Hypoplastic left heart syndrome: hemodynamic and angiographic assessment after initial reconstructive surgery and relevance to modified Fontan procedure

Division of Cardiology Children's Hospital of Philadelphia, Pennsylvania 19104.

After undergoing initial reconstructive surgery for hypoplastic left heart syndrome performed between August 1985 and March 1989, 59 patients (age range 3 to 27 months, mean 13.8 +/- 4.5) underwent elective cardiac catheterization in anticipation of a modified Fontan procedure. Five important hemodynamic and anatomic features considered to be components of successful reconstructive surgery were specifically addressed. 1) Interatrial communication: Only two patients had a measured pressure difference of greater than 4 mm Hg across the atrial septum. 2) Tricuspid valve function: Angiography demonstrated significant tricuspid valve regurgitation in only five patients (moderate in two and severe in three). 3) Aortic arch: Pressure tracings from the right ventricle to the descending aorta revealed a gradient greater than 25 mm Hg in only two patients. 4) Pulmonary vasculature: Ten patients had a calculated pulmonary vascular resistance greater than 4 U.m2; 51 (86%) of the 59 patients had no evidence of distortion (stenosis or hypoplasia) of either the left or the right pulmonary artery. 5) Right ventricular function: Five patients had an end-diastolic pressure in the right ventricle greater than 12 mm Hg and two patients had qualitative assessment of decreased ventricular function. Comparison of catheterization data between survivors and nonsurvivors of the subsequent modified Fontan procedure showed that only significant tricuspid regurgitation is a possible predictor of poor outcome. After first stage reconstructive surgery for hypoplastic left heart syndrome, most survivors have favorable anatomy and hemodynamics at follow-up cardiac catheterization for a subsequent Fontan procedure.

This article has been cited by other articles:

				M. Elmi, E. J. Hickey, W. G. Williams, G. Van Arsdell, C. A. Caldarone, and B. W. McCrindle Long-term tricuspid valve function after Norwood operation J. Thorac. Cardiovasc. Surg., December 1, 2011; 142(6): 1341 - 1347.e4. [Abstract] [Full Text] [PDF]

				J. A. Ballweg, T. E. Dominguez, S. Tabbutt, J. J. Rome, J. W. Gaynor, S. C. Nicolson, T. L. Spray, and C. Ravishankar Reintervention for arch obstruction after stage 1 reconstruction does not adversely affect survival or outcome at Fontan completion J. Thorac. Cardiovasc. Surg., September 1, 2010; 140(3): 545 - 549. [Abstract] [Full Text] [PDF]

				K. Takahashi, A. Inage, I. M. Rebeyka, D. B. Ross, R. B. Thompson, A. S. Mackie, and J. F. Smallhorn Real-Time 3-Dimensional Echocardiography Provides New Insight Into Mechanisms of Tricuspid Valve Regurgitation in Patients With Hypoplastic Left Heart Syndrome Circulation, September 22, 2009; 120(12): 1091 - 1098. [Abstract] [Full Text] [PDF]

				T. M. Ashcraft, K. Jones, W. L. Border, P. Eghtesady, J. M. Pearl, P. R. Khoury, and P. B. Manning Factors Affecting Long-Term Risk of Aortic Arch Recoarctation After the Norwood Procedure Ann. Thorac. Surg., April 1, 2008; 85(4): 1397 - 1402. [Abstract] [Full Text] [PDF]

				N. Ota, A. Ikai, K. Hirose, and K. Sakamoto Retrospective analysis of stage I Norwood procedures with tricuspid valve insufficiency in the past 5 years Interact CardioVasc Thorac Surg, February 1, 2007; 6(1): 121 - 123. [Abstract] [Full Text] [PDF]

				R. G. Ohye, C. A. Gomez, C. S. Goldberg, H. L. Graves, E. J. Devaney, and E. L. Bove Tricuspid valve repair in hypoplastic left heart syndrome J. Thorac. Cardiovasc. Surg., February 1, 2004; 127(2): 465 - 472. [Abstract] [Full Text] [PDF]

				J. A. Gaca, W. I. Douglas, and S. D. Barnes Anesthetic Implications of the Fontan Procedure for Single Ventricle Physiology Seminars in Cardiothoracic and Vascular Anesthesia, March 1, 2001; 5(1): 31 - 39. [Abstract] [PDF]

				I. Shiraishi, M. Yamagishi, T. Oka, A. Kawakita, and K. Hamaoka Intraoperative balloon angioplasty for aortic coarctation after Norwood operation Ann. Thorac. Surg., July 1, 2000; 70(1): 289 - 291. [Abstract] [Full Text] [PDF]

				C. I. Tchervenkov, S. A. Tahta, L. Jutras, and M. J. Beland SINGLE-STAGE REPAIR OF AORTIC ARCH OBSTRUCTION AND ASSOCIATED INTRACARDIAC DEFECTS WITH PULMONARY HOMOGRAFT PATCH AORTOPLASTY J. Thorac. Cardiovasc. Surg., December 1, 1998; 116(6): 897 - 904. [Abstract] [Full Text] [PDF]

				G. S. Shirali, C. E. Cephus, M. A. Kuhn, K. K. Ogata, L. K. Vander Dussen, R. E. Chinnock, N. F. Mulla, J. K. Johnston, L. L. Bailey, S. R. Gundry, et al. Posttransplant recoarctation of the aorta: a twelve year experience J. Am. Coll. Cardiol., August 1, 1998; 32(2): 509 - 514. [Abstract] [Full Text] [PDF]

				W. P. Santamore, O. Barnea, C. J. Riordan, M. P. Ross, and E. H. Austin Theoretical optimization of pulmonary-to-systemic flow ratio after a bidirectional cavopulmonary anastomosis Am J Physiol Heart Circ Physiol, February 1, 1998; 274(2): H694 - H700. [Abstract] [Full Text] [PDF]

				C. J. Riordan, F. Randsbaek, J. H. Storey, W. D. Montgomery, W. P. Santamore, and E. H. Austin III EFFECTS OF OXYGEN, POSITIVE END-EXPIRATORY PRESSURE, AND CARBON DIOXIDE ON OXYGEN DELIVERY IN AN ANIMAL MODEL OF THE UNIVENTRICULAR HEART J. Thorac. Cardiovasc. Surg., September 1, 1996; 112(3): 644 - 654. [Abstract] [Full Text]

				J. A. M. van Son, M. D. Black, K. Devoe, and G. S. Haas Organized thrombus in left main coronary artery in hypoplastic left heart syndrome Ann. Thorac. Surg., August 1, 1995; 60(2): 462 - 463. [Abstract] [PDF]

				W. I. Norwood Jr, M. L. Jacobs, and J. D. Murphy Fontan procedure for hypoplastic left heart syndrome Ann. Thorac. Surg., December 1, 1992; 54(6): 1025 - 1030. [Abstract] [PDF]

				D. M. Cohen Surgical Management of Congenital Heart Disease in the 1990s Arch Pediatr Adolesc Med, December 1, 1992; 146(12): 1447 - 1452. [Abstract] [PDF]