Liver Transplantation in Patient with Hereditary Hemorrhagic Telangiectasia or Rendu-Osler-Weber Disease

Barajas, Adriana Calderón; Levstek, Meta; Heras, Jesus Ruiz De las; Navacerrada, M.I. Real; Diez, Maria Guerrero; Salazar, Rafael; Guerrero, Manuel Cortés; Silvestre, Francisco Pérez-Cerdá

doi:10.1097/01.tp.0000543934.82482.c9

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“…Two studies were identified by hand-searching in the bibliography of retrieved articles [ 24 , 25 ]. Therefore 45 studies were finally included for analysis [ 10 , 11 , 13 , 15 , 16 , 17 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 ]. The PRISMA flow diagram is depicted in Figure 1 .…”

Section: Resultsmentioning

confidence: 99%

“…Perioperative complications within 30 days were described in 28 (33.7%) patients ( Appendix A Table A2 ). Bleeding complications occurred in 13 (15.6%) patients: gastrointestinal bleeding in three patients (one died on day 11), intracerebral bleeding in two (one died on day 2), hematoma in Morrison space in one patient, massive pulmonary bleeding in two patients (both died) and no specified in five patients (Case 3, [ 12 , 17 , 21 , 24 , 26 , 33 , 39 , 44 , 49 , 56 ]). Importantly, these last two patients with pulmonary bleeding were not screened for pulmonary AVM prior to LT [ 24 , 49 ].…”

Section: Resultsmentioning

confidence: 99%

“… -/4.9 BVZ Álamo, 2019 [ 30 ] 1 (F) 62 - 1 * (V) HOCF Severe heart failure 7.1/- BVZ Sildenafil Ejiri, 2019 [ 31 ] 1 (F) 52 - 4 HOCF and PHTN secondary to hepatic AVMs WHO class IV (dyspnea at rest) 9/- - Ionescu, 2018 [ 32 ] 1 (M) 59 2 * (E, V) HOCF MEN-I, transitory ischemic attack, and AF. 15.3/7.6 - Barajas, 2018 [ 33 ] 1 (F) 40 ACVRL1 3 * (E, T, V) HOCF + Hepatic encephalopathy Numerous admissions due to HOCF, respiratory infections and hepatic encephalopathy 12/- - Ahumada, 2017 [ 34 ] 1 (M) 51 - 2 * (T i V) …”

Section: Table A1mentioning

confidence: 99%

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Perioperative Complications and Long-Term Follow-Up of Liver Transplantation in Hemorrhagic Hereditary Telangiectasia: Report of Three Cases and Systematic Review

Riera‐Mestre

Cerdà

Guzmán

et al. 2022

JCM

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The aim was to describe three patients with hemorrhagic hereditary telangiectasia (HHT) requiring liver transplantation (LT) and to perform a systematic review focusing on surgical complications and long-term follow-up. Unrestricted searches of the Medline and Embase databases were performed through February 2022. Forty-five studies were selected including 80 patients plus the three new reported patients, 68 (81.9%) were female and mean age was 50 (27–72) years. Main indications for LT were high-output cardiac failure (n = 40; 48.2%), ischemic cholangitis (n = 19; 22.9%), and a combination of both conditions (n = 13;15.6%). Mean cold ischemic time and red blood cell units transfused during LT were 554 (300–941) minutes and 11.4 (0–88) units, respectively. Complications within 30 days were described in 28 (33.7%) patients, mainly bleeding complications in 13 patients, hepatic artery (HA) thrombosis in four and hepatic vein thrombosis in one. Mean follow-up was 76.4 (1–288) months, and during it, four new patients developed thrombotic complications in HA, HA aneurysm, celiac artery, and the portal–splenic–mesenteric vein. HHT relapse in the transplant allograft was detected in 13 (17.1%) patients after 1–19 years (including two fatal recurrences). Overall mortality was 12%. In conclusion, previous assessment of HA anatomy and hyperdynamic circulatory state could reduce LT complications. The risk of relapse in the hepatic graft supports a multidisciplinary follow-up for HHT patients with LT.

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