Dilatation Of Right Atrium Research Articles

Características de la insuficiencia tricúspide grave inducida por dispositivos de estimulación intracardiacos

Introduction and aimsTricuspid regurgitation (TR) induced by the implantation of cardiac implantable electronic devices (CIED) is an increasingly common cause of severe TR. Our aim was to describe the echocardiographic phenotypic characteristics of CIED-induced severe TR. MethodsRetrospective cohort study that included patients with severe TR related to CIED diagnosed in the cardiac imaging unit of a Spanish tertiary hospital. Results37 patients with severe TR induced by lead/electrode interference formed our study group. TR was predominantly severe (68%), followed by massive (21%) and torrential (11%). The leaflet most affected by the interference was the septal. 58% of the sample presented severe dilatation of the right atrium (RA) (mean RA area 28cm2). Mean tricuspid annulus measurement was 42mm. The usual parameters for quantifying RV systolic function were on average within the normal range (TAPSE mean 19mm, S’ wave 10mm, FAC 41%), while global RV strain (RVGLS -15%) and free wall strain (RVFWLS -19%) were found reduced. An incipient degree of ventricular/pulmonary arterial uncoupling was evident (mean TAPSE/PSAP 0.34, SGLVD/PSAP 0.27%/mmHg). ConclusionsOur patients with CIED-induced severe TR are characterized by a heterogeneous phenotype with a high prevalence of severe RA and tricuspid annulus dilatation. RVGLS, RVFWLS, and arterial ventricular coupling were the most sensitive parameters for early assessment of RV systolic dysfunction.

Predictive value of Cardiac Magnetic Resonance: new and old parameters in the natural history of repaired Tetralogy of Fallot

BackgroundPatients with repaired Tetralogy of Fallot (rTOF) often develop pulmonary regurgitation (PR) and right ventricle (RV) dysfunction, experiencing increased mortality and morbidity rates in adulthood. Pulmonary valve replacement (PVR) timing to address PR is controversial. Cardiac Magnetic Resonance (CMR) is the gold standard for morpho-functional evaluation of complex cardiopathies. This study aims to identify CMR parameters predictive of adverse outcomes to help defining the best therapeutic management of rTOF patients.Methods130 rTOF patients who underwent CMR (2006–2019) were enrolled in this retrospective single-center study. CMR, clinical, ECG and exercise data were analyzed. Univariate and multivariate analyses identified clinical and CMR parameters predictive of adverse outcomes both individually (e.g., death, arrhythmias, heart failure (HF), pharmacological therapy, QRS ≥ 160ms) and as composite outcome.ResultsUnivariate analysis confirmed RV volumes and RV ejection fraction corrected for PR as adverse outcome predictors and identified interesting correlations: pulmonary artery bifurcation geometry and abnormal interventricular septum (IVS) motion with arrhythmias (p < .001; p = .037), HF (p = .049; p = .005), composite outcome (p = .039; p = .009); right atrium (RA) dimensions with the composite outcome and the outcomes individually (p < .001). The best predictive models by multivariate analysis included sex (male), RV and RA dilation for QRS ≥ 160ms, time form repair to CMR, age at TOF repair and IVS fibrosis for pharmacological therapy.ConclusionsBesides RV volumes, new adverse prognostic factors could guide rTOF therapeutic management: pulmonary arteries morphology, abnormal IVS motion, RV dysfunction, RA dilation. Perspective multicentric evaluation is needed to specify their effective role.

ФАКТОРЫ РИСКА РАЗВИТИЯ ОСТРОГО ПОВРЕЖДЕНИЯ ПОЧЕК ПРИ ИНФАРКТЕ МИОКАРДА У МУЖЧИН МОЛОДОГО И СРЕДНЕГО ВОЗРАСТА, СТРАДАЮЩИХ ПОДАГРОЙ

