Liver Transplantation Trends and Outcomes for Hereditary Hemorrhagic Telangiectasia in the United States.

BACKGROUND: Liver arteriovenous malformations (AVM) in hereditary hemorrhagic telangiectasia (HHT) can necessitate liver transplantation. There is limited data on HHT patients undergoing liver transplantation (LT) in the United States. METHODS: Two sources of data were used: (1) Scientific Registry of Transplant Recipients (SRTR) database (1998-2016) (2) Single center liver transplant database (Mayo Clinic Rochester, MN). The aims of this study were (1) to determine trends in LT for HHT-related liver involvement in the United States using the SRTR database; (2) to identify clinical characteristics, indications, and outcomes for LT in HHT. RESULTS: Thirty-nine HHT patients were listed for LT in the SRTR database from 1998-2016 to 1998-2001 (n = 1); 2002-2005 (n = 4); 2006-2010 (n = 10), and 2011-2016 (n = 24). Twenty-four underwent LT at a median age of 47.5 years (interquartile range, 37.0-58.5 years). Median calculated MELD score at time of LT was 8.0 (interquartile range, 7.0-9.5), and 75% received an exception MELD score. Two status-1 patients died during transplant surgery. Nineteen (86%) patients were alive after a median post-LT follow-up of 48 months, whereas 2 patients were lost to follow-up. Five of the aforementioned HHT patients underwent LT at Mayo Clinic, 4 with high output cardiac failure, and 1 with biliary ischemia. All 5 were alive at the time of last follow-up with good graft function and resolution of heart failure. CONCLUSIONS: Outcomes after LT for HHT patients are excellent with 86% survival after a median follow-up of 48 months and resolution of heart failure. LT listing for HHT has increased in substantially in more recent eras.

Distribution and complications of native arteriovenous fistulas in maintenance hemodialysis patients: a single-center study.

BACKGROUND: The aim of this study was to investigate the use of vascular access in maintenance hemodialysis (MHD) patients and the complications of native arteriovenous fistulas (NAVFs) in a Chinese single center. METHODS: Patients (n=376) were recruited in our descriptive cross-sectional study, 350 of them had wrist NAVFs (wrist group),and 18 of them had elbow NAVFs (elbow group). We evaluated the complications associated with NAVFs and the prevalence of high-output cardiac failure between these 2 groups. All statistical analyses were performed with the use of the SPSS software package, version 17.0. Data were expressed as means ± SD; a p value <0.05 was assumed to be statistically significant. RESULTS: We found 97.87% of patients had NAVFs, among them 93.09% (350/376) of patients had wrist NAVFs, followed by elbow NAVFs 4.79% (18/376), while 1.33% (5/376) had graft AVFs and only 0.80% (3/376) of patients depended on a cuff catheter. The most frequent complications seen in our patients were thrombosis (13.86%), followed by aneurysm (12.23%), anastomotic stenosis (2.17%); arterial steal syndrome (1.63%); infection (0.54%); and venous hypertension (0.27%). Aneurysm was more common in the elbow group (66.67%) than in the wrist group (9.43%) (p<0.01). There were no differences in other complications, patency rate during 1-3 years or Kt/V between the 2 groups. We did not find high-output cardiac failure related to NAVFs in our patients. CONCLUSION: The prevalence of complications of NAVFs is high, and greater attention should be paid to the prevention of these complications. Early diagnosis and management of complications related to AVFs is essential to prevent loss of the vascular access. We think the wrist radiocephalic NAVF is the first choice for MHD patients; an elbow NAVF is a reasonable alternative for MHD access when a radiocephalic NAVF is not possible.

[High cardiac output in myeloma patients. Its prevalence and clinical characteristics. The Castile-León Cooperative Group for the Study of Monoclonal Gammapathies]. / Alto gasto cardíaco en pacientes con mieloma. Prevalencia y características clínicas. El Grupo Cooperativo Castellano-Leonés para el Estudio de las Gammapatías Monoclonales.

BACKGROUND: The study determines the prevalence of high cardiac output status in patients with multiple myeloma (MM) and its relationship with the more significant clinical and biological characteristics of the disease. PATIENTS AND METHODS: Cardiac output was determined in 28 patients with multiple myeloma by pulsed Doppler echocardiography. Patients with any other identifiable cause of high cardiac output were excluded. Mean age was 70 +/- 8 SD years (53-84). A stepwise regression with the cardiac output as dependent variable and age, performance status (ECOG), clinical stage (Durie and Salmon), scale of bone lesions, serum calcium, serum creatine, bone involvement (percentage), and M protein quantity as independent variables showed the following results. RESULTS: A high cardiac output state, defined as a cardiac index higher than 4.6 l/min/m2 was present in five patients (17.8%), four of them with severe bone involvement. Two patients developed heart failure. The stepwise regression revealed the scale of bone lesions was the unique variable associated with the risk of developing a high cardiac output status. CONCLUSIONS: A high cardiac output status may be fairly common in patients with multiple myeloma. The syndrome is associated with severe bone involvement, being the scale of bone lesions the only risk factor for this complication.