Hypertrophic Cardiomyopathy Flashcards¶
Anki-compatible flashcards for HCM board review.
Q: What is the echocardiographic definition of HCM? A: LV wall thickness ≥15 mm (adults) or Z-score ≥2 (children) not explained by loading conditions Tags: hcm, diagnosis, echo, high-yield
Q: What is the most common genetic cause of HCM? A: Mutations in sarcomere genes (most commonly MYH7 and MYBPC3, together ~70% of genetic cases) Tags: hcm, genetics, high-yield
Q: What is the inheritance pattern of HCM? A: Autosomal dominant Tags: hcm, genetics, inheritance
Q: What is the most important risk factor for SCD in pediatric HCM patients? A: Extreme LVH (massive hypertrophy) - more predictive in children than NSVT or syncope Tags: hcm, scd, risk, pediatric, high-yield
Q: What defines "massive" or extreme LVH in pediatric HCM? A: LV wall thickness Z-score ≥6 or >30 mm Tags: hcm, lvh, scd-risk
Q: Name the major risk factors for SCD in HCM (adult HCM Risk-SCD calculator factors) A: 1) Prior cardiac arrest/sustained VT, 2) Family history of SCD, 3) Unexplained syncope, 4) NSVT on Holter, 5) Maximal LV wall thickness, 6) LA size, 7) LVOT gradient Tags: hcm, scd, risk-factors
Q: What is HOCM and how is it distinguished from non-obstructive HCM? A: Hypertrophic Obstructive Cardiomyopathy - defined by LVOT gradient ≥30 mmHg at rest OR ≥50 mmHg with provocation Tags: hcm, hocm, lvot, gradient
Q: What maneuvers increase LVOT obstruction in HOCM? A: Valsalva maneuver, standing from squatting, exercise, dehydration, vasodilators, positive inotropes Tags: hcm, hocm, physiology
Q: What maneuvers decrease LVOT obstruction in HOCM? A: Squatting, passive leg elevation, handgrip, beta-blockers, phenylephrine Tags: hcm, hocm, physiology
Q: What is SAM and what causes it? A: Systolic Anterior Motion of the mitral valve; caused by Venturi effect from high-velocity flow in LVOT, pulling the anterior mitral leaflet toward the septum Tags: hcm, sam, mitral, physiology
Q: What are the first-line medications for symptomatic obstructive HCM? A: Beta-blockers (first choice) or non-dihydropyridine calcium channel blockers (verapamil, diltiazem) Tags: hcm, treatment, medications, high-yield
Q: What medications should be AVOIDED in obstructive HCM? A: Vasodilators, diuretics (if euvolemic), digoxin, and positive inotropes - all worsen obstruction Tags: hcm, treatment, avoid
Q: What is the role of disopyramide in HCM? A: Added to beta-blockers for refractory symptoms; negative inotrope that reduces LVOT gradient Tags: hcm, treatment, disopyramide
Q: What are the interventional options for drug-refractory obstructive HCM? A: Septal myectomy (surgical, gold standard) or alcohol septal ablation (transcatheter) Tags: hcm, treatment, myectomy, ablation
Q: What new medication was FDA-approved for obstructive HCM in 2022? A: Mavacamten (cardiac myosin inhibitor) Tags: hcm, treatment, mavacamten
Q: What ECG findings are commonly seen in HCM? A: LVH with repolarization abnormalities, deep narrow Q waves (septal), T wave inversions, possible WPW pattern (consider Pompe, Danon if present) Tags: hcm, ecg
Q: What sports participation recommendations apply to HCM patients? A: Historically disqualified from competitive sports; 2015+ guidelines allow shared decision-making with comprehensive evaluation Tags: hcm, sports, activity, high-yield
Q: What screening is recommended for first-degree relatives of HCM patients? A: Echo and ECG every 1-3 years during growth (children), then every 3-5 years (adults); genetic testing if proband's mutation known Tags: hcm, screening, family
Q: At what age should screening begin for children of HCM patients? A: Age 10-12 years (earlier if family history of early onset, symptoms, or competitive athlete) Tags: hcm, screening, pediatric
Q: What is the role of genetic testing in HCM? A: Recommended for ALL patients with clinical HCM; positive result allows cascade testing of relatives Tags: hcm, genetics, testing
Q: What percentage of HCM patients have identifiable genetic mutations? A: ~30-60% (higher in familial cases, lower in sporadic) Tags: hcm, genetics
Q: What syndrome combines HCM with WPW and intellectual disability? A: PRKAG2 syndrome (glycogen storage), Danon disease, or Pompe disease Tags: hcm, syndromic, wpw
Q: What is the prognosis of pediatric-onset HCM compared to adult-onset? A: Generally worse; earlier onset associated with more severe phenotype Tags: hcm, pediatric, prognosis
Q: A 15-year-old with HCM and ICD asks about sports. What do current guidelines recommend? A: Shared decision-making; may participate in some sports after comprehensive evaluation including imaging, genetic testing, and exercise testing. Intense physical activity increases arrhythmia risk. Tags: hcm, sports, guidelines, high-yield
Q: What are the indications for ICD in HCM? A: Primary prevention if high risk (calculate using HCM Risk-SCD or pediatric risk factors); Secondary prevention after cardiac arrest or sustained VT Tags: hcm, icd, scd
Q: A child with HCM has LV wall thickness Z-score of 8. What is the SCD risk category? A: HIGH RISK - extreme LVH (Z-score ≥6) is the most important pediatric risk factor Tags: hcm, scd, risk, pediatric, high-yield