The different surgical impact of the superior cavoatrial incision in children and adults.

INTRODUCTION: The single- and double-patch repairs are undoubtedly the most commonly used techniques for the surgical management of partial anomalous pulmonary venous connection associated with sinus venosus atrial septal defect. The aim of this study was to retrospectively compare early and long-term surgical outcomes in paediatric and adult patients, focusing in particular on the occurrence of ectopic atrial rhythm. MATERIAL AND METHODS: Seventy patients (male: 38, 54.2%) underwent surgical repair for partial anomalous pulmonary venous connection with sinus venosus atrial septal defect. Forty-nine patients (70%) underwent surgical repair in paediatric age (<16 years old), while 21 of (30%) patients were operated in adulthood. Thirty patients (42.8%) underwent single-patch repair and 39 patients (55.7%) underwent double-patch repair. In only one patient, the Warden procedure was performed (1.4%). Median follow-up time was 52 months (IQ 15.1-113). RESULTS: The type of surgical technique didn't affect the incidence of ectopic atrial rhythm (26.6% in single-patch group and 25.6% in double-patch groups, p = 0.9). At long-term follow-up, ectopic atrial rhythm, as an expression of sinoatrial node disturbance, was however significantly more frequent in the paediatric population (28.8% paediatric group and 4.7% adult group, p = 0.02). CONCLUSIONS: The higher incidence of ectopic atrial rhythm in children is probably related to the closer position of the sinus node to the superior cavoatrial incision, which makes irreversible iatrogenic traumatism more likely to occur. Surgical techniques that avoid any manipulation on the superior cavoatrial junction should, therefore, be preferred for children undergoing partial anomalous pulmonary venous connection repair.

Mitral regurgitation severity correlates with symptoms and extent of left atrial dysfunction: Effect of mitral valve repair.

PURPOSE: We aimed to assess the relationship between mitral regurgitation (MR) severity, symptoms, and left atrial (LA) structure and function, before and after mitral valve repair (MVR). METHODS: Global peak atrial longitudinal strain (PALS) was evaluated in 37 patients with severe symptomatic MR and preserved left ventricular (LV) ejection fraction (60.4% ± 4.6%) before and 3 months after MVR and was compared with values from 30 age- and gender-matched controls. RESULTS: Before surgery, PALS was worse in patients than in controls and indexed LA volume was greater (P < .0001 for both). After MVR, PALS deteriorated further and LA volume decreased (P = .001 and P = .05, respectively) as did LV ejection fraction, longitudinal strain (P = .05 and P < .001, respectively), and LV mass (P < .0001). Before surgery, LA volume correlated modestly with LV end-diastolic volume (R = 0.51; P = .01); effective regurgitant orifice area (EROA) correlated with PALS (R = -0.69, P < .001) and with LV longitudinal strain (R = 0.54, P = .01), and New York Heart Association class correlated with PALS (R = -0.69, P < .001), EROA (R = 0.69, P < .001), and LA volume (R = 0.51, P = .04). LA volume was the strongest predictor of global PALS reduction (P < .001), whereas global PALS was the main predictor of postoperative atrial fibrillation (AF) (P < .001). CONCLUSIONS: In patients with severe MR, EROA correlate with symptoms and LA PALS, which itself predicts the occurrence of postoperative AF. Strain values were superior to 2D data for the prediction of postoperative AF.

[Thrombotic storm in a teenager with previously diagnosed ulcerative colitis: a rare case of left ventricular thrombosis]. / Un raro caso di trombosi ventricolare sinistra in adolescente affetto da rettocolite ulcerosa.

Left ventricular thrombi usually occur in the setting of an acute myocardial infarction, left ventricular aneurysm, or dilated cardiomyopathy. In the absence of ventricular wall motion abnormalities, they are rare. We report the case of a patient with ulcerative colitis in whom two-dimensional echocardiography revealed a left intraventricular mass. Thrombosis in ulcerative colitis is a serious condition and can occur in a very young population. This case report also shows that left ventricular thrombi can occur in the active setting of ulcerative colitis.

