Giant isolated intracardiac thrombus presenting as acute heart failure secondary to right ventricular outflow tract obstruction in a patient with renal carcinoma.

Right-sided intracardiac thrombi are potential causes of right ventricular (RV) failure, particularly when tricuspid or pulmonary obstruction occurs. In most cases, RV thrombus develops in patients with RV dysfunction and concomitant thrombosis in the systemic veins. However, RV thrombosis can rarely present as an isolated mass and despite preserved RV function, particularly in patients with thrombophilic states. In this report, we describe an unusual case of giant isolated RV thrombus presenting with acute RV failure secondary to dynamic RV outflow tract obstruction in a patient with renal carcinoma. Bedside echocardiography allowed a rapid assessment of the hemodynamic effects of the mass. The possibility of a thrombotic RV outflow obstruction should be considered in patients with acute RV failure, even in those with no evidence of thrombosis in the venous district. This may be particularly important in patients with prothrombotic states, where the effectiveness of routine thromboembolic prophylaxis could be reduced.

Prognostic comparison between creatinine-based glomerular filtration rate formulas for the prediction of 10-year outcome in patients with non-ST elevation acute coronary syndrome treated by percutaneous coronary intervention.

BACKGROUND:: Estimated glomerular filtration rate (eGFR) is a predictor of outcome among patients with non-ST-elevation acute coronary syndrome (NSTE-ACS), but which estimation formula provides the best long-term risk stratification in this setting is still unclear. We compared the prognostic performance of four creatinine-based formulas for the prediction of 10-year outcome in a NSTE-ACS population treated by percutaneous coronary intervention. METHODS:: In 222 NSTE-ACS patients submitted to percutaneous coronary intervention, eGFR was calculated using four formulas: Cockcroft-Gault, re-expressed modification of diet in renal disease (MDRD), chronic kidney disease epidemiology collaboration (CKD-Epi), and Mayo-quadratic. Predefined endpoints were all-cause death and a composite of cardiovascular death, non-fatal reinfarction, clinically driven repeat revascularisation, and heart failure hospitalisation. RESULTS:: The different eGFR values showed poor agreement, with prevalences of renal dysfunction ranging from 14% to 35%. Over a median follow-up of 10.2 years, eGFR calculated by the CKD-Epi and Mayo-quadratic formulas independently predicted outcome, with an increase in the risk of death and events by up to 17% and 11%, respectively, for each decrement of 10 ml/min/1.73 m2. The Cockcroft-Gault and MDRD equations showed a borderline association with mortality and did not predict events. When compared in terms of goodness of fit, discrimination and calibration, the Mayo-quadratic outperformed the other formulas for the prediction of death and the CKD-Epi showed the best performance for the prediction of events (net reclassification improvement values 0.33-0.35). CONCLUSIONS:: eGFR is an independent predictor of long-term outcome in patients with NSTE-ACS treated by percutaneous coronary intervention. The Mayo-quadratic and CKD-Epi equations might be superior to classic eGFR formulas for risk stratification in these patients.

A Spiral in the Heart: Mitral Valve Endocarditis with Unusual Vegetation Shape Potentially Affecting Effectiveness of Antibiotic Therapy.

We report an unusual case of infective endocarditis (IE) in an 88-year-old woman, occurring on a prolapsing mitral valve and characterized by an atypical vegetation shape resembling a spiral-like appearance. After the patient refused surgical correction, persistent IE despite prolonged antibiotic therapy was observed, resulting in an ischemic stroke probably secondary to septic embolus. The importance of vegetation shape in the management of patients with IE was classically related to the increased risk of embolization associated with pedunculated, irregular, and multilobed masses. We hypothesize that the unusual spiral-like vegetation shape in our patient may have favored IE persistence by two mechanisms, namely, a decrease of the exposed vegetation surface with creation of an internal core where the penetration of antimicrobial agents was obstacled and the creation of blood turbulence within the vegetation preventing a prolonged contact with circulating antibiotics. These considerations suggest that vegetation shape might be considered of importance in patients with IE not only because of its classical association with embolization risk, but also because of its potential effect on the efficacy of antibiotic therapy.

Nightmare-induced atypical midventricular tako-tsubo cardiomyopathy.

Tako-Tsubo cardiomyopathy (TTC) is a reversible cardiomyopathy characterized by acute left ventricular segmental dysfunction, whose clinical presentation resembles that of acute myocardial infarction. The syndrome often follows a psychophysical stressful event and is characterized by echocardiographic evidence of akinesia of the left ventricular mid-apical segments. Atypical echocardiographic patterns of TTC have recently been described, often triggered by emotional stressors, rather than physical. In this report, we describe a case of atypical TTC triggered by an unusual stressor (recurrent nightmare) in a 45-year-old woman, with peculiar clinical presentation and evolution characterized by persistent loss of consciousness, neurological deterioration, absence of typical symptoms of TTC, and features suggestive of a hysterical crisis.

Advanced therapies in patients with congenital heart disease-related pulmonary arterial hypertension: results from a long-term, single center, real-world follow-up.

