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A one-year-old, French Bulldog was referred for the management of a severe form of pulmonary valve stenosis (PS) complicated by right-sided congestive heart failure. Echocardiography showed severe valvular PS with right ventricular concentric hypertrophy, dilatation and severe right atrial enlargement. A pulmonary balloon valvuloplasty (PBV) was performed with a balloon-to-pulmonary annulus ratio of 1.36. Echocardiography immediately after PBV showed a significant reduction in right atrial and ventricular size, improved opening and mobility of the pulmonary valve leaflets, and a 75% reduction in the pulmonary pressure gradient from 158 mmHg pre-operative to 40 mmHg post-operative. The dog recovered well from anesthesia, but two hours later suddenly showed severe respiratory distress. Focus cardiac ultrasound showed increased left cardiac size with echocardiographic signs of high left ventricular filling pressure. Bedside lung ultrasound showed diffuse numerous-to-confluent B lines, compatible with severe alveolar-interstitial syndrome. The dog was treated with furosemide, helmet continuous positive airway pressure and then mechanical ventilation but without success. At post-mortem evaluation, histological examination of the lung showed diffuse, severe broncho-alveolar edema with mixed leukocyte, fibrin and red blood cell infiltrate. Moreover, severe congestion and multifocal alveolar hemorrhages were evident. All findings were compatible with fatal acute lung injury after PBV secondary to pulmonary reperfusion-ischemia injury and increased pulmonary capillary hydrostatic pressure. Based on the present case, acute lung injury should be considered as a rare but serious complication of PBV.

Background: Rheumatic Heart disease (RHD) is the leading cause of acquired heart disease and more prevalent in developing countries. The objective of the study was to determine the clinical and echocardiographic profiles of patients with rheumatic heart disease (RHD). Materials and Methods: All patients above the age of 12 years of either gender diagnosed with Rheumatic heart disease from January 2018 to December 2019 at tertiary care hospital, Hyderabad were enrolled in the study. Detailed patient data was obtained from the hospital records. Information regarding gender, age, Electrocardiogram findings, clinical and echocardiographic manifestations was obtained. Results: Throughout the two-year study period, a total of fifty patients with RHD were studied. The mean age of the population was 33.71 years. Females (64%) outnumbered males and 60% were rural population. The clinical features observed were fever (80%), joint pain (64%), palpitation (60%), breathlessness (90%) and chest pain (50%), tachycardia (60%), raised JVP (10%), hypertension (20%). The common echocardiographic findings observed were mitral stenosis (50%), mitral regurgitation (24%) and pulmonary hypertension (36%), left atrial enlargement (56%), and right atrial enlargement (16%) whereas the majority of the patients (70%) have ejection fraction between 40-50% and thrombus was identified in 15 (30%) patients respectively. Conclusion: Common clinical findings observed were breathlessness and fever while the common echocardiographic findings were mitral stenosis and regurgitation, pulmonary hypertension, and left atrial enlargement. Population-based screening by echocardiography could be a potential route for early screening of rheumatic heart disease.

IIntroduction Isolated tricuspid valve prolapse (TVP) is a rare finding on transthoracic echocardiography. Right atrial enlargement or prominent ” v ” waves as a consequence of hemodynamic changes in severe tricuspid regurgitation (TR) are rarely seen with isolated TVP. Here is a case of isolated prolapse of anterior tricuspid leaflet presenting with giant C-V waves also known as Lancisi’s sign. Case Report A 66-year-old male presented with increasing exercise limitation and leg edema in recent months and was complaining about the persistent pulsation at his neck and elevated jugular venous pulse with prominent systolic pulsation that represents giant C-V waves, also known as ‘Lancisi’s sign’ consequence of severe TR due to isolated prolapse of the anterior leaflet.The patients’ symptoms resolved completely after tricuspid valve replacement. Discussion TVP is best defined at parasternal short axis view with more than 2 mm atrial displacement (AD) of leaflet/leaflets. TVP can also be detected from four chamber view with more than 2 mm AD or in right ventricular inflow view with more than 4 mm AD. As a consequence of TVP, the physiological jugular venous waveform alters due to severe TR.During severe TR; retrograde blood flow through right atrium during ventricular systole restrains x descent and produces a fusion of c and v waves that appears as a large pulsation in physical examination called as ‘Lancisi’s sign’ Conclusion ‘Lancisi’s sign’ is defined as a large visible systolic neck pulsation as a consequence of the c-v waves fusion by preventing x descent during severe TR.

Background The electrophysiological substrate underlying atrial fibrillation (AF) progression remains difficult to identify. Objective The goals of this study were to study the evolution of post–pulmonary vein isolation (PVI) AF inducibility (AFI) after AF ablation and to compare patients with organized atrial tachycardia recurrence (OATr) versus those with paroxysmal or persistent AF recurrence. Methods We studied 99 patients who underwent de novo AF ablation (p1) and redo ablation (p2) for AF recurrence (AFr) or OATr. Stepwise AF ablation was performed at p1 and p2: (1) PVI, (2) coronary sinus defragmentation, and (3) left atrial (LA) defragmentation. Burst pacing followed each step, with AFI defined as sustained AF >5 minutes, triggering the next step. Patients with OATr underwent OAT ablation and inducibility testing post-redo PVI. Inducibility progression (IP) was defined as AFI at further steps of p2 compared to p1. Results Among patients with AFr, 34 of 72 patients (47%) exhibited post-PVI IP vs 2 of 27 (7.4%) patients with OATr (P = .0002). Stratification for persistent AF/paroxysmal AF/OATr showed a consistent association between recurrence phenotype and IP. Pulmonary vein (PV) reconnection incidence was 90%, without association with recurrence type or IP. LA volume was larger in patients with IP than in those without IP (86.7 ± 25.3 mL vs 72.0 ± 28.9 mL; P = .001). Right atrial dimensions increased between p1 and p2 in patients with IP vs no IP and in patients with AFr vs OATr. Conclusion Patients with AFr after first ablation exhibit IP more frequently at redo ablation than do patients with OATr. IP correlates with more advanced AFr type, larger LA volumes, and progressive right atrial enlargement. PV reconnection is not associated with AFr. Changes in post-PVI AFI may accurately indicate progression of extra-PV AF-maintaining substrate.

OBJECTIVE We examined the atrial tachyarrhythmia (AT) burden among patients with congenital heart disease (CHD) following transcatheter (TC-) or surgical (S-) pulmonary valve replacement (PVR). DESIGN/SETTING This was a retrospective observational study of patients who underwent PVR from 2010 to 2016 at UCLA Medical Center. PATIENTS Patients of all ages who had prior surgical repair for CHD were included. Patients with a history of congenitally corrected transposition of the great arteries, underwent a hybrid PVR procedure, or had permanent atrial fibrillation (AF) without a concomitant ablation were excluded. OUTCOME MEASURES The primary outcome was a time-to-event analysis of sustained AT. Sustained ATs were defined as focal AT, intra-atrial reentrant tachycardia/atrial flutter, or AF lasting at least 30 seconds or terminating with cardioversion or antitachycardia pacing. RESULTS Two hundred ninety-seven patients (TC-PVR, n = 168 and S-PVR, n = 129) were included. During a median follow-up of 1.2 years, nine events occurred in TC-PVR group (5%) vs 23 events in S-PVR group (18%). In the propensity adjusted models, the following factors were associated with significant risk of AT after PVR: history of AT, age at valve implantation, severe right atrial enlargement, and S-PVR. In the secondary analysis, TC-PVR was associated with lower adjusted risk of AT events in the postoperative epoch (first 30 days), adjusted IRR 0.31 (0.14-0.97), P = .03, but similar risk in the short-term epoch, adjusted IRR 0.64 (0.14-2.94), P = .57. CONCLUSION There was an increased risk of AT in the first 30 days following S-PVR compared to TC-PVR. Additional factors associated with risk of AT events after PVR were a history of AT, age at valve implantation, and severe right atrial enlargement.

