TY - JOUR
T1 - Ventricular arrhythmias and acute left ventricular dysfunction as a primary and life-threatening manifestation of a mitochondrial crisis
T2 - A novel management strategy
AU - Vahdat, Khashayar K.
AU - Ilias-Basha, Haseeb
AU - Tung, Poyee P.
AU - Memon, Nada B.
AU - Hall, Andrea C.
AU - Koenig, Mary K.
AU - Meyers, Deborah E.
PY - 2012/8
Y1 - 2012/8
N2 - Mitochondrial disorders are genetic diseases that result in a deficiency of energy metabolism (ATP production). A " mitochondrial crisis" can occur in the setting of infection, dehydration, or physiologic stress. The hallmark of a mitochondrial crisis is failure of multiple individual organ systems. The mortality of mitochondrial crisis is high and therapy is supportive but involves a specific strategy of hydration with dextrose-containing IV fluids, avoidance of many medications known to worsen mitochondrial function, and limitations of oxygenation as this can promote free radical production. We report a case of a patient with known mitochondrial disease that presented with a mitochondrial crisis with prominent and life-threatening cardiac manifestations including long QT, ventricular arrhythmias, and acute left ventricular systolic dysfunction in addition to rhabdomyolysis, lactic acidosis, and an acute kidney injury. This patient was managed successfully with a specifically tailored supportive strategy, a high-dose metabolic cocktail, permissive hypoxia, and low-protein diet. At 10 weeks post discharge all electrocardiographic abnormalities resolved and ventricular recovery has been observed. Given the increased survival of this population of patients into adulthood it is important that these adjunctive therapeutic strategies require consideration by clinicians treating this group of patients.
AB - Mitochondrial disorders are genetic diseases that result in a deficiency of energy metabolism (ATP production). A " mitochondrial crisis" can occur in the setting of infection, dehydration, or physiologic stress. The hallmark of a mitochondrial crisis is failure of multiple individual organ systems. The mortality of mitochondrial crisis is high and therapy is supportive but involves a specific strategy of hydration with dextrose-containing IV fluids, avoidance of many medications known to worsen mitochondrial function, and limitations of oxygenation as this can promote free radical production. We report a case of a patient with known mitochondrial disease that presented with a mitochondrial crisis with prominent and life-threatening cardiac manifestations including long QT, ventricular arrhythmias, and acute left ventricular systolic dysfunction in addition to rhabdomyolysis, lactic acidosis, and an acute kidney injury. This patient was managed successfully with a specifically tailored supportive strategy, a high-dose metabolic cocktail, permissive hypoxia, and low-protein diet. At 10 weeks post discharge all electrocardiographic abnormalities resolved and ventricular recovery has been observed. Given the increased survival of this population of patients into adulthood it is important that these adjunctive therapeutic strategies require consideration by clinicians treating this group of patients.
KW - Arrhythmia
KW - Cardiomyopathy
KW - Clinical management
KW - Mitochondrial crisis
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U2 - 10.1016/j.jccase.2012.04.002
DO - 10.1016/j.jccase.2012.04.002
M3 - Article
AN - SCOPUS:84864759864
SN - 1878-5409
VL - 6
SP - e35-e38
JO - Journal of Cardiology Cases
JF - Journal of Cardiology Cases
IS - 2
ER -