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Background: Patient reported outcomes (PROs) are important measures in acquired heart disease but have not been well defined in Adult Congenital Heart Disease (ACHD). Our aim was to explore the discriminatory capacity of PRO survey tools in Fontan circulatory failure (FCF). Methods: Consecutive adults were enrolled from our ambulatory clinics. Inclusion criteria were age ≥18 years, a Fontan circulation or a hemodynamically insignificant shunt lesion, and sufficient cognitive/language abilities to complete PROs. A comprehensive package of PRO measures, designed to assess perceived health-related quality of life (HRQOL) was administered (including the Kansas City Cardiomyopathy Questionnaire [KCCQ-12], EuroQol-5-dimension [EQ5D], Short Form Health Status Survey [SF-12], self-reported New York Heart Association [NYHA] Functional Class, and Specific Activity Scale [SAS]). Results: We compared 54 Fontan patients (35 ± 10 years) to 25 simple shunt lesion patients (34 ± 11 years). The KCCQ-12 score was lower in Fontan versus shunt lesion patients (87 [IQR 79, 95] versus 100 [IQR 97, 100], p-value < 0.001). The FCF subgroup was associated with lower KCCQ-12 scores as compared with the non-FCF subgroup (82 [IQR 56, 89] versus 93 [IQR 81, 98], p-value = 0.002). Although the KCCQ-12 had the best discriminatory capacity for determination of FCF of all PRO tools studied (c-statistic 0.75 [CI 0.62, 0.88]), superior FCF discrimination was achieved when the KCCQ-12 was combined with all PRO tools (c-statistic 0.82 [CI 0.71, 0.93]). Conclusions: The KCCQ-12 questionnaire demonstrated good discriminatory capacity for the identification of FCF, which was further improved through the addition of complementary PRO tools. Further research will establish the value of PRO tools to guide management strategies in ACHD.
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A previously well 34-year-old man presents with electrical storm after performing headstands. A step-by-step review of clinical information and case development is presented with discussion. Ultimately, 2 rare diagnoses are discovered and their potential contribution to a cascade of complications resulting in ventricular arrhythmia are discussed. (Level of Difficulty: Intermediate.).
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Background: Coarctation of the aorta (CoA) is associated with intracranial aneurysms (IAs); however, the prevalence and risk factors (RFs) are not well described. Current practice guidelines offer inconsistent recommendations on screening for IAs in this patient population ranging from "not recommended" (European Society of Cardiology 2020) to "recommended" (American Heart Association 2018). Objectives: The purpose of this study was to determine the prevalence and RFs for IAs in patients with CoA. Methods: We completed a systematic review and meta-analysis of studies utilizing computed tomography or magnetic resonance angiographic screening for IAs in patients with CoA. Results: Five cohort studies were included, representing 442 patients. The pooled prevalence of IAs in patients with CoA was 3.8% [95% CI: 0.1%-12.3%]. The results met our prespecified definition for high heterogeneity. Of 5 RFs evaluated, only hypertension was associated with the development of IAs with an odds ratio of 3.1 [95% CI: 1.1-8.2; P = 0.03]. There was an observed downward trend over time in the prevalence of IAs among the studies included. Conclusions: The development of IAs is likely multifactorial in etiology and there may be modifiable RFs in their development. Considering the low prevalence of IAs in the pooled result, routine screening of patients with CoA for IAs is likely of low-value.
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An interesting case of respiratory failure secondary to occupational exposure in a 28-year-old male http://bit.ly/2SzR6dK.