Heart Failure & Pulmonary Hypertension:

What you need to knowIsh Kumar Singla , MD

National Heart Failure Awareness Week

February 9-15, 2014 

• Heart Failure• Pulmonary Hypertension

What you need to know

A Word of Anatomy

• Right side of heart gets blood from body.

• Right Ventricle sends blood to Lungs via pulmonary arteries for oxygenation.

• Left side of heart gets oxygenated blood from lungs.

• Left Ventricle pumps oxygen rich blood

to body.

What is Heart Failure

• Not able to pump enough blood to body

“ Heart failure is NOT heart attack”

• Muscle weakness or stiffness

Heart Failure is Very common

• Affects nearly 5 million

• 1% of Adults 50-60

• 10% of Adults over 80

• 8 million by 2030( 46% increase)

• 400,000 -700,000 new cases each year

• 5%-10% of all hospital admissions

Types of Heart Failure

• Systolic HF ( Muscle weakness )- Not able to pump enough blood

• Diastolic HF(Muscle thickening and stiffness)

- Not able to pump blood in normal way

• Right Sided Heart failure

Risk factors for Heart Failure

• Coronary Artery Disease (Heart attack )• Heart valve damage(Leaky or narrow valves)• Cardiomyopathy• Congenital heart disease• Hypertension • Obesity• Aging

Symptoms of Heart Failure

• Shortness of Breath• Fatigue• Leg edema• Weight gain• Cough• Not able to lie flat due to difficulty breathing

NYHA Classification of symptoms

• Class I: No Symptoms. No limitation in physical activity. Ordinary physical activity does not cause dyspnea or fatigue.

• Class II: Mild symptoms and Slight limitation in physical activity. Ordinary physical activity causes undue dyspnea or fatigue.

• Class III: Marked limitation in physical activity due to symptoms. Less than ordinary physical activity causes undue dyspnea or fatigue.

• Class IV: Severe limitations. dyspnea and/or fatigue may be present at rest.

Systolic Heart Failure

• Etiology- Coronary artery Disease ( CAD)- Myocardial Infarction (MI)- Cardiomyopathy ( Viral, Familial etc)- Valvular heart disease

Diagnosis of Heart Failure

• Echocardiogram ( Heart Ultrasound)• EKG• Chest X ray• Labs• Heart catheterization• Stress test

Diagnosis of Heart Failure

• Echocardiogram- Heart Ultrasound- Outpatient office procedure

- To measure EF(Ejection Fraction)- Normal EF is 55%-60%- Evaluate Heart valves- Evaluate Pressures in heart

Diagnosis of Heart Failure

• Left heart catheterization- Outpatient procedure ,done at hospital- Radial (wrist) or Femoral (groin) approach- Dye test to look at the heart arteries

Treatment of Systolic Heart Failure

• Diet Restriction• Exercise regimen• Lifestyle changes • Medications• Treatment of specific etiology• Surgical Rx

Dietary Modifications

• Salt Restriction- 2000 mg sodium/ day- 1 teaspoon of salt=2000 milligrams (mg)

• Fluid restriction- 2000 ml/day

How to Restrict Salt intake

How to Restrict Salt intake

• Stop using salt shaker• Salt substitutes• Eliminate salty foods • Read the labels• Eliminate canned foods• Fresh fruits and vegetables

“ Don’t eat anything that tastes good”

Fluid restriction

• 2000 ml /day ~ 64 oz of fluid a day• 1 cup ~ 250 ml of water

Fluid Retention

• Weigh daily• Call your doctor for weight gain more than 3 lbs

Lifestyle changes

• Quit smoking• Limit alcohol intake• Exercise regimen• Weight loss

Exercise

• Start aerobic exercise

• Avoid lifting weights more than 15-20lbs.

Medications

• Diuretics ( Fluid pills)- Furosemide, Torsemide, Bumetanide

• Beta blockers- Carvedilol, Metoprolol succinate

• Ace- Inhibtors- Lisinopril, Captopril, Enalapril

Medications

• Angiotensin Receptor Blockers• Aldosterone Inhibtors• Hydralazine and Nitrates• Digitalis

Cardiac Arrest

• EF < 35%-40%

- ICD( Implantable cardiac defibrillator)- BiV- pacemaker- Combined BiV- ICD

Diastolic Heart Failure

• Etiology- Hypertension- Obesity- Diabetes- Lack of exercise- Sleep Apnea

Diastolic Heart Failure

• Tests- Echocardiogram- Heart Catheterization- EKG- Sleep study- Labs

Treatment

• Diet Restriction• Exercise regimen• Loose weight• Quit smoking• Treatment of sleep apnea• Treatment of primary disease• ?? Medications

