Original Article
Efficacy and safety of coronary computed tomography angiography in diagnosing coronary lesions in children
- Sharon W. Gould, M. Patricia Harty, Mark Cartoski, Vijay Krishnan, Nicole Givler, John Ostrowski, Takeshi Tsuda
-
- Published online by Cambridge University Press:
- 25 October 2023, pp. 838-845
-
- Article
- Export citation
-
Introduction:
Identification of paediatric coronary artery abnormalities is challenging. We studied whether coronary artery CT angiography can be performed safely and reliably in children.
Materials:Retrospective analysis of consecutive coronary CT angiography scans was performed for image quality and estimated radiation dose. Both factors were assessed for correlation with electrocardiographic-gating technique that was protocoled on a case-by-case basis, radiation exposure parameters, image noise artefact parameters, heart rate, and heart rate variability.
Results:Sixty scans were evaluated, of which 96.5% were diagnostic for main left and right coronaries and 91.3% were considered diagnostic for complete coronary arteries. Subjective image quality correlated significantly with lower heart rate, increasing patient age, and higher signal-to-noise ratio. Estimated radiation dose only correlated significantly with choice of electrocardiographic-gating technique with median doses as follows: 2.42 mSv for electrocardiographic-gating triggered high-pitch spiral technique, 5.37 mSv for prospectively triggered axial sequential technique, 3.92 mSv for retrospectively gated technique, and 5.64 mSv for studies which required multiple runs. Two scans were excluded for injection failure and one for protocol outside the study scope. Five non-diagnostic cases were attributed to breathing motion, scanning prior to peak contrast enhancement, or scan acquisition during the incorrect portion of the R-R interval.
Conclusions:Diagnostic-quality coronary CT angiography can be performed reliably with a low estimated radiation exposure by tailoring each scan protocol to the patient’s body habitus and heart rate. We propose coronary CT angiography is a safe and effective diagnostic modality for coronary artery abnormalities in children.
Cardiac magnetic resonance predictors for successful primary biventricular repair of unbalanced complete common atrioventricular canal
- Andrea L. Jones, Brian R. White, Reena M. Ghosh, Antara Mondal, Steve Ampah, Deborah Y. Ho, Kevin Whitehead, Matthew A. Harris, David M. Biko, Sara Partington, Stephanie Fuller, Meryl S. Cohen, Mark A. Fogel
-
- Published online by Cambridge University Press:
- 18 July 2023, pp. 387-394
-
- Article
- Export citation
-
Background:
Patients with unbalanced common atrioventricular canal can be difficult to manage. Surgical planning often depends on pre-operative echocardiographic measurements. We aimed to determine the added utility of cardiac MRI in predicting successful biventricular repair in common atrioventricular canal.
Methods:We conducted a retrospective cohort study of children with common atrioventricular canal who underwent MRI prior to repair. Associations between MRI and echocardiographic measures and surgical outcome were tested using logistic regression, and models were compared using area under the receiver operator characteristic curve.
Results:We included 28 patients (median age at MRI: 5.2 months). The optimal MRI model included the novel end-diastolic volume index (using the ratio of left ventricular end-diastolic volume to total end-diastolic volume) and the left ventricle–right ventricle angle in diastole (area under the curve 0.83, p = 0.041). End-diastolic volume index ≤ 0.18 and left ventricle–right ventricle angle in diastole ≤ 72° yield a sensitivity of 83% and specificity of 81% for successful biventricular repair. The optimal multimodality model included the end-diastolic volume index and the echocardiographic atrioventricular valve index with an area under the curve of 0.87 (p = 0.026).
Conclusions:Cardiac MRI can successfully predict successful biventricular repair in patients with unbalanced common atrioventricular canal utilising the end-diastolic volume index alone or in combination with the MRI left ventricle–right ventricle angle in diastole or the echocardiographic atrioventricular valve index. A prospective cardiac MRI study is warranted to better define the multimodality characteristic predictive of successful biventricular surgery.
Trivial aortic valve regurgitation in children and adolescents with structurally normal hearts: physiologic or pathologic?
