Term
| disease of the small pulmonary arteries characterized by vascular proliferation and remodeling |
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Definition
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Term
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Definition
| increased pulmonary vascular resistance, right ventricular failure, and death |
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Term
|
Definition
| has uncertain clinical implications |
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Term
| greater than 25 mmHg at rest |
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Definition
| mean pulmonary artery pressure in pulmonary hypertension |
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Term
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Definition
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Term
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Definition
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Term
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Definition
| Idiopathic, heritable, drug and toxin-induced |
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Term
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Definition
| PH due to left heart disease |
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Term
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Definition
| PH due to lung disease/hypoxia |
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Term
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Definition
| Chronic thromboembolic pulmonary hypertension |
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Term
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Definition
| PH with unclear/multifactorial mechanisms |
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Term
| Type 1 PH associated with |
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Definition
| connective tissue disease; HIV infection; Portal hypertension; congenital heart disease |
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Term
| Type 2 PH associated with |
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Definition
| Systolic or diastolic dysfunction; valvular disease |
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Term
| Type 3 PH associated with |
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Definition
| COPD, Interstitial lung disease, Obstructive Sleep Apnea (OSA) |
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Term
| Type 5 PH associated with |
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Definition
| hematological disorders, vasculitis, sarcoidosis, metabolic disorder, tumor obstruction, dialysis |
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Term
| PH caused by familial factors (<10%) |
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Definition
| Transforming growth factor beta family; mutation in the bone morphogenic protein receptor-2 (BMPR2); Activin-like kinase 1 (ALK1) |
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Term
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Definition
| Appetitie suppressants, L-tryptophan, Illicit drugs |
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Term
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Definition
| fenfluramine (a component of Fen-Phen), Dexfenfluramine |
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Term
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Definition
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Term
| 0.5% of all patients develop PH |
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Definition
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Term
| 2-5% of all patients develop PH |
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Definition
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Term
| 6-60% of all patients develop PH |
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Definition
| Scleroderma, aka systemic sclerosis |
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Term
| 21% of all patients develop PH |
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Definition
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Term
| 4-14% of all patients develop PH |
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Definition
| Systemic lupus erythematosis |
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Term
| 20-40% of all patients develop PH |
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Definition
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Term
| imbalance of endogenous vasoactive mediators (increased endothelin, decreased NO, decreased prostacyclin) |
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Definition
| vasoconstriction; remodeling of vessel wall; thrombosis in situ (clots develop in the lungs) |
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Term
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Definition
| Patients with PH but without resulting limitations of physical activity. Ordinary physical activity does not cause undue fatigue or dyspnea, chest pain, or heart syncope |
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Term
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Definition
| Patients with PH resulting in slight limitation of physical activity. The yare comfortable at rest. Ordinary physical activity results in undue fatigue or dyspnea, chest pain, or heart syncope |
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Term
|
Definition
| patients with PH resulting in marked limitation of physical activity. They are comfortable at rest. Less than ordinary physical activity causes undue fatigue or dyspnea, chest pain, or heart syncope. |
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Term
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Definition
| Patients with PH resulting in inability to carry on any physical activity without symptoms. These patients manifest signs of right heart failure. Dyspnea and/or fatigue may be present even at rest. Discomfort is increased by physical activity |
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Term
| Common end point for drug studies |
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Definition
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Term
| Predictors of poor prognosis |
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Definition
| Age at presentation greater than 45; advanced heart failure, elevated right atrial pressure, poor exercise capacity; decreased oxygen saturation; increased pulmonary vascular resistance; underlying diagnosis of scleroderma spectrum disease |
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Term
| Treatment Options; General Measures |
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Definition
| Exercise/Rehab; Infection prevention; Avoidance of pregnancy; psychosocial support |
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Term
| Primary/Supportive Therapy |
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Definition
| Directed at the underlying causes of PH (Left heart disease, hypoxemia, thromboembolic occlusion) |
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Term
| Treatment of underlying heart disease (left heart disease) |
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Definition
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Term
| Correction of supplemental oxygen (Hypoxemia) |
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Definition
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Term
| Thromboembolic occlusion treatment |
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Definition
| Anticoagulation; surgical thromboendarterectomy |
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Term
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Definition
| Anticoagulation +/- diuretics +/- oxygen +/- Digoxin |
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Term
| Treatment algorithm summary |
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Definition
| supportive therapy; CCBs; Endothelin receptor antoagnists; phosphodiesterase 5 inhibitors; prostanoids; combination therapies; surgical considerations |
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Term
| Acute Vasoreactivity Testing (must be done prior to initiation of CCBs) |
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Definition
| Inhaled nitric oxide; IV epoprostenol, IV adenosine |
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