Search

Introduction: The diagnosis of Duchenne-linked cardiomyopathy may be challenging. Conventional echocardiographic measurements typically show deterioration beyond the second decade. Global longitudinal strain has been proposed as an earlier marker than left ventricular ejection fraction., Material and Methods: A prospective, observational, cross-sectional, case-control study was carried out. Both Duchenne patients and control subjects underwent transthoracic echocardiogram in order to assess left ventricle function. Bayesian linear regression was the main tool for inference. Age effects were parameterised by means of a spline function because of its simplicity to characterise continuous variables and smooth contributions. The posterior distribution of the marginal age effects was used to assess the earliest age of deterioration of each marker., Results: Sixteen Duchenne patients and twenty-two healthy male subjects were enrolled. On overage, cardiac function measures were found for ejection fraction and longitudinal strain among different groups. Age effects on global longitudinal strain are more reliably found at ages of 6 and above, while ejection fraction starts to deteriorate at an older age. Progressive left ventricular dysfunction in Duchenne patients is one of the key issues and starts at an early age with subtle symptoms., Conclusion: This cross-sectional study provides supporting evidence that global longitudinal strain is an earlier marker of disease progression than ejection fraction in Duchenne patients.

Background: Bronchopulmonary dysplasia (BPD) is still a major concern in preterm infants and adequate screening methods for secondary right ventricle (RV) failure are needed. Early detection could be aided by taking measurements of RV deformation using speckle tracking echocardiography. A prospective longitudinal study was carried out over 28 months at a tertiary care pediatric cardiology center. Preterm infants < 32 weeks gestational age (GA) were eligible for the study. Enrolled patients were separated in two groups: NO-BPD or BPD. At three timepoints over the first year of life, echocardiogram measurements were performed. Right ventricle strain was studied using speckle tracking analysis and compared to conventional function parameters., Results: Fifty patients were enrolled in the study, 22 in the NO-BPD group and 28 in the BPD group. RV strain showed no statistical differences between groups. However, the BPD group showed worse RV function than the NO-BPD group, using speckle tracking analysis and other conventional parameters. During the study follow-up, an improvement trend is shown in RV strain., Conclusions: RV longitudinal strain and strain rate derived by speckle tracking is feasible in preterm infants. Although there seems to be a good correlation between RV strain and BPD severity, the results of this study were not conclusive. More studies should be carried out to investigate the optimum echocardiographic screening model of RV dysfunction in BPD patients., (© 2023. The Author(s).)

Introduction: Pulmonary arterial hypertension (PAH) is a rare, multifactorial, chronic condition that requires ongoing monitoring and assessment. PAHcare™ is a novel, patient-centered digital platform that provides software intended for use on patients' mobile phones (downloadable application) and web-based dashboards for use by physicians and health coaches (HC). We describe herein the protocol of a clinical study aimed at evaluating the clinical benefit and safety of PAHcare™ for the routine management of patients with PAH., Methods and Analysis: In this prospective, single cohort, multicenter study, 50 patients with PAH will be recruited at six specialized PAH units from reference hospitals of the public Spanish healthcare system. The PAHcare™ digital health platform allows patients to log health and lifestyle information while also providing structured content for patient education, medication reminders, and behavioral and lifestyle coaching from a remote HC. Evaluation will be primarily focused on the impact of the platform use on the patient's health-related quality of life (HRQoL) via questionnaires completion through electronic patient-reported outcomes. Moreover, the analysis of the impact on the patient's functional status, signs and symptoms of PAH, patient costs and healthcare resource utilization, satisfaction, knowledge of the disease and its management, and adherence to and safety of the platform will be secondary outcomes. The clinical investigation started in July 2021 and is expected to end by September 2022., Discussion: The PAHcare™ platform is anticipated to provide direct benefits to healthcare professionals, patients, and caregivers. These include the simplification of the multidisciplinary approach needed to tailor routine PAH management, enhancement of the patient/healthcare professional interaction, patient's empowerment to become more actively involved in the management and treatment of the disease, and increase of the patient's and caregiver's knowledge on PAH., Competing Interests: PE has received consulting and lecture fees by Ferrer Internacional S.A. Barcelona, Spain, Acceleron, Janssen, MSD. GP has received consulting fees by Ferrer Internacional S.A. Barcelona, Spain. RA, HM, and GB are employees of Ferrer Internacional S.A. Barcelona, Spain. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Pérez Peñate, Ochoa Parra, Domingo Morera, Martínez Meñaca, López Ramón, Cadenas Menéndez, León Marrero, Gómara de la Cal, Ghadban Garrido, Royo Tolosana, Martin Puentes, Aldonza Aguayo, Mahdavi, Bacchini Jeanneret and Escribano Subías.)

