Medicine Marrow Pg 341-350 (Cardiology)
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What is considered the gold standard for the diagnosis of Hypertrophic Cardiomyopathy (HCM)?

  • Magnetic Resonance Imaging
  • Echocardiogram (correct)
  • Chest X-ray
  • Electrocardiogram

    • Propranolol is the only medication recommended for managing Hypertrophic Cardiomyopathy.

    False

    List one indication for the use of an Implantable Cardioverter Defibrillator (ICD).

    History of cardiac arrest

    The thickness of the Left Ventricle in Hypertrophic Cardiomyopathy is _____ mm.

    <p>215</p> Signup and view all the answers

    Match the following medications with their descriptions:

    <p>Propranolol = β-blocker, reduces heart rate response to exercise Verapamil = CCB, indicated if β-blockers are contraindicated Diltiazem = CCB, used for angina relief Disopyramide = Antiarrhythmic, used in combination with β-blockers or CCBs</p> Signup and view all the answers

    What is a characteristic finding in an ECG for cardiomyopathy?

    <p>Mimics acute coronary syndrome</p> Signup and view all the answers

    Short-term prognosis for cardiomyopathy is considered excellent.

    <p>False</p> Signup and view all the answers

    Name one medication used in the treatment of cardiomyopathy.

    <p>Carvedilol</p> Signup and view all the answers

    In a normal angiogram of cardiomyopathy, the _____ apex is noted, while the base is hypercontractile.

    <p>hypokinetic</p> Signup and view all the answers

    Match the complications of cardiomyopathy with their descriptions:

    <p>Shock = A critical condition where the body is not getting enough blood flow. LV rupture = Tearing of the left ventricle which can be fatal. Thromboembolism = The formation of a blood clot that travels to other parts of the body. Same as MI = Complications that resemble those of a myocardial infarction.</p> Signup and view all the answers

    What is the most common cause of chronic mitral regurgitation in developed countries?

    <p>Mitral valve prolapse</p> Signup and view all the answers

    Diastolic function is abnormal in cases of mitral regurgitation.

    <p>False</p> Signup and view all the answers

    What component of the mitral valve is primarily affected in acute mitral regurgitation due to papillary muscle rupture?

    <p>Papillary muscle</p> Signup and view all the answers

    In mitral regurgitation, there is a retrograde flow of blood from the left ventricle to the ______.

    <p>left atrium</p> Signup and view all the answers

    Match the types of mitral regurgitation with their characteristics:

    <p>Acute MR = Often due to papillary muscle rupture Chronic MR = Long-term valve dysfunction Primary MR = Caused by leaflet or chordae tendinae abnormality Secondary MR = Related to left ventricular dysfunction</p> Signup and view all the answers

    Which condition is most commonly associated with rheumatic heart disease (RHD)?

    <p>Mitral Regurgitation (MR)</p> Signup and view all the answers

    Eccentric hypertrophy of the left ventricle (LV) leads to an increase in the thickness of the LV wall.

    <p>False</p> Signup and view all the answers

    Name two autoimmune diseases that can lead to aortic regurgitation (AR).

    <p>Takayasu arteritis, Behcet's disease</p> Signup and view all the answers

    In mitral regurgitation, blood leaks from the left ventricle (LV) to the __________.

    <p>left atrium (LA)</p> Signup and view all the answers

    Match the following conditions with their characteristics:

    <p>Mitral Regurgitation = Incompetence of the mitral valve, allowing backflow to the LA Mitral Stenosis = Narrowing of the mitral valve opening Aortic Regurgitation = Backflow from the aorta to the LV Aortic Stenosis = Narrowing of the aortic valve opening</p> Signup and view all the answers

    Which of the following conditions are considered non-infiltrative causes of cardiomyopathy?

    <p>Diabetes Mellitus and Scleroderma</p> Signup and view all the answers

    Amyloidosis does not involve the heart in secondary AA types.

    <p>True</p> Signup and view all the answers

    What is the m/c cause of dilated cardiomyopathy (DCMP)?

