The Prevalence of and Predisposing Factors for Late Atrial Arrhythmias after Transcatheter Closure of Secundum Atrial Septal Defects in Children

Background: A 24 h Holter study in children after transcatheter secundum ASD (ASD II) closure was conducted to detect the prevalence of defects and/or device-related late atrial arrhythmias (LAAs). ASD II closure with an Amplatzer septal occluder (ASO) is an established procedure. Little is known about LAAs after device implantation. Methods: The eligible participants were children who had undergone ASO implantation, with a follow-up of ≥5 years, as well as one pre- and at least one post-procedural Holter ECG. Results: In total, 161 patients (mean age: 6.2 ± 4.3 years), with a mean follow-up of 12.9 ± 3.1 years (range 5–19), were included. A median of four Holter ECGs per patient were available. LAAs occurred before intervention in four patients (2.5%), and it was peri-interventional in four patients (2.5%), sustained in three patients (1.9%), and developed in three patients (1.9%). In patients with pre- and peri-interventional LAAs, the Qp/Qs ratio was higher (6.4 ± 3.9 vs. non-AA: 2.0 ± 1.1 (p = 0.002)) and the IAS/ASO ratio was lower (1.18 ± 0.27 vs. non-AA: 1.7 ± 0.4 (p < 0.001)). The patients with LAAs differed from those without LAAs in their Qp/Qs (6.8 ± 3.5 vs. 2.0 ± 1.3; p < 0.0001) and IAS/ASO ratios (1.14 ± 0.19 vs. 1.73 ± 0.45; p < 0.001). The patients with LAAs had a Qp/Qs ratio ≥2.94:1, and those who developed LAAs had an IAS/ASO ratio <1.15. Conclusions: LAAs occurred in 1.9% of patients and were sustained in another 1.9% of patients but persisted in those with large shunt defects and large occluders in relation to the atrial septal length. The predisposing factors for LAAs after ASD closure were a high Qp/Qs ratio, pre-existing atrial arrhythmias, and a low IAS/ASO ratio

Evaluating Cardiac Lateralization by MRI to Simplify Estimation of Cardiopulmonary Impairment in Pectus Excavatum

Background: The severity of pectus excavatum is classified by the Haller Index (HI) and/or Correction Index (CI). These indices measure only the depth of the defect and, therefore, impede a precise estimation of the actual cardiopulmonary impairment. We aimed to evaluate the MRI-derived cardiac lateralization to improve the estimation of cardiopulmonary impairment in Pectus excavatum in connection with the Haller and Correction Indices. Methods: This retrospective cohort study included a total of 113 patients (mean age = 19.03 ± 7.8) with pectus excavatum, whose diagnosis was verified on cross-sectional MRI images using the HI and CI. For the development of an improved HI and CI index, the patients underwent cardiopulmonary exercise testing to assess the influence of the right ventricle’s position on cardiopulmonary impairment. The indexed lateral position of the pulmonary valve was utilized as a surrogate parameter for right ventricle localization. Results: In patients with PE, the heart’s lateralization significantly correlated with the severity of pectus excavatum (p ≤ 0.001). When modifying HI and CI for the individual’s pulmonary valve position, those indices are present with greater sensitivity and specificity regarding the maximum oxygen-pulse as a pathophysiological correlate of reduced cardiac function (χ2 10.986 and 15.862, respectively). Conclusion: The indexed lateral deviation of the pulmonary valve seems to be a valuable cofactor for HI and CI, allowing for an improved description of cardiopulmonary impairment in PE patients

Comparative Study of 2D-Cine and 3D-wh Volumetry: Revealing Systemic Error of 2D-Cine Volumetry

This study investigates the crucial factors influencing the end-systolic and end-diastolic volumes in MRI volumetry and their direct effects on the derived functional parameters. Through the simultaneous acquisition of 2D-cine and 3D whole-heart slices in end-diastole and end-systole, we present a novel direct comparison of the volumetric measurements from both methods. A prospective study was conducted with 18 healthy participants. Both 2D-cine and 3D whole-heart sequences were obtained. Despite the differences in the creation of 3D volumes and trigger points, the impact on the LV volume was minimal (134.9 mL ± 16.9 mL vs. 136.6 mL ± 16.6 mL, p &lt; 0.01 for end-diastole; 50.6 mL ± 11.0 mL vs. 51.6 mL ± 11.2 mL, p = 0.03 for end-systole). In our healthy patient cohort, a systematic underestimation of the end-systolic volume resulted in a significant overestimation of the SV (5.6 mL ± 2.6 mL, p &lt; 0.01). The functional calculations from the 3D whole-heart method proved to be highly accurate and correlated well with function measurements from the phase-contrast sequences. Our study is the first to demonstrate the superiority of 3D whole-heart volumetry over 2D-cine volumetry and sheds light on the systematic error inherent in 2D-cine measurements.</jats:p

