primary pulmonary hypertension is...

Primary pulmonary hypertension is pulmonary hypertension with no or unknown cause. Some associations with co-existing diseases can be seen, such as the prior use of certain diet pills, cirrhosis of the liver, etc. In these cases, the cause of PPH is unknown.


some references from the current literature:

1.Loyd JE. Genetics and pulmonary hypertension. Chest. 2002 Dec;122(6 Suppl):284S-6S..

Latest on familial PPH

2.McLaughlin VV, Shillington A, Rich S. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation. 2002 Sep 17;106(12):1477-82.

More long-term evidence for the salutary effects of Flolan

3.Gaine S. Pulmonary hypertension. JAMA. 2000 Dec 27;284(24):3160-8.

A wonderful review of PPH, very current, nice diagram

4.Rubin LJ. Current Concepts: Primary pulmonary hypertension. New England Journal of Medicine, 1997 Jan 9, 336(2):111-7.

An older yet excellent review of PPH

5. Abenhaim L; Moride Y; Brenot F; Rich S; Benichou J; Kurz X; Higenbottam T; Oakley C; Wouters E; Aubier M; et al. Appetite-suppressant drugs and the risk of primary pulmonary hypertension. International Primary Pulmonary Hypertension Study Group. New England Journal of Medicine, 1996 Aug 29, 335(9): 609-16.

Important information on the risks of appetite suppressants

6.Barst RJ; Rubin LJ; Long WA; McGoon MD; Rich S; Badesch DB; Groves BM;Tapson VF; Bourge RC; Brundage BH ; et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study Group. New England Journal of Medicine, 1996 Feb 1, 334(5):296-302.

Excellent controlled clinical trial demonstarting the benefits of prostacyclin in patients with severe PPH.

7.Fishman AP. Etiology and pathogenesis of primary pulmonary hypertension: a perspective. Chest 1998 Sep;114(3 Suppl):242S-247S.

Review of current mechanisms and pathophysiology in PPH.

8.Simonneau G; Fartoukh M; Sitbon O; Humbert M; Jagot JL; Herve P. Primary pulmonary hypertension associated with the use of fenfluramine derivatives. Chest 1998 Sep;114(3 Suppl):195S-199S.

Outcomes of patients with exposure to appetite suppressants.

9. Conte JV; Gaine SP; Orens JB; Harris T; Rubin LJ. The influence of continuous intravenous prostacyclin therapy for primary pulmonary hypertension on the timing and outcome of transplantation. J Heart Lung Transplant 1998 Jul;17(7):679-85.

Review of 42 patients with PPH evaluated for lung transplant.

10. Frank H; Mlczoch J; Huber K; Schuster E; Gurtner HP; Kneussl M. The effect of anticoagulant therapy in primary and anorectic drug- induced pulmonary hypertension. Chest 1997 Sep;112(3):714-21.

Retrospective study from Austria showing the benefit of anticoagulation in PPH.

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