Hostname: page-component-78c5997874-m6dg7 Total loading time: 0 Render date: 2024-11-05T11:47:04.424Z Has data issue: false hasContentIssue false
  • English
  • Français

Cardiac diagnoses, procedures, and healthcare utilisation in inpatients with Ellis–van Creveld syndrome

Published online by Cambridge University Press:  29 October 2013

Matthew J. O’Connor ,
Xinyu Tang  and
R. Thomas Collins II
Matthew J. O’Connor*
Affiliation:
Department of Pediatrics, Pediatric Cardiology Division, University of Arkansas for Medical Sciences/Arkansas Children’s Hospital, Little Rock, Arkansas, United States of America
Xinyu Tang
Affiliation:
Biostatistics Program, Department of Pediatrics, University of Arkansas for Medical Sciences/Arkansas Children’s Hospital, Little Rock, Arkansas, United States of America
R. Thomas Collins II
Affiliation:
Department of Pediatrics, Pediatric Cardiology Division, University of Arkansas for Medical Sciences/Arkansas Children’s Hospital, Little Rock, Arkansas, United States of America
*
Correspondence to: Dr M. J. O’Connor, MD, Pediatric Cardiology Division, Arkansas Children’s Hospital, Slot 512-3, 1 Children’s Way, Little Rock, AR 72212, United States of America. Tel: (501) 364-1479; Fax: 501-364-3667; E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Introduction

Ellis–van Creveld syndrome is a rare condition associated with a very high incidence of congenital malformations of the heart. Prior reports have suggested increased morbidity and mortality following surgery for congenital malformations of the heart in patients with Ellis–van Creveld syndrome.

Materials and methods

The Pediatric Health Information System database, an administrative database containing data from 43 free-standing paediatric hospitals in North America, was queried to search for patients with the diagnostic code for Ellis–van Creveld syndrome between 2004 and 2011. Those patients who underwent cardiac procedures were compared with those who did not with respect to measures of healthcare utilisation.

Results

A total of 138 admissions occurred in 93 patients with Ellis–van Creveld syndrome during the study period. Of these, 74% had an underlying diagnosis of congenital malformations of the heart. Half of the patients in the sample underwent a cardiac surgical or interventional catheterisation procedure. Patients who underwent a cardiac procedure had a longer hospital length of stay, higher incidence of intensive care unit admission, and higher total and per day hospital charges than patients who did not undergo cardiac surgery during admission.

Conclusions

In a large group of inpatients with Ellis–van Creveld syndrome, the prevalence of congenital malformations of the heart was similar to that reported in prior studies. Cardiac surgical and interventional procedures appear to drive a substantial portion of healthcare utilisation in these patients.

Keywords

Ellis–van Creveld syndromecardiac surgeryinpatienthealthcare utilisation
Type
Original Articles
Information
Cardiology in the Young , Volume 25 , Issue 1 , January 2015 , pp. 95 - 101
Copyright
Copyright © Cambridge University Press 2013 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1. Baujat, G, Le Merrer, M. Ellis-van Creveld syndrome. Orphanet J Rare Dis 2007; 2: 27.Google Scholar
2. McKusick, VA, Eldridge, R, Hostetler, JA, Egeland, JA. Dwarfism in the Amish. Trans Assoc Am Physicians 1964; 77: 151–168.Google Scholar
3. Ellis, RWB, van Creveld, S. A syndrome characterized by ectodermal dysplasia, polydactyly, chondrodysplasia, and congenital morbus cordis: report of three cases. Arch Dis Child 1940; 15: 65–84.Google Scholar
4. Digilio, MC, Marino, B, Ammirati, A, Borzaga, U, Giannotti, A, Dallapiccola, B. Cardiac malformations in patients with oral-facial-skeletal syndromes: clinical similarities with heterotaxia. Am J Med Genet 1999; 84: 350–356.Google Scholar
5. Hills, CB, Kochilas, L, Schimmenti, LA, Moller, JH. Ellis-van Creveld syndrome and congenital heart defects: presentation of an additional 32 cases. Pediatr Cardiol 2011; 32: 977–982.Google Scholar
6. O’Connor, MJ, Rider, NL, Collins, RT II, Hanna, BD, Morton, DH, Strauss, KA. Contemporary management of congenital malformations of the heart in infants with Ellis-van Creveld syndrome: a report of nine cases. Cardiol Young 2011; 21: 145–152.CrossRefGoogle ScholarPubMed
7. Ruiz-Perez, VL, Blair, HJ, Rodriguez-Andres, ME, et al. Evc is a positive mediator of Ihh-regulated bone growth that localises at the base of chondrocyte cilia. Development 2007; 134: 2903–2912.Google Scholar
8. Digilio, MC, Marino, B, Giannotti, A, Dallapiccola, B, Opitz, JM. Specific congenital heart defects in RSH/Smith-Lemli-Opitz syndrome: postulated involvement of the sonic hedgehog pathway in syndromes with postaxial polydactyly or heterotaxia. Birth Defects Res A Clin Mol Teratol 2003; 67: 149–153.Google Scholar
9. Centers for Disease Control and Prevention. Hospital stays, hospital charges, and in-hospital deaths among infants with selected birth defects-United States, 2003. MMWR Morb Mortal Wkly Rep 2007; 56: 25–29.Google Scholar
10. Kim, YY, Gauvreau, K, Bacha, EA, Landzberg, MJ, Benavidez, OJ. Resource use among adult congenital heart surgery admissions in pediatric hospitals: risk factors for high resource utilization and association with inpatient death. Circ Cardiovasc Qual Outcomes 2011; 4: 634–639.Google Scholar
11. Pasquali, SK, Peterson, ED, Jacobs, JP, et al. Differential case ascertainment in clinical registry versus administrative data and impact on outcomes assessment for pediatric cardiac operations. Ann Thorac Surg 2013; 95: 197–203.Google Scholar