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.

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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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