0930 - Lee Pulm HTN
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Transcript of 0930 - Lee Pulm HTN
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PULMONARY HYPERTENSION
Augustine Lee, MD
Mayo Clinic Florida
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Definition
Hemodynamically defined
mPAP >25 mmHg at rest (or >30 with exercise)
PCWP <15 mmHg
(PVR > 3 wood units)
Diagnosis requires hemodynamic
assessment
Echocardiography alone is not sufficient
Right heart or PA catheterization required
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LUNGS
RA RV PA PC PV LA LV
PVR CO
PA Pressure PV Pressure
V = I R
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WHO Classification
1. PAH Idiopathic
Familial (6%): BMPR2, ALK1, Endoglin (HHT), KCNK3?
Drug and toxin-induced
Fenfluramine
Associated conditions (APAH):
Connective tissue disease: Scleroderma, SLE
HIV
Portal hypertension
CHD: Eisenmenger’s, Systemic-pulmonary shunt
Schistosomiasis
Chronic hemolytic anemia: Sickle cell anemia
1’. PVOD, Pulmonary capillary hemangiomatosis
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WHO Classification
2. Left heart disease (“venous”)
Systolic, diastolic, valvular
3. Lung disease and/or hypoxemia
COPD, ILD, Sleep disordered breathing, Hypoventilation syndromes, High altitude
4. Chronic thromboembolic pulmonary hypertension
(CTEPH)
5. Other
Sarcoidosis, PLCH, Neoplastic, Metabolic disorders, Myeloproliferative disorders, Fibrosing mediastinitis
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Breakdown (REVEAL)
McGoon, Eur Resp Rev 2012
ALL PH APAH
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Idiopathic
No risk factor
No family history or genetic mutation
Leading cause ~40%
Women > men
Mean age 52
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Associated conditions
CTD ~15% Systemic sclerosis (~50%) have worst prognosis
HIV ~6% Declining
Schistosomiasis Possibly most prevalent worldwide
Sickle cell ~20% Poor prognosis
COPD, IPF/ILD ~ 40% Worsens prognosis
Venothromboembolism Even after single “normotensive” acute PE ~27%
Might be less if treated with thrombolytics ~11%
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Europe: Galie, EHJ 2009
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Europe: Galie, EHJ 2009
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US: ACCF/AHA 2009
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ACCF/AHA 2009
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www.Pah-info.com
Right Heart Catheterization
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REVEAL Registry
“Registry to Evaluate Early and Long Term
PAH Disease Management”
United States
2006-2009
3515 subjects
Multiple publications
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Prognosis (REVEAL) Benza, Chest 2012.
McGoon, Eur Resp Rev 2012
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IPF and PAH Nadrous, Chest 2005
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COPD and PAH Oswald-Mammosser, Chest 1995
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Quick bad prognostic findings
Age > 45
WHO functinal class III or IV
Failure to drop a functional class after therapy
Echocardiography Pericardial effusion
Large right atrial size and RAP >20
Septal shift during diastole
RV dysfunction
Increased BNP
Prolonged QRS
Hypocapnia
Comorbid conditions: COPD, IPF, diabetes, sickle cell, HIV, etc.
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Causes of death (IPAH)
73%: RV failure
27% Other
Medication adverse effect
Surgery
Pneumonia
Stroke
D’Alonzo. AIM 1991. (NBHLBI)
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McGoon, Eur Resp Rev 2012
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Treatment: General Measures
Manage primary disorder E.g. Mitral stenosis, COPD, stop toxins
Manage aggravating conditions E.g. Sleep apnea, renal failure
Oxygen: Goal >90%
Anticoagulation Extrapolated survival data from IPAH
Management of RHF Diuretics, Digoxin
Exercise, rehabilitation
Move quicker & earlier to advanced therapy EARLY Study: Galie, Lancet 2008
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Special case: Vasodilator responsive
~ 13% will “Respond”
Absolute mPAP falls <40mmHg
Decrease in mPAP by at least 10mmHg
Vasodilator
Inhaled NO, Epoprostenol, CCB, Adenosine
Therapy (Optional)
CCB (diltiazem, nifedipine, amlodipine)
Sustained response ~54%
Requires regular monitoring
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ACCF/AHA 2009
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Pathophysiology: “Vasculopathy”
Smooth muscle cell & endothelial cell proliferation (plexiform lesions)
Growth inhibitors, mitogens
Vasoconstriction
Imbalance of vasodilators/constrictors
Thrombosis
Imbalance in local coagulation factors
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www.Pah-info.com
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Humbert, NEJM 2004
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Advanced Therapy
Prostanoid (“Gold standard”)
Epoprostenol (IV)
Treprostinil (IV, SQ, inhaled, [PO])
Iloprost (inhaled)
Endothelin receptor antagonists (ERA)
Bosentan, [macitentan], ambrisentan, sitaxsetan
PDE5 inhibitors
Sildenafil, tadalafil, vardenafil
Guanylate cyclase stimulant (sGC)
[Riociguat]
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Adverse effects
Prostanoids
Jaw pain, flushing, headache, nausea, diarrhea,
arthralgias, central line infection, skin reaction
ERA
Edema
Liver toxicity: sitaxsentan > bosentan >
ambrisentan
Teratogenicity
(Pregnancy is contraindicated)
ERA contraindicated. Prostanoid preferred.
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Combination therapy (REVEAL) McGoon, Eur Resp Rev 2012
BREATHE-2 TRIUMPH
FREEDOM-C
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Benza, Chest 2012
McGoon, Eur Resp Rev 2012
So are patients doing better? (REVEAL)
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Super-Advanced
Lung transplantation
Bilateral lung +/- heart
Median survival ~3 years
Pulmonary thromboendarterectomy
Can be curative
Expert centers only
Atrial septostomy
High procedural mortality (15-20%)
Worsening hypoxia from shunt
Palliative measure with goal to improve cardiac output in severe RHF
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Summary
PAH is bad news, by itself or when complicating other conditions
Use the WHO classification to guide diagnostic considerations, & specific therapeutics
Diagnose it early, and treat it early.
RHC is necessary for diagnosis.
Lots of therapeutic options now available & more coming
But prostanoids remain gold standard in severe PAH
Consider referral to advanced PH/Transplant center early