Clinical Pathology Conference “62 year old woman with weakness and shortness of breath”
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Clinical Pathology Clinical Pathology ConferenceConference
“62 year old woman with weakness “62 year old woman with weakness and shortness of breath”and shortness of breath”
Heather Henderson, MDHeather Henderson, MDInternal Medicine Resident, PGY-3Internal Medicine Resident, PGY-3
Scott & White/Texas A&M HSCScott & White/Texas A&M HSC
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Case PresentationCase Presentation
CC:CC: “I am so weak and short of breath” “I am so weak and short of breath”HPI:HPI: 62 year old white woman with 62 year old white woman with multiple sclerosis multiple sclerosis 2-3 days of worsening lower extremity 2-3 days of worsening lower extremity swellingswellingIncreasing shortness of breathIncreasing shortness of breathGeneralized weaknessGeneralized weaknessNo prior history of heart failure symptomsNo prior history of heart failure symptoms
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Case PresentationCase Presentation
Past Medical History:Past Medical History:– Relapsing, remitting multiple sclerosisRelapsing, remitting multiple sclerosis– HTNHTN– History of herpes zosterHistory of herpes zoster– Raynaud’s phenomenonRaynaud’s phenomenon– S/P TAH/BSOS/P TAH/BSO
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Case PresentationCase Presentation
Allergies: NKDAAllergies: NKDAMedications:Medications:– Amantadine 200 mg po qamAmantadine 200 mg po qam– Avonex 30 mcg IM qweeklyAvonex 30 mcg IM qweekly– Baclofen 10 mg po qidBaclofen 10 mg po qid– Amitriptyline 50 mg po qdayAmitriptyline 50 mg po qday– Oxybutynin 5 mg po bidOxybutynin 5 mg po bid– Maxzide 75/50 po daily, but has not taken for last Maxzide 75/50 po daily, but has not taken for last
weekweek– Conjugated estrogen 0.625 mg po dailyConjugated estrogen 0.625 mg po daily
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Case PresentationCase Presentation
Social History:Social History:– Tobacco: NoneTobacco: None– ETOH: NoneETOH: None– Lives alone in TempleLives alone in Temple– Inactive, but performs ADL’sInactive, but performs ADL’s
Family History:Family History:– Prostate Ca – brotherProstate Ca – brother– HTN - brotherHTN - brother
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Case PresentationCase Presentation
Review of Systems:Review of Systems:– Weak with poor appetiteWeak with poor appetite– Dyspnea on exertionDyspnea on exertion with some wheezing lately with some wheezing lately– No fever or chillsNo fever or chills– Constipated for last 2-3 daysConstipated for last 2-3 days– Nauseated at times without emesisNauseated at times without emesis– No headaches or blurred visionNo headaches or blurred vision– No dysuriaNo dysuria– No recent problem with Raynaud’sNo recent problem with Raynaud’s
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Physical ExaminationPhysical ExaminationVS:151/60, 120, 20, 93% RA, 36.1, Wt: 91kgVS:151/60, 120, 20, 93% RA, 36.1, Wt: 91kg
Gen: wn/wd white woman appearing chronically ill, weak, and Gen: wn/wd white woman appearing chronically ill, weak, and tired, but no acute distresstired, but no acute distress
HEENT: normal except HEENT: normal except JVP =10 cm of waterJVP =10 cm of water
Chest: bilateral breath sounds decreased bilateral lower lobes, Chest: bilateral breath sounds decreased bilateral lower lobes, bibasilar cracklesbibasilar crackles
CV:CV: PMI slightly displaced inferolaterally; tachycardic at 120 PMI slightly displaced inferolaterally; tachycardic at 120 bpm; normal S1 and S2 with normal splitting, no S3 or S4 bpm; normal S1 and S2 with normal splitting, no S3 or S4 gallop; II/VI diastolic decrescendo murmur at LLSB with patient gallop; II/VI diastolic decrescendo murmur at LLSB with patient sitting up; pulses 2+/2+, bilateral throughoutsitting up; pulses 2+/2+, bilateral throughout
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Physical ExaminationPhysical ExaminationBack: Back: pitting sacral edemapitting sacral edema
