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1、Pulmonary HypertensionPulmonary ModuleSteven W. Harris MHS, PA-C.Pulmonary HypertensionDefined as pressure within the pulmonary arterial system elevated above the normal range. l lgreater than 25 mm Hg at rest or 30 mm Hg greater than 25 mm Hg at rest or 30 mm Hg with exercisewith exercisePulmonary
2、arterial pressure (PAP)l lusually 12-15 mmHgusually 12-15 mmHgLeft atrial pressurel l6-10 mmHg6-10 mmHg7/22/20247/22/2024 2 2.Pulmonary hypertension develops when flow or resistance to flow across the pulmonary vascular bed increasesFour Categories of Pulmonary Hypertension1)1)Pulmonary hypertension
3、 from disorders of Pulmonary hypertension from disorders of the respiratory system or hypoxemia. the respiratory system or hypoxemia. 2)2)Pulmonary venous hypertension. Pulmonary venous hypertension. 3)3)Pulmonary hypertension from chronic Pulmonary hypertension from chronic thromboembolic disease.t
4、hromboembolic disease.4)4)Pulmonary arterial hypertension. (PAH)Pulmonary arterial hypertension. (PAH)7/22/20247/22/20243 3.Chronic pulmonary hypertension is an important cause of right ventricular failure in the United States. Many of the individuals who die each year of chronic obstructive pulmona
5、ry disease (COPD) succumb secondarily to right ventricular failure resulting from pulmonary hypertension. 7/22/20247/22/20244 4.In addition, greater than 200,000 deaths occur yearly from acute pulmonary embolism, the most common cause of sudden-onset pulmonary hypertension and acute right ventricula
6、r failure.7/22/20247/22/20245 5.7/22/20247/22/20246 6.7/22/20247/22/20247 7.7/22/20247/22/20248 8.In persons more than 50 years of age, Cor pulmonale the consequence of untreated pulmonary hypertension, is the third most common cardiac disorder (after coronary and hypertensive heart disease).7/22/20
7、247/22/20249 9.Entities that cause increased pulmonary blood flowCongenital Heart Diseasel lLeft to right shuntLeft to right shunt “ “Hyperkinetic Hyperkinetic pulmonarypulmonary hypertensionhypertension can be can be seen in patients with congenital heart disease who seen in patients with congenita
8、l heart disease who have extensive left-to-right cardiac shunts that have extensive left-to-right cardiac shunts that produce a large pulmonary blood flowproduce a large pulmonary blood flowIncreased cardiac output states l lsevere anemiassevere anemias7/22/20247/22/20241010.Entities that cause an i
9、ncreased resistance to flowPulmonary embolismIn situ pulmonary embolismPulmonary fibrosisSarcoidosis, scleroderma, or extensive pulmonary resectionSevere COPDThoracic deformitiesLarge tumor or infiltrate7/22/20247/22/20241111.Entities that cause changes in arteriolesHypoxia (altitude)COPDHypoventila
10、tion (sleep apnea)AcidosisDrugsPulmonary arterial hypertension (PAH)7/22/20247/22/20241212.Entities that cause venous pressure changes & vascular resistanceLeft atrial hypertension = Mitral StenosisLeft ventricular failurePulmonary venous thrombosisMediastinitis7/22/20247/22/20241313.Pulmonary HTNEl
11、evated pulmonary venous pressure in the setting of left ventricular failure or mitral stenosis is associated with an immediate increase in pulmonary arterial pressure which maintains forward blood flow through the lungs despite the increase in pulmonary venous pressure.7/22/20247/22/20241414.7/22/20
12、247/22/20241515.Entities that increase blood viscosityPolycythemia veraLeukemias with high WBCs7/22/20247/22/20241616.Entities that increase intra-thoracic pressureCOPDMechanical Ventilation: especially with PEEP7/22/20247/22/20241717.SymptomsMild to moderate pulmonary hypertension are often asympto
13、maticMore severe pulmonary hypertension usually complain of dyspnea on exertion secondary to exercise-induced decreases in cardiac output and increases in pulmonary arterial pressure.7/22/20247/22/20241818.SymptomsEasy Easy fatigability, lethargyfatigability, lethargyExertional chest discomfort Exer
14、tional chest discomfort Syncope with exertionSyncope with exertionCoughCoughHemoptysisHemoptysisHoarseness secondary to compression of the Hoarseness secondary to compression of the left recurrent laryngeal nerve by a dilated left recurrent laryngeal nerve by a dilated pulmonary artery(rare)pulmonar
