SARCOID CARDIOMYOPATHY with LIVER AND LYMPH NODE INVOLVEMENT
57-year-old male with pathology findings consistent with granulomatous hepatitis and non necrotizing granulomas in inguinal lymph nodes both consistent with sarcoidosis but without pulmonary findings. Lymphadenopathy is also present in the axillae and groins without involvement of the mediastinum
- Associated cardiovascular findings include findings consistent with hypertrophic cardiomyopathy with:
- Diabetes and Hypertension
- CXR showing LVE
- Abnormal stress test with concerning regions in RCA territory inferiorly
- Moderate LVH on echo with normal EF (68%) normal LA, RA PAP and RV function. LV mass was 127g/sq. m
- Focal nodular LGE in the anterior apical region in mid myocardial/subendocardial region and in the inferior mid myocardial wall medially
- Subsequently developed episodes of paroxysmal ventricular tachycardia with EP ablation and placement of a defibrillator (ICD)
- LH and RH catheterization performed 6 years after initial studies showed elevation of PC WP of 25 mmHg, mean RA pressure of 16 mmHg, mean PAP of 34 mmHg no CAD
- Subsequent CT showed mildly enlarged LA and RA with mild TR
- 6 years after initial presentation he had symptoms of biventricular failure with increasing dyspnea and pedal edema culminating in an acute episode of monomorphic VT episode, ectopic atrial flutter/fibrillation, and left bundle branch aberrancy requiring amiodarone and cardioversion. Underwent upgrade to biventricular upgrade to his ICD
PRESENTED WITH ABNORMAL LFT’S
Ashley Davidoff MD
LIVER BIOPSY
SARCOID CARDIOMYOPATHY with LIVER and LYMPH NODE INVOLVEMENT
57-year-old male with pathology findings consistent with granulomatous hepatitis and non-necrotizing granulomas in inguinal lymph nodes both consistent with sarcoidosis but without pulmonary findings. Lymphadenopathy is present in the axillae and groins without involvement of the mediastinum
Ashley Davidoff MD
SUBSEQUENT CT SHOWED LIVER NODULES
Ashley Davidoff MD
Normal Lung Parenchyma
Ashley Davidoff MD
Adenopathy
CT scan shows axillary adenopathy (red arrowheads right upper and lower image) and inguinal adenopathy (red arrowheads, left upper and lower image)
Ashley Davidoff MD
Ultrasound of Cervical, and Submandibular Adenopathy
Ashley Davidoff MD
MRI
LV THICKENING – HYPERTROPHY vs INFILTRATION
Normal LA RA and RV
Ashley Davidoff MD
LV – LVE Normal EF
LVE
MRI 4 CHAMBER PROJECTION
Ashley Davidoff MD
Normal RV
RV NORMAL THICKNESS AND EJECTION FRACTION
Ashley Davidoff MD
Delayed Gadolinium Sequences
Ashley Davidoff MD
Ashley Davidoff MD
Had Single Lead Pacemaker Placed but Subsequent V Fib Requiring Cardiovesrion
6 years after initial presentation he had symptoms of biventricular failure with increasing dyspnea and pedal edema culminating in an acute episode of monomorphic VT episode, ectopic atrial flutter/fibrillation, and left bundle branch aberrancy requiring amiodarone and cardioversion. Underwent upgrade to biventricular upgrade to his ICD. CXR shows LVE and LAE , with prominent azygos vein suggesting right heart failure, with cephalisation of the vessels indicating CHF. Defibrillator pad in right upper chest.
Ashley Davidoff MD
Placement of Biventricular Pacemaker and Defibrillator
o Subsequently developed episodes of paroxysmal ventricular tachycardia with EP ablation and placement of a defibrillator (ICD)
o LH and RH catheterization performed 6 years after initial studies showed elevation of PC WP of 25 mmHg, mean RA pressure of 16 mmHg, mean PAP of 34 mmHg no CAD
o Subsequent CT showed mildly enlarged LA and RA with mild TR
o 6 years after initial presentation he had symptoms of biventricular failure with increasing dyspnea and pedal edema culminating in an acute episode of monomorphic VT episode, ectopic atrial flutter/fibrillation, and left bundle branch aberrancy requiring amiodarone and cardioversion. Underwent upgrade to biventricular upgrade to his ICD
Ashley Davidoff MD