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6/15/2017

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PULMONARY HYPERTENSION EVALUATION JUNE 14TH, 2017

32ND ANNUAL FAMILY MEDICINE UPDATE

HISHAM QUTOB, MD (PULMONARY / CRITICAL CARE MEDICINE)

FINANCIAL DISCLOSURE

• I do not have any relevant financial disclosures or relationships to any part of

this presentation

KEY OBJECTIVES

• Define Pulmonary Hypertension

• Classification of Pulmonary Hypertension

• Symptoms and Exam findings

• Workup of Pulmonary Hypertension

• Discuss role of Right Heart Cath

• Treatment of Pulmonary Hypertension

• Cases

• Prognosis Calculator

DEFINITION OF PULMONARY HYPERTENSION

• Fifth World Symposium on Pulmonary Hypertension update in 2013

• Pulmonary Hypertension definition:

• #1,Resting mean pulmonary artery pressure > 25 mmHg

• #2,Pulmonary artery occlusion pressure / LVEDP less than 15-16 mmHg

• Pulmonary vascular resistance > 3 wood units (not a part of the definition)

• Borderline pulmonary hypertension, resting mean PAP 21-24 therapeutic importance

remains unknown

• Exercise-induced pulmonary hypertension has not been reinstated

Hoeper et al, definition and diagnosis of pulmonary hypertension,J Am Coll Cardiol 2013;62

PULMONARY HYPERTENSION W.H.O. GROUPS

• Group 1, pulmonary arterial hypertension…

• Group 2, heart associated…

• Group 3, lung associated…

• Group 4, thromboembolic associated…

• Group 5, miscellaneous

Fifth world symposium diagnostic classification of pulmonary hypertension, 2009

FIFTH WORLD SYMPOSIUM DIAGNOSTIC CLASSIFICATION OF PULMONARY HYPERTENSION, 2013

• Group 1: Pulmonary arterial hypertension:

• 1.1, Idiopathic PAH

• 1.2, Hereditary PAH

• 1.3, Drug and toxin induced

• 1.4, Connective tissue disease associated, HIV infection, Portal pulmonary hypertension,

Congenital heart disease, Schistocytosis

• Group 1’: Pulmonary venoocclusive disease and/or pulmonary capillary

hemangiomatosis

• Group 1”: Persistent pulmonary hypertension of the newborn

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• Group 2: Pulmonary arterial hypertension: “heart disease associated”

• Chronic systolic cardiomyopathy

• Chronic diastolic cardiomyopathy

• Chronic valvular disease


• Group 3: Pulmonary arterial hypertension: “lung disease associated”

• Chronic obstructive pulmonary disease

• Interstitial lung disease

• Mixed Restrictive and Obstructive lung disease

• Sleep-disordered breathing

• Chronic exposure to high altitude

• Developmental abnormalities

• Alveolar hypoventilation


• Group 4: Pulmonary arterial hypertension: “thromboembolic disease

associated”

• Chronic thromboembolic pulmonary hypertension


• Group 5: Pulmonary arterial hypertension: “miscellaneous basket”

• Hematologic ( myeloproliferative, splenectomy)

• Sarcoidosis

• Pulmonary Langerhans cell histiocytosis

• Lymphangioleiomyomatosis

• Neurofibromatosis

• Chronic vasculitis

• Glycogen-storage disease

• Gaucher disease

• Thyroid disease

• Obstructive ( tumor, fibrosing mediastinitis)

• End stage renal disease on dialysis

PATHOPHYSIOLOGY

Murphy and Nadel’s text book of respiratory medicine Intimal hyperplasia

Medial hypertrophy

Adventitial fibrosis

PATHOPHYSIOLOGY

Medial hypertrophy of pulmonary artery

Medial hypertrophy with cellular intimal proliferation

Medial hypertrophy with cellular fibrosis

Progressive general vascular dilatation and occlusion by fibrosis

Dilatation lesions and vein like branches, plexiform lesions

Necrotizing of the pulmonary artery

Heath D, Edwards JE: the pathology of hypertensive pulmonary vascular disease, 1958

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PATHOPHYSIOLOGY

• There has been a significant change of understanding from vasoconstriction to

that of growth and proliferation. Progressive and essentially irreversible

• Research ongoing, bone morphogenic protein receptor 2 (BMPR2) is

associated to be a failure of apoptosis, familial causes of PAH

MISSED DIAGNOSIS

• Must be on the lookout for this diagnosis

• Not one disease (ie, World Health Organization classification)