15、y artery(rare)7/22/20247/22/20241919.Physical ExamIncreased intensity of the pulmonic component Increased intensity of the pulmonic component of the second heart sound (P2)of the second heart sound (P2)Systolic ejection murmur from tricuspid Systolic ejection murmur from tricuspid regurgAdvanced Dis
16、easeregurgAdvanced DiseaseDiastolic murmur of pulmonic regurgitation in Diastolic murmur of pulmonic regurgitation in patients with severe pulmonary hypertensionpatients with severe pulmonary hypertensionEvidence of right ventricular dilatation (left Evidence of right ventricular dilatation (left pa
17、rasternal lift or heave)parasternal lift or heave)A pulse prominent a wave in jugular venous A pulse prominent a wave in jugular venous systemsystem7/22/20247/22/20242020.PE (contd)Signs of right ventricular failure: jugular venous distensionRight ventricular S4 (increased loudness of the S4 on insp
18、iration)HepatomegalyAscites, and/or peripheral edema 7/22/20247/22/20242121.CaveatsPatients with severe emphysema and increased thoracic anteroposterior diameter may not display the findings usually associated with advanced pulmonary hypertension because chest expansion make palpation and auscultati
19、on more difficult.The JVD may actually be above the jaw-line!7/22/20247/22/20242222.Incidence of S & Sl lLoud P2 ( 80%) Loud P2 ( 80%) l lRight ventricular lift ( 80%) Right ventricular lift ( 80%) l lDyspnea ( 75%) Dyspnea ( 75%) l lMurmur of tricuspid insufficiency (50-80%) Murmur of tricuspid ins
20、ufficiency (50-80%) l lIncreased jugular venous pressure (50-80%) Increased jugular venous pressure (50-80%) l lRight ventricular S4 (50-80%) Right ventricular S4 (50-80%) l lChest pain ( 50%) Chest pain ( 50%) l lFatigue ( 50%) Fatigue ( 50%) 7/22/20247/22/20242323.S & S contdl lPalpitations ( 50%)
21、 Palpitations ( 50%) l lSyncope; dizziness ( 50%) Syncope; dizziness ( 50%) l lCough ( 50%) Cough ( 50%) l lRaynauds phenomenon ( 10%) Raynauds phenomenon ( 10%) l lHepatomegaly ( 50%) Hepatomegaly ( 50%) l lPulmonic ejection click ( 50%) Pulmonic ejection click ( 50%) 7/22/20247/22/20242424.S & S c
22、ontdl lRight ventricular S3 ( 50%) Right ventricular S3 ( 50%) l lMurmur of pulmonic insufficiency ( 50%) Murmur of pulmonic insufficiency ( 50%) l lLower extremity edema ( 50%) Lower extremity edema (males (1.7:1)7/22/20247/22/20242626.Causes of PAHUnknown (IPAH)HIVDrugsl lAppetite suppressants(fen
23、fluramine and Appetite suppressants(fenfluramine and dexfenfluramine) dexfenfluramine) l lAmphetamines Amphetamines l lCocaineCocaine7/22/20247/22/20242727.7/22/20247/22/20242828.Diagnostic Testsl lChest x-ray, pulmonary function testsChest x-ray, pulmonary function testsl lALL should have a V/Q sca
24、n r/o PEALL should have a V/Q scan r/o PEl lEKGEKGl l2-D echocardiography with Doppler flow study2-D echocardiography with Doppler flow studyl ldisease disease l lSleep studySleep studyl l6 minute walk6 minute walkl lCardiac catheterization - right heart Cardiac catheterization - right heart cathete
25、rization is necessary to measure catheterization is necessary to measure pulmonary artery pressures and pulmonary artery pressures and hemodynamics; rule out underlying cardiachemodynamics; rule out underlying cardiac7/22/20247/22/20242929.EKG FindingsRVH (late finding)l lRADRADRBBBRV strain (acute
26、ie Pulm embolus)l lS wave in lead IS wave in lead Il lQ wave/ inverted T in Lead IIIQ wave/ inverted T in Lead III7/22/20247/22/20243030.7/22/20247/22/20243333.7/22/20247/22/20243434.Diagnostic Testsl lPulmonary angiography - should be done if Pulmonary angiography - should be done if segmental or l
27、arger defect on V/Q scan. segmental or larger defect on V/Q scan. Caution in pulmonary hypertension as can Caution in pulmonary hypertension as can lead to hemodynamic collapse; use low lead to hemodynamic collapse; use low osmolar agents, subselective angiograms. osmolar agents, subselective angiog
28、rams. l lLung biopsy not recommended Lung biopsy not recommended 7/22/20247/22/20243535.7/22/20247/22/20243636.World Health Organization Classification of Functional Status of Patients with PHClass I - patients with PH who experience no limitation of Class I - patients with PH who experience no limi
