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Basics

Basics

Definition

Inappropriate concentric hypertrophy of the ventricular free wall and/or the interventricular septum of the non-dilated left ventricle. The disease occurs independently of other cardiac or systemic disorders.

Pathophysiology

  • Diastolic dysfunction results from a thickened, non-compliant left ventricle.
  • High left ventricular filling pressure develops, causing left atrial enlargement.
  • Pulmonary venous hypertension causes pulmonary edema. Some cats develop biventricular failure (i.e., pulmonary edema, pleural effusion, small volume pericardial effusion without tamponade, and, rarely, ascites).
  • Stasis of blood in the large left atrium predisposes the patient to ATE.
  • Dynamic aortic outflow obstruction and systolic anterior mitral motion (SAM) with secondary mitral insufficiency may occur.

Systems Affected

  • Cardiovascular-CHF, ATE, and arrhythmias
  • Pulmonary-dyspnea if CHF develops
  • Renal/urologic-prerenal azotemia

Genetics

Some families of cats have been identified with a high prevalence of the disease, and the disease appears to be an autosomal dominant trait in Maine coon cats and ragdoll cats, due to a mutation in the MyBPC gene. The genetics have not been definitively determined in other breeds; however, the Maine coon and ragdoll mutations have not been identified in affected Sphynx, Norwegian forest cats, Bengals, Siberians, or British shorthair cats.

Incidence/Prevalence

Unknown, but relatively common. May be as high as 15% of the population.

Signalment

Species: Cat

Breed Predilections

Maine coon cats, ragdolls, Sphynx, British and American shorthairs, and Persians.

Mean Age and Range

5–7 years with reported ages of 3 months–17 years. Some breeds of cats including ragdolls and Sphynx may develop the disease at a younger age (average of 2 years of age). HCM is most often a disease of young to middle-aged cats; unexplained murmurs in geriatric cats are more likely associated with hyperthyroidism or hypertension.

Predominant Sex: Male

Signs

Historical Findings

  • Dyspnea.
  • Anorexia.
  • Exercise intolerance.
  • Vomiting.
  • Collapse.
  • Sudden death.
  • Coughing is uncommon in cats with cardiomyopathy and usually suggests pulmonary disease.

Physical Examination Findings

  • Gallop rhythm (S3 or S4).
  • Systolic murmur in many animals.
  • Apex heartbeat may be exaggerated.
  • Muffled heart sounds, lack of chest compliance, and dyspnea characterized by rapid shallow respirations may be associated with pleural effusion.
  • Dyspnea and crackles if pulmonary edema is present.
  • Weak femoral pulse.
  • Acute pelvic limb paralysis with cyanotic pads and nailbeds, cold limbs, and absence of femoral pulse in animals with ATE. Emboli rarely affect thoracic limbs.
  • Arrhythmia in some animals.
  • May have no clinical signs.

Causes

  • Usually unknown-multiple causes exist
  • MyBPC mutations in some cats with HCM

Possible Causes

  • Abnormalities of the contractile protein myosin or other sarcomeric proteins (e.g., troponin, myosin binding proteins, tropomyosin).
  • Abnormality affecting catecholamine-influenced excitation contraction coupling.
  • Abnormal myocardial calcium metabolism.
  • Collagen or other intercellular matrix abnormality.
  • Growth hormone excess.
  • Dynamic left ventricular outflow obstruction may contribute to secondary left ventricular hypertrophy.

Risk Factors

Offspring of animals with familial mutations of MyBPC

Diagnosis

Diagnosis

Differential Diagnosis

  • Other forms of cardiomyopathy
  • Hyperthyroidism
  • Aortic stenosis
  • Systemic hypertension
  • Acromegaly
  • Non-cardiac causes of pleural effusion

CBC/Biochemistry/Urinalysis

  • Results usually normal
  • Prerenal azotemia in some animals

Other Laboratory Tests

  • MyBPC assay. Mutation differs for Maine coon cats and ragdoll cats.
  • In cats > 6 years old, check thyroid hormone concentration. Hyperthyroidism causes myocardial hypertrophy that might be confused with HCM.
  • Serum BNP concentrations are higher in cats with HCM than in normal cats, and higher still in cats with symptomatic HCM. The positive predictive valve of this test to differentiate normal cats from those with asymptomatic HCM is unknown in the general population, and this test should therefore not be used to screen all asymptomatic cats. Serum BNP testing is useful, in identifying cats with a high suspicion of HCM from an asymptomatic population of cats with abnormal physical exam findings (e.g., murmur).

