Right Ventricular Enlargement Research Articles

Disease severity-related alterations of cardiac microRNAs in experimental pulmonary hypertension.

Right ventricular (RV) failure is the primary cause of death in pulmonary arterial hypertension (PAH). We hypothesized that heart‐relevant microRNAs, that is myomiRs (miR‐1, miR‐133a, miR‐208, miR‐499) and miR‐214, can have a role in the right ventricle in the development of PAH. To mimic PAH, male Wistar rats were injected with monocrotaline (MCT, 60 mg/kg, s.c.); control group received vehicle. MCT rats were divided into two groups, based on the clinical presentation: MCT group terminated 4 weeks after MCT administration and prematurely terminated group (ptMCT) displaying signs of terminal disease. Myocardial damage genes and candidate microRNAs expressions were determined by RT‐qPCR. Reduced blood oxygen saturation, breathing disturbances, RV enlargement as well as elevated levels of markers of myocardial damage confirmed PH in MCT animals and were more pronounced in ptMCT. MyomiRs (miR‐1/miR‐133a/miR‐208a/miR‐499) were decreased and the expression of miR‐214 was increased only in ptMCT group (P < 0.05). The myomiRs negatively correlated with Fulton index as a measure of RV hypertrophy in MCT group (P < 0.05), whereas miR‐214 showed a positive correlation (P < 0.05). We conclude that the expression of determined microRNAs mirrored the disease severity and targeting their pathways might represent potential future therapeutic approach in PAH.

Understanding tricuspid valve remodelling in atrial fibrillation using three-dimensional echocardiography.

Atrial fibrillation (AF) has been associated with tricuspid annulus (TA) dilation in patients with severe functional tricuspid regurgitation (TR); however, the impact of AF is less clear in patients without severe TR. Our aim was to characterize TA remodelling in patients with AF in the absence of severe TR using 3D transoesophageal echocardiography (TOE). Ninety patients underwent clinically indicated transthoracic and TOE: non-structural (NS)-AF (n = 30); AF with left heart disease (LHD) (n = 30), and controls in sinus rhythm (n = 30). Three-dimensional TOE datasets were analysed to measure TA dimensions using novel dedicated tricuspid valve software. The NS-AF group showed biatrial dilatation and normal right ventricular (RV) size with decreased longitudinal function compared to controls, whereas the LHD-AF group showed biatrial dilatation, RV enlargement, decreased biventricular function, and higher systolic pulmonary artery pressure compared with the other groups. Indexed TA area, minimum diameter, maximum diameter, and total perimeter were significantly larger in the NS-AF group than in controls (measurements in end-diastole: 6.4 ± 1.1 vs. 5.0 ± 0.6 cm2/m2, 1.8 ± 0.3 vs. 1.6 ± 0.2 cm/m2, 2.1 ± 0.3 vs. 1.9 ± 0.2 cm/m2, and 6.6 ± 0.9 vs. 5.9 ± 0.7 cm/m2, respectively, all P < 0.05). There was no significant difference in any indexed TA parameter between AF groups. TA circularity index (ratio between minimum and maximal diameters) and TA fractional area change between end-diastole and end-systole were no different among the three groups. AF is associated with right atrial and tricuspid annular remodelling independent of the presence of LHD in patients with intrinsically normal tricuspid leaflets without severe TR.

P1326 Recurrent isolated pulmonary valve endocarditis complicated by heart failure, septic pulmonary embolism and multiple lung abscesses in an intravenous drug abuser

Abstract Background Right-sided infective endocarditis (IE) ( 5-10% of all IE cases)is the most frequently observed in intravenous drug abusers (IVDA) with tricuspid valve as the most predominant site. Isolated pulmonary valve IE is an extremely rare entity in adults. Case report 27-year-old women, IVDA for 15 years, was admitted to the hospital due to fever and severe shortness of breath. Her past history was significant for isolated pulmonary valve IE of methicillin-susceptible Staphylococcus aureus 9 months before with mild pulmonary regurgitation and normal right ventricular (RV) function at discharge after medical treatment. At admission on physical examination, the patient showed tachycardia 140/’, tachypnoea, hypotension and crackles. Initial chest X-ray documented massive bilateral infiltrates. Laboratory tests showed highly elevated inflammatory markers and anaemia. Transthoracic echocardiography (TTE) revealed large (36 x 20 mm) very mobile vegetations on the pulmonary valve (Fig A), pulmonary trunk dilatation, mild pulmonary regurgitation and moderate pericardial effusion. No sign of IE was found on the tricuspid valve (Fig C), which presented anterior leaflet prolapse with mild regurgitation. Computed tomography (CT) revealed numerous cavitary infiltrates in both lungs surrounded by ground-glass opacification. Blood cultures were positive for methicillin-resistant Staphylococcus aureus. The patient was started on vancomycin. On day seven the patient status worsened with symptoms of heart failure and elevated pro-BNP up to 2833pg/ml. TTE revealed severe pulmonary regurgitation (Fig B) with pulmonary hypertension and RV dysfunction (Fig D). Repeated CT showed pulmonary embolism (PE), RV enlargement and multiple abscesses in both lungs. The patient was consulted with cardio surgeon but the intervention was denied and medical treatment was continued with vancomycin, gentamicin, rifampin, glicocorticoids, enoxaparin, betablockers, digoxin and diuretics. Under serial TTE and CT control antibiotics were continued up to 10 weeks. Ultimately patient was discharged in relatively good condition without any symptoms of heart or respiratory failure with smaller vegetation (15-16mm) on the pulmonary valve but with severe pulmonary regurgitation and mild RV enlargement and dysfunction in TTE and significant regression of pulmonary lesions and PE in CT. Discussion With respect to the high recurrence rate of right-sided IE in IVADs surgery should generally be avoided. A prolonged course of parental antibiotics is recommended in cases with lung abscesses. Conclusions We report a case of recurrent isolated pulmonary valve IE and extensive complications but with a positive outcome. Pulmonary valve IE is very rare in IVADs but requires early as well as serial multimodality imaging in the diagnostic workup. Abstract P1326 Figure. TTE of complicated pulmonary valve IE