Clinical Presentation A full-term neonate was referred to our institution because of respiratory distress. CXR was significant for right lung hypoplasia and mild cardiomegaly. ECG showed normal sinus rhythm, right atrial enlargement, and right ventricular hypertrophy with no signs of ischemia. Imaging Findings The initial echocardiogram demonstrated PAPVD with the right upper pulmonary vein draining into IVC/RA junction with flow acceleration (mean gradient= 7 mmHg), moderate ASD, small muscular VSD with left-right shunting, moderate PDA with bidirectional shunting. Forward flow was seen in the proximal part of left main coronary artery (LMCA). RV systolic pressure was supra-systemic with a qualitatively moderately reduced RV systolic function. The patient was taken to the catheterization lab where MPA angiography revealed an antegrade flow from the RPA into LMCA supplying both the anterior descending and the circumflex arteries. A selective injection within the scimitar vein showed drainage of the right lung into a vertical vein connecting with stenosis to IVC. A follow up echocardiogram to re-examine the coronary origin revealed an anomalous origin of LMCA from proximal RPA; 3 mm distal to branch pulmonary artery bifurcation with mainly antegrade low velocity flow into LMCA and LAD. (Image 1) Role of Imaging in Patient Care - Imaging of the coronary origin in patients with ALCAPA can be challenging especially if the LMCA originates from RPA. Also, the presence of pulmonary hypertension might contribute to maintain coronary perfusion and lead to misinterpretation of the antegrade flow in LMCA and its branches. - In certain situations, cardiac catheterization is essential to make the diagnosis of ALCAPA which prevented a potentially catastrophic outcome. Catheter intervention with a series of balloon dilations of the stenotic scimitar vein was successful in relieving the stenosis. Summary/Discussion Points: - Extensive review of the available literature revealed only three cases of Scimitar syndrome associated with ALCAPA. In all of these cases, the LMCA originated from the posterior sinus of MPA. Our case is the first to report ALCAPA from RPA in association with Scimitar syndrome. This presentation might have led to the initial misinterpretation of the echocardiography images. - The presence of pulmonary hypertension in our patient maintained an adequate antegrade flow across the LMCA preventing significant coronary steal and signs of myocardial ischemia. - The report highlights the challenges in making the diagnosis of ALCAPA with echocardiograms. Moreover, we discuss the role of cross-sectional and invasive imaging to rule out potential coronary arteries anomalies in patients with Scimitar syndrome, as this a rare although a very significant association that may have important implications in their outcomes. Abstract P1488 Figure. ALCAPA origin from RPA

Background Sports activity has become extremely popular among amateurs. Electrocardiography is a useful tool in screening for cardiac pathologies in athletes; however, there is little data on electrocardiographic abnormalities in the group of amateur athletes. Objectives The aim of this study was to analyze the abnormalities in resting and exercise electrocardiograms (ECGs) in a group of amateur athletes, and try to determine whether the criteria applied for the general population or for athletes' ECGs should be implemented in this group. Material and methods In 40 amateur male marathon runners, 3 consecutive 12-lead ECGs were performed: 2-3 weeks before (stage 1), just after the run (stage 2) and 2-3 weeks after the marathon (stage 3). Resting (stage 1) and exercise (stage 2) ECGs were analyzed following the refined criteria for the assessment of athlete's ECG (changes classified as training-related, borderline or training-unrelated). Results In resting ECGs, at least 1 abnormality was found in 92.5% of the subjects and the most common was sinus bradycardia (62.5%). In post-exercise ECGs, at least 1 abnormality was present in 77.5% of the subjects and the most common was right atrium enlargement (RAE) (42.5%). Training-related ECG variants were more frequent at rest (82.5% vs 42.5%; p = 0.0008), while borderline variants - after the run (22.5% vs 57.5%; p = 0.0004). Training-unrelated abnormalities were found in 15% and 10% of the subjects, respectively (p-value - nonsignificant), and the most common was T-wave inversion. Conclusions Even if the refined criteria rather than the criteria used for normal sedentary population were applied, the vast majority of amateur runners showed at least 1 abnormality in resting ECGs, which were mainly training-related variants. However, at rest, in 15% of the subjects, pathologic training-unrelated abnormalities were found. The most frequent post-exercise abnormality was right atrial enlargement. General electrocardiographic screening in amateur athletes should be taken into consideration.

Twenty patients with secundum atrial septal defect, who had undergone open heart surgery were studied retrospectively. Girls were more affected than boys; the sex ratio was 1.5 : I. Associated cardiac defects were diagnosed in two patients, one with moderate valvular pulmonic stenosis and the other one with small ventricular septal defect. Typical clinical findings consisted of loud first heart sound, widely fixed split second heart sound and soft ejection systolic murmur at the upper left sternal border were heard in all cases. Mid diastolic murmur due to relative tricuspid stenosis was detected in most cases (75%). Electrocardiographic findings included right axis deviation, prolonged PR-interval and right atrial enlargement were found in 50%, 15% and 60% of cases, respectively. Incomplete right bundle branch block and right ventricular enlargement were found in all cases, as was cardiomegaly with increased vascular markings were found in all cases. Paradoxical ventricular septal motion and visualization of the atrial septal defect were seen in 95% and 75% of cases, respectively. Cardiac catheterization was performed in 19 patients (95%). The pulmonary-systemic flow ratio (Qp/Qs) ranged from 1.7 to 6.3 (mean 2.9 ± 0.67), and was correlated to the presence of mid diastolic tricuspid flow murmur and paradoxical ventricular septal motion. Simple closure of the defect was the procedure of choice, but in one patient (5%) pericardial patch was used to close the very large defect. The mortality rate was 10 percent. Physical retardation was found in all boys and 50% of girls, before surgery. Body weight percentile increased in most cases (61.1 %), while body height percentile increased in only 5.6% of cases, postoperatively. Ejection systolic murmur at the upper left sternal border was still detected in one patient (5.6%). lncomplele right bundle branch block persisted in all cases, while cardiomegaly was still found in 5. 6% of cases followed up six months to five years after surgery. There was no residual left ventricular dysfunction in all cases.

Sinus venosus atrial septal defect (SVASD) is a rare adult congenital heart disease which permits shunting of blood from the systemic to the pulmonary circulation and is commonly associated with anomalous pulmonary venous return. We report a case of a 27-year-old man with a history of premature birth and unilateral cryptorchidism who was admitted for syncope. Electrocardiogram (ECG) demonstrated atrial fibrillation (AF)and S1Q3T3 pattern along with an incomplete right bundle branch block. Transthoracic echocardiography (TTE) suggested the presence of right ventricular pressure and volume overload and severe right ventricular and right atrial enlargement. The agitated saline study was negative suggesting no inter-atrial communication. Transesophageal echocardiography (TEE) demonstrated a superior SVASD and raised the possibility of an anomalous pulmonary venous connection. Chest computed tomography identified the right superior pulmonary vein connection to the superior vena cava. The diagnosis of SVASD poses multiple challenges from the variety of symptoms to the selection of appropriate imaging and the complexity of surgical treatment.