Imaging

Radiography

  • Dorsal ventral radiographs often reveal a valentine-appearing heart because of biatrial enlargement and a left ventricle that comes to a point.
  • Pulmonary edema, pleural effusion, or both in some animals.
  • Radiographs may be normal in asymptomatic cats.
  • The different forms of cardiomyopathy cannot be reliably differentiated by radiography.

Echocardiography

  • Hypertrophy of the interventricular septum or the left ventricular posterior wall (diastolic wall thickness >6 mm).
  • Hypertrophy may be symmetric (affecting IVS and posterior wall) or asymmetric (affecting IVS or posterior wall, but not both).
  • Hypertrophy of the papillary muscles.
  • Normal or high fractional shortening.
  • Normal or reduced left ventricular lumen.
  • Left atrial enlargement.
  • Systolic anterior motion of the mitral valve (some animals).
  • Left ventricular outflow obstruction (some animals). Specialized Doppler studies performed by experienced sonographers often reveal left ventricular relaxation abnormalities (e.g., mitral inflow E:A wave reversal).
  • Thrombus in the left atrium (rare).
  • Note: There is some overlap between normal cats (especially ketaminized and dehydrated) and cats with mild HCM. Correlate echo findings with physical findings. Presence of left atrial enlargement favors HCM.

Diagnostic Procedures

Electrocardiography

  • Sinus tachycardia (HR >240) is common with heart failure; however, some cats with severe heart failure and hypothermia are bradycardic.
  • Atrial and ventricular premature complexes seen more often in cats with cardiomyopathy, but are also occasionally seen in normal cats.
  • Atrial fibrillation is seen in some advanced cases.
  • A left axis deviation is often seen.
  • Cannot differentiate different forms of cardiomyopathy. May be normal.

Systemic Blood Pressure

  • Normotensive or hypotensive.
  • Evaluate blood pressure in all patients with myocardial hypertrophy to rule out systemic hypertension as the cause of hypertrophy.

Pathologic Findings

  • Non-dilated left ventricle with hypertrophy of intraventricular septum or left ventricular free wall.
  • Hypertrophy of papillary muscles.
  • Left atrial enlargement.
  • Mitral valve thickening.
  • Myocardial hypertrophy with disorganized alignment of myocytes (myofiber disarray).
  • Interstitial fibrosis.
  • Myocardial scarring.
  • Hypertrophy and luminal narrowing of intramural coronary arteries.

Treatment

Treatment

Appropriate Health Care

Cats with CHF should be hospitalized.

Nursing Care

  • Minimize stress
  • Oxygen if dyspneic
  • Warm environment if hypothermic

Activity

Restricted with CHF

Diet

Modest to moderate sodium restriction in animals with CHF

Chf Client Education

  • Many cats diagnosed while asymptomatic eventually develop CHF and may develop ATE and die suddenly.
  • If cat is receiving warfarin, dalteparin, lovenox, or a combination of clopidogrel and any of those medications, minimize potential for trauma and subsequent hemorrhage.

Medications

Medications

Drug(s) Of Choice

Furosemide

  • Dosage-1–2 mg/kg PO, IM, IV q8–24h.
  • Critically dyspneic animals often require high dosage (4 mg/kg IV). This dose can be repeated in 1 hour if the cat is still severely dyspneic. Indicated to treat pulmonary edema, pleural effusion, and ascites.
  • Cats are sensitive to furosemide and prone to dehydration, prerenal azotemia, and hypokalemia.
  • Once pulmonary edema resolves, taper to the lowest effective dose.

Pimobendan

  • Dosage-0.25–0.3 mg/kg PO q12h.
  • Appears to be useful in the management of congestive heart failure (e.g., pulmonary edema or pleural effusion) in cats with HCM, possibly by enhancing diastolic function. Pimobendan is not used in the management of asymptomatic HCM at this time.

ACE Inhibitors

  • Dosage-enalapril or benazepril 0.25–0.5 mg/kg PO q24h.
  • Indications in cats with HCM not well defined-authors currently use for CHF.

Beta-Blockers

  • Dosage-atenolol (6.25–12.5 mg/cat PO q12h).
  • Beneficial effects may include slowing of sinus rate, correcting atrial and ventricular arrhythmias, platelet inhibition.
  • More effective than diltiazem in controlling dynamic outflow tract obstruction.
  • Role in asymptomatic patients unresolved, but authors generally use if dynamic outflow obstruction and hypertrophy present.
  • Contraindicated in the presence of CHF.