• Found within other co-morbid disease process

• Different severity of disease is found

• No perfect screening test

SYMPTOMS

• Dyspnea is found and 95% of patients

• Begins usually a exertional dyspnea, insidious onset

• Exertional chest pain is also noted frequently

• Hemoptysis can be seen with idiopathic pulmonary arterial hypertension

• Syncope and fatigue

SIGNS / EXAM FINDINGS

• Late sign of tachycardia (HR ~90-105), relative hypotension

• Rarely a right ventricular heave is noted

• Left sternal border tricuspid regurgitation murmur

• Late finding, stasis dermatitis and evidence of right heart failure with peripheral

edema and abdominal distention

• EKG can show RBBB, right axis deviation, right ventricular hypertrophy

• Lungs sounds are usually normal

NOW YOUR CONCERNED YOU HAVE A PATIENT WITH PULMONARY HYPERTENSION

• Categorize prior to testing

• Does this patient have heart failure? (JVD, crackles, lower extremity edema, orthopnea, PND)

• Does this is patient have COPD? (prolonged expiratory phase…)

• Does this is patient have pulmonary fibrosis? (crackles on exam, dyspnea on exertion)

• Does this patient have connective tissue disease? (rash, joint swelling, reflux, sclerodactyly)

• Is this patient Young and may have pulmonary arterial hypertension?

• Is this patient a drug user (methamphetamine, cocaine, HIV) ? (track marks)

• Does this patient have cirrhosis ? (telangiectasias, spider nevi, palmar erythema)

• Does this patient have OSA? (Mallampati, neck obesity, snoring, poor sleep..)

• Does this patient have a history of pulmonary embolism and deep vein thrombosis ?

• Does this patient have sickle cell disease ?

WORKUP

• 90% of patients with idiopathic pulmonary arterial hypertension have an

abnormal chest x-ray

Rich S at L, primary pulmonary hypertension, Ann internal medicine 1987

Murray and Nadel textbook of respiratory medicine

Engorged pulmonary artery

Cardiomegaly

Loss of retrosternal airspace

Chronic interstitial disease, heart failure…

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WORKUP

• Pulmonary function test (Key to diagnosis)

• Reduction in FEV1 = COPD

• Diffusion capacity for carbon monoxide (DLCO)

• Reduction in FVC and DLCO = Interstitial lung disease

WORKUP

• High-resolution CT scan:

• Concern for emphysema, interstitial lung disease, chronic thromboembolic pulmonary

hypertension, patient with possible pulmonary arterial hypertension

L.J.

•

R.H.

WORKUP

• Echocardiogram :

• Evaluate for systolic and diastolic dysfunction, left ventricular hypertrophy

• Order with a bubble study

• Evaluate right ventricular systolic function

• Must include heart valve evaluation ( i.e. not limited echocardiogram)

• Estimation of pulmonary arterial pressure ( based on the peak velocity jet of tricuspid

regurgitation )

• Use echocardiogram mainly to evaluate for heart disease and heart valve disease,

Echocardiogram can miss pulmonary hypertension

WORKUP

• Echocardiogram :

• Accuracy of echocardiogram

in patients with advanced lung disease:

Selim M et al, echocardiographic assessment of pulmonary hypertensionin patients with advanced lung disease,

Am J Resp Crit Med 2003, 167:735-740

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WORKUP

• Concern for chronic thromboemolic disease

• Argument for VQ scan: Screening method of choice for CTEPH. Negative VQ scan

excludes CTEPH due to high sensitivity ( if the radiologist knows how to read them)

• Positive VQ scan can be seen in pulmonary venoocclusive disease

• PVOD: positive VQ scan, mediastinal lymphadenopathy, pleural effusions, intralobular septal

thickening, diffuse groundglass ( look for a history of previous chemotherapy, history of stem

cell transplant, autoimmune connective tissue diseases )

Tunariu N et al, A ventilation-perfusion scintigraphy is more sensitive than multidetector CTPA in detecting chronic

thromboembolic pulmonary disease as a treatable cause of pulmonary hypertension, J Nucl Med 2007