- James J. Joyce, Thomas R. Joyce, Sunita J. Ferns
-
- Published online by Cambridge University Press:
- 31 May 2023, pp. 131-136
-
- Article
-
- You have access Access
- Open access
- HTML
- Export citation
-
Background:
Trivial regurgitation from a normal aortic valve is rarely seen in healthy children and adolescents. The aim of this study was to evaluate timing of presentation, associated conditions, and medium-term prognosis of this incidental finding.
Methods:Paediatric patients observed to have trivial aortic valve regurgitation with otherwise normal echocardiograms were retrospectively analysed. Clinical and echocardiographic parameters were measured and categorised on presentation and follow-up.
Results:Sixty patients (39 males) were identified over a 13-year period. Age at presentation was 14.8 years (IQR 12.9–16.0), height z-score was + 0.71 (95% CI + 0.48– + 0.94), and body mass index z-score was + 0.66 (95% CI + 0.40– + 0.92). Median aortic regurgitation vena contracta diameter was 1.0 mm (IQR 0.8–1.3). Aortic valve strands were visualised in 28% and physiologic mitral regurgitation in 32%. Aortic annulus, sinotubular junction, and mid-ascending aorta diameters were normal, and mean aortic sinus diameter was only slightly increased (z-score + 0.23, 95% CI + 0.02– + 0.44). Follow-up data were obtained in 36 patients from 1 to 6.7 years later (median 2.1). Aortic regurgitation was no longer detectable in 28%, and none exhibited worsening. Mitral regurgitation prevalence was lower in those with aortic regurgitation resolution versus persistence (10% versus 50%, p = 0.03).
Conclusions:Trivial aortic regurgitation in paediatric patients with normal hearts is more common in adolescents and is associated with an increased prevalence of aortic valve strands and physiologic mitral regurgitation. These findings do not worsen during growth and may resolve consistent with being physiologic rather than pathologic.
Differentiating primary sarcomeric hypertrophic cardiomyopathy from Noonan syndrome: can the electrocardiogram be of use?
- Robert W. L. Hauptmeijer, Lea Lippert, Floris E. A. Udink ten Cate, Zina Fejzic, Erika Leenders, Cordula M. Wolf, Jos M. T. Draaisma
-
- Published online by Cambridge University Press:
- 31 August 2023, pp. 597-603
-
- Article
-
- You have access Access
- Open access
- HTML
- Export citation
-
Noonan syndrome is a multi-system genetic disorder and patients may suffer from hypertrophic cardiomyopathy. Previous studies have identified electrocardiographic features that may support a diagnosis of Noonan syndrome. In this two-centre retrospective study, we analysed typical Noonan syndrome-related electrocardiographic features in 30 patients with Noonan syndrome with hypertrophic cardiomyopathy and compared these with the electrocardiographic features in 15 children with sarcomeric hypertrophic cardiomyopathy. Typical Noonan syndrome-related electrocardiographic features are a negative aVF, small left precordial R-waves, large right precordial S-waves, and abnormal Q-wave. We also analysed electrocardiographic features of hypertrophic cardiomyopathy: ST-segment abnormalities and T-wave abnormalities. A negative aVF was seen in 83% of patients with Noonan syndrome-related hypertrophic cardiomyopathy in contrast to 27% of patients with primary sarcomeric hypertrophic cardiomyopathy (p < 0.001). An extreme QRS axis in the north-west was seen only in patients with Noonan syndrome-related hypertrophic cardiomyopathy. This QRS axis deviation is likely to be determined by the Noonan syndrome-related hypertrophic cardiomyopathy and not by the type of hypertrophic cardiomyopathy. There were no differences between the two groups in the frequency of large right precordial S-waves and small R-waves in the left precordial leads V5 and V6. However, an abnormal R/S ratio was more often seen in patients with Noonan syndrome-related hypertrophic cardiomyopathy (p < 0.001). Pathologic Q-waves were seen statistically more frequently in patients with sarcomeric hypertrophic cardiomyopathy (p = 0.009). The occurrence of ST-segment and T-wave pathology did not statistically differ between the two groups. Electrography can be of use in differentiating sarcomeric hypertrophic cardiomyopathy from Noonan syndrome-related hypertrophic cardiomyopathy.
