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

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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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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Limited Guideline-Recommended Therapy

Slide Courtesy Marc Pfeffer, MD, PhD

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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

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

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

Paulus WJ, et al. Eur Heart J 2007; 28:2539-50

HF signs and Symptoms

Near Normal EF

Abnormal Filling or Relaxation

• 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

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

Etiologies of Cor Pulmonale

• Acute

– Pulmonary Thromboembolism

• Chronic

– COPD/Emphysema

– Interstitial Lung Disease

– Pulmonary Arterial Hypertension

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

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)

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

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

• 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…

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

Predominant Right Heart Failure

Constrictive Pericarditis

Restrictive Cardiomyopathy

Cor Pulmonale Primary RV Failure/TR

54 yo normotensive man with AF and Heart Failure

Not Just HF-PEF

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

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

When to Pursue Right Heart Catheterization in HF-PEF

• Hemodynamics Uncertain

• Unexplained pulmonary hypertension (suspected PAH)

• Constrictive/Restrictive Disease

• Infiltrative Disease Suspected (bx)

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.

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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