A cohort study of childhood hypertrophic cardiomyopathy
Improved survival following high-dose beta-adrenoceptor antagonist treatment
Ingegerd Östman-Smith, MD, FRCPa,
G.öran Wettrell, MD* and
Tomas Riesenfeld, MD
a Pediatrics and Cardiology, John Radcliffe Hospital, Oxford, United Kingdom
* Division of Pediatric Cardiology, University Hospital, Lund, Sweden
Division of Pediatric Cardiology, Uppsala Academic Hospital, Uppsala, Sweden
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Figure 1 Box-and-whisker plot illustrating the beta-blocker dose in propranolol equivalents among patients receiving beta-blockers, comparing survivors with nonsurvivors. The box encloses the middle 50% of data values (i.e., 25th to 75th centile) with the median indicated by a horizontal line, and the whiskers extend to minimum and maximum values.
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Figure 2 Kaplan-Meier survival curves comparing the survival of the different treatment groups described in Table 2. Survival time analysis with each patient survival time (in years) indicated with a symbol at the time of death or when censored alive. (A) NST group (n = 20, filled circles); the monotherapy CDßB group (n = 15, open triangles); and the monotherapy HDßB group (n = 16, filled triangles). (B) Total CDßB group (n = 18) is shown with circles around patients with additional anti-arrhythmic therapy (n = 3); total HDßB group (n = 26) is shown with filled diamonds for patients with additional disopyramide therapy (n = 10). The NST group is shown for reference (filled circles). See Results for statistical comparisons using log-rank t test.
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Figure 3 Kaplan-Meier survival time analysis of total survival after diagnosis. Patients in the HDßB group (n = 26; filled triangles) are compared with all the other patients, "conventional management" (n = 40; filled squares), showing a significantly improved survival in the patients on HDßB (log-rank p = 0.0015).
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Figure 4 Comparison of treatment effects in HCM patients with or without Noonan’s syndrome. Kaplan-Meier analysis of total survival from diagnosis. (A) Noonan patients with conventional management (n = 17; filled squares) compared with Noonan patients from the total HDßB group (n = 8; filled triangles); log-rank p = 0.03. (B) Non-Noonan HCM patients with conventional management (n = 21; open squares) compared with non-Noonan HCM patients from the total HDßB group (n = 18; open triangles); log-rank p = 0.02. Two patients in the conventional management group with some dysmorphic features suggesting Noonan’s syndrome but of average or above average height, one survivor and one nonsurvivor, could not retrospectively be assigned as either Noonan or non-Noonan and were omitted.
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Figure 5 The 24-h ECG recordings from patients with HCM and varying degrees of beta-receptor blockade. (A) A 24-h ECG trace from 9-month-old infant with severe HCM before starting therapy. (B) Same infant on 5 mg/kg/day of propranolol; maximum heart rate is lower, but still up to 140 beats/min, and there is considerable variability. (C) Same infant, after serial increases to a dose of 20 mg propranolol/kg/day in order to abolish a dynamic outflow gradient, now shows good beta-blockade, with awake heart rates mostly between 90 and 110 beats/min. (D) A young adult, with HCM first diagnosed at age 13 years, now on 14 mg/kg of propranolol/day. Despite the very flat heart rate response (between 60 to 70 beats/min) she has no problems coping with full-time employment.
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