Background: Right atrium (RA) enlargement in uncorrected atrial septal defect (ASD) is due to chronic volume overload. Several electrocardiogram (ECG) criteria had been proposed for screening RA enlargement. This study aimed to compare the accuracy of ECG criteria in detecting RA enlargement in adults with uncorrected ASD. Methods: This was a cross-sectional study involving 120 adults with uncorrected secundum ASD. The subjects underwent ECG examination, transthoracic echocardiography, and right heart catheterization. An RA enlargement was determined with RA volume index by transthoracic echocardiography. Various ECG and combined ECG criteria were evaluated. Statistical analysis was performed to analyze the sensitivity, specificity, accuracy, positive predictive value (PPV), and negative predictive value (NPV). Results: An RA enlargement was detected in 64.2% subjects. The P wave height > 2.5 mm in lead II criterion had the best specificity (100%) and PPV (100%), but low sensitivity (19%) and accuracy (48%). The combined 2 ECG criteria (QRS axis > 90°, R/S ratio > 1 in V1) had 82% sensitivity, 56% specificity, 73% accuracy, 77% PPV, and 63% NPV. The combined 3 ECG criteria (QRS axis > 90°, R/S ratio > 1 in V1, and P wave height > 1.5 mm in V2) had 35% sensitivity, 86% specificity, 53% accuracy, 82% PPV, and 43% NPV. Conclusions: The combined 2 ECG criteria (QRS axis > 90° and R/S ratio > 1 in V1) had increased sensitivity, better accuracy, and more balance of PPV and NPV as compared with P wave > 2.5 mm in II criterion and combined 3 ECG criteria to diagnose RA enlargement in adults with uncorrected ASD.

Background Patients with pulmonary hypertension caused by chronic lung disease (Group 3 PH ) have disproportionate right ventricle ( RV ) dysfunction, but the correlates and clinical implications of RV dysfunction in Group 3 PH are not well defined. Methods and Results We performed a cohort study of 147 Group 3 PH patients evaluated at the University of Minnesota. RV systolic function was quantified using right ventricular fractional area change ( RVFAC ) and + dP /dt max /instantaneous pressure. Tau and RV diastolic stiffness characterized RV diastolic function. Multivariate linear regression was used to define correlates of RVFAC . Kaplan‐Meier and Cox proportional hazards analyses were used to examine freedom from heart failure hospitalization and death. Positive correlates of RVFAC on univariate analysis were pulmonary arterial compliance, cardiac index, and left ventricular diastolic dimension. Conversely, male sex, N‐terminal pro‐brain natriuretic peptide, heart rate, right atrial enlargement, mean pulmonary arterial pressure, and pulmonary vascular resistance were negative correlates. Male sex was the strongest predictor of lower RVFAC , after adjusting for pulmonary vascular resistance and pulmonary arterial compliance. When comparing sexes, males had lower RVFAC (26% versus 31%, P =0.03) both overall and for any given mean pulmonary arterial pressure and pulmonary vascular resistance value. Males exhibited a reduction in + dP /dt max /instantaneous pressure as pulmonary vascular resistance increased, whereas females did not. There were no sex differences in RV diastolic function. RV dysfunction ( RVFAC CI : 1.04–3.10, P =0.035). Conclusions Male sex is associated with RV dysfunction in Group 3 PH , even after adjusting for RV afterload. RV dysfunction ( RVFAC PH patients at risk for poor outcomes.

BACKGROUND The underlying molecular aetiology of congenital heart defects is largely unknown. The aim of this study was to explore the genetic basis of non-syndromic severe congenital valve malformations in two unrelated families. METHODS Whole-exome analysis was used to identify the mutations in five patients who suffered from severe valvular malformations involving the pulmonic, tricuspid and mitral valves. The significance of the findings was assessed by studying sporulation of yeast carrying a homologous Phospholipase D (PLD1) mutation, in situ hybridisation in chick embryo and echocardiography and histological examination of hearts of PLD1 knockout mice. RESULTS Three mutations, p.His442Pro, p.Thr495fs32* and c.2882+2T>C, were identified in the PLD1 gene. The mutations affected highly conserved sites in the PLD1 protein and the p.His442Pro mutation produced a strong loss of function phenotype in yeast homologous mutant strain. Here we show that in chick embryos PLD1 expression is confined to the forming heart (E2-E8) and homogeneously expressed all over the heart during days E2-E3. Thereafter its expression decreases, remaining only adjacent to the atrioventricular valves and the right ventricular outflow tract. This pattern of expression follows the known dynamic patterning of apoptosis in the developing heart, consistent with the known role of PLD1 in the promotion of apoptosis. In hearts of PLD1 knockout mice, we detected marked tricuspid regurgitation, right atrial enlargement, and increased flow velocity, narrowing and thickened leaflets of the pulmonic valve. CONCLUSIONS The findings support a role for PLD1 in normal heart valvulogenesis.

Aorta-right atrial tunnel is a vascular anomaly that originates from the aortic sinus and terminates in either the superior vena cava or the right atrium. The patency of the tunnel can result in volume overload in both ventricles, bacterial endocarditis, aneurysm formation, and spontaneous rupture. Transesophageal echocardiography was performed in a 42-year-old male patient diagnosed with infectious endocarditis, and vegetation of the mitral and aortic valves, right atrial enlargement, and an extracardiac blood vessel connecting the aorta to the right atrium were discovered. Therefore, we were able to diagnose an aorta-right atrial tunnel leading to infectious endocarditis and proceeded with surgical treatment. Together with a review of the literature, we present a case report of a patient with aorta-right atrial tunnel accompanied by infectious endocarditis. (Korean J Med 2016;90:140-143)

We present an extraordinary case of congenital enlargement of the right atrium diagnosed at 31 weeks of gestation. This case emphasizes the fact that timing of the detection of this particular cardiac malformation is of capital importance to tract the optimal treatment strategy in order to monitor further progression (in this case accompanying thrombosis) and prevent complications.

Löffler endocarditis with hypereosinophilic syndrome is rare but can cause critical ventricular obliteration by endomyocardial fibrosis. A 52-year-old woman experienced severe right heart failure with extreme shrinkage of her right ventricle, severe tricuspid regurgitation and marked right atrial enlargement. Preoperative tests showed identical pressures in the right atrium and pulmonary artery. Endocardial stripping was done, and to enlarge the right ventricle, we relocated the anterior and posterior tricuspid leaflets cephalad, up the right atrium wall, to ‘ventricularize’ a portion of the right atrium, with autologous pericardial augmentation of the tricuspid leaflets. An annuloplasty ring was added to reinforce the relocated tricuspid attachment. Right heart pressures normalized postoperatively. The patient recovered uneventfully. She has received corticosteroid therapy continuously and has shown no recurrence of heart failure in the 5 years since surgery.

