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it has a wide range of clinical presentations that range from asymptomatic electrocardiogram changes to sudden cardiac death .
ventricular aneurysms and ventricular tachycardia are seen late in the disease , and are rarely the presenting manifestation of the disease .
we report a 35-year - old patient who presented with sustained ventricular tachycardia and st - elevation on electrocardiogram .
cardiac mri was done and showed late gadolinium enhancement in the septum , apex and lateral wall .
the patient was diagnosed with cardiac sarcoidosis and treated with immune suppression and antiarrhythmic agent .
our case highlights the importance of suspecting cardiac sarcoidosis in young patients presenting with electrocardiogram changes , and present an atypical presentation of this disease .
studies have suggested that it might be an immunological response to unidentified antigenic triggers . the highest incidence is reported in northern european and african individuals , especially in women . in the united states ,
the annual incidence is estimated to be 10.9 per 100,000 in whites and 35.5 per 100,000 in african americans .
cardiac sarcoidosis ( cs ) can precede , follow , or occur simultaneously with other organs involvement .
autopsy studies estimate the prevalence of cardiac involvement to be at least 25% of the patients with sarcoidosis .
imaging studies with late gadolinium enhancement - cardiac magnetic resonance ( lge - cmr ) show asymptomatic cardiac involvement in 19 - 25.9% of patients with extra - cardiac sarcoidosis .
cs alters the prognosis for those who are affected , and accounts for as many as 13 - 25% of deaths from sarcoidosis .
a 35-year - old african american female , with a history of pulmonary sarcoidosis , presented to the emergency department with a 1-hour history of palpitations and light headedness .
no chest pain , shortness of breath , loss of consciousness , other significant symptoms , or similar previous episodes were reported .
no history of smoking , drinking , or drug abuse was reported . on examination , she was alert and oriented .
her vital signs showed a heart rate of 224 beats / minute , blood pressure of 138/92 mmhg , respiratory rate of 18 , temperature of 98.2 , and oxygen saturation of 99% on room air .
12-lead electrocardiogram ( ekg ) showed a wide - complex tachycardia , ventricular rate of 225/minute , extreme axis , right - bundle pattern in lead v1 , and q - waves in the lateral leads .
subsequent ekg showed normal sinus rhythm and st - segment elevations in the lateral leads .
left ventriculography showed global left ventricular hypokinesis with an estimated ejection fraction ( ef ) of 35% .
additionally , an anteroapical segment aneurysm was identified [ figure 1 ] . left ventriculography , right - anterior - oblique cranial view :
anteroapical segment aneurysm 2d - echocardiogram showed dilated left ventricle with moderate concentric hypertrophy and an echogenicity involving the basal to mid septum , consistent with an infiltrative disease [ figure 2 ] . due to the high likelihood of an underlying cs , a cmr with gadolinium enhancement was done and showed thickening of the left ventricular myocardium , seen particularly in the septum , and decreased ef of 40% .
delayed images showed diffuse , patchy , transmural and subepicardial gadolinium enhancement involving the septum , apex and lateral wall [ figures 3 and 4 ] .
2d - echocardiogram , parasternal long axis view : dilated left ventricle , moderate concentric hypertrophy .
echogenicity in the basal to mid septum lge - cmr , two - chamber view : diffuse patchy transmural as well as subepicardial late gadolinium enhancement lge - cmr , shortaxis view : diffuse patchy transmural as well as subepicardial late gadolinium enhancement cmr , two - chamber view , t2 : aneurysm in anteroapical ventricular wall with evidence of dyskinesia the patient was diagnosed with cardiac sarcoidosis complicated by ventricular aneurysm formation and ventricular tachycardia .
she was switched to oral amiodarone and underwent aicd implantation for secondary prevention of life - threatening arrhythmias .
the clinical presentations of cs are wide and variable ; they can range from asymptomatic electrocardiographic changes to sudden cardiac death .
conduction abnormalities occurin up to 62% of patients during the disease course and usually present as complete heart block .
they usually result from direct granulomatous involvement of the myocardium and might persist despite medical treatment . unlike the conduction abnormalities that develop in the inflammatory phase of the disease , ventricular arrhythmias
are not closely related to the disease activity , and frequently develop in the advanced stages .
they are usually the result of atrial dilatation due to left ventricular dysfunction , cor - pulmonale , or direct granulomatous involvement of the atria .
other findings on the electrocardiogram include pseudoinfarction patterns that mimic myocardial infarction and non - specific repolarization changes .
ventricular aneurysms result from direct myocardial involvement , which unlike ischemic aneurysms ; do not follow a coronary artery distribution .
mitral valve regurgitation is the most common encountered valvular lesion in cs , and is usually the result of papillary muscles dysfunction or direct granulomatous involvement of the valve leaflets .
sudden cardiac death is the most feared cardiac manifestation of cs and usually results from either ventricular arrhythmias or complete heart block .
it is the most common cause of death in cs and responsible for 24%-64% of all deaths in the usa .
progressive heart failure is the second - most common cause of death and accounts for up to 25% of all the deaths in cs .
the most important independent predictors of mortality in cs are the new york heart association ( nyha ) functional class , left ventricular end diastolic diameter , and presence of sustained vt .
furthermore , patients presenting with ventricular aneurysm have been found to have a worse prognosis .
heart rhythm society expert consensus recommendations in july , 2014 , identify cs by two pathways .
the first requires the presence of non - caseating granulomas on histological examination of myocardial tissues with no other alternative cause .
