Portopulmonary Hypertension: An Updated Review.

Portal hypertension may have major consequences on the pulmonary vasculature due to the complex pathophysiological interactions between the liver and lungs. Portopulmonary hypertension (PoPH), a subset of group 1 pulmonary hypertension (PH), is a serious pulmonary vascular disease secondary to portal hypertension, and is the fourth most common subtype of pulmonary arterial hypertension. It is most commonly observed in cirrhotic patients; however, patients with noncirrhotic portal hypertension can also develop it. On suspicion of PoPH, the initial evaluation is by a transthoracic echocardiogram in which, if elevated pulmonary pressures are shown, patients should undergo right heart catheterization to confirm the diagnosis. The prognosis is extremely poor in untreated patients; therefore, management includes pulmonary arterial hypertension therapies with the aim of improving pulmonary hemodynamics and moving patients to orthotopic liver transplantation (OLT). In this article, we review in detail the epidemiology, pathophysiology, process for diagnosis, and most current treatments including OLT and prognosis in patients with PoPH. In addition, we present a diagnostic algorithm that includes the current criteria to properly select patients with PoPH who are candidates for OLT.

Bilateral Proximal Forearm Transplantation: Case Report at 7 Years.

BACKGROUND: Although return of function has been reported in patients undergoing proximal forearm transplantations (PFTs), reports of long-term function are limited. In this study, we evaluated the clinical progress and function 7 years postoperatively in a patient who underwent bilateral PFT. CASE PRESENTATION: A 58-year-old man underwent bilateral PFT in May 2012. Transplantation involved all of the flexor and extensor muscles of the forearm. Neurorrhaphies of the median, ulnar, and radial nerves were epineural and 7 cm proximal to the elbow. Immunosuppressive maintenance medications during the first 3 years postoperatively were tacrolimus, mycophenolate, and steroids, and later, tacrolimus, sirolimus, and steroids. Forearm function was evaluated annually using the Disabilities of the Arm, Shoulder, and Hand; Carroll; Hand Transplantation Score System; Short Form-36; and Kapandji scales. We also evaluated his grip and pinch force. RESULTS: Postoperatively, the patient developed hypertriglyceridemia and systemic hypertension. He experienced 6 acute rejections, and none were resistant to steroids. Motor function findings in his right/left hand were: grip strength: 10/13 kg; key pinch: 3/3 kg; Kapandji score: 6/9 of 10; Carroll score: 66/80; Hand Transplantation Score System score: 90/94. His preoperative Disabilities of the Arm, Shoulder, and Hand score was 50 versus 18, postoperatively; his Short Form-36 score was 90. This function improved in relation with the function reported in the second year. CONCLUSIONS: Seven years following PFT, the patient gained limb strength with a functional elbow and wrist, although with diminished digital dexterity and sensation. Based on data presented by other programs and our own experience, PFT is indicated for select patients.

Liver transplantation center in México with low-volume and excellent results / Centro de trasplante hepático en México con bajo volumen y excelentes resultados

Background: Orthotopic liver transplantation (OLT) is the treatment of choice for end stage liver disease. Many studies show an inverse relationship between the number of procedures and operative mortality. Objective: The objective of the study is to show the results of our center and determine if it can have comparable results to high volumen centers. Method: This is a retrospective study which analyzed the information of patients with OLT at our institution from 1985 to December 31, 2012. Depending on date of transplantation, the study was divided into three stages. Stage 1: from 1985 to 1999. Stage 2: from 2000 to 2007. Stage 3: from 2008 to 2012. In the 1, 2 and 3 stage 22, 37 and 56 OLT were performed respectively. Results: Perioperative mortality was significantly lower between Stage 3 vs. Stage 1 and 2 (3.5% vs. 50% and 21.7%, p = 0.001). Patient survival was also better at 1 and 5 years at Stage 3 (94.4%, 87.8%) vs. era 2 (77.6%, 66.17%) and Stage 1 (47% and 29%) (p = 0.001). Conclusion: In conclusion, the present results of OLT at our program are excellent despite being a low-volume center.

Antecedentes: El trasplante hepático ortotópico (THO) es el tratamiento de elección para la insuficiencia hepática terminal. Numerosos estudios muestran una relación inversa entre el número de procedimientos y la mortalidad operatoria. Objetivo: El objetivo de este estudio es mostrar los resultados de nuestro centro y determinar si puede tener resultados equiparables a los obtenidos en centros de alto volumen. Método: Es un estudio retrospectivo en el que se analizó la información de pacientes con THO en nuestra institución, de 1985 al 31 de diciembre de 2012. Dependiendo de la fecha del THO, el estudio se dividió en tres etapas: etapa 1, de 1985 a 1999; etapa 2, de 2000 a 2007; y etapa 3, de 2008 a 2012. En las etapas 1, 2 y 3 se realizaron 22, 37 y 56 THO, respectivamente. Resultados: La mortalidad perioperatoria fue menor de manera significativa en la etapa 3 en comparación con las etapas 1 y 2 (3.5 vs. 50 y 21.7%; p = 0.001). La supervivencia de los pacientes a 1 y 5 años fue mejor en la etapa 3 (94.4 y 87.8%) que en la etapa 2 (77.6 y 66.17%) y en la etapa 1 (47 y 29%) (p = 0.001). Conclusión: En conclusión, los resultados actuales en THO en nuestro programa son excelentes, a pesar de ser un centro de bajo volumen.