Nailfold videocapillaroscopy: a novel possible surrogate marker for the evaluation of peripheral microangiopathy in pulmonary arterial hypertension.

Nailfold videocapillaroscopy (NVC) changes in systemic sclerosis (SSc) are correlated with vascular complications, such as pulmonary arterial hypertension (PAH), supporting a potential link between peripheral and internal organ vasculopathy. The current stage of knowledge regarding NVC and PAH is discussed, focusing on the assessment of peripheral microangiopathy and a potential relationship with functional, echocardiographic, and haemodynamic markers of cardiac dysfunction. A comprehensive literature search was carried out to identify all studies focusing on NVC findings in patients with PAH, diagnosed with right heart catheterization. The majority of the studies examined NVC findings in patients with SSc-PAH, while three studies reported NVC abnormalities in patients with idiopathic PAH. Besides the pulmonary vasculature, a systemic component of microangiopathy seems to be involved in PAH. Well-designed prospective trials are warranted to validate NVC as a biomarker, with clinical implications in the diagnostic evaluation, risk stratification, and overall management of PAH in the daily clinical setting.

Impact of stent implantation on endothelial shear stress.

BACKGROUND: Endothelial shear stress (ESS) may play a key role in the pathobiology of stent restenosis (SR). Nevertheless, limited data are available about ESS and its relation to SR. PATIENTS AND METHODS: We enrolled 14 patients who underwent successful percutaneous coronary intervention (PCI) in this study. Three-dimensional (3D) reconstruction of 14 coronary arteries before and after stent implantation was performed. Using computational fluid dynamics, mean ESS was calculated proximally, in tertiles within and distal to the stent, both before and after stent implantation. RESULTS: Stent implantation resulted in a significant ESS decrease in the entire atherosclerotic lesion (1.83 vs. 1.26 Pa, p = 0.02). Regarding the five territories in which the entire lesion was divided, ESS decrease was marginally significant in the area of the second in-stent tertile, and in the area 5 mm distal to the stent, whereas ESS decrease was not significant in the area 5 mm proximal to the stent, and in the area of the first and third in-stent tertile. At 12 months, two patients had SR, but restenosis was not related to ESS decrease. CONCLUSION: ESS decreases after stent implantation but not uniformly, with the major reduction being in the middle tertile of the stent, and distal to the stent. In-stent ESS decrease may create local hemodynamic conditions leading to in-stent and in-segment restenosis.

Aortic balloon valvuloplasty before transcatheter valve replacement in high-risk patients with aortic stenosis. Cardiac catheterization and echocardiographic hemodynamic study.

BACKGROUND: Balloon aortic valvuloplasty (BAV) has been revived as a bridge to transcatheter aortic valve replacement (TAVR). The aim of the current prospective study was to define a safe time period from BAV to TAVR and to determine hemodynamic variables that predict event-free survival after BAV. PATIENTS AND METHODS: The present prospective study included 68 consecutive patients with severe aortic stenosis who were treated initially with BAV from 2009 to 2012. Echocardiographic and invasive hemodynamic assessments were performed before BAV. The patients were followed up at regular intervals and events were defined as cardiac hospitalization or death. RESULTS: Invasive hemodynamic evaluation yielded more favorable results than echocardiographic assessment: aortic stenosis was less severe, cardiac output was higher, and pulmonary capillary wedge pressure (PCWP) was lower. Post-BAV event-free survival was 80.4 % at 30 days, 64.5 % at 6 months, 37 % at 1 year, 22.3 % at 2 years, and 9.3 % at 3 years. After excluding pre-discharge deaths (n = 7), the 30-day event-free survival rate was 90 %. Predictors of events after BAV were atrial fibrillation, cardiogenic shock, elevated euroSCORE (European System for Cardiac Operative Risk Evaluation), elevated PCWP, and elevated pulmonary artery systolic pressure. Invasively measured PCWP was the only independent predictor of events (hazard ratio, 1.07; 95 % confidence interval, 1.03-1.11; p = 0.001). CONCLUSION: A 30-day post-BAV period may be considered a bridge to TAVR. Furthermore, invasive assessment of PCWP before BAV is an independent hemodynamic predictor of events after BAV.