P1467 Natural history and outcome of a patient with untreated congenital pulmonary stenosis added value of 3d echo in decision making

Abstract INTRODUCTION congenital pulmonary valve stenosis (PVS) accounts for approximately 7% of all congenital heart defects. In less severe and untreated asymptomatic forms, it might have variable outcomes with progressive stenosis or development and worsening of pulmonary regurgitation . Atrial septal defect (ASD) might be associated in up to 20% of the cases rendering an even more unpredictable outcome and efect on right ventricular (RV) volumes and function. Cardiac magnetic ressonance is considered the gold standard method to measure RV function and volumes. Three dimensional echocardiography (3D echo) emerges as a valuable, redproducible and portable alternative not only for RV volume and function analysis but also for structural evaluation. PURPOSE To describe the added value of 3D echo for the diagnostic work-up, decision making and post surgical outcome in a patient with longstanding untreated PVS associated with ASD. CASE PRESENTATION a 56 year old asymptomatic , non syndromic man , was referred by the cardiac surgeon for a comprehensive echocardiographic evaluation (CEE) due to a previous diagnosis of PVS .The patient was aware of this diagnosis since the age of 17 and no intervention was undertaken at that time. More recently a conventional echocardiogram described a moderate PVS with RV enlargement (RVE) and no futher details regarding RV function or surgical feasibility. CEE showed RVE due to volume overload (VO) with indexed end diastolic volume (EDV) of 144ml/m2 (upper limit 76ml/m2), end sistolic volume (ESV) of 57ml/m2 (upper limit 37ml/m2) and an RV ejection fraction (RVEF) of 60% (lower limit 45%) on 3D echo. RV free wall strain (RVFWS) was -25,3% , TAPSE 32mm and fractional area change 46%, all paramenters within the normal range except for RV volumes. Anatomical analysis showed PVS with three leaflets, partial comissural fusion and retraction of the edges yelding a closure gap of at least 8mm and consequent severe regurgitation. Peak transvalvular gradient was 38mmHg and pulmonary artery was dilated measuring 6.4cm. An ostium secundum atrial septal defect (ASD) with left to right shunt measuring 15x9mm by 3D echo contributed for futher RVVO. With these information , surgical intervention was undertaken and consisted of PV replacement, excision and reconstruction of the pulmonary trunk dilation and correction of the ASD. The patient recovered well and returned three months after, for a new evaluation. At that time RV volumes reduced dramatically with EDV and ESV respectively of 44ml/m2 and 19ml/m2 RVEF of 55,7% and RWFWS of -25,6%. CONCLUSION untreated congenital PVS associated with ASD may have a variable outcome . Follow-up is mandatory. In the present case a CEE including more recent technologies like 3D echo and strain imaging helped in defining anatomical findings and RV function analysis thus stating the appropriatness for surgical intervention. Abstract P1467 Figure. 3Decho pre and post surgical analysis

P335 Evaluation of systolic right ventricular function with 3D echocardiography in patients with isolated right ventricular pacing induced left ventricle dysfunction

Abstract Background Right ventricular (RV) pacing may worsen left ventricular (LV) systolic function causing heart failure, but the exact mechanism of the LV dysfunction is unknown. The purpose of this study was to examine the right ventricle by three-dimensional echocardiography in patients with LV dysfunction accompanied by long-term RV pacing. Methods We analysed consecutive patients receiving permanent pacemaker (PPM) due to atrioventricular block from 2015 January to 2017 March (n = 335). During the mean follow-up period (27 months) 4 patients were selected with at least 5% decrease in the LV ejection fraction measured by two-dimensional echocardiography (B group). Control (K) group contains 4 age-, sex-, concomitant disease matched patients without the sign of LV dysfunction from the same time interval. Right ventricle function was assessed by 3D echocardiography. Results In both groups, there were 3 men, and the mean age was similar (B: 68 ± 6 y vs. K: 66 ± 10 y; p = 0.65). Right ventricular ejection fraction (EF) was significantly higher in controls compared to patients (K: 49 ± 7.8% vs. B: 36 ± 3.1%; p = 0.02), while the right ventricular volumes [end-systolic (K: 79 ± 47 ml vs. B: 71 ± 7 ml; p = 0.77), end-diastolic (K: 151 ± 73 ml vs. B: 111 ± 11 ml; p = 0.36) and stroke volumes (K: 58 ± 44 ml vs. B: 40 ± 6 ml; p = 0.5)] did not differ significantly. We did not find any important differences between the groups regarding the permanent right ventricle pace rate (K: 93 ± 5.6% vs. B: 84 ± 19.5%; p = 0.5), systolic pulmonary pressure (K: 34 ± 6 mmHg vs. B: 35 ± 18 mmHg; p = 0.92), or the severity of tricuspid regurgitation. Conclusion The left ventricular dysfunction after permanent right ventricular pacing results in right ventricular systolic dysfunction. The decrease of RV ejection fraction is not associated with RV enlargement or increase of pulmonary pressure.

