LECTURE №3. The syndrome of inflammatory changes an endocardium, myocardium and pericardium.

Myocarditis. Pericarditis. Rheumatic fever.

Cardiomyopathy.

The information block Inflammatory process can develop in the endocardium, a

myocardium and a pericardium. Most often the cardiac muscle is involved in inflammatory process.

Syndrome of an inflammation of a cardiac muscle (myocarditis) – this is clinico-laboratory-instrumental complex caused by inflammatory process in a myocardium.

• The causes:- Infectious (virus almost in 70 %, bacterial, rickettsiosis,

fungoid, scarlet fever parasitic, etc.);- Not infectious and allergic, caused by influence physical

and chemical factors (toxic, radiating).

CLINICAL PICTURE

• Complaints • pains in the field of heart, boring or pricking character, often

constant; • pains can arise without relation to the physical activity and do

not vary on intensity at physical or emotional loadings, nitroglycerine taking;

• pains without accurate irradiation, irregulatory in heart work,• breathlessness at first at more physical activity; • at illness progressing – at the minimal physical activity, then in

rest, • at night there is cough in horizontal position, • a palpitation, attacks of an asthma, • a pain and weight in right side of abdomen, oedema of the feet, • increase in stomach volume (аscites), • fast fatigue, the general weakness, especially at physical activity.

CLINICAL PICTURE• The above described complaints can

be accompanied by a so-called syndrome of an inflammation and an intoxication: increase of temperature, absence of appetite, sweating, weakness.

Survey.• Pallor of skin, sometimes light acrocyonosis,

swelling and a pulsation of cervical veins, oedema of feet.

Palpation: apex beat• Decreased, displaced to the left, • Pulsesoft, frequent. Percussion:• Expansion of the heart relative dullness

borders , mainly to the left.

Survey.Auscultation:• Soft S1 on the heart apex,

• accentuated S2 over a pulmonary artery,• «a gallop rhythm», • a tachycardia, • systolic murmur on an apex or in the field of

III-IV intercostals space at the left at a breast.

• The blood pressure it is lowered, especially systolic, it is reduced pulse pressure.

Laboratory and instrumental Inspection methods of investigation:

• Common blood count: • increase of leucocytes, increase of ESR, hyperglobulinemia

(alpha and gamma-globulinemia), increase of levels of cardiac enzymes (creatine phosphatekinase, lactate dehydrogenase, asparate aminotransferase, troponins).

• Electrocardiogram: the sinus tachycardia, extrasistoly, heart block, atrial fibrillation.

• Pfonocardiography: S1 - reduction of the amplitude, registration of "a gallop rhythm», systolic murmure on apex of the heart.

• Echocardiography: decreased cardiac output.• Roentgenological research: increase in the sizes of heart

(cardiomegaly).

MYOCARDITIS CRITERIA

MAJOR CRITERIA:PATHOLOGICAL EKG changes ( Rhythm and

conductions diisturbencies)Sarcoplasmatic enzymes and isoenzymes

activity increasing (LDH, CPK)CONGESTIVE HEART FAILURE or CARDIOGENIC

SHOCK.

MYOCARDITIS CRITERIAMINOR:TACHICARDIASOFT S1

Gallop Rhythm ADDITIONAL CRYTERIA• ECHO –local myocardial asynergy• Scan with Gallyi-67• Myocardium biopsyTwo major or one plus two minor plus

preceeding infection evidence for diagnosis nesessary

Rheumatic fever

• Is an inflammatory, possibly autoimmune disease, in nature, involves many tissues, including the heart, joints, skin, and central nervous system.• Preceding infection of the upper

respiratory tract with group A Streptococcus is a prerequisite to the development of acute rheumatic fever.

