A Late-Detected Paraganglioma in a Young Patient with Resistant Hypertension and Severe Aortic Regurgitation-A Case Report and Review of the Literature.

BACKGROUND: Paraganglioma is a rare neuroendocrine tumor derived from chromaffin cells. The overproduction of catecholamines accounts for the presenting symptoms and cardiovascular complications. The clinical presentation frequently overlaps with the associated cardiac diseases, delaying the diagnosis. Multimodality imaging and a multidisciplinary team are essential for the correct diagnosis and adequate clinical management. CASE SUMMARY: A 37-year-old woman with a personal medical history of long-standing arterial hypertension and radiofrequency ablation for atrioventricular nodal reentry tachycardia presented with progressive exertional dyspnea and elevated blood pressure values, despite a comprehensive pharmacological treatment with six antihypertensive drugs. The echocardiography showed a bicuspid aortic valve and severe aortic regurgitation. The computed tomography angiography revealed a retroperitoneal space-occupying solid lesion, with imaging characteristics suggestive of a paraganglioma. The multidisciplinary team concluded that tumor resection should be completed first, followed by an aortic valve replacement if necessary. The postoperative histopathology examination confirmed the diagnosis of paraganglioma. After the successful resection of the tumor, the patient was asymptomatic, and the intervention for aortic valve replacement was delayed. DISCUSSION: This was a rare case of a late-detected paraganglioma in a young patient with resistant hypertension overlapping the clinical presentation and management of severe aortic regurgitation. A multimodality imaging approach including transthoracic and transesophageal echocardiography, computed tomography, and magnetic resonance imaging had an emerging role in establishing the diagnosis and in guiding patient management and follow-up. The resection of paraganglioma was essential for the optimal timing of surgical correction for severe aortic regurgitation. We further reviewed various cardiovascular complications induced by pheochromocytomas and paragangliomas.

Body size influences heart rate in children aged 6 to 18 years old.

Previous research established age-related normal limits for children's heart rates (HRs). However, children of the same age can have significantly different HRs, depending on whether they are overweight or underweight, tall or short. Studies on children HR have failed to find a clear correlation between HR and body size. The goal of our study was to create Z scores for HR based on weight (W), height (H), body mass index (BMI), and body surface area (BSA) and compare them to normal age-related HR limits. Electrocardiograms were recorded from a total of 22,460 healthy children ranging in age from 6 to 18 years old using BTL machines. A comparison was made between different age groups, in function of W, H, BMI, and BSA, based on the HR that was automatically detected by using the digitally stored electrocardiogram. Z scores were computed for each of the categories that were mentioned. Incremental Z score values between -2.5 and 2.5 were calculated to establish upper and lower limits of HR. The BSA's estimation of HR is the most accurate of the available methods and can be utilized with accuracy in clinical practice. Z scores for HR in children differ in function of the age, W, H, BMI and BSA. The best estimation is based on BSA. The novelty of our study is that we developed Z scores for HR in relation to body size, age and sex, producing a standardized, consistent, and reproducible result without requiring practitioners to learn and remember cutoff values for a wide range of variables across age groups and sexes. Z scores minimize observer and institutional bias, hence generating uniform and reproducible standards.

Low Atrial Rhythm in a Large Cohort of Children from Transylvania, Romania.

Low atrial rhythm (LAR) is an ectopic rhythm originating in the lower part of the right or left atrium. Prior observational studies attempted to quantify the prevalence of low atrial rhythm in the pediatric population, but the observed prevalence was highly variable with relatively small sample sizes. We aimed to characterize low atrial rhythm and determine its prevalence in a large population of 24,316 asymptomatic children from northwestern Transylvania. We found a prevalence of 0.6% (145 children) for low atrial rhythm. Children with LAR had a significantly lower heart rate (mean 78.6 ± 8.3 bpm), than the control sinus rhythm group (85.02 ± 4.5 bpm). Furthermore, a shorter PR interval was seen in children with LAR (132.7 ± 12.7 ms) than in the children from the control group (141.7 ± 5.4; p = 0.0001).There was no significant association between gender and the presence of left LAR (LLAR) or right LAR (RLAR) (p = 0.5876). The heart rate of children with LLAR was significantly higher (81.7 ± 11.6 bpm) than that of the children with LRAR (77.6 ± 11.1 bpm) (p = 0.037). Pediatric cardiologists should recognize low atrial rhythm and be aware that asymptomatic, healthy children can exhibit this pattern, which does not require therapeutic intervention.