P1729 Abnomral course of pulmonary vein causing right heart dilatation

Abstract Funding Acknowledgements None Introduction Partial anomalous pulmonary venous return is a rare congenital cardiac anomaly that usually involves the right pulmonary vein and an atrial septal defect. It is accounted for 0.7%. Isolated partial anomalous pulmonary venous return with an intact atrial septum is even rarer, and this condition is usually treated surgically in younger patients. We report a case of a young female, incidentally found to have a right upper pulmonary vein draining into the right atrium, confirmed by TOE and CT Venography pulmonary, with moderate right ventricular enlargement, without pulmonary hypertension, intra-cardiac shunt or accompanied congenital anomaly. A decision of surgical correction was made. We conclude that it is important to suspect PAPVC in patients with right ventricular volume overload, even in absence of ASD. Case presentation A 26-year-old female, not known to have any chronic illness. She was referred because of poor appetite and low weight, to cardiology evaluation for accidental murmur finding during examination. She had no other symptoms. The TOE was done which showed left pulmonary veins are normal, the right lower pulmonary vein is normal, and there is high suspicion of aberrant right upper pulmonary vein draining in the right atrium, no evidence of intra-cardiac shunt. CT Venography pulmonary revealed anomalous (Giant) right superior pulmonary vein to the superior vena cava and draining to the right atrium. Figure 1. Patient was referred for surgical correction. Discussion Partial anomalous pulmonary venous return is a type of left to right shunt. It is a rare congenital abnormal cardiac defect in which the pulmonary veins draining into the right atrium (RA) directly or indirectly by venous connection. Its accounted for 0.4 to 0.7%. However, as this rate is based upon autopsy data, the true prevalence of PAPVR may actually be higher. PAPVR is frequently associated with other congenital heart disease, most commonly ASD, in &amp;gt;75% of patients. It is estimated that 10% to 15% of patients with ASD and up to 85% of patients with sinus venosus ASD have PAPVC. Exclusive of ASD, other more complex cardiac malformations occur in ∼20% of patients. PAPVR can also be an isolated defect with intact atrial septum. TOE is more sensitive than TTE in detecting PAPVR. A potential limitation of echocardiography is the availability of acoustic windows. If all four PVs are not identified emptying into the left atrium on TEE, CCT and CMR may provide complementary data . CONCLUSION In conclusion, missed diagnosis always occurs in PAPVR, the debauched and blood flow of pulmonary vein should be over-viewed carefully during TEE examination. In a patient with unexplained RV enlargement PAPVC (one or more pulmonary veins) should be considered in the differential diagnosis. Multi-modality cardiac imaging using echocardiography, CT angiography and CMR may provide a comprehensive noninvasive evaluation of PAPVC. Abstract P1729 Figure. Fig1 TOE (Top),CT (Bottom) of RUPV

P876 I may still steal you : huge coronary cameral fistula (non-dominant right coronary artery to right ventricle) in a neonate causing heart failure

Abstract Funding Acknowledgements Not applicable OnBehalf Not applicable A 25 days old neonate, weighing 2.8kg was brought with complaints of feeding difficulties, not gaining birth weight and rapid breathing. The parents had not noticed any cyanosis. He was further referred to us as the treating paediatrician heard a pansystolic murmur over the left sternal border. When examined, baby was afebrile, tachypnoea (70 per minute), regularly tachycardia(180bpm). We did not appreciate any dysmorphism. On cardiovascular examination we found a mild cardiomegaly, RV S3, mild hepatomegaly with no signs of active infection. Systemic saturations were 98% in all limbs. Other systemic examinations were essentially normal. 12 lead ECG showed, physiological RV dominance, with no signs of LV volume overload. Chest radiograph showed cardiomegaly of RV type with adequate PBF. 2 D echocardiogram : The intra-cardiac structures were found to be normal. A huge coronary-cameral fistula originating from RCA, traversing in right AV groove and draining into the RV with mild RV enlargement. No signs of PAH. Biventricular function was preserved. CT coronary angiogram : CT confirmed the presence and anatomy of the RCA—RV fistulous tract seen on echocardiography. The fistula measured ∼ 6mm at the origin and 3.5mm at the point of entry into RV. As we could not see any PDA arising from RCA and a twig from LCX traversing in interventricular groove, we thought it’s a left dominant coronary circulation. Hemodynamics in the index case The symptoms of heart failure in the index neonate is due to the volume overload on the RV. The non-dominance of RCA is probably a blessing in disguise for this baby in preventing coronary steal. The "steal phenomenon" will be apparent once the physiological (high) PVR comes down. Plan Baby is on diuretics and nutritional support, under weekly follow up. We will be performing catheter study (around 12 weeks of life or as per clinical indications) to look for dominance and need/feasibility of device closure. Abstract P876 Figure.

