The Common Vein

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

LIVER BIOPSY

 

SUBSEQUENT CT SHOWED LIVER NODULES

Normal Lung Parenchyma

Adenopathy

Ultrasound of Cervical, and Submandibular Adenopathy

MRI

LV THICKENING – HYPERTROPHY vs INFILTRATION

 

LV – LVE Normal EF

Normal RV

RV NORMAL THICKNESS AND EJECTION FRACTION
Ashley Davidoff MD

Delayed Gadolinium Sequences

Had Single Lead Pacemaker Placed but Subsequent V Fib Requiring Cardiovesrion

Placement of Biventricular Pacemaker and Defibrillator