Abd: soft, nd/nt; nabs, no abd bruit, no hepatosplenomegalyAbd: soft, nd/nt; nabs, no abd bruit, no hepatosplenomegaly
Ext: no clubbing/cyanosis; Ext: no clubbing/cyanosis; positive 0.5cm depth pitting positive 0.5cm depth pitting edemaedema of the lower extremities L>R to the level of the upper of the lower extremities L>R to the level of the upper tibiatibia
Skin: chronic venous stasis changes bilateral lower Skin: chronic venous stasis changes bilateral lower extremitiesextremities
Neuro: bulk and tone normal in UE’s, LE’s with decrease Neuro: bulk and tone normal in UE’s, LE’s with decrease motor strength with patient unable to lift left leg (known to motor strength with patient unable to lift left leg (known to be chronic); no new sensory deficitsbe chronic); no new sensory deficits
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Laboratory EvaluationLaboratory Evaluation
BNP=1940BNP=1940TnI=0.07, CK=35, CK-MB=1.8TnI=0.07, CK=35, CK-MB=1.8Na=140, K=3.7, Cr=1.0, BUN=6 Na=140, K=3.7, Cr=1.0, BUN=6 TSH=1.3TSH=1.3WBC=12.7, Hbg=13.9, Plat=391kWBC=12.7, Hbg=13.9, Plat=391kChol=178, TG=152, HDL=67, LDL=81Chol=178, TG=152, HDL=67, LDL=81
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ElectrocardiogramElectrocardiogram
NSR, rate = 84 NSR, rate = 84 Marked t-wave inversion in the anterior Marked t-wave inversion in the anterior leads and scooping of the ST segment in leads and scooping of the ST segment in the inferior leads, suggests ischemiathe inferior leads, suggests ischemiaNo voltage criteria for LVHNo voltage criteria for LVH
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Chest X-rayChest X-ray
CardiomegalyCardiomegalyPulmonary vascular congestionPulmonary vascular congestionSmall bilateral pleural effusionsSmall bilateral pleural effusions
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EchocardiogramEchocardiogram
LV enlargement with EF=25% LV enlargement with EF=25% (globally depressed) (globally depressed) Normal LV wall thickness Normal LV wall thickness Increased echo densities in the LV apex Increased echo densities in the LV apex suggesting possible thrombus suggesting possible thrombus LA enlargement with mild MR LA enlargement with mild MR Moderate to severe AI with normal aortic Moderate to severe AI with normal aortic root sizeroot size
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Transesophageal Transesophageal EchocardiogramEchocardiogram
AI was mild to moderateAI was mild to moderateProminent myocardial trabeculationsProminent myocardial trabeculationsNo LV thrombusNo LV thrombus
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Coronary AngiogramCoronary Angiogram
Normal coronary anatomyNormal coronary anatomyNo significant angiographic coronary No significant angiographic coronary artery diseaseartery disease
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Problem ListProblem List
WeaknessWeaknessShortness of breathShortness of breathMultiple SclerosisMultiple SclerosisCongestive Heart Congestive Heart FailureFailureDilated Dilated Cardiomyopathy Cardiomyopathy EF =25%EF =25%
TachycardiaTachycardiaAortic Insufficiency Aortic Insufficiency with normal aortic rootwith normal aortic rootProminent myocardial Prominent myocardial trabeculationstrabeculationsNo significant No significant Coronary Artery Coronary Artery DiseaseDisease
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Things are not as they appearThings are not as they appear
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ObjectivesObjectives
Define cardiomyopathyDefine cardiomyopathyDiscussion of causes of dilated cardiomyopathyDiscussion of causes of dilated cardiomyopathyDiagnostic evaluation of a dilated Diagnostic evaluation of a dilated cardiomyopathy cardiomyopathy Review of the literature for a correlation between Review of the literature for a correlation between multiple sclerosis and cardiomyopathymultiple sclerosis and cardiomyopathyA discussion of a rare cause of dilated A discussion of a rare cause of dilated cardiomyopathycardiomyopathy
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Cardiomyopathy DefinedCardiomyopathy Defined