Paraoxonase 1 and atrial fibrillation: Is there a relationship?

Atrial fibrillation (AF) is associated with oxidative stress and inflammation. Paraoxonase-1 (PON1), circulates in blood bound to high-density lipoproteins and reduces systemic oxidative stress. The aim of this study was to evaluate PON1 serum concentration and PON1 arylesterase activity (AREase) in patients with AF. We studied a group of 67 patients with symptomatic paroxysmal or persistent AF admitted for cardioversion and a control group of 59 patients without AF. Clinical parameters, lipid profile, PON1 concentration and AREase were evaluated. A significant difference in serum PON1 concentration and in AREase was found among the two groups. In a multivariate linear regression model, the presence of AF was associated with low PON1 concentration (P = .022). The body mass index was also independently associated with PON1 values (P < .001). Only the high-density lipoproteins-cholesterol level was independently associated with AREase (P = .002). PON1 serum concentrations and AREase were diminished in patients with AF, and the presence of AF was independently associated with low PON1 values.

Formula to estimate left atrial volume using antero-posterior diameter in patients with catheter ablation of atrial fibrillation.

ABSTRACT: In patients undergoing atrial fibrillation (AF) ablation, an enlarged left atrium (LA) is a predictor of procedural failure as well as AF recurrence on long term. The most used method to assess LA size is echocardiography-measured diameter, but the most accurate remains computed tomography (CT).The aim of our study was to determine whether there is an association between left atrial diameters measured in echocardiography and the left atrial volume determined by CT in patients who underwent AF ablation.The study included 93 patients, of whom 60 (64.5%) were men and 64 (68.8%) had paroxysmal AF, who underwent AF catheter ablation between January 2018 and June 2019. Left atrial diameters in echocardiography were measured from the long axis parasternal view and the LA volume in CT was measured on reconstructed three-dimensional images.The LA in echocardiography had an antero-posterior (AP) diameter of 45.0 ±â€Š6 mm (median 45; Inter Quartile Range [IQR] 41-49, range 25-73 mm), longitudinal diameter of 67.5 ±â€Š9.4 (median 66; IQR 56-88, range 52-100 mm), and transversal diameter of 42 ±â€Š8.9 mm (IQR 30-59, range 23-64.5 mm). The volume in CT was 123 ±â€Š29.4 mL (median 118; IQR 103-160; range 86-194 mL). We found a significant correlation (r = 0.702; P < .05) between the AP diameter and the LA volume. The formula according to which the AP diameter of the LA can predict the volume was: LA volume = AP diam3 + 45 mL.There is a clear association between the left atrial AP diameter measured on echocardiography and the volume measured on CT. The AP diameter might be sufficient to determine the increase in the volume of the atrium and predict cardiovascular outcomes.

Value of Left Atrial Appendage Function Measured by Transesophageal Echocardiography for Prediction of Atrial Fibrillation Recurrence after Radiofrequency Catheter Ablation.

Atrial fibrillation (AF) recurrence after radiofrequency catheter ablation (RFCA) remains a challenging issue. This study aims to explore the left atrial appendage function by transesophageal echocardiography (TEE) and assess its value in predicting AF recurrence following RFCA in paroxysmal AF patients. Eighty-one patients with paroxysmal AF that underwent RFCA were recruited. TEE was performed before ablation with the assessment of left atrial appendage emptying flow velocity (LAAeV). AF recurrence occurred in 24 patients (29.6%) within 12 months after RFCA. The left atrium diameter (LAD) and left atrium volume index (LAVI) were both significantly higher in the recurrence group compared to the non-recurrence group, while the LAAeV was significantly lower in the recurrence group. LAD, LAVi and LAAeV were univariately significant risk factors for AF recurrence after ablation. Based on receiver operating curve (ROC), LAAeV < 40.5 cm/s, LAVi > 40.5 mL and LAD > 41 mm were identified as cut-off values for predicting AF recurrence. In multivariate regression analysis LAAeV < 40.5 cm/s (HR 8.194, 95% CI 2.980-22.530, p < 0.001) was identified as the only statistically significant independent predictor of AF recurrence, as the statistical significance threshold was not achieved for LAVI > 40.5 mL and LAD > 41 mm (p = 0.319; p = 0.507, respectively). A low LAAeV was the only important independent predictor of AF recurrence within 1 year after first RFCA.