Signs and symptoms• Joint symptoms ranging from arthralgias to frank

arthritis (75%)• Joints involved are medium to large, e.g., ankles,

knees, wrists• Joint involvement is classically migratory• Joint symptoms usually disappear in 1 – 4 weeks

without permanent deformities• Carditis (65%), mild or severe with murmurs• Cardiac involvement may include pericarditis,

myocarditis, and/or valvular insufficiency. Appears within 2 weeks and lasts 6 weeks to 6 months

Mitral valve in the acute phase of Rheumatic fever

Signs and symptoms• Valvular damage may be permanent. • P – R prolongation on ECG• Erythema marginatum (classic rash) <5%• Subcutaneus nodules (painless, hard swellings

overlying bony prominences) 5 – 10%• Chorea is often a late finding but may be a

presenting complaint. It occurs in 10 – 15% of patients, and its duration is not altered by treatment.

• Fever 101 – 104 0F (38.3 – 40.0 0C)• Abdominal pain is common. It may be severe.

Signs and symptoms

• Epistaxis (historycally important, but rarely seen in acute rheumatic fever)

• Facial tics• Facial grimace

Pericarditis• The commonest causes are viral or bacterial infection, Ml, or

uraemia.• Pain, not related to movement or exertion• Friction rub of a pericardium, • electrocardiogram-change, • effusion in a pericardium with development tamponade of

heart • and paradoxical pulse - the basic signs of an inflammation of

a pericardium.• The pain represents the major symptom. Usually it is marked

at the acute infectious process, connected with hypersensitivity or autoimmunity processes, however often is absent at slowly developing tuberculosis, postradiating and uraemic pericarditis.

• The pain-сonstant soreness, compressing, • as a rule, is localised retrosternal ,• irradiation to one, or in both hands and reminds

these an ischemic pain.• Often has character pleural, increase, worse at a

breath (inspiration -pleuritic) and is acute, • It is accompanied by cough, varies at change of

position of a body. • It is characteristic, that the pain relieved slightly by

sitting forwards.

Pericarditis

Pericarditis• Pericardial friction rub - important physycal sign,

sometimes it comes to light only at strong pressure of a diaphragm with stethoscope on a chest wall.

• This is a scratching sound, comparable with creaking leather, heard with each heartbeat caused by inflamed pericardial membranes rubbing against each other.

• Pericardial friction rub is heard best in exhalation, louder as the patient is sitting up, leaning forward, or in lying position on the left side.

• Pericardial friction rub usually has changeable, passing character. The loud creaking sound can disappear within several hours and appear then next day.

Clinical features:

• These are mainly of right heart failure with JVP (with prominent x and y descents,);

• Kussmaul's sign (JVP rising paradoxically with inspiration);

• soft, diffuse apex beat;

• quiet heart sounds; muffled S1 & S2.

• S3;

• diastolic pericardial knock, • hepatosplenomegaly,• ascites, • and oedema.

• Electrocardiogram - at acute process without massive effusion usually is revealing concave (saddle-shaped) ST segment elevation , in 2,3 standard leads and from V2 to V6, with depression SТ in aVR and V1, without QRS complexes change,

• is rare against the lowered voltage of ECG, but may be normal or non-specific (10%).

• Fluid in a pericardium cavity - heart sounds become decreased. • The pericardial friction rub can disappear or is clear be listened. • Apex beat can disappear, but sometimes is palpatedmedially

from the left border of cardiac dullness. • On the the chest X ray film (CXR) shows an enlarged, globular

heart (if >250mL fluid), decreased ventricular pulsations.

Pericarditis

Pericarditis• EchoCG -echo-free space (>2cm, or >1cm if acute),

between a posterior part of pericardium and posterior part epicardium left ventricule means fluid presence in a pericardial cavity (inflammatory effusion).

• diastolic collapse of right atrium and right ventricle

• Paradoxical pulse is more expressed, than in norm (10 mm hg) reduction the systolic blood pressure at a breath. At a breath at palpation reveal easing or disappearance of arterial pulse during a breath.

Management:

• Seek expert help. The pericardial effusion needs urgent drainage .

• Send fluid for culture, ZN stain/TB culture & cytology.

EKG

Infective endocarditis• The infectious inflammation of the endocardium - (a

septic condition), a bacterial inflammation of the endocardium.

• Etiology:– Is due to microbial infection of a heart valve, the lining of

a cardiac chamber or blood vessel, or a congenital anomaly ( septal defect).

