Complexities of Management of Atypical Ventricular Fibrillation Storm in a Young Patient With TANGO2.

TANGO2 deficiency disorder, a rare autosomal recessive genetic disorder characterised by biallelic loss-of-function variants in the TANGO2 gene, was first described in 2016. This disorder involves the transport and Golgi organisation homologue, impacting Golgi membrane redistribution into the endoplasmic reticulum. Clinically, affected individuals exhibit a multiorgan phenotype, with prominent neurological manifestations such as developmental delay and regression.

COVID-19 in congenital heart disease (COaCHeD) study.

Background: COVID-19 has caused significant worldwide morbidity and mortality. Congenital heart disease (CHD) is likely to increase vulnerability and understanding the predictors of adverse outcomes is key to optimising care.

Objective: Ascertain the impact of COVID-19 on people with CHD and define risk factors for adverse outcomes.

When Adenosine Does Not Work: Apparent and Real Adenosine-Resistant Tachycardia.

Supraventricular tachycardia (SVT) is the most common arrhythmia in the pediatric population. Adenosine is widely accepted as the first-line pharmacological treatment for hemodynamically stable SVT, constituting a class I recommendation in the 2020 American Heart Association guidelines for pediatric life support (2020 American Heart Association Guidelines for cardiopulmonary resuscitation and emergency cardiovascular care). As most pediatric SVTs are dependent on the atrioventricular node (AVN) for their propagation, and adenosine acts primarily on the AVN, adenosine will frequently terminate the arrhythmia.

A zebrafish screen reveals Renin-angiotensin system inhibitors as neuroprotective via mitochondrial restoration in dopamine neurons.

Parkinson's disease (PD) is a common neurodegenerative disorder without effective disease-modifying therapeutics. Here, we establish a chemogenetic dopamine (DA) neuron ablation model in larval zebrafish with mitochondrial dysfunction and robustness suitable for high-content screening. We use this system to conduct an in vivo DA neuron imaging-based chemical screen and identify the Renin-Angiotensin-Aldosterone System (RAAS) inhibitors as significantly neuroprotective.