Medications

• Diuretics ( Fluid pills)- Furosemide, Torsemide, Bumetanide

• ?? Beta blockers- Carvedilol

• ?? Aldosterone antagonist- Spironolcatone

Surgical Treatment of Heart Failure

• Heart Transplant - Needed for more than 4000 patients

- 2500 heart donors yearly

- Scarcity of donors

- “It is not a cure, it is an exchange of one set of problems for the other” (Dr Bourge)

• Left Ventricle Assist Device( Mechanical Heart Pump)

Right Sided Heart Failure

• Pulmonary Hypertension• Cardiomyopathy• Congenital heart disease• Heart attack

Pulmonary Artery Hypertension

Pulmonary Hypertension

• Definition• Epidemiology• Classification• Symptoms• Etiology• Treatment

Pulmonary Hypertension

• Normal Mean PA pressures are 12-16.

• Definition: Mean Pulmonary Artery (PA)

pressure > 25 mm Hg at rest

• Chronic condition with no cure.

• Without treatment only 34% survival at 5 years.

Severity

• Degree of disease ( Mean PAP mmHg)

• Mild : 25 – 40

• Moderate: 41 – 55

• Severe: >55

Pulmonary Hypertension

VC RA RV PA PVPC

LA LV Ao

2.Pulmonary Venous HTN

HTN

Left sided heart failure

1.Arterial Mitral Valve Disease

4. Embolic3. Hypoxic Lung Diseases

Pulmonary Hypertension Lesion

Aortic Valve Disease

Epidemiology

• Pulmonary Venous HTN - very common• PAH - rare disease• Hypoxic - 1-2%• Embolic - 5%• Miscellaneous

PH Classification

• Based on similarities in their pathophysiology and responses to treatment.

• Classify patients accurately to ensure appropriate treatment.

PH Classification

• Group I (Arterial ) Idiopathic, Familial, HIV, Scleroderma, drugs, toxins, Congenital heart disease, Sickle cell disease

• Group II (Venous ) Pulmonary venous hypertension due to left sided

heart disease.

• Group III (Hypoxia) COPD, Interstitial lung disease, Sleep apnea

• Group IV (Embolic) Chronic thromboembolic disease

• Group V (Misc) Sarcoidosis, Compression of pulmonary artery by tumor

Pulmonary Artery Hypertension

• Idiopathic - Incidence 2-3 /million per year- Female: Male 3:1

• Common with Rheumatological diseases- Scleroderma 50%- Rheumatoid Arthritis 20%- Lupus 10%

• Congenital heart disease : 30% - 50%

Pulmonary Artery Hypertension

• Sickle cell disease 25%• Liver disease 5%• HIV 0.5%• Dexfenfluramine (fen-phen) Risk 20 times if

taken for more than 3 months• Cocaine or amphetamine use risk by 3 fold

Symptoms

• Gradual onset of Shortness of breath

- Often results in delayed diagnosis ~ 18 months

• Fatigue

• Chest pain

• Syncope/presyncope

• Peripheral edema or ascites

• Raynaud’s in about 10%

Diagnosis

• Echocardiography is excellent screening tool• No treatment based on echocardiography alone• Right heart catheterization for accurate diagnosis

and appropriate treatment• Labs• CT scan

NORMAL REVERSIBLE DISEASE IRREVERSIBLE DISEASE

Pathogenesis of Pulmonary ArterialHypertension

WHO functional classification PAH

• Class I: No limitation in physical activity. Ordinary physical activity does not cause undue dyspnea or fatigue, chest pain or near syncope.

• Class II: Slight limitation in physical activity. Ordinary physical activity causes undue dyspnea or fatigue, chest pain or near syncope.

• Class III: Marked limitation in physical activity. Less than ordinary physical activity causes undue dyspnea or fatigue, chest pain or near syncope.

• Class IV: Inability to perform any physical activity without symptoms. Signs of right heart failure. Dyspnea and/or fatigue may be present at rest. H/o Syncope.

Clinical Sequeale

• Increased pressure load on Right Ventricle.

• Right Ventricle failure.

• Once RV failure is present prognosis poor.

