Cardiac Transplantation in a Pediatric Patient with Systemic Sclerosis

Jonathan N. Johnson, Bonnie S. Kvistad, Ann M. Reed, Co Burn J Porter, Patrick W. O'Leary, David J. Driscoll

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

Introduction. Diffuse cutaneous systemic sclerosis (SSc) is rare in children, but has a poor prognosis when cardiomyopathy is present. Methods. We reviewed the case of a 14-year-old female with progressive skin thickening/tightness and dyspnea on exertion who was diagnosed with SSc. Results. Our patient was found to have severe restrictive cardiomyopathy with poor left ventricular systolic function (ejection fraction = 20%), unresponsive to the immunosuppression used to treat her SSc. There was no evidence of pulmonary fibrosis. The patient underwent orthotopic cardiac transplantation, with improvement in systemic symptoms. Two years after transplantation, she had elevated filling pressures during a surveillance catheterization, with no evidence of cellular rejection or coronary artery vasculopathy. Four months later, she developed severe ventricular dysfunction and dyspnea, despite a negative biopsy and negative C4d immunofluorescence staining. Her immunosuppression was intensified with improvement of cardiac function. Despite this, 1 month later she had sudden pulseless cardiac arrest from which she could not be revived. The family declined an autopsy. Discussion. We report a 14-year-old female with SSc who had cardiac transplantation. The etiology behind her recurrent restrictive physiology, cardiac dysfunction, and subsequent cardiac arrest remains unclear.

Original languageEnglish (US)
JournalCongenital Heart Disease
Volume8
Issue number1
DOIs
StatePublished - Jan 2013

Fingerprint

Systemic Scleroderma
Heart Transplantation
Pediatrics
Dyspnea
Immunosuppression
Restrictive Cardiomyopathy
Ventricular Dysfunction
Diffuse Scleroderma
Pulmonary Fibrosis
Sudden Cardiac Death
Heart Arrest
Cardiomyopathies
Left Ventricular Function
Catheterization
Fluorescent Antibody Technique
Autopsy
Coronary Vessels
Transplantation
Staining and Labeling
Biopsy

Keywords

  • Cardiac transplant
  • Humoral rejection
  • Restrictive cardiomyopathy
  • Scleroderma
  • Systemic sclerosis
  • Transplant

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Pediatrics, Perinatology, and Child Health
  • Surgery
  • Radiology Nuclear Medicine and imaging

Cite this

Johnson, J. N., Kvistad, B. S., Reed, A. M., Porter, C. B. J., O'Leary, P. W., & Driscoll, D. J. (2013). Cardiac Transplantation in a Pediatric Patient with Systemic Sclerosis. Congenital Heart Disease, 8(1). https://doi.org/10.1111/j.1747-0803.2011.00554.x

Cardiac Transplantation in a Pediatric Patient with Systemic Sclerosis. / Johnson, Jonathan N.; Kvistad, Bonnie S.; Reed, Ann M.; Porter, Co Burn J; O'Leary, Patrick W.; Driscoll, David J.

In: Congenital Heart Disease, Vol. 8, No. 1, 01.2013.

Research output: Contribution to journalArticle

Johnson, JN, Kvistad, BS, Reed, AM, Porter, CBJ, O'Leary, PW & Driscoll, DJ 2013, 'Cardiac Transplantation in a Pediatric Patient with Systemic Sclerosis', Congenital Heart Disease, vol. 8, no. 1. https://doi.org/10.1111/j.1747-0803.2011.00554.x
Johnson, Jonathan N. ; Kvistad, Bonnie S. ; Reed, Ann M. ; Porter, Co Burn J ; O'Leary, Patrick W. ; Driscoll, David J. / Cardiac Transplantation in a Pediatric Patient with Systemic Sclerosis. In: Congenital Heart Disease. 2013 ; Vol. 8, No. 1.
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