– The causative organism is usually a bacterium streptococci, staphylococci, gram-negative bacilli, heamophilus, anaerobes), may be a rickettsia, chlamydia or fungus.

Classification• 50% of all endocarditis occurs on normal valves. It

follows an acute course, and presents with acute heart failure.

• Endocarditis on abnormal valves tends to run a subacute course. Predisposing cardiac lesions: aortic or mitral valve disease; tricuspid valves in IV drug users; coarctation; patent ductus arteriosus; VSD; prosthetic valves. Endocarditis on prosthetic valves may be early (acquired at the time of surgery, poor prognosis) or late (acquired haematogenously

Causes

• Bacteria: • Any cause of bacteraemia exposes valves to the risk of bacterial

colonization (eg dental work; UTI; urinary catheterization; cystoscopy; respiratory infection; endoscopy (controversial); colonic carcinoma; gall bladder disease; skin disease; IV cannulation; surgery; abortion; fractures).

• Quite often, no cause is found. • Streptococcus viridans is the commonest (35-50%). Others:

enterococci; Staphylococcus aureus/epidermidis; diphtheroids and microaerophilic streptococci.

• Rarely: HACEK group of Gram -ve bacteria (Haemophilus“Actinobacillus Cardiobacterium EikenellaKingella); Coxiella burnetii; Chlamydia.

• Fungi: These include Candida, Aspergillus, and Histoplasma. Other causes: SLE (Libman-Sacks endocarditis); malignancy.

Clinical features

• The patient may present with any of the following:• Signs of infection: Fever, rigors, night sweats, malaise,

weight loss, anaemia, splenomegaly, and clubbing. • Cardiac lesions: Any new murmur, or a change in the

nature of a • Vegetations may cause valve destruction, and severe

regurgitation, or valve obstruction. An aortic root abscess causes prolongation of the PR interval, and may lead to complete AV block.

• LVF is a common cause of death.• Immune complex deposition: Vasculitis may affect any

vessel. • Microscopic haematuria is common;

Clinical features• Glomerulonephritis and acute renal failure may

occur.• Roth spots (boat-shaped retinal haemorrhage with

pale centre); • splinter haemorrhages (on finger or toe nails); • Osler's nodes (painful pulp infarcts in fingers or

toes) and Janeway lesions (painless palmar or plantar macules) are pathognomonic.

• Embolic phenomena: Emboli may cause abscesses in the relevant organ, eg brain, heart, kidney, spleen, GI tract. In right-sided endocarditis, pulmonary abscesses may occur.

Duke criteria for infective endocarditis

• Major criteria:• Positive blood culture:– typical organism in 2 separate cultures or– persistently +ve blood cultures, eg 3, >12h apart (or

majority if 4)• Endocardium involved:– positive echocardiogram (vegetation, abscess,

dehiscence of prosthetic valve) or– new valvular regurgitation (change in murmur not

sufficient).

Duke criteria for infective endocarditis• Minor criteria:• Predisposition (cardiac lesion; IV drug abuse)• Fever >38В°C• Vascular/immunological signs• Positive blood culture that do not meet major criteria• Positive echocardiogram that does not meet major

criteria.

• How to diagnose:• Definite infective endocarditis: 2 major or 1 major

and 3 minor or all 5 minor criteria (if no major criterion is met).

• Features of haemodynamics play the big role in development of a septic inflammation of endocardium. Blood-groove infringement in sites, damaging to other structural changes or anomalies, promotes surface change of endotelium and to formation of thrombotic imposings, which then become the centre of sedimentation of microorganisms.

• Microorganisms circulating in blood are attached to endotelium, then become covered by fibrin imposing, forming vegetations. Microorganisms fastly cause destruction of valves, them forming as emboli. Therefore following basic pathological features may be seen:

Local destructive effects of intracardiac infection:- Destruction of the valve cusp, produssing ulceration and ultimately

regurgitation.

- Rupture of chordae tendineae.

- Paravalvular abscess leading to destruction of conduction system ( causing arrytmias) and purulent pericarditis.

- Large vegetations may causer functional valvular stenosis.

• Embolism

-Embolisation fragments of vegetations producing splenic, renal, myocardial or pulmonary infarctions.