Goals of Therapy• Alleviate symptoms, improve exercise capacity and

quality of life

• Delay time to clinical worsening

• Reduce morbidity and mortality

Treatment of Pulmonary Hypertension

• Lifestyle modifications• Oxygen• Treatment of specific etiology• Medications• Transplantation

Dietary Modifications

• Salt Restriction- 2000 mg sodium/ day- 1 teaspoon of salt=2000 milligrams (mg)

• Fluid restriction- 2000 ml/day

Lifestyle Modifications

• Sodium restriction

• Abstinence from smoking

• Avoid high altitude

• <4,000 feet above sea level

• Avoid physical exertion in setting of pre-

syncope sx

• Avoid pregnancy

PAH: Therapy

• Supplemental O2

• Anticoagulation

• Diuretics

• Vasodilators Therapy

Targets for Therapy in PAH

Endothelin cells

PAH: Therapy

• Calcium-channel blockers-pills

• Endothelial receptor antagonists (ERAs)- pills

• Phosphodiesterase-5 Inhibitors (PDE5-I)

-pills

• Prostanoids-Inhalers, intravenous, subcutaneous

Epoprostenol (Flolan)

Barst RJ et al. NEJM, 334: 296-301, 1996.

• Intravenous Prostacyclin

• First drug, became available in 1990s.

• Delivered via continuous infusion

• PPH study

• Improvement in survival

SQ or IV Treprostinil (Remodulin)

• Stable Prostacyclin, t ½ 4 hours.

• Continuous subcutaneous infusion

• Approved 2002

• Significant pain at infusion site limits use

• NYHA class II,III,IV

• Group I PAH• Stable at room temperature.

Simonneau et al. Am. J. Respir. Crit. Care Med., March 2002, 800-804

Bosentan (Tracleer)• Endothelin Receptor Antagonist

• First oral Drug,Approved 2002

• Dose 125mg BID

• Risk of Liver Toxicity

• Group I PAH (Idiopathic, CTD and congenital)

Rubin L et al. NEJM 2002

Ambrisentan (Letairis)

• Selective Type A ERA.• Oral dose 10 mg once daily • Much less risk of liver toxicity• Monthly monitoring not required• Approved 2005• Group I PAH (Idiopathic and CTD )

Badesch et al. J Am Coll Cardiol. 2005;46(3):529.

Macitentan (Opsumit)

• Non-selective ERA• Dose 3mg or 10mg po daily• Approved 2013• CBC and LFTs monitoring• Group I PAH (Idiopathic,congenital,CTD)• Improvement in mortality and morbidity.

Pulido et al. N Engl J Med Aug 2013; 369:809-818

Sildenafil (Revatio)

• PDE-5 inhibtor• FDA approved dose 20 mg po tid• Approved 2005• Hypotension, Sudden hearing loss• First dose in monitored setting• Contraindicated with nitrates • Group I PAH(Idiopathic,CTD,Congenital)

Ghofrani et al. N Engl J Med. 2005;353(20):2148.

Tadalafil ( Adcirca )

• PDE-5 inhibtor• FDA approved dose 40 mg po daily• Approved 2009• Hypotension, Headaches, Myalgias• Contraindicated with nitrates • Group I PAH(Idiopathic and CTD)

Brundage et al. Circulation. 2009;119(22):2894.

Riociguat (Adempas )

• Oral sGC stimulant.• First in Class• Dose 1.5mg to 2.5 po TID• Approved 2013• Group IV PAH• Group I PAH

D Armini et al.N Engl J Med. 2013 Jul;369(4):319-29.

• Synthetic PGI 2

• Selective pulmonary vasodilator

• Inhaled form 6-9 times while patient awake

• Approved 2002

• Exerts preferential vasodilatation in well- ventilated regions.

• No known contraindications.

Olschewski et al, NEJM 2002, 347:322-9

Inhaled Iloprost ( Ventavis )

Inhaled Treprostinil (Tyvaso)

• Synthetic Prostacyclin• Inhaler,4 doses used 4 hrs apart while awake.• Approved 2011• Group I PAH (Idiopathic, CTD, HIV,Fen-phen)• NYHA III pt• Background therapy with oral drugs• SE: dizziness,cough,headache,flushing

McLaughlin et al . J Am Coll Cardiol. 2010; 55(18):1915-1922

Effi

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Treatment Summary

Conclusion

Progressive disease

Significant morbidity and mortality

Right heart failure marks disease progression

Therapies with proven benefit in improving hemodynamics, functional class and exercise tolerance

Continuous IV Flolan is reserved for advanced (class IV) disease where there is a proven survival benefit

Acknowledgements

• Dr Robert C. Bourge (Vice Chair ,Dept of Medicine ,UAB, Birmingham)

• UAB Heart Failure and Transplant Division• Springfield Clinic

References

• www.heart.org• www.phaassociation.org• www.abouthf.org• www.escardio.org• http://www.nutrition411.com• www.hfsa.org• http://lungs.wikispaces.com• http://www.acponline.org• http://www.cardiachealth.org• https://encrypted-tbn0.gstatic.com/images • http://www.lsuagcenter.com/• https://www.unitypoint.org

Thank You