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Case Rounds. Laura Miles March 3 2012. 6 month old female Several day hx of cough and increased work of breathing. What do you want to know?. Any investigations?. CXR. Further Testing…. EGC Evidence of LVH and possible RVH Inferior T wave inversion Echo Globally poor LV function - PowerPoint PPT Presentation

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  • Case RoundsLaura MilesMarch 3 2012

  • 6 month old femaleSeveral day hx of cough and increased work of breathing

  • What do you want to know?

  • Any investigations?

  • CXR

  • Further TestingEGCEvidence of LVH and possible RVHInferior T wave inversionEchoGlobally poor LV functionDilated and globular LVSF 11 % and EF 22%Normal coronaries

  • ???

  • CardiomyopathyClassificationPathophysiologyCausesManagement

  • ClassificationDilated Cardiomyopathy

    Hypertrophic Cardiomyopathy

    Restrictive Cardiomyopathy

  • Dilated CardiomyopathyDilation and impaired systolic function of left or both ventriclesMost common type of cardiomyopathy in childrenUp to 50%0.5 - 8 per 100 000 children

  • PresentationInitial signs and symptoms may be very vagueIrritabilityAnorexiaAbdominal painCough (from pulmonary congestion)

  • PresentationSigns and symptoms of CHFPoor systolic functionLimited cardiac outputVentricles enlarge and myocardium hypertrophies in an effort to maintain cardiac outputEnlargement of ventricles increases systolic wall tension in ventricle and increases myocardial oxygen consumption

  • PresentationDecreased cardiac output decreases renal blood flowEnhances renin-angiotensin systemFluid retention (necessary to maintain output)Stimulation of the sympathetic nervous system increased heart rateValvular regurg dilation of the valve annulusFurther myocardial oxygen demand

  • Physical ExamSigns of CHFCardiomegalyHepatomegalyTachycardiaTachypneaDecreased arterial pulse pressureNarrow pulse pressureGallopMurmur (MR)

  • Further SequelaeAbnormal myocytes conduction abnormalitiesNonspecific ST and T wave changesAtrial arrhythmiasVentricular arrhythmiasIntraventricular thrombus formation low flow state

  • A HUGE Differential!!Majority are idiopathic20-50% familialAutosomal dominant is most common5-10% are X linkedSmall number of autosomal recessiveMitochondrialViral myocarditis

  • A HUGE Differential!!NeuromuscularDuchennes Muscular DystrophyBeckers Muscular DystrophyLater onset and slower progressionMyotonic Dystrophy

  • Other less common causes:Infectious (excluding viral myocarditis)MetabolicStorage diseasesNutritional deficienciesEndocrineHypo/hyperthyroidismPheochromocytoma

  • Other less common causes:Coronary artery abnormalitiesCongenitalAbnormal placement of the right coronary artery between the great vesselsAcquiredKawasakisCardiotoxinsAnthracyclinesDoxorubicinDonorubicinCan occur anywhere from months after treatment completion to years later

  • Other less common causes:Connective tissue diseasesTachyarrhythmiasFamilial hypercholesterolemiaHIV associatedIron overloadSevere Hypertension

  • TreatmentLook for an identifiable cause and treat it!Majority of patients however have no specific etiology identified

  • TreatmentAfterload reduction to improve forward failureDiuretics as needed for symptoms of backward failureInotropic agents to improve symptomatic forward failure

  • TreatmentTreatment is supportiveEither the process resolvesThe heart improvesOr the heart is transplanted

  • Natural HxExtremely variableTransient dysfunction with complete recovery and minimal symptomsRapid progression to cardiogenic shock and deathAnything in betweenMost deaths occur within the first 6 months to 2 years after presentation

  • Hypertrophic CardiomyopathyLeft ventricular diseaseThickening of the ventricular wallNondilated

  • Hypertrophic CardiomyopathyCan be symmetric or asymmetricAsymmetric hypertrophy can obscure outflow tract (HOCM hypertrophic obstructive cardiomyopathy)Diagnosis is appropriate only after other causes of left ventricular hypertrophy are excluded

  • CausesCan be divided into:Primary (genetic mutation)SecondaryObstructive congenital heart diseaseInborn error of metabolism

  • CausesFamilialMost often autosomal dominantWide variability in penetranceOften denovoGenerally worse prognosis with early signs of disease

  • CausesTransientInfant of a diabetic motherCan develop with corticosteroid use in premsMetabolicGlycogen storage diseasePompe

  • PresentationUp to 50% of cases present asymptomaticallyEvaluation of a murmurScreening due to a family historyChest painExercise intoleranceArrhythmiaNear death event

  • TreatmentSupportiveImportant to maintain preload and avoid hypovolemiaNo role for afterload reduction or inotropic support (systolic function usually ok)Use of beta blockers, calcium channel blockers Prevention of sudden death debatableMay reduce symptoms

  • TreatmentIndications for ICD treatmentFamily historySevere hypertrophyHistory of ventricular arrhythmiaNear sudden death

  • Restrictive CardiomyopathyRestriction of flow into the ventriclesAccounts for less than 5% of cardiomyopathies in children

  • PathophysiologyAbnormal relaxation of ventricular myocardiumDecreased ventricular complianceAltered diastolic fillingIncreased ventricular end-diastolic pressureElevated atrial pressureEnlargement of atrium

  • CausesIdiopathicSystemic diseaseSclerodermaAmyloidosisSarcoidosisInborn errors of metabolismMucopolysaccharidosisHypereosinophiliaRadiationMalignancyNoncompaction of the left ventricular myocardium

  • TreatmentMedical treatment has limited successPay close attention to fluid balanceHigh atrial pressures required for adequate preloadIf atrial pressures too high, may lead to congestionDifficult to achieve adequate balanceUsually require early consideration for transplant

  • Questions??

  • References

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