core curriculum h fp ef, hfref, and infiltrativerestrictive cardiomyopathies
TRANSCRIPT
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Is this Cor Pulmonale or is this HF-PEF?
Akshay S. Desai, MD, MPH
Director, Heart Failure Disease Management Program
Cardiovascular Division
Brigham and Women’s Hospital
Assistant Professor of Medicine
Harvard Medical School
Boston, MA
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Objectives
• Explore Heterogeneity of Clinical Phenotypes amongst patients with heart failure and normal ejection fraction
• Discuss role of right heart catheterization in facilitating workup of pulmonary hypertension
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3
Spironolactone
Placebo
TOPCAT
CHARM-Preserved PEP-CHF
I-PRESERVE
HR = 0.89 (0.77 – 1.04), p=0.138
Outcome Trials in HF-PEF
Yusuf, et al. Lancet 2003; Cleland, et al. Eur Heart J 2006; Massie, et al. N Engl J Med 2008; Pitt, et al. N Engl J Med 2014
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4
Limited Guideline-Recommended Therapy
Slide Courtesy Marc Pfeffer, MD, PhD
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5
Restrictive CMP
Pericardial Disease
RV Failure
HF-PEF
HF Signs and Symptoms Normal LVEF
Hypertrophic CMP
Storage Disease
• Constrictive Pericarditis • Constrictive-Effusive Disease • Post-Pericardiotomy Syndrome
• Amyloidosis • Hemochromatosis • Endomyocardial Fibrosis • Radiation-Induced • Chemotherapy-induced • Idiopathic
• PAH/cor pulmonale • ARVC • Sarcoidosis • TR
• Fabry • LAMP2 • PRKAG2
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Patient #1
• 74 y/o nurse
• PMHx: HTN and DM
• Progressive DOE, orthopnea, PND
• Exam: BP 190/88, JVP↑, rales, edema
• ECG: AF
• EF 66%, RVSP 60
Images courtesy Rick Nishimura, MD
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Patient #2
• 65 yo man with emphysema, O2 dependent
• Progressive dyspnea, fatigue
• Exam: BP 90/60, JVP↑, no rales, ascites, edema
• EF 65%, RVSP 70
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Paulus WJ, et al. Eur Heart J 2007; 28:2539-50
HF signs and Symptoms
Near Normal EF
Abnormal Filling or Relaxation
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• 74 y/o nurse
• PMHx: HTN and DM
• Progressive DOE, orthopnea, PND
• Exam: BP 190/88, JVP↑, rales, edema
• ECG: AF
• EF 66%, RVSP 60
• 65 yo man with emphysema, O2 dep
• Progressive dyspnea, fatigue
• Exam: BP 90/60, JVP↑, no rales, ascites, edema
• EF 65%, RVSP 70
HF with PH and normal LVEF
HF-PEF Cor Pulmonale
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Cor Pulmonale
• Alteration in structure and function of right ventricle caused by a primary disorder of the respiratory system
• Typically associated with pulmonary hypertension
• Distinguish from RV dysfunction caused by a primary abnormality of the left side of the heart or congenital heart disease
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Etiologies of Cor Pulmonale
• Acute
– Pulmonary Thromboembolism
• Chronic
– COPD/Emphysema
– Interstitial Lung Disease
– Pulmonary Arterial Hypertension
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How do I Identify whether PH/RV failure is due to Left Heart Disease?
• Suggestive Features – Left Sided Valve Disease
– Structural LV abnormalities
• Evidence of Alternative cause of PH – Severe Lung Disease, Pulmonary Emboli
• If concern for PAH after noninvasive evaluation, right heart catheterization
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Suspected PAH (mPAP>25 mm Hg)
PCWP ≤ 15 PVR > 3 WU
PCWP > 15 PVR ≤ 3 WU
Pulmonary Arterial Hypertension
Group 2 Pulmonary Hypertension
(or mixed)
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Suspected PAH (mPAP>25 mm Hg)
PCWP ≤ 15 PVR > 3 WU
PCWP > 15 PVR ≤ 3 WU
Pulmonary Arterial Hypertension
Consider: HF-PEF
Restrictive CMP HCM
Constrictive Pericarditis
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Is this Cor Pulmonale or HF-PEF? • 50 yo woman with prior mantle XRT for Hodgkin’s
Lymphoma
• Known Radiation-induced lung disease
• Progressive edema, ascites, fatigue
• BP 90/60, elevated JVP, clear lungs
• EF 66%
Images courtesy Rick Nishimura, MD
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• RA 20, PA 50/20 (mean 30), PCWP 20 • CO 4.0 L/min • PVR = 2.5 Wood Units
Right Heart Catheterization
HF-PEF, right?
But, there is elevation and equalization of filling pressures in diastole…
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Waveforms
120
80
40
120 mm Hg
LV
RV
Expiration
Inspiration
0 mm Hg
LV Pressures Decrease, RV Pressures Increase Discordant Changes with Inspiration Ventricular Interdependence
Constrictive Pericarditis
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Predominant Right Heart Failure
Constrictive Pericarditis
Restrictive Cardiomyopathy
Cor Pulmonale Primary RV Failure/TR
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54 yo normotensive man with AF and Heart Failure
Not Just HF-PEF
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Specific Diagnostic ‘Clues’ • Constrictive Pericarditis
– Prior XRT, Prior cardiac surgery
• Amyloidosis
– Periorbital bruising/petechiae, low QRS voltage on ECG, peripheral neuropathy, orthostasis, carpal tunnel syndrome
• Fabry Disease
– Proteinuria, Acroparesthesias, angiokeratomas, neuropathy
• Endomyocardial Fibrosis/Loeffler’s Endocarditis
– Eosinophilia, ventricular thrombi, tropical origin
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When to look closer at HF-PEF
• Atypical history
– Younger age, absence of HTN/DM
• Atypical ECHO features
– Normal Wall thickness/LA size
– Massive Hypertrophy
– Normal Diastolic function
• ‘Flash’ Pulmonary Edema
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When to Pursue Right Heart Catheterization in HF-PEF
• Hemodynamics Uncertain
• Unexplained pulmonary hypertension (suspected PAH)
• Constrictive/Restrictive Disease
• Infiltrative Disease Suspected (bx)
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A 70-year-old woman with a long-standing history of hypertension is your office for a second opinion regarding management of pulmonary hypertension. She was recently admitted to the hospital for an acute heart failure exacerbation. A TTE obtained during that admission demonstrated a normal LV ejection fraction (LVEF) with an LV posterior wall thickness of 1.2 cm. Her estimated PA systolic pressure was 70 mm Hg based on TR jet velocity. Right heart catheterization revealed the following: RA pressure: 7 mm Hg PA pressure: 70/30 mm Hg with a mean PA pressure of 45 mm Hg PCWP: 35 mm Hg Cardiac output: 5 L/min Cardiac index: 2.5 L/min/m2 While she is uncertain for her medical treatment in the hospital following her heart catheterization, she feels better. Her current medications include lisinopril 40 mg a day, furosemide 40 mg bid, and amlodipine 5 mg a day. She states that she has read about the risks associated with pulmonary hypertension on the Internet, as well as potential therapies, and wants your opinion on most appropriate treatment.
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Question
Which of the following is the most appropriate recommendation?
A. Add sildenafil 20 mg tid.
B. Add IV epoprostenol at 3 ng/kg/min.
C. Add bosentan at 62.5 mg bid.
D. Add carvedilol 3.125 mg bid.
E. Continue her present therapy.
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