Injecting a ventricular tachycardia into the heart-Α unique case report.

INTRODUCTION: A 52-year-old woman presented with a complex ventricular arrhythmia in an intraoperative context, during kyphoplasty for an osteoporotic fracture of a lumbar vertebra. The subject showed no indications of a previous cardiovascular condition. METHODS AND RESULTS: Causes of arrhythmias associated with the procedure were excluded. Due to her positive family history for dilated cardiomyopathy, upcoming thoughts were made for unmasking a previous asymptomatic cardiomyopathy. Nevertheless, an intracardiac cement embolism was diagnosed and, finally, the patient underwent an open-heart surgery with successful removal of the cardiac cement. Νo new arrhythmia recorded during follow up. CONCLUSION: To the best of our knowledge, this is the first reported case of ventricular arrhythmogenic presentation of a cardiac cement embolus after a KP procedure.

Cardiac Paraganglioma: Multimodality Imaging of a Rare Tumor.

Paragangliomas are rare tumors of chromaffin cells arising from an extra-adrenal location. Unlike pheochromocytomas, they are seldom functional. We present a case of pericardial paraganglioma incidentally encountered on an echocardiographic study, focusing on the characteristic features the tumor demonstrates on different imaging modalities. (Level of Difficulty: Intermediate.).

HEllenic Registry on Myocarditis SyndromES on behalf of Hellenic Heart Failure Association: The HERMES-HF Registry.

AIMS: Despite the existence of many studies, there are still limited data about the characteristics of myocarditis in Greece. This led to the creation of the Greek Myocarditis Registry aiming to document the different symptoms and treatment of myocarditis, assess possible prognostic factors, and find similarities and differences to what is already published in literature. This paper is a preliminary descriptive analysis of this Registry. METHODS AND RESULTS: We analysed data for the hospitalization period of all patients included in the Registry from December 2015 until November 2017. Statistics are reported as frequency (%) or median and inter-quartile range (IQR) as appropriate. In total, 146 patients were included; 83.3% of the patients reported an infection during the last 3 months. The most common symptom, regardless of the underlying infection, was chest pain (82.2%) followed by dyspnoea (18.5%), while the most common finding in clinical examination was tachycardia (26.7%). Presentation was more frequent in the winter months. ECG findings were not specific, with the repolarization abnormalities being the most frequent (60.3%). Atrial fibrillation was observed in two patients, both of whom presented with a reduced ventricular systolic function. Left ventricular ejection fraction changed significantly during the hospitalization [55% (IQR: 50-60%) on admission vs. 60% (IQR: 55-60%) on discharge, P = 0.0026]. Cardiac magnetic resonance was performed in 88 patients (61%), revealing mainly subepicardial and midcardial involvement of the lateral wall. Late gadolinium enhancement was present in all patients, while oedema was found in 39 of them. Only 11 patients underwent endomyocardial biopsy. Discharge medication consisted mainly of beta-blockers (71.9%) and angiotensin-converting enzyme inhibitors (41.8%), while 39.7% of the patients were prescribed both. CONCLUSIONS: This preliminary analysis describes the typical presentation of myocarditis patients in Greece. It is a first step in developing a better prognostic model for the course of the disease, which will be completed after the incorporation of the patients' follow-up data.

Troponin-I levels as a potential prognostic biomarker of sacubitril/valsartan treatment response in heart failure with reduced ejection fraction: Who will benefit most?