The role of intracardiac echocardiography in reducing radiation exposure during atrial fibrillation ablation.

AIMS: Intracardiac echocardiography (ICE) is a relatively young technique used during complex electrophysiology proce-dures, such as atrial fibrillation (AF) ablation. The aim of this study was to assess whether the use of ICE modifies the radia-tion exposure at the beginning of the learning curve in AF ablation. MATERIALS AND METHODS: In this retrospective study, 52 patients, in which catheter ablation for paroxysmal or persistent AF was performed, were included. For 26 patients we used ICE guidance together with fluoroscopy, whereas for the remaining 26 patients we used fluoroscopy alone, all supported by electroanatomical mapping. We compared total procedure time and radiation exposure, including fluoroscopy dose and time between the two groups and along the learning curve. RESULTS: Most of the patients included were suffering from paroxysmal AF (40, 76%), pulmonary vein isolation being performed in all patients, without secondary ablation sites. The use of ICE was associated with a lower fluoroscopy dose (11839.60±6100.6 vs. 16260.43±8264.5 mGy, p=0.041) and time (28.00±12.5 vs. 42.93±12.7 minutes, p=0.001), whereas the mean procedure time was similar between the two groups (181.54±50.3 vs 197.31±49.8 minutes, p=0.348). Radiation exposure was lower in the last 9 months compared to the first 9 months of the study (p<0.01), decreasing gradually along the learning curve. CONCLUSIONS: The use of ICE lowers radiation exposure in AF catheter ablation from the beginning of the learning curve, without any difference in terms of acute safety or efficacy. Aware-ness towards closest to zero radiation exposure during electrophysiology procedures should increase in order to achieve better protection for both patient and medical staff.

ECG Markers of Cardiovascular Toxicity in Adult and Pediatric Cancer Treatment.

When a cardiologist is asked to evaluate the cardiac toxic effects of chemotherapy, he/she can use several tools: ECG, echocardiography, coronary angiography, ventriculography, and cardiac MRI. Of all these, the fastest and easiest to use is the ECG, which can provide information on the occurrence of cardiac toxic effects and can show early signs of subclinical cardiac damage. These warning signs are the most desired to be recognized by the cardiologist, because the dose of chemotherapeutics can be adjusted so that the clinical side effects do not occur, or the therapy can be stopped in time, before irreversible side effects. This review addresses the problem of early detection of cardiotoxicity in adult and pediatric cancer treatment, by using simple ECG recordings.

Efficacy of amiodarone for the prevention of atrial fibrillation recurrence after cardioversion.

The restoration and maintenance of sinus rhythm is a desirable strategy for many patients with atrial fibrillation (AF) since it has been associated with improvement in symptoms and a better quality of life. Sinus rhythm can be achieved by pharmacological or electrical cardioversion or after catheter ablation of AF. Despite high rates of successful cardioversion, AF recurrence remains a major challenge. Anti-arrhythmic drug therapy currently plays a significant role in maintaining sinus rhythm after cardioversion. Amiodarone is the most commonly prescribed anti-arrhythmic drug for patients with AF. This is due to its particular electrophysiological properties and superior anti-arrhythmic effects in comparison with other anti-arrhythmic drugs. Understanding the cardiac electrophysiology and arrhythmogenesis mechanisms may result in identification of new targets for anti-arrhythmic therapy. The aim of this article was to review amiodarone's clinical pharmacology and evaluate evidence supporting amiodarone for treatment and prevention of AF recurrence after cardioversion.

