[A case of pulmonary artery sarcoma and review of the literature]. / Un caso di angiosarcoma polmonare e revisione della letteratura.

Pulmonary artery sarcoma (PAS) is a highly malignant tumor that originates in the pulmonary artery. This disease has a poor prognosis. Early diagnosis followed by radical surgical resection constitutes the only chance of survival for patients. However, owing to the rare and nonspecific clinical manifesta-tions and imaging findings, PAS is frequently misdiagnosed as various pulmonary thromboembolic diseases. We report the case of a 49-year-old woman who presented to our emergency department for dyspnea, hemoptysis, cough, and asthenia. A diagnosis of pulmonary thromboembolism was initially postulated. However, the rapid clinical progression of the disease, characterized by multiorgan involvement, together with the persistence of pulmonary filling defects despite appropriate anticoagulation therapy, induced to a late diagnosis of metastasized PAS. The peculiarity of our case consists of two main factors: the first is the atypical clinical presentation characterized by severe neurological impairment that finally led to patient's death; the second is the histopathological aspect of the lesion with a prevalent histiocytic cell component that is described in the literature as the less frequent pathological variant of this tumor.

Left Atrial Function After Radiofrequency Catheter Ablation of Atrial Fibrillation--Can Pre-Ablation Function Predict Contractile Improvement During Follow-up?

BACKGROUND: Data are lacking on the effect of radiofrequency catheter ablation (RFCA) on atrial function. The aim of this study was to determine a cut-off of pre-ablation left atrial (LA) function in order to predict atrial functional recovery after RFCA. METHODS AND RESULTS: A total of 64 atrial fibrillation (AF) patients who underwent RFCA were enrolled (age, 59.05±12.09 years; 36% persistent AF; LA volume 37.8±13.6 ml/m(2)). LA emptying fraction (LAEF), LA active fraction (LAAEF), LA passive emptying fraction (LAPEF) and LA expansion index (LAEI) were evaluated in sinus rhythm before and 48 h, 15 days, 1, 2, 3 and 9 months after ablation. LA function improvement was defined as any positive increase in LAEF compared with baseline. On univariate and multivariate analysis only baseline atrial function proved to be an independent predictor of LA function improvement after ablation (P=0.002; OR=0.001; 95% CI: 0.000-0.099). On receiver operating characteristic analysis (AUC=0.70), cut-off for baseline LAEF was 40%. At 9 months, patients with LAEF <40% had significant improvement in atrial performance (LAEF, P=0.01; LAAEF, P=0.036; LAEI, P=0.004); a significant negative correlation between baseline LAEF and LA function improvement was observed (r=-0.62; 95% CI: -0.83 to -0.26; P(r=0)<0.002). CONCLUSIONS: Baseline LAEF is an independent predictor of LA function recovery after RFCA. The beneficial effect of AF ablation is most evident in patients with LAEF<40%.

New echocardiographic insights in short QT syndrome: More than a channelopathy?

BACKGROUND: Short QT syndrome (SQTS) is a congenital ion channel disease characterized by an increased risk of sudden cardiac death. Little is known about the possibility that accelerated repolarization alters mechanical function in SQTS. OBJECTIVES: The study investigated the presence of left ventricular dysfunction and mechanical dispersion, assessed by tissue Doppler imaging (TDI) and speckle tracking echocardiography (STE), and their correlation with QT interval duration and genetics. METHODS: Fifteen SQTS patients (7 with HERG and 3 with KCNQ1 mutation) were studied. Electrocardiographic and echocardiographic parameters were compared with age- and sex-matched healthy controls. RESULTS: When compared to the control group, SQTS patients showed reduced left ventricular contraction (global longitudinal strain: -16.0% ± 3.4% vs -22.6% ± 1.7%, P < .001; myocardial performance index 0.59 ± 0.17 vs 0.34 ± 0.08, P < .001) and a higher incidence of ejection fraction <55% (odds ratio 11, 95% confidence interval 1.045-374, P = .04). Mechanical dispersion assessed by TDI (P < .01) and STE (P < .001) was higher in the SQTS group than in controls; each parameter showed a significant inverse correlation with QT interval but not with QT dispersion. CONCLUSION: This study showed that in SQTS systolic function may also be affected. SQTS patients presented a significant dispersion of myocardial contraction. TDI and STE could become part of the evaluation of this rare disease.

One-year follow-up of conduction disturbances following transcatheter aortic valve implantation.

AIMS: To describe the postprocedural and 1-year follow-up incidence of heart conduction disturbances in patients with severe symptomatic aortic stenosis undergoing transcatheter aortic valve implantation (TAVI). METHODS: Ninety-five patients were enrolled from 2008 to 2011 (mean age 81.8 ±â€Š7.2 years, 63.1% women). Clinical and ECG data were recorded at admission, discharge, and 3, 6 and 12 months following TAVI. RESULTS: Fifty-seven Edwards SAPIEN (31 transapical, 26 transfemoral) and 38 transfemoral CoreValve implants were used. Two (2.1%) patients died during the procedure and 11 (11.6%) patients received a pacemaker prior to discharge (7 CoreValve, 3 transapical, 1 transfemoral SAPIEN; P = 0.18). Among the surviving patients not receiving a pacemaker, TAVI increased the PQ interval (176 ±â€Š29 vs. 188 ±â€Š36 ms; P = 0.001), QRS width (90 ±â€Š15 vs. 108 ±â€Š26 ms; P < 0.001), and first-grade atrioventricular block (17 vs. 29%; P < 0.001). Postprocedural complete left bundle branch block was reported most in transapical (from 10 to 36%; P = 0.01) and CoreValve (from 8 to 64%; P < 0.001) recipients compared to the transfemoral SAPIEN group. At the 12-month follow-up, 24 (25.3%) patients had died [two (2.1%) sudden deaths] and four (4.2%) required pacemaker implantation. Among the survivors not receiving a pacemaker at the 1-year follow-up, the PQ interval (178 ±â€Š27 vs. 188 ±â€Š36 ms; P = 0.39) remained unchanged in the ECG, whereas the QRS width (100 ±â€Š22 vs. 108 ±â€Š26 ms; P = 0.008) decreased compared to measurements taken at discharge. CONCLUSION: Conduction disturbances following TAVI primarily develop during hospitalization and subsequently stabilize. However, the small percentage of patients suffering sudden death or pacemaker implantation requires attention.

