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primary pulmonary hypertension (PPH)

Primary pulmonary hypertension (PPH) is an uncommon disease of unknown etiology. It occurs most often in young to middle-aged women & usually results in death within several years of diagnosis. Etiology: 1) genetic form(s) 2) may occur in association with anorexiant use 3) idiopathic Epidemiology: 1) uncommon 2) may occur from infancy throughout life 2) primarily in young to middle-aged women - mean age at onset of 36 years Diagnosis: by exclusion Pathology: - disease localized to small pulmonary arterioles resulting in high pulmonary vascular resistance - plexiform lesions of proliferating endothelial cells in pulmonary arterioles - lesions lead to elevated pulmonary arterial pressure, right ventricular failure, & death Clinical manifestations: 1) clinical course is variable 2) most patients present with advanced stages of disease 3) gradual progressive dyspnea (90%) 4) fatigue (25%) 5) dizziness 6) syncope (10%) 7) right-sided angina (20%) 8) hemoptysis (10%) 9) cough (30%) 10) hoarseness 11) Raynaud's phenomenon (10%) 12) loud P2 13) right ventricular heave (80%) 14) pulmonary ejection murmur (70%) 15) S3 or S4 (50%) 16) pulmonary regurgitation (20%) 17) hepatomegaly (20%) 18) peripheral edema 19) ascites 20) cyanosis Laboratory: 1) antinuclear antibody (ANA) may be positive (40%) 2) arterial blood gas (ABG) - exercise-induced hypoxia 3) also see pulmonary hypertension Special laboratory: 1) pulmonary function testing - isolated decrease in DLCO with normal TLC, FEV & FEV1 2) echocardiography 3) electrocardiogram: a) right axis deviation b) tall R waves in V1, V2 c) ST segment depression d) T wave inversion e) right bundle branch block may occur f) peaked T waves may be seen 4) pulmonary artery catheterization with vasodilator test a) IV vasodilator (adenosine, epoprostenol) b) inhaled nitric oxide c) vasoreactivity verified by 1] reduction in mean pulmonary artery pressure of at least 10 mm of Hg to a value <= 40 mm Hg 2] no decrease in cardiac output with vasodilation [3] 5) polysomnography as indicated 6) open lung biopsy may be indicated 7) also see pulmonary hypertension Radiology: 1) chest radiograph a) normal in 6% of patients b) enlarged pulmonary arteries (90%) c) pruning of peripheral vessels (50%) d) clear lung fields 2) V/Q scan to exclude multiple pulmonary emboli 3) pulmonary angiography may be appropriate 4) high-resolution lung CT to rule out interstitial lung disease 5) also see pulmonary hypertension Management: 1) supplemental oxygen in patients that are hypoxemic (SaO2 < 90%) 2) chronic anticoagulation - chronic thromboembolic pulmonary hypertension (lifelong therapy) - this recommendation in the absence of thromboembolism not well understood 3) calcium channel antagonists a) invasive hemodynamic monitoring required to establish vasoreactivity before initiating therapy b) effective in some patients with primary pulmonary hypertension & vasoreactivity on testing c) may improve [1] right ventricular function [2] oxygen delivery [3] survival d) adverse effect of possibly worsening gas exchange 4) if vasoreactivity testing negative - sildenafil, tadalafil - endothelin receptor antagonists (bosentan, ambrisentan) 5) prostanoids reserve for patients with advanced disease a) treprostinil c) inhaled iloprost d) intravenous prostacyclin (epoprostenol) 6) diuretics 7) lung transplantation 8) prognosis is poor: - generally results in death within several years of diagnosis

Interactions

disease interactions

Specific

familial primary pulmonary hypertension

General

pulmonary hypertension

References

  1. Manual of Medical Therapeutics, 28th ed, Ewald & McKenzie (eds), Little, Brown & Co, Boston, 1995, pg 256
  2. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 779
  3. Medical Knowledge Self Assessment Program (MKSAP) 11, 14, American College of Physicians, Philadelphia 1998, 2006