No abstract available. Article truncated after first 150 words. A 97-year-old woman was repeatedly admitted for dyspnea, hypoxemia and treated with antibiotics for presumed left lower lobe pneumonia. She has a past medical history of atrial fibrillation, congestive heart failure and sick sinus syndrome with placement of a cardiac pacemaker. Notably on physical examination, she had heart rate of 110 beats/minute, temperature of 98.8°F, blood pressure of 122/72 mm Hg, and a respiratory rate of 27 breaths/minute. She had a sternal heave, a grade 4/6 "blowing" holosystolic murmur, a loud S2, jugular venous distension and an enlarged liver. Chest x-ray showed obscuration of the left lower lobe - the left heart border cannot be seen, and the L mainstem bronchus is straightened and lifted superiorly (Figure 1). An image of the heart is shown from a CT abdomen obtained 6 months previously, showing cardiomegaly, particularly massive atrial enlargement (Figure 2). An ultrasound showed bilateral atrial enlargement with moderate mitral …

Background: Total anomalous pulmonary venous return (TAPVR) is an uncommon congenital cardiovascular anomaly with poor natural prognosis without proper intervention. It has been detected more frequently in recent year due to the advent of echocardiography. The aim of this study is to evaluate the clinical manifestations, age at diagnosis and short term outcomes in TAPVR patients. Methods: From 1st January 2013 to 31st December 2013, a total of 34 cases with TAPVR were admitted in pediatric cardiac centre at Dhaka Shishu Hospital, Dhaka, Bangladesh. All of them were evaluated with 2-dimensional (2-D) and color Doppler echocardiography examination. CXR and ECG were also done. Patient’s sex, age at diagnosis, types of TAPVR, clinical manifestations, radiological finding, ECG findings and outcomes were compiled and analyzed. Results: In 34 patients with TAPVR, 23 (67.6%) were male and 11 (32.4%) were female with male to female ratio of 2.09:1. Most of the patients were diagnosed between 0-6 months of age that is 13 (38.2%) cases were in 0-2 month’s age group, 14 (41.2%) cases were in more than 2 month’s to 6 month’s age group. Tachypnea and cyanosis were more common symptoms. The types of TAPVR was supra-cardiac 18 (52.9%), cardiac 11 (32.4%), infra-cardiac 3 (8.8%) and mixed in 2 (5.9%) cases. Pulmonary hypertension was present in 31 (91.2%) of 34 cases. Among them, 20 (58.8%) patients had severe pulmonary hypertension. The most common associated intra-cardiac lesions of TAPVR patients were ASD 13 (38.2%) and PFO 13 (38.2%). ECG findings of TAPVR, 18 (52.9%) patient had right axis deviation (RAD), right ventricular hypertrophy (RVH) and 14 (41.2%) had right axis deviation (RAD), right ventricular hypertrophy (RVH), right atrial enlargement (RAE). X-ray findings of TAPVR patients, 32 (94.1%) patients had Cardiomegaly and increased pulmonary vascularity. Among admitted patient, 3 (8.8%) patients died due to pneumonia and intractable heart failure, 31 (91.2%) patients referred to advanced cardiac centre for operative treatment. Conclusions: Tachypnea and Cyanosis were an obvious clinical symptom of TAPVR. 2-D and color Doppler echocardiography can provide quick and accurate diagnostic information of TAPVR. Death rate is high in TAPVR patient in spite of adequate medical management. So, early detection and definitive surgical treatment of TAPVR is much needed. Bangladesh Journal of Medical Science Vol.14(3) 2015 p.270-273

Objective:Heart chymase rather than angiotensin converting enzyme has higher specificity for angiotensin (Ang) I conversion into Ang II in humans. A new pathway for direct cardiac Ang II generation has been revealed through the demonstration that Ang-(1-12) is cleaved by chymase to generate Ang II directly. We address here whether Ang-(1-12) and chymase gene expression and activity are detected in the atrial appendages of 44 patients (10 females) undergoing heart surgery for the correction of valvular heart disease, resistant atrial fibrillation or ischemic heart disease.Methods and results:Immunoreactive Ang-(1-12) expression was 54% higher in left atrial compared with right atrial appendages. This was associated with higher abundance of left atrial appendage chymase gene transcripts and chymase activity, but no differences in angiotensinogen mRNA. Atrial chymase enzymatic activity was highly correlated with left atrial but not right atrial enlargement as determined by echocardiography, while both tyrosi...

Background Right atrial area (RAA) is a prognostic factor in human patients with pulmonary arterial hypertension (PAH). Reference intervals for RAA have been described in healthy dogs. Objectives To evaluate RAA indexed to the body surface area in dogs with PAH as an indicator of right atrial size, PAH severity and right‐sided congestive heart failure (R‐CHF). Animals A total of 119 client‐owned dogs, 48 dogs with PAH and 71 control dogs. Methods Prospective observational study. Pulmonary arterial hypertension was classified according to the tricuspid regurgitation pressure gradient (TRPG) as mild (36–50 mmHg), moderate (51–75 mmHg), or severe (>75 mmHg). The RAA index was calculated as the RAA divided by body surface area. Results The RAA index was higher in dogs with moderate PAH (13.3 cm2/m2; range, 3.4–24.7 cm2/m2) and severe PAH (12.1 cm2/m2; range, 5.4–21.8 cm2/m2) than in those with mild PAH (6.7 cm2/m2; range, 4.8–10.7 cm2/m2) or in controls (7.3 cm2/m2; range, 4.2–10.2 cm2/m2; P 12.3 cm2/m2 (sensitivity, 100%; specificity, 89.5%). In dogs with PAH, severity of tricuspid regurgitation (TR) was the only independent predictor of RAA index based on multivariate analysis (P

BackgroundIn 2010, an Italian project was launched aimed at using a telecardiology device in order to perform early diagnosis of young students at risk of sudden cardiac death.MethodsOur retrospective observational study was conducted on a population of 13,016 students, aged between 16 and 19 years, in different Italian regions. It consisted of analysis of data recorded during a telecardiology pilot study. The recorded data were electrocardiograms and data concerning lifestyle habits and family history of cardiovascular diseases. In total, 14 alterations in the electrocardiogram signal have been considered in this study. Some of these alterations are as follows: ventricular ectopic beats, atrioventricular block, Brugada-like electrocardiogram pattern, left anterior/posterior fascicular block, left/right ventricular hypertrophy, long/short QT interval, left atrial enlargement, right atrial enlargement, short PQ interval, and ventricular pre-excitation Wolff–Parkinson–White syndrome. On the basis of the collected data, we implemented this retrospective observational study.ResultsThe analysed data showed that 13.60% of students had a family history for cardiovascular diseases, 22.43% reported smoking habits, 26.23% reported alcohol consumption, and 7.24% reported abuse of drugs. A total of 24% of students had at least one of the 14 electrocardiogram pathological alterations considered in our study and 32% had electrocardiogram values within the normal range.ConclusionsThis retrospective observational study analysed data registered during our telecardiology activity. This activity permitted to maximise data collection and minimise the costs for collecting such data. This activity of screening is being continued and in the next few years it will allow us to have a greater mass of data.

