Tuesday, June 28, 2022
Monday, May 9, 2022
Age range 1 day - 36 yrs)
Treated with IV Sotalol (Average dose 1 mg/kg/dose; Range = 0.5 - 1.8 mg/kg/dose).
over a median period of 60 min (Range 30 min - 5 hours).
Successful in 49%, Improved in 30% (Improved = HR decreased to the extent of allowing overdrive atrial pacing).
QTc prolonged >465 ms in 16% (Prolonged >500 ms in 4%).
Conlcusion: Safe as effective.
Successful or Improved in 79%.
Most common dose is 1 mg/kg/dose, infused over 60 min.
Link to reference: Mollory-walton LE at al. JAHA 2022.
FDA approved for 2 pediatric indications:
1) Venous thromboembolism in children - after at least 5 days of parenteral anticoagulant treatment.
2) Thromboprophylaxis in pediatric patients < 2 yrs of age after Fontan operation.
FDA approved Mavacamten (Camzyos from Bristol-Myers Squibb) for HOCM for children (May 2022).
Mavacamten is allosteric inhibitor of Myosin
Explorer-HCM trial data showed
(i) improved in peak VO2 and
(ii) stabilization or improvement in NYHA function class
compared to placebo.
Long-term extension study showed that benefits lasted at 1-year follow up. There was improvement in QoL reported by patients.
Valor-HCM - addition of Mavacamten to maximally-tolerated medical management, reduced the need for surgical or cath intervention for septal reduction.
Sunday, August 28, 2016
(UCLA, Jeruselam, Groningen - Netherlands & Toronto)
Good review articles covering most important lesions.
Part I - JACC 2016;68(4): 396-410.
Part II - JACC 2016;68(5): 502-16.
Friday, August 26, 2016
Divided into two surgical era: 1953-89 & 1990-2009.
Cardiac causes of mortality:
Sudden death (Aortic rupture, Arrhythmia, MI - majority were unexplained)
Death after reoperation
Non-cardiac causes of mortality:
Respiratory disease (esp. pneumonia associated with mental disability)
Neurologic and infectious diseases
Neoplasm (more common compared to general population)