Myocardial deformation assessment in patients with precapillary pulmonary hypertension: A cardiac magnetic resonance study.

PURPOSE: The purpose of this study was to investigate right atrial and ventricular strain parameters on cardiac magnetic resonance (CMR) in patients with precapillary pulmonary hypertension (PPH) and whether they can aid in the assessment of PPH prognosis. MATERIALS AND METHODS: Adult patients with groups 1 and 4 PPH were invited to participate in the study. Age- and sex-matched healthy volunteers were also recruited as controls. At baseline, patients underwent clinical examination, N-terminal pro-B-type natriuretic peptide measurement and CMR with feature tracking post-processing (CMR-FT). Healthy controls underwent only CMR-FT. The study's primary endpoint was clinical failure, defined as death, hospitalization or demonstrable clinical deterioration during follow-up. Patients who were unable to perform 6-minute walking test due to musculoskeletal disorders were excluded from the study. RESULTS: Thirty-six patients (8 men, 28 women; mean age, 50.6±13.8 [SD] years [range: 18.6-78.5years]) and 12 healthy control subjects (5 mean, 7 women; mean age, 40.6±13.5 [SD] years [range: 23.1-64.4years]) were recruited. Right ventricular global longitudinal strain (GLS) was significantly impaired in PPH patients (-20.2±5.3 [SD] % [range: -28.8 to -9.1%] vs. -28.4±3.1% [-33.7 to -22.7%] respectively, P<0.001). The right atrial GLS was significantly impaired in PPH compared to healthy controls (-19.9±4.5% [range: -28.6 to -3.6%] vs. -26.5±4.2% [range: -32.8 to -15.8%] respectively) (P<0.001). Clinical failure occurred in 19 (19/36, 53%) of patients. Right ventricular GLS predicted clinical failure most reliably among CMR parameters (-22.6±3.8 [SD] % [range: -27.6 to -12.7%] for patients without clinical failure vs. -18±5.6 [SD] % [range: -28.8 to -9.1%] for patients with clinical failure; hazard ratio [HR]=1.85; P=0.007; area under the AUC curve=0.75). Lower absolute right atrial GLS was significantly associated with clinical failure (-22.7±3.0 [SD] % [range: -28.6 to -17.7%] for patients without clinical failure vs. -16.9±5.8 [SD] % [range: -24.2 to -3.6%] for patients with clinical failure) (HR=1.53; P=0.035). CONCLUSION: CMR feature tracking-derived myocardial strain parameters of both the right atrium and ventricle can assist clinicians in the prognosis of PPH.

Left ventricular function in elite rowers in relation to training-induced structural myocardial adaptation.

To examine left ventricular (LV) function in elite young athletes in relation to structural adaptation to prolonged intense training. Conventional echocardiography and tissue Doppler imaging (TDI) were performed in 15 elite rowers and 12 sedentary matched controls. Rowers had increased LV mass index, septal (12 vs 10 mm, P<0.005) and posterior wall thicknesses (12 vs 9 mm, P<0.001) and increased relative wall thickness. Septal and lateral systolic velocities were enhanced in rowers (septal S(m)=8.5 vs 6.3 cm/s, P<0.001; lateral S(m)=11.4 vs 8.0 cm/s, P<0.005), representing a 35% and 42% increase, respectively. Similarly, septal and lateral early diastolic velocities were enhanced (septal E(m)=12.1 vs 9.5 cm/s, P<0.01; lateral E(m)=16.6 vs 11.6 cm/s, P<0.001), representing a 27% and 43% increase, respectively. Systolic and early diastolic TDI velocities of the lateral wall showed a positive correlation (r=0.65, P<0.01) in athletes indicating a parallel improvement of systolic and diastolic function, while LV stiffness was decreased [(E/E(m))/(LV end-diastolic diameter)=1.13 vs 1.57, P<0.005). Both systolic and diastolic LV function were improved in elite rowers, despite a pattern of concentric hypertrophy.