Epidemiological and mortality trends in infective endocarditis, a 17-year population-based prospective study.

BACKGROUND: The population at risk, the clinical and microbiological features of infective endocarditis (IE) have changed. Aim of our study was to evaluate the contemporary epidemiological trends, over a 17-year period in a definite region of Tuscany, Italy, to analyze the clinical outcomes and associated prognostic factors. METHODS: From 1 January 1998 to 31 December 2014, all patients with a definite diagnosis of IE were prospectively entered in a data-base. The Health-Care system data-base was interrogated to capture patients who could have been missed. The final dataset derived by the merging of the two data-bases. RESULTS: Incidence rate of IE was 4.6/100,000/y with a significant linear incidence increase. In hospitalized patients the incidence was 1.27/1,000 admissions. Over age 65 incidence rate was 11.7/100,000/y. Male/female ratio was 1.54:1. A temporal trend towards an increase in the mean population age was found (P=0.033). There was an increase in the incidence of Health-care associated IE, P=0.016. The most common microorganisms were staphylococcus aureus (25%) and coagulase-negative staphylococci (22%). In-hospital mortality was 24%. A trend towards an increase in mortality rate was found (P=0.055). Independent predictors of mortality were older age, S. aureus infection, heart failure, septic shock and persistent bacteremia. CONCLUSIONS: Our study confirms an increasing mortality trend in IE, although with a borderline significance. Elderly forms are associated with poor prognosis and higher than 1-year mortality rate even in the multivariate analysis. Ageing population, increase in healthcare-associated and staphylococcal infections, may explain the rise of IE incidence and of the mortality trend.

Incidence rate of primary cardiac tumors: a 14-year population study.

AIMS: Incidence of primary cardiac tumors (PCTs) is not known. Literature data derive from autoptic studies or echocardiographic registries. An incidence of 1 of 1000 autoptic and 1.5 of 1000 echocardiographic study is reported but data from a general population are not available. The aim of our study was to evaluate the incidence rate of PCTs in the general population. METHODS: All patients with suspected cardiac mass were evaluated with basal echocardiogram and/or transesophageal echocardiogram/cardiac magnetic resonance by Grosseto's cardiology department, the county referral center for both adult and pediatric populations. Diagnosis was confirmed at surgical excision (32), autoptic specimens (3) or by multimodal imaging when surgery was not indicated (7). The database of the county health system was interrogated to identify residents with The International Classification of Diseases-9 codes of PCT. Forty-two consecutive cases of PCTs were diagnosed from 1 January 1998, through 31 December 2011, among residents in Grosseto's county. RESULTS: Incidence rate of PCTs was 1.38 of 100,000 inhabitants per year. PCTs were benign in 38 patients (90.5%) and malignant in four (9.5%). Twenty myxomas were found (48%), followed by seven fibroelastomas (15%), six lipomas (15%), three rhabdomyomas (8%), two hemangioma (5%), two sarcomas (5%), one lymphoma (2%) and one pericardial hemangiopericytoma (2%). Incidence of benign PCT was 1.24 of 100,000/year; referring only to myxomas we found an incidence of 0.68 of 100,000/year. CONCLUSION: This is the first population study on PCT, a rare disease with an incidence rate of 1.38 new cases per 100,000 residents per year.

Multimodality Imaging of a Silent Cardiac Hemangioma.

