Prospective evaluation of patients with non-cirrhotic portal hypertension: A single centre study.

BACKGROUND: Non-cirrhotic portal hypertension (NCPH) is a spectrum of liver diseases, including porto-sinusoidal vascular disorder, with portal hypertension (PH) in the absence of cirrhosis. The natural history and diagnostic approach to NCPH are not well understood. AIM: We aimed to evaluate disease progression and outcomes in NCPH. METHODS: Patients with or at risk for NCPH were enrolled in a single centre prospective study; two groups were formed based on the presence of specific features of PH, such as varices, collaterals, portal hypertensive gastropathy or portal hypertensive bleeding. All participants underwent a baseline liver biopsy. Liver stiffness measurement (LSM), and imaging were repeated every 6-12 months. RESULTS: Fifteen patients without specific features of PH (Group I), and 35 patients with specific features (Group II) were enrolled. The median follow-up time was 50 months. Group II had higher hepatic venous pressure gradients, non-invasive measures of PH and a lower platelet count (PLT) when compared to Group I. Rates of survival and decompensation were similar in both groups. Patients with PLT ≤100 K/mcL had lower survival compared to those with PLT >100 K/mcL. Patients with LSM ≥10 kPa had lower survival and survival without decompensation when compared to patients with LSM <10 kPa. CONCLUSIONS: Patients irrespective of specific features of PH had similar survival or survival without decompensation. Patients without specific features are at risk for disease progression and should be monitored closely. Thrombocytopenia and increased LSM are associated with severe forms of liver disease, which are strongly associated with outcomes.

Dobutamine stress echocardiography in patients undergoing orthotopic liver transplantation: a pooled analysis of accuracy, perioperative and long term cardiovascular prognosis.

Pre-transplant evaluation for orthotopic liver transplantation (OLT) commonly includes a cardiac evaluation using dobutamine stress echocardiography (DSE). We performed a quantitative systematic review assessing DSE's use in detecting coronary artery disease (CAD) and predicting perioperative and long term cardiac events in patients undergoing OLT. Published studies in pubmed were accessed using keyword searches and bibliographic review. Included studies evaluated the use of DSE in patients undergoing OLT, including its accuracy for detection of CAD, and in predicting perioperative and long term cardiac prognosis for both hard (myocardial infarction, cardiac death, cardiac arrest, and asystole) and soft cardiac events (all other events that were cardiovascular in nature). We calculated DSE's sensitivity, specificity, negative predictive value (NPV), and positive predictive value (PPV) in the above areas. We identified 7 studies, including a total of 580 patients, which included 4 accuracy studies (n = 110 patients), 4 perioperative studies, and 3 long term studies. Accuracy for CAD included a sensitivity of 0.32, specificity of 0.78, PPV of 0.37, and NPV of 0.75. Accuracy for prediction of perioperative hard and soft cardiac events was a sensitivity of 0.20 and 0, specificity of 0.99 and 0.99, PPV of 0.33 and 0, and NPV of 0.98 and 0.89, respectively. For long term hard and soft cardiac events, sensitivity was 0.5 and 0, specificity 0.99 and 0.98, PPV 0.33 and 0, and NPV 0.99 and 0.96, respectively. DSE has a limited accuracy for the detection of CAD in candidates for OLT. However, among those patients selected for OLT, the negative predictive value of DSE for both perioperative and long term cardiac events is high.