1、Pulmonary Hypertension,Pulmonary Module Steven W. Harris MHS, PA-C,Pulmonary Hypertension,Defined as pressure within the pulmonary arterial system elevated above the normal range. greater than 25 mm Hg at rest or 30 mm Hg with exercise Pulmonary arterial pressure (PAP) usually 12-15 mmHg Left atrial
2、 pressure 6-10 mmHg,10/19/2018,2,Pulmonary hypertension develops when flow or resistance to flow across the pulmonary vascular bed increases,Four Categories of Pulmonary Hypertension Pulmonary hypertension from disorders of the respiratory system or hypoxemia. Pulmonary venous hypertension. Pulmonar
3、y hypertension from chronic thromboembolic disease. Pulmonary arterial hypertension. (PAH),10/19/2018,3,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 pulmonary disease (COPD) su
4、ccumb secondarily to right ventricular failure resulting from pulmonary hypertension.,10/19/2018,4,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 ventricular failure.,10/19/2018,5,10/19
5、/2018,6,10/19/2018,7,10/19/2018,8,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).,10/19/2018,9,Entities that cause increased pulmonary blood flow,Congen
6、ital Heart Disease Left to right shunt “Hyperkinetic“ pulmonary hypertension can be seen in patients with congenital heart disease who have extensive left-to-right cardiac shunts that produce a large pulmonary blood flow Increased cardiac output states severe anemias,10/19/2018,10,Entities that caus
7、e an increased resistance to flow,Pulmonary embolism In situ pulmonary embolism Pulmonary fibrosis Sarcoidosis, scleroderma, or extensive pulmonary resection Severe COPD Thoracic deformities Large tumor or infiltrate,10/19/2018,11,Entities that cause changes in arterioles,Hypoxia (altitude) COPD Hyp
8、oventilation (sleep apnea) Acidosis Drugs Pulmonary arterial hypertension (PAH),10/19/2018,12,Entities that cause venous pressure changes & vascular resistance,Left atrial hypertension = Mitral Stenosis Left ventricular failure Pulmonary venous thrombosis Mediastinitis,10/19/2018,13,Pulmonary HTN,El
9、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.,10/19/2018,14,10/19/2018,15,E
10、ntities that increase blood viscosity,Polycythemia vera Leukemias with high WBCs,10/19/2018,16,Entities that increase intra-thoracic pressure,COPD Mechanical Ventilation: especially with PEEP,10/19/2018,17,Symptoms,Mild to moderate pulmonary hypertension are often asymptomatic More severe pulmonary
11、hypertension usually complain of dyspnea on exertion secondary to exercise-induced decreases in cardiac output and increases in pulmonary arterial pressure.,10/19/2018,18,Symptoms,Easy fatigability, lethargy Exertional chest discomfort Syncope with exertion Cough Hemoptysis Hoarseness secondary to c
12、ompression of the left recurrent laryngeal nerve by a dilated pulmonary artery(rare),10/19/2018,19,Physical Exam,Increased intensity of the pulmonic component of the second heart sound (P2) Systolic ejection murmur from tricuspid regurgAdvanced Disease Diastolic murmur of pulmonic regurgitation in p
13、atients with severe pulmonary hypertension Evidence of right ventricular dilatation (left parasternal lift or heave) A pulse prominent a wave in jugular venous system,10/19/2018,20,PE (contd),Signs of right ventricular failure: jugular venous distension Right ventricular S4 (increased loudness of th
14、e S4 on inspiration) Hepatomegaly Ascites, and/or peripheral edema,10/19/2018,21,Caveats,Patients 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 ausc
15、ultation more difficult. The JVD may actually be above the jaw-line!,10/19/2018,22,Incidence of S & S,Loud P2 ( 80%) Right ventricular lift ( 80%) Dyspnea ( 75%) Murmur of tricuspid insufficiency (50-80%) Increased jugular venous pressure (50-80%) Right ventricular S4 (50-80%) Chest pain ( 50%) Fati
