SMP Student Researcher: Parm Khakh

Southern Medical Program Student: Parm Khakh, Class of 2021
Research Supervisor:
Dr. Jasmine Grewal, Clinical Assistant Professor, UBC Department of Medicine, Division of Cardiology

What’s your research about?

The pathophysiology of Fontan-Associated Liver Disease is not well-documented aside from the state of chronic passive congestion caused by the disturbance in the liver’s blood supply and drainage. Studies have shown that these patients commonly develop histological liver abnormalities; therefore, effective screening methods need to be in place to ensure the patients experiencing these complications are identified early to facilitate quicker treatment and decrease morbidity associated with this condition. There are no robust guidelines in the utilization of the biomarker Alpha-Fetoprotein (AFP), and its requisition varies widely between physicians. Currently, the Pacific Adult Congenital Heart (PACH) Clinic at St. Paul’s Hospital, does not utilize AFP until there is an identified abnormality on imaging, which is most often assessed by abdominal ultrasonography (US). Due to substantial inter and intraobserver variability in abdominal US and the reduced sensitivity in patients with early-onset liver disease, it is understandable why clinical examination, laboratory data, and imaging, with or without histological evaluation, are essential in ensuring hepatological complications are not overlooked.

Although the Fontan procedure has significantly reduced early childhood mortality for these patients, it is also important to avoid premature death in adulthood. Therefore, this retrospective chart review assessed and compared patients’ current age, liver enzymes (alkaline phosphatase (ALP), gamma glutamyl transpeptidase (GGT), aspartate aminotransferase (AST), alanine aminotransferase (ALT) from 2014 to present, as well as AFP and imaging studies completed over the same time frame.

What were your findings?

The retrospective chart analysis showed that there were a number of patients that were not getting the full workup during their annual visits. Of the one-hundred and two patients included in the study, nine patients had clinically elevated AFP levels, but only three had diagnostic imaging studies done. Ten patients had abnormally high levels of AFP, and only one had gotten a diagnostic imaging study, which was completed more than five years ago. There were nineteen patients that had never had an AFP level assessed. This finding indicated that a large proportion of the individuals were not getting adequately worked up, and potential complications of the Fontan procedure were not being investigated. Of these nineteen patients, none had received diagnostic imaging studies. All of this information together, strongly indicates that hepatic complications could be occurring in these patients, but no evaluations were being done to explore this potentially deleterious outcome. Of these patients, thirteen had abnormal liver US findings, indicated by hepatic cirrhosis, hepatic congestion, hepatocellular carcinoma or chronic liver disease. This finding correlates with the literature which emphasizes that liver enzymes are not an accurate reflection of abnormal US findings and why more workup needs to be assessed. Only twenty-two of the 102 patients have had diagnostic imaging done in the last six years, with seventeen of them having them done in the last three years. This shows that active liver surveillance is improving as physicians’ protocols become more similar, but a lot more work needs to be done to ensure that every patient with this complex circulatory system is getting adequately monitored.

Once the patients with inadequate evaluations were identified, it was decided to have them followed up to ensure that important findings were not missed. Nineteen patients were given imaging requisitions due to the combination of their elevated AFP and abnormal liver enzymes; the imaging study requested was a triple phase CT scan of their livers. Nineteen patients were given lab requisitions to ensure there AFP levels were assessed, so they too could have diagnostic imaging studies should they be necessary. The QI study showed that although active steps are being made to ensure every patient follow-up is similar and consistent, a more thorough assessment of these patients is necessary to ensure their long-term well-being.

This research project was supported through the Southern Medical Program studentship program.