Abstract. Relevance. Acute kidney injury (AKI) in myocardial infarction worsens the prognosis and increases the duration of treatment of patients. Aim. To evaluate predictors of acute kidney injury in myocardial infarction in men younger than 60 years of age with gout to assess opportunities to improve prevention. Material and methods. The study included men 42-59 years old (y.o.) with type I MI and gout. Patients were divided into two age-comparable groups: I - study group, with AKI - two pa-tients; II - control, without it - 24 patients. Comparative evaluation of data on cardiovascular risk factors, parameters of the disease course in selected groups was performed. Quantitative parameters were determined twice, in the first 48 hours (1) and at the end of the third week (2) of MI. Pearson's chi-square method was used to assess the absolute (AR) and relative (RR) risks of developing AKI under the influence of the listed factors. Results. A significant effect on the possibility of developing AKI in the examined patients was obtained for: a history of urolithiasis (AR 50.0%; p=0.01), MI in the winter period (AR 40.0%; p=0.03 ); levels of low-density lipoproteins1&lt;2.5 mmol/l (AR 50.0%; p=0.04), ratios of total cholesterol1/high-density lipoproteins1&lt;3.7 (AR 50.0%; p=0.04), glucose1 ≥8.9 mmol/l (AR 100.0%; RR 14.0; p=0.008); potassium1 ˂3.8 mmol/l (AR 100.0%; RR 13.0; p=0.01), glomerular filtration rate (СKD-EPI)1≥123.0 ml/min/1.73 m2 (AR 100.0%; RR 14.0, p=0.008); polytopic extrasystole (AR 100.0%; RR 14.0; p=0.008), thromboembolism (AR 100.0%; RR 14.0; p=0.08) in the structure of MI complications; levels of systolic blood pres-sure (BPsyst)1≥200 mm Hg. (AR 66.7%; p=0.002), mean BP1≥133.0 (AR 66.7%; p=0.002), stroke index1≥39.8 ml/m2 (AR 66.7%; p=0.002), transverse dimension of the right atrium (RA)1 ˃52 mm (AR 50.0%; p=0.01). Conclusions. The most important for the risk of AKI development during MI in men under 60 y.o. with gout were arterial hypertension (BPsyst≥200 mHg), hyperglycemia (≥8.9 mmol/l), RA dilation (˃52 mm), history of urolithiasis. Combinations of the above parameters should be used to form high-risk groups for AKI development in this category of patients to improve preventive measures, as well as for predictive modeling.

P87 MEDIASTINAL SYNDROME REVEALED HEART LOCALIZATION OF A PRIMARY MEDIASTINAL B–CELL LYMPHOMA

Abstract Background Primary mediastinal B–cell lymphoma (PMBCL) is an aggressive B–cell lymphoma that represents 2–3% of non –Hodgkin lymphoma cases and typically affects young adult Caucasian women. Diagnosis can be difficult and often need a multidisciplinary approach. Case a 75 year old female, with history of hypertension, came to ER for severe dyspnea. She complains also neck and left arm oedema. Blue swelling of face and trunk were described. Heart PoCUS was inconclusive, describing only a hypoechoic dilation of right atrium while the other three heart chambers were reduced in dimensions. Chest TC revealed a huge mediastinal mass next to a thrombosis of superior cava vein and right atrium (7 cm diameter) and a diffuse subsegmental pulmonary embolism. Arm and neck Lymphnodes were also enlarged . Low molecular weight heparin was given twice a day. Due the respiratory failure a high–flow nasal cannula oxygen treatment was performed. PET revealed FDG uptake in antero–superior mediastinum, but there was the same uptake in heart right atrium without a connection with the previous described. Therefore, this was no more considered as thrombus but as mass. The micro–bubble test revealed right to left shunt. She refused heart MRI. The patient was not suitable for an open–surgery biopsy, thus she was scheduled for a TC guided one, in order to obtain a pathological diagnosis for a suspected right atrium sarcoma. Due to the high risk we choose to perform the less invasive as possible way to have a histological sample. Thus, and intravascular biopsy of atrial mass by femoral vein was performed. The patient did not present any sequelae after procedure and she continued anticoagulation. The subsequent histological analysis revealed a PMBCL with a primary localization in Right atrium. Unfortunately, a sepsis by multiple resistant pseudomonas Aeruginosa and Aspergillus spp. arose and the patient died three weeks after biopsy. Conclusion A PMBCL is a rare and aggressive disease. Like in our patient, mediastinal syndrome is often associated to the disease. However, intracardiac localization are even rarer events. In large case series, less than 1% of intracardiac masses were lymphomas. In our patient, open–surgery was not suitable for multiple comorbidities. However, intravascular approach was less aggressive but effective to obtain enough tissue for diagnosis. Unfortunately, the immune suppression related to PMBCL and opportunistic infections drive a mortal sepsis.