16、gue ( 50%),10/19/2018,23,S & S contd,Palpitations ( 50%) Syncope; dizziness ( 50%) Cough ( 50%) Raynauds phenomenon ( 10%) Hepatomegaly ( 50%) Pulmonic ejection click ( 50%),10/19/2018,24,S & S contd,Right ventricular S3 ( 50%) Murmur of pulmonic insufficiency ( 50%) Lower extremity edema ( 50%) Sup
17、erficial thrombophlebitis (5%),10/19/2018,25,Pulmonary Arterial Hypertension (PAH),Incurable disease characterized by medial hypertrophy, intimal fibrosis and in situ thrombi Very rare 1-2 cases per million Age 34-36 & again 50-59 Femalesmales (1.7:1),10/19/2018,26,Causes of PAH,Unknown (IPAH) HIV D
18、rugs Appetite suppressants(fenfluramine and dexfenfluramine) Amphetamines Cocaine,10/19/2018,27,10/19/2018,28,Diagnostic Tests,Chest x-ray, pulmonary function tests ALL should have a V/Q scan r/o PE EKG 2-D echocardiography with Doppler flow study disease Sleep study 6 minute walk Cardiac catheteriz
19、ation - right heart catheterization is necessary to measure pulmonary artery pressures and hemodynamics; rule out underlying cardiac,10/19/2018,29,EKG Findings,RVH (late finding) RAD RBBBRV strain (acute ie Pulm embolus) S wave in lead I Q wave/ inverted T in Lead III,10/19/2018,30,10/19/2018,33,10/
20、19/2018,34,Diagnostic Tests,Pulmonary angiography - should be done if segmental or larger defect on V/Q scan. Caution in pulmonary hypertension as can lead to hemodynamic collapse; use low osmolar agents, subselective angiograms. Lung biopsy not recommended,10/19/2018,35,10/19/2018,36,World Health O
21、rganization Classification of Functional Status of Patients with PH,Class I - patients with PH who experience no limitation of usual physical activity; ordinary physical activity does not cause increased dyspnea, fatigue, chest pain or presyncope Class II - patients with PH who have mild limitation
22、of physical activity. There is no discomfort at rest, but normal physical activity causes increased dyspnea, fatigue, chest pain or presyncope Class III - patients with PH who have marked limitation of physical activity. There is no discomfort at rest, but less than ordinary activity causes increase
23、d dyspnea, fatigue, chest pain, or presyncope Class IV - patients with PH who are unable to perform any physical activity at rest and who may have signs of right ventricular failure. Dyspnea and/or fatigue may be present at rest, and symptoms are increased by almost any physical activity,10/19/2018,
24、37,Tx of Pulmonary HTN,Treat the cause! Phosphodiesterase inhibitors (sildenafil) Revatio Prosanoids (prostacyclin analogues) Flolan, Iloprost Endothelin receptor antagonists Bosentan Vasodilators L-arginine? Coumadin,10/19/2018,38,Tx of Pulmonary HTN,Phosphodiesterase inhibitors (sildenafil) Revati
25、o Nitric oxide stimulation of endothelium increases cGMP resulting in vasorelaxation Phosphodiesterase breaks down cGMP,10/19/2018,39,Tx of Pulmonary HTN,Prosanoids (prostacyclin analogues) Flolan, Iloprost Prostacyclin is a potent vasodilator produced in the vascular endothelium . Also inhibits gro
26、wth of smooth muscle cells,10/19/2018,40,Tx of Pulmonary HTN,Endothelin receptor antagonists Bosentan Endothelan-1 is a potent vasoconstrictor, induces fibosis and leads to proliferation of smooth-muscle cells,10/19/2018,41,Tx of Pulm HTN,Treatment of heart failure (e.g., diuretics) Oxygen supplemen
27、tation is indicated for rest, exercise, or nocturnal hypoxemia Calcium channel blockers (nifedipine, diltiazem Only in selected patients that qualify via a vasoreactivity study. Digoxin (afterload reduction & increase cardiac output) Surgery of thrombolic emboli Low salt diet Cautious exercise Heart
28、-lung transplant,10/19/2018,42,Prognosis,Survival at 1 year 63% Survival at 2 years 45% Improves to 87% survival at 2 years with targeted therapy Mean age at diagnosis 34 years Mode of death: Most from Right Vent failure,10/19/2018,43,Easy Reading,http:/www.mayoclinic.org/pulmonaryhypertension-rst/,10/19/2018,44,