European Guidance for screening for Sudden Cardiac Death in athletes

 European Journal of Preventive Cardiology 2015

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.

Link to manufacturer information page and IFU.

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.

News from TCTMD dt. 4/29/22

Thoracic duct redirection to left atrium - Fontan

High-Risk Cardiac Disease in Pregnancy

Uri Elkayam, Sorel Goland, Petronella Pieper and Candice Silverside.
(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.

Late Causes of Death After Pediatric Cardiac Surgery

Alireza Raissadati et al. JACC 2016;68:487-98.
From Finland.

1953-2009
Divided into two surgical era: 1953-89 & 1990-2009.

Cardiac causes of mortality:
Heart failure
Pulmonary hypertension
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)
Accidents (alcohol-related)