eMedicine Specialties > Radiology > Chest

Pulmonary Hypertension: Follow-up

Author: Davinder Jassal, MD, BSc, FACC, FRCPC, Clinical and Research Cardiac Echocardiography Fellow, Department of Cardiology, Massachusetts General Hospital, Harvard Medical School
Coauthor(s): Sat Sharma, MD, FRCPC, Professor and Head, Division of Pulmonary Medicine, Department of Internal Medicine, University of Manitoba; Site Director, Respiratory Medicine, St. Boniface General Hospital; Bruce Maycher, MD, Director of Pulmonary Radiology, St Boniface General Hospital; Associate Professor, Department of Radiology, University of Manitoba
Contributor Information and Disclosures

Updated: Apr 21, 2009

Intervention

Unfortunately, primary pulmonary hypertension is a progressive disease that has no cure. Although spontaneous remission has been reported, this is exceptionally rare. Approximately 25-35% of individuals with primary pulmonary hypertension respond to the acute administration of vasodilators and/or anticoagulants. In a meta-analysis of randomized, controlled trials, active treatments were found to reduce mortality by 43%; sensitivity analysis confirmed a 38% reduction in mortality.6  


Vasodilation

Although vasodilator therapy may decrease pulmonary vasculature resistance, few studies show long-term clinical improvement in patients with pulmonary hypertension.

Vasodilators that are short acting include nitrous oxide, epoprostenol (prostacyclin),7 and adenosine. If the aforementioned drugs can reduce the pulmonary artery pressures, thereby increasing cardiac output, the morbidity and mortality of this disease may theoretically improve.

From the perspective of evidence-based medicine, short-term therapy with calcium channel blockers has conflicting results in terms of improving pulmonary hypertension. Long-term benefits have not been demonstrated.

Longer-acting medications include calcium channel blockers, such as diltiazem and dihydropuridines, which improve morbidity in 20-30% of individuals. Verapamil, with its negative inotropic effects, is not used in the treatment of PPH.

Anticoagulation

Patients with PPH are at an increased risk of thrombosis. Therefore, anticoagulation therapy is recommended for patients at high risk for thromboembolism; the more general use of warfarin therapy is not recommended. Studies have shown that anticoagulation with warfarin, adjusting for an international normalized ratio (INR) of 2-3, prolongs life in individuals with PPH.

Special Concerns

  • Issues in pregnancy
    • Physiologic changes that occur in pregnancy may activate primary pulmonary hypertension and result in considerable morbidity and mortality for both the mother and fetus.
    • As the circulating blood volume increases, so does oxygen consumption, leading to an increase in right ventricular volume.
    • Syncope and cardiac arrest may occur during active labor and delivery, as well.
    • For the aforementioned reasons, pregnancy should be discouraged in women with PPH.
 


More on Pulmonary Hypertension

Overview: Pulmonary Hypertension
Imaging: Pulmonary Hypertension
Follow-up: Pulmonary Hypertension
Multimedia: Pulmonary Hypertension
References
Further Reading
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References

  1. [Best Evidence] Condliffe R, Kiely DG, Peacock AJ, Corris PA, Gibbs JS, Vrapi F, et al. Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med. Jan 15 2009;179(2):151-7. [Medline].

  2. Revel MP, Faivre JB, Remy-Jardin M, Delannoy-Deken V, Duhamel A, Remy J. Pulmonary hypertension: ECG-gated 64-section CT angiographic evaluation of new functional parameters as diagnostic criteria. Radiology. Feb 2009;250(2):558-66. [Medline].

  3. Koito H. [The findings of computed tomography (CT) and magnetic resonance imaging (MRI) in pulmonary arterial hypertension]. Nippon Rinsho. Nov 2008;66(11):2114-23. [Medline].

  4. Devaraj A, Wells AU, Meister MG, Corte TJ, Hansell DM. The effect of diffuse pulmonary fibrosis on the reliability of CT signs of pulmonary hypertension. Radiology. Dec 2008;249(3):1042-9. [Medline].

  5. Kovacs G, Reiter G, Reiter U, Rienmüller R, Peacock A, Olschewski H. The emerging role of magnetic resonance imaging in the diagnosis and management of pulmonary hypertension. Respiration. 2008;76(4):458-70. [Medline].

  6. [Best Evidence] Galiè N, Manes A, Negro L, Palazzini M, Bacchi-Reggiani ML, Branzi A. A meta-analysis of randomized controlled trials in pulmonary arterial hypertension. Eur Heart J. Feb 2009;30(4):394-403. [Medline].

  7. 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].

  8. Abman SH. Neonatal pulmonary hypertension: A physiologic approach to treatment. Pediatr Pulmonol. Feb 2004;37 Suppl 26:127-8.

  9. Aschner JL. New therapies for pulmonary hypertension in neonates and children. Pediatr Pulmonol. Feb 2004;37 Suppl 26:132-5.

  10. Christman BW, McPherson CD, Newman JH, et al. An imbalance between the excretion of thromboxane and prostacyclin metabolites in pulmonary hypertension. N Engl J Med. Jul 9 1992;327(2):70-5. [Medline].

  11. Frank H, Mlczoch J, Huber K, et al. The effect of anticoagulant therapy in primary and anorectic drug- induced pulmonary hypertension. Chest. Sep 1997;112(3):714-21. [Medline].

  12. Fraser RG, Pare JA, Pare PD. Pulmonary hypertension and edema. In: Diagnosis of Diseases of the Chest. Vol 3. 4th ed. Philadelphia: WB Saunders;. 1999.

  13. Gaine SP, Rubin LJ. Primary pulmonary hypertension [published erratum appears in Lancet 1999 Jan 2;353(9146):74]. Lancet. Aug 29 1998;352(9129):719-25. [Medline].