Left ventricular diastolic dysfunction in idiopathic pulmonary fibrosis: a tissue Doppler echocardiographic [corrected] study.

It was hypothesised that, apart from right ventricular (RV) dysfunction, patients with idiopathic pulmonary fibrosis (IPF) also exhibit left ventricular (LV) impairment, which may affect disease progression and prognosis. The aim of the present study was to evaluate LV performance in a cohort of IPF patients using conventional and tissue Doppler ECG. IPF patients exhibiting mild-to-moderate pulmonary arterial hypertension (mean age 65+/-9 yrs; n = 22) and healthy individuals (mean age 61+/-6 yrs; n = 22) were studied. Conventional and tissue Doppler ECG were used for the evaluation of RV and LV systolic and diastolic function. In addition to the expected impairment in RV function, all patients showed a characteristic reversal of LV diastolic filling to late diastole compared with controls (early diastolic peak filling velocity (E)/late diastolic peak filling velocity 0.7+/-0.2 versus 1.5+/-0.1, respectively). Patients with IPF also exhibited lower peak myocardial velocities in early diastole (E(m); 5.7+/-1.1 versus 10.3+/-1.6 cm x s(-1), respectively), higher in late diastole (A(m); 8.9+/-1.3 versus 5.5+/-0.8 cm x s(-1), respectively), lower E(m)/A(m) ratio (0.6+/-0.1 versus 1.9+/-0.5, respectively) and higher E/E(m) ratio (10.8+/-3 versus 6+/-0.6, respectively), all indicative of LV diastolic dysfunction. Moreover, LV propagation velocity was significantly lower in IPF patients (46+/-13 versus 83+/-21 cm x s(-1), respectively). Physicians should be aware that patients with idiopathic pulmonary fibrosis exhibit early impairment of left ventricular diastolic function.

Asymmetrical dimethylarginine in systemic sclerosis-related pulmonary arterial hypertension.

OBJECTIVES: SSc is a CTD characterized by vascular involvement, with generalized disturbance of the microcirculation, which may lead to pulmonary artery hypertension (PAH). Asymmetrical dimethylarginine (ADMA) is an endogenous nitric oxide (NO) inhibitor. Increased concentrations of plasma ADMA may also contribute to endothelial dysfunction in patients with pulmonary vascular disease. The aim of our study was to elucidate the possible relationship between serum ADMA and PAH in patients with SSc. Moreover, we sought to investigate the effect of ADMA levels on the functional capacity of these patients. METHODS: Plasma ADMA levels were measured in 66 patients with SSc (63 females, mean age 57.8 +/- 12.8 yrs, median duration of disease 9.9 yrs, 47 with lcSSc and 19 with dcSSc disease) and 30 healthy controls (29 females, mean age 58.2 +/- 8.4 yrs). Systolic pulmonary artery pressure (sPAP) assessed by echocardiography, lung function tests, 6-min walk test (6MWT) and serum ADMA levels were recorded from patients. RESULTS: In 24 patients, the diagnosis of PAH was established. Mean value of ADMA for SScPAH patients was 0.44 +/- 0.22 micromol/l compared with 0.26 +/- 0.18 micromol/l for patients without PAH and 0.25 +/- 0.20 micromol/l for controls (P = 0.001). ADMA levels were inversely correlated with the 6MWT (r = -0.55, P = 0.005). CONCLUSIONS: In patients with SScPAH, increased ADMA serum levels and their negative association with exercise capacity suggests that the NO pathway is involved in the development of pulmonary vascular disease.

