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Page 1: 5/98MedSlides.com1 Pulmonary (Arterial) Hypertension

5/98 MedSlides.com 1

Pulmonary (Arterial)

Hypertension

www.medslides.com

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Definitions

• Pulmonary arterial hypertension– acute (pulmonary embolism, ARDS)– chronic (clinically more common)

• Pulmonary venous hypertension– pulmonary venous (LA pressure) > 12 mmHg– Hallmarks: pulmonary congestion and edema– Invariably, PVH elicits PAH

• Cor Pulmonale– pulm HTN with RV hypertrophy and dilatation

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Survival as a Function of Mean Pulmonary Arterial Pressure

0

20

40

60

80

100

120

0 1 2 3 4 5

Years

% S

urv

ival <25

25-30

30-45

45+

Mean PAPressure

Prog Resp Res 1975, 9:10

mm Hg

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Pulmonary Circulation

• Before birth– pulmonary and systemic pressure are

equal in the order of 70/40 (mean 50)

• After birth– ductus arteriousus closes, lung expands,

PA pressure falls to 1/2 of systemic level– PA pressure gradually decrease over

weeks to reach adult level

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Pulmonary Circulation

• Pulmonary circulation is “protected” from being hypertensive– large capacity– great distensibility– low resistance– modest amounts of smooth muscle in the

small arteries and arterioles

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Pulmonary Hemodynamics

PA pressure (mmHg) 20/12, 15 38/14, 25

LA pressure (mmHg) 5.0 5.0

Cardiac Output 6 L/min 6 L/min

PVR (R Unit) 1.7 3.3

PVR = -------------------- ; CGS units = R units x 80PA - LA

Cardiac Output

Sea AltitudeLevel 15,000 ft

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Pulmonary Arterial Hypertensionpathophysiology

• Decrease in total cross-sectional area– lung resection

• Narrowing of the lumen of the small muscular arteries and arterioles resistance vessels (P = Q x R)– chronic hypoxia with vasoconstriction

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Primary or Idiopathic Pulmonary Hypertension

• Plexogenic

• Thromboembolic (small emboli)

• Veno-occlusive disease

• Pulmonary capillary hemangiomatosis

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Secondary Pulmonary Hypertension

• Hypoxic– chronic lung disease, sleep apnea

• Pulmonary Venous Hypertension– mitral stenosis, left ventricular failure

• Congenital Heart Disease– Eisenmanger’s, PDA, ASD, VSD

• Vascular Obstruction– thromboembolism, systemic vasculitis

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Evaluation

• Right heart catheterization– “gold standard” for the diagnosis of PH

• Chest Radiography– findings depends on the duration and etiology– enlargement of the pulmonary trunk and hilar

vessels, pruning of the peripheral tree, RV – hyperinflation, flattening of the diaphragm

• Electrocardiogram– RV enlargement

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Evaluation

• Ultrasound– assess RV wall thickness and function– estimate pulmonary pressure from TR jet

• Lung Scan– rule out pulmonary thromboembolic disease

• Radionuclide Studies– evaluate RV function

• Lung Biopsy– identify interstial or collagen vascular disease

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Evaluation

• Arterial blood gas• Pulmonary function test• Collagen vascular serology• Lower extremity venogram or compressive

ultrasonography• CBC and peripheral blood smear• Stool and urine for parasites• Polysomnogram

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Left-heartfailure

Congenitalheart disease

Increasedblood flow

Reversedshunt

Passive Pulm HTN

Abnormalvasoconstrictive

response

Hypoxemia

ErythrocytosisPulmonary Hypertension

RVH / Cor PulmonaleRV Failure

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Pulmonary Venous Hypertension

• Elevated LV diastolic pressure– LV systolic or diastolic failure– constrictive pericarditis

• Left atrial hypertension– mitral valve disease– Cor triatriatum– left atrial myxoma or thrombus

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Left ventricular failure

• LV failure is the most common cause of pulmonary hypertension

• LV failure is the most common cause of right ventricular failure - attributable to failure of the muscle in the shared ventricular septum

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Congenital Heart Disease

• Pulmonary hypertension is associated with many types of CHD, and often a major determinant of it’s clinical course

• Large left-to-right shunt ( blood flow)– atrial septal defect, ventricular septal

defect, patent ductus arteriosus• Right-to-left shunt ( vasomotor tone)

– hypoxia with in red cell mass

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LV-RV Inter-dependance Normal Heart

RV

LV ED Volume

ED

Pre

ssu

re RVLV

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LV-RV Inter-dependance Acute RV Distention

Dilated RV

Compressed“D” shaped LV

LVED VolumeL

VE

D P

ress

ure

Normal RV

Distended RV

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Thromboembolic Disease

• A form of occlusive pulm vascular disease– thromboembolic (U.S. and Europe)– schistosomiasis (Egypt)– filariasis (Asia)

• Categorization by arterial segment occlusion – small (attributable to organized thrombi)– intermediate (clots from upper legs and thighs)– large (chronic proximal thromboembolism)

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Respiratory Disease and Disorder

Chronic Lung Disease

PulmonaryHypertension

RV Dilatationand Hypertrophy

Hypoxia

Polycythemia

Restricted Pulmonary

Vascular Bed

HypercapniaAcidosis

RV Failure

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Respiratory Disease and Disorder

• Intrinsic disease of lung and/or airways• Interstitial fibrosis• Chronic obstructive lung disease• Conglomerate fibrosis, emphysema, and

chronic bronchitis• Collagen vascular disease • Alveolar hypoventilation (sleep apnea,

abnormal respiratory control, chest bellows)

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Miscellaneous Causes

• residence at high altitude

• hemoglobinopathies

• intravenous drug abuse

• alveolar proteinosis

• Takayasu’s disease

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References

• Hurst’s The Heart, 8th Edition,1994Schlant RC, Alexander RW, O’Rourke RA, Roberts R, Sonnenblick EH

• Hypoxia and pulmonary hypertension in chronic bronchitis.Bishop JM. Prog Resp Res 1975, 9:10