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Duchenne muscular dystrophy is associated with progressive cardiorespiratory failure, including left ventricular dysfunction.
Methods and Results:
Males with probable or definite diagnosis of Duchenne muscular dystrophy, diagnosed between 1 January, 1982 and 31 December, 2011, were identified from the Muscular Dystrophy Surveillance Tracking and Research Network database. Two non-mutually exclusive groups were created: patients with ≥2 echocardiograms and non-invasive positive pressure ventilation-compliant patients with ≥1 recorded ejection fraction. Quantitative left ventricular dysfunction was defined as an ejection fraction <55%. Qualitative dysfunction was defined as mild, moderate, or severe. Progression of quantitative left ventricular dysfunction was modelled as a continuous time-varying outcome. Change in qualitative left ventricle function was assessed by the percentage of patients within each category at each age. Forty-one percent (n = 403) had ≥2 ejection fractions containing 998 qualitative assessments with a mean age at first echo of 10.8 ± 4.6 years, with an average first ejection fraction of 63.1 ± 12.6%. Mean age at first echo with an ejection fraction <55 was 15.2 ± 3.9 years. Thirty-five percent (140/403) were non-invasive positive pressure ventilation-compliant and had ejection fraction information. The estimated rate of decline in ejection fraction from first ejection fraction was 1.6% per year and initiation of non-invasive positive pressure ventilation did not change this rate.
In our cohort, we observed that left ventricle function in patients with Duchenne muscular dystrophy declined over time, independent of non-invasive positive pressure ventilation use. Future studies are needed to examine the impact of respiratory support on cardiac function.
Children with congenital cardiac defects may have associated chromosomal anomalies, airway compromise, and/or pulmonary hypertension, which can pose challenges to adequate sedation, weaning from mechanical ventilation, and successful extubation. Propofol, with its unique properties, may be used as a bridge to extubation in certain cardiac populations.
Materials and methods
We retrospectively reviewed 0–17-year-old patients admitted to the Cardiac Intensive Care Unit between January, 2007 and September, 2008, who required mechanical ventilation and received a continuous infusion of propofol as a bridge to extubation. Medical charts were reviewed for demographics, associated comorbidities, as well as additional sedation medications and haemodynamic trends including vital signs and vasopressor support during the peri-infusion period. Successful extubation was defined as no re-intubation required for respiratory failure within 48 hours. Outcomes measured were successful extubation, evidence for propofol infusion syndrome, haemodynamic stability, and fluid and inotropic requirements.
We included 11 patients for a total of 12 episodes. Propofol dose ranged from 0.4 to 5.6 milligram per kilogram per hour with an average infusion duration of 7 hours. All patients were successfully extubated, and none demonstrated worsening metabolic acidosis suggestive of the propofol infusion syndrome. All patients remained haemodynamically stable during the infusion with average heart rates and blood pressures remaining within age-appropriate ranges. One patient received additional fluid but no increase in vasopressors was needed.
This study suggests that propofol infusions may allow for successful extubation in a certain population of children with congenital cardiac disease. Further studies are required to confirm whether propofol is an efficient and safe alternative in this setting.
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