    <p>Titin mutation</p> Signup and view all the answers

    In Fabry's disease, the presence of ______ bodies is a notable feature.

    <p>Lamellar</p> Signup and view all the answers

    Match the following types of amyloidosis with their characteristics:

    <p>1° AL = Involves heart and kidneys 2° AA = No heart involvement, only kidney Familial (ATTR) = Transthyretin related amyloidosis Wild type = Senile systemic amyloidosis</p> Signup and view all the answers

    Which of the following is a common characteristic of the murmur associated with mitral regurgitation?

    <p>Pansystolic murmur</p> Signup and view all the answers

    An S4 heart sound is typically present in cases of mitral regurgitation.

    <p>False</p> Signup and view all the answers

    What is the most common demographic profile for mitral valve prolapse?

    <p>Females aged 15-30 years</p> Signup and view all the answers

    The treatment for mitral regurgitation usually involves _____ over replacement when considering surgical options.

    <p>mitral valve repair</p> Signup and view all the answers

    Match the following features to the appropriate conditions:

    <p>Pansystolic murmur = Mitral Regurgitation Floppy valve = Mitral Valve Prolapse Universal S3 = Mitral Regurgitation Posterior leaflet prolapse = Mitral Valve Prolapse</p> Signup and view all the answers

    Which side effect is associated with Disopyramide?

    <p>QT prolongation</p> Signup and view all the answers

    Restrictive Cardiomyopathy is more commonly genetic than Hypertrophic Cardiomyopathy.

    <p>False</p> Signup and view all the answers

    What surgical management is indicated for refractory HCM when gradient is greater than 50 mmHg?

    <p>Septal myomectomy</p> Signup and view all the answers

    In Restrictive Cardiomyopathy, the ventricles are described as ___ and non-compliant.

    <p>stiff</p> Signup and view all the answers

    Match the following features with their corresponding consequences:

    <p>Atrial stretch and dilation = Increased risk of atrial fibrillation Increased systemic blood pressure = Systemic congestion High end diastolic pressure = Right and left ventricle pressure mismatch Both atria enlarged = Almost normal systolic function</p> Signup and view all the answers

    What is a characteristic finding in the ECHO for arrhythmogenic RV dysplasia?

    <p>Fibrofatty replacement of RV myocardium</p> Signup and view all the answers

    Takotsubo cardiomyopathy is also known as broken heart syndrome.

    <p>True</p> Signup and view all the answers

    What kind of dysfunction is observed in Takotsubo cardiomyopathy?

    <p>Transient LV dysfunction</p> Signup and view all the answers

    The mutation leading to arrhythmogenic RV dysplasia is found in proteins called ______.

    <p>desmosomes</p> Signup and view all the answers

    Match the following terms with their descriptions:

    <p>Takotsubo cardiomyopathy = Stress-induced CMP Recurrent tachycardia = Tachycardia Induced CMP Right ventricular dysplasia = Autosomal recessive condition Myocardial stunning = Neurogenic myocardial stunning</p> Signup and view all the answers

    What effect does nitroglycerin (NTG) have on afterload in cases of acute mitral regurgitation (MR)?

    <p>Decreases afterload</p> Signup and view all the answers

    Patients with severe mitral regurgitation often have a normal ejection fraction.

    <p>True</p> Signup and view all the answers

    What is the effect of decreased wall thickness on the required filling pressure of the left ventricle in mitral regurgitation?

    <p>Less filling pressure is required.</p> Signup and view all the answers

    In patients with mild to moderate mitral regurgitation, the usual symptom presentation is __________.

    <p>asymptomatic or palpitations</p> Signup and view all the answers

    Match the following parameters with their respective conditions in mitral regurgitation:

    <p>Ejection Fraction (EF) = Normal in mild to moderate MR; Normal/↓ in severe MR LV End Diastolic Pressure (LVEDP) = Normal in mild to moderate MR; ↑↑ in severe MR Symptoms = Asymptomatic in mild to moderate MR; Dyspnea +++ in severe MR LV End Systolic Pressure (LVESP) = Normal in mild to moderate MR; ↑↑ in severe MR</p> Signup and view all the answers

    Which of the following symptoms may be associated with excess leaflet tissue buckling into the left atrium?

