Cardiology 2 Flashcards
Diseases that involve myocardium directly and not result of conditions such as HTN, congenital, valvular, arterial or pericardial abn
2 forms
Cardiomyopathy
Primary - unknown cause
Secondary - known assoc with organ dysfunction
Genetic alteration in cardiomyopathy
Dystrophin complex
Basic structural and fxn unit of muscle
Sarcomere protein
Ryanodine receptors of SR
Disease
L type calcium ch
Malignant hyperthermia
Extrinsic protein on RBC membrane and myocyte
Spectrin
Most common cause of myocarditis
Coxsackie B
Viral replication in myocyte -> myocyte lysis -> delayed apoptosis
Glycogen storage disease with cardiac involvement
Pompe Disease
Bacteria notorious for causing myocarditis
Metachromatic granules
Diptheria
Most common cause of heart transplant
Diminished myocardial contractility, systolic pump function resulting in impaired ventricles leading to cardiac enlargement
Dilated cardiomyopathy
Most common cause of dilated cardiomyopathy
Idiopathic
- peripartum
- postmyocarditis
Alcoholic 2nd
Doxorubicin, cocaine
Infiltrative: amyloid
Most common assoc toxin with chronic dilated cardiomyopathy
Tx abstinence
Alcohol
This amount of consumption may develop DCM resembling idiopathic or familial DCM
> 90g/d
Alcohol consumption regularly during weekend or holiday
Acute cardiac rhythm or conduction disturbance mc Supraventricular Tachyarhythmia assoc with heavy ethanol without other clinical evidence of heart disease
Appears after binge drinking
Considered in px without structural heart disease and with new onset artrial fibrillation
Holiday heart syndrome
Abstinence resolution
Most frequently seen arrhythmia in holiday heart syndrome
Atrial fibrillation
Atrial flutter
Ventricular premature dep PVCs
A fib causes
Ethanol Valvular heart disease Ischemia Cardiomyopathy Cocaine Thyroid
Pathophysiology of holiday heart syndrome
1 inc secretion of epi and norepi
2 inc sympathetic output
3 rise in level of plasma free fatty acid
4 indirect effect through acetaldehyde (primary metabolite) or fatty acid ethyl ester
5 dec sodium current and affect intracellular pH
Most common symptom of holiday heart syndrome
palpitation
Only PE abnormality in Holiday Heart Syndrome
Irregular or thready pulse
Rapid heart rate
Cardiac dilation and CHF developing during last trimester of pregnancy within 6 mos after delivery
Peripartum cardiomyopathy
Peripartum cardiomyopathy rf
Multiparous, over 30 Twin pregnancy Malnutrition Tocolytic use (terbutaline, ritodrine) Premature labor Preeclampsia (vasospasm)
Most common drug that causes cardiotoxicity
Rf
>70
Tx
Doxorubicin
Danorubicib
Dexrazoxane
Myocardial hypertrophy
Abnormal diastolic filling
Intermittent ventricular outflow obstruction
Idiopathic hypertrophic subaortic stenosis
Hypertrophic obstructive cardiomyopathy (HOCM)
Cause of sudden cardiac death in young athletes
Thick walled heavy hypercontracting heart
Hypertrophic cardiomyopathy
Differential: Brugada syndrome
Hypertrophic cardiomyopathy pathophysiologic abnormality
Diastolic dysfunction
Most common mutation in hypertrophic cardiomyopathy
Half with HCM have family history
Cardiac b myosin heavy chain gene on ch 14
HOCM hallmark
systolic murmur at left sternal border as well as at apex
Most common complaint in HOCM
dyspnea
Inherited metabolic cardiomyo with LVH
Cardiac danon disease
Glycogen storage cardiomyo
Fabry disease
Friedrich ataxia -Frataxin gene
Abnormal diastolic function
Dec ventricular compliance/restriction of cardiac filling
Ventricular walls are excessively rigid and impede ventricular force
Caused by fibrosis, infiltration and hypertrophy
Restrictive cardiomyopathy
Most frequent cause of hemochromatosis
B thalassemia
Multiple transfusion and hemoglobinopathy
Marked subendocardial fibrosis
encroachment of lumen, dec ventricular filling and cardiac failure