A group of disorders in which the dominant A group of disorders in which the dominant feature is direct involvement of the heart feature is direct involvement of the heart muscle. muscle. (Not the result of pericardial, hypertensive, (Not the result of pericardial, hypertensive, congenital, or valvular diseases)congenital, or valvular diseases)
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Classification of Primary Classification of Primary CardiomyopathiesCardiomyopathies
Dilated cardiomyopathyDilated cardiomyopathy
Hypertrophic cardiomyopathyHypertrophic cardiomyopathy
Restrictive cardiomyopathyRestrictive cardiomyopathy
Arrhythmogenic right ventricular Arrhythmogenic right ventricular cardiomyopathycardiomyopathy
Unclassified cardiomyopathyUnclassified cardiomyopathy
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Specific CardiomyopathiesSpecific CardiomyopathiesIschemic cardiomyopathyIschemic cardiomyopathyValvular cardiomyopathyValvular cardiomyopathyHypertensive cardiomyopathyHypertensive cardiomyopathyInflammatory cardiomyopathyInflammatory cardiomyopathyMetabolic cardiomyopathyMetabolic cardiomyopathyGeneral-systemic disease cardiomyopathyGeneral-systemic disease cardiomyopathyMuscular dystrophiesMuscular dystrophiesNeuromuscular disordersNeuromuscular disordersSensitivity and toxic reactionsSensitivity and toxic reactionsPeripartal cardiomyopathyPeripartal cardiomyopathy
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Dilated CardiomyopathyDilated Cardiomyopathy
5-8 cases per 100,000 population/year5-8 cases per 100,000 population/year10,000 deaths each year in the US10,000 deaths each year in the US46,000 hospitalizations each year in the 46,000 hospitalizations each year in the United StatesUnited States¼ of the cases of congestive heart failure ¼ of the cases of congestive heart failure in the United Statesin the United States75 different diseases cause DCM75 different diseases cause DCM
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ObjectivesObjectives
Define cardiomyopathyDefine cardiomyopathyDiscussion of causes of dilated cardiomyopathyDiscussion of causes of dilated cardiomyopathyDiagnostic evaluation of a dilated Diagnostic evaluation of a dilated cardiomyopathy cardiomyopathy Review of the literature for a correlation between Review of the literature for a correlation between multiple sclerosis and cardiomyopathymultiple sclerosis and cardiomyopathyA discussion of a rare cause of dilated A discussion of a rare cause of dilated cardiomyopathycardiomyopathy
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Causes of Dilated Causes of Dilated CardiomyopathyCardiomyopathy
IschemiaIschemiaInfectious diseasesInfectious diseases– CoxsackievirusCoxsackievirus– CytomegalovirusCytomegalovirus– HIVHIV– VaricellaVaricella– HepatitisHepatitis– Epstein-BarrEpstein-Barr– EchovirusEchovirus– Streptococci-rheumatic Streptococci-rheumatic
feverfever
– Typhoid feverTyphoid fever– DiphtheriaDiphtheria– BrucellosisBrucellosis– PsittacosisPsittacosis– Rickettsial diseaseRickettsial disease– Lyme diseaseLyme disease– HistoplasmosisHistoplasmosis– CryptococcosisCryptococcosis– ToxoplasmosisToxoplasmosis– TrypanosomiasisTrypanosomiasis– ShistosomiasisShistosomiasis– TrichinosisTrichinosis
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Causes of Dilated Causes of Dilated CardiomyopathyCardiomyopathy
MedicationsMedications– Chemotherapeutic agentChemotherapeutic agent
AnthracyclinesAnthracyclinesCyclophosphamideCyclophosphamideTrastuzumabTrastuzumab
– Antiretroviral drugsAntiretroviral drugsZidovudineZidovudineDidanosineDidanosineZalcitabineZalcitabine
– PhenothiazinesPhenothiazines– ChloroquineChloroquine– ClozapineClozapine
ToxinsToxins– EthanolEthanol– CocaineCocaine– AmphetaminesAmphetamines– CobaltCobalt– LeadLead– MercuryMercury– Carbon MonoxideCarbon Monoxide– BerylliumBeryllium
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Causes of Dilated Causes of Dilated CardiomyopathyCardiomyopathy
Rheumatologic diseasesRheumatologic diseases– Systemic lupusSystemic lupus– SclerodermaScleroderma– Giant cell arteritisGiant cell arteritis
