Pediatric Cardiology

, Volume 32, Issue 6, pp 828–833

Connective Tissue Disease Presenting With Signs and Symptoms of Pulmonary Hypertension in Children

  • Shinichi Takatsuki
  • Jennifer B. Soep
  • Michelle Calderbank
  • David Dunbar Ivy
Case Report

DOI: 10.1007/s00246-011-9950-7

Cite this article as:
Takatsuki, S., Soep, J.B., Calderbank, M. et al. Pediatr Cardiol (2011) 32: 828. doi:10.1007/s00246-011-9950-7

Abstract

Our case series describes three children who were initially diagnosed as having severe pulmonary arterial hypertension (PAH) and subsequently found to be positive for specific autoantibodies suggestive of an underlying autoimmune process. The signs and symptoms of PAH are subtle and may be part of the initial presentation of childhood connective tissue disease (CTD). Evaluation for connective tissue disease in the newly diagnosed pulmonary hypertension (PH) patient is important because early diagnosis of PH as well as CTD is crucial in the successful management of these complex patients. Ongoing monitoring for CTD in patients with severe PAH is warranted.

Keywords

Pulmonary arterial hypertension Autoantibody Pulmonary vasodilator therapies Antinuclear antibody Connective tissue disease Children 

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  • Shinichi Takatsuki
    • 1
  • Jennifer B. Soep
    • 1
  • Michelle Calderbank
    • 1
  • David Dunbar Ivy
    • 1
  1. 1.Department of Pediatrics, The Children’s HospitalUniversity of Colorado Denver School of MedicineAuroraUSA

Personalised recommendations