BACKGROUND: Echocardiography is the easiest way to evaluate the functions of parts of the heart like ventricles and valves. Some of the studies have shown normal echocardiography in Chronic Obstructive Pulmonary Disease (COPD) but others have shown pulmonary hypertension and cor pulmonale in most of the studies both of which are associated with decreased survival. Our study is also an effort to look for the heart changes in COPD patients and to correlate them with the duration and severity of the disease. MATERIAL AND METHODS: It was a cross-sectional study done in the Medicine department of Chirayu Medical College and Hospital, Bhopal during the period from January 2012 to June 2012. 60 cases of COPD were selected for study. Almost all the cases have varying degrees of overlapping picture of chronic bronchitis and emphysema taken as single entity of COPD. Echocardiography was done in all the 60 patients and looked for Right Atrial Enlargement (RAE), Right Ventricular Dilatation (RVD), Tricuspid Regurgitation (TR) and its severity, Right ventricular systolic pressure, and Pulmonary Arterial Hypertension (PAH). RESULTS: On Echocardiography, patients with moderate and severe grade COPD show RAE and RVD in more than 50%, whereas, patients with mild grade COPD showed RAE in about 40% and RVD in about 60%. In very severe grade COPD patient having TR also had severe grade PHT whereas mild grade COPD patients usually have mild grade PHT. CONCLUSION: Physicians and scientists will need to be encouraged to conduct research that will help develop newer, easier and better diagnostic methods and develop newer drugs and inhalation devices, that will not only help further improve symptoms and quality of life, but will also halt, and if possible, revert the progression of the disease.

INTRODUCTION: Chronic obstructive pulmonary disease (COPD) is a major cause of morbidity and mortality in India especially in rural areas. COPD is the 5th leading cause of death worldwide. COPD causes hypoxia in lungs triggering the pulmonary vasoconstriction which leads to increase in pulmonary vascular resistance finally involving the heart and causes right sided cardiac complications which can be evidenced by Electrocardiography (ECG). Therefore, the present study was conducted to evaluate the diagnostic values of ECG changes among COPD patients. MATERIAL AND METHODS: This was a cross-sectional study done in the Medicine department of Chirayu Medical College and Hospital, Bhopal during the period from January 2012 to June 2012. 60 cases of COPD were selected for study. The diagnosis of COPD is based upon the clinical history, clinical examination, X-ray chest and Pulmonary Function Test (PFT). ECG was done in all the 60 patients and looked for heart axis, Right Ventricular Enlargement (RVE), Right Atrial Enlargement (RAE) and Right Bundle Branch Block (RBBB). RESULTS: ECG analysis shows the features suggestive of RAE in >40% of COPD patients having duration of >10 yrs. Out of total 60 patients 24 patients had Forced Expiratory Volume First(FEV1) ≥80% (mild COPD), 30 had FEV1 50 % to 79% (moderate COPD), 5 patients had FEV1 30-49% (severe COPD) and 1 patient had FEV1 < 30% (very severe COPD). When the disease severity is compared with the Manifestations in ECG RAE, RVE and RBBB was found in 3 (60%) of the patients out of 5 patients who had severe disease. CONCLUSION: The prevalence of ECG abnormalities related to cardiac diseases, in general is higher in those with more severe pulmonary obstruction and ECG findings should be taken into consideration when diagnosing these patients of COPD for delivering a more integrated pulmonary and cardiovascular care.

A 30-year-old man with a known history of heart failure with preserved ejection fraction and paroxysmal atrial fibrillation diagnosed at 24 years of age was referred to our service due to progressive heart failure. His history was unremarkable for drug use, travel, occupational exposure, and lung disease and was negative for familial cardiomyopathy. His vitals were 108/72 mm Hg, heart rate 60 beats per minute; jugular venous pressure was elevated with a prominent y-descent and positive hepatojugular reflux, and he had mild peripheral edema. Biochemical analysis revealed plasma brain natriuretic peptide=975 pg/mL, estimated glomerular filtration rate=121 mL/min per 1.73 m2 and hemoglobin=134 g/L. Comparison of previous 12-lead ECGs over the past 3 years revealed progressive right atrial enlargement culminating into atrial fibrillation with his previous ECG showing low voltage QRS complexes associated with incomplete right bundle branch block and right axis deviation (Figure 1A). We optimized his medical therapy which included furosemide, spironolactone, bisoprolol, enalapril, and warfarin. Figure 1. ECG shows normal sinus rhythm, right atrium (RA) enlargement, low voltage QRS complex, right axis deviation, and incomplete right bundle branch block ( Ai ). Twelve-lead ECG of a same patient 3 years later showing progression into atrial fibrillation ( Aii ). Transthoracic echocardiogram showing severe dilation of the left atrium and RA and normal-sized ventricles based on the 4-chamber view with a “D”-shaped septum representative of right ventricular (RV) pressure and volume-overload ( Bi ). There was restrictive left ventricle (LV) filling based on transmitral Doppler filling with the deceleration time=100 ms and E-wave amplitude=135 cm/s ( Bii ). Right atrial hemodynamics showed v-wave only with a prominent y-descent ( Ci ), whereas the left- and right-sided catheterization showed elevated LV and RV filling pressures with a clear separation and a dip and plateau morphology ( Cii ). A thorough series of investigations, including transthoracic echocardiogram, coronary angiogram, invasive hemodynamic cardiac catheterization, endomyocardial …

Functional tricuspid regurgitation (FTR) is typically due to tricuspid annulus (TA) dilation and right ventricular or right atrial enlargement and dysfunction. FTR is frequently observed in patient...

PURPOSE: To assess the role of long-axis (LA) and short-axis (SA) measurements of the right atrium (RA) and right ventricle (RV) at non–electrocardiographically (ECG) gated thoracic CT angiography for identification of RA enlargement and RV enlargement. MATERIALS AND METHODS: This study was a retrospective case review of 138 patients who underwent both non–ECG-gated CT angiography and ECG-gated CT angiography concurrently from November 2016 through November 2018. The SA and LA of the RA and RV were measured by two observers blinded to the ECG-gated CT angiography data. ECG-gated CT angiography–derived RA end-systolic and RV end-diastolic volumes were used as standard of reference to derive cutoff values for diagnosis of RA and RV enlargement. RESULTS: In this study, 138 patients were evaluated (70 men, 68 women; mean age, 70.0 years ± 18.4 [standard deviation]; mean body mass index, 29.3 kg/m(2) ± 8.1). Of these patients, ECG-gated CT angiography revealed 36.2% had RA enhancement and 19.0% had RV enhancement. The best predictor of RA enhancement was the product of atrial LA and SA measurements, for which a threshold value of 3210 mm(2) yielded a 94% sensitivity and 81.8% specificity (area under the curve [AUC], 0.92). A threshold of 55.5 mm for LA diameter had 86% sensitivity and 78.4% specificity in identifying RA enlargement. RV enlargement could be predicted if the SA diameter was greater than 48.5 mm (76.9% sensitivity and 64.9% specificity) and with a body surface area indexed value of 27.0 mm/m(2) (92.3% sensitivity and 74.8% specificity [AUC, 0.87]). CONCLUSION: RA and RV enlargement can be accurately diagnosed by using non–ECG-gated CT angiography. © RSNA, 2019 Supplemental material is available for this article.

Background— Functional tricuspid regurgitation (TR) with a structurally normal tricuspid valve (TV) may occur secondary to chronic atrial fibrillation (AF). However, the clinical and echocardiographic differences according to functional TR subtypes are unclear. Therefore, characterization of functional TR because of chronic AF (AF-TR) remains undetermined. Methods and Results— To investigate the prevalence of AF-TR, 437 patients with moderate to severe TR underwent 3-dimensional (3D) transesophageal echocardiography. TR severity was determined by the averaged vena contracta width on apical and parasternal inflow views. The prevalence of AF-TR was 9.2%, whereas that of functional TR because of left-sided heart disease was 45.3%. Clinical features of AF-TR included advanced age, female sex, greater right atrial than left atrial enlargement and lower systolic pulmonary artery pressure compared with left-sided heart disease-TR with sinus rhythm (all P P P 2 ; P P Conclusions— AF-TR is not rare and is associated with advanced age and right atrial enlargement. TV deformations and their association with right heart remodeling differ between AF-TR and left-sided heart disease-TR. Our results suggest that in patients with TR secondary to AF, TV annuloplasty should be effective because this entity has annular dilatation without leaflet deformation.