Late Presentation of Arrhythmogenic Right Ventricular Cardiomyopathy : Role of Non Invasive Modalities for The Diagnosis

Background : Arrhythmogenic right ventricular cardiomyopathy(ARVC) is an inherited myocardial disease affecting predominantly young people and manifests as sustained ventricular tachycardia, sudden cardiac death (SCD) or heart failure. However, its first manifestation in older patients is infrequent. Diagnosis of ARVC remains a clinical challenge and need further investigation. Our case report investigated role of non invasive modalities for diagnosis of ARVC patient.&#x0D; Case Presentation : A 65 year old man was admitted to the hospital with symptoms of palpitationand near syncope. An Electrocardiogram (ECG) showed a sustained VT with LBBB morphology and inferior axis. The patient was cardioverted to sinus rhytm with a single 100J shock. Postcardioversion ECG showed an epsilon wave in right precordial leads. Echocardiography revealed extensive RV enlargement and reduce function. Our patient had three major (RV aneurysm, epsilon wave and T wave inversion) and one minor criteria (sustained LBBB type-VT with inferior axis)making the diagnosis of ARVC definite according to the revised Task Forced Criteria.&#x0D; Conclusion: ARVC may have a very late presentation and this diagnosis should be considered as a potential cause of sustained VT of RV origin among the elderly. ECG and echocardiography as non invasive modalities have an important role for the diagnosis of patients with suspected ARVC.&#x0D; Keywords :Arrhythmogenic right ventricular cardiomyopathy,ventricular tachycardia, sudden cardiac death, diagnosis.

P6592Novel heart-specific LncRNAs identified by deep sequencing contribute to pathogenesis in arrhythmogenic cardiomyopathy

Abstract Background Arrhythmogenic Cardiomyopathy (AC) is one of main causes of sudden cardiac death in young people with limited understanding of molecular mechanism. LncRNAs are emerging as important regulators of cardiovascular diseases, but their roles in AC remain elusive. Purpose This study is to identify novel lncRNAs in AC genome wide and investigate their functions. Methods and results Extremely deep RNA-seq was performed for AC right ventricles (RVs, n=9) and left ventricles (LVs, n=6), normal RVs (n=5) and LVs (n=6), and dilated cardiomyopathy (DCM) RVs (n=4). Novel lncRNAs were accurately identified with epigenetic annotations. In comparison with normal and DCM, 1,925 novel lncRNAs transcripts specifically upregulated in AC RVs were identified. Most of AC-specific lncRNAs were highly expressed in heart, located in distal regions (&gt;3 kb) from coding gene and marked by H3K4me1 or H3K27ac, indicating that these lncRNAs might act as cis-elements to regulate gene expression. Enrichment analysis revealed that AC-specific lncRNAs were associated with AC phenotypes like RV enlargement and dysfunction. Interestingly, their potential target genes were significantly enriched in lipid metabolism and calcium signaling pathways. In-vitro experiments demonstrated that LncRNA AC1, a novel AC-specific lncRNA, was significantly positively correlated with adipogenic genes expression. And overexpression of LncRNA AC1 promoted adipogenic genes expression and droplet deposition in human derived iPSC-CM and cardiac fibroblast. ESC-AC lncRNA study abstract Conclusion We identified novel AC-specific lncRNAs related to adipogenesis and validated them in-vitro. Our findings also provide fundamental resource for the potential development of basic translation study for AC. Acknowledgement/Funding This study was supported by CAMS Innovation Fund for Medical Sciences (2016-I2M-1-015)

Echocardiographic Predictors of Long-Term Mortality in Patients Presenting With Acute Pulmonary Embolism

Pulmonary embolism (PE) is associated with a high mortality; whether echocardiographic evaluation at presentation predicts long-term adverse outcomes is of importance. We sought to determine if a composite of routinely obtained echocardiographic parameters could determine long-term adverse events in PE patients. Right ventricular (RV) size and function and right atrial (RA) size were retrospectively evaluated in 233 consecutive PE patients with an inpatient echocardiogram, and compared with 70 healthy controls; mortality at 3 years was confirmed. PE patients had increased RV size (RV parasternal long-axis diameter [RVPLAX] and RV end-diastolic volume [p < 0.001 for both]) and RA area (p < 0.001). RV function was reduced in PE patients (RV fractional area change and RV ejection fraction [p <0.001 for both]). Peak tricuspid regurgitation (TR) velocity was higher in the PE group. At follow-up (3.0 ± 2.1 years), 61 patients died; multivariable analysis demonstrated RVPLAX diameter >37 mm (hazard ratio [HR] 2.3, 95% confidence interval [CI] 1.3 to 4.2; p = 0.005), RA area >20 cm2 (HR 2.0, 95% CI 1.1 to 3.5; p = 0.016), and TR velocity >2.9 ms-1 (HR 1.9, 95% CI 1.1 to 3.4; p = 0.021), were independent echocardiographic predictors of mortality. Patients with all 3 "risk markers" had ∼17-fold increased mortality compared with those with no "risk markers" (HR 16.9, 95% CI 6.1 to 47.2; p < 0.001). In conclusion, a composite of routinely collected echocardiographic parameters, namely an enlarged RA and RV (RVPLAX diameter), and TR velocity, were independent predictors of mortality in PE patients, with an exponential increase in mortality when all 3 parameters were significantly altered. Prospective validation is required to confirm these preliminary observations.

