Term
| Pulmonary Arterial Hypertension (PAH) |
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Definition
| syndrome resulting from restricted flow through pulmonary arterial circulation resulting in increased pulmonary vascular resistance & ultimately right HF; |
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Term
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Definition
| exertional dyspnea; fatigue; weakness; complaints of exertion intolerance; dyspnea at rest with disease progression; anginal chest pain; syncope; |
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Term
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Definition
| leg swelling; abdominal bloating & distention; anorexia; plethora; profound fatigue; |
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Term
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Definition
| accentuated component of S2 audible at apex of heart; early systolic ejection click; midsystolic ejection murmur; palpable left parasternal lift; right ventricular S4 gallop; prominent "a" wave; |
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Term
| Risk Factors associated with PAH |
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Definition
Drugs & toxins: amiorex, fenfluramine, dexfenfluramine, toxic rapeseed oil, amphetamines, L-tryptophan, meta-amphetamines, cocaine;
Gender (females > males);
Pregnancy;
Systemic HTN;
Diseases: HIV, portal HTN/liver dx, connective tissue dx, congenital systemic-pulmonary cardiac shunts, thryoid disorders, hematological conditions; |
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Term
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Definition
restricted flow through pulmonary arterial ciculation resulting in increased pulmonary vascular resistance (PVR) and ultimately right heart failure;
caused by increased PVR:
- loss of vascular luminal cross section due to vascular remodeling;
- excessive vasoconstriction; |
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Term
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Definition
| pts w/ NO limitation of usual physical activity; ordinary activity DOES NOT cause increased S/Sx |
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Term
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Definition
| pts have mild limitation of physical activity; NO discomfort at rest, BUT normal physical activity causes increased S/sx |
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Term
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Definition
| Pts who have MARKED limitation of physical activity; NO discomfort at rest, but LESS THAN NORMAL physical activity causes S/Sx |
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Term
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Definition
| pts who are unable to perform ANY physical activity at rest and may show Sx of righty ventricular failure; S/Sx may be present at rest and are increased by any physical activity; |
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Term
| Acute Vasodilator Testing |
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Definition
administration of pharmacologic agnest to test presence of pulmonary vasoreactivity;
Acute responder: reduction in mPAP of 10 mmHg to an absolute mPAP of <40 mmHg w/o a decrease in cardiac output;
Positive Result: candidate for long-term CCB therapy;
Perform in ALL Idiopathic PAH pts;
C/I: pts w/ over right-sided HF or hemodynamic instability |
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Term
| More advanced PAH & requirement of parenteral or combo therapy |
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Definition
| requires office visit every 3 months (or more frequently) |
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Term
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Definition
| Requires an office visit every 3 to 6 months |
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Term
| Hemodynamic Diagnosis of PAH |
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Definition
REQUIREMENTS:
- ELEVATED PVR & ELEVATED mPAP in setting of normal left heart filling pressure;
Diagnosis of Exclusion:
- exclude: lung dx, thromboembolic dx, LV dx, valvular dx;
AND:
- mPAP >25 mmHg, PCWP or LVEDP <15 mmHg, PVR >3 Wood units; |
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Term
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Definition
alleviation of symptoms;
improvement of QOL;
prevention of dx progression;
improvement in survival; |
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Term
| Nonpharmacological Approach to PAH |
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Definition
Use positive airway pressure in pts w/ sleep disorder breathing;
Immunizations;
Avoid hypobaric hypoxia (commercial airplanes);
Limit Na intake (<2400 mg/day);
Cardiopulmonary rehab;
Support groups & psychological counseling to help improve QOL;
AVOID: meds that interact w/ warfarin; NSAIDs;
Use w/ Caution in pts w/ PAH: ACEIs, ARBs, Beta-blockers; |
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Term
| Surgical Approach to Treatment |
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Definition
apical septostomy;
pulmonary thromboendarterectomy;
lung or heart & lung transplant; |
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Term
| General Pharmacological Care Interventions for PAH |
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Definition
oral anticoagulation (goal INR: 1.5-2.5);
diuretics;
oxygen;
digoxin; |
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Term
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Definition
pts w/ PAH display multiple risk factors for venous thromboembolism;
pts w/ PAH demonstrate thrombotic predisposition & thrombotic changes in pulmonary circulation;
INR goal: 1.5-2.5 |
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Term
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Definition
used in pts w/ decompensated right heart failure;
Symptomatic pts:
- increased central venous pressure;
- abdominal organ congestion;
- peripheral edema & ascites; |
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Term
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Definition
| maintain O2 saturation at >90%; |
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Term
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Definition
used in PAH based on clinical judgement, not scientific evidence;
used in pts w/ right HF & PAH as adjunctive therapy to control sx & for atrial flutter to slow ventricular rate;
Goal plasma conc.: 0.5-0.8 ng/mL |
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Term
| Dihydropyridine CCBs (amlodipine, felodipine) |
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Definition
used in small pop. of pts w/ a POSITIVE response to acute vasodilator test;
Idiopathic PAH pts most likely to respond to acute vasodilators & CCBs;
Preferred because of lack in inotropic effects;
Assess therapy after 3 months;
If no improvement in functional Class I or II, alternative therapy should be instituted --> remove from drug as pt progresses; |
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Term
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Definition
potent & highly selective PDE-V inhibitor;
Reduces mPAP & improves pts functional class;
MoA: increases intracellular cGMP --> vasorelaxation & antiproliferative effects on vascular smooth muscle cells;
Recommended for Class II pts w/ PAH |
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Term
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Definition
induces potent vasodilation of all vascular beds;
potent inhibitor of plt aggregation;
possess cytoprotective & antiproliferative properties; |
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Term
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Definition
prostacyclin analog;
Short half-life: 3-5 min;
Give by continuous infusion;
ADR: increased risk of bloodstream infections;
Recommended for Class III pts w/ PAH; |
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Term
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Definition
prostacyclin analog given SC or IV;
Major ADV: ease of use, less potential for bloodstream infections if given SC;
ADR: SC injection site pain;
Improves clinical outcomes;
2nd or 3rd line for pts w/ Class II, III, or IV PAH |
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Term
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Definition
prostacyclin analog given by inhalation;
utilized as add-on therapy with bosentan;
Recommended for Class III & IV PAH |
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Term
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Definition
| powerful vasoconstrictor & mitogen for smooth muscle; activation of ET-1 system has been shown in both plasma & lung in PAH pts; |
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Term
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Definition
endothelin receptor antagonist;
improves exercise capacity, functional class, hemodynamics, ECHO, & Doppler variables;
ADR: increases LFTs;
If LFTs are 3-5x normal limit, reduce daily dose or D/C tx;
Monitor LFTs at baseline & monthly;
Pregnancy Cat. X;
Recommended for 1st line in Class III or IV PAH |
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Term
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Definition
orphan drug;
endothelin receptor antagonist;
improves exercise capacity, hemodynamics, & clinical events in PAH;
may increase INR in pts taking warfarin; |
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