Endocrinologic disordersEndocrinologic disorders– Hypo/HyperthyroidismHypo/Hyperthyroidism– Growth hormone excess or Growth hormone excess or
deficiencydeficiency– PheochromocytomaPheochromocytoma– Diabetes MellitusDiabetes Mellitus– Cushing’s diseaseCushing’s disease
Neuromuscular diseasesNeuromuscular diseases– Duchenne’s Muscular Duchenne’s Muscular
DystrophyDystrophy– Myotonic dystrophyMyotonic dystrophy– Friedreich’s ataxiaFriedreich’s ataxia
Deposition DiseaseDeposition Disease– HemochromatosisHemochromatosis– AmyloidosisAmyloidosis
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Causes of Dilated Causes of Dilated CardiomyopathyCardiomyopathy
Electrolyte abnormalitiesElectrolyte abnormalities– HypocalcemiaHypocalcemia– HypophosphatemiaHypophosphatemia– UremiaUremia
Nutritional deficienciesNutritional deficiencies– ThiamineThiamine– SeleniumSelenium– CarnitineCarnitine
MiscellaneousMiscellaneous– Peripartum Peripartum
cardiomyopathycardiomyopathy– TachycardiaTachycardia– SarcoidosisSarcoidosis– FamilialFamilial– Sleep ApneaSleep Apnea– Autoimmune myocarditisAutoimmune myocarditis– RadiationRadiation– Calcium OverloadCalcium Overload– Oxygen free radical Oxygen free radical
damagedamage
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Frequency of Different CausesFrequency of Different CausesIdiopathic – 50 percentIdiopathic – 50 percentMyocarditis – 9 percentMyocarditis – 9 percentIschemic heart disease – 7 percentIschemic heart disease – 7 percentInfiltrative disease – 5 percentInfiltrative disease – 5 percentPeripartum cardiomyopathy – 4 percentPeripartum cardiomyopathy – 4 percentHIV infection – 4 percentHIV infection – 4 percentConnective tissue disease – 3 percentConnective tissue disease – 3 percentSubstance abuse – 3 percentSubstance abuse – 3 percentDoxorubicin – 1 percentDoxorubicin – 1 percentOther – 10 percentOther – 10 percent
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Differential DiagnosisDifferential Diagnosis
Dilated CardiomyopathyDilated Cardiomyopathy– Idiopathic dilated cardiomyopathyIdiopathic dilated cardiomyopathy– Valvular cardiomyopathyValvular cardiomyopathy– MedicationsMedications– Multiple SclerosisMultiple Sclerosis– Left ventricular noncompactionLeft ventricular noncompaction
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ObjectivesObjectives
Define cardiomyopathyDefine cardiomyopathyDiscussion of causes of dilated cardiomyopathyDiscussion of causes of dilated cardiomyopathyDiagnostic evaluation of a dilated Diagnostic evaluation of a dilated cardiomyopathy cardiomyopathy Review of the literature for a correlation between Review of the literature for a correlation between multiple sclerosis and cardiomyopathymultiple sclerosis and cardiomyopathyA discussion of a rare cause of dilated A discussion of a rare cause of dilated cardiomyopathycardiomyopathy
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Noninvasive Laboratory EvaluationNoninvasive Laboratory Evaluation
CaCaPhosPhosCreatinine, BUNCreatinine, BUNThyroid function studiesThyroid function studiesIron studiesIron studiesHIVHIV
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Invasive EvaluationInvasive Evaluation
Endomyocardial biopsyEndomyocardial biopsy– May be of benefit in certain situationsMay be of benefit in certain situations– Definite clinical benefitDefinite clinical benefit
Infiltrative disordersInfiltrative disordersAnthracycline toxicityAnthracycline toxicityCardiac transplant rejectionCardiac transplant rejection
– No definitive pattern histologically in DCMNo definitive pattern histologically in DCM– Estimated that a specific diagnosis is obtained by Estimated that a specific diagnosis is obtained by
biopsy in fewer than 10 percent of patientsbiopsy in fewer than 10 percent of patientsCardiac Catheterization and AngiographyCardiac Catheterization and Angiography– To determine ischemic diseaseTo determine ischemic disease
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ObjectivesObjectives
Define cardiomyopathyDefine cardiomyopathyDiscussion of causes of dilated cardiomyopathyDiscussion of causes of dilated cardiomyopathyDiagnostic evaluation of a dilated Diagnostic evaluation of a dilated cardiomyopathy cardiomyopathy Review of the literature for a correlation between Review of the literature for a correlation between multiple sclerosis and cardiomyopathymultiple sclerosis and cardiomyopathyA discussion of a rare cause of dilated A discussion of a rare cause of dilated cardiomyopathycardiomyopathy
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Multiple Sclerosis and Multiple Sclerosis and Cardiomyopathy: Is there a link?Cardiomyopathy: Is there a link?Subclinical left ventricular dysfunction in Subclinical left ventricular dysfunction in multiple sclerosis, per Akgulmultiple sclerosis, per Akgul41 patients with MS and 32 healthy 41 patients with MS and 32 healthy controlscontrolsLV ejection fraction was decreased in MS LV ejection fraction was decreased in MS patients compared with controls (p<0.05)patients compared with controls (p<0.05)
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MedicationsMedications
AmantadineAmantadine<1% CHF<1% CHF1%-10% orthostatic hypotension, 1%-10% orthostatic hypotension, peripheral edemaperipheral edemaUse in caution in patients with heart Use in caution in patients with heart failure, peripheral edema, or orthostatic failure, peripheral edema, or orthostatic hypotensionhypotensionTriamterene has been reported to increase Triamterene has been reported to increase the potential for toxicity with amantadinethe potential for toxicity with amantadine
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More MedicationsMore Medications
Interferon beta 1a – AvonexInterferon beta 1a – Avonex<1% cardiomyopathy, CHF<1% cardiomyopathy, CHF1%-10% chest pain, vasodilatation1%-10% chest pain, vasodilatationUse in caution in patients with pre-existing Use in caution in patients with pre-existing cardiovascular diseasecardiovascular diseaseInterferons increase the adverse effects of Interferons increase the adverse effects of ACE inhibitors, specifically the ACE inhibitors, specifically the development of granulocytopeniadevelopment of granulocytopenia
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More MedicationsMore Medications
AmitriptylineAmitriptyline– Rare cause of cardiomyopathyRare cause of cardiomyopathy– 2 case reports in the literature:2 case reports in the literature:
Case report: Cardiomyopathy developed during Case report: Cardiomyopathy developed during treatment with imipramine, recovered after treatment with imipramine, recovered after withdrawal, recurred 9 years later during treatment withdrawal, recurred 9 years later during treatment with amitriptylinewith amitriptylineCase report: Cardiomyopathy in a patient on Case report: Cardiomyopathy in a patient on amitriptyline and perphenazineamitriptyline and perphenazine
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More MedicationsMore Medications
MitoxantroneMitoxantroneCause of cardiomyopathyCause of cardiomyopathyDose related, approved cumulative dose is Dose related, approved cumulative dose is 140 mg/m2140 mg/m2Prospective study in Germany in 73 patients Prospective study in Germany in 73 patients showed no significant change in end-showed no significant change in end-diastolic diameter, end-systolic diameter, diastolic diameter, end-systolic diameter, fractional shortening, or EF in 23 month fractional shortening, or EF in 23 month follow up with mean dose of 114 mg/m2follow up with mean dose of 114 mg/m2
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ObjectivesObjectives
Define cardiomyopathyDefine cardiomyopathyDiscussion of causes of dilated cardiomyopathyDiscussion of causes of dilated cardiomyopathyDiagnostic evaluation of a dilated Diagnostic evaluation of a dilated cardiomyopathy cardiomyopathy Review of the literature for a correlation between Review of the literature for a correlation between multiple sclerosis and cardiomyopathymultiple sclerosis and cardiomyopathyA discussion of a rare cause of dilated A discussion of a rare cause of dilated cardiomyopathycardiomyopathy
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This is not a zebra!This is not a zebra!