Atrial high-rate episodes: a comprehensive review.

Cardiac electronic implantable devices (CIEDs) have the ability to monitor, store and interpret complex arrhythmias, which has generated a new arrhythmic entity: atrial high-rate episodes (AHRE). AHRE are atrial tachyarrhythmias, detected only by CIEDs. They are widely considered a precursor to atrial fibrillation (AF) but can also be represented by other kinds of supraventricular arrhythmias such as atrial flutter or atrial tachycardia. CIED-detected AHRE are associated with an increased risk of stroke, but the risk is significantly lower than the stroke risk of clinical AF. Moreover, there seems to be no temporal correlation between AHRE and thromboembolic events. Because of the current gaps in evidence, the appropriate management of this arrythmia can be challenging. In this review we take into account the epidemiology behind AHRE, predictive factors, clinical impact and management of this arrhythmia.

An Exploratory Association Analysis of ABCB1 rs1045642 and ABCB1 rs4148738 with Non-Major Bleeding Risk in Atrial Fibrillation Patients Treated with Dabigatran or Apixaban.

(1) Background: The approach of bleeding complications in patients treated with non-vitamin K oral anticoagulants (NOACs) represents an important issue in clinical practice. Both dabigatran and apixaban are substrates for P-glycoprotein and, therefore, ABCB1 gene variations may be useful in individualizing NOACs treatment, especially in high-risk patients. (2) Methods: ABCB1 rs1045642 and rs4148738 were determined in 218 atrial fibrillation patients treated with dabigatran or apixaban (70.94 ± 9.04 years; 51.83% men). (3) Results: Non-major bleeding appeared in 7.34% NOACs-treated patients. The logistic tested models based on the four genetic models revealed no significant association between the variant genotype of two ABCB1 SNPs and the risk of bleeding (p > 0.05). Among the four two-locus haplotypes, TA and CA haplotypes had the highest frequency in NOACs-treated patients with bleeding, involving a possible positive association with the susceptibility of bleeding complications (OR = 1.04 and OR = 1.91, respectively). The logistic model found no significant association of estimated haplotypes with bleeding (p > 0.05) except for the TG haplotype which had a trend toward statistical significance (p = 0.092). Among the risk factors for bleeding, only age > 70 years and stroke/TIA showed a tendency toward statistical significance. (4) Conclusions: We found no significant associations between the studied ABCB1 variant genotypes with non-major bleeding risk in NOACs-treated patients. A trend of association between TG haplotype with bleeding risk was observed, implying a protective role of this haplotype against bleeding in patients treated with dabigatran or apixaban.

Catheter ablation of a latent accessory pathway under continuous infusion of adenosine: A case report.

RATIONALE: In absence of conduction over the accessory pathway (AP) during the electrophysiological study, mapping and ablation is impossible. Various techniques can be used to activate absent conduction. In this presentation we describe the first case of latent AP ablation performed under continuous infusion of adenosine. PATIENT CONCERNS: A 65-year-old man, presented to emergency department with atrial fibrillation and antegrade conduction through a left lateral AP. He had palpitations and lightheadedness that occurred every 2 to 3 weeks. DIAGNOSIS: The electrophysiological study confirmed a latent left-side AP. INTERVENTIONS: Catheter ablation could not be performed because of absent conduction through AP. Therefore, a continuous infusion of adenosine was used to activate AP. Ablation was performed at the left lateral mitral ring. OUTCOMES: After catheter ablation and a new adenosine bolus there was no conduction through AP. LESSONS: In case of a latent AP when ablation is difficult to perform because of absent conduction at the time of electrophysiological study, adenosine can be used in doses of 1.5 mg/kg over 5 minutes continuous infusion.

Evaluating Physician Adherence to Antithrombotic Recommendations in Patients with Atrial Fibrillation: A Pathway to Better Medical Education.