BACKGROUND: Despite robust data on the benefits of sacubitril/valsartan (LCZ696) in patients with chronic heart failure with reduced ejection fraction (HFrEF), there is no evidence yet on prespecified predictive markers of its efficacy. Hypothesis The objective of this study was to identify potential prognostic factors of LCZ696 treatment response. METHODS: We included 48 symptomatic patients with chronic HFrEF (left ventricular ejection fraction ≤35%) and New York Heart Association (NYHA) class II/III: Group A (N = 23) received LCZ696 (105 ± 30 mg twice daily), whereas it was not prescribed in group B (N = 25) according to physician's judgment. Analysis of biochemical parameters, cardiopulmonary exercise testing, and echocardiographic evaluation was performed at baseline and 6 months later. RESULTS: The baseline serum troponin-I levels (TnI) and peak oxygen uptake (VO2 max) were positively associated with the increase in VO2 max (ΔVO2 max = +14.11%, P < 0.05 vs group B) after sacubitril/valsartan treatment (r = 0.68, P = 0.001 and r = 0.57, P = 0.004, respectively). Positive correlations were reported between ΔVO2 max and the improvements in the ratio of early diastolic filling to myocardial tissue velocity (ΔE/E') and the tricuspid annular peak systolic velocity (ΔSa) in group A (r = 0.58, P = 0.004 and r = 0.60, P = 0.002, respectively). In multiple regression analysis, ΔVO2 max was correlated significantly with TnI (beta = 0.35, P = 0.048), ΔE/E' (beta = 0.36, P = 0.031) and ΔSa (beta = 0.37, P = 0.035). CONCLUSIONS: TnI levels may be an independent predictive marker of sacubitril/valsartan efficacy in HFrEF.

Adult-type ALCAPA syndrome: A rare coronary artery anomaly.

Anomalous origin of the left coronary artery arising from the pulmonary artery (ALCAPA or Bland-White-Garland syndrome) is a rare but serious congenital coronary artery anomaly, with a poor prognosis without surgical repair. There are two types of ALCAPA syndrome: infant type and adult type. We present a rare case of a 63-year-old female patient, with isolated left anterior descending artery origin from the pulmonary artery. Coronary computed tomography angiography revealed giant and tortuous coronary arteries with many collaterals between the left and right coronary system. The patient refused any surgical treatment.

Two-dimensional global and segmental longitudinal strain: are the results from software in different high-end ultrasound systems comparable?

To compare the peak global longitudinal myocardial strain (PGLS) and peak segmental longitudinal myocardial strain (PSLS) values by speckle-tracking echocardiography (STE) obtained using two different echocardiography devices. STE is an emerging quantitative ultrasound technique that allows an accurate evaluation of global and segmental myocardial function. However, there is a lack of standardization of the acquired data among different manufacturers. Sixty-three subjects, mean age 56.2±10.4 years, underwent complete echocardiographic studies with two different devices (Philips IE33 and General Electric VIVID E9) performed by the same operator. Thirty-one of them had known cardiac disease, with estimated left ventricular ejection fraction <50%, while 32 were free of any cardiovascular disease (control subjects). All images were digitally stored and analyzed using off-line post processing with QLAB 9 and EchoPAC 11 Software packages. PSLS and PGLS were calculated. A strong relationship between QLAB and EchoPAC was found for PGLS (r=0.91, P<0.001), PSLS-4 chamber (CH; r=0.79, P<0.001), PSLS-2CH (r=0.73, P<0.001), and PSLS-3CH (r=0.78, P<0.001) QLAB. Bland-Altman analysis showed absolute differences vs average of -0.16, -0.37, -0.21, and -0.16 for PGLS, PSLS-4CH, PSLS-2CH, and PSLS-apical long-axis views respectively. Segmental analysis showed a good agreement between the apical segments, whereas poor correlations were found for the basal segments. Receiver operating characteristic curve analysis showed that cutoff values for PGLS of -17.5 and -17.75% with Philips or GE systems gave a sensitivity and specificity of 93.5 and 87.5%, and 90 and 87.5%, respectively, in the discrimination of the patients from the controls. Both Philips and GE echo stations were found to give comparable results for PGLS, with approximately the same cutoff values, suggesting that their PGLS results may be interchangeable.

Alterations in the expression of genes related to contractile function and hypertrophy of the left ventricle in chronically paced patients from the right ventricular apex.