A 74-year-old man underwent echocardiographic exam for hypertension screening. A fixed plurilobulated mass originating from the right ventricular lateral wall and occupying half of the cavity was incidentally diagnosed. On cardiac magnetic resonance (CMR) it appeared homogeneous, intermediate-to-high signal on T1-weighted, and diffusely hyperintense on T2-weighted images. First pass enhancement was late and heterogeneous and no late gadolinium enhancement was present. Computed tomography (CT) showed no extracardiac infiltration, the feeding artery was a branch of therightcoronary artery. The tumor was excised and histological examination demonstrated a hemangioma of the cavernous type. The postoperative course was uneventful. From 1998 to 2014, four cardiac hemangiomas have been diagnosed in our Department, accounting for 8.7% of all primary cardiac tumors and for 9.5% of all benign forms; estimated population prevalence was 0.11/100.000 inhabitants/year. The hemodynamic consequences of unoperated cardiac hemangiomas cannot be predicted and therefore, resection is recommended.

Pre-operative left atrial strain predicts post-operative atrial fibrillation in patients undergoing aortic valve replacement for aortic stenosis.

Post-operative atrial fibrillation (AF) is a common and serious complication in patients undergoing aortic valve replacement (AVR). Speckle tracking echocardiography (STE) has recently enabled the quantification of longitudinal myocardial left atrial (LA) deformation dynamics. Our aim was to investigate LA preoperative mechanical function in patients undergoing AVR for aortic stenosis using STE and determine predictors of post-operative AF. 76 patients with aortic stenosis in sinus rhythm, undergoing AVR, were prospectively enrolled. Conventional echocardiographic parameters, and peak atrial longitudinal strain (PALS) were measured in all subjects the day before surgery. PALS values were obtained by averaging all segments in the 4- and 2-chamber views (global PALS). All patients received biological valve prostheses and a standard postoperative care. Postoperative AF occurred in 15 patients (19.7 %). On univariate analysis among all clinical and echocardiographic variables, global PALS showed the highest diagnostic accuracy (HR 6.55 p < 0.0001; AUC of 0.89) with a cut-off value <16.9 %, having sensitivity and specificity of 86 and 91 %, respectively, in predicting postoperative AF. LA volume indexed and E/e' ratio had lower diagnostic accuracy (AUC 0.76 and 0.51, respectively). On multivariate analysis global PALS remains a significant predictor of postoperative AF (p < 0.0001). STE analysis of LA myocardial deformation is considered a promising tool for the evaluation of LA subclinical dysfunction in patients undergoing AVR, giving a potentially better risk stratification for the occurrence of postoperative AF.

Usefulness of atrial deformation analysis to predict left atrial fibrosis and endocardial thickness in patients undergoing mitral valve operations for severe mitral regurgitation secondary to mitral valve prolapse.

In patients with severe mitral regurgitation (MR) referred for cardiac surgery, left atrial (LA) remodeling and enlargement are accompanied by mechanical stress, mediated cellular hypertrophy, and interstitial fibrosis that finally lead to LA failure. Speckle tracking echocardiography is a novel non-Doppler-based method that allows an objective quantification of LA myocardial deformation, becoming useful for LA functional analysis. We conducted a study to evaluate the relation between the traditional and novel atrial indexes and the extent of ultrastructural alterations, obtained from patients with severe MR who were undergoing surgical correction of the valvular disease. The study population included 46 patients with severe MR, referred to our echocardiographic laboratory for a diagnostic examination before cardiac surgery. The global peak atrial longitudinal strain (PALS) was measured in all subjects by averaging all atrial segments. LA tissue samples were obtained from all patients. Masson's trichrome staining was performed to assess the extent of the fibrosis. The LA endocardial thickness was measured. A close negative correlation between the global PALS and grade of LA myocardial fibrosis was found (r = -0.82, p <0.0001), with poorer correlations for the LA indexed volume (r = 0.51, p = 0.01), LA ejection fraction (r = 0.61, p = 0.005), and E/E' ratio (0.14, p = NS). Of these indexes, global PALS showed the best diagnostic accuracy to detect LA fibrosis (area under the curve 0.89), and it appears to be a strong and independent predictor of LA fibrosis. Furthermore, we also demonstrated an inverse correlation between the global PALS and LA endocardial thickness (r = -0.66, p = 0.0001). In conclusion, in patients with severe MR referred for cardiac surgery, impairment of LA longitudinal deformation, as assessed by the global PALS, correlated strongly with the extent of LA fibrosis and remodeling.

