JACC
HOME SUBSCRIPTIONS CURRENT ISSUE PAST ISSUES CARDIOSOURCE SEARCH HELP FEEDBACK
QUICK SEARCH:   [advanced]


     

J Am Coll Cardiol, 2000; 36:908-916
© 2000 by the American College of Cardiology Foundation
This Article
Right arrow Figures Only
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Gilljam, T.
Right arrow Articles by Freedom, R. M.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Gilljam, T.
Right arrow Articles by Freedom, R. M.

CLINICAL STUDY

Outcomes of left atrial isomerism over a 28-year period at a single institution

Thomas Gilljam, MD, PhD* {dagger}, Brian W. McCrindle, MD, MPH, FACC* {dagger}, Jeffrey F. Smallhorn, MBBS, FACC* {dagger}, William G. Williams, MD, FRCS(C)* {dagger} and Robert M. Freedom, MD, FACC* {dagger}

* Division of Cardiology, the Department of Pediatrics, the Hospital For Sick Children and the University of Toronto Faculty of Medicine, Toronto, Ontario, Canada
{dagger} Division of Cardiovascular Surgery, the Department of Surgery, the Hospital For Sick Children and the University of Toronto Faculty of Medicine, Toronto, Ontario, Canada

Manuscript received June 17, 1999; revised manuscript received April 3, 2000, accepted April 11, 2000.

Reprint requests and correspondence: Dr. Robert M. Freedom, the Hospital For Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada
rfreedom{at}sickkids.on.ca

OBJECTIVES

We determined long-term outcomes in a large cohort with left atrial isomerism (LAI).

BACKGROUND

Left atrial isomerism is associated with a complex spectrum of cardiac and noncardiac anomalies that may impact on outcomes.

METHODS

The records of all patients with LAI, born between 1970 and 1998, and treated at one center were reviewed. Kaplan-Meier survival was estimated, and independent factors associated with time-related death were identified.

RESULTS

There were 163 patients (63% women), and extracardiac anomalies were noted in 36%, including biliary atresia in 10%. Cardiac defects included interrupted inferior caval vein in 92%, anomalous pulmonary veins in 56%, atrioventricular septal defect in 49%, pulmonary atresia or stenosis in 28% and aortic coarctation in 16%, with congenital atrioventricular block in 7%. Of 22 patients with a normal heart, 18% died of extracardiac anomalies. Of 71 patients with hearts suitable for biventricular repair, 62 (87%) had surgery, with survival of 80% at one year, 71% at five years, 66% at 10 years and 63% after 15 years. Of 70 patients with unbalanced cardiac defects suitable for single-ventricle palliation, 47 (67%) had surgery, with survival of 73% at one year, 61% at five years, 53% at 10 years and 48% at 15 years (p < 0.001). Independent factors associated with time-related death included congenital atrioventricular block, aortic coarctation, single ventricle, biliary atresia and other gastrointestinal malformations.

CONCLUSIONS

Both cardiac and noncardiac anomalies contribute to a high mortality with LAI. Cardiac transplantation may need to be a considered a primary option for selected high-risk patients.

Abbreviations and Acronyms
  AV = atrioventricular
  BV = biventricular
  LAI = left atrial isomerism
  N = normal
  SV = single ventricle




This article has been cited by other articles:


Home page
 Ann. Thorac. Surg.Home page
M. Imamura, U. Dyamenahalli, R. Sachdeva, E. R. Kokoska, and R. D.B. Jaquiss
Hypoplastic Left Heart Syndrome, Interrupted Inferior Vena Cava, Biliary Atresia
Ann. Thorac. Surg., November 1, 2007; 84(5): 1746 - 1748.
[Abstract] [Full Text] [PDF]

Home page
 CirculationHome page
M. P. Kennedy, H. Omran, M. W. Leigh, S. Dell, L. Morgan, P. L. Molina, B. V. Robinson, S. L. Minnix, H. Olbrich, T. Severin, et al.
Congenital Heart Disease and Other Heterotaxic Defects in a Large Cohort of Patients With Primary Ciliary Dyskinesia
Circulation, June 5, 2007; 115(22): 2814 - 2821.
[Abstract] [Full Text] [PDF]