• Metastatic infection

-Septic emboli may cause infection in any organ or tissue producing abscess there.

• Deposition of immune complexes

-Deposition of immune complexes ( antigen antibody complexes) in various tissues producing extracardiac manifestations e. g. arthaldia, Roth spots, Janeway lesions, focal glomerulonephritis, acute vasculitis.

TYPES:

1. Subacute endocarditis

2. Acute endocarditis

3. Prothetic endocarditis

• At circulation in blood of microbes and blood crops define a kind and number of bacteria. Occurrence emboli - the important sign, is more often observed emboli of vessels of a brain, a spleen,GIT, hearts, finitenesses kidneys.

• Survey - petechiae - displays acute vasculitis, immunopositive by the nature. Other defeats of a skin accompanied by pains, pressure , can be a consequence of emboli.

• Complaints: the general weakness and fatigue, reduction of weight of a body, a fever, sweat at night, appetite loss, arthralgia. The emboly can cause paralyses, pulmonary infarction, heart (a pain in a chest), and also a painful syndrome depending on where has got emboli.

• Physical signs: in early terms can be any signs. At the chronic - there is a pallor of skin, a fever. Increase of pulse rate. Skin changes petechiae- the small size, the red colour, looking like hemorrhages, not growing white at press, not strained, painless. They are localised on mucous of throat, oral cavity, conjunctivus, on a skin of the upper part of a thorax. On mucous a mouth and conjunctiva in the centre the pale ( Roth’s spots), gradually they get brown colour and disappearance. Under nails linear hemorrage, as at a trauma. As consequence emboli on palms, fingers of hands, heels, other places - erytromatous, painful, intense small nodes (Osler’s). The emboli in larger arteries can cause a gangrene of fingers of finitenesses, change of fingers of hands in the form of clubbing, in rare instances – jaundice.

On soft tissues of fingers of hands - erhytromatous not intense hypodermic maculopapulous defeats ( Jeneway’s lesions), inclined to ulceration.

• Heart area - considerable changes of character of cardiac noise, for the first time arisen dyastolic noise - owing to the ulceration valve, rupture of valvular chords or formation of very big vegetations. Destruction of heart valves can become complicated rupture valvular chords or punching of shutters, that quickly leads to progressing heart failure. At addicts - it is damaged the right atrio-ventricular valve with signs right side cardiac failure.

• Laboratory investigation.-CBC: leucocytosis, neutrophylosis, increase ESR. At a chronic course - an

anaemia, and then a syndrome of hyperspleen.- The positive hemoculture (revealing of the activator and its sensitivity to

antibiotics).- ECHO: vegetations.

• Pathology:• The acute phase of rheumatic fever is characterized by

exudative and рroliferative inflammatory reactions involving connective or collagen tissue. Heart, joints, brain, skin and subcutaneous tissues are primarily affected.

• In rheumatic carditis,_principally affected part is heart valve especially the aortic and mitral valves. The characterjstic lesion of rheumatic carditis, is the Aschoff nodule, which is a granulomatous lesion with a central necrotic area. The valve cusps become tickelied by ederma and_by the infiltration of capillaries. Later a row of tiny vegetations is foimed along the lines of closure of the valve leaflets.

Criteria for Rheumatic fever

Diagnostic categories

Criteria

Major manifestations

Carditis, polyarthritis, chorea, erythema marginatum, subcutaneous nodules

Minor manifestations

clinical: fever, polyarthralgia laboratory: elevated acute phase reactants (erythrocyte sedimentation rate or leukocyte count) electrocardiogram: prolonged P-R interval

* Supporting evidence of а

preceding streptococcal

infection

elevated or rising antistreptolysin-O or other streptococcal antibody, or а positive throat culture, or rapid antigen test for gr. А streptococci, or recent scarlet fever.