Myocardial deformation by speckle tracking echocardiography is a novel method for evaluating cardiac function. To test the hypothesis that right ventricular and left ventricular function have age-specific patterns of development, we tracked the evolution of ventricular strain mechanics by speckle tracking echocardiography in the fetus. We conducted a retrospective cross sectional echocardiography study in 154 healthy fetuses, and characterized cardiac function by measuring right and left ventricles global longitudinal strain and strain rate. Comparison of the data of both ventricles according to gestational age was carried out. The magnitudes of right and left ventricle global longitudinal strain show wide range values and decreased throughout gestation. Strain values are higher in left ventricle compared to the right one throughout pregnancy. Strain rate values were similar over gestation in each ventricle, but the magnitudes declined overtime in the right and left ventricle. The maturational patterns of left and right strain are gestational specific. With accepted physiological maturation patterns in healthy subjects, these myocardial deformation parameters can provide a valid basis that allows comparison between health and disease.

Ataxia-telangiectasia is a disorder characterized by cerebellar ataxia, telangiectasia, immunodeficiency, and increased predisposition to cancer susceptibility. Mutations in the ataxia telangiectasia mutated gene seem to play an important role in normal cell function and in cardiovascular remodeling. We report a case of a 14-year-old boy with ataxia-telangiectasia and high-grade B-non-Hodgkin lymphoma who remained in continuous complete remission after chemotherapy and who was admitted into our Emergency Room presenting with episodes of presyncope. At admission he presented a complete atrioventricular block that evolved into asystole and required placement of a pacemaker. Cumulative cardiotoxic drugs received were at low risk. However, it is possible that this chronic degenerative disease may affect the cardiac conduction system over time. In the reviewed literature there are no or unknown reports of ataxia-telangiectasia with malignant cardiac arrhythmias., (Sociedad Argentina de Pediatría.)

Percutaneous closure is nowadays considered the treatment of choice of ostium secundum atrial septal defects (ASD). However, transcatheter closure can be highly challenging when we face an ASD with complex morphological features. The combination of different imaging modalities can be very helpful. This case shows the great value of using both intracardiac and real time 3D transesophageal echocardiography for the percutaneous closure of a complex iatrogenic ASD after device embolization., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Acute pericarditis is the most common disease of the pericardium encountered in clinical practice. It is diagnosed in 0.1% of all admissions and 5% of emergency room admissions for chest pain. In developed countries, it is usually due to a benign cause. Idiopathic and infectious pericarditis are more common than secondary to surgical pericardiotomy or neoplastic causes, whereas tuberculosis is the dominant cause in developing countries. The most common symptoms of pericarditis are characteristic chest pain and fever. Since pericarditis presents a benign outcome because of self-limiting and good response to conventional anti-inflammatory therapy, it can be safely managed on outpatient basis unless a specific cause is suspected or the patient has high-risk features to avoid complications such as pericardial effusion, cardiac tamponade or recurrent pericarditis. We report a case of pericarditis, diagnosed 9 months after surgical closure of an atrial septal defect, in a 7-year-old boy with favorable evolution., (Sociedad Argentina de Pediatría.)

Background: The impact of high exercise loads on a previously healthy heart remains controversial. We examined the consequences of decades of strenuous endurance exercise at the highest competition level on heart dimensions and volumes as well as on serum biomarkers of cardiac fibrosis/remodeling., Methods and Results: We compared echocardiographic measurements and serum biomarkers of cardiac fibrosis/remodeling [troponin I, galectin-3, matrix metallopeptidase-2 and -9, N-terminal pro-brain natriuretic peptide, carboxy-terminal propeptide of type I procollagen, and soluble suppressor of tumorigenicity-2 (sST-2)/interleukin(IL)-1R4] in 53 male athletes [11 former professional ('elite') and 42 amateur-level ('sub-elite') cyclists or runners, aged 40-70years] and 18 aged-matched controls. A subset of 15 subjects (5 controls, 3 sub-elite and 7 elite athletes) also underwent cardiac magnetic resonance imaging (cMRI). Elite and sub-elite athletes had greater echocardiography-determined left ventricular myocardial mass indexed to body surface area than controls (113±22, 115.2±23.1 and 94.8±21g/m(2), respectively, p=0.008 for group effect), with similar results for left (50.5±4.4, 48.2±4.3 and 46.4±5.2mm, p=0.008) and right (38.6±3.8, 41.1±5.5 and 34.7±4.3mm, p<0.001) ventricular end-diastolic diameter, and cMRI-determined left atrial volume indexed to body surface area (62.7±8.1, 56.4±16.0 and 39.0±14.1ml/m(2), p=0.026). Two athletes showed a non-coronary pattern of small, fibrotic left ventricular patches detected by late gadolinium enhancement. No group effect was noted for biomarkers., Conclusions: Regardless of their competition level at a younger age, veteran endurance athletes showed an overall healthy, non-pathological pattern of cardiac remodeling. Nonetheless, the physiopathology of the ventricular fibrotic patches detected warrants further investigation., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Although extrarenal manifestations of haemolytic-uraemic syndrome are not frequent, myocardial dysfunction should be given special consideration because of the importance of proper early haemodynamic management and potential complications. We report the case of a 21-month-old child with haemolytic-uraemic syndrome who developed clinical signs of poor myocardial function with depressed myocardial function noted by bedside echocardiography and significant elevation of biomarkers. Early intervention and supportive treatment for the patient were crucial during the acute phase of cardiac failure, and repeated monitoring of biomarkers and ecocardiography were useful diagnostic tools that provided relevant information throughout the patient's evolution.

