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Management of a child with pulmonary arterial hypertension presenting with systemic hypertension

  • Saul Flores (a1), Joshua Daily (a1), Jayant “Nick” Pratap (a1) (a2), Michelle C. Cash (a1) and Russel Hirsch (a1)...

Abstract

We describe the course and management of a 12-year-old girl with severe pulmonary arterial hypertension who initially presented with severe systemic hypertension. Successful therapy included pulmonary vasodilators and an atrial septostomy, while ensuring adequate maintenance of her systemic vascular resistance to maintain cardiac output. Clear understanding of the physiology and judicious medical management in patients with severe pulmonary arterial hypertension using extreme compensatory mechanisms is vitally important.

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Corresponding author

Correspondence to: S. Flores, Heart Institute, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229, United States of America. Tel: 513 803 3124; Fax: 513-636-3952; E-mail: saul.flores@cchmc.org

References

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2. Mallory, GB Jr, Hanna, BD, Ivy, DD, Shardonofsky, F, Farber, HJ. Management and controversies in pediatric pulmonary hypertension. Pediatr Allergy Immunol Pulmonol 2011; 22: 139150.
3. Gomberg-Maitland, M, Bull, TM, Saggar, R, et al. New trial designs and potential therapies for pulmonary artery hypertension. J Am Coll Cardiol 2013; 62: D82D91.
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5. McLaughlin, VV, Shillington, A, Rich, S. Survival in primary pulmonary hypertension the impact of epoprostenol therapy. Circulation 2002; 106: 14771482.
6. Sitbon, O, Humbert, M, Nunes, H, et al. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension Prognostic factors and survival. J Am Coll Cardiol 2002; 40: 780788.
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Cardiology in the Young
  • ISSN: 1047-9511
  • EISSN: 1467-1107
  • URL: /core/journals/cardiology-in-the-young
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