New England Journal of Medicine
01/05/2023
Vol 388; Pages 71-78
The journal feature illustrates clinical problem-solving through the case of a 68-year-old female who was brought to the hospital in the statute of an emergency. The female presented with initial signs like lightheadedness, dyspnea, and palpitations. However, this was not the first time the patient had presented with these symptoms, as she was later found to have a 2 years history of transient lightheadedness and shortness of breath. Moreover, she also had experienced hypertension and obstructive sleep apnea (temporary relaxation of muscles supporting the tissues in your throat). Her record showed her father to have also been diagnosed with coronary artery disease and an aortic aneurysm. The history and her symptoms were primarily indicative of three conditions which included:
- Pulmonary Embolism
- Myocardial Ischemia
- Arrhythmia
However, the doctors then conducted a thorough physical examination to reach a more definitive diagnosis. She was found to have a regular heartbeat, non-elevated jugular venous pressure, clear lungs, and a stable wide complex tachycardia – previously found in the Emergency department. There can be two types of factors causing Ventricular Tachycardia which include:
- Chronic Ischemia
- Non Ischemic Factors
- Acute Myocarditis
- Genetic Cardiomyopathic Syndromes
- Arrhythmogenic Syndromes
- Drug intakes
- Imbalance of Electrolytes
The patients underwent a coronary angiogram and echocardiogram to investigate which of these factors may have been responsible for ventricular Tachycardia. While the coronary angiogram was negative for obstructive coronary artery disease, the echocardiogram showed a preserved ejection fraction (when the ejection fraction is greater than or equal to 50%). Finally, the patient was given Verapamil and was diagnosed with idiopathic fascicular ventricular Tachycardia, also referred to as Verapamil-sensitive Ventricular Tachycardia.
However, 10 days later, the patient presented to the emergency department again with similar symptoms. She was given electrical cardioversion with amiodarone and Lidocaine, which was successful. Electrophysiological results showed ventricular Tachycardia to be present. She was then transferred to a cardiac intensive care unit and was diagnosed to be affected by a Ventricular Tachycardia Storm. This is when the patient experiences three or more episodes of sustained ventricular Tachycardia, either within 24 hours or recurrent episodes, with an interval of 5 minutes. The patient then underwent a basic metabolic panel and echocardiogram. She was found to have high troponin levels, an ejection fraction of 55%, and ventricular systolic pressure of 40 mm Hg.
A cardiac MRI was also obtained, which showed large amounts of Subendocardial Late Gadolinium Enhancement in the left ventricle and diffused myocardial edema. This can be indicative of the following:
- Ischemic dilated cardiomyopathy
- Sarcoidosis,
- Myocarditis
- Chagas disease
- Idiopathic Dilated Cardiomyopathy
The patient underwent a Cardiac FDG PET to reach a definitive diagnosis, which showed a perfusion defect and intense FDG uptake. This was suggestive of Sarcoidosis a therapy of 60 mg prednisolone was started.
But the patient presented to the emergency department 4 months later with an ICD shock. Further testing showed an even increased FDG uptake and perfusion metabolism mismatch. An endomyocardial biopsy was also performed, which showed multinucleated giant cells – which is a finding consistent with giant cell myocarditis. She was then discharged with Azathioprine, Cyclosporine, and Prednisolone, and the patient was instructed to continue the three immunosuppressives indefinitely.