Aims Prevalence and clinical significance of right atrial enlargement (RAE) has been poorly characterized in hypertrophic cardiomyopathy.Methods One hundred and sixty consecutive patients with hypertrophic cardiomyopathy (35.5 +/- 20 years; 64% men) were studied. They underwent clinical examination, standard ECG, M-mode, 2D and Doppler echocardiography, stress test and ECG Holter monitoring. Major adverse cardiac events were considered: cardiac death (sudden death, heart failure death); cardiac transplant; resuscitated cardiac arrest or appropriate implantable cardioverter defibrillator discharge. Genetic analysis of eight sarcomeric genes was performed using Sanger sequencing.Results RAE was observed in 22 patients (14%), associated with left atrial enlargement in all cases. Patients with RAE were likely to have restrictive mitral pattern (P < 0.001) and had higher New York Heart Association (P < 0.001), N-terminal prohormone of brain natriuretic peptide (P< 0.001), left atrial volume index (P < 0.001), lateral (P=0.04) and septal (P=0.002) E/e', systolic pulmonary artery pressure (P< 0.001) and lower ejection fraction (all P < 0.001). On cardiopulmonary exercise testing, peak VO2 was lower and VE/VCO2 higher in patients with RAE (P < 0.001). During a mean follow-up of 4 +/- 2.1 years, 30 major adverse cardiac events in 24 patients (15%) were observed. Cox proportional hazards regression analysis identified RAE as an independent predictor of major adverse cardiac events (odds ratio=2.6; confidence interval 1.5-4.6; P=0.001). In patients with RAE who were genetically tested, there was a higher prevalence of sarcomeric genemutations (68%), doublemutations (16%) and troponin T mutations (21%).Conclusion RAE is present in a small subset of patients with hypertrophic cardiomyopathy, and largely reflects increased pulmonary pressures because of severe diastolic and/or systolic left ventricular dysfunction. Patients with RAE had a higher prevalence of sarcomeric gene mutations, troponin T mutations and complex genotypes. In conclusion, RAE may serve as a very useful marker of disease progression and adverse outcome in patients with sarcomeric hypertrophic cardiomyopathy.

Introduction: Atrial myxomas are rare cardiac tumours. This case report illustrates a case of myxoma in the right atrium, which is a rare location for this condition. Case Presentation: A 55 year old Myanmar (Burmese) man had two syncopes within one week. Cardiac evaluation showed changing cardiac murmur. Right atrial enlargement was obvious on electrocardiogram. Transthoracic echocardiogram revealed an echogenic mass in the right atrium which prolapsed through tricuspid valve. The myxoma was successfully excised, the base of which was attached to the interatrial septum at the lower margin of fossa ovalis. Conclusion: In this case report, we emphasize the uncommon site of myxomas in the right atrium. Clinical suspicion will lead to early diagnosis by echocardiogram and surgical excision is the curative treatment.

Background— Pulmonary vascular disease associated with left-side heart failure and preserved ejection fraction (PH-HFpEF) is an increasingly common cause of pulmonary hypertension. The distinction between PH-HFpEF and pulmonary arterial hypertension (PAH) is important because therapies indicated for PAH can be detrimental in HFpEF. The characteristic features of PH-HFpEF are understudied. Methods and Results— In a cross-sectional study, we compared the clinical, echocardiographic, and hemodynamic features of PH-HFpEF (n=100), with PAH (n=522), and HFpEF without pulmonary vascular disease (n=45). We determined the clinical characteristics that best differentiated PH-HFpEF from PAH. Compared with patients with PAH, patients with PH-HFpEF were older; had a higher prevalence of cardiovascular comorbidities; had worse exercise capacity and renal function; more frequently had left atrial enlargement; and less frequently had right atrial enlargement. PH was less severe in PH-HFpEF patients than in PAH patients (pulmonary vascular resistance 4.8 [interquartile range 3 to 8.4] versus 10.9 [interquartile range 7.4 to 15.7] Wood units; P Conclusions— These data should help better identify PH-HFpEF, an entity that has become increasingly recognized and difficult to treat.

Pulmonary hypertension (PH) is defined as an increased mean pulmonary artery pressure (P̄(pa)) >25 mmHg at rest as assessed by right heart catheterisation (RHC). However, this technique is invasive and noninvasive alternatives are desirable for early diagnosis of PH. Although estimation of systolic pulmonary arterial pressure is easily obtained using Doppler echocardiography, cases of under- and over-estimations are not rare and direct measurement of P̄(pa) is not possible using this method. Therefore, echocardiography should be considered as a tool for assessment of the likelihood rather than the definite presence or absence of PH. Transthoracic echocardiography may be useful for noninvasive screening of patients at risk of PH. On the basis of an echocardiographic assessment, patients showing signs suggestive of PH can be referred for a confirmatory RHC. A number of variables measured during echocardiography reflect the morphological and functional consequences of PH and have prognostic value. The presence of pericardial effusion, reduced tricuspid annular plane excursion and right atrial enlargement are associated with a poorer prognosis. Echocardiography is also an important procedure for monitoring the response of patients to therapy, and is recommended 3−4 months after initiation of, or a change in, therapy. Echocardiographic assessment as part of a goal-oriented approach to therapy is essential for the effective management of PH patients.

Pulmonary hypertension, which may lead to right ventricular (RV) failure, increases with left ventricular (LV) diastolic dysfunction severity. The prevalence and determinants of RV failure were analyzed in 120 patients admitted with acute left heart (LH) failure. Patients were divided into RV failure (n=50) and non-RV failure (n=70) groups. The prevalence of RV failure was found to be 42%. In both groups, two thirds of the patients had isolated LV diastolic dysfunction and the rest had combined LV systolic and diastolic dysfunction. Patients in the RV failure group were characterized by higher LV diastolic grade (2.2 ± 0.6 vs 1.84 ± 0.7; P=.0070), pulmonary artery systolic pressure (PASP; 57.8 ± 15.3 vs 50.14 ± 12.1 mm Hg; P=.0028), right atrial enlargement (92% vs 25.7%; P=.000001), and more-than-moderate tricuspid regurgitation (58% vs 27.1%; P=.0006). RV failure is a frequent finding in patients with advanced LH failure. It is strongly associated with the severity of LV diastolic dysfunction and the severity of PASP.