Blood Flow Simulation to Determine the Risk of Thrombosis in the Fontan Circulation: Comparison between Atriopulmonary and Total Cavopulmonary Connections

Three-dimensional computational fluid dynamics (CFD) simulations were performed in the anastomotic region of the Fontan route between the venae cava and pulmonary arteries to investigate the risk of thrombosis due to blood stasis in the Fontan circulation. The finite volume method based on the time-averaged continuity and Navier–Stokes equations combined with the k-ω SST turbulent model was used in the CFD simulations. Low shear rate (SR) and SR on the wall (WSR) of &lt;10 s−1 were used as markers to assess blood stasis as a cause of blood coagulation. Simulated blood flow velocity and both SR and WSR were reduced in the right atrium (RA) as the cavity of a flow channel in the atriopulmonary connection (APC) Fontan model, whereas the values increased in the total cavopulmonary connection (TCPC) Fontan model, which has no cavity. The volume of SR &lt;10 s−1 and wall surface area of WSR &lt;10 s−1 were, respectively, 4.6–261.8 cm3 and 1.2–38.3 cm2 in the APC Fontan model, and 0.1–0.3 cm3 and 0.1–0.6 cm2 in the TCPC Fontan model. The SR and WSR increased in the APC model with a normal-sized RA and the TCPC model as the flow rate of blood from the inferior vena cava increased with exercise; however, the SR and WSR in the RA decreased in the APC model with a dilated RA owing to the development of a recirculating flow. These findings suggest that the APC Fontan has a higher risk of thrombosis due to blood stasis than the TCPC Fontan and a higher RA dilation is associated with a higher risk of thrombosis from a fluid mechanics perspective.

Bidirectional Glenn shunt with tricuspid valve resection in patients with infective endocarditis.

Bidirectional Glenn shunt with tricuspid valve resection in patients with infective endocarditis.

Acute thoracic aortic dissection: a case report

Introduction: Aortic dissection (AD) is the most common aortic emergency, result in a catastrophic outcome. Patients usually asymptomatic, some of them present with sudden severe, tearing or sharp back or anterior chest pain and haemodynamic disturbance. Early and accurate diagnosis and treatment determine the patient outcome.Case Report: A case of 40 years old man complained of acute, sharp, chest pain since 4 days before admission which worsen instead of medical treatment, with a history of smoking since a few years ago. A vital sign was within normal limit. Laboratory result showed high CKMB, troponin T, LDH result. Occasional ventricular extrasystoles and inferior ischaemic was found in the ECG examination. From echocardiography, the patient suspected to have aortic dissection Stanford type A since an intimal flap on ascending aorta was found, with left ventricle hypertrophy, dilatation of right atrium and right ventricle, severe aortic regurgitation, moderate mitral regurgitation, severe tricuspid regurgitation, moderate pulmonary regurgitation and moderate pulmonary hypertension. From plain radiography, the patient showed mediastinum widening and cardiomegaly, CT angiography showed Stanford type A aortic dissection (DeBakey type I). The patient refused to seek further treatment for operation as suggested.Conclusion: It is still challenging to differentiate aortic dissection (AD) and another acute chest emergency. Since clinical presentation and laboratory result may be similar between those illnesses. Therefore careful history taking and further imaging examination should be considered to increase the patient outcome.

Strategies for diagnosis of fetal right atrium dilation: based on fetal cardiac anatomy and hemodynamics

BackgroundFetal right atrium (RA) dilation is frequently detected in routine screenings while it remains a challenge to clarify the reasons. This study aimed to analyze the cardiac anatomy and hemodynamics of fetal RA dilation and the changes of hemodynamic indexes.MethodsIn the retrospective study, 420 fetuses with RA dilation were included, which were classified into the physiological group (n = 202), volume overload group (n = 142), and the pressure overload group (n = 76). The ratio of right atrium to left atrium (RA/LA) were measured at four-chamber view. Peak velocity of tricuspid regurgitation (VTR) was recorded in each case, if existed.ResultsThe RA/LA ratio in the volume overload group is significantly higher than both the pressure overload group and the physiological group (both P = 0.000) throughout the pregnancy while no difference presents between the pressure overload group and the physiological group (P = 0.694 for 19–31 GW, and P = 0.974 for 32–36 GW, respectively). The VTR in the pressure overload group (3.29 ± 0.58 m/s) is significantly higher than both the volume overload group (1.85 ± 0.45 m/s, P = 0.000) and the physiological group (0.88 ± 0.45 m/s, P = 0.000). The volume overload group shows a significantly higher VTR than the physiological group (P = 0.000). In the volume overload group, the ductal contraction/closure shows a significantly higher VTR than that in the pulmonary valve stenosis/atresia (3.98 ± 0.41 m/s vs. 3.03 ± 0.38 m/s, P = 0.000).ConclusionsA strategy proposed herein is useful to clarify the reasons for RA dilatation by systematically assessing fetal hemodynamics, which may facilitate the sonographers to make an accurate diagnosis of congenital heart disease.