Evaluation of preterm infants having bronchopulmonary dysplasia with echocardiography and serum biomarkers
- O. Pamukcu, N. Narin, S. Sunkak, A. Tuncay
-
- Published online by Cambridge University Press:
- 31 May 2023, pp. 137-144
-
- Article
- Export citation
-
Background and objectives:
Pulmonary hypertension is frequent in infants with bronchopulmonary dysplasia. Echocardiography is easy to perform, non-invasive, and recommended by guidelines even though solely it is not enough. Catheterisation is gold standard but invasive, expensive, and not cost effective. Therefore, we aimed to assess to find out the role of biomarkers besides echocardiography in the diagnosis of pulmonary hypertension in preterm with bronchopulmonary dysplasia.
Methods:This study is done during the time period January 2016–2017. The diagnosis of pulmonary hypertension was assessed by echocardiography at 36 weeks later repeated at 3rd and 6th months. We also repeated biomarkers at 3rd and 6th months. The infants born ≤ 28 weeks in Erciyes University hospital who were diagnosed bronchopulmonary dysplasia were included. Infants with genetic syndromes, structural lung, and CHDs were excluded. Patients without bronchopulmonary dysplasia but having pulmonary hypertension due to other reasons and patients having echocardiograms without adequate images were excluded.
Results:At initial, 21/59 patients had bronchopulmonary dysplasia-pulmonary hypertension (Group 1), 21/59 had no bronchopulmonary dysplasia-pulmonary hypertension (Group 2), and 17/59 had bronchopulmonary dysplasia without pulmonary hypertension (Group 3). Systolic pulmonary artery pressure and pulmonary vascular resistance were found high in Group 1 (36 mmHg; p <0.001, 1.25 Woods Unit; p < 0.0017, respectively). Tricuspid annular plane systolic excursion values of Group 1 were low. Median serum kallistatin levels of Group 1 were lower than the other groups (230.5 (114.5–300.5) µg/ml; p < 0.005). During the study period, pulmonary hypertension of 14/21 bronchopulmonary dysplasia-pulmonary hypertension resolved, six patients in Group 3 developed pulmonary hypertension. However, there was no difference in the biomarkers of these six patients.
Conclusion:In the diagnosis and the follow-up of pulmonary hypertension in bronchopulmonary dysplasia patients, besides echocardiography kallistatin, gelsolin, NT-probrain natriuretic peptide, homocysteine, and cystatin-C levels can be used. Further studies were required with large sample sizes.
Cardiovascular remodelling in response to exercise training in patients after the Fontan procedure: a pilot study
- Maria Bano, Tarique Hussain, Mitchel R. Samels, Ryan J. Butts, Richard Kirk, Benjamin D. Levine
-
- Published online by Cambridge University Press:
- 04 September 2023, pp. 604-613
-
- Article
-
- You have access Access
- Open access
- HTML
- Export citation
-
Background:
The cardiovascular adaptations associated with structured exercise training in Fontan patients remain unknown. We hypothesised that short-term training causes cardiac remodelling and parallel improvement in maximal exercise capacity (VO2 max) in these patients.
Methods and Results:Five patients, median age 19.5 (17.6–21.3) years, with a history of Fontan operation meeting inclusion/exclusion criteria, participated in a 3-month training programme designed to improve endurance. Magnetic resonance images for assessment of cardiac function, fibrosis, cardiac output, and liver elastography to assess stiffness were obtained at baseline and after training. Maximal exercise capacity (VO2 max) and cardiac output Qc (effective pulmonary blood flow) at rest and during exercise were measured (C2H2 rebreathing) at the same interval. VO2 max increased from median (IQR) 27.2 (26–28.7) to 29.6 (28.5–32.2) ml/min/kg (p = 0.04). There was an improvement in cardiac output (Qc) during maximal exercise testing from median (IQR) 10.3 (10.1–12.3) to 12.3 (10.9–14.9) l/min, but this change was variable (p = 0.14). Improvement in VO2 max correlated with an increase in ventricular mass (r = 0.95, p = 0.01), and improvement in Quality-of-life inventory (PedsQL) Cardiac scale scores for patient-reported symptoms (r = 0.90, p = 0.03) and cognitive problems (r = 0.89, p = 0.04). The correlation between VO2 max and Qc showed a positive trend but was not significant (r = 0.8, p = 0.08). No adverse cardiac or liver adaptations were noted.
