Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

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Right Ventricular Outflow TractVentricular Tachycardia10% of all Ventricular Tachycardias, major differential in patientswith LBBB morphology with inferior axisBenign process in absence of structural heart diseaseElectrical defect that results in ventricular extra beats orsustained tachycardiaPresents with palpitationsProposed mechanism: cAMP triggered activity, resulted fromintense exerciseDiagnosis s of exclusion, and ARVC must be ruled outRVOT do not usually have EKG changes associated with ARVCLong term prognosis is good, and SCD is rare
History/EpidemiologyNewly recognized disease, clinical profile first publish in1982Autosomal dominant with penetrance in family members25-30% and 50% in Northern region of Italy.Exact incidence and prevalence is unknown, estimated1:5000– One group of patients diagnosed based on symptoms, EKGchanges and arrhythmias– Other group of clinically silent disease and asymptomatic untilthey manifest as sudden cardiac death– Pt that with CHF where dysplasia was not considered can bemistakenly diagnosed for idiopathic dilated cardiomyopathy
Page 1 and 2: Arrhythmogenic Right VentricularDys
Page 3 and 4: Learning ObjectivesCharacteristics
Page 5: Case PresentationChief Complaint: I
Page 9 and 10: Physical ExamVitals: Temp 96.7, BP
Page 11 and 12: Wide Complex Regular Tachycardia+ Q
Page 13 and 14: LabsTroponins: 0.120.21.3 2.20 1.8
Page 15 and 16: CCU CourseContinued on amiodarone g
Page 17 and 18: Cardiac MRIRevealed right ventricle
Page 19 and 20: Cardiac MRIShort axis viewdemonstra
Page 21 and 22: Final OutcomeEvaluated by EP, s/p I
Page 23 and 24: Mechanical Stress on impaired desmo
Page 25: Coronary Syndrome XPatients have an
Page 29 and 30: Diagnosis: Task Force CriteriaI. Gl
Page 31 and 32: Epsilon waves,which h arereproducib
Page 33 and 34: V. ArrhythmiasMinor:- LBBB type ven
Page 35 and 36: Evaluation of Risk Factors SCD in A
Page 37 and 38: Discussion:- ARVD typically present
Page 39 and 40: Genetic TestingFamily ScreeningFami
Page 41 and 42: ReferencesMcNally E, MacLeod H, Del

Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

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