the second requires the presence of a histological diagnosis of extra - cardiac sarcoidosis , excluding other causes of cardiac manifestations , and one of the followings : steroid and/or immunosuppressant responsive cardiomyopathy or heart block , unexplained reduced lvef ( < 40% ) , unexplained sustained ( spontaneous or induced ) vt , mobitz type ii second - degree heart block or third - degree heart block , patchy uptake on dedicated cardiac 18f - flurodeoxyglucose - positron emission tomography ( fdg - pet ) , lge - cmr , or a positive gallium uptake .
cmr has an excellent sensitivity and specificity for the diagnosis of cs despite the lack of pathognomonic patterns .
setting , inflammation and edema are demonstrated by myocardial thickening and increased t2 signal with a subepicardial or midmyocardial enhancement patterns . in the chronic setting , granulomas cause focal areas of myocardial thinning resulting from scar formation .
this is mostly seen as delayed contrast enhancement in areas of granuloma and scar formation that is frequently patchy and nodular .
this is atypical for myocardial infarction , which typically shows preferential hyper - enhancement of the subendocardial layer .
similar to cmr , no pathognomonic findings for cs are seen on fdg - pet .
cs is most typically associated with focal fdg uptake either in isolation or on a background of mild diffuse uptake with or without resting perfusion defects and wall motion abnormalities .
lung or lymph node biopsies are usually done first in patients with extra - cardiac sarcoidosis due to their higher yield and lower risks . however , in cases of isolated cs or negative extra - cardiac biopsies , endomyocardial biopsy may be required to confirm the diagnosis .
non - caseating granulomas are found in less than 25% of patients due to the focal nature of the disease .
results can be enhanced by utilizing electrophysiological , pet , or cmr image - guided biopsies .
corticosteroids might halt the progression of cardiac disease and improve survival by slowing the progression of inflammation and fibrosis .
superior results might be achieved if they are initiated early in the course of the disease .
they aremost beneficial in cs patients with mobitz - ii or third - degree heart blocks , frequent ventricular ectopy , non - sustained vt or sustained ventricular arrhythmias in the presence of an evidence of myocardial inflammation ( class iia recommendations ) .
nevertheless , lower initial doses ( < = 30 mg daily ) can be used without effecting long - term prognosis .
prednisone can be gradually tapered , once the disease is responding , over a period of six months and eventually discontinued .
other immunosuppressive agents , such as infliximab , methotrexate , azathioprine , or cyclophosphamide , can be used in patients who can not tolerate or do not respond to prednisone therapy .
atrial and ventricular arrhythmias in patients with cs can be controlled with beta - clockers , calcium channel blockers , sotalol , dofetilide , and amiodarone .
class i agents are not recommended due to their adverse outcomes in patients with structural heart disease .
stepwise approach can be used in the management of ventricular arrhythmias ; initial treatment with immunosuppression is followed by anti - arrhythmic medications , and finally catheter ablation in refractory or incessant cases ( class iia recommendation ) .
vt / vf storm can be managed with combination therapy of anti - arrhythmic agents , usually amiodarone , and immunosuppression .
cardioverter - defibrillator implantation is recommended in patients with spontaneous sustained ventricular arrhythmias , cardiac arrest , or in patients with lved < = 35% despite optimal medical therapy and immunosuppression ( class i recommendation ) .
icd can be useful in patients with an indication for permanent pacemaker implantation , unexplained syncope or near syncope that is felt to be arrhythmic in origin , or inducible sustained ventricular arrhythmias ( class iia recommendation ) .
icd can be considered in patients with lvef of 36 - 49% and/or rvef < 40% despite optimal medical therapy for heart failure and a period of immunosuppression ( class iib recommendation ) .
heart failure due to cs is treated in the same way of that of other causes .
patients with advanced heart failure or persistent ventricular arrhythmias not amenable to ablation might be considered for cardiac transplantation .
recipients show better one - year post - transplant survival when compared to cardiac transplant for all other indications .
screening for cardiac involvement is vital in patients with biopsy proven extra - cardiac sarcoidosis .
this can be done initially by history , 12-lead electrocardiogram ( class i recommendation ) and 2d - echocardiogram ( class iia recommendation ) .
abnormalities can be investigated by advanced cardiac imaging with cmr or fdg - pet ( class iia recommendation ) .
additionally , patients younger than 60 years with unexplained second - degree ( mobitz ii ) or third - degree av block can be screened for cs with high resolution ct chest and/or advanced cardiac imaging ( cmr or fdg - pet ) .
positive initial screening command extra - cardiac , if feasible , or image guided endomyocardialbiopsies ( class iia recommendation ) .
context : cardiac sarcoidosis ( cs ) is a rare , potentially fatal disease . it has a wide range of clinical presentations that range from asymptomatic electrocardiogram changes to sudden cardiac death . 
 ventricular aneurysms and ventricular tachycardia are seen late in the disease , and are rarely the presenting manifestation of the disease . 
 diagnosis of cs is challenging and often missed or delayed.case report : we report a 35-year - old patient who presented with sustained ventricular tachycardia and st - elevation on electrocardiogram . 
 cardiac catheterization showed normal coronaries and left ventricular aneurysm . 
 subsequent 2d - echocardiography showed an infiltrative disease pattern . 
 cardiac mri was done and showed late gadolinium enhancement in the septum , apex and lateral wall . 
 the patient was diagnosed with cardiac sarcoidosis and treated with immune suppression and antiarrhythmic agent . 
 in addition underwent aicd implantation.conclusion:our case highlights the importance of suspecting cardiac sarcoidosis in young patients presenting with electrocardiogram changes , and present an atypical presentation of this disease .