Managing Hypertrophic Cardiomyopathy with Imaging. Gisela C. Mueller University of Michigan Department of Radiology

Transcription

1 Managing Hypertrophic Cardiomyopathy with Imaging Gisela C. Mueller University of Michigan Department of Radiology

2 Disclosures Gadolinium contrast material for cardiac MRI

3 Acronyms Afib CAD Atrial fibrillation Coronary Artery Disease PVC RF Premature ventricular contraction Radiofrequency HCM Hypertrophic Cardiomyopathy SAM Systolic anterior motion of mitral valve LA Left atrium SAX Short axis view LV Left ventricle SCD Sudden cardiac death LVOT Left ventricular outflow tract Vfib Ventricular fibrillation NSVT Non-sustained ventricular tachycardia Vtach Ventricular tachycardia

4 Agenda I. HCM: Management and Diagnostic Issues II. III. IV. Prevention of SCD CMR: Late Gadolinium Enhancement Dynamic LVOT and Mid-cavity Obstruction V. Heart Failure and Atrial Fibrillation VI. VII. Early Markers of HCM Differential Diagnoses VIII. References

5 I. Management and Diagnostic Issues

6 Br Heart J : 1-8 Autosomal dominant LV Hypertrophy: unexplained = Absence of disease that would be capable of producing the magnitude of hypertrophy evident in a given patient Non dilated ventricular chamber

7 Prognosis Most common: normal life expectancy, and no major complications

8 Complication Prevention or Intervention Vtach/Vfib => SCD (most common 35 years of age) ICD for SCD prevention Heart failure (diastolic and/or systolic) Atrial fibrillation (associated with stroke) Heart failure drugs surgical myomectomy or alcohol septal ablation if heart failure 2 nd to LVOT obstruction Heart transplant Anticoagulation, heart rate/rhythm control RF ablation Surgical maze procedure Gersh et al: Circulation. 2011;124:e

9 Management Issues Counseling (lifestyle, genetic testing) SCD Risk Assessment and Prevention Diagnosis and treatment of dynamic LVOT and cavity obstruction Prevention, diagnosis, and treatment of heart Failure and atrial fibrillation Family counseling and screening of family members of HCM patients Monitoring of gene carriers for development of the HCM phenotype

10 HCM Management: Role of Imaging SCD Risk Stratification Diagnosis and monitoring of heart failure Evaluation of dynamic LVOT and cavity obstruction Pulmonary Vein Imaging for treatment of Afib Family Screening Monitoring of Gene Carriers for Development of Hypertrophy CMR, Echo Echo, CMR Echo, CMR CT, CMR Echo, CMR Echo, CMR

11 Diagnostic Issues Other Conditions with LV Hypertrophy Athlete s Heart Diagnostic Methods EKG, Echo, CMR, deconditioning Systemic Hypertension Blood pressure monitoring and treatment, EKG, Echo, CMR Aortic Valve Stenosis Amyloid Echo, CMR, CT, EKG, Echo, CMR, pathology Glycogen Storage Disease EKG, Echo, CMR, pathology Noncompaction Echo, CMR

12 II. Prevention of SCD

13 Secondary prevention: after the patient had an arrest Primary prevention: before the patient had an arrest

14 Personal History: Vfib; sustained Vtach; aborted SCD Yes ICD recommended No Risk Stratification for primary SCD prevention ICD reasonable indeterminate ICD not reasonable Assess potential risk modifiers

15 Risk Stratification for Primary Prevention of SCD ICD is reasonable if one of the following applies: maximal myocardial thickness 3 cm Family history of HCM related SCD in one or more firstdegree relative Personal history of one or more recent unexplained syncopal episodes ICD can be useful, particularly if other risk factors or risk modifiers present, if NSVT = Ventricular tachycardia (3 or more beats at 120 bpm) with a duration of less than 30 seconds) Systolic blood pressure rises < 20 mm Hg or decreases during exercise Gersh et al: Circulation. 2011;124:e

16 Maximal Myocardial Thickness ECHO Limited visualization: large patients, apex, anterior wall Most common site of maximal thickness: anterior septum parasternal long axis view Sometimes difficult delineation of RV border of septum CMR Entire myocardium visualized Most common sites of maximal thickness: intersection of anterior septum and anterior wall; inferior septum Short axis view Good delineation of RV border of septum

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18 Potential SCD Risk Modifiers Are considered if conventional risk stratification is indeterminate Delayed enhancement of myocardium on CMR Marked LVOT obstruction Double and compound mutations

19 CMR: Late Gadolinium Enhancement

20 Delayed Enhancement > 50% of patients with HCM Thought to represent replacement fibrosis Caveat: sparse pathologic data, limited to end-stage disease) More common in hypertrophied areas of myocardium and related to extent of hypertrophy Inversely related to LVEF Associated with NSVT and PVCs (Holter monitoring)

21 FWHM (dual threshold): Core tissue: 1.10g ( 1%) Grayzone: 74.62g (41%) 6 SD/ 4SD (dual threshold) Core tissue: 39.47g (21%) Grayzone: 21.12g (11%)

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