Hypertensive Heart DiseaseHypertensive Heart DiseaseCSBR.Prasad, MD.,CSBR.Prasad, MD.,

May-2015-CSBRP 1

• Hypertrophy• Causes for LVH• Causes for RVH• Complications of systemic HTN• Name some causes for cardimegaly?

May-2015-CSBRP 2

Systemic (Left-Sided) Hypertensive Heart Disease

• Hypertrophy of the heart is an adaptive response to the pressure overload of chronic hypertension

• In the course of time, compensatory changes may be ultimately maladaptive and can lead to myocardial dysfunction, cardiac dilation, CHF or sudden death

May-2015-CSBRP 3

Systemic Hypertensive Heart Disease

The minimal pathologic criteria for the diagnosis of systemic HHD are:

1. Left ventricular hypertrophy and 2. Pathologic evidence of hypertension in

other organs (e.g., kidney)

Even mild hypertension (levels only slightly above 140/90 mm Hg)—if sufficiently prolonged—induces left

ventricular hypertrophy

May-2015-CSBRP 4

Systemic Hypertensive Heart Disease

Hypertension induces:• Left ventricular hypertrophy

– Initially concentric hypertrophy – Left ventricular wall thickening >2cm– Increase in weight of the heart >500gm

• Left atrial enlargement (due to diastolic filling)Microscopically:

– Increase in transverse diameter of the cardiac muscle fiber

– Interstitial fibrosis

May-2015-CSBRP 5

May-2015-CSBRP 6

Systemic Hypertensive Heart Disease

• Asymptomatic, if compensated– ECG / Echo evidence of LVH

• Many patients present with – Atrial fibrillation– CCF

Complications:– IHD– Stroke– Renal damage– Progressive cardiac failure

• Effective control of HTN reduces the chances of complications

May-2015-CSBRP 7

Pulmonary (Right-Sided) Hypertensive HeartDisease (Cor Pulmonale)

• Chronic cor pulmonale is characterized by:– RVH– Right sided heart failure

• Acute cor pulmonale can follow massive pulmonary embolism

MORPHOLOGY:Acute CP - marked dilation of the RVChronic CP

RVH =/>1cm (Normal thickness 0.3-0.5cm)Thickening of moderator bandRVH may encroach on to left ventricle and cause fibrous thickening

of Tricuspid valve and TR

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Acute Cor Pulmonale

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Pulmonary embolism (PE)

• The most common ECG finding in the PE is sinus tachycardia

• However, the "S1Q3T3" pattern of acute cor pulmonale is classic

• This is termed the McGinn-White sign

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Pulmonary (Right-Sided) Hypertensive HeartDisease (Cor Pulmonale)

It should also be remembered that PHT most commonly occurs as a complication

of left-sided heart disease

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Hypertensive heart disease

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Cardiomyopathies

• They are primary diseases of the myocardium • due to genetic causes• Associated with inappropriate ventricular

hypertrophy or dilatation • Causing mechanical and/or electrical

dysfunction • often leading to cardiovascular death or

progressive heart failure

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Cardiomyopathies

Presentations:• CCH• Arrhythmias Types:• Primary cardiomyopathies (genetic or

acquired diseases of myocardium) • Secondary cardiomyopathies (myocardial

involvement as a component of a systemic or multiorgan disorder)

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Exclusion

Ventricular dysfunction due to:• Ischemia• Valvular Abnormalities• Hypertension should not be denoted as cardiomyopathies

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ClassificationClassificationCan be classified according to a variety of

criteria:1.Underlying genetic basis2.Arrhythmia-inducing channelopathies3.Those producing anatomic abnormalities

– Three pathologic patterns:• Dilated cardiomyopathy (including ARVH) [90%]• Hypertrophic cardiomyopathy• Restrictive cardiomyopathy [Rarest]

May-2015-CSBRP 19

Can you name some channelopathies?

• Cystic fibrosis• Familial periodic paralysis• Long QT syndrome• Myesthenia gravis• Myotonia congenita

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Three major morphologic patterns of

cardiomyopathy

Note: Only left heart is shown in the diagram

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Dilated Cardiomyopathy

Characterized by:• Dilatation• Hypertrophy• Contractile dysfunction

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DCM - PathogenesisDCM - Pathogenesis• Genetic Influences

– 30-40% of cases– Mutations in >20 genes [AD > XR > Mitochondrial]

• Myocarditis– Coxsackie B

• Alcohol and other toxins– Acetaldehyde, Cobalt, Doxorubicin

• Childbirth – Peripartum cardiomyopathy

• Iron overload - Hemochromatosis

• Supraphysiologic stress - Excess Thyroxin, catecholamines

May-2015-CSBRP24

Takotsubo cardiomyopathy

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Takotsubo cardiomyopathy

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Schematic of a myocyte, showing key proteins mutated in dilated cardiomyopathy (red labels), hypertrophic cardiomyopathy (blue labels), or both (green labels).

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Causes and consequences of dilated and hypertrophic cardiomyopathy

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DCM - MORPHOLOGY

• Heavy (2-3x normal) and flabby heart• Mural thrombi

– Thromboembolism• Valvular regurgitationHistologic abnormalities in DCM are nonspecific

– Hypertrophy – Nucleomegaly, irregular nuclei– Endocardial fibrosis

May-2015-CSBRP29

Dilated cardiomyopathy

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30

What is “cor bovinum” ?

• Bovine heart [>1000gm]• Seen in Syphilis, • AR due to other causes• Dilated cardiamyopathy

May-2015-CSBRP 31

Name some causes for globular heart?

• Wet Beri beri• AR due to any cause [Tertiary Syphilis]• Dilated cardimyopathy• Chaga’s disease

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Arrhythmogenic right ventricular cardiomyopathy (ARVC)

• Inherited disease, AD • Right ventricular failure and rhythm

disturbances – Ventricular tachycardia or fibrillation

• Sudden deathMorphology:• Fatty infiltration and • Fibrosis

May-2015-CSBRP 33

Arrhythmogenic right ventricular cardiomyopathy (ARVC)

May-2015-CSBRP 34

Name some causes for fatty heart?[Fatty change of the heart]

• Anemia• ARVH• Diphtheria

May-2015-CSBRP 35

Hypertrophic Cardiomyopathy• Inherited disease, AD• Many genes are involved

– HCM is a disease caused by mutations in proteins of the sarcomere

– Most common is the gene encoding β-myosin heavy chain (β-MHC)

• Myocardial hypertrophy• Poorly compliant LV > defective diastolic filling• Intermittent ventricular out flow obstrction

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Hypertrophic Cardiomyopathy

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Restrictive Cardiomyopathy

• Characterized by a primary decrease in ventricular compliance, resulting in impaired ventricular filling during diastole

• Causes:– Radiation fibrosis– Amyloidosis– Sarcoidosis– Metastatic tumors– inborn errors of metabolism

May-2015-CSBRP 38

Restrictive Cardiomyopathy

Several other restrictive conditions:

Endomyocardial fibrosisLoeffler endomyocarditisEndocardial fibroelastosis

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E N D

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Takotsubo cardiomyopathy

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