cvs misc-2-csbrp
TRANSCRIPT
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Hypertensive Heart DiseaseHypertensive Heart DiseaseCSBR.Prasad, MD.,CSBR.Prasad, MD.,
May-2015-CSBRP 1
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• Hypertrophy• Causes for LVH• Causes for RVH• Complications of systemic HTN• Name some causes for cardimegaly?
May-2015-CSBRP 2
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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
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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
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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
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May-2015-CSBRP 6
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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
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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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May-2015-CSBRP13
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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
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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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May-2015-CSBRP 22
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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
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Takotsubo cardiomyopathy
May-2015-CSBRP 25
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Takotsubo cardiomyopathy
May-2015-CSBRP 26
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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).
May-2015-CSBRP
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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
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Dilated cardiomyopathy
May-2015-CSBRP
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What is “cor bovinum” ?
• Bovine heart [>1000gm]• Seen in Syphilis, • AR due to other causes• Dilated cardiamyopathy
May-2015-CSBRP 31
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Name some causes for globular heart?
• Wet Beri beri• AR due to any cause [Tertiary Syphilis]• Dilated cardimyopathy• Chaga’s disease
May-2015-CSBRP 32
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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
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Arrhythmogenic right ventricular cardiomyopathy (ARVC)
May-2015-CSBRP 34
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Name some causes for fatty heart?[Fatty change of the heart]
• Anemia• ARVH• Diphtheria
May-2015-CSBRP 35
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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
May-2015-CSBRP
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Hypertrophic Cardiomyopathy
May-2015-CSBRP 37
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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
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Restrictive Cardiomyopathy
Several other restrictive conditions:
Endomyocardial fibrosisLoeffler endomyocarditisEndocardial fibroelastosis
May-2015-CSBRP 39
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May-2015-CSBRP40
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E N D
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May-2015-CSBRP 42
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Takotsubo cardiomyopathy
May-2015-CSBRP 43