Obliterative cardiomyopathy
Apical ballooning syndrome
Severe chest discomfort preceded by very stressful emotional or physical event
Women >50
ST segment elevation and T wave inversion in chest leads
Reversible within 3-7d
No long term cardiac dysfunction
Takotsubo stress cardiomyopathy
Firm rubbery waxy non compliant heart Diastolic dysfxn Systolic dsyfxn Arrhythmias OH
Amyloid cardiomyopathy
Parasite migrates to myocaridium and larvae causes necrosis
Heterophyes
Immune mediated myocarditis from post strep infection (rf) strep pyogenes bec of
M protein
Major causes of myocarditis
Coxsackie B
Diptheria
T cruzi
Myocarditis sx
Dx
Best dx
Dyspnea Fatigue Arrhythmia Chest pain Murmur
Degree of heartblock on ECG
Elevation of cardiac enzyme
Endomyocardial biopsy
Normal amount of pericardial fluid
Thin clear straw colored fluid in pericardial sac ie ultrafiltrate of the plasma
15- 50ml
Restricts anatomic position of heart
Minimizes friction between surrounding structures
Prevents displacement of heart and kinking of great vessel
Pericardium
Most common form of pericardial effusion
serous
ex CHF, hypoproteinemia
Pericardial effusion of blunt chest trauma
Serosanguinous
ex CPR
Pericarditis infectious agent
Pericarditis immunologically mediated
Virus
TB
Fungi
Parasite
Scleroderma
Postcardiotomy
PostMI Dressler syndrome
Drug hypersensitivity
Post myocardial infarction pericarditis
Dressler syndrome
Drug induced SLE can produce pericarditis
Procainamide (anti arrhythmic)
Hydralazine
Isoniazid
Methyldopac
Drug causes constrictive pericarditis
Methysergide
Miscellaneous causes of pericarditis
MI Uremia Ff cardiac surgery Neoplasia Trauma Radiation
Cancer that metastizes to the pericardium
Lung ca
Most often cause of myocarditis
Virus
Inflammatory reaction in epicardial and pericardial surface with scant numbers of PMN lympho and macrophage
Serous pericarditis
RF, SLE, scleroderma, tumor, uremia
Most frequent type of pericarditis
Serous fluid mixed with fibrinous exudate
Cause: MI, Dressler, uremia, chest radia, RF, SLE and trauma
Clinical presentation:
Fibrous/serofibrinous pericarditis
Pericardial friction rub
Fibrinous pericarditis is also known as
fibrinous pericarditis
Denotes invasion of pericardial space by infective organism
Route:
direct extension from neighboring inflamm
Thin to creamt pus 400-500 ml
Serosal surface reddened granular and coated with exudate
Acute inflamm reaction
Purulent or suppurative
Blood mixed with fibrinous or suppurative effusion
Most commonly causes by
Others: bacterial, diasthesis, TB(caseous) and cardiac surgery
Hemorrhagic
Malignancy
Healing of acute lesion resolution or pericardial fibrosis ranging from thick pearly nonadherent epicardial plaque to thin delicate adhesions to massive adhesions
soldier’s plaque
Pericardial sac obliterated and parietal layer tethered to mediastinal tissue
Adhesive mediastinopericarditis
Chronic or healed
Perocarditis symptoms
Chest pain in acute infectious type related to hypersensitivity
Pericardial friction rub
Reduction in voltage with large effusion on ECG
Elevation of ST in ECG returning to normal within hours
Pericardial effusion leads to cardiac tamponade
Water bottle appearance of cardiac silhouette on radio
Most common cause of tamponade
Neoplastic disease
Idiopathic pericarditis
Uremia
3 principal features of tamponade
Elevation of intracardiac pressure
Limitation of ventricular filling
Reduction in cardiac output
Cardiac involvemeny manifesting valvular abnormality from recurrent rheumatic fever
Rheumatic heart disease
Immune mediated disease triggered by antecedent infection assoc with
RHD
Most antecedent event: Group A Streptococcus Pyogenes
Group A Beta Hemolytic Streptococci GABHS
MC Cause of acquired heart disease in children/young adults
Peaks
Rheumatic fever
5-15 years old
rare in >30?