AIM: Long-term right ventricular apical (RVA) pacing may lead to left ventricular (LV) remodelling and heart failure. This study assessed changes in the expression of genes regulating LV contractile function and hypertrophy, after permanent RVA pacing and investigated whether such changes proceed or even predict LV remodelling. METHODS AND RESULTS: We enrolled 52 consecutive patients (age 79.1 ± 7.7 years, 34 males) who underwent pacemaker implantation for bradycardic indications: Group A, 24 individuals with atrioventricular conduction disturbances and group B, 28 patients with sinus node disease. In group A, peripheral blood mRNA levels of gene sarcoplasmic reticulum calcium ATPase decreased at 3, 6, and 12 months' follow-up, while α-myosin heavy chain (MHC) decreased and ß-MHC increased until 6 months follow-up. In this group, 25% of patients demonstrated significant LV remodelling. At 4 years, LV end-systolic diameter increased from 29.67 ± 3.39 mm at baseline to 35.38 ± 4.22 mm, LV end-diastolic diameter increased from 50 ± 4.95 to 56.71 ± 5.52 mm, and ejection fraction declined from 63.04 ± 10.22 to 52.83 ± 10.81%. Early alterations in gene expression were associated with a deterioration in LV function and geometry that became apparent months later. In group B, echocardiographic indexes and mRNA levels of the evaluated genes demonstrated no statistically significant changes. CONCLUSIONS: Permanent RVA pacing in patients with preserved ejection fraction is associated with alterations in the expression of genes regulating LV contractile function and hypertrophy, measured in the peripheral blood. These alterations are traceable at an early stage, before echocardiographic changes are apparent and are associated with LV remodelling that becomes evident in the long term.

Isolated left ventricular apical hypoplasia: a newly recognized unclassified cardiomyopathy.

We describe two relatively asymptomatic cases diagnosed with the newly recognized unclassified cardiomyopathy, isolated left ventricular apical hypoplasia. The disease has been described mainly in cardiac magnetic resonance, whereas this study presents the echocardiographic characteristics of this new cardiomyopathy.

Myocardial gene expression alterations in peripheral blood mononuclear cells of patients with idiopathic dilated cardiomyopathy.

AIMS: To assess cardiac gene expression in peripheral blood cells of patients with idiopathic dilated cardiomyopathy (IDCM) and its relationship to echocardiographic left ventricular (LV) function. METHODS AND RESULTS: A complete echocardiographic study and blood sampling were performed in 65 consecutive stable IDCM patients with LV ejection fraction (LVEF) 31.76 +/- 10.07% and chronic mild to moderate heart failure (NYHA functional class II to III) for > or =9 months. Blood samples from 19 healthy individuals were included for comparison. Transcript levels of myocardin, GATA4, alpha- and beta-myosin heavy chain (MHC), sarcoplasmic reticulum calcium ATPase 2 (SERCA2), and phospholamban were determined by quantitative real-time reverse transcription-polymerase chain reaction. Myocardin (24.88 +/- 4.93 vs. 3.98 +/- 1.12, P = 0.0048) and GATA4 (17.85 +/- 4.85 vs. 0.45 +/- 0.15, P = 0.0069 x 10(-5)) were upregulated in IDCM patients compared with controls, whereas SERCA2 (5.11 +/- 0.42 vs. 8.93 +/- 1.07, P = 0.001) was downregulated. In IDCM patients, myocardin (r = 0.279, P = 0.025), GATA4 (r = 0.314, P = 0.011), beta-MHC (r = 0.444, P=0.0002), and alpha-MHC (r = 0.272, P = 0.034) showed positive correlations, whereas SERCA2 (r = -0.264, P = 0.034) exhibited a negative correlation with LVEF. Patients with elevated LV filling pressures had lower myocardin (15.06 +/- 3.10 vs. 43.12 +/- 12.03, P = 0.048), GATA4 (8.96 +/- 2.17 vs. 34.38 +/- 12.60, P = 0.026), beta-MHC (10.59 +/- 4.05 vs. 16.43 +/- 4.91, P = 0.013), and alpha-MHC (0.27 +/- 0.08 vs. 0.79 +/- 0.20, P = 0.033) and higher SERCA2 (5.65 +/- 0.54 vs. 3.90 +/- 0.61, P = 0.037) levels. Patients with atrial fibrillation (AF) had higher SERCA2 levels compared with sinus rhythm patients (6.75 +/- 0.84 vs. 4.54 +/- 0.45, P = 0.017). CONCLUSION: Our data indicate that cardiac gene expression alterations in peripheral blood cells of IDCM patients may reflect alterations in LV function, whereas the presence of AF may be associated with increased SERCA2 levels in these patients.

