title: Severe symptoms in mid and apical hypertrophic cardiomyopathy.
abstract: BACKGROUND
  We analyzed the clinical and quantitative echocardiographic characteristics of patients with sub-basal hypertrophic cardiomyopathy (HCM) to define the characteristics of patients (pts) with severe symptoms.
  
  
  METHODS
  Of 444 pts in a referral-based HCM program, 22 (5%) had midventricular or apical HCM. Quality of life (QoL) questionnaire was administered as an independent confirmer of symptomatic state.
  
  
  RESULTS
  Ten pts were NYHA III and IV, and 12 pts were NYHA I and II; QoL scores (41 +/- 26 vs. 10 +/- 13, P = 0.001) confirmed a priori division of two groups based on NYHA classification. Pts with more severe symptoms were more likely female (70% vs. 25%, P = 0.001) with atrial fibrillation (40% vs. 0%, P = 0.02). They more frequently had midventricular HCM 60% versus 8% (P = 0.01) (mid-LV thickness 17 +/- 6 vs. 12 +/- 2 mm, P = 0.03) and had much smaller LV diastolic volumes 68 +/- 12 versus 102 +/- 22 ml (39 +/- 4 vs. 53 +/- 12 ml/m(2), P = 0.001). Septal E/E' was higher in the severely symptomatic pts (15 +/- 5 vs. 7 +/- 3, P = 0.001) indicating higher estimated LV filling pressure. Midobstruction with apical akinetic chamber was noted in 4/10 pts who developed refractory symptoms. Cardiac mortality was higher in the severely symptomatic patients, 4/10 who had midventricular HCM as compared to 0/12 in the mildly symptomatic apical HCM group (P = 0.03).
  
  
  CONCLUSIONS
  In subbasal HCM, pts with severe symptoms have midventricular hypertrophy, with encroachment of the LV cavity and consequent very small LV volumes that may be complicated by mid-LV obstruction. Pts with mid-LV hypertrophy are more symptomatic than those with apical HCM, are often refractory to therapy, and have higher mortality.
authors.affiliation: Division of Cardiology, St. Luke's-Roosevelt Hospital Center, College of Physicians and Surgeons, Columbia University, New York City, New York, USA.
authors.forename: Ajay
authors.initials: A
authors.lastname: Shah
authors.forename: Karl
authors.initials: K
authors.lastname: Duncan
authors.forename: Glenda
authors.initials: G
authors.lastname: Winson
authors.forename: Farooq A
authors.initials: FA
authors.lastname: Chaudhry
authors.forename: Mark V
authors.initials: MV
authors.lastname: Sherrid
mesh_terms: D000328:Adult; D002312:Cardiomyopathy, Hypertrophic / Q000150:complications* / Q000000981:diagnostic imaging*; D004452:Echocardiography / Q000379:methods*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging* / Q000209:etiology*