Skip to main content Accessibility help

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)...


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.


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:


Hide All
1. Berger, RM, Beghetti, M, Humpl, T, et al. Clinical features of paediatric pulmonary hypertension: a registry study. Lancet 2012; 379: 537546.
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.
4. Simonneau, G, Gatzoulis, MA, Adatia, I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2013; 62.
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.
Recommend this journal

Email your librarian or administrator to recommend adding this journal to your organisation's collection.

Cardiology in the Young
  • ISSN: 1047-9511
  • EISSN: 1467-1107
  • URL: /core/journals/cardiology-in-the-young
Please enter your name
Please enter a valid email address
Who would you like to send this to? *



Full text views

Total number of HTML views: 0
Total number of PDF views: 0 *
Loading metrics...

Abstract views

Total abstract views: 0 *
Loading metrics...

* Views captured on Cambridge Core between <date>. This data will be updated every 24 hours.

Usage data cannot currently be displayed