Conclusion:Short-term training improved exercise capacity in this Fontan pilot without any adverse cardiac or liver adaptations. These results warrant further study in a larger population and over a longer duration of time.
Trial Registration Number:NCT03263312, Unique Protocol ID: STU 122016-037; Registration Date: 18 January, 2017.
Atrial septal defect-associated pulmonary hypertension with decompensated heart failure: outcomes after fenestrated device closure
- Ahmed Deniwar, Jason Hernandez, Enrique O. Aregullin, Sana K. Khan, Sihong Huang, Michael T. Finn, Joseph J. Vettukattil
-
- Published online by Cambridge University Press:
- 19 July 2023, pp. 395-400
-
- Article
- Export citation
-
Background:
Up to 90% of adults with untreated atrial septal defect will be symptomatic by 4th decade, and 30-49% will develop heart failure. 8–10% of these patients have pulmonary arterial hypertension with a female predominance regardless of age. We aimed to demonstrate that fenestrated closure can be safely performed in patients with decompensated heart failure and atrial septal defect-associated pulmonary arterial hypertension with improved outcome.
Methods:Transcatheter fenestrated atrial septal defect closures (Occlutech GmbH, Jena, Germany) were performed on a compassionate-use basis in 5 consecutive adult patients with atrial septal defect-associated pulmonary arterial hypertension and severe heart failure with prohibitive surgical mortality risks. Change in systemic oxygen saturation, 6-minute walk test, NYHA class, echocardiographic and haemodynamic parameters were used as parameters of outcome.
Results:All patients were female, mean age 48.8 ± 13.5 years, followed up for a median of 29 months (max 64 months). Significant improvements observed in the 6-minute walk test, and oxygen saturation comparing day 0 time point to all other follow-up time points data (B = 1.32, SE = 0.28, t (22.7) = -4.77, p = 0.0001); and in the haemodynamic data (including pulmonary vascular resistance and pulmonary pressure) (B = –0.60, SE = 0.22, t (40.2) = 2.74, p = .009). All patients showed improved right ventricular size and function along with NYHA class. There were no procedure-related complications.
Conclusion:Fenestrated atrial septal defect closure is feasible in adults with decompensated heart failure and atrial septal defect-associated pulmonary arterial hypertension. It results in sustained haemodynamic and functional improvement
Contemporary transesophageal echocardiography practice patterns among paediatric cardiology centres in the United States and Canada
- Hunter C. Wilson, M. Eric Ferguson, William L. Border, Ritu Sachdeva
-
- Published online by Cambridge University Press:
- 31 October 2023, pp. 846-853
-
- Article
-
- You have access Access
- Open access
- HTML
- Export citation
-
Objective:
To characterise transesophageal echocardiography practice patterns among paediatric cardiac surgical centres in the United States and Canada.
Methods:A 42-question survey was sent to 80 echocardiography laboratory directors at paediatric cardiology centres with surgical programmes in the United States and Canada. Question domains included transesophageal echocardiography centre characteristics, performance and reporting, equipment use, trainee participation, and quality assurance.