Quadricuspid aortic valve associated with non-obstructive sub-aortic membrane: a case report and review of the literature.

Quadricuspid aortic valve (QAV) is a rare congenital cardiac entity. The recognition of QAV has clinical significance because QAV causes aortic valve dysfunction, commonly aortic regurgitation. QAV is often associated with other congenital cardiac abnormalities. Our case represents, to the best of our knowledge, the first reported case of QAV with a non-obstructive sub-aortic membrane.

Central aortic stiffness in patients with nonischemic dilated cardiomyopathy: relationship with neurohumoral activation.

BACKGROUND: Increased aortic stiffness has been found in heart failure (HF), but the underlying mechanisms remain to be elucidated. The aim of the present study was to examine the association between aortic stiffness and neurohumoral activation in patients with nonischemic dilated cardiomyopathy (NIDC). METHODS AND RESULTS: We examined 101 patients with NIDC, New York Heart Association Class II-III, LVEF 33.3 +/- 11.6%, and 33 controls. All subjects underwent blood sampling for plasma concentrations of renin, aldosterone, C-reactive protein (CRP), and brain natriuretic peptide (BNP). We evaluated the pulse wave velocity (PWV) of the proximal aorta in the region of the aortic arch with a new echo application. Patients showed increased PWV (P < .001), and increased plasma levels of log-renin (P < .001), log-aldosterone (P = .01), CRP (P = .01), and log-BNP (P = .01) compared with controls. PWV was correlated with log-BNP (r = 0.63, P < .001) and log-aldosterone (r = 0.34, P < .001) levels, with LV end-diastolic (r = 0.27, P = .01) and end-systolic (r = 0.33, P = 0.003) volumes, and the PW-tissue Doppler imaging systolic wave (r = -0.27, P = .006) and the E/e' ratio (r = 0.45, P < .001). Linear regression analysis showed that log-BNP levels were independently associated with PWV. CONCLUSIONS: In patients with HF from NIDC, there is evidence of increased aortic stiffness that is correlated with LV shape and function. Although aldosterone levels seem to influence the aortic PWV, BNP levels are the best independent predictor of increased PWV.

Prosthetic aortic valve endocarditis complicated with annular abscess, sub-aortic obstruction and valve dehiscence.

We present a 76-year-old woman with infective endocarditis of a prosthetic aortic valve. The course of her illness started with an ischaemic stroke and she was admitted with prolonged fever and an episode of loss of consciousness. Echocardiography revealed acute aortic regurgitation and dehiscence of the prosthetic valve with excessive "rocking motion", aortic abscesses and left ventricular outflow obstruction caused by a semilunar shelf of tissue probably due to endocarditis vegetations. She underwent an urgent surgical procedure that confirmed the echocardiographic findings. Our case report reinforces the value of early diagnosis in the presence of a high clinical suspicion of prosthetic valve endocarditis. An extended workup, including transoesophageal echocardiography, in such a patient with a mechanical valve is mandatory.

Proximal aortic stiffness is related to left ventricular function and exercise capacity in patients with dilated cardiomyopathy.