Renal cell carcinoma with rhabdoid features. Divergent differentiation of conventional (clear cell) carcinoma.

A rare case of renal cell carcinoma (RCC) with rhabdoid features in an adult man is presented. This tumor with rhabdoid features, also known as composite neoplasm, represents a pattern of progression emerging from specific histologic RCC types. RCC with rhabdoid features is a highly aggressive neoplasm and its malignant behavior may be due to the high-cell proliferation activity of the rhabdoid areas. Rhabdoid features in RCC may represent a final pathway of differentiation in clonal progression to a high-grade, aggressive biologic state.

Association of brain natriuretic peptide and adrenomedullin plasma levels with left ventricular filling pressures in end-stage renal disease patients on hemodialysis.

OBJECTIVE: Adrenomedullin (ADM) and brain natriuretic peptide (BNP) are known to be associated with elevated left ventricular filling pressures. However, little is known about this association in hemodialysis (HD) patients with preserved left ventricular ejection fraction (LVEF). Our objective was to evaluate the potential association between E/e' ratio and plasma levels of BNP and ADM in end-stage renal disease (ESRD) patients with preserved LVEF undergoing chronic hemodialysis. PATIENTS AND METHODS: The study group enrolled 62 ESRD patients treated with hemodialysis three times weekly. BNP and ADM plasma concentration measurements and echocardiographic examination were performed 30 minutes after hemodialysis. E/e' ratio, evaluated by Tissue Doppler imaging and measured at the basal septum, was used as a surrogate marker for assessing left ventricular filling pressures. RESULTS: The mean age of patients was 62 ± 25 years. The mean BNP and ADM values after hemodialysis were 0.40 ± 6.73 ng/ml and 0.06 ± 2.12 ng/ml, respectively. Elderly patients with hypertrophied left ventricles and larger left atria displayed higher E/e' values. BNP (r = 0.324. p = 0.018) and ADM (r = 0.319, p = 0.042) plasma levels were positively and significantly associated with E/e΄. Multivariate regression analysis including BNP, ADM, age, hemodialysis duration, left ventricular end-systolic volume index, LVEF, left ventricular mass index and left atrium volume index, revealed that ADM (p-value 0.025) but not BNP levels, were independently associated with the E/e' ratio. CONCLUSIONS: ADM, but not BNP, was independently associated with septal E/e' in HD patients with preserved LVEF. ADM plasma levels can be used as a surrogate index to assess left ventricular filling pressures in HD patients.

Impact of Post-Exodontia Bleeding in Cardiovascular Patients: A New Classification Proposal.

BACKGROUND: Exodontia (dental extraction), being the most frequent minor surgical procedure in the general population, inevitably involves a large number of patients on antithrombotic medication. Current experience shows that there is a degree of confusion in managing these patients. DESCRIPTION: Post-exodontia bleeding, a natural consequence of every dental extraction with no or minor clinical significance in the vast majority of cases, often appears to be of major concern to both patients and healthcare practitioners (dentists or physicians), either because of the alarming nature of oral bleeding itself or because of the distorted perception about its importance. These concerns are enhanced by the lack of a universal standardized definition of post-exodontia bleeding and by the fact that all currently available post-exodontia bleeding definitions bear intrinsic limitations and tend to overestimate its clinical significance. CONCLUSION: In order to overcome the aforementioned issues, this article presents an overview of post-extraction bleeding and proposes a classification, based on the well-recognized Bleeding Academic Research Consortium (BARC) bleeding definition, aiming at reducing heterogeneity in this field.

Changes in cardiac function and structure in newly diagnosed Graves' disease. A conventional and 2D-speckle tracking echocardiography study.

