How Common Is Liver Fibrosis in Adult Patients with Ebstein's Anomaly and Can It Serve as an Additional Criterion for Surgical Indication?

Background: Little is known about the prevalence of liver fibrosis and cirrhosis in patients with Ebstein's anomaly (EA). Known and established surgical criteria of EA are impaired right ventricular (RV) function with high-grade tricuspid regurgitation (TI). Could existing liver fibrosis or cirrhosis be considered an additional criterion for surgery?

Method: We performed a prospective study to verify structural liver changes in EA and tested a correlation of fibrosis/cirrhosis to severity of EA according to Carpentier, to RV function, and to TI.43 patients with native EA (28 w), median age = 43 years (range: 18–78 years) were studied. Noninvasive liver stiffness measurement was determined by the Acoustic Radiation Force Impulse Imaging (ARFI), RV function and TI by MRI, and Carpentier's classification by echocardiography. Results of ARFI measurement were classified according to the following values:

Fibrosis	ARFI (m/sec)	Ebstein's patients (n)
No	<1.185	21
Mild	≥1.185	2
Moderate	≥1.215	11
Severe	≥1.540	2
Cirrhosis	≥1.940	6

Results: A total of 11 patients were classified as mild (A), 19 as moderate (B), and 13 as marked (C) form of EA according to Carpentier. Forty-two of 43 patients received ultrasonography of the liver by ARFI. The mean value of liver stiffness measurement was 1.19 m/s (range: 0.83–3.76 m/s). Forty-one of 43 patients received MRI. RV ejection fraction was 52% (range: 23–74%), regurgitation fraction of TI (directly measured) was 37% (range: 0–83%) at median, and 39.5% (range: 0–80%) by indirect (calculated) method. Severity of EA, RV-EF, and TI did not correlate with liver changes detected by ARFI.

Conclusion: Noninvasive liver examination by ARFI revealed higher grade changes in liver structure in 45% of native patients with EA in terms of fibrotic/cirrhotic processes. However, no correlation with the severity of EA, right ventricular pumping efficiency, or extent of tricuspid regurgitation could be detected. From the present data, ARFI measurements for liver fibrosis or cirrhosis cannot be considered an additional criterion for surgery.

Publication History

Article published online:
12 February 2022