AIMS: Patients with heart failure (HF) show abnormal arterial stiffening. METHODS AND RESULTS: We examined 60 patients (52.1 +/- 12, 8 years) with non-ischaemic dilated cardiomyopathy (NIDC), New York Heart Association II-III, in sinus rhythm, left ventricular ejection fraction 30.1 +/- 8.6%, and 44 normals. All subjects underwent an echocardiographic study and a cardiopulmonary exercise test. We evaluated the segmental proximal aorta (AO) pulse wave velocity (PWV) in the region of aortic arch with a new echo-method: from the suprasternal view, the distance between ascending and descending AO was measured with two-dimensional ultrasound, and the aortic flow wave transit time (TT) was measured with pulsed-wave Doppler. Pulse wave velocity was calculated as aortic distance/TT. Patients showed increased PWV (7.4 +/- 2.9 vs. 4.8 +/- 1.1 m/s, P < 0.001), compared with controls. Patients with advanced left ventricular (LV) (restrictive or pseudo-normal filling pattern) diastolic dysfunction showed increased PWV (8.6 +/- 2.6 vs. 6.6 +/- 2.9 m/s, P = 0.01) and reduced peak and predicted (for age, sex, and body mass) VO(2) (both P < 0.001), compared with those with mild diastolic dysfunction (delayed relaxation filling pattern). Pulse wave velocity was significantly correlated with the LV mass (r = 0.32, P = 0.01), the peak spectral tissue Doppler imaging systolic wave (r = -0.34, P = 0.006), the LV diastolic filling pattern (r = 0.42, P = 0.001), and the peak (r = -0.47, P < 0.001) and predicted VO(2) (r = -0.579, P < 0.001). CONCLUSION: Patients with NIDC showed increased proximal aortic stiffness, which relates to LV systolic and diastolic function and exercise capacity. The echocardiographic assessment of the regional aorta PWV seems to be clinically important.

CRT and exercise capacity in heart failure: the impact of mitral valve regurgitation.

Cardiac resynchronization therapy (CRT) has emerged as an established therapy for patients with end-stage heart failure (HF) and symptoms refractory to optimal medication. Resynchronization of the ventricles leads to reduced functional mitral regurgitation (MR) both acutely and chronically and to a better haemodynamic and energetic profile. In addition, large clinical trials have confirmed its beneficial effect on exercise performance and quality of life. The link between the pathophysiological mechanisms of CRT and its positive effect on clinical status has not been fully elucidated. Therefore, this review will focus on the effect of CRT on the haemodynamic role of functional MR and its impact on exercise performance in patients with chronic HF.

Noncompaction myocardium imaging with multiple echocardiographic modalities.

Noncompaction myocardium of the left ventricle (LV) is a genetically heterogeneous congenital cardiomyopathy, that has only been described during the last two decades. Echocardiography plays a pivotal role as a first line diagnostic tool of this rare abnormality. The present case describes the imaging of the noncompacted myocardium using varying echocardiographic modalities.

Right atrial myxoma: echocardiographic appearance.

A 67-year-old asymptomatic male was admitted for evaluation of his arterial hypertension. The routine echocardiographic study revealed a large tumour in the dilated right atrium. The mass appeared to arise from the posterior wall of the right atrium. After infusion of a contrast agent, the mass appeared to fill with the contrast agent, ruling out the possibility of the mass being a clot. Transesophageal study revealed a round mass arising from the posterior right atrial wall just adjacent to the extrusion of the superior vena cava. The patient subsequently underwent cardiac surgery and a cardiac tumour was excised that proved to be a cardiac myxoma. In this case we present echocardiographic images and the macro- and microscopic view of the right atrial myxoma.

NT-proBNP response to dobutamine stress echocardiography predicts left ventricular contractile reserve in dilated cardiomyopathy.

BACKGROUND: Brain natriuretic peptide (BNP) and left ventricular (LV) inotropic reserve are major prognostic indexes in heart failure (HF). AIMS: To investigate the relationship between N-terminal-proBNP (NT-proBNP) changes in response to dobutamine stress echocardiography (DSE) and the LV inotropic reserve, in HF patients with dilated cardiomyopathy (DC). METHODS: We studied 41 patients with DC, LVEF 31.6+/-7.7%, NYHA class II-III and 15 controls. Plasma NT-proBNP levels were measured before and 60 min after three 5-min stages of dobutamine (5 to 15 microg/kg/min). RESULTS: Based on NT-proBNP changes in response to dobutamine, patients were categorized into two groups: In Group A circulating NT-proBNP levels fell (-16.6+/-7.8%), and in Group B they increased (8.4+/-9.1%). Group A had a marked improvement in WMSI compared to Group B (32.1+/-9.7% vs. 18.8+/-15.9%, p<0.001). Multivariate analysis showed that NT-proBNP changes were an independent predictor of LV inotropic reserve (b= -0.55, p<0.001). A reduction of 21.3% in plasma NT-proBNP levels in response to dobutamine predicted an improvement in WMSI of >25% with a sensitivity of 100% and a specificity of 92.3%. CONCLUSIONS: NT-proBNP changes in response to dobutamine reflect improvement in LV contractility and constitute an independent predictor of LV inotropic reserve in patients with DC.

