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Pulmonary Arterial Hypertension
Aka: Pulmonary Arterial Hypertension, Primary Pulmonary Hypertension, Pulmonary Hypertensive Crisis, Idiopathic Pulmonary Arterial Hypertension, Familial Pulmonary Arterial Hypertension
- See Also
- Pulmonary Arterial Hypertension Crisis
- Pulmonary Hypertension
- Pulmonary Hypertension Causes
- Pulmonary Hypertension Diagnosis
- Pulmonary Hypertension Management
- Epidemiology
- Incidence: 1-2 cases per 1 million (rare)
- Female predominance after Puberty in ratio of 1.7 to 1
- Most prevalent in ages 20 to 40 years old
- Causes
- See Pulmonary Arterial Hypertension Causes
- Idiopathic or familial
- Genetic associations
- BMPR2 gene
- ALK1 gene
- Endoglin (with or without Hereditary Hemorrhagic Telangiectasia)
- Associated Conditions
- Collagen Vascular Disease (e.g.` Systemic Sclerosis)
- HIV Infection
- Includes Persistent Pulmonary Hypertension of the Newborn
- Predisposing medication use (see Pulmonary Arterial Hypertension Causes)
- Pathophysiology
- Progressive narrowing of distal pulmonary arteries
- Processes
- Inflammation
- Hypercoagulability
- Pre-capillary pulmonary arteriole disorder
- Endothelial dysfunction
- Excess cell proliferation and dysfunctional apoptosis
- Vasconstriction
- Mediators
- Nitric oxide
- Prostacyclin
- Reduces platelet aggregation and causes vasodilation
- Reduced in PAH
- Cyclic guanosine monophosphate (cGMP)
- Vasodilator
- cGMP is rapidly degraded by PDE5 (Phosphodiesterase 5) which present in right ventricle of PAH patients
- Endothelin-1
- Vasoconstricor and smooth muscle mitogen
- Increased in PAH
- Pulmonary circulation obstruction results from underlying factors
- Increased pulmonary vascular resistance
- Contrast with the normal low-resistance, high compliance pulmonary circulation
- Right Ventricular Afterload
- Right ventricle gradually compensates for increasing right Ventricular Afterload increases
- Right ventricular increased pressure and volume force interventricular septum to bow into left ventricle
- Results in decreased left ventricular filling and ejection fraction
- Ultimately compensation fails, resulting in Right Ventricular Failure
- Right ventricle is unable to compensate for acute increases to Afterload resulting in PAH Crisis
- Symptoms
- See Pulmonary Hypertension
- Dyspnea (60%)
- Fatigue (19%)
- Syncope or Presyncope (13%)
- Chest Pain
- Palpitations
- Signs
- See Pulmonary Hypertension
- Signs of underlying cause (e.g. Portal Hypertension, Connective Tissue Disease)
- Signs of right ventricular Heart Failure
- Jugular Venous Distention
- Hepatomegaly
- Ascites
- Peripheral Edema
- Tricuspid regurgitation murmur
- Diagnosis
- See Pulmonary Hypertension Diagnosis for labs, imaging and diagnostics
- Management
- See Pulmonary Arterial Hypertension Management
- Complications
- See Pulmonary Arterial Hypertension Crisis (PAH Crisis)
- Prevention
- Avoid predisposing factors (e.g. weight loss drugs)
- Avoid pregnancy (mortality risk approaches 50% in some studies)
- Vaccinations
- Influenza Vaccine
- Pneumococcal Vaccine
- Advanced Directives: Severe Pulmonary Arterial Hypertension
- Advise Do-Not-Resuscitate (very poor outcomes for ROSC)
- Discuss end-of-life wishes
- Prognosis
- Median survival from diagnosis
- Prior medications: 2.8 years
- New medications: may exceed 10 years
- References
- Meter (2013) Crit Dec Emerg Med 27(5): 2-10
- Dunlap (2016) Am Fam Physician 94(6):463-9 [PubMed]
- Galie (2009) Eur Heart J 30(20): 2493-537 [PubMed]
- Simmonneau (2009) J Am Coll Cardiol 54(1 Suppl): S43-54 [PubMed]