ABSTRACT
Brugada syndrome (BS) is an inherited cardiac ion channelopathy that is a rare, but treatable, cause of sudden cardiac death (SCD). There are many studies that explore the management of symptomatic BS, but few trials have been conducted regarding management of asymptomatic Brugada patients. Asymptomatic BS patients are shown to be at increased risk (0.5%-1.5%) for SCD compared to the general population and account for nearly 20% of deaths from SCD in patients with structurally normal hearts. Treatment for asymptomatic BS patients is often debated with the current guidelines allowing for management decisions to be made on a case-by-case basis. Therapies include either anti-arrhythmic medications, implantable cardioverter-defibrillator (ICD) placement, or no active treatment. This review intended to assess whether ICD placement benefits asymptomatic BS patients and what criteria may be useful in selecting patients for ICD placement. Results showed that ICD placement can reduce mortality in select asymptomatic patients. There were certain risk factors that increased the likelihood that an asymptomatic patient would experience SCD and thus benefit from an ICD. These factors include an electrocardiogram(ECG) demonstrating spontaneous type 1 Brugada Syndrome and inducibility of ventricular tachyarrhythmias during electrophysiological study. Other variables including gender, family history of SCD, and the presence of SCN5A mutation were not predictive of arrhythmic events. Moreover, many patients can suffer complications from ICDs that can affect the quality of life including inappropriate shocks, device malfunction, infection, mental health problems, and difficulties with replacements. Guidelines for quantifying the risk of SCD relative to the risks associated with ICD placement are still poorly defined. These complications and risk factors should be taken into consideration in the context of a patient-centered discussion regarding ICD placement in asymptomatic patients.
ABSTRACT
BACKGROUND: Upper tract urothelial carcinoma (UTUC) is a rare disease with a highly heterogeneous biologic behavior. Accurate individualized prediction of the behavior of UTUC could help guide personalized clinical decision-making regarding optimal therapy. METHODS: A MEDLINE literature search was performed on UTUC predictive tools. We recorded input variables, prediction form, number of patients used to develop the prediction tools, outcomes being predicted, prediction tool-specific features, predictive accuracy, and whether internal or external validations were performed. Each prediction tool was classified according to the clinical disease state it addressed and the outcome it predicted. RESULTS: The literature search generated five published tools for UTUC staging and prognostication. None of these prediction tools have undergone external validation yet. Two tools focused on the clinical decision-making regarding conservative management versus radical nephroureterectomy (RNU), lymphadenectomy versus not, and neoadjuvant systemic therapy versus not. Three tools focused on the prognosis after RNU, thereby helping in the decision-making regarding adjuvant systemic chemotherapy. CONCLUSIONS: Management of UTUC is challenging, and there are no high-level data to guide physicians and patients. Prognostic tools relying on data from large cohorts of patients are currently the best source of information for evidence-based management of UTUC patients.