P1336PROPHYLACTIC CLOSING OF AV FISTULA PREVENTS HIGH OUTPUT HEART FAILURE - A SINGLE-CENTER RANDOMISED CONTROLLED TRIAL

Abstract Background and Aims AV fistulas are the favoured way ensuring a safe long-term access for patients with need for hemodialysis. Various studies, including a prior retrospective trial at our department, showed that AV fistulas are an additional burden on the cardiac-vascular system leading to frequent fistula closure in post-transplant patient. In literature, there is a widespread approach in dealing with high-flow fistulas in post-transplant patients with stable kidney function is reported. Method For this trial, patients with a stable graft function (min. 3 months post-transplant) and a brachial artery flow of min. 1,500 ml/min were recruited. Initially planned for multi-centric analysis, 28 patients were randomised in Innsbruck. Exclusion criteria were a pre-existing cardiac failure with ejection fraction less than 25%, NT-pro-BNP &amp;gt; 1,400 ng/l and NYHA score ≥ III. These patients were randomised in a 1:1 ratio in intervention group with immediate fistula ligature after randomisation, and control group with no surgical intervention. Patients in the control group had fistula ligature only if at least two criteria for ligature occurred. These criteria were selected in agreement with of the department for cardiology and encompassed dilation of right atrium, of inferior vena cava or of pulmonary artery, left-ventricular eccentricity index &amp;lt; 1 or a systolic pulmonary artery pressure of &amp;gt; 35 mmHg. The main endpoint high-output heart failure was defined by min. 2 echocardiographic criteria and signs of congestive heart failure such as worsening in NYHA score. A follow-up procedure of 24 months with quarterly measurements of kidney function, NT-proBNP and lactat dehydrogenase as well as a biannual echocardiographic check-up were performed. Statistics included paired t-test and Wilcoxon-rank test for median/mean comparison and chi-quadrat test for absolute frequency. Results The main endpoint high-output heart failure referable to high fistula flow was reached in five of 13 control patients (38.5%), whereas in the intervention group no study patient showed a worsening in clinical and echocardiographic signs to reach criteria for ligature. Prophylactic ligature of high-flow fistulas avoided right heart failure in our patient collective (p-value 0.013, x?test). Three patients had to undergo ligature due to off-study complications such as progressive aneurysma formation and steal phenomenon. One patient with dyspnoe preferred fistula ligature but was not fulfilling echocardiographic criteria. In total nine patients in the control group were assigned to fistula ligature. Matching all conducted fistula closures median NT-proBNP was 317 ng/l pre-ligature and 223 ng/l post-ligature (p 0.003, Wilcoxon). In total, 18 of 21 patients (excluding 2 patients with lack of data) showed falling NT-proBNP values. Creatinine levels showed a decrease from 1.69 mg/l to 1.60 mg/l (p-value 0.059, paired t-test). The intervention group included 14 patients with a gender ratio of 12 men to 2 women and a mean age of 52.5 years; the same age and gender distribution was seen in the control group. Fistula flow per m?body surface did not differ significantly (980 ml/min vs. 1000 ml/min). Echocardiographic findings showed improvement in ligature patients (e.g. a drop of sPAP in 7/8 ligature patients with available data) but did not reach statistical significance. Conclusion A prophylactic ligature of high-flow AV fistulas can avoid high-output heart failure and a more liberal approach to closure AV fistulas may be discussed in future.

Impact of right atrium dimension on adverse outcome after pulmonary valve replacement in repaired Tetralogy of Fallot patients

The hemodynamic impact of residual pulmonary regurgitation (PR) in repaired Tetralogy of Fallot (rTOF) has been well demonstrated. However, markers driving the decision making process to indicate the ideal timing of pulmonary valve replacement (PVR) are still uncertain. Furthermore, very few studies have included the right atrium (RA) dilatation as a preoperative risk factor for post-PVR clinical adverse outcome. The aim of this study was to investigate the impact of pre-PVR right atrial dilation on adverse outcomes in rTOF. We retrospectively reviewed from our CMR database all rTOF patients who underwent CMR study before and after PVR. Detailed clinical and surgical history were collected, in addition to imaging data. The composite primary and secondary post-PVR end points were also recorded. The study cohort consisted of 41 patients (mean age at PVR repair 27.4 ± 10years). As expected, end-diastolic and end-systolic right ventricle (RV) volumes significantly decreased after PVR (p < 0.001). The RV reverse remodeling, defined by ΔRVEDVi and ΔRVESVi, was associated with both pre-PVR RVEDVi and RVESVi. The higher the pre-PVR RV volumes, more the RV reverse remodeling will be obtained post-surgery. Patients who experienced an adverse outcome were older at pre-PVR, they had a higher Nt-ProBNP, worse VO2/kg/min, more significant tricuspid regurgitation and more dilated RA. The association with the RA dilatation persists and becomes even more significant if we exclude patients who had tricuspid repair beside RVOT surgical reconstruction. Besides RV volumes and function, RA dimensions may play a pivotal role in the decision making of TOF patients.