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Isolated Left Ventricular Isolated Left Ventricular NoncompactionNoncompaction
Characteristics of Isolated Left Ventricular Characteristics of Isolated Left Ventricular ConcompactionConcompactionPrevalencePrevalenceGeneticsGeneticsNoncompaction associated with other diseasesNoncompaction associated with other diseasesClinical ManifestationsClinical ManifestationsImagingImagingManagmentManagment
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Isolated Left Ventricular Isolated Left Ventricular NoncompactionNoncompaction
Characterized by the following feautures:Characterized by the following feautures:– Altered myocardial wallAltered myocardial wall
– Prominent trabeculae and deep Prominent trabeculae and deep intertrabecular recessesintertrabecular recesses
– Thickened myocardium with two layers Thickened myocardium with two layers consisting of compacted and noncompacted consisting of compacted and noncompacted myocardiummyocardium
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Isolated Left Ventricular Isolated Left Ventricular NoncompactionNoncompaction
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Isolated Left Ventricular Isolated Left Ventricular NoncompactionNoncompaction
Also Characterized by the following Also Characterized by the following feautures:feautures:– Continuity between the left ventricular cavity Continuity between the left ventricular cavity
and the deep intratrabecular recesses, which and the deep intratrabecular recesses, which are filled with bloodare filled with blood
– No communication to epicardial coronariesNo communication to epicardial coronaries
– Decreased coronary flow reserveDecreased coronary flow reserve
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Prevalence of Isolated Left Prevalence of Isolated Left Ventricular NoncompactionVentricular Noncompaction
A rare form of cardiomyopathyA rare form of cardiomyopathyAll adult echocardiograms with global LV All adult echocardiograms with global LV dysfunction and an EF of <45% were dysfunction and an EF of <45% were reviewed for signs of LV compactionreviewed for signs of LV compaction3.7% prevalence for LVEF <45%3.7% prevalence for LVEF <45%0.26% for all patients0.26% for all patientsA review from Switzerland identified 34 A review from Switzerland identified 34 cases in 15 yearscases in 15 years
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Genetics of Isolated Left Ventricular Genetics of Isolated Left Ventricular NoncompactionNoncompaction
LVNC can be familialLVNC can be familialMutations have been found in the following Mutations have been found in the following genesgenes– G4.5G4.5– P121LP121L– Cypher/ZASPCypher/ZASP– Chromosome 11p15Chromosome 11p15
Family ScreeningFamily Screening
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Left Ventricular NoncompactionLeft Ventricular NoncompactionCongenital right or left ventricular outflow tract Congenital right or left ventricular outflow tract abnormalitiesabnormalities– Pulmonary atresia with intact ventricular septumPulmonary atresia with intact ventricular septum
Rarely seen with other congenital cardiac Rarely seen with other congenital cardiac disordersdisorders– Ebstein’s anomalyEbstein’s anomaly– Bicuspid aortic valveBicuspid aortic valve– Aorta-to-left ventricular tunnelAorta-to-left ventricular tunnel– Congenitally corrected transpositionCongenitally corrected transposition– Isomerism of the left atrial appendageIsomerism of the left atrial appendage– VSDVSD
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Left Ventricular NoncompactionLeft Ventricular Noncompaction
LVNC is associated with Neuromuscular LVNC is associated with Neuromuscular diseasesdiseases86 patients with LVNC underwent 86 patients with LVNC underwent neurological evaluationneurological evaluation– Metabolic myopathy(14), Leber’s hereditary Metabolic myopathy(14), Leber’s hereditary
optic neuropathy(3), myotonic(2), Becker(1), optic neuropathy(3), myotonic(2), Becker(1), Duchenne(1), NMD of unknown etiology in 32, Duchenne(1), NMD of unknown etiology in 32, normal in 13, 20 patients refusednormal in 13, 20 patients refused
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LV Noncompaction and NMDLV Noncompaction and NMD