Impact of premature birth on cardiopulmonary function in later life

Pulmonary function is reduced in children after preterm birth. The variety of subgroups ranges from early to late preterm births. Limitations in pulmonary function can be observed even after late preterm birth without signs of bronchopulmonary dysplasia and/or history of mechanical ventilation. Whether this reduction in lung function is reflected in the cardiopulmonary capacity of these children is unclear. This study aims to investigate the impact of moderate to late premature birth on cardiopulmonary function. Cardiopulmonary exercise testing on a treadmill was performed by 33 former preterm infants between 8 and 10 years of age who were born between 32 + 0 and 36 + 6 weeks of gestation and compared with a control group of 19 children born in term of comparable age and sex. The former preterm children achieved comparable results to the term-born controls with respect to most of the cardiopulmonary exercise parameters (V˙O2peak43.9±6.6mlkgminminvs.41.9±8.8mlkg/min). The only differences were in a slightly higher oxygen uptake efficiency slope (OUESof1.6±0.4vs1.4±0.4) and higher peak minute ventilation V˙Epeakof55.2±11.3ml/minvs.49.1ml±8.8/min) in the group of children born preterm. With respect to heart rate recovery (-35.3±13.8bpmvs.-37.2bpm±14.0after1min) and breathing efficiency (V˙E/V˙CO2of35.9±4.1vs34.0±4.6), there were no significant differences.Conclusion: Children born preterm did not show limitations in cardiopulmonary function in comparison with matched controls. What is Known:• Preterm birth is associated with reduced pulmonary function in later life, this is also true for former late preterms.• As a consequence of being born premature, the lungs have not finished their important embryological development. Cardiopulmonary fitness is an important parameter for overall mortality and morbidity in children and adults and a good pulmonary function is therefore paramount.What is New:• Children born prematurely were comparable to an age- and sex-matched control group with regards to almost all cardiopulmonary exercise variables.• A significantly higher OUES, a surrogate parameter for VO2peak was found for the group of former preterm children, most likely reflecting on more physical exercise in this group. Importantly, there were no signs of impaired cardiopulmonary function in the group of former preterm children.Open Access funding enabled and organized by Projekt DEAL.Friedrich-Alexander-Universität Erlangen-Nürnberg (1041

Heart and Cardiovascular Involvement in Patients with Mucopolysaccharidosis Type IVA (Morquio-A Syndrome).

Mucopolysaccharidosis (MPS) IVA is a rare lysosomal storage disorder with multiple skeletal and non-skeletal abnormalities requiring multiple surgical interventions. It is well known that patients with MPS IVA suffer from tachycardia, but cardiac and hemodynamic alterations have not been reported to date. We investigated the cardiovascular and hemodynamic alterations in patients with MPS IVA and developed a possible patho-mechanism for cardiovascular deterioration during anesthesia.In this observational study, serial cardiac examinations were performed in 54 patients with MPS IVA who were followed at the Children's Hospital of the Mainz Medical University (Mainz, Germany) between 1991 and 2014 (follow-up 1-24 years; median 5.8 years). Results were compared with data from a large central European cohort of more than 2000 healthy infants and children.None of the patients had arterial hypertension, but 4% had evidence of increased pulmonary artery pressure. Patients developed aortic root extension up to 6.9 standard deviations above normal. Left-sided valve leaflet thickening occurred in 26 patients (five with valve disease). Patients had lower left ventricular dimensions (z: -1.02±0.1), lower stroke volumes (z: -2.3±0.17), lower left ventricular mass (z: -1.5±0.21), but higher wall thickness (z: +0.8±0.16), and higher work index (z: +2.5±0.2) compared to healthy control subjects. Cardiac output was preserved by an increase in heart rate of 21%. Sixty % of patients showed impaired diastolic filling; heart rate (99.0±1.8 vs. 92.0±2.1 bpm), age (18.0±1.8 vs. 14.2±1 years), and cardiothoracic ratio (61.6±3.6% vs. 55±4.2%) of these patients were higher compared to those with normal filling.The results of this study suggest an age-progressive disproportion of the intra-thoracic organs of patients with MPS IVA, which is accompanied by aortic root extension and thickened left ventricles, with reduced stroke volumes, impaired diastolic filling patterns, and increased heart rates

Cumulative Prevalence of Valve Disease in MPS IVA Patients.

<p>Patients presenting with (dotted line) or without (solid line) valve thickening at first examination. Bars on the line indicate patients without valve thickening at first examination. Early valve thickening predisposes to the development of valve disease at later age. Created using SPSS software.</p

Correlation Between Heart Rate and Cardiothoracic Ratio (CTR) in MPS IVA Patients.

<p>Patients with higher CTR had increased heart rates (r² = 0.52; CTR = 0.171*HR + 42.43; ANOVA F = 9.9, p = 0.04). Created using SPSS software.</p

Baseline Biometric Characteristics of MPS IVA Patients (n = 54).

<p>Baseline Biometric Characteristics of MPS IVA Patients (n = 54).</p

Cardiac Data for MPS IVA Patients with and without Valvular Thickening.

<p>Cardiac Data for MPS IVA Patients with and without Valvular Thickening.</p

Valve Involvement in MPS IVA Patients (n = 54).

<p>Valve Involvement in MPS IVA Patients (n = 54).</p