Usual presentation of RF
Pharyngitis (sore throat, odynophagia)
Strep skin infection
Most common cause of mitral stenosis 2/3 worldwide
rheumatic fever
Peaks at 25-40
Predominant at females
RHD
Most common cause of heart disease in children in developing countries
95% of ARF cases and RHD occur in developing
RHD
ARF is caused by infection of upper respiratory tract with group A strep pyogenes whose virulence factor is
Pathogenesis HLA
M protein
HLA DR7 HLA DR4(RA)
Immune response targeted at strep antigens aka
of Group A streptococcal carbohydrate a process called recognizing human cell (myocyte)
M protein
N-acetylglucosamine
Molecular mimicry
Demonstrates cross reactivity with M protein
Protein that is helical coiled
Antibodies to cardiac valve tissue cross react with leading to valvular damage
In IV users most common valve involved
Myosin
Laminin
N acetylglucosamine targetting mitral valve
Tricuspid valve
Modified JONES criteria for RF
(MAJOR)
CASES
Carditis Arthritis Sydenham chorea Erythema marginatum Subcutaneous nodule
Modified JONES Minor Criteria:
PEACH FEVER
Prolonged PR Elevated ESR Arthralgia C-RP Hx of preceding sore throat or recent strep infection Fever
RF Dx
Two major
One major and 2 minor
+
supporting evidence of preceding Group A Strep infection
Most common feature
Polyarthritis 60-75 Carditis 50-60 Chorea <2-30 Erythema marginatum and Subcutaneous nodules are rare <5
Exception
Presence of this alone with history of recent streptococcal infection is enough to diagnose RF
chorea
Inflammation of endocardium
valves, myocardium and pericardium (pancarditis)
Carditis
Diastolic rumble and opening snap
Mitral stenosis
Most common valves involved in rf
Mitral 2/3
Aortic 25%
Tricuspid infrequent
Pulmonic rare
Most common joints affected in rf
Occur in 75%
Pain is debilitating
Highly responsive to NSAIDs and salicylate
Knees, ankles, hip, elbow
Classic rash of ARF
Painless small mobile lumps seen overlying bony prominences
Erythema marginatum
Subcutaneous nodules
Erythema chronicum migrans
Lyme disease
Bull’s eye / Target rash
Histologic hallmark of subcutaneous nodules
Also a histologic marker of rheumatic activity
Aschoff bodies attached to tendon sheaths
Assoc with severe carditis
T lymhpocytic plasma cell
Aschoff bodies
Pathognomonic for RF
Caterpillar cells
Pancarditis
Histiocytes of cardiac myocyte
Anitschkow cell
Irregular thickening induced by subendocardial lesions exacerbated by regurgitant jets usually in L atrium
MacCallum Plaque
1-2mm small vegetation
Serve as nidus for infection
Verrucae
Infective endocarditis
Chronic RHD thickening the commisural fusion and shortening thickening and fusion of the tendinous cords
Calcification and fibrous bridging across valvular commisures
Fishmouth
Buttonhole appearance
Basal ganglia and caudate nucleus involvement
Present as single manifestation of RF
Sydenham’s chorea
St. Vitus dance
Trinucleotide CAG repeat
Atrophy of caudate nucleus
Huntington’s disease
Gradual onset
Involuntary uncoordinated purposely jerky movements aggravated under stress when patient is awake with emotional instability
Speech is indistinct halting and explosive
Milkmaids hand
Handwriting affected
Darting tongue
Sydenham’s chorea
Drug of choice for rheumatic f and prophy
Benthanzine penicillin
Orally BID
Amox 50mg/kg max 1g daily
drug of choice Used for treatment of arthritis, arthralgia and fever
Aspirin
May also take NSAID
Mainstay of primary prevention for ARF
Primary prophylaxis timely and complete Group A strep sorethroat with antibiotics
Mainstay of controlling ARF and RHD
Secondary prevention
Benzanthine pen G
1.2M units
600,000 u if <27 kg
