title: Treatment of obstructive hypertrophic cardiomyopathy symptoms and gradient resistant to first-line therapy with β-blockade or verapamil.
abstract: BACKGROUND
  There is controversy about preferred methods to relieve obstruction in hypertrophic cardiomyopathy patients still symptomatic after β-blockade or verapamil.
  
  
  RESULTS
  Of 737 patients prospectively registered at our institution, 299 (41%) required further therapy for obstruction for limiting symptoms, rest gradient 61 ± 45, provoked gradient 115 ± 49 mm Hg, and followed up for 4.8 years. Disopyramide was added in 221 (74%) patients and pharmacological control of symptoms was achieved in 141 (64%) patients. Overall, 138 (46%) patients had surgical relief of obstruction (91% myectomy) and 6 (2%) alcohol septal ablation. At follow-up, resting gradients in the 299 patients had decreased from 61 ± 44 to 10 ± 25 mm Hg (P<0.0001); New York Heart Association class decreased from 2.7 ± 0.7 to 1.8 ± 0.5 (P<0.0001). Kaplan-Meier survival at 10 years in the 299 advanced-care patients was 88% and did not differ from nonobstructed patients (P=0.28). Only 1 patient had sudden death, a low annual rate of 0.06%/y. Kaplan-Meier survival at 10 years in the advanced-care patients did not differ from that expected in a matched cohort of the US population (P=0.90).
  
  
  CONCLUSIONS
  Patients with obstruction and symptoms resistant to initial pharmacological therapy with β-blockade or verapamil may realize meaningful symptom relief and low mortality through stepped management, adding disopyramide in appropriately selected patients, and when needed, by surgical myectomy.
authors.affiliation: Hypertrophic Cardiomyopathy Program, St. Luke's-Roosevelt Hospital Center, Columbia University, College of Physicians and Surgeons, New York, NY, USA. msherrid@chpnet.org
authors.forename: Mark V
authors.initials: MV
authors.lastname: Sherrid
authors.forename: Aneesha
authors.initials: A
authors.lastname: Shetty
authors.forename: Glenda
authors.initials: G
authors.lastname: Winson
authors.forename: Bette
authors.initials: B
authors.lastname: Kim
authors.forename: Dan
authors.initials: D
authors.lastname: Musat
authors.forename: Carlos L
authors.initials: CL
authors.lastname: Alviar
authors.forename: Peter
authors.initials: P
authors.lastname: Homel
authors.forename: Sandhya K
authors.initials: SK
authors.lastname: Balaram
authors.forename: Daniel G
authors.initials: DG
authors.lastname: Swistel
mesh_terms: D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D000368:Aged; D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D006348:Cardiac Surgical Procedures; D002312:Cardiomyopathy, Hypertrophic / Q000188:drug therapy* / Q000401:mortality / Q000601:surgery; D003131:Combined Modality Therapy; D004206:Disopyramide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D017211:Treatment Failure; D014700:Verapamil / Q000627:therapeutic use
chemical_list: D000319:Adrenergic beta-Antagonists; D000889:Anti-Arrhythmia Agents; D002121:Calcium Channel Blockers; D014700:Verapamil; D004206:Disopyramide
keywords: cardiac surgical procedures; cardiomyopathy, hypertrophic; disopyramide; drug therapy