Objectives Pulmonary arterial hypertension (PAH) is a rare disorder with unfavorable prognosis despite implementation of specific PAH-oriented therapy. The aim of the study was to define predictors of poor prognosis in patients from one center treated according to the Polish National Health Fund program. Patients and methods Forty-seven consecutive patients (30 women; aged 39±17 years) with PAH diagnosis were enrolled to the study. Clinical assessment, laboratory measurements, electrocardiogram, echocardiography, 6-minute walk test, 24-hour Holter monitoring, cardiopulmonary exercise test and microvolt T-wave alternans test were performed during routine visits. Eight patients died during 2.6±1.7 years follow-up. Results Parametrs which differentiated patients who died were brain natriuretic peptide (BNP) concentration ≥330 pg/mL (sensitivity 88%, specificity 92%, area under the ROC curve [AUC] 0.92); bilirubin concentration ≥1.2 mg/dL (sensitivity 88%, specificity 81%, AUC 0.85); right atrial area ≥21 cm2 (sensitivity 86%, specificity 69%, AUC 0.84), right ventricular (RV) dimension in the apical 4-chamber view ≥47 mm (sensitivity 86%, specificity 86%, AUC 0.85) and RV to left ventricular diastolic diameter ratio ≥1.5 (sensitivity 83%, specificity 84%; AUC 0.85). In multivariate analysis, independent predictors of mortality were higher BNP (p = 0.04) and bilirubin level (p = 0.03), higher right atrial area (p = 0.02) and lower tricuspid annular plane systolic excursion (p = 0.03). Conclusions In PAH patients treated with specific PAH-oriented therapy right atrial enlargement, impaired right ventricular systolic function, as well as increased BNP and bilirubin concentration was associated with an increased mortality risk.

A 16-year-old boy with a double-outlet right ventricle, D-malposed great vessels, and a subpulmonary ventricular septal defect status-post surgical ventricular septal defect patch closure and arterial switch procedure at 2 months of age, reported progressive exercise intolerance. He was found to have moderate right atrial enlargement, mild dilation of right and left ventricles, and a persistent residual left ventricle to right atrium (LV-RA) intracardiac shunt on echocardiographic assessment (similar physiology to a Gerbode-type defect; Figure A and Movie I in the online-only Data Supplement). Cardiac MRI delineated the LV-RA shunt (steady-state free precession cine; Figure B and Movie I in the online-only Data Supplement), with an estimated Qp:Qs of 1.4:1 (velocity-encoded MRI). Cardiac MRI–derived left-ventricular end-diastolic volume was 132 mL/m2 ( z score …

Background— Severe tricuspid valve (TV) dysfunction may lead to surgical TV replacement with a biological valve prosthesis in patients with congenital heart disease. To expand the lifetime of this valve and reduce the number of surgeries, percutaneous TV implantation (PTVI) may be an effective alternative to repeated surgery. We report on our 2-center experience with PTVI. Methods and Results— Between 2008 and 2014, 17 percutaneous valves were implanted in 16 patients with TV bioprosthesis dysfunction (9 females) from 2 centers. Median age and weight were 31.3 years (5–77.2) and 65.2 kg (17.7–107); 14 patients had congenital heart disease (univentricular heart with a right atrial to right ventricle bioprosthesis in 3, Ebstein’s anomaly of the TV in 5, and other in 6), and 2 had acquired TV dysfunction. All procedures were successful (Melody n=7, Sapien 26 mm valve n=4, Sapien XT 29 mm valve n=6). One valve showed early dysfunction. It was replaced surgically and shortly after that a repeated PTVI was performed. The median duration of follow-up was 2.1 years (3 days to 6.3 years). The percutaneous valve was performing well in 15 of 16 patients. Conclusions— PTVI was safe and effectively improved TV function in all but 1 patient at midterm follow-up. We think that PTVI is a good alternative to repeated surgical TV replacements and that it may reduce the total number of open heart surgeries in these patients.

Information about a real patient is presented in stages (boldface type) to an expert clinician (Dr Ammash), who responds to the information, sharing his reasoning with the reader (regular type). A discussion by the authors follows. A 21-year-old man with a history of Ebstein anomaly and atrial fibrillation who had previously undergone tricuspid valve repair, pulmonary venous isolation, and the Cox maze III procedure was referred from an outside institution for the evaluation of an acute onset of left-sided upper- and lower-extremity weakness. The patient had initially presented 3 years earlier with abdominal pain and symptomatic palpitations. Physical examination at that time revealed a jugular venous pressure of 12 cm without a prominent V wave. Cardiac palpation noted a sternal heave. On auscultation, a grade II holosystolic murmur was appreciated at the left sternal border that increased with inspiration. Abdominal inspection noted an enlarged liver that was not pulsatile. A standard 12-lead ECG demonstrated sinus rhythm with first-degree atrioventricular block(Figure 1), and a chest x-ray noted enlargement of the right atrium(Figure 2). A 24-hour Holter monitor noted that the predominant rhythm was atrial fibrillation interrupted by brief periods of sinus rhythm. Transthoracic echocardiography demonstrated severe right atrial enlargement, dilatation of the tricuspid annulus, apical displacement of the tricuspid valve (displacement index, 10.2 mm/m2), large anterior tricuspid valve leaflet, and severe tricuspid regurgitation consistent with Ebstein anomaly. Figure 1. Resting 12-lead ECG demonstrating sinus rhythm with first-degree atrioventricular block and nonspecific T-wave abnormalities. Figure 2. Patient chest x-ray demonstrated enlargement of the cardiac silhouette, particularly the right atrium (arrow). Dr Ammash: Ebstein anomaly is a rare disorder accounting for

A 12-year-old boy presented to our outpatient department with progressive dyspnoea and palpitations of 6-month duration. Cardiovascular system examination revealed a short ejection systolic murmur at the left lateral heart border. Chest X-ray showed gross cardiomegaly. Two-dimensional ECHO revealed massive aneurysmal right atrial (RA) enlargement, low-pressure tricuspid regurgitation and dysplastic tricuspid valve with compression of the left atrium (LA) and left ventricle (LV). Cardiac CT showed grossly enlarged RA compressing the LA, LV, right pulmonary artery and right upper lobe pulmonary vein. Initially, the patient refused surgery. On follow-up, a thrombus was seen in the giant RA and the patient agreed to and underwent successful surgery. Absence of pericardium was specifically excluded on cardiac MRI and on surgery. Resected atrial tissue showed wall thinning and focal fibrosis establishing idiopathic dilation of the RA. The patient is on regular follow-up.

Objectives The purpose of this study was to investigate the predictor of non-pulmonary vein (PV) ectopic beats initiating paroxysmal atrial fibrillation (PAF). Background Non-PV ectopic beats can initiate PAF in some patients and play an important role in the recurrence of PAF after PV isolation. Information on the predictors of non-PV ectopic beats initiating PAF is unknown. Methods This study included 293 patients (215 men and 78 women, age 60 ± 14 years) with clinically documented drug-refractory PAF. Of the 94 patients with non-PV ectopic beats initiating PAF, 38 (40%) patients had superior vena cava (SVC) ectopic beats and 32 (34%) had left atrial posterior free wall (LAPFW) ectopic beats. Results In a univariate analysis, only female gender was related to the presence of non-PV (p = 0.016) and SVC ectopic beats (p = 0.012). Right atrial enlargement (p = 0.005) and left atrial enlargement (p Conclusions The location of non-PV ectopic beats initiating PAF can be predicted by both gender and left atrial enlargement.