Home page
 J Am Coll CardiolHome page
L. Grosse-Wortmann, A. Al-Otay, H. Woo Goo, C. K. Macgowan, J. G. Coles, L. N. Benson, A. N. Redington, and S.-J. Yoo
Anatomical and Functional Evaluation of Pulmonary Veins in Children by Magnetic Resonance Imaging
J. Am. Coll. Cardiol., March 6, 2007; 49(9): 993 - 1002.
[Abstract] [Full Text] [PDF]

Home page
 J Am Coll CardiolHome page
P. J. Bartz, D. J. Driscoll, J. A. Dearani, F. J. Puga, G. K. Danielson, P. W. O'Leary, M. G. Earing, C. A. Warnes, D. O. Hodge, and F. Cetta
Early and Late Results of the Modified Fontan Operation for Heterotaxy Syndrome: 30 Years of Experience in 142 Patients
J. Am. Coll. Cardiol., December 5, 2006; 48(11): 2301 - 2305.
[Abstract] [Full Text] [PDF]

Home page
 Asian Cardiovasc. Thorac. Ann.Home page
K. Takeuchi, A. Murakami, Y. Hirata, K. Kitahori, Y. Doi, and S. Takamoto
Surgical Outcome of Heterotaxy Syndrome in a Single Institution
Asian Cardiovasc Thorac Ann, December 1, 2006; 14(6): 489 - 494.
[Abstract] [Full Text] [PDF]

Home page
 Ann. Thorac. Surg.Home page
M. S. Cohen, A. H. Schultz, Z.-Y. Tian, D. D. Donaghue, P. M. Weinberg, J. W. Gaynor, and J. Rychik
Heterotaxy Syndrome with Functional Single Ventricle: Does Prenatal Diagnosis Improve Survival?
Ann. Thorac. Surg., November 1, 2006; 82(5): 1629 - 1636.
[Abstract] [Full Text] [PDF]

Home page
 Ann. Thorac. Surg.Home page
S.-J. Kim, W.-H. Kim, H. G. Lim, C.-H. Lee, and J. Y. Lee
Improving results of the fontan procedure in patients with heterotaxy syndrome.
Ann. Thorac. Surg., October 1, 2006; 82(4): 1245 - 1251.
[Abstract] [Full Text] [PDF]

Home page
 Asian Cardiovasc. Thorac. Ann.Home page
M. Koudieh, E D. McKenzie, and C. D Fraser Jr
Outcome of Glenn Anastomosis for Heterotaxy Syndrome with Single Ventricle
Asian Cardiovasc Thorac Ann, June 1, 2006; 14(3): 235 - 238.
[Abstract] [Full Text] [PDF]

Home page
 J. Thorac. Cardiovasc. Surg.Home page
T.-J. Yun, O. O. Al-Radi, I. Adatia, C. A. Caldarone, J. G. Coles, W. G. Williams, J. Smallhorn, and G. S. Van Arsdell
Contemporary management of right atrial isomerism: Effect of evolving therapeutic strategies
J. Thorac. Cardiovasc. Surg., May 1, 2006; 131(5): 1108 - 1113.
[Abstract] [Full Text] [PDF]

Home page
 J. Thorac. Cardiovasc. Surg.Home page
A. Hoskote, D. Bohn, C. Gruenwald, D. Edgell, S. Cai, I. Adatia, and G. Van Arsdell
Extracorporeal life support after staged palliation of a functional single ventricle: Subsequent morbidity and survival
J. Thorac. Cardiovasc. Surg., May 1, 2006; 131(5): 1114 - 1121.
[Abstract] [Full Text] [PDF]

Home page
 CirculationHome page
J. S.L. Lim, B. W. McCrindle, J. F. Smallhorn, F. Golding, C. A. Caldarone, M. Taketazu, and E. T. Jaeggi
Clinical Features, Management, and Outcome of Children With Fetal and Postnatal Diagnoses of Isomerism Syndromes
Circulation, October 18, 2005; 112(16): 2454 - 2461.
[Abstract] [Full Text] [PDF]

Home page
 HeartHome page
Y Maeno, W Himeno, A Saito, S Hiraishi, O Hirose, M Ikuma, N Inamura, M Kawataki, A Mizukami, M Ota, et al.
Clinical course of fetal congenital atrioventricular block in the Japanese population: a multicentre experience
Heart, August 1, 2005; 91(8): 1075 - 1079.
[Abstract] [Full Text] [PDF]