Complications

• Congestive heart failure• Rheumatic heart disease• Arrhythmias• Pericarditis• Pericardial effusion• Rheumatic pneumonitis

INVESTIGATIONS

Although the diagnosis of rheumatic fever is clinical, following investigations may be helpful:

Throat swab culture• Throat swab culture for group A streptococcus.Antistreptolysin О titer (ASOT):• The_rising_ titer of antibodies against streptococci (ASOT)

indicates a recent streptococcal infection. ASOT is elevated in about 80% of cases. If ASOT is normal, it should be rechecked after a week, as it may then be elevated. If not elevated then anti-DNase В is performed. Both types of titers are elevated for weeks or months.

ESR and C-reactive protein:• ESR and C-reactive protein are the acute phase

reactants indicating tissue inflammation and are elevated during the acute stages of the disease.

• Erythrocyte sedimentation rate (ESR) is useful in monitoring the course of the didease; it usually returns to normal as the rheumatic activity subsides. ESR may be elevated in patients with anemia and may be supressed to normal levels in patients with_congestive heart failure. Unlike the ESR, the CRP level is unaffected by anemia or cardiac failure.

Blood CP• Leukocytosis • Anemia is usually mild to moderate and is normocytic

normochromic in morphology.Chest x-ray: may be normal or indicates cardiomegaly,

pulmonary edema and increased pulmonary vascularity.ECG: may indicate prolonged PR interval, heart block,

features of pericarditis and myocarditis.

Echocardiography: It may show mitral regurgitation due to prolapse of anterior mitral leaflet, heart dilatation and valve abnormality. Myocardial dysfunction and pericardial effusion may also be seen.

ACUTE MYOCARDITIS

Acute myocarditis causes focal or diffuse inflammation of the myocardium. Majority of cases are due to infection caused by viral (most common), bacterial, rickettsial, fungal, or parasitic agents; but toxins, drugs and immunologic disorders can also cause myocarditis.

CAUSES OF MYOCARDITIS Primary myocarditis • Myocarditis caused by acute viral infection is called primary myocarditis.• Viruses: Coaxsackie A and B, adenovirus, influenza virus, HlV virus, Epstein-

Barr_yirus, herpes virus, cytomegalovirus, mumps virus, respiratory syncytial virus and rubella virus.

Secondary myocarditis• Myocarditis caused by non-viral cause is called secondary myocarditisInfections• Bacterial: Diphtheria, brucellosis, H.influenza, mycoplasma,

pneumococci, salmonella, streptococcus, staphylococcus.• Protozoal: Entamoeba, trypanosomiasis .• Fungal: Candida, actmomyces, aspergillus

Endocrine and metabolic disorders

• Diabetes, hypo and hyperthyroidism, acromegaly, carcinoid syndrome, inherited siorage diseases.

Connective tissue diseases Scleroderma, SLE, polyarteritis nodosa.Infiltrative disorders• Hemochromatosis, hemosiderosis, sarcoidosis, amyloidosis.Endomyocardial fibrosis and eosinophilic heart disease.Toxins Neuromuscular disorders

Clinical features• The clinical presentation of infectious myocarditis is variable.• Many patients are asymptomatic and will have a complate

resolution of myocarditis without complications.• Patients may present with heart failure several days to a few

weeks after the onset of an acute febrile illness or a respiratory infection; or heart failure without antecedent symptoms.

• Pleural chest pain secondary to pericarditis is common.• Patient may present with life threatending atthythmias or

embolic events.• Patients may present after months or years of development of

dilated cardiomyopathy.

On examination

• Tachycardia • Pericardial friction rub may be heard• Features of congestive cardiac failure• S3 gallop murmurs of tricuspid and mitral

regurgitation may be present (due top dilatation od chambers)

Investigation• ECG: Non-specific ST segment and T wave abnormalities,

arrhythmias or conduction delay.• X-ray: shows normal heart size initially and then cardiomegaly.• Cardiac enzymes: may be elevated if the patient presents acutely

usually within one month of symptoms.• Echocardiography: trashtoracic echo is usefulto assess the size of

chambers and their function. In fulminant myocarditis there is increased ventricular wall thickness due to inflammation associated with interstitial edema.

• MRI: has sensitivity of 100% while specifity 90-100% in the diagnosis of acute myocarditis.

• Endomyocardial biopsy: It is the gold standard for diagnosing muocarditis and is useful when a treatable cause is found.