BACKGROUND: Atrial fibrillation is a major health problem due to the stroke risk associated with it. To reduce stroke risk, oral anticoagulants (OAC) are prescribed using the CHA2DS2-VASc (Congestive heart failure; Hypertension; Age ≥75 years; Diabetes Mellitus; Stroke; Vascular disease; Age 65-74 years; Sex category) risk score, a clinical probability assessment that includes a combination of risk factors predicting the probability of a stroke. Not all patients with high risk are receiving this treatment. The aim of this study was to assess physician adherence to clinical guidelines concerning the OAC treatment and to identify the factors that were associated with the decision to prescribe it. METHODS: Registry data from 784 patients with non-valvular atrial fibrillation were evaluated in this retrospective cross-sectional study. Demographic data, subtype of AF, comorbidities associated with higher stroke and bleeding risk, and antithrombotic treatment received were recorded. We compared stroke and bleeding risk in patients with and without OAC treatment to determine if the clinicians followed guidelines: prescribed when necessary and abstained when not needed. RESULTS: OAC treatment was administered in 617 (78.7%) patients. Of the 167 patients who did not receive OAC, 161 (96.4%) were undertreated according to their risk score, as opposed to those who received OAC in which the percentage of overtreated was 3.2%. Most undertreated patients (60.5%, p < 0.001) were with paroxysmal atrial fibrillation subtype. CONCLUSIONS: The decision to use anticoagulants for stroke prevention was based on the type of atrial fibrillation, rather than on the risk of stroke as quantified by CHA2DS2-VASc as per the recommended guidelines.

Transesophageal and intracardiac ultrasound in arrhythmogenic right ventricular dysplasia/cardiomyopathy: Two case reports.

RATIONALE: Two-dimensional echocardiography (2D echo) is a major tool for the diagnosis of Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). However 2D echo can skip regional localized anomalies of the right ventricular wall. We aimed to determine whether transesophageal and intracardiac ultrasound can provide additional information, on the right ventricular abnormalities compared to 2D echo. PATIENT CONCERNS: Case 1 is a 30-year-old patient that presented in the Emergency Department with multiple episodes of fast monomorphic ventricular tachycardia (VT) manifested by palpitations and diziness. Case 2 is a 65-year-old patient that also presented with episodes of ventircular tachycardia associated with low blood pressure. DIAGNOSIS: Both patients had a clear diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy confirmed by cardiac magnetic resonance imaging. INTERVENTION: In both patients transesophageal and intracardiac ultrasound was performed, which brought more information on the diagnosis of ARVD/C compared to transthoracic echocardiograpy. OUTCOMES: The first patient was implanted with an internal cardiac defibrillator and treated with Sotalol for VT recurrences. He presented episodes of VT during follow-up, treated with antitachycardia pacing. The second patient was implanted with an internal cardiac defibrillator and treated with Sotalol without any VT recurrence at 18 month-follow-up. LESSONS: Transesophageal echocardiography and intracardiac echocardiography can provide additional information on small, focal structural abnormalities in patients with ARVD/C: bulges, saculations, aneurysms with or without associated thrombus, partial or complete loss of trabeculations and hypertrophy of the moderator band. These changes are particularly important in cases with "concealed" form of the disease in which no morphological abnormalities are evident in transthoracic echocardiograpy.

What is the optimal duration of the TILT after administration of 0.4 mg nitroglycerin spray?

