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Cardiac crises: Cardiac arrhythmias and cardiomyopathy during TANGO2 deficiency related metabolic crises

Christina Y Miyake¹, Erica J Lay², Cheyenne M Beach³

¹Department of Pediatrics, Division of Pediatric Cardiology, Texas Children's Hospital and Baylor College of Medicine, Houston, Texas; Department of Molecular Physiology and Biophysics, Baylor College of Medicine, Houston Texas.

|May 14, 2022

Abstract

Background:

TANGO2 deficiency disorder (TDD) is an autosomal recessive disease associated with metabolic crisis, lethal cardiac arrhythmias, and cardiomyopathy. Data regarding treatment, management, and outcomes of cardiac manifestations of TDD are lacking.

Objective:

The purpose of this study was to describe TDD-related cardiac crises.

Methods:

Retrospective multicenter chart review was made of TDD patients admitted with cardiac crises, defined as development of ventricular tachycardia (VT), cardiomyopathy, or cardiac arrest during metabolic crises.

Results:

Twenty-seven children were admitted for 43 cardiac crises (median age 6.4 years; interquartile range [IQR] 2.4-9.8 years) at 14 centers. During crisis, QTc prolongation occurred in all (median 547 ms; IQR 504-600 ms) and a type I Brugada pattern in 8 (26%). Arrhythmias included VT in 21 (78%), supraventricular tachycardia in 3 (11%), and heart block in 1 (4%). Nineteen patients (70%) developed cardiomyopathy, and 20 (74%) experienced a cardiac arrest. There were 10 deaths (37%), 6 related to arrhythmias. In 5 patients, recalcitrant VT occurred despite use of antiarrhythmic drugs. In 6 patients, arrhythmias were controlled after extracorporeal membrane oxygenation (ECMO) support; 5 of these patients survived. Among 10 patients who survived VT without ECMO, successful treatment included intravenous magnesium, isoproterenol, and atrial pacing in multiple cases and verapamil in 1 patient. Initiation of feeds seemed to decrease VT events.

Conclusion:

TDD-related cardiac crises are associated with a high risk of arrhythmias, cardiomyopathy, cardiac arrest, and death. Although further studies are needed, early recognition and appropriate treatment are critical. Acutely, intravenous magnesium, isoproterenol, atrial pacing, and ECMO as a last resort seem to be the best current treatment options, and early initiation of feeds may prevent VT events.

Insights

TANGO2 deficiency disorder (TDD) cardiac crises are life-threatening, causing arrhythmias, cardiomyopathy, and arrest. Prompt treatment with magnesium, isoproterenol, pacing, and ECMO, alongside early feeding, improves survival in TDD patients.

Area of Science:

Cardiology
Genetics
Metabolic Disorders

Background:

TANGO2 deficiency disorder (TDD) is a severe autosomal recessive condition.
Cardiac complications, including arrhythmias and cardiomyopathy, are significant features of TDD.
Limited data exists on the management and outcomes of cardiac issues in TDD.

Purpose of the Study:

To characterize the cardiac crises associated with TANGO2 deficiency disorder.
To describe the clinical presentation, treatment, and outcomes of TDD-related cardiac events.

Main Methods:

Retrospective multicenter chart review of TDD patients experiencing cardiac crises.
Cardiac crises defined as ventricular tachycardia, cardiomyopathy, or cardiac arrest during metabolic crises.
Data collected from 14 centers involving 27 children across 43 admissions.

Main Results:

All patients exhibited QTc prolongation during crises; 26% showed a Brugada pattern.
Ventricular tachycardia (78%) and cardiomyopathy (70%) were common, with 74% experiencing cardiac arrest.
Mortality was high (37%), with 6 deaths linked to arrhythmias; ECMO was crucial for some survivors.

Conclusions:

TDD cardiac crises pose a high risk of life-threatening arrhythmias, cardiomyopathy, and death.
Early recognition and intervention are critical for managing TDD cardiac events.
Intravenous magnesium, isoproterenol, atrial pacing, ECMO, and early feeding are key therapeutic strategies.

Read full analysis

Keywords:

Arrhythmia Brugada syndrome Cardiac arrest Cardiomyopathy Crisis Death Long QT syndrome TANGO2 Treatment Ventricular tachycardia

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