Clinical pathological types of myocarditis

• Fulminant myocarditis• Subacute myocarditis• Chronic active myocarditis• Chronic persistent myocarditis

Cardiomyopathy• The cardiomyopathy is a general term indicating disease

of the cardiac muscle primarity, not associated with the major causes of cardiac disease such as ischemic heart disease, hypertension, pericardial disease, valvular disease, or congenital defects. While some have specific causes, many cases are idiopathic.

• TYPES:• Dilated cardiomuopathy (ventricular dilatation)• Hypertrophic cardiomyopathy (myocardial hypertrophy)• Restrictive cardiomyopathy (impaired ventricular filling)

Dilated cardiomyopathy

• Is characterized by dilatation and impaired ventricular contraction (left ventricular dysfunction) leading to progressive left-sided and later, right-sided failure. Functional mitral and/or tricuspid regurgitation may occur. Arrhythmias are common. Cause cannot be identified in most of the patients; myocarditis and chronic alcohol abuse are probably freqent causes of dilated cardimyopethy.

• Possible causes of dilaed cardiomyopathy:• Idiopathic ( most common)• Alcohol• Viral myocarditis• Familial and genetic factors• Peripartium cardiomyopathy• Diabetes mellitus• Sarcoidosis, hemochromatosis• Connective tissue disease (multiple sclerosis)

Clinical features:• Features of cardiac failure, arrhythmias or emboly• Cardiac failure, S3 gallop rhythm• Ventricular dilatation leads to functional mitral or tricuspid valvular

regurgitation

Investigations:X-ray chest: shows large flask-shaped heart (massive cardimegaly)Echocardigraphy: reveals dilatation of left ventricle, dilatation of right

ventricle with poor global contraction. It helps in assessment of degree of left ventricular function, and axclusion of concominant valvular or pericardial disease.

ECG: tachycardia, conduction abnormalities, ST- segment and T wave changes, ventricular ectopics.

Cardiac biopsy: fibrosis and non- specific leukocyte infiltration.Cardiac catheterization: left ventricular dilatation and dysfunction, high end-

diastolic pressure, low cardiac output.

Hypertrophic cardiomyopathy• Is characterized by marked hypertrophy of the left and/or right ventricle

particularly the interventricular septum in the absence of a cardiac or systemic cause (asymmetrical septal hypertrophy).

• The hypertrophied septum and the anterior movement of mitral valve across the outflow tract making with the ventricular septum midsystole result in mechanical obstruction to left ventricular ejection. Some degree of mitral regurgitation may develop.The left ventricular outflow tract is narrowed during systole between the bulging septum and systole anterior motiob (SAM) of the anterior mitral leaflet causing obstruction to left ventricular emptying therefore called obstructive cardiomyopathy. The obstruction is worsened by factors that increase myocardial contractility or that decrease left ventricular filling.

• Inabout 50% cases it is an onherited as autosomal dominant trait, soma patients have prolonged history of hypertension and soma cases are sporadics. It is_the most common genetically transmitted cardiac disorder. Incidence is I in 500 of the gcnerl population.

• This type of hypertrophy usually manifests in adolescents and young adults, most of the patients are identified during screening of relatives of patients with hypertrophic cardiomyopathy. It may manifest 4thor 5th

decade and sometimes in elderly.• The physiological characteristic of HOCM is diaslolic dysfunction (while

systolic dysfunction in dilated cardiomyopathy) resulting in abnormal sliffness of left ventricle with resultantl impaired ventricular filling. Left ventricular end-diastolic pressure increases resulting in pulmonary congestion and dyspnea.

CLINICAL FEATURES • Symptoms: • Patient may be asymptomatic, diagnosed on echo during screening.• Dyspnea: it is due to pulmonary congestion resulting from elevations in pulmonaru

venous and left atrial pressure because of stiffness of hepertrophic ventricles (diastolic dysfunction).

• Chest pain: This occurs on exertion or at rest due to compression of intramyocardial coronary arteries and increased oxygen requirement due to increased myocardial contraction and muscle mass. Chest pain usually does not respond to sublingual nitroglycerine.