The right heart of the elite senior rugby football league athlete.

Right heart enlargement is common in the athletes' heart phenotype; however, few data exist regarding interpretation of normal athletic adaptation during Preparticipation Cardiac Screening (PCS) of Rugby Football League (RFL) athletes. Echocardiography is utilized during PCS and thus the primary aim of this study was to establish the normal right ventricular (RV) phenotype in elite RFL athletes using standard 2-D echocardiography and myocardial mechanics. The secondary aim was to describe right atrial (RA) structure and function using 2-D echocardiography. 139 male RFL athletes underwent echocardiographic evaluation of the right heart including RV strain (ɛ) and strain rate (SR) imaging using speckle tracking echocardiography (STE). Nonathletic males were used for comparison and allometric scaling was applied for conventional echocardiographic parameters. Scaled RV dimensions were larger in athletes (P<0.05) with the exception of the mid-cavity. No differences (P>0.05) in RV fractional area change (FAC) and RV longitudinal ɛ were observed between groups. Tissue Doppler imaging (TDI)-indexed parameters and global strain rate (SR) were lower (P<0.05) in athletes with HR and weight found to have co-variance with SR. The RA was larger in athletes (P<0.001) with no functional difference (P>0.05) observed by volume assessment. Reduction in SR and indexed TDI are partly associated with lower HR and increased body mass and are likely to represent normal physiological adaptation in RFL athletes. RA enlargement appears proportional to RV enlargement. These data may aid interpretation of normal athletic adaptation during PCS of RFL athletes.

Myocardial segmental thickness variability on echocardiography is a highly sensitive and specific marker to distinguish ischemic and non-ischemic dilated cardiomyopathy in new onset heart failure

The aim of this study was to determine non-invasive diagnostic markers by echocardiography that differentiate ischemic dilated (ICM) from non-ischemic dilated cardiomyopathy (NICM) in patients with new onset heart failure. We identified 100 consecutive new heart failure patients with dilated cardiomyopathy (valvular etiology excluded). Clinical risk factors, medication history, serum biomarkers, ECG and echocardiographic variables were compared between the ICM and NICM groups (as confirmed by coronary angiography). Mean age, left ventricular size and ejection fraction were 56 years, 6.1 cm and 26% respectively. A total of 24% had ICM. Patients with ICM were older (65 vs. 53 years; P < 0.001). No significant difference was observed between ICM and NICM among 18 clinical variables, 7 laboratory tests, 6 EKG parameters and 10 of the 13 echocardiographic markers evaluated. Segmental wall thickness variability, regional wall motion abnormality and RV enlargement on echocardiogram (echo) differentiated ICM from NICM. Segmental thickness variability outperformed wall motion abnormality in diagnosing ICM with a sensitivity and specificity of 79.2 and 98.7% versus 62.5 and 84.2% respectively. RV enlargement was not sensitive but 90.6% specific for predicting NICM. Myocardial segmental thickness variability on echo, resulting from thinned infarcted or hibernating myocardium, is a highly sensitive and specific marker to differentiate ICM from NICM in new onset heart failure.

Physicians’ Ability to Visually Estimate Left Ventricular Ejection Fraction, Right Ventricular Enlargement, and Paradoxical Septal Motion After a 2-Day Focused Cardiac Ultrasound Training Course

Focused cardiac ultrasound (FCU) can provide useful information for the management of shock and acute respiratory distress syndrome. This study aimed to determine whether a 2-day focused cardiac ultrasound training course could enable critical care physicians to interpret ultrasound images in terms of left ventricular ejection fraction (LVEF), ratio of right ventricular end-diastolic area to left ventricular end-diastolic area (R/LVEDA), and septal kinetics. A prospective analysis of an image test score. Ultrasound training programs in 7 regions across China. Two hundred forty-seven critical care physicians. All participants received a 2-day FCU training, including 4 sessions of basic heart function appraisal, 3 sessions of hands-on practice, and 1 session of image interpretation. The post-training total scores were considerably higher than those of pretraining (75.6% v 58.9%, respectively, p < 0.001). After the course, the trainees obtained considerably higher scores on images with LVEF <30% than on images with LVEF 30% to 54% and LVEF ≥55% (100% v 60.0% and 60.0%, respectively, p < 0.001). The trainees obtained considerably higher scores on images with R/LVEDA >1 than on images with R/LVEDA 0.6 to 1 and R/LVEDA <0.6 (90.0% v 80.0% and 80.0%, p = 0.042 and p < 0.001, respectively). The trainees obtained considerably higher scores on images with paradoxical septal movement (PSM) than on images without PSM (100% v 75.0%, respectively, p < 0.001). The physicians' abilities to assess LVEF, RV enlargement, and PSM improved after the training course, and they demonstrated more accurate estimations of the most obviously abnormal images.