P1582 Outcomes, predictors and treatment of intraatrial reentrant tachycardias in Adult with Congenital Heart Disease

Abstract Introduction and objectives Atrial tachycardia (AT) is a major late complication in congenital heart disease (CHD) after surgery. These arrhythmias arise between areas of anatomical barriers or in fibrotic tissue/scars. In our study we examined the clinical predictors of AT. Methods A retrospective study of cases and controls in adult patients with CHD was performed. We have included consecutively 71 patients with AT and 71 controls matched by gender, age and type of CHD located in the same database. Medical records, electrocardiograms and echocardiograms were reviewed. Multivariate analysis of the risk factor (logistic regression) and analysis of adverse cardiovascular events (Kaplan-Meier) were performed. Results Systolic pressure of subpulmonary ventricle ≥40mmHg (HR:7, 95%CI:2.4-18, p = 0.001), right atrium (RA) dilatation≥21cm2 (HR:3, 95%CI:1.2-7.6, p = 0.005), significant tricuspid regurgitation (HR:4, 95%CI:1.3-10, p = 0.018) were identified as main risk factors for AT. Patients with AT had worse outcomes with more major adverse cardiovascular events (86% vs 14%, P = 0.006), and a 58% free survival events compared to 98% of patients without AT at 8 years of follow-up (p = 0.01). The treatment of the first episode of AT was electrical cardioversion (38%), pharmacological cardioversion (13%), heart rate control (20%). Antiarrhythmic treatment was not been given in 30% of the patients 73% of them had percutaneous ablation as first choice treatment. Ablation was performed in 40 pacientes and 6 patients have recurrences. In total, 24 patients have recurrences of AT. Previous Maze (OR 9 (IC al 95% 1.5, 50), p = 0.016) and surgical paliative shunt (OR 21 (IC al 95% 2.3,192), p = 0.007) were identified as main risk factor for AT recurrences. Conclusions The main risk factors identified for AT were RA dilatation, raised systolic pressure of subpulmonary ventricle and significant tricuspid regurgitation. The main risk factors for AT recurrences were previous Maze and surgical paliative shunt. Early treatment of these arrhythmic problems is mandatory in order to improve the prognosis in this group of patients. Abstract P1582 Figure. Central illustration of AT in CHD

47 Identification of threshold values to define right chamber enlargement consistent with severe tricuspid regurgitation

Abstract Background Right ventricle (RV), tricuspid anulus (TA) and right atrium (RA) dilatation, are listed among the supportive signs to grade severe tricuspid regurgitation (TR) according to current EACVI and ESC guidelines. However, at present, there is no cut-off value to define RV, RA and TA dilatation associated to severe TR. Purpose Accordingly, we sought to identify the threshold values of RV, RA and TA size associated to severe TR. Methods 302 patients (59 ± 13 years, 54 % women) with functional TR underwent three- (3D) and two-dimensional (2D) echocardiography to obtain: 3D RV end diastolic volume (RVEDVi) indexed for body surface area (BSA), 3D RV end systolic volume indexed for BSA (RVESVi), 3D RA max volume indexed for BSA (3DRAi), 2D RA systolic volume indexed for BSA (3DRAi), 2D RV basal diameter (2DRVd), 2D RV basal diameter indexed for BSA (2DRVdi), 2D TA measured in the apical 4-chamber view and 2D TA measured in the apical 4-chamber view indexed for BSA. To identify the threshold values of the parameters that discriminate patients with right chamber enlargement associated to severe TR, we selected the probability which returns the best sum of sensitivity and specificity on the ROC curve of the model. Results According to EACVI multiparametric approach, 50/302 pts (17%) were found to have severe TR. As shown in Figure, 3DRAi &amp;gt; 45 ml/m2 and 2DRAi &amp;gt; 45 ml/m2 identified patients with RA enlargement associated to severe TR. RVEDVi and RVESVi did not show any predictive value for severe TR. Conversely, 2DRVd &amp;gt; 52 mm (or &amp;gt;30 mm/m2) was associated to severe TR. 2DTA &amp;gt; 42 mm ( or &amp;gt;24 mm/m2) was the selected threshold value for TA dilatation. Conclusions Our study provided the threshold values to define the right chamber and TA dilatation associated to severe TR. Implementation of those values in current guidelines can help clinicians to improve their accuracy to identify patients with severe TR. Abstract 47 Figure.

