An Unusual Case of Cirrhosis

A 45-year-old female presents with chest pressure and dyspnea at rest for two days, followed by a syncopal episode. Her medical history is significant for obstructive sleep apnea, and former tobacco and alcohol abuse (3-4 drinks per day, quit 5 years ago).

Prior to her admission (about 1 year), she presented to her local primary care physician complaining of palpitations, fatigue and low blood pressure readings (BP range 96/70 – 100/70mmHg; HR 80 – 90bpm).

Laboratory work up at that time was significant for TSH 2.41µIU/mL (0.35-4.94µIU/mL), Total T4 7.2ng/dL (4.9-11.7ng/dL), Free T4 1.28ng/dL (0.7 – 1.48ng/dL), WBC 3.8x103 µL-1, Hgb 13.5g/dL, platelets 95x103 µL-1. An electrocardiogram (ECG) showed nonspecific ST and T wave abnormalities. She underwent a nuclear stress test that was also unremarkable. By that time, she was placed on midodrine 5mg twice daily and presented again four months later complaining of varicose veins and leg edema. On that occasion, blood pressure had improved, midodrine was stopped, and she was started on furosemide 20mg daily as needed for edema. Four months later she presented once again with worsening edema, and furosemide dose was increased.

Concurrently, she underwent work-up for an ovarian cyst with high CA-125. As part of the work-up, abdominal and pelvic computed tomography (CT) incidentally demonstrated heterogeneous appearance of the liver and mild surface nodularity suggesting mild/early cirrhosis, mild-to slightly moderate ascites, and also moderate pericardial calcification extending from the base of the heart towards the apices of the ventricles, without pericardial effusion.

Further abdominal magnetic resonance imaging (MRI) characterized the cyst as a corpus luteum cyst. Esophagogastroduodenoscopy (EGD) revealed hypertensive gastropathy, non-bleeding erosive gastropathy, non-bleeding duodenal ulcers with clean base, and grade 1 esophageal varices. Comprehensive laboratory evaluation showed no evidence of underlying liver diseases such as viral or autoimmune hepatitis, hemochromatosis, Wilson's disease, primary biliary cirrhosis or primary biliary cholangitis.

At her current presentation, she denied fevers, chills, nausea, or abdominal pain. She also denied worsening of the edema.

Vitals: BP 129/83mmHg; HR 89bpm, RR 18bpm, Temp 36.2 C, SpO2 99% on room air.

On exam, she was dyspneic, though in no acute distress. Cardiac auscultation was significant for Kussmaul's sign and a pericardial knock.

Initial laboratory work-up showed CK 68U/L, MB 1%, Troponin T<0.01ng/mL, BNP 86pg/mL, INR 1.1, WBC 3.9x103 µL-1, Hgb 12.5g/dL, platelets 101x103 µL-1, BUN 15mg/dL, Cr 0.87mg/dL, K 3.8mEq/L, Na 141mEq/L, total bilirubin 0.7mg/dL, alkaline phosphatase 80U/L, ALT 21U/L, AST 20U/L, WSR 8mm/h, CRP 0.1mg/L.

Electrocardiogram (ECG), additional imaging, and left and right heart catheterization were performed and are shown below (ECG 1 and Figure 1):



Figure 1

Figure 1
Figure 1: (A) Double oblique short axis orientation on cardiac CT demonstrating significant calcium burden along the lateral atrioventricular groove. (B) Circumferential pericardial calcification demonstrated on fluoroscopy (C) Short axis view on T2 short tau inversion recovery cardiac MRI sequence showing no pericardial enhancement. (D) Short axis view on delayed hyper enhancement sequences showing no increased pericardial signal to suggest pericardial inflammation (E) Tissue Doppler velocities of the septal and lateral mitral annulus. Doppler velocity with a compensatory increase in medial annular tissue Doppler velocity commonly referred to as "annulus reversus" (F) Apical 4 chamber window on transthoracic echocardiogram demonstrating conical/tubular deformity of the ventricles, suggestive of constrictive physiology.

Left Heart Catheterization
Left Main Coronary artery No disease
Left Anterior Descending Artery No disease
Left Circumflex Artery No disease
Right Coronary Artery Dominant, tortuous, with no disease.
Right Heart Catheterization
Right Atrium 20mmHg (with deep y descent)
Right Ventricle 44/7mmHg
Pulmonary Artery 40/20mmHg (29 mmHg)
PCWP 22mmHg
Cardiac output 7L/min
Cardiac index 3.6L/min/m2

Simultaneous LV/RV pressure measurements revealed ventricular interdependence, with a systolic area index of 1.3, and presence of a square root sign.

What is the most appropriate next step in management?

Show Answer