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GUIDELINE REVIEW AUGUST 8, 2003 NOTE: An ACC Guideline on Hypertrophic Cardiomyopathy has now been published. SOURCES: HYPERTROPHIC CARDIOMYOPATHY - JAMA MARCH 2002 MANAGEMENT OF HYPERTROPHIC CARDIOMYOPATHY - NEJM MARCH, 1997 ACC/AHA/NASPE 2002 UPDATE - PACEMAKERS AND ANTIARRHYTHMIA DEVICES
1) HCM PREVALENCE A) 1/100 B) 1/500 C) 1/1000 D) 1/1,000,000
2) ___ OF THOSE WITH HCM HAVE RESTING OUTFLOW TRACT GRADIENTS A) 25 % B) 65 C) 75 D) > 90
3) INHERITANCE A) AUTOSOMAL RECESSIVE B) AUTO DOMINANT C) SEX-LINKED (Y) D) UNKNOWN, BUT APPEARS TO RUN IN FAMILIES
4) SCREENING OF FIRST DEGREE RELATIVES A) NOT NECESSARY B) BEGIN WITH PE, IF NO MURUR SUGGESTING OUTFLOW TRACT GRADIENT (REST OR PROVOCATION) - NO IMMEDIATE WORK-UP C) Hx,PE AND ECHO (ECG TOO INSENSITIVE) D) Hx,PE, ECG, ECHO ENCOURAGED
5) ECG A) MAJORITY OF HCM (75-95%) HAVE ABNORMAL ECGs B) MOST HAVE LBBB C) MOST HAVE RBBB D) YEARLY ECGs RECOMMENDED
6) PATTERNS OF LVH A) MOST HAVE SYMMETRIC LVH B) FREQUENTLY SEGMENTAL, ASYMMETRIC WITH THE ANTERIOR SEPTUM PREDOMINANT BUT STILL NO SINGLE “TPICAL” PATTERN C) APICAL/”JAPANESE”/SPADE HEART IS REALLY A DIFFERENT CLINICAL CONDITION FROM HCM D) ABSENCE OF ANY OF THE MORE COMMON ECHO FINDINGS BY ADULTHOOD - ESSENTIALLY RULES OUT HCM
7) BETA BLOCKERS A) USE PROPHYLACTICALLY IN GENOTYPE POSITIVE / PHENOTYPE NEGATIVE B) USE FOR GENOTYPE POSITIVE, EVEN IF ASYMPTOMATIC C) PHENOTYPE POSITIVE, SYMPTOMATIC IF OUTFLOW OBSTRUCTION ESTABLISHED D) ALL OF THE ABOVE - B BLOCKERS REDUCE MORTALITY E) NONE OF ABOVE - RESERVE FOR SYMPTOMATIC - WITH OR WITHOUT OBSTRUCTION - BUT “NO EVIDENCE . . . PROTECTS . . . FROM SUDDEN DEATH” (‘97)
8) ENDOCARDITIS PROPHYLAXIS A) NEVER NEEDED B) ONLY IF ACCOMPANIED BY MR C) OUTFLOW TRACT GRADIENT CONFERS HIGHER RISK TO PRIMARILY AORTIC VALVE D) OUTFLOW GRADIENT PRIMARILY INCREASES RISK TO MITRAL VALVE
9) WELL ESTABLISHED TO REDUCE MORTALITY IN HCM A) BETA BLOCKERS , DISOPYRAMIDE OR VERAPAMI; B) MYECTOMY C) DUAL CHAMBER PACING D) ETOH SEPTAL ABLATION E) AMIODARONE IN HIGH RISK Pts F) ICD IN HIGH RISK G) NONE OF ABOVE
10)WHICH ARE TRUE QUOTES A) “At present. . . icd appears to be the most effective treatment modality for the high-risk HCM patient” (jama-2002) B) “Sudden death prevention with the ICD is most strongly warranted for patients with prior cardiac arrest or ustained spontaneous ventricular tachycardia” (jama-2002) C) CLASS IIb FOR ICD PLACEMENT: “Familial or inherited conditions with a high risk of life- threatening ventricular tachyarrhythmias such as . . . hypertrophic cardiomyopathy.” (ACC/AHA/NAPSE 2002) D) ALL OF ABOVE E) NONE OF ABOVE
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