P764 Right ventricular basal diameter, but not volume, can predict severe tricuspid regurgitation

Abstract Background According to current EACVI guidelines, right ventricle (RV), tricuspid anulus (TA) and right atrium (RA) dilatation are supportive signs to identify severe functional tricuspid regurgitation (TR) by echocardiography. However, the ranking by which those parameters should be considered to identify severe TR remains to be clarified. Purpose Accordingly, the aim of this study is to compare RV, RA and TA association with severe TR and to rank them in order of importance to predict severe TR. Methods 302 patients (59 ± 13 years, 54 % women) with functional TR underwent two- and three-dimensional echocardiography. Using the nonparameteric Variable Importance (VIMP) software package, we assessed the relative importance of 6 differerent parameters (indexed by body surface area) to identify severe TR: 3D RV end diastolic volume (RVEDVi), 3D RV end systolic volume (RVESVi), 3D RA max volume (3DRAi), 2D RA systolic volume (3DRAi), 2D RV basal diameter (2DRVdi) and 2D TAi measured in the apical 4-chamber view. Results According to EACVI multiparametric approach, 50/302 pts (17%) were found to have severe TR. 3DRAi (VIMP = 0.075) was the most important predictor of severe TR. 2DRVdi (VIMP= 0.005) was the second most important parameter and was the only parameter of RV dilation (RVEDVi= -0.0011 and RVESVi= -0.0012) associated to severe TR. Also, 2DRAi (VIMP= 0.023), and 2D TAi (VIMP= 0.004) showed good predictive ability. Conclusions Among the various right heart structures undergoing remodeling in patients with functional TR, RA dilation was the most important predictor of severe TR. Also the RV basal diameter, but not the volumes, was a predictor of severe TR. This underlines the importance of the shape, more than the volume of the RV as a predictor of severe TR.

Isolated acute right heart failure syndrome in a young male with severe sepsis: An unusual pathophysiology in a normal heart

Acute right heart failure is being increasingly recognized in intensive care unit (ICU) and when severe, can contribute to hemodynamic instability. Sepsis-induced myocardial dysfunction is also a known entity in critical care ICU. However, isolated right ventricle (RV) dysfunction in patients with sepsis after excluding chronic heart and lung disease is underdiagnosed and reported uncommonly. Whether RV is a bystander or a major contributor to hemodynamic instability in that scenario has not been studied clearly. Here, we report a 42-year-old male patient with no major cardiovascular risk factors who presented to us in shock with a history of low-grade fever, generalized weakness, and decreased urine output for 3 days. On physical examination, he had pedal edema and neck vein distension. He was found to have neutrophilic leukocytosis, deranged renal function test, and liver function test. His chest X-ray posteroanterior view showed cardiomegaly with pulmonary congestion. His electrocardiography (ECG) showed right bundle branch block (RBBB). He was anuric and started on dialysis for severe acidosis. In view of shock, renal failure and RBBB on ECG, a transthoracic echocardiography was done which showed gross dilatation of right atrium, and RV with severe RV dysfunction with normal left ventricle function. Contrast-enhanced computerized tomography chest was negative for pulmonary thromboembolism. His blood culture has grown Escherichia coli. With intravascular (IV) fluids, ionotropic support, renal replacement therapy, IV antibiotics, and other supportive therapy, he gradually improved. His serial ECGs showed resolution of RBBB. Two-dimensional echo showed improved RV function. He was discharged on day 9 and his 2-week follow-up echo showed normalization of RV function. During follow-up, his coronary angiogram showed normal coronaries. This case illustrates the importance of identifying acute RV failure in patients with severe sepsis with shock. It also underscores the necessity for more studies and research on understanding the pathophysiology of RV dysfunction in critical care patients and its contribution to hemodynamic instability.

Atrial involvement in arrhythmogenic right ventricular cardiomyopathy patients referred for ventricular arrhythmias ablation.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable myocardium disorder that predominantly affects the ventricle. Little is known about atrial involvement. This study aimed to assess atrial involvement, especially the role of genotype on atrium in ARVC. The incidence, characterization and predictors of atrial involvement were investigated. Nine known ARVC-causing genes were screened and the correlation between genotype and atrial involvement was assessed. Right atrium (RA) dilation, left atrium (LA) dilation, and sustained atrial tachyarrhythmias (ATa) were found in 45, 16 and 3 patients, respectively. Gene mutations were identified in 64 (64.0%) patients. Mutation carriers showed more RA dilation than noncarriers (54.7% vs. 27.8%, P=0.009), and no difference in LA dilation and ATa. Multivariate analysis showed tricuspid regurgitation (OR: 18.867; 95% CI: 1.466-250.000; P=0.024) increased the risk of RA dilation and decreased left ventricular ejection fraction (LVEF) (OR: 1.134; 95% CI: 1.002-1.272; P=0.031) correlated with LA dilation, whereas genotype showed no significant effect. At a median follow-up time of 91 months, 7 patients died and 1 patient accepted heart transplantation. New-onset RA dilation, LA dilation, and sustained ATa were found in 8, 7, and 6 patients, respectively. Atrial involvement was not associated with the long-term survival. Despite mutation carriers showing more RA dilation, Kaplan-Meier analysis showed genotype was not associated with atrial involvement. Atrial involvement was common in ARVC. Tricuspid regurgitation and decreased LVEF increased the risk for atrial dilation. Genotype was not associated with atrial involvement.