Overproduction of thyroid hormones leads to structural as well as functional cardiac changes. Conventional echocardiography but also recently developed sophisticated two-dimensional echocardiography speckle (2D-STE) tracking allow elaborate evaluation of these changes. Our purpose was to investigate the effects of thyroid hormones overproduction on the heart in patients with Graves' disease and changes that occur after 6 months thyrostatic therapy. We conducted a prospective, case-control study of 6 months duration. Full echocardiographic assessment at diagnosis and after 6 months of thyrostatic therapy were performed in 44 patients with Graves' disease, aged 37.6 ± 9.1 years. Additionally, 43 euthyroid controls were studied for the same time period. Left ventricular end diastolic diameter (LVEDD) and left ventricular end systolic diameter (LVESD) were higher in the patient group while triscupid annular plane systolic excursion (TAPSE) was lower in the patient group. Moreover, left ventricular (LV) mass index and left atrium (LA) volume index were higher in the Graves' disease group. Diastolic impairment as assessed with conventional echocardiography including tissue Doppler was present in the patient group (E/A ratio 0.87 ± 0.10,). 2D-STE analysis, revealed an increase in the strain rate at the isovolumic relaxation time (SRIVRT, 0.310 ± 0.07 patients versus 0.298 ± 0.09 s-1 controls). Improvement in diastolic and right systolic function as well as in left ventricular structural parameters was observed after restoration of euthyroidism (E/A ratio from 0.87 ± 0.10 versus 0.9 ± 0.08, p < 0.05). Patients with newly diagnosed Graves' showed an improvement in diastolic function, right systolic function and structural parameters after 6 months of thyrostatic treatment.

Adult congenital heart disease in Greece: Preliminary data from the CHALLENGE registry.

BACKGROUND: The majority of patients with congenital heart disease (CHD), nowadays, survives into adulthood and is faced with long-term complications. We aimed to study the basic demographic and clinical characteristics of adult patients with congenital heart disease (ACHD) in Greece. METHODS: A registry named CHALLENGE (Adult Congenital Heart Disease Registry. A registry from Hellenic Cardiology Society) was initiated in January 2012. Patients with structural CHD older than 16years old were enrolled by 16 specialized centers nationwide. RESULTS: Out of a population of 2115 patients with ACHD, who have been registered, (mean age 38years (SD 16), 52% women), 47% were classified as suffering from mild, 37% from moderate and 15% from severe ACHD. Atrial septal defect (ASD) was the most prevalent diagnosis (33%). The vast majority of ACHD patients (92%) was asymptomatic or mildly symptomatic (NYHA class I/II). The most symptomatic patients were suffering from an ASD, most often the elderly or those under targeted therapy for pulmonary arterial hypertension. Elderly patients (>60years old) accounted for 12% of the ACHD population. Half of patients had undergone at least one open-heart surgery, while 39% were under cardiac medications (15% under antiarrhythmic drugs, 16% under anticoagulants, 16% under medications for heart failure and 4% under targeted therapy for pulmonary arterial hypertension). CONCLUSIONS: ACHD patients are an emerging patient population and national prospective registries such as CHALLENGE are of unique importance in order to identify the ongoing needs of these patients and match them with the appropriate resource allocation.

Parachute-like mitral valve as a cause of mitral regurgitation.

BACKGROUND: Parachute mitral valve (PMV) is commonly associated with mitral valve stenosis but may occasionally be associated with mitral valve regurgitation. CASE REPORT: We present a case of an asymptomatic 41-year-old female with a background of incomplete Shone's complex displaying a PMV regurgitation pattern. CONCLUSION: Cardiovascular imaging with the use of transoesophageal echocardiogram and cardiac magnetic resonance were essential tools for the diagnosis and quantification of the severity of mitral valve regurgitation. Hippokratia 2016, 20(3): 238-240.

Left atrial appendage pericardial fluid: contrast-enhanced transesophageal echocardiography makes it visible.

