eMedicine Specialties > Pulmonology > Pulmonary Hypertension

Pulmonary Hypertension, Primary: Follow-up

Author: Ronald J Oudiz, MD, FACP, FACC, Associate Professor of Medicine, Division of Cardiology, The David Geffen School of Medicine at UCLA; Director, Liu Center for Pulmonary Hypertension, LA Biomedical Research Institute at Harbor-UCLA Medical Center
Contributor Information and Disclosures

Updated: Aug 13, 2009

Follow-up

Further Inpatient Care

  • Patients on EPO or intravenous treprostinil therapy must have a central venous catheter placed surgically and receive their initial dose in an inpatient setting. This allows for monitoring of acute adverse effects and provides the opportunity for the patient and support personnel to master the drug preparation and administration technique before discharge.

Further Outpatient Care

  • Currently, no precise dosage adjustment algorithm is available for patients with idiopathic pulmonary arterial hypertension (IPAH) who are on vasodilator therapy.
  • Monitor the patient with frequent physical examinations, and focus the history on heart failure symptoms and adverse effects of medications.
  • Echocardiography has been used in several studies to serially monitor changes in the right ventricular–right atrial pressure gradient and the right and left ventricular chamber sizes.
  • Findings from other noninvasive modalities (eg, electron-beam CT measurements of cardiac chamber sizes) correlate with hemodynamic improvements in pulmonary physiology.
  • More recently, cardiopulmonary exercise testing, serial invasive hemodynamic testing, and 6-minute walk testing have been used to monitor the disease status of patients with IPAH.

Complications

  • Advanced right-sided heart failure with hepatic congestion
  • Pedal edema
  • Pleural effusions
  • Ascites
  • Worsening dyspnea on exertion

Prognosis

  • The mortality rate for untreated IPAH is approximately 50% at 3 years (varies with severity at presentation). With EPO therapy, this has increased to higher than 65% at 5 years. Data on long-term survival in patients treated with other pulmonary vascular therapies are emerging. Patients whose disease progresses and is unresponsive to medical treatments either undergo transplantation or die of progressive right-sided heart failure.

Patient Education

  • Patient and physician education about this rare fatal disease is paramount.
  • If applicable, instruct patients on how to administer their daily parenteral medication.
  • For excellent patient education materials, see eMedicine's Lung and Airway Center and Heart and Blood Vessels Center.

Miscellaneous

Medicolegal Pitfalls

  • Treating PAH requires significant education regarding and exposure to the available therapies for IPAH and the potential complications. Because IPAH is relatively rare, management is best left to expert personnel at centers with regular exposure to these patients. Failure to heed this advice can result in medicolegal pitfalls should patient outcome be less than optimal.
 


More on Pulmonary Hypertension, Primary

Overview: Pulmonary Hypertension, Primary
Differential Diagnoses & Workup: Pulmonary Hypertension, Primary
Treatment & Medication: Pulmonary Hypertension, Primary
Follow-up: Pulmonary Hypertension, Primary
Multimedia: Pulmonary Hypertension, Primary
References
[ CLOSE WINDOW ]

References

  1. Dresdale DT, Schultz M, Michtom RJ. Primary pulmonary hypertension. I. Clinical and hemodynamic study. Am J Med. Dec 1951;11(6):686-705. [Medline].

  2. Abenhaim L, Moride Y, Brenot F, et al. Appetite-suppressant drugs and the risk of primary pulmonary hypertension. International Primary Pulmonary Hypertension Study Group. N Engl J Med. Aug 29 1996;335(9):609-16. [Medline].

  3. Tolle JJ, Waxman AB, Van Horn TL, Pappagianopoulos PP, Systrom DM. Exercise-induced pulmonary arterial hypertension. Circulation. Nov 18 2008;118(21):2183-9. [Medline].

  4. [Guideline] McGoon M, Gutterman D, Steen V, et al. Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. Jul 2004;126(1 Suppl):14S-34S. [Medline].

  5. [Guideline] Rubin LJ. Diagnosis and management of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. Jul 2004;126(1 Suppl):4S-6S. [Medline].

  6. [Guideline] McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: developed in collaboration with the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association. Circulation. Apr 28 2009;119(16):2250-94. [Medline].

  7. Torbicki A, Kurzyna M, Kuca P, et al. Detectable serum cardiac troponin T as a marker of poor prognosis among patients with chronic precapillary pulmonary hypertension. Circulation. Aug 19 2003;108(7):844-8. [Medline].

  8. Oudiz RJ, Farber HW. Dosing considerations in the use of intravenous prostanoids in pulmonary arterial hypertension: an experience-based review. Am Heart J. Apr 2009;157(4):625-35. [Medline].

  9. Shapiro SM, Oudiz RJ, Cao T, et al. Primary pulmonary hypertension: improved long-term effects and survival with continuous intravenous epoprostenol infusion. J Am Coll Cardiol. Aug 1997;30(2):343-9. [Medline].

  10. Oudiz RJ, Schilz RJ, Barst RJ, et al. Treprostinil, a prostacyclin analogue, in pulmonary arterial hypertension associated with connective tissue disease. Chest. Aug 2004;126(2):420-7. [Medline].

  11. Simonneau G, Barst RJ, Galie N, et al. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial. Am J Respir Crit Care Med. Mar 15 2002;165(6):800-4. [Medline].