Right ventricular enlargement predicts responsiveness to tolvaptan in congestive heart failure patients with reduced ejection fraction

BackgroundTolvaptan is a vasopressin type 2 receptor antagonist used in heart failure (HF) with refractory diuretic resistance. However, since tolvaptan is also ineffective in some HF patients with reduced ejection fraction (HFrEF), the identification of responders is important. MethodsThe study population consisted of 51 HFrEF patients who were administered tolvaptan (EF, 28 ± 7%). We defined responders as patients with a ≥50% increase in urine volume during the 24-hours after administration of tolvaptan. All patients underwent comprehensive transthoracic echocardiography before administration of tolvaptan. Patients were followed for 120 days to ascertain secondary events (cardiac death and rehospitalization for HF). ResultsMultiple regression analysis indicated that right ventricular (RV) enlargement (defined as basal RV diameter > 41 mm and midlevel RV diameter > 35 mm, according to guidelines) remained a predictor of response after adjustment for age, sex, starting dosage of tolvaptan, and estimated glomerular filtration rate (odds ratio, 4.88; 95%-confidence interval, 1.26–18.9; P < 0.05), whereas left ventricular parameters and RV dysfunction were not. Kaplan-Meier curves indicated responsiveness to tolvaptan was associated with better prognosis among the overall population (P < 0.05); similar trends were observed among patients with RV dilatation (P = 0.056). ConclusionsThese findings suggest that RV enlargement, which represents right-sided volume overload, elevated filling pressure, and diastolic dysfunction similar to that seen in constrictive pericarditis, predicts responsiveness to tolvaptan in patients with HFrEF. Moreover, administration of tolvaptan may have the potential to improve the reportedly poor prognosis for HFrEF patients with RV dilatation.

ANGIOIMMUNOBLASTIC T-CELL LYMPHOMA AS A CAUSE OF PERSISTENT PULMONARY HYPERTENSION

SESSION TITLE: Medical Student/Resident Pulmonary Vascular Disease 1 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Angioimmunoblastic T-cell lymphoma (AITL) is rare, accounting for 15-20% of peripheral T-cell lymphomas. Diagnosis can be challenging, as AITL may present with symptoms such as rash and arthralgias more suggestive of an autoimmune disorder (1). There are very few published reports of lymphoma-associated pulmonary hypertension (PH), most involving intravascular B-cell lymphomas (2). Here we present a case of AITL-associated PH that did not resolve with lymphoma remission. CASE PRESENTATION: A previously healthy 63-year-old woman presented after weeks of fevers, arthralgias, rashes, and dyspnea on exertion. Pulmonary function testing and CT chest were initially unremarkable. After an equivocal work-up she was provisionally diagnosed with Still’s disease, but saw no improvement on colchicine or anakinra. FDG-PET obtained a year after presentation revealed new pleural and pericardial effusions, splenomegaly, and diffuse lymphadenopathy, but no abnormal uptake in the lungs. Echocardiogram revealed mild RV enlargement with reduced function and an estimated RVSP of 73. A lymph node biopsy was consistent with AITL, a diagnosis further supported by the presence of a TCR gene rearrangement. She was started on chemotherapy with CEOP and had complete response after 2 cycles. Despite her excellent response to chemotherapy, her dyspnea rapidly progressed and repeat PFTs showed interval development of a moderately reduced diffusion capacity. Repeat CT chest was again negative for pulmonary infiltrates and V/Q scan did not suggest chronic embolism. On physical exam she now had JVD, 2+ pitting edema to her knees, and cool extremities. Right heart catheterization confirmed severe PH with mPAP 46 mmHg, normal PAWP, and PVR 13 WU. A thorough evaluation for other causes of PH was unrevealing. The patient’s symptoms improved on PDE5i and ERA therapy; HRCT did not reveal signs of PVOD nor did she develop hypoxia on treatment. DISCUSSION: Lymphoma is a rare cause of PH, and to our knowledge this is only the second reported case of PH due to AITL (3). In that case, as well as in other cases of lymphoma-associated PH, the PH improved or resolved with lymphoma remission. Our patient’s PH continued even after complete response to chemotherapy, and we hypothesize that the delay in diagnosis allowed time for fixed remodeling of the pulmonary arterial endothelium. CONCLUSIONS: Lymphoma may be an underappreciated cause of PH, and should be considered in patients with unexplained PH. AITL, in particular, should be considered in patients with PAH and symptoms suggestive of a systemic rheumatologic disorder. Imaging devoid of interstitial changes does not preclude this diagnosis, particularly once PE, PVOD, and pulmonary capillary hemangiomatosis have been considered. Treatment with PAH therapy was safe and effective in this case. Reference #1: de Leval et al. Br J Haematol. 2010 Mar; 148(5): 673-89 Reference #2: Nelson et al. J Thorac Oncol. 2012 Dec; 7(12): e34-5 Reference #3: Hua J et al. J Hematol Malig. 2012; 2(3): 26. DISCLOSURES: No relevant relationships by Anna Bracewell, source=Web Response No relevant relationships by Sachin Gupta, source=Web Response