Myocardial sympathetic innervation in patients with impaired glucose tolerance: relationship to subclinical inflammation.

AIM: This study was designed to assess cardiac adrenergic nerve activity, using iodine (I)-123-labeled metaiodobenzylguanidine (MIBG), in patients with impaired glucose tolerance (IGT) and to investigate its relation to circulating levels of proinflammatory cytokines. METHODS: We studied 22 patients with IGT (aged 34-68 years) and 18 age-matched healthy controls, using I-123 MIBG cardiac imaging. The early (10 min) and late (4 h) heart to mediastinum MIBG uptake (H/M) ratio and washout were calculated. Levels of interleukin-6 (IL-6), tumor necrosis factor-alpha (TNF-a), and its soluble receptor [soluble TNF receptor II (sTNFRII)] were measured by immunoassay of blood samples from patients and controls. RESULTS: The early and late MIBG uptake was lower (both P<.001) and the WR was higher (P<.001) in patients than in controls. The analysis showed innervation defects in 20 of the 22 patients. Nearly half (45.4%) showed severe adrenergic innervation defects in both the inferior wall and the apex. Regarding cytokines, patients showed significantly elevated TNF-a (P=.005), sTNFRII (P<.001), and IL-6 (P<.001) levels compared to controls. IL-6 and sTNFRII were found to correlate with the WR (r=0.468, P=.028 and r=0.455, P=.034, respectively). CONCLUSION: Patients with IGT show reduced MIBG cardiac uptake with a segmental pattern. The reduced cardiac sympathetic innervation was related to the elevated proinflammatory cytokine levels and could be considered an index of early atherosclerotic process in these patients.

Altered expression of early cardiac marker genes in circulating cells of patients with hypertrophic cardiomyopathy.

BACKGROUND: Early cardiac marker genes, such as cardiac-specific homeobox (Csx/Nkx2.5), myocardin, homeodomain only protein, GATA4, and myocyte enhancer factor 2C, are thought to participate in cardiomyocyte differentiation and to contribute to heart hypertrophy in animal models. In this study, we investigated whether the expression of early cardiac genes is altered in the peripheral blood of patients with hypertrophic cardiomyopathy. METHODS: Peripheral blood mononuclear cells were isolated from 30 consecutive hypertrophic cardiomyopathy patients and 20 healthy controls, and gene expression was determined by quantitative real-time reverse transcription-polymerase chain reaction. RESULTS: Csx/Nkx2.5, myocardin, and GATA4 expressions were significantly higher in hypertrophic cardiomyopathy patients by 5.14+/-0.89 (P<.001), 1.65+/-0.21 (P<.05), and 2.04+/-0.41 (P<.04) times, respectively, while homeodomain only protein showed a fourfold decrease in expression (P<.02) compared to controls. In addition, expression of the differentiation-specific marker genes beta-myosin heavy chain and smooth muscle myosin heavy chain was significantly higher in hypertrophic cardiomyopathy patients by 3.72+/-0.82 (P<.02) and 2.57+/-0.72 (P<.05) times, respectively, compared to controls. Myocyte enhancer factor 2C expression was not different between patients and controls. Furthermore, increased expression of GATA4, myocardin, and beta-myosin heavy chain positively correlated with increased left ventricular mass. CONCLUSIONS: In conclusion, we found altered expressions of early cardiac marker genes and differentiation-specific marker genes in peripheral blood mononuclear cells of hypertrophic cardiomyopathy patients compared to control individuals, possibly reflecting changes in response to disease.