Right Atrial Dysfunction in the Fetus with Severely Regurgitant Tricuspid Valve Disease: A Potential Source of Cardiovascular Compromise

In severe right heart obstruction (RHO), redistribution of cardiac output to the left ventricle (LV) is well tolerated by the fetal circulation. Although the same should be true of severely regurgitant tricuspid valve disease (rTVD) with reduced or no output from the right ventricle, affected fetuses more frequently develop hydrops or suffer intrauterine demise. We hypothesized that right atrium (RA) function is altered in rTVD but not in RHO, which could contribute to differences in outcomes. Multi-institutional retrospective review of fetal echocardiograms performed over a 10-year period on fetuses with rTVD (Ebstein's anomaly, tricuspid valve dysplasia) or RHO (pulmonary atresia/intact ventricular septum, tricuspid atresia) and a healthy fetal control group. Offline velocity vector imaging and Doppler measurements of RA size and function and LV function were made. Thirty-four fetuses with rTVD, 40 with RHO, and 79 controls were compared. The rTVD fetuses had thelargest RA size and lowest RA expansion index, fractional area of change, and RA indexed filling and emptying rates compared with fetuses with RHO and controls. The rTVD fetuses had the shortest LV ejection time and increased Tei index with a normal LV ejection fraction. RA dilation (odds ratio, 1.27; 95% CI, 1.05-1.54) and reduced indexed emptying rate (odds ratio, 2.49; 95% CI, 1.07-5.81) were associated with fetal or neonatal demise. Fetal rTVD is characterized by more severe RA dilation and dysfunction compared with fetal RHO and control groups. RA dysfunction may be an important contributor to reduced ventricular filling and output, potentially playing a critical role in the worsened outcomes observed in fetal rTVD.

Atrial Remodeling and Atrial Tachyarrhythmias in Arrhythmogenic Right Ventricular Cardiomyopathy

Less is known about atrial remodeling and atrial tachyarrhythmias (ATa) in arrhythmogenic right ventricular cardiomyopathy (ARVC); this cross-sectional study aimed to determine the prevalence, characterization, and predictors of atrial remodeling and ATa in a large series of patients with ARVC. From February 2004 to September 2014, 294 consecutive patients who met the task force criteria for ARVC were enrolled. The prevalence, characterization, and predictors of atrial dilation and ATa were investigated. Right atrium (RA) dilation was identified in 160 patients (54.4%) and left atrium dilation in 66 patients (22.4%). Both RA and left atrium dilation were found in 44 patients (15.0%). Twenty-five patients (8.5%) had atrial fibrillation (AF), whereas 19 patients (6.5%) had atrial flutter (AFL). Of which, 7 patients (2.4%) had both AF and AFL. Multivariate analysis showed that AFL (odds ratio [OR] 10.309; 95% confidence interval [CI] 2.770 to 38.462; p <0.001), hypertension (OR 9.174; 95% CI 2.364 to 35.714; p= 0.001), and RA dilation (OR 6.993; 95% CI 1.623 to 30.303; p= 0.009) were associated with increased risk for AF. AF (OR 10.526; 95% CI 2.786 to 40.000; p= 0.001) increased the risk of AFL. Inconclusion, atrial remodeling and ATa were common in patients with ARVC.