BACKGROUND: Contrast-enhanced transesophageal echocardiography (TEE) for the opacification of left atrial appendage (LAA) is not clearly suggested by the current guidelines and only scarce data are available. CASE PRESENTATION: A patient with a membrane-like structure in the LAA cavity is presented. Contrast-enhanced TEE revealed LAA pericardial effusion and this diagnosis was confirmed by cardiac magnetic resonance that offered comparable images and tissue characterization. CONCLUSION: Contrast-enhanced TEE has been demonstrated to safely and accurately enlighten LAA anatomy. Hippokratia 2016, 20(3): 235-237.

Doppler echocardiographic evaluation of right ventricular diastolic function in isolated valvular aortic stenosis.

BACKGROUND AND AIM OF THE STUDY: Left ventricular diastolic function (LVDF) in patients with aortic stenosis (AS) has been adequately studied, in contrast to right ventricular diastolic function (RVDF). In this study, RVDF in patients with AS was evaluated using pulsed-wave Doppler echocardiography. METHODS: The study population comprised 20 patients with isolated AS (mean age 53.7 +/- 6.5 years) and 20 healthy volunteers (control group, mean age 52.6 +/- 8.8 years). The diastolic indices of right ventricular (RV) function were calculated using transtricuspid and transpulmonary Doppler flow velocities. Statistical analysis was performed using Student's t-test. There was no statistically significant difference between patients and controls with regard to age, height, bodyweight, heart rate, systolic and diastolic blood pressures, end-diastolic and end-systolic left ventricular (LV) diameter, LV fractional shortening and RV end-diastolic diameter. RESULTS: RV diastolic indices in patients (versus controls) were as follows: E/A ratio of transtricuspid flow waves was significantly lower (0.88 +/- 0.20 versus 1.25 +/- 0.33, p < 0.001); deceleration time of E wave was significantly longer (184 +/- 3 versus 127 +/- 3 ms, p < 0.001); atrial filling fraction was significantly augmented (43.1 +/- 7.7 versus 33.6 +/- 7.6%, p < 0.001); and isovolumic relaxation time was significantly prolonged (116 +/- 73 versus 31 +/- 15 ms, p < 0.001). There was no statistically significant correlation between diastolic indices and interventricular septum thickness and LV mass index. CONCLUSIONS: RVDF in AS patients is impaired, reflecting abnormal relaxation.

Primary aldosteronism of late onset: a case report.

BACKGROUND: Primary Aldosteronism is the commonest cause of secondary arterial hypertension and is due to uncontrollable aldosterone secretion by a series of adrenal disorders. DESCRIPTION OF THE CASE: We describe a case of a 71-year-old female patient with uncontrollable hypertensive peaks in soils of pre-existing arterial hypertension, who was diagnosed with Primary Aldosteronism. CONCLUSION: Both late onset of the disease and bilateral presence of adrenal nodes are rarely described in the literature and selection of treatment mainly depends on adrenal vein catheterization.

Myocardial crypts as a preclinical sign of hypertrophic cardiomyopathy.

BACKGROUND: The identification by cardiac magnetic resonance of myocardial crypts in the left ventricle (LV) of individuals carrying hypertrophic cardiomyopathy causative mutations, but without overt hypertrophy, has been proposed as an early sign of the disease. Myocardial crypts are usually identified in the offsprings of patients with a complete penetrance of the disease. CASE DESCRIPTION: We present a case of familial hypertrophic cardiomyopathy displaying an unusual pattern of disease distribution; the 14-year-old child was affected, demonstrating a typical LV asymmetrical hypertrophy, his grandfather, and the 2 brothers of his mother were also affected, but the 41-year-old mother was unaffected (no hypertrophy) displaying 3 myocardial crypts in inferior LV wall, suggesting a preclinical involvement. CONCLUSION: The findings underscore the diverse clinical spectrum of the disease, even in a single family and also the need to revise the diagnostic criteria of hypertrophic cardiomyopathy.Hippokratia 2014; 18 (4): 359-361.