Diltiazem

  • Dosage-7.5–15 mg/cat PO q8h or 10 mg/kg PO q24h (Cardizem CD) or 30 mg/cat q12h (Dilacor XR).
  • Beneficial effects may include slower sinus rate, resolution of supraventricular arrhythmias, improved diastolic relaxation, coronary and peripheral vasodilation, platelet inhibition.
  • May reduce hypertrophy and left atrial dimensions in some cats.
  • Role in asymptomatic patients unresolved.

Aspirin

  • Dosage-81 mg/cat q2–3 days if severe atrial enlargement.
  • Depresses platelet aggregation, hopefully minimizing the risk of thromboembolism.
  • Warn owners that thrombi can still develop despite aspirin administration. Aspirin appears to be not as effective as clopidogrel (1/4 of a 75-mg tablet PO q24h) in the prevention of ATE, at least in cats with a previous embolic episode.

Nitroglycerin Ointment

  • Dosage-one-fourth inch/cat topically applied q6–8h or 2.5 mg/24-hour patch.
  • Often used in the acute stabilization of cats with severe pulmonary edema or pleural effusion.
  • When used intermittently, it may be useful for long-term management of refractory cases.

Contraindications

Avoid beta-blockers in cats with emboli; these agents cause peripheral vasoconstriction. If beta-blockers must be used in this setting for arrhythmia control, choose a beta-1 selective blocker such as atenolol.

Precautions

Use ACE inhibitors cautiously in azotemic animals.

Alternative Drug(s)

Spironolactone

  • Dosage-1 mg/kg q12–24h.
  • Used in conjunction with furosemide in cats with CHF.
  • May cause facial pruritis.

Warfarin and Low Molecular Weight Heparin

  • Used sometimes in cats at high risk for thromboembolism.
  • See chapter, Aortic Thromboembolism.

Clopidogrel

Dosage-18.75 mg/cat/day. Platelet function inhibitor, superior to aspirin in cats with previous ATE.

Beta-Blocker Plus Diltiazem

  • Cats that remain tachycardic on a single agent can be treated cautiously with a combination of a beta-blocker and diltiazem.
  • Monitor for bradycardia and hypotension.

Follow-Up

Follow-Up

Patient Monitoring

  • Observe closely for dyspnea, lethargy, weakness, anorexia, and painful posterior paralysis or paresis.
  • If treating with warfarin, monitor prothrombin time.
  • If treating with an ACE inhibitor or spironolactone, monitor renal function and electrolytes.
  • Repeat echocardiogram in 6 months to assess efficacy of treatment for hypertrophy. If a beta-blocker or diltiazem was prescribed in an asymptomatic animal and there is evidence of progressive hypertrophy/left atrial enlargement, consider switching to another class of medications (or adding ACEI) and recheck 4–6 months later.
  • Echocardiographic evaluations that reveal LA diameters >2 cm or loss of LV systolic function should prompt more aggressive prophylaxis against ATE (e.g., clopidogrel with low molecular weight heparin).

Prevention/Avoidance

Avoid stressful situations that might precipitate CHF.

Possible Complications

  • Heart failure
  • ATE and paralysis
  • Cardiac arrhythmias/sudden death

Expected Course and Prognosis

  • Animals homozygous for MyBPC mutations more likely to develop severe HCM and at earlier age than heterozygous animals.
  • Prognosis varies considerably, probably because there are multiple causes. In one study of cats with HCM living at least 24 hours following presentation:
    • Asymptomatic cats: median survival 563 days (range 2–3,778 days)
    • Cats with syncope: median survival 654 days (range 28–1,505 days)
    • Cats with CHF: median survival 563 days (range 2–4,418 days).
    • Cats with ATE: median survival 184 days (range 2–2,278 days).
    • Older age and larger left atria predicted shorter survival.

Miscellaneous

Miscellaneous

Associated Conditions

Aortic thromboembolism

Pregnancy/Fertility/Breeding

  • High risk of complications
  • Avoid aspirin

Abbreviations

  • ACE = angiotensin converting enzyme
  • ATE = aortic thromboembolism
  • HCM = hypertrophic cardiomyopathy
  • IVS = interventricular septum
  • MyBPC = myosin binding protein C

Authors Francis W.K. Smith, Jr., Bruce W. Keene, and Kathryn M. Meurs

Client Education Handout Available Online