Cardiomyopathies Flashcards
Definition cardiomyopathy
Heart muscle disease of unknown cause in which the heart muscle is structurally/ functionally abnormal leading to mechanical/ electrical dysfunction resulting in dilated, hypertrophic pathophysiology of heart
Classification of cardiomyopathies
1- dilated cardiomyopathy
2- hypertrophic cardiomyopathy
3- restrictive cardiomyopathy
4- Arrythmogenic right ventricular cardiomyopathy
5- unclassified cardiomyopathy
Categories of cardiomyopathy
Primary involving only the heart
Secondary involving other organs
What is the moges classification of cardiomyopathies
M- morpho functional phenotype
O- involved organs
G - Genetic
E- etiology
S- functional status
4 major types of primary cardiomyopathy
HCM
DCM
ARVD
RCM
cardiomyopathies with genetics as primary cause
HCM
ARVC
LVNC
cardiomyopathies with Mixed etiologies
DCM
RCM
Steps to diagnose cardiomyopathies
History
Examination
Echocardiographic evaluation (2Decho, Doppler echo)
MRI and CT scan In some cases
Cases when to further investigate wirh mri and Ct scan
Local fat
Iron
Amyloid infiltration
Scar
Fibrosis
Focal hypertrophy
Iv apical aneurysm
Fv structure / function
What characterizes DCM
Dilatation and impaired contraction of one or both ventricles
Pathophysiology of DCM
Dilated ventricles with impaired contraction
Increased ventricular mass with normal or reduced wall thickness
Can lead to valve incompetence
This modifiable risk factor is involved in many cases of dilated cardiomyopathy
Alcohol
25% cases of DCM are inherited as autosomal dominant trait, true or false
True
Common causes of DCM i
Idiopathic
Infections
Drugs
Alcohol
Inflammatory dx
Pregnancy
Poor nutrition
Hereditary / genetic
Toxic substances
Autoimmune disorders
Main presentation of DCM
Heart failure
Reduced ejection fraction
Arrhythmia
Thromboembolism
Sudden death
Sporadic chest pain
Main differential of DCM
Ventricular dysfunction due to CAD
Most useful investigations test in DCM
Cardiac MRI
Echocardiogram
Pharmacotherapy in DCM
RAAS blockers ( ACEIs, ARBs, ARNIs)
Bblockers
Mineralocorticoid receptor antagonists
SGLT2 inhibitors
Loop diuretics
Vasodilators
Devices and surgeries done in DCM
ICD - implantable cardioverter defribillator
CRT- cardiac resynchronization therapy
VAD- ventricular assist device
Heart transplant
What is peripartum cardiomyopathy PPCM
Pregnancy associated cardiomyopathy occurring in late pregnancy or early puerperium which presents with heart failure towards end of pregnancy or within 5 months after delivery
Ejection fraction in peripartum cardiomyopathy
Less than 45 %
Risk factors in peripartum cardiomyopathy
More than 30 yo
African descent
Pregnancy with multiple fetuses
Preeclampsia, eclampsia , postpartum hypertension history
Long term oral tocolytic more than 4 weeks with beta agonists
Maternal cocaine abuse
Selenium deficiency
What is hypertrophic cardiomyopathy
Increase in LV wall thickness not explained solely by abnormal loading conditions
Form of inheritance in hypertrophic cardiomyopathy
Autosomal dominant trait with variable expression
This cardiomyopathy is the leading cause of sudden death in pre adolescent and adolescent children
Hypertrophic cardiomyopathy
Hallmark of hypertrophic cardiomyopathy
Inappropriate , asymmetric myocardial hypertrophy
Abscess of obvious inciting stimulus
Main area of the heart affected by hypertrophic cardiomyopathy
Interventricular septum with LVOT obstruction
Thickness of wall in hypertrophic cardiomyopathy
More than 15mm
2 types of hypertrophic cardiomyopathy
Obstructive (mid systolic obstruction of flow)
Non obstructive
Symptoms of HCM
Asymptomatic
Sudden cardiac death
Dyspnea
Syncope
Presyncope
Chest pain
Palpitations
Fatigue
Signs of HCM
Double carotid arterial pulse
Double apical impulse
S4 possibly heard
Systolic ejection crescendo decrescendo murmur due to LVOT obstruction
Holosystolic murmur at apex and axilla due to mitral regurgitation
Diastolic decrescendo murmur of AR
Complication of HCM
Heart failure
Ventricular arrhythmia
Infective endocarditis of mitral valve
Atrial fibrillation with mural thrombus formation
Sudden death
Investigation of HCM
2D echo
Chest MRI in selected cases
CxR
Cardiac catheterization
Electrophysiologic studies
Genetic testing
Management of HCM
BBlockers
NDHP CCBs (verapamil, diltiazem )
Disopyramide
Amiodarone
Antitussives
Diuretic
Management to avoid in HCM
Inotropic drugs
Nitrates
Vasodilators
Digitalis
Surgical options in HCM
Surgical myectomy
Catheter alcohol septal ablation
Mitral valve replacement
ICD placement
Heart transplant
What is Restrictive cardiomyopathy
Non compliant ventricular walls that resist diastolic filling
Part of the heart most commonly affected with RCM
Left ventricle
RCM types
Obliterative due to fibrosis of endocardium and subendocardium
Non obliterative due to MI by abnormal substance such as amyloid
Possible etiology of RCM
Genetic or acquired
Systemic disease (amyloidosis, hemochromatosis)
Sarcoidosis
Fabry disease
Loiffler syndrome
Genetic abnormalities (fabry dx, gaucher dx, hemochromatosis )
Connective tissue dx (amyloidosis , systemic sclerosis, Endocardial fibroelastosis )
Carcinoid tumors
Endomyocardial fibrosis
Hypereosinophilic syndrome
Sarcoidosis
Différent possible consequence of RCM
Endocardial thickening or myocardial/papillary infiltration with or without myocyte death
Hypertrophy and fibrosis in one or both ventricles
Mitral or tricuspid malfunction -> regurgitation
SA and AV nodes malfunction -> SA and AV block
Main hemodynamic consequence -> Diastolic dysfunction with rigid non compliant ventricle and impaired diastolic filling -> pulmonary venous hypertension
Mural thrombi possible -> systemic emboli
Investigations done in RCM
Echocardiogram
MRI
Cardiac CT scan
ECG
Chest X ray
L and R heart catheterization
Cardiac biopsy
Lab test
Biopsy
What do you see on echo of RCM
Both atria Dilated
LVEF may be normal
Elevated filling pressure on Doppler
Myocardial hypertrophy
Findings on echo of amyloidosis with RCM
Bright echo pattern from myocardium
Treatment of RCM
Diuretics
Bblockers
Anticoagulation
Avoid nitrates
People at risk of Endomyocardial fibrosis
Children and adolescents in tropics
Age at risk of Endomyocardial fibrosis
10 and 30 yo
Clinical manifestation of Endomyocardial fibrosis
Right or left heart failure