77 The Pathological Disease Spectrum of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) in Sudden Cardiac Death Emphasising Biventricular Involvement and Challenges in Diagnosis

<h3>Introduction</h3> Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disease of the heart muscle characterised by fibrofatty replacement of the myocardium. It is a major cause of sudden death in the young, specifically athletes. ARVC is largely a genetic disease with implications for living family members and cardiological screening. We present the largest detailed pathological study from the UK of arrhythmogenic right ventricular cardiomyopathy (ARVC) diagnosed at post-mortem following sudden death. <h3>Methods and results</h3> We identified 121 cases of sudden cardiac death due to ARVC in our UK referral sudden death database. Cases were identified using our database compiled from 2873 sudden cardiac death cases received in our specialist national referral centre between 1994 and 2012. This study highlights clinical history, initial pathological opinion, macroscopic and microscopic findings on specialist examination. Individuals were predominantly male (82%) from 8–66 years with highest prevalence at 21–30 years. Cardiac arrest occurred mainly during/immediately after exertion (45%) and at rest (38%). Macroscopic examination showed dilatation of right atrium and ventricle with thinning (44%) and dilatation of left atrium and ventricle (19%). There were pale areas with fat replacement of epicardial muscle in both ventricular walls (70%) and septum (12%). The right ventricular outflow tract (RVOT) was involved in all biventricular cases. Left ventricular hypertrophy showed increased epicardial fat (49%) and right ventricular fatty hypertrophy (15%). 12% were macroscopically normal. Thrombi in the right ventricle occurred in 10% of cases. Histologically fibrofatty replacement with degenerate myocytes was seen in all cases, Biventricular disease predominated (78%). Above the midventricle there was always involvement of the epicardial surface of RVOT. Left ventricular involvement was epicardial and circumferentially with predominant involvement of the posterobasal wall. The biventricular disease cases had left dominance in 21% or right dominance in 17%. Of the 53 opinions by referring pathologists, only 27 were identified as ARVC highlighting the difficulty of diagnosis. <h3>Conclusion</h3> ARVC ranges from normal appearing hearts to widespread biventricular disease. ARVC has genetic implications and correct pathological diagnosis is vital to ensure that family members at risk are screened to prevent further sudden deaths.

PO-0490 Echocardiographic Predictors Of Outcome In Persistent Pulmonary Hypertension In Newborn (pphn): A Prospective Study

Aim To evaluate prospectively the echocardiographic predictors of outcome in persistent pulmonary hypertension of the newborn (PPHN). Patients and methods We realised a prospective, monocentric study from April 2012 to April 2013 in a paediatric intensive care unit. Were enrolled newborns who were less of 28 days of life, born at term or preterm, with clinical and echocardiographic signs of pulmonary hypertension. Were excluded patients with congenital heart disease. Two echocardiographies were performed by a trained intensivist, first at the diagnostic of PPHN, second after therapeutic optimisation. Were measured : sense of the septal curve, evaluation of systolic pulmonary pressure on tricuspid regurgitation, presence and direction of ductal and atrial shunts, right and left ventricular function (with maximal aortic and pulmonary velocity and visual aspect of dilatation of right atrium and ventricle). Results 27 newborns (median age 0 day: 0–15 days of life) were enrolled. Six died (22%). At the first echocardiography, we find that the newborn who died presented more frequently a right to left ductal shunt (83% vs. 28%,p = 0.043), a dilated right atrium (83% vs. 33%, p = 0,043) and a smallest maximal velocity in pulmonary artery (0,53 m/s vs. 0,64 m/s, non significative) than those who survived. The direction of the atrial shunt were not in correlation with death. Second echocardiography showed that the diminution of the measured pulmonary pressures isn’t a good sign of predictive good outcome. Conclusion We have to confirm in largest studies that echocardiography can be used to estimate prognosis in PPHN.

Mirror image atrial dilatation in adult patients with atrial fibrillation and congenital heart disease

BackgroundAtrial fibrillation (AF) is largely regarded to be initiated from left atrial (LA) dilatation, with subsequent dilatation of the right atrium (RA) in those who progress to chronic AF. We hypothesized that in adult patients with right-sided congenital heart disease (CHD) and AF, RA dilatation will predominate with subsequent dilatation of the left atrium, as a mirror image. MethodsAdult patients with diagnosis of right-sided, ASD or left-sided CHD who had undergone an echocardiographic study and electrocardiographic recording in 2007 were included. RA and LA area were measured from the apical view. AF was diagnosed from a 12-lead electrocardiogram or Holter recording. A multivariate logistic regression model was used to identify predictors of AF and linear regression models were performed to measure relationship between RA and LA area and AF. ResultsA total of 291 patients were included in the study. Multivariate analysis showed that age (p=0.0001), RA (p=0.025) and LA area (p=0.0016) were significantly related to AF. In patients with pure left-sided pathologies, there was progressive and predominant LA dilatation that paralleled the development of AF from none to paroxysmal to chronic AF. In patients with pure right-sided pathologies, there was a mirror image of progressive and predominant RA dilatation with the development of AF. ConclusionWe observed a mirror image atrial dilatation in patients with right sided disease and AF. This may provide novel mechanistic insight as to the origin of AF in these patients and deserves further studying in the form of targeted electrophysiological studies.