Tuesday, June 28, 2022
Monday, May 9, 2022
IV Sotalol - Registry study in children
85 patients
Age range 1 day - 36 yrs)
Treated with IV Sotalol (Average dose 1 mg/kg/dose; Range = 0.5 - 1.8 mg/kg/dose).
Infused
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.
Rivaroxaban (Xarelto, Janssen Pharmaceuticals, Inc)
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.
Mavacamten for HOCM
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.