Performing a head-up tilt test can be of great value for the diagnosis of vasovagal syncope. The European Society of Cardiology recommends a drug challenge phase duration of 15 to 20 minutes, with either Isoprenaline or Nitroglycerin administration.We sought to investigate the outcome of a ten-minute active phase with Nitroglycerin in patients suspected of vasovagal syncope and determine the percentage of loss in the positivity rate, using this short duration approach.We consecutively enrolled patients presenting with syncope undergoing the head-up tilt test (HUTT), with a clinical suspicion of vasovagal syncope between the years 2009 to 2019. The HUTT consisted of 2 successive phases: passive and active. During the passive phase, the patients were tilted at 70° for 20 minutes. If negative, the test was continued after administration of 0.4 mg sublingual Nitroglycerin. Applying the (VASIS) Vasovagal Syncope International Study classification of the vasovagal syncope international study and comparing for age and gender, positive responses were categorized into 3 types.A number of 306 patients (age = 43.5 +/-20.3; male = 140 [45.7%]) with suspected vasovagal syncope, undergoing HUTT, were enrolled in the years of 2009 to 2019. Of those, 245 (80.2%) presented a positive test, with 200 patients (82.0%) during the being positive during the active phase of the test. The results were as follows: 116 subjects (47.2%) presented with a mixed response (VASIS I), 52 (21.3%) showed a cardio inhibitory response (VASIS II), and 77 (31.5%) displayed a vasodepressor response (VASIS III). We found no relationship between the type of syncope with neither gender, nor age of the patient. Three minutes represented the median time to positivity, after Nitroglycerine administration. The time distribution showed a peak incidence appearing between minutes 3 and 5, ranging from 1 to 20 minutes. Only 3 patients tested positive after minute 10.Shortening the active phase to 10 minutes would result in a positivity rate loss of 1.5%.

The Impact of Pulmonary Vein Anatomy on the Outcomes of Catheter Ablation for Atrial Fibrillation.

Background and Objectives: Prior studies have identified a number of predictors for Atrial fibrillation (AF) ablation success, including comorbidities, the type of AF, and left atrial (LA) size. Ectopic foci in the initiation of paroxysmal AF are frequently found in pulmonary veins. Our aim was to assess how pulmonary vein anatomy influences the recurrence of atrial fibrillation after radiofrequency catheter ablation. Materials and Methods: Eighty patients diagnosed with paroxysmal or persistent AF underwent radiofrequency catheter ablation (RFCA) between November 2016 and December 2017. All of these patients underwent computed tomography before AF ablation. PV anatomy was classified according to the presence of common PVs or accessory PVs. Several clinical and imagistic parameters were recorded. After hospital discharge, all patients were scheduled for check-up in an outpatient clinic at 3, 6, 9, and 12 months after RFCA to detect AF recurrence. Results: A total of 80 consecutive patients, aged 53.8 ± 9.6 years, 54 (67.5%) men and 26 (32.5%) women were enrolled. The majority of patients had paroxysmal AF 53 (66.3%). Regular PV anatomy (2 left PVs, 2 right PVs) was identified in 59 patients (73.7%), a left common trunk (LCT) was detected in 15 patients (18.7%), an accessory right middle pulmonary vein (RMPV) was found in 5 patients (6.25%) and one patient presented both an LCT and an RMPV. The median follow-up duration was 14 (12; 15) months. Sinus rhythm was maintained in 50 (62.5%) patients. Age, gender, antiarrhythmic drugs, and the presence of cardiac comorbidities were not predictive of AF recurrence. The diagnosis of persistent AF before RFCA was more closely associated with an increase in recurrent AF after RFCA than after paroxysmal AF (p = 0.01). Longer procedure times (>265 minutes) were associated with AF recurrence (p = 0.04). Patients with an LA volume index of over 48.5 (mL/m2) were more likely to present AF recurrence (p = 0.006). Multivariate analysis of recurrence risk showed that only the larger LA volume index and variant PV anatomy were independently associated with AF recurrence. Conclusion: The study demonstrated that an increased volume of the left atrium was the most important predictive factor for the risk of AF recurrence after catheter ablation. Variant anatomy of PV was the only other independent predictive factor associated with a higher rate of AF recurrence.

Intracardiac echocardiography for transseptal puncture. A guide for cardiac electrophysiologists.

The key to a successful catheterization of the left heart chambers is the safe transseptal puncture. Intracardiac echocardiography (ICE) is an attractive tool used in cardiac catheterization and electrophysiology labs to provide detailed images thatcan facilitate transseptal puncture. ICE permits a direct visualization of the endocardium and precisely locates the needle and the sheath against the interatrial septum. Two different ICE catheters are available: a phased array and a mechanical transducer, both being currently used in clinical practice. This paper describes the technique used for guiding transseptal puncture with ICE. Due to its advantages, ICE has currently become an important tool used to maximize the safety of the transseptal puncture and increase efficacy of interventional cardiology procedures.