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Liver Disease in Patients After the Fontan Operation

Pundi, Krishna, BS ; Pundi, Kavitha N., MD ; Kamath, Patrick S., MD ; Cetta, Frank, MD ; Li, Zhuo, BS ; Poterucha, Joseph T., MD ; Driscoll, David J., MD ; Johnson, Jonathan N., MD

The American journal of cardiology, 2016-02, Vol.117 (3), p.456-460 [Peer Reviewed Journal]

United States: Elsevier Inc

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Title:
Liver Disease in Patients After the Fontan Operation
Author: Pundi, Krishna, BS ; Pundi, Kavitha N., MD ; Kamath, Patrick S., MD ; Cetta, Frank, MD ; Li, Zhuo, BS ; Poterucha, Joseph T., MD ; Driscoll, David J., MD ; Johnson, Jonathan N., MD
Subjects: Adolescent ; Adult ; Biopsy ; Cardiac arrhythmia ; Cardiovascular ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Fontan Procedure - adverse effects ; Forecasting ; Gastroenterology ; Heart ; Heart Defects, Congenital - surgery ; Humans ; Incidence ; Infant ; Liver - pathology ; Liver cirrhosis ; Liver Cirrhosis - diagnosis ; Liver Cirrhosis - epidemiology ; Liver Cirrhosis - etiology ; Liver diseases ; Magnetic Resonance Imaging ; Male ; Medical imaging ; Middle Aged ; Mortality ; NMR ; Nuclear magnetic resonance ; Postoperative Complications ; Pulmonary arteries ; Retrospective Studies ; Risk Factors ; Survival analysis ; Time Factors ; Ultrasonic imaging ; United States - epidemiology ; Variables ; Young Adult
Is Part Of: The American journal of cardiology, 2016-02, Vol.117 (3), p.456-460
Notes: ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
content type line 23
Description: We reviewed records of all patients with an initial Fontan operation or revision from 1973 to 2012 at our institution (n = 1,138); 195 patients had postoperative liver data available. Cirrhosis was identified by histopathology or characteristic findings on imaging with an associated diagnosis of cirrhosis by a hepatologist. Of 195 patients with biopsy or imaging, 10-, 20-, and 30-year freedom from cirrhosis was 99%, 94%, and 57%, respectively. There were 40 of 195 patients (21%) diagnosed with cirrhosis (mean age at Fontan 10.7 ± 8 years). On multivariate analysis, hypoplastic left heart syndrome was associated with increased risk of cirrhosis (n = 2 of 16, p = 0.0133), whereas preoperative sinus rhythm was protective (p = 0.009). Survival after diagnosis of cirrhosis was 57% and 35%, at 1, and 5 years, respectively. The cause of death was known for 9 patients (5 multiorgan failure, 2 liver failure, and 2 heart failure). In conclusion, there is an incremental occurrence of cirrhosis after the Fontan, which should be considered when designing follow-up protocols for patients after Fontan operation.
Publisher: United States: Elsevier Inc
Language: English

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Liver Disease in Patients After the Fontan Operation

Pundi, Krishna, BS ; Pundi, Kavitha N., MD ; Kamath, Patrick S., MD ; Cetta, Frank, MD ; Li, Zhuo, BS ; Poterucha, Joseph T., MD ; Driscoll, David J., MD ; Johnson, Jonathan N., MD

United States: Elsevier Inc

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