Noncompaction and neuromuscular Noncompaction and neuromuscular disease in a nonageriandisease in a nonagerian– 94 year old male presented with a surprising 94 year old male presented with a surprising
find of left ventricle hypertrabeculationfind of left ventricle hypertrabeculation– Upon neurologic investigation, patient had a Upon neurologic investigation, patient had a
polyneuropathy and possible myopathypolyneuropathy and possible myopathy
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Clinical ManifestationsClinical Manifestations
Report from Switzerland on 34 patientsReport from Switzerland on 34 patients– At the time of diagnosis, clinical At the time of diagnosis, clinical
manifestations included:manifestations included:Dyspnea – 27 (79%)Dyspnea – 27 (79%)NYHA Class III or IV heart failure – 12 (35%)NYHA Class III or IV heart failure – 12 (35%)Chest Pain – 9 (26%)Chest Pain – 9 (26%)Chronic Atrial Fibrillation – 9 (26%)Chronic Atrial Fibrillation – 9 (26%)
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ECG in NoncompactionECG in Noncompaction
No characteristic changesNo characteristic changesUsually abnormalUsually abnormal
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Diagnosis of NoncompactionDiagnosis of Noncompaction
EchocardiographyEchocardiography
Cardiac MRICardiac MRI
Cardiac CT ScanCardiac CT Scan
Left VentriculographyLeft Ventriculography
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Echocardiographic Criteria for Echocardiographic Criteria for DiagnosisDiagnosis
Absence of coexisting cardiac abnormalitiesAbsence of coexisting cardiac abnormalities
Segmental thickening of the left ventricular myocardial Segmental thickening of the left ventricular myocardial wall consisting of two layers; a ratio of noncompacted to wall consisting of two layers; a ratio of noncompacted to compacted myocardium of >2:1 and end-systole with compacted myocardium of >2:1 and end-systole with thickening of the myocardial wallthickening of the myocardial wall
Predominant localization of the pathology in the apical Predominant localization of the pathology in the apical mid-lateral, and mid-inferior regions of the left ventriclemid-lateral, and mid-inferior regions of the left ventricle
Color doppler evidence of flow within the deep perfused Color doppler evidence of flow within the deep perfused intertrabecular recessesintertrabecular recesses
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EchocardiogramEchocardiogram
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EchocardiogramEchocardiogram
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MRI in NoncompactionMRI in Noncompaction
Noninvasive way to evaluate the presence Noninvasive way to evaluate the presence and extent of myocardial fibrosisand extent of myocardial fibrosis
Cardiac MRI shows trabecular delayed Cardiac MRI shows trabecular delayed hyperenhancement in left ventricle hyperenhancement in left ventricle noncompactionnoncompaction
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Cardiac MRICardiac MRI
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ManagementManagement
No specific therapyNo specific therapy
– Treat heart failure, arrhythmias, etcTreat heart failure, arrhythmias, etc
Holter monitoring once a yearHolter monitoring once a year
Heart transplantationHeart transplantation
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What is?What is?
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What is the answer?What is the answer?
Final DiagnosisFinal Diagnosis
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What is the answer?What is the answer?
Final DiagnosisFinal DiagnosisLeft Ventricular Noncompaction Left Ventricular Noncompaction associated with Multiple Sclerosisassociated with Multiple Sclerosis
vs.vs.
Idiopathic Dilated CardiomyopathyIdiopathic Dilated Cardiomyopathy
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What is the answer?What is the answer?
Final DiagnosisFinal DiagnosisLeft Ventricular Noncompaction Left Ventricular Noncompaction associated with Multiple Sclerosisassociated with Multiple Sclerosis
vs.vs.
Idiopathic Dilated CardiomyopathyIdiopathic Dilated Cardiomyopathy
Diagnostic Study/ProcedureDiagnostic Study/ProcedureCardiac MRICardiac MRI