Prediction of atrial fibrillation recurrence after cardioversion in patients with left-atrial dilation.

AIMS: Little is known about the impact of left-ventricular (LV) diastolic dysfunction on risk of atrial fibrillation (AF) recurrence in patients with left-atrial (LA) dilation. To evaluate, in patients with symptomatic persistent AF and LA dilation, the incremental role of LV diastolic dysfunction in predicting early AF recurrence after cardioversion (CV). METHODS AND RESULTS: From July 2011 to July 2013, 175 patients with persistent AF referred to our centre for CV were screened. Inclusion criteria were: European Heart Rhythm Association (EHRA) class ≥2 despite optimal medical treatment and heart rate at rest ≤80 bpm, LA volume ≥34 mL/m(2), EF > 35%, absence of untreated ischaemic disease and significant valvular disease, successful CV. Finally, 127 patients (age 64 ± 10 years, 60% EHRA ≥3, LA volume 42 ± 15 mL/m(2)) were enrolled. At 3 months, 37 (29%) patients presented AF recurrence. At univariate analysis, AF duration >90 days before CV (P < 0.01), septal e' <8 cm/s (P 0.03), and septal E/e' ratio ≥11 (P < 0.001) but no LA dimensions significantly correlated with AF recurrence. Logistic regression analysis confirmed septal E/e' ratio ≥11 as the best predictor of recurrence (OR 3.25 95% CI 1.19-8.86 P 0.001) together with an AF duration >90 days before the CV (OR 2.69 95% CI 1.01-7.53 P 0.04). At ROC curve analysis, the septal E/e' ratio ≥11 showed the best diagnostic accuracy (AUC 0.66, 95% CI 0.55-0.76, P 0.007). CONCLUSION: In this population with symptomatic persistent AF and LA enlargement, septal E/e' ratio ≥11 and AF duration >90 days predicted AF recurrence at 3 months.

Berberine behind the thriller of marked symptomatic bradycardia.

Patients with chronic aortic dissections are at high risk of catheter-induced complications. We report a Berberine is used in traditional Chinese medicine for the treatment of congestive heart failure, hypertension, diabetes, and dyslipidaemia and has a good safety profile. We report a case of a 53-year-old sportsman referred to our hospital for the onset of fatigue and dyspnoea upon exertion after he started berberine to treat hypercholesterolaemia. An electrocardiogram showed sinus bradycardia (45 bpm), first-degree atrioventricular block, and competitive junctional rhythm. An ergometric stress test showed slightly reduced chronotropic competence and the presence of runs of competitive junctional rhythm, atrial tachycardia, and sinus pauses in the recovery. After 10 d of wash-out from berberine, the patient experienced a complete resolution of symptoms, and an ergometric stress test showed good chronotropic competence. An electrocardiogram Holter showed a latent hypervagotonic state. This is the first case report that shows that berberine could present certain side effects in hypervagotonic people, even in the absence of a situation that could cause drug accumulation. Therefore, berberine's use should be carefully weighed in hypervagotonic people due to the drug's bradycardic and antiarrhythmic properties, which could became proarrhythmic, exposing patients to potential health risks.

Telomere length variation in juvenile acute myocardial infarction.

Leukocyte telomere length (LTL) provides a potential marker of biological age, closely related to the endothelial dysfunction and consequently to the atherosclerotic process. To investigate the relationship between the LTL and the risk of premature acute myocardial infarction and to evaluate the predictive value of LTL on the onset of major cardiovascular events, 199 patients from 18 to 48 years old with first diagnosis of acute myocardial infarction were enrolled and were matched with 190 controls for sex and age (± 1 year). Clinical data and coronary artery disease were evaluated at enrollment and at follow up. LTL was measured at enrollment using a quantitative PCR-based method. No significant differences were observed in LTL between cases and controls (p = 0.20) and with the presence of coronary artery disease in patients (p = 0.47). Hypercholesterolemic cases presented LTL significantly longer than cases without hypercholesterolemia (t/s: 0.82 ± 0.16 p = 0.79 and t/s norm: 0.79 ± 0.19 p = 0.01), as confirmed in multivariate regression analysis (p = 0.005, ß = 0.09). Furthermore, multivariate regression analysis showed LTL significantly shorter in hypertensive cases than in normotensive cases (p = 0.04, ß = -0.07). One hundred seventy-one cases (86%) ended the average follow up of 9 ± 5 years, 92 (54%) presented a major cardiovascular event. At multivariate regression analysis the LTL detected at enrollment did not represent a predictive factor of major cardiovascular events nor it significantly impacted with cumulative events. Based on present cohort of young Italian patients, the LTL did not represent a marker of acute myocardial infarction nor had a predictive role at medium term follow up.