    <p>Palpitation</p> Signup and view all the answers

    A high-pitched, whooping murmur is typically heard at the left ventricle apex.

    <p>False</p> Signup and view all the answers

    What is the effect of squatting on mitral valve prolapse?

    <p>It decreases prolapse.</p> Signup and view all the answers

    When the chordae ruptures, it may lead to acute mitral __________.

    <p>regurgitation</p> Signup and view all the answers

    Match the following types of murmurs with the conditions associated with them:

    <p>Non-ejection click = MVP Systolic crescendo-decrescendo = Mitral regurgitation High-pitched, whooping = Aortic regurgitation S3 gallop = Heart failure</p> Signup and view all the answers

    Study Notes

    Hypertrophic Cardiomyopathy (HCM)

    • Gold standard in diagnosis: Echocardiogram (ECHO)
    • ECHO findings: Left Ventricle (LV) thickening of 215 mm with no dilatation in the absence of other causes (valvular, systemic, cardiac)
    • Doppler: Dagger sign
    • Medical Management: Beta-blockers (Propranolol) and Calcium Channel Blockers (Verapamil, Diltiazem)
    • Implantable Cardioverter Defibrillator (ICD) indications: History of cardiac arrest, family history of sudden cardiac death, sustained ventricular tachycardia, abnormal Holter study, unexplained syncope, LVH > 30 mm

    Mitral Regurgitation

    • Most common heart disease due to Rheumatic Heart Disease (RHD): Mitral Stenosis (MS)
    • Most common type of rheumatic heart disease: Mitral Regurgitation (MR)
    • Causes of Aortic Regurgitation (AR) due to autoimmune diseases: Takayasu arteritis, Behcet's disease, Ankylosing spondylitis
    • Pathophysiology: Incompetence of mitral valve leaflets leading to blood leakage (from LV to LA), causing increased blood volume in LA and increased blood flow from LA to LV.
    • LV: Eccentric hypertrophy with dilatation due to increased radius
    • Classification: Acute MR (e.g., papillary muscle rupture due to MI), Chronic MR (primary or secondary)

    Chronic MR

    • Primary (organic) MR: Leaflet/chordae tendinae abnormality (RHD, Mitral valve prolapse) or autoimmune disorders (SLE, RA).
    • Secondary (functional) MR: Annular abnormality, LV dysfunction, annular dilatation, HCM.

    Medical Management for HCM

    • Beta-blockers (DOC): Propranolol (2mg/kg, max 480mg)
      • Decreases heart rate response to exercise
      • Decreases LVOT gradient during rest and exercise
      • Relieves angina by reducing myocardial oxygen demand
      • Increases diastolic filling
    • Calcium Channel Blockers (CCB): Used if contraindications for beta-blockers or intolerance to side effects.
    • Disopyramide (Class Ia antiarrhythmic): Added to beta-blockers/CCB, may prolong QT interval, has anticholinergic side effects, and can decrease ejection fraction.
    • Surgical Management: Septal myomectomy or Alcohol septal ablation (for refractory HCM with gradient > 50 mmHg despite medical therapy)

    Restrictive Cardiomyopathy (RCM)

    • Non-genetic: More common than HCM
    • Causes: Constrictive pericardium (chronic constrictive pericarditis), Restrictive CMP
    • Features: Stiff, non-compliant ventricles, increased pressure to fill, elevated right ventricular end-diastolic pressure (RVEDP), elevated right atrial pressure (RAP), normal-sized or small ventricles, normal ventricular thickness.
    • Consequences: Increased systemic blood pressure, atrial stretch and dilatation, increased risk of atrial fibrillation, systemic congestion (JVP, ascites, edema, hepatomegaly)
    • Systolic function: Almost normal with normal or slightly decreased dimensions, both atria massively enlarged, high end diastolic pressure (↑ RVEDP > ↑LVEDP)
    • First sign: Subtle exercise intolerance, severe diastolic dysfunction
    • Causes: Non-infiltrative (DM, Scleroderma), Infiltrative (Iron accumulation, Glycosphingolipid accumulation, Glycogen accumulation, Amyloidosis, Sarcoidosis, Loeffler's syndrome, Tropical endomyocardial fibrosis, Ascaris lumbricoides)

