primary pulmonary hypertension (PPH)
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Introduction
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
- genetic form(s)
- may occur in association with anorexiant use
- idiopathic
Epidemiology
- uncommon
- may occur from infancy throughout life
- primarily in young to middle-aged women
- mean age at onset of 36 years
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
- clinical course is variable
- most patients present with advanced stages of disease
- gradual progressive dyspnea (90%)
- fatigue (25%)
- dizziness
- syncope (10%)
- right-sided angina (20%)
- hemoptysis (10%)
- cough (30%)
- hoarseness
- Raynaud's phenomenon (10%)
- loud P2
- right ventricular heave (80%)
- pulmonary ejection murmur (70%)
- S3 or S4 (50%)
- pulmonary regurgitation (20%)
- hepatomegaly (20%)
- peripheral edema
- ascites
- cyanosis
Diagnostic criteria
by exclusion
Laboratory
- antinuclear antibody (ANA) may be positive (40%)
- arterial blood gas (ABG) - exercise-induced hypoxia
- also see pulmonary hypertension
Diagnostic procedures
- pulmonary function testing
- echocardiography
- electrocardiogram:
- right axis deviation
- tall R waves in V1, V2
- ST segment depression
- T wave inversion
- right bundle branch block may occur
- peaked T waves may be seen
- pulmonary artery catheterization with vasodilator test
- IV vasodilator (adenosine, epoprostenol)
- inhaled nitric oxide
- vasoreactivity verified by
- reduction in mean pulmonary artery pressure of at least 10 mm of Hg to a value <= 40 mm Hg
- no decrease in cardiac output with vasodilation[3]
- polysomnography as indicated
- open lung biopsy may be indicated
- also see pulmonary hypertension
Radiology
- chest radiograph
- normal in 6% of patients
- enlarged pulmonary arteries (90%)
- pruning of peripheral vessels (50%)
- clear lung fields
- V/Q scan to exclude multiple pulmonary emboli
- pulmonary angiography may be appropriate
- high-resolution lung CT to rule out interstitial lung disease
- also see pulmonary hypertension
Management
- supplemental oxygen in patients that are hypoxemic (SaO2 < 90%)
- chronic anticoagulation
- chronic thromboembolic pulmonary hypertension (lifelong therapy)
- this recommendation in the absence of thromboembolism not well understood
- calcium channel antagonists
- invasive hemodynamic monitoring required to establish vasoreactivity before initiating therapy
- effective in some patients with primary pulmonary hypertension & vasoreactivity on testing
- may improve[1] right ventricular function[2] oxygen delivery[3] survival
- adverse effect of possibly worsening gas exchange
- if vasoreactivity testing negative
- prostanoids reserve for patients with advanced disease
- treprostinil
- inhaled iloprost
- intravenous prostacyclin (epoprostenol)
- diuretics
- lung transplantation
- prognosis is poor:
- generally results in death within several years of diagnosis
More general terms
More specific terms
References
- ↑ 1.0 1.1 Manual of Medical Therapeutics, 28th ed, Ewald & McKenzie (eds), Little, Brown & Co, Boston, 1995, pg 256
- ↑ 2.0 2.1 Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 779
- ↑ 3.0 3.1 3.2 Medical Knowledge Self Assessment Program (MKSAP) 11, 14, American College of Physicians, Philadelphia 1998, 2006