Intravenous leiomyoma is a rare tumor of smooth muscle with invasion to veined channels that affects women at reproductive age. The case of a 45-year-old woman is described, with history of oophorectomy and hysterectomy, in addition to abdominal surgery due to mesenteric tumor. Several months later, the patient developed syncope and dyspnea secondary to pulmonary embolism. Echocardiographic study reported a mass in right side of heart proceding from inferior vena cava. The patient underwent tumor surgical resection from left iliac vein and histologic study concluded intravascular leiomyomatosis.

The Fontan procedure (FP) is the standard surgical treatment for Univentricular heart diseases. Over time, the Fontan system fails, leading to pathologies such as protein-losing enteropathy (PLE), plastic bronchitis (PB), and heart failure (HF). FP should be considered as a transitional step to the final treatment: heart transplantation (HT). This systematic review and meta-analysis aims to establish the risk of death following HT according to the presence of FP complications. There was a total of 691 transplanted patients in the 18 articles, immediate survival 88% (n = 448), survival from 1 to 5 years of 78% (n = 427) and survival from 5.1 to 10 years of 69% (n = 208), >10 years 61% (n = 109). The relative risk (RR) was 1.12 for PLE (95% confidence interval [CI] = 0.89–1.40, p = 0.34), 1.03 for HF (0.7–1.51, p = 0.88), 0.70 for Arrhythmias (0.39–1.24, p= 0.22%), 0.46 for PB (0.08–2.72, p = 0.39), and 5.81 for CKD (1.70–19.88, p = 0.005). In patients with two or more failures, the RR was 1.94 (0.99–3.81, p = 0.05). After FP, the risk of death after HT is associated with CKD and with the presence of two or more failures.

Objective: Magnetic resonance imaging (MRI) and cardiac catheterization are diagnostic tools for right ventricle dysfunction (RVD), but those are expensive and often unavailable techniques. Thus, our objective was to identify clinical and/or echocardiographic variables capable of predicting a catheterization-based diagnosis of RVD.Design: This was cross-sectional, diagnostic test accuracy study, considering the catheterization-based diagnosis of RVD as the gold standard.Patients: Pediatric patients with non-repaired CHD with overload pressure were evaluated. Clinical variables (edema and functional class), transthoracic echocardiography (right heart dimensions, systolic and diastolic function, Doppler velocities), and cardiac catheterization (pressures and right ventricle systolic work measurements) were obtained during the same hospitalization.Results: We included 253 patients with tetralogy of Fallot (39.9%), pulmonary atresia with ventricular septal defect (33.9%), type C Ebstein's anomaly (15.8%), or pulmonary stenosis (10.4%). Among clinical (vascular congestion, functional class derangement) and echocardiographic (indexed right ventricle diameter, fractional area change, tricuspid annular plane systolic excursion, S' wave, Tei index) variables, the Tei index (defined as the ratio of isovolumetric contraction time to ejection time) was the sole variable that exhibited high diagnostic capability, with 98.5% sensitivity, 97.4% specificity, 97.8% positive predictive value, and 98.3% negative predictive value, with 98.0% overall performance. Multivariate logistic regression confirmed that Tei index alone predicted the catheterization-based diagnosis of RVD.Conclusions: Tei index is the best parameter that can be employed for the non-invasive identification of RVD in patients with CHD.

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