Home page
 J Ultrasound MedHome page
C. Berg, A. Geipel, D. Kamil, M. Knuppel, J. Breuer, M. Krapp, A. Baschat, U. Germer, M. Hansmann, and U. Gembruch
The Syndrome of Left Isomerism: Sonographic Findings and Outcome in Prenatally Diagnosed Cases
J. Ultrasound Med., July 1, 2005; 24(7): 921 - 931.
[Abstract] [Full Text] [PDF]

Home page
 Ann. Thorac. Surg.Home page
R. Formigari, R. M. Di Donato, G. Gargiulo, D. Di Carlo, C. Feltri, F. M. Picchio, and B. Marino
Better surgical prognosis for patients with complete atrioventricular septal defect and Down's syndrome
Ann. Thorac. Surg., August 1, 2004; 78(2): 666 - 672.
[Abstract] [Full Text] [PDF]

Home page
 Ann. Thorac. Surg.Home page
E. A. Bacha, J. Hardin, D. C. Cronin, L. Brady, M. J. Millis, J. P. Starr, P. Koenig, S. Daves, and M. Kahana
Open-heart surgery in pediatric patients with end-stage liver disease
Ann. Thorac. Surg., August 1, 2004; 78(2): e30 - e33.
[Abstract] [Full Text] [PDF]

Home page
 J. Thorac. Cardiovasc. Surg.Home page
A. Azakie and I. A. Russell
Gender differences in pediatric cardiac surgery: The surgeon's perspective
J. Thorac. Cardiovasc. Surg., July 1, 2004; 128(1): 4 - 6.
[Full Text] [PDF]

Home page
 Eur. J. Cardiothorac. Surg.Home page
G. Michielon, F. Parisi, D. Di Carlo, C. Squitieri, A. Carotti, M. Buratta, and R. M. Di Donato
Orthotopic heart transplantation for failing single ventricle physiology
Eur. J. Cardiothorac. Surg., October 1, 2003; 24(4): 502 - 510.
[Abstract] [Full Text] [PDF]

Home page
 CirculationHome page
G. Michielon, F. Parisi, C. Squitieri, A. Carotti, G. Gagliardi, L. Pasquini, and R. M. Di Donato
Orthotopic Heart Transplantation for Congenital Heart Disease: An Alternative for High-Risk Fontan Candidates?
Circulation, September 9, 2003; 108(90101): II-140 - 149.
[Abstract] [Full Text] [PDF]

Home page
 Eur. J. Cardiothorac. Surg.Home page
S. P. McGuirk, D. S. Winlaw, S. M. Langley, O. F. Stumper, J. V. de Giovanni, J. G. Wright, W. J. Brawn, and D. J. Barron
The impact of ventricular morphology on midterm outcome following completion total cavopulmonary connection
Eur. J. Cardiothorac. Surg., July 1, 2003; 24(1): 37 - 46.
[Abstract] [Full Text] [PDF]

Home page
 EuropaceHome page
A. Frogoudaki, R. Sutton, and M. A. Gatzoulis
Pacing for adult patients with left atrial isomerism: efficacy and technical considerations
Europace, January 1, 2003; 5(2): 189 - 193.
[Abstract] [PDF]

Home page
 Ann. Thorac. Surg.Home page
C. Stamm, I. Friehs, L. F. Duebener, D. Zurakowski, J. E. Mayer Jr, R. A. Jonas, and P. J. del Nido
Improving results of the modified Fontan operation in patients with heterotaxy syndrome
Ann. Thorac. Surg., December 1, 2002; 74(6): 1967 - 1978.
[Abstract] [Full Text] [PDF]

Home page
 Ann. Thorac. Surg.Home page
A. Azakie, S. L. Merklinger, W. G. Williams, G. S. Van Arsdell, J. G. Coles, and I. Adatia
Improving outcomes of the Fontan operation in children with atrial isomerism and heterotaxy syndromes
Ann. Thorac. Surg., November 1, 2001; 72(5): 1636 - 1640.
[Abstract] [Full Text] [PDF]


HOME SUBSCRIPTIONS CURRENT ISSUE PAST ISSUES CARDIOSOURCE SEARCH HELP FEEDBACK
Copyright © 2000 by the American College of Cardiology Foundation.