  12. Olschewski H, Simonneau G, Galie N, et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med. Aug 1 2002;347(5):322-9. [Medline].

  13. Galie N, Hinderliter AL, Torbicki A, et al. Effects of the oral endothelin-receptor antagonist bosentan on echocardiographic and doppler measures in patients with pulmonary arterial hypertension. J Am Coll Cardiol. Apr 16 2003;41(8):1380-6. [Medline].

  14. Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med. Mar 21 2002;346(12):896-903. [Medline].

  15. [Best Evidence] Galie N, Olschewski H, Oudiz RJ, et al. Ambrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2. Circulation. Jun 10 2008;117(23):3010-9. [Medline].

  16. [Best Evidence] Simonneau G, Rubin LJ, Galie N, et al. Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trial. Ann Intern Med. Oct 21 2008;149(8):521-30. [Medline].

  17. Galie N, Brundage BH, Ghofrani HA, et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation. Jun 9 2009;119(22):2894-903. [Medline].

  18. Barst RJ, Langleben D, Frost A, et al. Sitaxsentan therapy for pulmonary arterial hypertension. Am J Respir Crit Care Med. Feb 15 2004;169(4):441-7. [Medline].

  19. [Guideline] Badesch DB, Abman SH, Ahearn GS, et al. Medical therapy for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. Jul 2004;126(1 Suppl):35S-62S. [Medline].

  20. [Guideline] Badesch DB, Abman SH, Simonneau G, Rubin LJ, McLaughlin VV. Medical therapy for pulmonary arterial hypertension: updated ACCP evidence-based clinical practice guidelines. Chest. Jun 2007;131(6):1917-28. [Medline].

  21. [Guideline] Galie N, Torbicki A, Barst R, et al. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology. Eur Heart J. Dec 2004;25(24):2243-78. [Medline].

  22. Barst RJ, Rubin LJ, Long WA, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study Group. N Engl J Med. Feb 1 1996;334(5):296-302. [Medline].

  23. Gaine S. Pulmonary hypertension. JAMA. Dec 27 2000;284(24):3160-8. [Medline].

  24. Rich S, Dantzker DR, Ayres SM, Bergofsky EH, et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med. Aug 1987;107(2):216-23. [Medline].

  25. Sun XG, Hansen JE, Oudiz RJ, Wasserman K. Exercise pathophysiology in patients with primary pulmonary hypertension. Circulation. Jul 24 2001;104(4):429-35. [Medline].

  26. Sun XG, Hansen JE, Oudiz RJ, Wasserman K. Gas exchange detection of exercise-induced right-to-left shunt in patients with primary pulmonary hypertension. Circulation. Jan 1 2002;105(1):54-60. [Medline].

  27. Sun XG, Hansen JE, Oudiz RJ, Wasserman K. Pulmonary function in primary pulmonary hypertension. J Am Coll Cardiol. Mar 19 2003;41(6):1028-35. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

primary pulmonary hypertension, idiopathic pulmonary arterial hypertension, IPAH, idiopathic pulmonary hypertension, elevated pulmonary artery pressure, thrombotic pulmonary arteriopathy, TPA, plexogenic pulmonary arteriopathy, PPH, precapillary pulmonary hypertension, endothelin receptor antagonists, ERAs, endothelin-receptor antagonists, pulmonary hypertension, portal hypertension, pulmonary arteriopathy, pulmonary artery hypertension, PAH, pulmonary arterial hypertension, portopulmonary hypertension, CREST syndrome

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Ronald J Oudiz, MD, FACP, FACC, Associate Professor of Medicine, Division of Cardiology, The David Geffen School of Medicine at UCLA; Director, Liu Center for Pulmonary Hypertension, LA Biomedical Research Institute at Harbor-UCLA Medical Center
Ronald J Oudiz, MD, FACP, FACC is a member of the following medical societies: American College of Cardiology, American College of Chest Physicians, American College of Physicians, American Heart Association, and American Thoracic Society
Disclosure: Actelion Grant/research funds Clinical Trials + honoraria; Encysive Grant/research funds Clinical Trials + honoraria; Gilead Grant/research funds Clinical Trials + honoraria; Pfizer Grant/research funds Clinical Trials + honoraria; United Therapeutics Grant/research funds Clinical Trials + honoraria; Lilly Grant/research funds Clinical Trials + honoraria; LungRx  Clinical Trials + honoraria

Medical Editor

Oleh Wasyl Hnatiuk, MD, Program Director, National Capital Consortium, Pulmonary and Critical Care, Walter Reed Army Medical Center; Associate Professor, Department of Medicine, Uniformed Services University of Health Sciences
Oleh Wasyl Hnatiuk, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, and American Thoracic Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

,, Kathy Roarty Placeholder
Disclosure: Nothing to disclose.

CME Editor

Timothy D Rice, MD, Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, Saint Louis University School of Medicine
Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians
Disclosure: Nothing to disclose.

Chief Editor

Zab Mosenifar, MD, Director, Division of Pulmonary and Critical Care Medicine, Director, Women's Guild Pulmonary Disease Institute, Executive Vice Chair, Department of Medicine, Cedars Sinai Medical Center; Professor of Medicine, David Geffen School of Medicine at UCLA
Zab Mosenifar, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, and American Thoracic Society
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.