Background: In this research, an extension to our previous work published in the Clinical Journal of Sports Medicine in 2009, we studied subjects that differed in terms of age and training status and assessed the impact of prolonged exercise on systolic and left ventricular diastolic function and cardiac biomarkers levels, recognized as identifiers of cardiac damage and dysfunction. We also assessed the possible influence of event duration, exercise intensity and weight loss (dehydration) on left ventricular diastolic function., Findings: Ninety-one male cyclists were assessed by echocardiography and serum biomarkers before and after the 2005 Quebrantahuesos cycling event (206 km long and with an accumulated slope of 3800 m). Cardiac function was assessed by echocardiography and cardiac biomarkers were assessed in blood serum. Echocardiograms measured left ventricular internal dimension during diastole and systole, left ventricular posterior wall thickness during diastole, interventricular septum thickness during diastole, left ventricular ejection fraction and diastolic filling. The heart rate of 50 cyclists was also monitored during the race to evaluate exercise intensity. Echocardiograph results indicated that left ventricular diastolic and systolic function decreased after the race, with systolic function reduced to a significant degree. Left ventricular ejection fraction was below 55% in 29 cyclists. The decrease in left ventricular systolic and diastolic function did not correlate with age, training status, race duration, weight loss or exercise intensity., Conclusions: Left ventricular systolic and diastolic function was reduced and cardiac biomarkers were increased after the cycling event, but the mechanisms behind such outcomes remain unclear.

Endomyocardial fibrosis is a restrictive cardiomyopathy of unknown etiology prevalent in tropical regions. It is characterized by fibrotic obliteration of the apex of one or both ventricles, which can be extended to the ventricular inlet chamber. It predominantly affects children and young adults. There is no specific treatment and usually has a poor prognosis. It is uncommon in Spain, but due to immigration, it appears to be increasing. Four cases of endomyocardial fibrosis in children diagnosed and treated in Spain are presented, as a well as a review of this disease., (Copyright © 2012 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.)

Objective: To determine the influence of age, training status, race time, and exercise intensity on the appearance of cardiac biomarkers after a cycle-touring event., Design: Pre-post exercise measurements., Setting: University of Zaragoza, Zaragoza, Spain., Participants: Ninety-one amateur cyclists., Intervention: The 2005 Quebrantahuesos cycle-touring event (distance: 206 km; altitude difference: 3800 m)., Main Outcome Measures: Cardiac troponin I (cTnI) and N-terminal pro-brain natriuretic peptide (NT-pro-BNP) were measured the day before and immediately after the race. Heart rate (HR) monitoring was performed on 55 cyclists during the race to evaluate exercise intensity., Results: cTnI and NT-pro-BNP were significantly elevated immediately post race, with 43% of participants exhibiting cTnI levels greater than 0.04 microg/L and 65% of the participants measuring NT-pro-BNP levels greater than 125 ng/L. The cTnI increase was significantly associated with mean exercise intensity HR (r = 0.36, P < 0.01) but was not associated with age, training status, or race time. The increase in NT-pro-BNP correlated with race time (r = 0.40, P < 0.001) and training status (r = -0.36, P < 0.001) but was not associated with age or exercise intensity HR measures., Conclusions: These results suggest that, in part, exercise intensity (HR) is responsible for the cTnI increase and that race time is responsible for the increase in NT-pro-BNP. Future standardized endurance exercise trials are recommended to further elucidate the potentially differential effects of training status, exercise time, and intensity on post-exercise increases in cTnI and NT-pro-BNP.