Pulmonary Hypertension: Management Update
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Transcript of Pulmonary Hypertension: Management Update
Pulmonary Hypertension: Management Update
Robert Vassallo, MDDivision of Pulmonary and Critical Care MedicineMayo Clinic, Rochester, MN.
KUWAIT 2014
Disclosures
• I have nothing to disclose with respect to this presentation.
Key objectives of this presentation
• Review WHO updated classification of pulmonary hypertension.
• Review diagnostic approach and caveats with testing for pulmonary hypertension.
• Outline a clinical rationale for the management of pulmonary hypertension.
• Discuss situations when expert opinion and referral to specialized centers may be necessary.
Pulmonary Hypertension
Concept…
…progressive increase in the blood pressure in the pulmonary vascular bed
Consequence…
…right heart failure and death
Criteria for Pulmonary Hypertension
• Mean Pulmonary Artery Pressure MPAP > 25 mmHg at rest with pulmonary capillary wedge pressure ≤15 mm Hg
4th World Symposium on Pulmonary Hypertension, Dana Point, CA 2008
Potential sites to induce Pulmonary Hypertension
Post-capillaryPre-capillary
Updated Clinical Classification of Pulmonary Hypertension
1. Pulmonary arterial hypertension (PAH)1. idiopathic (“primary pulmonary hypertension”)2. secondary to systemic disorders
2. Pulmonary hypertension due to left heart disease (pulmonary venous hypertension)
3. Pulmonary hypertension associated with respiratory disease and/or hypoxia
1. COPD, Interstitial lung disease, OSA
4. Chronic thromboembolic/embolic pulmonary hypertension
5. Pulmonary hypertension from unclear mechanisms
Journal of the American College of CardiologyVol. 62, No. 25, Suppl D, 2013
0 5 10 15 20
Cardiac Output [L/min]
90
60
30
0
Mean PA pressure
REST EXCERCISE
Mean pulmonary artery pressure and cardiac output relationship
Normal
Mild to moderate
Severe
Signs and symptoms of Pulmonary Hypertension
• Symptoms and signs are usually not specific; delay in diagnosis very common.
• Early – dyspnea with activity.
• Late symptoms:• syncope• chest pain / chest pressure• leg edema (signs of right heart failure)• abnormal findings on chest X ray or ECG
Diagnostic studies: Chest radiography
Radiographic findings are subtle and often missed
ECG abnormalities
• Right heart strain (RVH and RA enlargement)
• Non-specific
• Right axis deviation
• ST depression and T wave inversion V1-V3
Screening for pulmonary hypertension
• There is no perfect screening tool.
• Transthoracic echocardiography
• Goals: • 1. determine right ventricular size
and systolic function• 2. estimate right ventricular systolic
pressure
Diagnostic testing: Trans-thoracic echocardiography
RA LA
LV
RV
Echo transducerEcho transducerEcho transducerEcho transducer
PASP = (4 x [TRV]2) + RAPPASP = (4 x [TRV]2) + RAPPASP = (4 x [TRV]2) + RAPPASP = (4 x [TRV]2) + RAP
CP900234-1
Echocardiography uses Doppler ultrasound to estimate the pulmonary artery systolic pressure
Pulmonary hypertension diagnosis via right heart catheterization
From Mayo Clinic Right Heart Catheterization Training Manual – Cardiology Rotation
Clinical approach to suspect pulmonary hypertension
• If the echocardiogram suggests the presence of pulmonary hypertension
• 1. perform careful evaluation for secondary causes
• 2. consider whether you should proceed to right heart catheterization
Once you have identified pulmonary hypertension, look for causes
1. Pulmonary arterial hypertension (PAH)1. idiopathic (“primary pulmonary hypertension”)2. secondary to systemic disorders
2. Pulmonary hypertension due to left heart disease (pulmonary venous hypertension)
3. Pulmonary hypertension associated with respiratory disease and/or hypoxia
1. COPD, Interstitial lung disease, OSA
4. Chronic thromboembolic/embolic pulmonary hypertension
5. Pulmonary hypertension from unclear mechanisms
Journal of the American College of CardiologyVol. 62, No. 25, Suppl D, 2013
Once pulmonary hypertension is detected, look for causes!