    Amyloidosis

    • Heart involved in all except: Secondary AA (no heart involvement, only kidney)
    • Classification: Primary AL (AL Amyloidosis), Secondary AA (AA Amyloidosis), Familial (ATTR)
      • Wild type: Senile systemic amyloidosis
      • Mutant: Familial amyloid polyneuropathy
    • Features: Localized amyloidosis, thickened ventricles, biatrial dilatation, interatrial septal thickening, heart failure (diastolic > systolic)

    Mitral Valve Prolapse (MVP)

    • Features: Leaflet redundancy, buckling into LA, flail, posterior leaflet most common, female > male (15-30 yrs), mostly asymptomatic.
    • Investigations: ECG (low voltage complexes), ECHO (pseudo-infarct pattern, left ventricular hypertrophy), Cardiac MRI (glittering, hyperrefractile myocardium d/t interatrial septal thickening)
    • Prognosis: Poor

    Arrhythmogenic Right Ventricular Dysplasia

    • Autosomal Recessive: Mutated protein: Desmosomes
    • Pathology: Fibrofatty replacement of RV myocardium, predisposed to arrhythmias and sudden cardiac death.

    Tachycardia-Induced Cardiomyopathy

    • Recurrent tachycardia leads to disruption of myocardial fibres and development of cardiomyopathy and atrial fibrillation.

    Takotsubo Cardiomyopathy

    • Synonyms: Broken heart syndrome, Transient apical ballooning, Stress-induced cardiomyopathy, Catecholamine-induced cardiomyopathy
    • Presentation: Middle-aged females, history of chronic stress, transient left ventricular dysfunction with ballooned apex due to sympathetic overactivity.

    Laplace's Law

    • Wall stress = Pressure x radius / 2 x thickness.
    • Thin-walled LV in MR requires less filling pressure.
    • Nitroglycerin (NTG) and sodium nitroprusside dilate arteries, decrease afterload, and increase blood flow to aorta (used in acute MR)
    • Strict blood pressure control is needed in MR to decrease afterload and increase blood flow to aorta.

    Mitral Regurgitation (MR): Symptoms and Complications

    • Symptoms:
      • Mild to moderate MR: Asymptomatic, palpitations, fatigue
      • Severe MR/Decompensated MR: Dyspnea, increased LV end diastolic pressure, increased LV end systolic pressure.
    • Normal EF in MR: 70%
    • Complications: Excess leaflet tissue buckling, annulus weakening/dilatation/calcification, chordae rupture, acute MR

    Auscultation

    • MVP: Associated with non-ejection click (NEC) and murmur.
    • Murmur Characteristics: High-pitched, whooping (cooing dove), mid-systolic crescendo-decrescendo, heard at apex.
    • Radiation: Anterior leaflet prolapse: Axilla, Posterior leaflet prolapse: Aortic area.
    • Dynamic Auscultation:
      • Valsalva, standing (↓preload/↓afterload): Click moves closer to S1 and murmur becomes longer.
      • Squatting, leg raising, standing to supine, isometric exercises (↑preload/↑afterload): Click moves away from S1 and murmur becomes shorter.

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    Description

    Explore critical concepts in cardiology focusing on Hypertrophic Cardiomyopathy (HCM) and Mitral Regurgitation. Understand diagnosis techniques like Echocardiograms and Doppler tests, along with medical management options and indications for implantable cardioverter defibrillators. This quiz also delves into the common heart diseases arising from Rheumatic Heart Disease.

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