severe right ventricular coronary
dependency, birth weight, and the date and type of initial procedure to be
risk factors for time-related death. Subsequent procedures were performed
in 51% of patients. Among patients undergoing an initial procedure that did
not include a systemic-pulmonary artery shunt, only 49% did not receive a
shunt in the subsequent 1 month; small size of the tricuspid valve was the
only risk factor for receiving the subsequent shunt. Ninety-eight percent
of living patients whose initial procedure did not include a transannular
patch were free of a subsequently placed transannular patch within 1 month,
but only 45% were free of it 3 years later; no risk factors were
identified. Eighteen percent of living patients had received a
one-ventricle repair within 3 years, and 32% had received a two-ventricle
repair; the remainder (50%) had incompletely separated pulmonary and
systemic circulations. The only patient-specific risk factor for not
receiving a two-ventricle repair was the Z-value of the tricuspid valve.
ARTICLES
Outcomes in neonatal pulmonary atresia with intact ventricular septum. A multiinstitutional study
Department of Cardiac Surgery, Children's Hospital, Harvard Medical School, Boston, MA.
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J. Rychik, H. Levy, J. W. Gaynor, W. M. DeCampli, and T. L. Spray OUTCOME AFTER OPERATIONS FOR PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM J. Thorac. Cardiovasc. Surg., December 1, 1998; 116(6): 924 - 931. [Abstract] [Full Text] [PDF]
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M. L. Jacobs, E. H. Blackstone, L. L. Bailey, and The Congenital Heart Surgeons Society INTERMEDIATE SURVIVAL IN NEONATES WITH AORTIC ATRESIA: AMULTI-INSTITUTIONAL STUDY J. Thorac. Cardiovasc. Surg., September 1, 1998; 116(3): 417 - 431. [Abstract] [Full Text]
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R. M. Freedom and G. S. Van Arsdell Biventricular hearts not amenable to biventricular repair Ann. Thorac. Surg., August 1, 1998; 66(2): 641 - 643. [Abstract] [Full Text] [PDF]
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G. S. Van Arsdell, W. G. Williams, and R. M. Freedom A practical approach to 11/2 ventricle repairs Ann. Thorac. Surg., August 1, 1998; 66(2): 678 - 680. [Abstract] [Full Text] [PDF]
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V. M. Reddy, D. B. McElhinney, N. H. Silverman, S. M. Marianeschi, and F. L. Hanley Partial biventricular repair for complex congenital heart defects: an intermediate option for complicated anatomy or functionally borderline right complex heart J. Thorac. Cardiovasc. Surg., July 1, 1998; 116(1): 21 - 25. [Abstract] [Full Text]
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C. Ovaert, S. A. Qureshi, E. Rosenthal, E. J. Baker, and M. Tynan Growth of the right ventricle after successful transcatheterpulmonary valvotomy in neonates and infants with pulmonary atresia and intactventricular septum J. Thorac. Cardiovasc. Surg., May 1, 1998; 115(5): 1055 - 1059. [Abstract] [Full Text] [PDF]
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H. K. Najm, W. G. Williams, J. G. Coles, I. M. Rebeyka, and R. M. Freedom Pulmonary Atresia With Intact Ventricular Septum: Results of the Fontan Procedure Ann. Thorac. Surg., March 1, 1997; 63(3): 669 - 675. [Abstract] [Full Text]
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G. S. Van Arsdell, W. G. Williams, C. M. Maser, K. S. Streitenberger, I. M. Rebeyka, J. G. Coles, and R. M. Freedom SUPERIOR VENA CAVA TO PULMONARY ARTERY ANASTOMOSIS: AN ADJUNCT TO BIVENTRICULAR REPAIR J. Thorac. Cardiovasc. Surg., November 1, 1996; 112(5): 1143 - 1149. [Abstract] [Full Text]
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N. Ad, E. Birk, J. Barak, S. Diamant, E. Snir, and B. A. Vidne A ONE-WAY VALVED ATRIAL SEPTAL PATCH: A NEW SURGICAL TECHNIQUE AND ITS CLINICAL APPLICATION J. Thorac. Cardiovasc. Surg., April 1, 1996; 111(4): 841 - 848. [Abstract] [Full Text]
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K. Miyaji, M. Shimada, A. Sekiguchi, A. Ishizawa, T. Isoda, and M. Tsunemoto Pulmonary Atresia With Intact Ventricular Septum: Long-Term Results of ``One and a Half Ventricular Repair'' Ann. Thorac. Surg., December 1, 1995; 60(6): 1762 - 1764. [Abstract] [Full Text]
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G. B. Luciani, S. Swilley, and V. A. Starnes Pulmonary atresia, intact ventricular septum, and major aortopulmonary collaterals: Morphogenetic and surgical implications J. Thorac. Cardiovasc. Surg., September 1, 1995; 110(3): 853 - 854. [Full Text]
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J. D. Waldman, R. B. Karp, J. J. Lamberti, M. E. Sand, D. G. Ruschhaupt, and B. Agarwala Tricuspid Valve Closure in Pulmonary Atresia and Important RV-to-Coronary Artery Connections Ann. Thorac. Surg., April 1, 1995; 59(4): 933 - 940. [Abstract] [Full Text]
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T. Akiba and A. E. Becker Disease of the left ventricle in pulmonary atresia with intact ventricular septumThe limiting factor for long-lasting successful surgical intervention? J. Thorac. Cardiovasc. Surg., July 1, 1994; 108(1): 1 - 8. [Abstract] [Full Text]
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C. Bull, M. Kostelka, K. Sorensen, and M. de Leval Outcome measures for the neonatal management of pulmonary atresia with intact ventricular septum J. Thorac. Cardiovasc. Surg., February 1, 1994; 107(2): 359 - 366. [Abstract] [Full Text]
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