European Heart Journal (2009) 30, 2493–2537
European Heart Journal (2009) 30, 2493–2537
History and Exam LFT, CBC Autoimmune screen Overnight oximetry Echocardiography Chest CT V/Q scan PFT HIV serology
Other testing as suggested by H&P
PAH (Group 1) Medication Options
Mild Moderate Severe
25 <MPAP< 35 35 <MPAP< 50 MPAP> 50
� Ca++ Channel Blocker X
� Endothelin antagonist X X
� PDE-5 inhibitor X X
� Prostanoids X X � Inhaled iloprost X X� SQ treprostinil X X � IV epoprostenol X X
Group 1: “Primary” pulmonary arterial hypertension
• Remember PAH (Group 1) and PH (Groups 2-5) are different entities
• PAH is a progressive disease with a 50% survival at 2.8 years
• Can lead to sudden death
• Often affects young adults: mean age 45 years
• Rarely familial
Group 1: Secondary pulmonary artery hypertension
• Collagen vascular disease• scleroderma • Systemic lupus erythematosus
• Liver disease
• Congenital heart
• Drugs/toxins
• HIV infection
Therapy
• Treat any underlying disease.
• Treat cardiac disease (left sided).
• Treat lung disease and hypoxemia.
• Treat obstructive sleep apnea or any associated sleep disorder.
• Treat thromboembolic disease.
• Remember: Groups 2-5 are managed differently and vasodilator therapy is NOT recommended.
Intravenous Vasodilators
Pulmonary Arterial Respiratory Disease Pulmonary Venous
Increased ShuntPulmonary Edema
Adjunctive therapy
• Salt restriction.
• Diuretics as needed.
• Oxygen at rest or with activity.
• Flu and pneumococcal vaccination.
• Digoxin ? Role if any.
• Anticoagulation ?
Group 1 PAH therapy
Drug of choice?
• Right heart catheterization vasodilator “challenge”
� 20% acute drop in MPAP and PVR? ...begin with ca++ channel blocker
Right Heart Catheterization and Vasodilator Trial
Improved
Acute Responder Non-Responder
Calcium Channel Blocker
NYHA II-IV
Vasodilator therapy ERA or PDE5-inh or prostacyclin analog
Not Improved
Transplant
Consider combination therapy
Involve a specialist with expertise
Calcium Channel Blockers
• PAH therapy is based on severity of disease as determined by right heart catheterization and responsiveness to vasoactivity testing.
• Although not FDA approved for the treatment of PAH, CCBs commonly used are nifedipine, diltiazem, and amlodipine.
• All agents are titrated to clinical effect.
Objective measures to monitor therapy…every 3-6 months
• 6 minute walk (goal > 300 meters)
• NT-ProBNP (Brain Naturetic Peptide)
• Sequential echocardiography
• Other studies depending on context
…other therapeutic options
• If chronic pulmonary emboli…� pulmonary thromboendarterectomy
surgery
• If all meds fail…� atrial septostomy� organ transplantation
• heart-double lung• double lung
Riociguat (a stimulator of soluble guanylate cyclase) significantly improved exercise capacity and pulmonary vascular resistance in patients with chronic thromboembolic pulmonary hypertension.
N Engl J Med 2013;369:319-29.
Final “take home” messages
• New onset exertional dyspnea…think of pulmonary hypertension.
• Screen by Transthoracic Doppler Echo but make sure you tell the cardiologist that you are thinking of PH!
• Definitive diagnosis by right heart catheterization.
• Several pharmacologic options now available: selection a function of PAH severity, expertise, cost assessment, and other factors.
• Pregnancy may cause significant challenges.
• PH patients are increasingly older with potential multiple ongoing diagnoses.
Thank you so much for the invitation.