Right-Sided Heart Structural and Functional Remodeling in Mitral Regurgitation Secondary to Mitral Valve Prolapse

Preoperative evaluation of the mitral valve but also of tricuspid valve and right ventricular (RV) function is mandatory in primary mitral regurgitation (MR) secondary to mitral valve prolapse (MVP). Tricuspid annulus (TA) diameter plays a pivotal role in the surgical decision to perform preventive combined tricuspid valve annuloplasty. Cardiac magnetic resonance (CMR) is the gold standard for the assessment of RV size and function. Based on 70 consecutive patients (17 women; mean age 64 ± 12) with severe MR secondary to MVP referred for CMR, we sought to assess RV geometry and function and TA dimensions and to study the interaction between TA dilatation and right-sided cardiac chambers. Frequency of RV dilatation, RV systolic dysfunction, and TA dilatation (TA diameter ≥ 40 or 21 mm/m²) were 11%, 51%, and 49%, respectively. Left ventricular (LV) end-diastolic volume index was the only independent predictor of RV dilatation. Presence of symptoms, larger LV end-diastolic volume index, and LV ejection fraction <60% were independently associated with RV dysfunction. Absolute TA diameter was 36 ± 6 mm and TA diameter index was 20 ± 3 mm/m². Reproducibility TA diameter measurement was excellent (coefficient of variation ≤10%). TR velocity >220 cm/s (odds ratio = 20.17; [3.57 to 113.90]; p = 0.001 and right atrial volume index ≥ 38 ml/m² (odds ratio = 13.44; [3.57 to 50.54]; p = 0.0001) were independent predictors of TA diameter ≥40 or 21 mm/m². CMR provides accurate right-sided cardiac chambers assessment and may help surgical planning of concomitant tricuspid valve annuloplasty before mitral valve repair in severe MR secondary to MVP. In conclusion, TA dilatation, RV enlargement, and dysfunction are related to pulmonary pressure and left-sided cardiac chambers enlargement, reflecting the long-standing consequences of severe MR.

Echocardiographic evaluation of the right ventricular dimension and systolic function in dogs with pulmonary hypertension.

BackgroundRight ventricular (RV) enlargement and dysfunction are associated with prognosis in humans with pulmonary hypertension (PH).Hypothesis/ObjectivesTo assess RV size and systolic function in dogs with PH and to determine if they are associated with disease severity and right‐sided congestive heart failure (R‐CHF).Animals89 dogs with PH and 74 healthy dogs.MethodsProspective observational study. PH was classified according to the tricuspid regurgitation pressure gradient. RV end‐diastolic area (RVEDA) index was calculated as RVEDA divided by body surface area. RV systolic function was assessed with the tricuspid annular plane systolic excursion (TAPSE) and the RV fractional area change (FAC) normalized for body weight (TAPSEn and FACn, respectively).ResultsRVEDA index was higher in dogs with moderate PH (10.8 cm2/m2; range, 6.2‐14.4 cm2/m2) and severe PH (12.4 cm2/m2; range, 7.7‐21.4 cm2/m2) than in those with mild PH (8.4 cm2/m2; range, 4.8‐11.6 cm2/m2) and control dogs (8.5 cm2/m2; range, 2.8‐11.6 cm2/m2; P < .001). RVEDA index was significantly higher in dogs with R‐CHF (13.7 cm2/m2; range, 11.0‐21.4 cm2/m2) than in dogs without R‐CHF (9.4 cm2/m2; range, 4.8‐17.1 cm2/m2; P < .001). The severity of tricuspid regurgitation (TR) was the only independent predictor of the RVEDA index (P < .001). TAPSEn and FACn were not significantly different among varying degrees of PH severity and between dogs with and without R‐CHF.Conclusions and Clinical ImportanceThe RVEDA index can be used to evaluate RV size in dogs. It can provide additional information in dogs with PH and predict R‐CHF. Severity of TR is the main determinant of RV enlargement in dogs with PH.

Slow Conducting ElectroanatomicIsthmuses: An Important Link BetweenQRS Duration and Ventricular Tachycardia inTetralogyof Fallot.