Pluripotency regulators in human mesenchymal stem cells: expression of NANOG but not of OCT-4 and SOX-2.

Mesenchymal stem cells (MSCs) are adult multipotent cells able to differentiate toward mature mesodermal lineages. In spite of more than a decade of investigation, little is known about the molecular mechanisms regulating the undifferentiated state and the identity of distinct functional subpopulations in these cells. Transcription factors that regulate the maintenance of the pluripotent state in embryonic stem cells, including NANOG, SOX2, and OCT4, have been proposed to play a similar role also in adult stem cells, although with conflicting results. We performed a critical evaluation of expression of these 3 transcription factors and found that NANOG, but not OCT-4 and SOX-2, is expressed in cultured human adult MSCs. Actually, NANOG was not expressed in freshly isolated MSCs, but was detected only after in vitro culture. NANOG was detected only in proliferating cells, but not in MSCs induced to differentiate. The percentage of cells expressing NANOG was maintained throughout early passages of MSCs, but then started to decrease in late passages in MSCs from adipose tissue and heart but not from bone marrow. However, the number of NANOG-expressing cells did not associate with the proliferative and differentiative capabilities of MSC populations, neither its expression appeared to identify cells having stem or progenitor cell properties. Accordingly, we propose that activation of NANOG expression in MSCs is associated with, although cannot directly regulate, the transition from in vivo quiescence to adaptation to in vitro growth conditions.

Interleukin 6 activity associated with a cardiac lymphoma.

Primary cardiac B-cell lymphoma is an extremely rare heart tumor that may be difficult to diagnose because of nonspecific clinical manifestations. Cardiac myxomas and mediastinal lymphomas show increased levels of serum cytokines, which correlate with symptoms and tumor size. We present a case of an intracardiac large B-cell lymphoma in a 75-year-old woman who had high serum levels of interleukin 6 that decreased after tumor excision. These data suggest a possible correlation between cardiac B lymphoma symptoms and interleukin 6 overproduction.

Congenital mitral disease: anomalous mitral arcade in a young man.

We present the case of a 33-year-old man referred to our institution with a diagnosis of severe mitral valvular stenosis and insufficiency. We realized the valvular disease was due to an "anomalous mitral arcade," a rare congenital malformation of the mitral tensor apparatus characterized by enlarged papillary muscles connected to mitral leaflets by a typical fibrous tissue bridge. This arrangement creates a fibrous continuity between valvular and subvalvular apparatus. The reported echocardiographic images shows in detail the anatomic and functional features of this rare condition.

Echocardiographic Assessment of Ebstein's Anomaly in a 60-Year-Old Man.

We present an echocardiographic evaluation of an elderly man affected with Ebstein's anomaly. In the natural history of this congenital disease only 5% of patients survive beyond the fifth decade. The patient presented severe right heart failure and he was refered to our institution for heart transplantation.

Cardiogenic shock complicating myocardial infarction in a doped athlete.

Abuse of doping agents may pose a higher risk for heart disease including acute myocardial infarction. We report the case of a 50-year-old body-builder Caucasian man with a long-standing abuse of nandrolone and erythropoietin that developed a ventricular septal defect following acute myocardial infarction. This mechanical complication led to cardiogenic shock ultimately treated with the implantation of a circulatory support by means of extracorporeal membrane oxygenation. The patient subsequently underwent orthotopic heart transplantation. The association of intense isometric exercise, abuse of erythropoietin and nandrolone is likely to have predisposed to coronary thrombus formation and acute myocardial infarction, as the patient presented no traditional cardiovascular risk factors.

Impaired angiotensin II-extracellular signal-regulated kinase signaling in failing human ventricular myocytes.