29、tation of usual physical activity; ordinary physical activity does not usual physical activity; ordinary physical activity does not cause increased dyspnea, fatigue, chest pain or cause increased dyspnea, fatigue, chest pain or presyncope presyncope Class II - patients with PH who have mild limitati
30、on of Class II - patients with PH who have mild limitation of physical activity. There is no discomfort at rest, but physical activity. There is no discomfort at rest, but normal physical activity causes increased dyspnea, normal physical activity causes increased dyspnea, fatigue, chest pain or pre
31、syncope fatigue, chest pain or presyncope Class III - patients with PH who have marked limitation of Class III - patients with PH who have marked limitation of physical activity. There is no discomfort at rest, but less physical activity. There is no discomfort at rest, but less than ordinary activi
32、ty causes increased dyspnea, fatigue, than ordinary activity causes increased dyspnea, fatigue, chest pain, or presyncope chest pain, or presyncope Class IV - patients with PH who are unable to perform Class IV - patients with PH who are unable to perform any physical activity at rest and who may ha
33、ve signs of any physical activity at rest and who may have signs of right ventricular failure. Dyspnea and/or fatigue may be right ventricular failure. Dyspnea and/or fatigue may be present at rest, and symptoms are increased by almost present at rest, and symptoms are increased by almost any physic
34、al activity any physical activity 7/22/20247/22/20243737.Tx of Pulmonary HTNl lTreat the cause!Treat the cause!l lPhosphodiesterase inhibitors (sildenafil)Phosphodiesterase inhibitors (sildenafil) RevatioRevatiol lProsanoids (prostacyclin analogues)Prosanoids (prostacyclin analogues) Flolan, Ilopros
35、tFlolan, Iloprostl lEndothelin receptor antagonistsEndothelin receptor antagonists BosentanBosentanl lVasodilatorsVasodilators L-arginine?L-arginine?l lCoumadinCoumadin7/22/20247/22/20243838.Tx of Pulmonary HTNPhosphodiesterase inhibitors (sildenafil) RevatioRevatiol lNitric oxide stimulation of end
36、othelium Nitric oxide stimulation of endothelium increases cGMP resulting in vasorelaxationincreases cGMP resulting in vasorelaxationl lPhosphodiesterase breaks down cGMPPhosphodiesterase breaks down cGMP7/22/20247/22/20243939.Tx of Pulmonary HTNProsanoids (prostacyclin analogues) Flolan, IloprostFl
37、olan, Iloprostl lProstacyclin is a potent vasodilator produced Prostacyclin is a potent vasodilator produced in the vascular endothelium . Also inhibits in the vascular endothelium . Also inhibits growth of smooth muscle cells growth of smooth muscle cells 7/22/20247/22/20244040.Tx of Pulmonary HTNE
38、ndothelin receptor antagonists BosentanBosentanl lEndothelan-1 is a potent vasoconstrictor, Endothelan-1 is a potent vasoconstrictor, induces fibosis and leads to proliferation of induces fibosis and leads to proliferation of smooth-muscle cellssmooth-muscle cells7/22/20247/22/20244141.Tx of Pulm HT
39、NTreatment of heart failure (e.g., diuretics) Treatment of heart failure (e.g., diuretics) Oxygen supplementation is indicated for rest, Oxygen supplementation is indicated for rest, exercise, or nocturnal hypoxemia exercise, or nocturnal hypoxemia Calcium channel blockers (nifedipine, diltiazemCalc
40、ium channel blockers (nifedipine, diltiazeml lOnly in selected patients that qualify via a Only in selected patients that qualify via a vasoreactivity study. vasoreactivity study. Digoxin (afterload reduction & increase cardiac Digoxin (afterload reduction & increase cardiac output)output)Surgery of
41、 thrombolic emboliSurgery of thrombolic emboliLow salt dietLow salt dietCautious exerciseCautious exerciseHeart-lung transplantHeart-lung transplant7/22/20247/22/20244242.PrognosisSurvival at 1 year 63%Survival at 2 years 45%Improves to 87% survival at 2 years with targeted therapyMean age at diagnosis 34 years Mode of death: Most from Right Vent failure7/22/20247/22/20244343.Easy Readinghttp:/www.mayoclinic.org/pulmonaryhypertension-rst/7/22/20247/22/20244444.