This study sought to evaluate the influence of slow conducting anatomic isthmuses (SCAI) as dominant ventricular tachycardia (VT) substrate on QRS duration. QRS prolongation has been associated with VT in repaired tetralogy of Fallot. Seventy-eight repaired tetralogy of Fallot patients (age 37 ± 15 years, 52 male, QRS duration 153 ± 29 ms, 67 right bundle branch blocks [RBBB]) underwent programmed stimulation and electroanatomic activation mapping during sinus rhythm. Right ventricular (RV) surface, RV activation pattern, RV activation time, conduction velocity at AI, and remote RV sites were determined. Twenty-four patients were inducible for VT (VT+); SCAI was present in 22 of 24 VT+ but only in 2 of 54 patients without inducible VT (VT-). Conduction velocity through AI was slower in VT+ patients (median of 0.3 [0.3 to 0.4] vs. 0.7 [0.6 to 0.9] m/s; p < 0.01) but conduction velocity in the remote RV did not differ between groups. In non-RBBB, QRS duration was similar in VT+ patients (n= 6) and VT- patients (n= 5), but RV activation within SCAI exceeded QRS offset in VT+ patients (37 ± 20 ms vs. -5 ± 9 ms, p< 0.01). In RBBB, both QRS duration and RV activation time were longer in VT+ patients (n= 18, 17 of 18 QRS > 150 ms) compared with VT- patients (n= 49, 27 of 49 QRS > 150 ms) (173± 22 ms vs. 156 ± 20 ms; p< 0.01; 141 ± 22 ms vs. 129± 21 ms; p= 0.04). In VT+ patients, QRS prolongation >150ms (n= 17) was due to SCAI or blocked isthmus in 15 patients (88%) and 1 (6%). In contrast, in VT- patients, QRS prolongation >150 ms (n= 27) was due to enlarged RV or blocked isthmus in 10 patients (37%) and 8 (30%), but due to SCAI in only 1 (4%). After exclusion of a severely enlarged RV, a QRS duration >150 ms was highly predictive for SCAI/blocked AI (OR: 17; 95% CI: 3.3 to 84; p< 0.01). A narrow QRS interval does not exclude VT-related SCAI. In the presence of RBBB, SCAI further prolongsQRS duration. QRS duration >150 ms is highly suspicious for SCAI or isthmus block distinguishable by electroanatomic mapping.

Brief cardiovascular imaging with pocket-size ultrasound devices improves the accuracy of the initial assessment of suspected pulmonary embolism

Pulmonary embolism onset is frequently neglected due to the non-specific character of its symptoms. Pocket-size imaging devices (PSID) present an opportunity to implement imaging diagnostics into conventional physical examination. The aim of this study was to test the hypothesis that supplementation of the initial bedside assessment of patients with suspected pulmonary embolism (PE) with four-point compression venous ultrasonography (CUS) and right ventricular size assessment with the use of PSID equipped with dual probe could positively influence the accuracy of clinical predictions. A single-centre, prospective analysis was conducted on 100 patients (47 men, mean age 68 ± 13 years) with suspected PE. Clinical assessment on the basis of Wells and revised Geneva score and physical examination were supplemented with CUS and RV measurements by PSID. The mean time of PSID scanning was 4.9 ± 0.8 min and was universally accepted by the patients. Fifteen patients had deep venous thrombosis and RV enlargement was observed in 59 patients. PE was confirmed in 24 patients. If the both CUS was positive and RV enlarged, the specificity was 100% and sensitivity 54%, ROC AUC 0.771 [95% CI 0.68–0.85]. The Wells rule within our study population had the specificity of 86% and sensitivity of 67%, ROC AUC 0.776 (95% CI 0.681–0.853, p < 0.0001). Similar values calculated for the revised Geneva score were as follows: specificity 58% and sensitivity 63%, ROC AUC 0.664 (95% CI 0.563–0.756, p = 0.0104). Supplementing the revised Geneva score with additional criteria of CUS result and RV measurement resulted in significant improvement of diagnostic accuracy. The difference between ROC AUCs was 0.199 (95% Cl 0.0893–0.308, p = 0.0004). Similar modification of Wells score increased ROC AUC by 0.133 (95% CI 0.0443–0.223, p = 0.0034). Despite the well-acknowledged role of the PE clinical risk assessment scores the diagnostic process may benefit from the addition of basic bedside ultrasonographic techniques.

Large animal model of acute right ventricular failure with functional tricuspid regurgitation

BackgroundFunctional tricuspid regurgitation (FTR) commonly arises secondary to conditions affecting the left heart and is associated with right ventricular dysfunction and tricuspid annular dilatation. We set out to establish an animal model of acute RV failure (RVF) with FTR resembling the clinical features. MethodsTen adult sheep had pressure sensors placed in the LV, RV, and right atrium while sonomicrometry crystals were implanted around tricuspid annulus and on the RV. Animals were studied open-chest to assess for RV function and FTR after: (1) volume infusion, (2) pulmonary artery constriction, (3) 5 min posterior descending artery occlusion, and (4) combination of all interventions. Hemodynamic, echocardiographic, and sonomicrometry data were collected at baseline and after every intervention. RV dimensions, RV strain, and annular area, perimeter, and size were calculated from crystal coordinates. The model was validated in six additional sheep studied only before and after combined interventions. ResultsNeither volume infusion, pulmonary hypertension, nor ischemia were associated with RVF or clinically significant TR when applied separately but combined resulted in RVF and greater than moderate FTR. In the validation group, maximal RV volume increased (62 ± 14 vs 70 ± 16 ml, p = 0.006), contractility decreased (20 ± 6 vs 12 ± 2%, p = 0.02), and strain increased. FTR increased from 0.4 ± 0.5 to 2.5 ± 0.8 (p < 0.001) and annular area from 652 ± 87 mm2 to 739 ± 87 mm2 (p = 0.005). ConclusionsThe developed ovine model of acute RVF was associated with significant annular and RV enlargement and FTR. This novel and clinically pertinent research platform offers insight into the acute RVF pathophysiology and can be utilized to evaluate treatment interventions.