Help in patient case

[Tim Schreuder]

Dear all,

I would like to have your opinion on the next case seen in our liver clinic; she is a 65 yrs old female known with Rendu Osler Weber’s disease. In July 15 she presented with cholecystitis. An abnormal ECG and significant impairment in left ventricular function and dilation with estimated EF 30% was found while being worked up for cholecystectomy. Additional abdominal CT-scanning revealed, as it appeared, large arteriovenous malformations. In order to diminish further deterioration of the dilated cardiomyopathy, angiography with coiling was attempted. However, during this procedure no AV-malformations were found but instead a hypertrophic hepatic artery was seen. No intervention was performed and a liver biopsy done; histological abnormalities are highly probable consistent with ROW.
My questions are whether some of you have more extensive experience with this category of patients? What would be the best next step? In my opinion liver transplantation is the best treatment option. However her current cardiac status probably makes this impossible.

Looking forward to your opinion(s),

Kind regards,

Tim Schreuder, consultant transplant hepatologist
University Medical Center Groningen, the Netherlands

[Elisabetta Buscarini]

Dear Tim,
the features of this patient (dilative cardiomyopathy, decreased FE, dilated hepatic artery) go for cardiac failure secondary to severe liver VMs, which is typically a dilative cardiomyopathy, initially at high output.
Some fundamental data are missing for understanding better the patient condition:
how’s the patient? (NYHA class?)
other cardiac performance data: CI, pulmonary pressures in particular? atrial fibrillation?
How large is hepatic artery?
CT data (VMs:where?) conflict with angiographic data, which however finds a dilated hepatic artery (typically associated to liver VMs); will you verify this discrepancy?
How about her LFTs? cholestasis?
How’s hemoglobin?
If the high output cardiac failure secondary to severe liver VMs is confirmed, patient would require a more extensive assessment (including heart cath for pulmonary pressure assessment and search for other visceral VMs, lung and brain in particular); you should avoid cholecystectomy which could precipitate devastating ischemic cholecisto-cholangitis; start intensive cardiological treatment including appropriate anemia correction; start evaluation for OLT provided heart and pulmonary pressures are permissive (cardiac function improves markedly after OLT); bevacizumab 5 mg/kg every 21 days, 6 times for induction cycle is an option either as a bridge to OLT if there is a substantial hemodynamic response or as treatment (and in this case she will require maintenance therapy).
Hope these synthetic suggestions can be of some help; I also suggest you to read EASL guidelines re vascular disorders of liver just appeared on Journal of Hepatology, section Hereditary hemorrhagic telangiectasia.
Elisabetta Buscarini
HHT Center of reference, Crema, Italy

[Andrea De Gottardi]

Dear Elisa

Thank you very much for your comments on Tim’s patient. This is very helpful for all of us and an excellent learning opportunity. I very much enjoyed this function in the forum.

With kindest regards

Andrea

Secretary of VALDIG
Swiss Liver Center, Bern, Switzerland

[Tim Schreuder]

Dear all,

Hereby I would like to have additional advice on this case I presented previous. Since then she has been in a stable clinical condition. I would like to provide questions raised by Elisabetta Buscarini.
– Patient can be classified as NYHA Class II
– Patient hasn’t been suffering from atrial fibrillation, right-sided catheterization in July
revealed CO 10.3 L/min, PAP 43/21 mm Hg, wedge 16 mm Hg and caval vein saturation infrahepatic 88%
and suprahepatic 69%, thoracic and abdominal CT demonstrates no other collaterals besides those in
the liver
– Her liver enzymes are completely normal including bilirubin and INR, hemoglobin is 6.1 mmol/l

After having an extensive look at our EASL guideline and discussions with transplant surgeons and anesthesiologists I got somewhat stuck in how to proceed. Do you think I should persuade the transplant team to have her on the waiting list for transplantation (of course if pulmonary pressures are acceptable and if not bevacizumab used as a bridge to transplantation)? Or do I have to follow a wait-and-see approach with regular cardiology check-ups?
I would be more than happy if one of you could help me out.

Kind regards,
Tim

[Elisabetta Buscarini]

Dear Tim,
on the basis of this update, patient condition seems very improved, I guess due to an aggressive cardiological treatment. She definitely needs a better correction of anaemia; some more cardiological data are important: pulmonary resistances? PAP you quote is systolic/diastolic?
If pulmonary resistances are compatible with OLT, I would start enlisting process; I would continue aggressive cardiological treatment (especially diuretics); I wouldn’t accept hb lower than 8.5.
If patient shows a further improvement you can wait and eventually de-list the patient.
All my best! Elisa

[Tim Schreuder]

Dear Elisa,

Thanks for your fast reply.
You are completely right regarding her clinical condition. She is in an exceptional good clinical condition. The PAP systolic and diastolic are indeed 43/21 mm Hg. Her pulmonary resistance is 85 dynes.
I’ll have a new discussion with the team.

I’ll keep you posted!

Kind regards, Tim

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