Pulmonary Hypertension
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More Specific Terms
Introduction
- pulmonary artery pressure of > 25 mm Hg at rest or > 30 mm Hg with exercise. At least 50% of the vascular bed must be occluded to produce clinical pulmonary hypertension.
Etiology
- (see causes of pulmonary hypertension)
- left atrial hypertension
- mitral stenosis
- heart failure, left ventricular failure (most common USA)
- chronic hypoxemia - COPD
- severe nocturnal hypoxemia ( sleep apnea)
- pulmonary fibrosis
- left to right shunts - atrial septal defect
- vascular diseases of the lung
- chronic thromboembolism
- connective tissue disease
- AIDS
- schistosomiasis (world-wide most common cause)
- sickle cell disease (older patients) [8]
- familial, congenital
- pharmaceutical agents: analeptics
- idiopathic ( primary pulmonary hypertension)
Pathology
- vascular
- arterial intimal proliferation & arteriolar constriction secondary to alveolar hypoxia
- increased pulmonary blood flow from left to right shunts
- increased pulmonary venous pressure from left heart failure
- perivascular parenchymal changes resisting blood flow
- intravascular obstruction
- cor pulmonale may result from right ventricular overload
Clinical-manifestations
- (also see primary pulmonary hypertension)
- exertional dyspnea
- fatigue
- syncope
- peripheral edema
- persistent unexplained hoarseness
- right ventricular heave
- loud pulmonic valve component of the 2nd heart sound P2
- systolic murmur of tricuspid regurgitation
- right-sided S3 heart sound
Laboratory
- antinuclear antibody ( ANA)
- HIV testing
- serum pro-BNP improves predictive value in patients with sickle cell disease [8]
Diagnostic-procedures
-
- elevated pulmonary artery pressure
- normal range: 15-30/5-13 ( mean 10-18) mm Hg
- rule out mitral stenosis
- bubble- contrast or transesophageal echocardiogram for congenital heart disease
- electrocardiogram: ( EKG)
- right ventricular hypertrophy
- RVH by EKG found in only 1/3 of patients with COPD & cor pulmonale
- RBBB often occurs
- right axis deviation
- T wave changes [9]
- 6- minute walk improves predictive value in patients with sickle cell disease [8]
- pulmonary artery catheterization (see primary pulmonary hypertension)
- acute vasoreactivity study
- assess response to vasodilator(s)
- polysomnography to rule out severe nocturnal hypoxemia
- open lung biopsy may be indicated
Radiology
-
- enlarged right ventricle
- dilated pulmonary arteries [9]
- V/Q scan to rule out pulmonary embolism
- pulmonary angiography may be appropriate if VQ scan is equivoval
Complications
Management
- treat underlying cause
- supplemental oxygen in patients that are hypoxemic
- anticoagulation with warfarin unless contraindicated [3]
- this recommendation in the absence of thromboembolism not well understood
- effective in some patients with primary pulmonary hypertension (pulmonary Raynaud's phenomenon)
- invasive hemodynamic monitoring required prior to initiating therapy to verify vasoreactivity (see primary pulmonary hypertension)
- adverse effect of possibly worsening gas exchange
- nifedipine 120-240 mg QD
- diltiazem 540-900 mg QD
- patients with severe pulmonary hypertension not responding to calcium channel antagonists
- endothelin receptor antagonists
- prostacyclin analogues
- sildenafil improves exercise capacity, functional class, & hemodynamics [5] in patients with mild pulmonary hypertension
- nitrates may have short-term benefit
- diuretics
- pulmonary embolism
- anticoagulation if pulmonary embolism suspected
- surgical thromboendarterectomy if indicated in patients with pulmonary embolism
- chronic anticoagulation
- inferior vena cava interruption ( Greenfield filter)
- lung or heart/ lung transplantation
- advise against pregnancy if severe; maternal mortality 30-50%
- screen for portal hypertension prior to liver transplantation
- screening:
- 1st degree relatives of patients with familial pulmonary hypertension
- congenital heart disease with systemic-to-pulmonary shunt
More General Terms
Additional Terms
- causes of pulmonary hypertension
- cor pulmonale (right heart failure, right ventricular failure)
- pulmonary artery pressure (PAP)
- right ventricular hypertrophy (RVH)
References
- Manual of Medical Therapeutics, 28th ed, Ewald & McKenzie (eds), Little, Brown & Co, Boston, 1995, pg 256
- Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 778-81
- Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15 American College of Physicians, Philadelphia 1998, 2006, 2009
- Smith R., Jewish Home for the Aging, Reseda CA, 2001, unpublished
- Galie N et al, Sildenafil citrate therapy for pulmonary arterial hypertension N Engl J Med 353(20):2148, 2005 PMID: [1]
- Badesch DB, Abman SH, Simonneau G, Rubin LJ, McLaughlin VV. Medical Therapy for Pulmonary Arterial Hypertension: Updated ACCP Evidence-Based Clinical Practice Guidelines. Chest. 2007 Jun;131(6):1917-28. <PubMed> PMID: [2] <Internet> [3]
- McLaughlin VV et al ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension. A Report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association. Circulation. 2009 Mar 30 <PubMed> PMID: [4] <Internet> [5]
- Parent F et al. A hemodynamic study of pulmonary hypertension in sickle cell disease. N Engl J Med 2011 Jul 7; 365:44. PMID: [6]
- Geriatrics at your Fingertips, 13th edition, 2011 Reuben DB et al (eds) American Geriatric Society
- National Guideline Clearinghouse Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology. ngc-guideline: [7] (1) Medical therapy for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. (2) 2007 addendum. American College of Chest Physicians ngc-guideline: [8]