Pulmonary arterial hypertension (PAH) is a common finding in patients with congenital heart disease (CHD), and has relevant prognostic implications. The recent introduction of advanced therapies (AT) considerably improved the clinical outcome of these patients, but real-world data are still lacking. We aimed at reporting the results of a long-term follow-up of CHD patients with PAH undergoing AT, followed at a tertiary Center during the two last decades. The study population included a total of 34 patients with an established diagnosis of CHD-related PAH. In addition to conventional treatment, 97% of patients started AT during the follow-up. Over a median follow-up of 9 [3-31] years, 11 (32.4%) patients died: 7 of them were affected by Eisenmenger syndrome and the majority of patients were in NYHA class ≥3 at the time of death. Among the 23 patients who were alive at the last follow-up, the majority were in NYHA class I-II. Oxygen saturation and 6-min walking distance improved in all subjects within the first 6 months after starting of AT. One patient with ventricular septum defect and high pulmonary resistances was successfully treated with AT to lower resistances and underwent defect closure. A good clinical outcome was also observed in the subset (n = 8) with Down syndrome. The results of this real-world experience suggest that, despite a relatively high mortality rate mostly related to late commencement of AT, the clinical outcome of subjects with CHD-related PAH undergoing AT are characterized by a good quality of life and clinical improvement in most patients.

Determinants and Regression Equations for the Calculation of z Scores of Left Ventricular Tissue Doppler Longitudinal Indexes in a Healthy Italian Pediatric Population.

Aim. We investigated the predictors of tissue Doppler left ventricular (LV) longitudinal indexes in a healthy Italian pediatric population and established normative data and regression equations for the calculation of z scores. Methods and Results. A total of 369 healthy subjects aged 1-17 years (age of 6.4 ± 1.1 years, 49.1% female) underwent echocardiography. LV peak longitudinal velocity at systole (s (')), early diastole (e (')), and late diastole (a (')) was determined by tissue Doppler. The ratio of peak early diastolic LV filling velocity to e (') was calculated. Age was the only independent determinant of s (') (ß = 0.491, p < 0.0001) and the strongest determinant of e (') (ß = 0.334, p < 0.0001) and E/e (') (ß = -0.369, p < 0.0001). Heart rate was the main determinant of a (') (ß = 0.265, p < 0.0001). Male gender showed no effects except for a weak association with lateral s ('), suggesting no need of gender-specific reference ranges. Age-specific reference ranges, regression equations, and scatterplots for the calculation of z scores were determined for each index. Conclusion. In a pediatric Italian population, age was the strongest determinant of LV longitudinal dynamics. The availability of age-specific normality data for the calculation of z scores may allow for correctly detecting LV dysfunction in pediatric pathological populations.

Clinical outcome, valve dysfunction, and progressive aortic dilation in a pediatric population with isolated bicuspid aortic valve.

The aim of this study was to explore the medium-term clinical outcome and the risk of progression of aortic valve disease and aortic dilation in pediatric patients with isolated bicuspid aortic valve (BAV). 179 pediatric patients with isolated BAV were prospectively followed from January 1995 to December 2010. Patients with severe valve dysfunction at baseline were excluded. Clinical outcome included cardiac death, infective endocarditis, aortic complications, cardiac surgery and percutaneous valvuloplasty. Echocardiographic endpoints were: progression of aortic stenosis (AS) or regurgitation (AR) and progressive aortic enlargement at different levels of the aortic root, evaluated as z-score. The median age at diagnosis was 7.8 [2.7-12.0] years. After a median followup of 5.4 [2.3-9.2] years, all patients were alive. The clinical endpoint occurred in 4 (2.2 %) patients (0.41 events per 100 patient-years). A progression of AS and AR was observed in 9 (5.0 %) and 29 (16.2 %) patients, respectively. The z-scores at the end of follow-up were not significantly different from baseline at the annulus, Valsalva sinuses and sinotubular junction, whereas a slight increase was observed at the level of the ascending aorta (1.9 vs 1.5, p = 0.046). Significant progressive aortic dilation occurred in a minority of patients (10.6, 5.6, 9.5, and 19.0 % respectively). The clinical outcome in pediatric patients with isolated BAV is favourable and the progression of aortic valve dysfunction and aortic dilation is relatively slow. These findings may be taken into account to better guide risk assessment and clinical follow-up in these patients.

Subclavian steal syndrome presenting as recurrent pulmonary oedema associated with acute left ventricular diastolic dysfunction.

Subclavian steal syndrome typically presents as angina in patients with internal mammary artery grafts. Atypical clinical presentations have been rarely described. We report an unusual case of subclavian steal syndrome presenting as pulmonary oedema with acute left ventricular diastolic dysfunction and preserved ejection fraction in a patient with internal mammary artery graft and severe stenosis of the proximal left subclavian artery. After successful angioplasty and stenting of subclavian artery, the patient remained asymptomatic for six months, but then experienced acute diastolic dysfunction and recurrent pulmonary oedema associated with critical subclavian in-stent restenosis with stent deformation. This report points out that, in patients with internal mammary-to-LAD grafts, subclavian steal syndrome may present as acute left ventricular diastolic dysfunction and pulmonary oedema even in the presence of normal ejection fraction.