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Dear Andrea,
our Center of reference for HHT (Maggiore Hospital , Crema , Italy) belongs to VASC-ERN, and I am co-chair of the VASCERN-HHT group. So , one more Valdig member in the ERN. I would be more than happy to foster collaboration between VALDIG and VASC-ERN if you feel appropriate.
All my best !
ElisaDear 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! ElisaDear 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