• Syncope: Especially post-exertional due to inadequate cardiac output with exertion or from cardiac arrhythmia.

• Palpitation: It is_due to arrhythmias. Atrial fibrillation js_common and poor prognostic sign. Ventricular arrhythmias are also common and sudden death may occur.

• Sudden death: This can occur at any age but the highest rates occur in adolescents and young adults. ~ "

• Congestive heart failure •-

On CVS Examination Palpation:• Apex beat is displaced laterally, forceful and diffuse. A double apical pulsation

(forceful atrial contraction produces a palpable fourth heart sound).Auscultation: • S1 is normal, often preceded by S4, normal S2.• Late systolic murmur beast heard between the apex and left sternal border

radiating to lower sternal border, ахillа and base of heart but not into the neck vessel (that differentiates it from murmur of aortic stenosis). It is produced due to left ventricular outflow obstruction in late systole. This murmur is increased by Valsalva maneuver and by standing while decreased by squatting.

• A pansystolic murmur at apex may be heard due to mitral regurgitation as a result of systolic anterior motion of anterior leaflet of mitral valve).

• Reversed splitting of second-heart sound.• A fourth heart sound.Pulse: A jerky carotid pulse with sharp upstroke (because of rapid ejection and

sudden obstruction to left ventricular outflow during systole).JVP: prominent a wave due to forceful atrial contraction.

INVESTIGATIONS X –ray chest: Heart usually not greatly enlarged ECG:• ECG demonstrates left ventricular hypertrophy• Large abnormal Q waves in 20-50% of patients in leads II, III, aVF or V2-V6

(pseudoinfarction), lelt axis deviation.• Occasionally LBBB OR RBBB, APCs, PVCs, short PR may be present.Echocardiography: It is diagnostic showing:• Asymmetric left ventricular hypertrophy.• Systolic anterior motion (SAM) of anterior leaflet of mitral valve.• Small left ventricular cavity size.• Dilated left atrium.• Left ventricular diastolic dysfunction.Cardiac catheterization• Small hypercontractile left ventricle• Dynamic left ventricular outflow obstruction• Diastolic dysfunction

RESTRICTIVE CARDIOMYOPATHY

• Restrictive cardiomyopathy is characterized by impaired diastolic ventricular tilling due to stiffness of ventncIes with preserved systolic contractile function. Because of the-abnormallу elevated right atrial and ventricular filling pressures, cardiac filling is diminished. As a result, the clinical manifestations of restrictive cardiomyopathy usually are predominantly right-sided. There is high atrial pressure with atrial hypertrophy, dilatation and later atrial fibrillation.

• Causes of restrictive cardiomyopathyAmyloidosis, sarcoidosis, hemochromatosis

radiation, carcinoid syndrome, scleroderma,myocardial fibrosis after open-heart surgery, Loffler's syndrome.

• Symptoms: 1. Dyspnea and fatigue (due to low cardiac output). 2. Abdominal discomfort (due to hepatic congestion).3. Restriction to ventricular filling results in persistently elevated venous

pressures and consequent hepatic enlargement, ascites and dependent edema (right sided failure).

• Signs: Pgysical signs are simular to those of constrictive pericarditis e.g.-Raised JVP with diastolic collapse (Friendreinch’ signs) and raised JVP on

inspiration (Kussmaul’ signs).-Cardiac enlargement with a third or fourth heart sound is common.

Investigations:• Chesty X-rays: It shows mild to moderate cardiomegaly.• ECG: Usually has low-voltage, conduction abnormalities, ST segment

and T wave abnormalities.• Echocardiography: It shows symmetrical myocardial

thickening,usually a normal systolic function, but impaired ventricular filling.

• Cardiac catheterization: It shows higher diastolic pressure. Right and left ventricular pressure tracings demonstrate a characteristic "square root sign"—a deep and rapid early decline at the onset of diastole followed by a rapid rise to a plateau. Normal or mildly reduced left yentricular function.

Cardiac catheterization helps in distinction from constrictive pericarditis. • Endomyocardial biopsy: helpful in diagnosis

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Maneuvers

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Echocardiography