WORKUP

• Concern for chronic thromboembolic disease

• Argument for CT pulmonary angiogram:

• Radiologist comfortable reading CT pulmonary angiogram (acute PE, bands, webs, intimal

irregularities, loss of perfusion distally)

• Evaluate for pulmonary artery endarterectomy

WORKUP

• Lab test:

• CBC, CMP, TSH

• HIV test

• Anti-nuclear Antibody

• Anticentromere antibody ( limited scleroderma )

• Anti-double-stranded DNA, anti-SM ( systemic lupus )

• Anti-RNP ( mixed connective tissue disease )

• Anti-phospholipid antibody, lupus anticoagulated, anti-cardiolipin antibodies (CTEPH)

WORKUP

• Polysomnogram

• Prefer in lab sleep study with pulse oximetry

• Once diagnosed, ensure adequate compliance

DO I HAVE TO DO A RIGHT HEART CATHETER

• MUST be done to confirm pulmonary arterial hypertension

• MUST have a experienced operator to achieve adequate useful numbers

• Attempt to make patient euvolemic prior to right heart catheter

• Fick method is preferred over thermodilution

• Need to obtain superior vena cava, pulmonary artery, systemic arterial blood

oxygen saturation to calculate pulmonary vascular resistance

DO I HAVE TO DO A RIGHT HEART CATHETER

• Right heart catheter should be performed if 1 or more of the following

conditions exist:

• Evaluation for lung transplantation

• Worsening clinical status or gas exchange

• Need to understand prognosis

• Severe pulmonary hypertension is suspected and further therapy is being considered

• Suspicion of left heart dysfunction

Seeger W at our, pulmonary hypertension and chronic lung disease, J Am Coll Cardiol, 2013

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WHAT DOES THE RHC TELL YOU

• Cardiac index = mean 3.4 ( 2.8–4.2 )

• Mean wedge pressure = 9 ( 6–15 )

• Pulmonary artery systolic and diastolic = 24/10 ( 15–28 / 5–16 )

• Pulmonary artery mean pressure = 16 ( 10–22 )

• Trans-pulmonary gradient = ( less than 12 )

• Pulmonary vascular resistance = > 3 Wood units is elevated

WHAT DOES THE RHC TELL YOU

• Pulmonary vasoreactivity testing

• Calcium channel blocker responsiveness testing

• Recommended only for idiopathic pulmonary arterial hypertension

• However finding positive calcium channel responders are rare and not a perfect

correlation to clinical improvement

Sitbon O et al, long-term response to calcium channel blockers and idiopathic pulmonary arterial hypertension. Circulation 2005

OUT OF PROPORTION PULMONARY HYPERTENSION

• Typical pulmonary patient:

• 74-year-old male with:

** past medical history of coronary artery disease status post PCI / CABG, hypertension,

hyperlipidemia, COPD, obesity, obstructive sleep apnea, peripheral arterial disease, stage III

chronic kidney disease.

** social history: 40 pack year history of smoking, drinks 5-6 beers per weekend

• Referral: Evaluate for pulmonary hypertension

• Thoughts: Congestive heart failure, emphysema, obstructive sleep apnea, kidney disease,

cirrhosis…


• Typical pulmonary patient:

• 74-year-old male with:

** past medical history of coronary artery disease status post PCI / CABG, hypertension, hyperlipidemia, COPD, obesity, obstructive

sleep apnea, peripheral arterial disease, stage IV chronic kidney disease.

** social history: 40 pack year history of smoking, drinks 5-6 beers per weekend

• Referral: Evaluate for pulmonary hypertension

• Thoughts: Congestive heart failure, emphysema, obstructive sleep apnea, kidney disease, cirrhosis…

• Right heart catheter:


• Use of transpulmonary gradient is helpful in this case

• Concomitant heart failure and/or mitral valve disease ( mitral stenosis )

• Presence of pulmonary hypertension and mitral stenosis is an indication for

mitral valve repair or mitral valve replacement

• Main concern of decreasing pulmonary vascular resistance is increasing venous

return to the dysfunctional left ventricle

Bonow RO et al, ACC/AHA guidelines for management of patients with fibrillary heart disease, J Am Coll Cardiol. 1998