Objective Most of the data about the prevalence of pulmonary arterial hypertension (PAH) are from tertiary centers that are biased toward seeing more severe cases; therefore, the true prevalence of PAH among patients with connective tissue disease is unknown. We sought to determine the point prevalence of undiagnosed PAH in community-based rheumatology practices. Methods The study design was a multicenter, prospective and retrospective survey and analysis of clinical cases in 50 community rheumatology practices. We evaluated a total of 909 patients with either scleroderma (systemic sclerosis [SSc]) or mixed connective tissue disease (MCTD). If a subject had not been diagnosed as having PAH, then a new Doppler echocardiogram was obtained to measure cardiac parameters, including estimated right ventricular systolic pressure (ERVSP), and a full review of medical records was done. Results Of 909 screened patients, 791 were evaluable and completed the study; 669 had not previously been studied for PAH. Of these 669 patients, 89 (13.3%) were found by Doppler echocardiography to have an ERVSP of ≥40 mm Hg. Of these 89 patients, 82 (92.1%) had SSc and 7 (7.9%) had MCTD. The total prevalence of PAH in the survey was 26.7% (211 of 791 patients, including 122 with known PAH and 89 newly diagnosed as having PAH). Doppler echocardiographic data showed 20 of 89 patients (22.5%) with ERVSP of ≥50 mm Hg, 20 of 89 patients (22.5%) with increased RV dimension, and 25 of 89 patients (28.1%) with right atrial enlargement. Patients with ERVSP ≥40 mm Hg had decreased exercise tolerance compared with those with ERVSP

Background There is a common assumption that a normal ECG or a normal heart size on chest X-ray virtually rules out a diagnosis of heart failure. Aims To assess the value of the electrocardiogram and chest X-ray in identifying patients with chronic heart failure in the community. Methods and results This study was a secondary analysis of data prospectively collected at the time of patient's enrolment in the EPICA study, an epidemiological study of the prevalence of heart failure in Portugal. A total of 6300 subjects were clinically evaluated. Patients who presented with symptoms or signs of heart failure, and/or were receiving diuretics for chronic heart failure (CHF) had a chest X-ray, ECG, and echocardiogram. The diagnosis of heart failure was confirmed in 551 cases. Patients with right atrial enlargement, atrial flutter, atrial fibrillation, 2nd degree-Mobitz I atrioventricular block, 1st degree atrioventricular block, left bundle branch block, lung interstitial oedema, and bilateral pleural effusion were more likely to be diagnosed with heart failure. For the diagnosis of heart failure, in the Portuguese population aged over 25 years, an abnormal electrocardiogram had an estimated sensitivity of 81%, and negative predictive value of 75%; an abnormal chest X-ray had an estimated sensitivity of 57%, and negative predictive value of 83%. Twenty five percent of patients with CHF had a normal ECG or chest X-ray. Conclusion Our results show that electrocardiographic and roentgenographic features are not sufficient to allow heart failure to be reliably predicted in the community and support the recommendation that all patients with suspected heart failure should undergo echocardiography.

Chagasic heart disease has been documented in non-human primates, but noninvasive characterization of systolic and diastolic function has not been previously reported. Seventeen seropositive (12 females; mean age, 20) and 13 age- and gender-matched seronegative baboons underwent Doppler echocardiography. Systolic function indices included left ventricular (LV) fractional shortening (FS %), velocity of circumferential fiber shortening (VCF, circ/sec), LV mass index, and left and right ventricular ejection fractions (RVEF %). Diastolic function indices included trans- mitral E-wave, A-wave, E/A ratio, E-deceleration time, and isovolumic relaxation time. Twelve-lead electrocardio- graphic (ECG) recordings were obtained. There were no significant differences between groups for body size or blood pressure. Seropositive and seronegative groups revealed diffuse non-specific T wave changes precluding differentiation; however, tall "P" waves were seen in four seropositive and two seronegative baboons. Four of the 17 (24%) seropositive baboons had decreased FS (25 ± 8% versus 40 ± 5%, P < 0.005) and VCF (1.05 ± 0.36 circ/sec versus 1.84 ± 0.23 circ/sec, P < 0.0001), prolonged isovolumic relaxation time (71 ± 16 msec versus 5 5±9m sec, P < 0.02), and reduced RVEF (44 ± 9% versus 54 ± 4%, P < 0.05), as compared with the other seropositive baboons. We conclude that chagasic heart disease is present in 24% of the naturally infected baboons in this study. ECG evidence of right atrial enlargement was more common in the seropositive animals. There were systolic and diastolic abnormalities of both ventricles. The LV systolic dysfunction may be segmental or diffuse.

Previous attempts to validate ECG criteria for right atrial (RA) enlargement (RAE) have been limited by sample sizes and lack of accepted standards for measuring RA size. New guidelines have recommended that RA volume (RAV) be used to determine RA size. Since these guidelines were released, no studies have been published that correlate RAE by ECG to RAV using the new standards. We aimed to validate previously proposed ECG criteria for RAE, commonly called P pulmonale, and to establish whether a correlation exists between P wave amplitudes and RAV as determined by echocardiogram in patients from the pulmonary hypertension (PHT) clinic. We identified patients from the PHT clinic that had an echocardiogram and ECG done at most 30days apart. We defined increased P wave amplitude as ≥2mm in lead II and ≥1mm in lead V1. The RA was determined to be enlarged if the RAV index (RAVI) was ≥39mL/m2 for men and ≥33mL/m2 for women. Patients were stratified into four groups: those with P II≥2mm, those with P V1≥1mm, those that met both criteria, and those that met neither. Right atrial volumes were then compared. Sixty-three patients were included in the study (7 men, 56 women). Three men and 36 women had an ECG that met criteria for P pulmonale. Five men and 28 women had an enlarged RA on echocardiogram. Sixty-nine percent of ECGs that met criteria for RAE were associated with increased RAV by echocardiogram. The specificity of each of the ECG criteria for P pulmonale at detecting RAE was 100% for men. The criterion with the highest specificity among women was P II≥2mm AND P V1≥1mm (94%). The least specific criterion for women was P II≥2mm (70%). The sensitivity of each criterion was much lower. The most sensitive criteria for men and women were P V1≥1mm (66.6%) and P II≥2mm (48%), respectively. The correlation of P wave amplitude in leads II and V1 and RAVI was not statistically significant for any of the ECG criteria for P pulmonale. In patients from the PHT clinic, the specificity of P pulmonale for detecting RAE is high, but the sensitivity is relatively low. These results suggest that in PHT, P pulmonale can be used to confirm that the RA is enlarged, but it is not a reliable test for diagnosing RAE.

A 17-year-old male patient presented with cyanosis, repeated squatting since childhood and haemoptysis since the past 1 month. He had central cyanosis with clubbing. Cardiovasular examination revealed ejection systolic murmur in the pulmonary area with single S2. ECG showed right ventricular hypertrophy (RVH) with right atrial enlargement and first-degree heart block. Two-dimensional echo showed ventricular septal defect, overriding aorta, RVH, right ventricular enlargement (RVE) and right atrial enlargement with infundibular and valvular pulmonary stenosis and 1.9 cm ostium secondum atrial septal defect. There was no evidence of atrioventricular canal defect. The patient was diagnosed with pentology of Fallots. Follow-up ECG showed complete heart block (CHB) that again reverted to first-degree heart block. A diagnosis of pentology of Fallot with intermittent CHB was made with an awake heart rate of 50/min. This case report shows association of CHB with tetralogy of Fallot.

In this article, we describe a double-chambered left ventricle (LV) associated with a functional right ventricular (RV) aneurysm and right atrial (RA) enlargement in an asymptomatic 24-year-old woman with a family history of sudden cardiac death. We will discuss the differential diagnosis, genetic testing and possible prognostic implications.