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ReferencesReferencesUptodateUptodateKasper, Braunwald, Fauci, Hauser, Longo, Jameson. Harrison’s Principles of Kasper, Braunwald, Fauci, Hauser, Longo, Jameson. Harrison’s Principles of Internal Medicine. 16Internal Medicine. 16thth edition. 2005 edition. 2005Zipes, Libby, Bonow, Braunwald. Braunwald’s Heart Disease Textbook of Zipes, Libby, Bonow, Braunwald. Braunwald’s Heart Disease Textbook of Cardiovascular Medicine. 7Cardiovascular Medicine. 7thth edition. 2005 edition. 2005Kuhn H, Lawrenz T, Beer G. Indication for Myocardial Biopsy in myocarditis Kuhn H, Lawrenz T, Beer G. Indication for Myocardial Biopsy in myocarditis and dilated cardiomyopathy. Med Klin. 2005 Sep15;100(9):553-61.and dilated cardiomyopathy. Med Klin. 2005 Sep15;100(9):553-61.Alsaileek AA, Syed I, Seward JB, Julsrud P. Myocardial fibrosis of left Alsaileek AA, Syed I, Seward JB, Julsrud P. Myocardial fibrosis of left ventricle: Magnetic resonance imaging in noncompaction. J Magn Reson ventricle: Magnetic resonance imaging in noncompaction. J Magn Reson Imaging. 2008 Jan 24Imaging. 2008 Jan 24Bruder O, et al. Detection and characterization of left ventricular thrombi by Bruder O, et al. Detection and characterization of left ventricular thrombi by MRI compared to transthoracic echocardiography. Rofo. 2005 Mar;177(3):344-MRI compared to transthoracic echocardiography. Rofo. 2005 Mar;177(3):344-9.9.Sandhu R, et al. Prevalence and characteristics of left ventricular Sandhu R, et al. Prevalence and characteristics of left ventricular noncompaction in a community hospital cohort of patients with systolic noncompaction in a community hospital cohort of patients with systolic dysfunction. Echocardiography. 2008 Jan;25(1):8-12.dysfunction. Echocardiography. 2008 Jan;25(1):8-12.Zaragoza MV, et al. Noncompaction of the left ventricle: primary Zaragoza MV, et al. Noncompaction of the left ventricle: primary cardiomyopathy with an elusive genetic etiology. Curr Opin Pediatr. 2007 cardiomyopathy with an elusive genetic etiology. Curr Opin Pediatr. 2007 Dec;19(6):619-27.Dec;19(6):619-27.
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ReferencesReferencesFinsterer J, et al. Noncompaction and neuromuscular disease with positive Finsterer J, et al. Noncompaction and neuromuscular disease with positive troponin-T in a nonagenerian. Clin Cardiol. 2007 Oct;(10):527-8.troponin-T in a nonagenerian. Clin Cardiol. 2007 Oct;(10):527-8.Dodd JD, et al. Quantification of left ventricular noncompaction and Dodd JD, et al. Quantification of left ventricular noncompaction and trabecular delayed hyperenhancement with cardiac MRI: correlation with trabecular delayed hyperenhancement with cardiac MRI: correlation with clinical severity. AJR AM J Roentgenol. 2007 Oct;189(4):974-80.clinical severity. AJR AM J Roentgenol. 2007 Oct;189(4):974-80.Briec F, et al. Recurrence of dilated cardiomyopathy after re-introduction of Briec F, et al. Recurrence of dilated cardiomyopathy after re-introduction of a tricyclic antidepressant. Arch Mal Coeur Vaiss. 2006 Oct;99(10):933-5.a tricyclic antidepressant. Arch Mal Coeur Vaiss. 2006 Oct;99(10):933-5.Ansari A, et al. Drug induced toxic myocarditis. Tex Heart Inst J. Ansari A, et al. Drug induced toxic myocarditis. Tex Heart Inst J. 2003;30(1):76-9.2003;30(1):76-9.Akgul F, et al. Subclinical left ventricular dysfunction in multiple sclerosis. Akgul F, et al. Subclinical left ventricular dysfunction in multiple sclerosis. Acta Neurol Scand. 2006 Aug;114(2):114-8.Acta Neurol Scand. 2006 Aug;114(2):114-8.Cohen BA, Mikol DD. Mitoxantrone treatment of multiple sclerosis: safety Cohen BA, Mikol DD. Mitoxantrone treatment of multiple sclerosis: safety considerations. Neurology. 2004 Dec 28;63(12 Suppl 6):s28-32.considerations. Neurology. 2004 Dec 28;63(12 Suppl 6):s28-32.Zingler VC, et al. Assessment of potential cardiotoxic side effects of Zingler VC, et al. Assessment of potential cardiotoxic side effects of mitoxantrone in patients with multiple sclerosis. Eur Neurol. 2005;54(1):28-mitoxantrone in patients with multiple sclerosis. Eur Neurol. 2005;54(1):28-3333Stollberger C, Winkler-Dworak M, et al. Cardiology. 2007;107(4):374-9.Stollberger C, Winkler-Dworak M, et al. Cardiology. 2007;107(4):374-9.
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Thank youThank you
Dr. ScottDr. ScottDr. PruettDr. PruettDr. HagerDr. HagerDr. MockDr. MockDr. BrustDr. Brust
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Questions?Questions?