• Suspicion for diastolic heart failure

• All testing normal, exceptional exertional dyspnea

• Administration of 500 mL of saline over 5 minutes during right heart catheter

may help identify diastolic heart failure

• Conducting right heart catheter during treadmill exercise testing

Robbins IM et al, Association of the metabolic syndrome with pulmonary venous hypertension, chest 2009, 136:31-6

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TREATMENT OF OUT OF PROPORTION PULMONARY HYPERTENSION

• Maximized management of primary condition

• Treat valvular heart disease with surgery

• Heart failure management

• Diuresis

• Use of sildenafil or endothelial antagonist should be avoided

Advances in PH journal, volume 5, No 1, spring 2006

TREATMENT OF OUT OF PROPORTION PULMONARY HYPERTENSION


TREATMENT OF PULMONARY HYPERTENSION

• Make sure you do not have left-sided heart failure as a cause

• Advanced treatment for pulmonary arterial hypertension must have a right heart catheter

prior to beginning therapy

• Must conduct a 6 minute walk test prior to use objective data in evaluating the clinical

response to advanced treatment

• Insurance hurdles in obtaining prior approvals for these therapies

• Pregnancy concerns in these patients (bosentan and riocuquat are absolutely contraindicated)

• Patients with World Health Organization functional class II, III, IV symptoms despite PH

treatment shall be referred to tertiary care center with PH specialization


Gen. considerations:


• Pulmonary arterial hypertension (portion of group 1)

• Idiopathic and heritable PAH should undergo vaso-reactivity testing during RHC

• Treatment world health organization functional class II, III, IV

• 3 treatment arms:

• 1.Prostacyclin analogues (Epoprostenol, Treprostinil, Iloprost, Selexipag)

• 2. Endothelin antagonist (Ambrisentan, Bosentan, Macitentan)

• 3. Phosphodiesterase inhibitors (Sildenafil, Tasalafil)

• Other: Guanylate cyclase stimulant (Riocuquat )

• Combination therapy is becoming standard

SILDENAFIL/MONOTHERAPY DOES NOT CUT IT

AMBITION study, Galie N et al, Initial use of ambrisentan plus tadalafil in pulmonary arterial hypertension, NEJM, 2015

AMBITION study


• Pulmonary arterial hypertension ( group 2 )

• In general, advance pulmonary hypertension therapy is not to be used in Group 2

• Out of proportion pulmonary hypertension can be a consideration for advanced

pulmonary hypertension medications

• Worse mortality has been seen with the use of epoprostenol

• Treatment can potentiate a CHF exacerbation

ACCF/AHA 2009 expert consensus, J Am Coll Cardiology. 2009

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• Pulmonary arterial hypertension ( group 3 & 5 )

• No significant use of advanced therapy in these groups of pulmonary hypertension

patient’s

• Oxygen supplementation for COPD as hypoxia can cause pulmonary vasoconstriction and

elevation of pulmonary vascular resistance

TREATMENT OF PULMONARY HYPERTENSION • Pulmonary arterial hypertension ( group 4 )

• 1st consider surgical therapy pulmonary artery endarterectomy in patients with central clot

• 2nd consider Medical therapy: Riociguat

PATENT-1 trial CHEST-1 trial


• Lung transplantation for pulmonary arterial hypertension

• Reserved for patients that have failed medical management

• Early referral is appropriate especially in the young, pulmonary arterial hypertension

WHEN SHALL YOU SEND TO PULMONARY

• Group 1, Group 2 (out of proportion PH), Group 4, Group 5

• World Health Organization functional class II through IV

• Progressive symptoms

• Suspected pulmonary veno-occlusive disease

MONITORING THESE PATIENTS

• Oxygen saturation

• 6 Minute Walk Test

• B-Type Naturetic Peptide (? Echocardiogram)

• CPAP compliance

• Pregnancy tests

• Liver enzymes (hepatotoxcity with Pulmonary HTN medications)

CASES:

• Real patients here in Chattanooga:

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O.M.

• 61 year old female

• 1st seen in 2014, hx ox obesity, diabetes, asthma, lupus, diastolic dysfunction,

heart failure, and chronic hypoxia.

• Referral: shortness of breath

• Symptoms: insidious onset of dyspnea, oxygen did not help, uses a walker, has

significant worsening of symptoms with ambulation

• Exam: Distant breath sounds, lower extremity edema with stasis dermatitis

O.M.