Angiotensin II was reported to induce insulin-like growth factor-I and endothelin-1 gene expression and peptide release by ventricular cardiomyocytes. However, the progression from cardiac hypertrophy to failure in humans is characterized by a reduced myocyte expression of insulin-like growth factor-I and endothelin-1, notwithstanding the enhanced cardiac generation of angiotensin II. In the present study we investigated the functional status of the signaling pathways responsible for angiotensin II-induced endothelin-1 and insulin-like growth factor-I formation in human ventricular myocytes isolated from patients with dilated (n = 19) or ischemic (n = 14) cardiomyopathy and nonfailing donor hearts (n = 6).In human nonfailing ventricular myocytes, angiotensin II (100 nmol/l) induced insulin-like growth factor-I and endothelin-1 gene expression, and peptide release was mediated by extracellular signal-regulated kinase activation and inhibited by extracellular signal-regulated kinase antagonism (PD98059, 30 micromol/l), endothelin-1 formation being partially reduced also by c-Jun N-terminal kinase inhibition (SP600125, 10 micromol/l); insulin-like growth factor-I and endothelin-1 formations were unaffected by the inhibition of p38 mitogen-activated protein kinase (SB203580, 10 micromol/l) and Janus tyrosine kinase 2 (AG490, 10 micromol/l). In failing myocytes, angiotensin II failed to induce insulin-like growth factor-I and endothelin-1 formation; angiotensin II-induced extracellular signal-regulated kinase activation was significantly impaired (-88% vs. controls) although c-Jun NH2-terminal kinase activation was preserved. The impaired extracellular signal-regulated kinase phosphorylation in failing myocytes was associated with increased myocyte levels of mitogen-activated protein kinase phosphatases.Therefore, the altered growth factor production in failing myocytes is associated with a significant derangement in intracellular signaling.

Reduced expression of thyroid hormone receptors and beta-adrenergic receptors in human failing cardiomyocytes.

An altered thyroid hormone profile has been reported in patients with congestive heart failure. However, information regarding the status of thyroid hormone receptors in human failing cardiomyocytes is lacking. Therefore the expression of thyroid hormone and beta-adrenergic receptors was investigated in human ventricular cardiomyocytes isolated from patients with end-stage heart failure (FM, n=12), or from tentative donors (C, n=4). The expression of thyroid (TRalpha1, and TRbeta1) and beta-adrenergic receptors (ARB1 and ARB2) was measured at both the gene, and at the protein level. In FM the reduced mRNA expression of ARB1 (p<0.05, -37%) and ARB2 (p<0.05, -42%) was associated with a reduction of the messenger for TRalpha1 (p<0.05, -85%) and TRalpha2 (p<0.05, -73%). These findings were confirmed at the protein level for ARB1, ARB2 and TRalpha1. These data reveal that in human heart failure the reduction of beta-adrenergic receptors is associated with reduced expression of both TRalpha1 and TRalpha2 isoforms of thyroid hormone receptors.

Early cardiac remodeling after repair of sinus venosus atrial septal defect.

We report the case of a 24-year-old man who underwent surgical repair of a sinus venosus atrial septal defect. Transthoracic echocardiograms performed on Day 3 and 6 after surgery demonstrated a very early cardiac remodeling with dramatic reduction of the right ventricular and atrial dimensions paralleled by an increase in the size of the left ventricle. This case provides the first demonstration of a very early cardiac remodeling after repair of sinus venosus atrial septal defect.

Primary hemangioma of the mitral valve: an unusual presentation.

Cardiac hemangiomas are rare tumors, the presence of these neoplasms in heart valves is an exceptional finding, mainly because of their histological features. Normally, transesophageal echocardiography is used to establish a diagnosis of cardiac tumor, though careful interpretation is needed to avoid diagnostic errors. Herein is described an unusual presentation of a primary hemangioma of the mitral valve which was diagnosed as a prolapsing left atrial myxoma. The patient underwent successful surgical excision of the neoplasm, followed by mitral valve repair.