  14. Gonçalves RC, Buschpigell CA, Lopes AA. Circulating blood volumes in pulmonary hypertension associated with erythrocytosis--the effects of therapeutic hemodilution. Cardiol Young. Dec 2003;13(6):544-50. [Medline].

  15. Grossman W, Braunwald E. Pulmonary hypertension. In: Heart Disease: A Textbook of Cardiovascular Medicine. 4th ed. Philadelphia: WB Saunders;. 1997.

  16. Lilienfeld DE, Rubin LJ. Mortality from primary pulmonary hypertension in the United States, 1979-1996. Chest. Mar 2000;117(3):796-800. [Medline].

  17. Miller SW, Waltman AC. The pulmonary circulation. In: Radiology: Diagnosis, Imaging, Intervention. Philadelphia: Lippincott;. 2001.

  18. Morgan JM, Griffiths M, du Bois RM, Evans TW. Hypoxic pulmonary vasoconstriction in systemic sclerosis and primary pulmonary hypertension. Chest. Mar 1991;99(3):551-6. [Medline].

  19. Pistolesi M, Milne ENC. Radiology of cardiopulmonary hemodynamic disturbances. In: Radiology: Diagnosis, Imaging, and Intervention. Philadelphia: Lippincott;. 2001.

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

  21. Rich S, Kaufmann E, Levy PS. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N Engl J Med. Jul 9 1992;327(2):76-81. [Medline].

  22. Rich S, Levitsky S, Brundage BH. Pulmonary hypertension from chronic pulmonary thromboembolism. Ann Intern Med. Mar 1988;108(3):425-34. [Medline].

  23. Rosenzweig EB, Kerstein D, Barst RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation. Apr 13 1999;99(14):1858-65. [Medline].

  24. Rubin LJ. Primary pulmonary hypertension. Chest. Jul 1993;104(1):236-50. [Medline].

  25. Rubin LJ. Primary pulmonary hypertension. N Engl J Med. Jan 9 1997;336(2):111-7. [Medline].

  26. Weiss BM, Zemp L, Seifert B, Hess OM. Outcome of pulmonary vascular disease in pregnancy: a systematic overview from 1978 through 1996. J Am Coll Cardiol. Jun 1998;31(7):1650-7. [Medline].

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Keywords

pulmonary hypertension, pulmonary heart disease, vascular disease, hypertension, persistent fetal circulation syndrome, cardiovascular disease, primary pulmonary hypertension, PPH, secondary pulmonary arterial hypertension, SPAH

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Contributor Information and Disclosures

Author

Davinder Jassal, MD, BSc, FACC, FRCPC, Clinical and Research Cardiac Echocardiography Fellow, Department of Cardiology, Massachusetts General Hospital, Harvard Medical School
Davinder Jassal, MD, BSc, FACC, FRCPC is a member of the following medical societies: American College of Cardiology, American College of Physicians-American Society of Internal Medicine, American Heart Association, and Canadian Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Sat Sharma, MD, FRCPC, Professor and Head, Division of Pulmonary Medicine, Department of Internal Medicine, University of Manitoba; Site Director, Respiratory Medicine, St. Boniface General Hospital
Sat Sharma, MD, FRCPC is a member of the following medical societies: American Academy of Sleep Medicine, American College of Chest Physicians, American College of Physicians-American Society of Internal Medicine, American Thoracic Society, Canadian Medical Association, Royal College of Physicians and Surgeons of Canada, Royal Society of Medicine, Society of Critical Care Medicine, and World Medical Association
Disclosure: Nothing to disclose.

Bruce Maycher, MD, Director of Pulmonary Radiology, St Boniface General Hospital; Associate Professor, Department of Radiology, University of Manitoba
Bruce Maycher, MD is a member of the following medical societies: American Roentgen Ray Society, Canadian Medical Association, Radiological Society of North America, and Society of Thoracic Radiology
Disclosure: Nothing to disclose.

Medical Editor

Judith K Amorosa, MD, FACR, Clinical Professor and Program Director, Department of Radiology, University of Medicine and Dentistry of New Jersey, Robert Wood Johnson Medical School; Consulting Staff, Department of Radiology, Robert Wood Johnson University Hospital
Judith K Amorosa, MD, FACR is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, Association of University Radiologists, Radiological Society of North America, and Society of Thoracic Radiology
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

Managing Editor

John D Newell, Jr, MD, FACR, FCCP, FASER, Co-Director of Thoracic Imaging, UCDHSC; Director of Lung Imaging Center, Professor of Radiology and Professor of Medicine, Department of Radiology, University of Colorado Health Sciences Center, National Jewish Medical and Research Center; Univ. Colorado Hospital
John D Newell, Jr, MD, FACR, FCCP, FASER is a member of the following medical societies: American College of Chest Physicians, American College of Radiology, American Roentgen Ray Society, American Thoracic Society, Association of University Radiologists, Radiological Society of North America, and Society of Thoracic Radiology
Disclosure: Siemens Medical Grant/research funds Consulting; Forevision Technologies Ownership interest Consulting; Vida Corporation Ownership interest Board membership; TeraRecon Grant/research funds Consulting; eMedicine Honoraria Consulting

CME Editor

Robert M Krasny, MD, Consulting Staff, Department of Radiology, Resolution Imaging Medical Corporation
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

Eugene C Lin, MD, Consulting Radiologist, Virginia Mason Medical Center; Clinical Assistant Professor of Radiology, University of Washington School of Medicine
Eugene C Lin, MD is a member of the following medical societies: American College of Nuclear Medicine, American College of Radiology, Radiological Society of North America, and Society of Nuclear Medicine
Disclosure: Nothing to disclose.

 
 
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