• Testing:

• PFT: FEV1 86%, FVC 84%, DLCO 36%

• Left Pleural effusion with atelectasis (small), no fibrosis / no emphysema

• Echocardiogram: Normal LV / EF, No diastolic dysfunction, No right to left shunt, trace TR

• B-Type Naturetic Peptide: 400+

• ABG: 7.40 / 39 / 75 on an FiO2 of 28%

• Question: “ Where is the hypoxia? “

“ Where is the CHF / Diastolic Dysfunction “

“ Where is the restrictive lung disease “

“ Where is the pulmonary hypertension on echo? “

O.M. RIGHT HEART CATH

• Pulmonary Artery Pressure: 92/34 (mean 57)

• Wedge: ~ 15

• Right Ventricular Pressure: 90/5

• Pulmonary Vascular Resistance 10 wood units

Right Heart Cath diagnostic criteria :

Mean Pulmonary Artery Pressure ≥ 25 mmHg

Pulmonary Vascular Resistance > 3 Wood units

Mean Pulmonary Capillary wedge pressure < 15 mmHg

L.M.

• 63 year old female

• Past history of factor VII deficiency, palpitations and sinus tachycardia,

exertional dyspnea

• Comes in for second opinion as cardiology has no etiology of supra ventricular

tachycardia / palpitations / exertional dyspnea

L.M.

• Symptoms:

• Dyspnea when walking the dogs in the yard

• Significantly worse with going up a hill

• Starts about 5-7 minutes after walking

• Testing:

• Echocardiogram: all normal

• CT Chest: all normal

• VQ scan: Normal ventilation / perfusion

• PFT:

• Rheumatologic labs: all normal

L.M.

• Right Heart Cath at Cleveland Clinic:

REST EXERCISE

Mean PA pressure : 18 mmHg Mean PA pressure : 80 mmHg

Wedge pressure: 14 Wedge pressure: 35

Oxygen saturation: 95 % Oxygen saturation: 88 %

Diagnosis: Exercise-induced pulmonary venous hypertension due to diastolic dysfunction

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L.J.

• 52 year old female with multiple sclerosis and decreased ambulation

• Few pulmonary symptoms, came to ED with malaise and shortness of breath

• Vitals: HR 105, BP 143/85, Respiratory rate: 35

• ABG: 7.45 / 22 / 60

• LE Doppler: negative bilaterally

• Sent to the unit for acute pulmonary embolism

L.J.

•

L.J.

• ABG: 7.45 / 22 / 60 7.18 / 145 / 37

R.H.

• 30 year old male

• Diagnosed with OSA, has exertional dyspnea.

• Patient reports exertional dyspnea, started after he cut grandfather’s farm grass /

hay from May through August

• Patient has a workup for Dyspnea on Exertion.

• SpO2 in clinic 92%

• Echo, normal with no Right to Left shunt

• Overnight pulse ox showed continued hypoxia

• CXR clear

• Spirometry clear

R.H.

• 30 year old male, diagnosed with OSA and nocturnal hypoxia is told he

needs to lose weight and continue to use CPAP

• ? Oxygen saturation at 92-94%

• Lead to more testing in clinic:

• DLCO is 39%

• 6 MWT on Room Air with oxygen saturation at 76%, required 6 L to maintain

• CT scan:

R.H.

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R.H.

R.H.

J.M.

• 85 year old male presents in 2013 for Dyspnea on Exertion

• PmHx of CAD, CABG, CHF. “crackles on exam”

• PFTs 2014, FEV1 99%, FVC 82%, DLCO 53%

J.M.

• DLCO: 53% 33%

J.M.

• Echocardiogram at a DLCO level of 33%:

• EF 55%

• Mild Diastolic Dysfunction

• Normal RV and Function

• Moderate Tricuspid Regurgitation with a TR gradient at 36

• Estimated Pulmonary Artery Systolic Pressure: 41 mmHg

• Positive Right to Left shunt “bubble study”

MORTALITY PROGNOSIS RISK CALCULATION

• REVEAL Registry risk score calculator in patients newly diagnosed with

pulmonary arterial hypertension

• Poor prognostic factors: Familial PTAH, portopulmonary hypertension, male greater than

60, functional class III & IV, pericardial effusion, DLCO less than 32, pulmonary vascular

resistance greater than 30 Wood units, evidence of right heart failure, BNP greater than

180.

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