Results:Fifty of the 80 centres (62.5%) responded to the survey. Most settings were academic (86.0%) with 42.0% of centres performing > 350 surgical cases/year. The median number of transesophageal echocardiograms performed/cardiologist/year was 50 (26, 73). Pre-operative transesophageal echocardiography was performed in most surgical cases by 91.7% of centres. Transesophageal echocardiography was always performed by most centres following Norwood, Glenn, and Fontan procedures and by < 10% of centres following coarctation repair. Many centres with a written guideline allowed transesophageal echocardiography transducer use at weights below manufacturer recommendations (50.0 and 61.1% for neonatal and paediatric transducers, respectively). Most centres (36/37, 97.3%) with categorical fellowships had rotations which included transesophageal echocardiography participation. Large surgical centres (>350 cases/year) had higher median number of transesophageal echocardiograms/cardiologist/year (75.5 [53, 86] versus 35 [20, 52], p < 0.001) and more frequently used anaesthesia for diagnostic transesophageal echocardiography ≥ 67% of time (100.0 versus 62.1%, p = 0.001).
Conclusions:There is significant variability in transesophageal echocardiography practice patterns and training requirements among paediatric cardiology centres in the United States and Canada. Findings may help inform programmatic decisions regarding transesophageal echocardiography expectations, performance and reporting, equipment use, trainee involvement, and quality assurance.
Neuromental health aspects in adults with CHD after cardiopulmonary bypass intervention during childhood
- Julia Remmele, Milka Pringsheim, Nicole Nagdyman, Renate Oberhoffer-Fritz, Peter Ewert
-
- Published online by Cambridge University Press:
- 31 May 2023, pp. 145-150
-
- Article
-
- You have access Access
- Open access
- HTML
- Export citation
-
Objective:
It is often assumed, that adult patients with CHD (ACHD) have impairments regarding their cognitive function (CF) and health-related quality of life. In particular, it seems reasonable to assume that cyanosis may have a potential impact on CF as well as surgical or drug treatment into adulthood. This study assesses neuromental health aspects such as CF and health-related quality of life in ACHD patients.
Methods:Seventy-eight ACHD patients (female n = 39 (50%); 34.1 ± 12.9 years; cyanotic CHD n = 49 (62.8%) with a cyanosis duration of 159.8 ± 196.2 month) who underwent open heart surgery as first intervention were asked to participate during routinely follow-up in 2018. Wechsler Intelligence Scale IV was used for CF and the Short Form 36 Health Survey to assess health-related quality of life.
Results:Intelligence quotient measures showed significant differences comparing never cyanotic and with a cyanotic phase in verbal comprehension (p = 0.013). There was no association of CF with cyanosis duration, number of surgery or catheter, CHD severity, and time of first surgery. The group of early surgery showed significantly better results in physical function (p = 0.040) of health-related quality of life, and in comparison with their assigned reference, both groups showed significantly reduced results in all domains except in bodily pain and mental health. Full-Scale intelligence quotient correlates with physical function of health-related quality of life.
Conclusions:The results show normal CF in ACHD. Health-related quality of life was weak in comparison with the reference. There is a need to improve the well-being of our ACHD with structured programmes, including physical activity programmes. This growing ACHD population should be focused in order of their needs, medical ones on one hand and on the other hand psychosocial matters.
Outcomes of atrioventricular septal defects with and without down syndrome: analysis of the national inpatient database
- Safwat Aly, Ibrahim Qattea, Hasan Othman, Hoang H. Nguyen, Hany Z. Aly
-
- Published online by Cambridge University Press:
- 05 September 2023, pp. 614-623
-
- Article
- Export citation
-
Background:
Controversial data exist about the impact of Down syndrome on outcomes after surgical repair of atrioventricular septal defect.
Aims:(A) assess trends and outcomes of atrioventricular septal defect with and without Down syndrome and (B) determine risk factors associated with adverse outcomes after atrioventricular septal defect repair.
Methods:We queried The National Inpatient Sample using International Classification of Disease codes for patients with atrioventricular septal defect < 1 year of age from 2000 to 2018. Patients’ characteristics, co-morbidities, mortality, and healthcare utilisation were evaluated by comparing those with versus without Down syndrome.
Results:In total, 2,318,706 patients with CHD were examined; of them, 61,101 (2.6%) had atrioventricular septal defect. The incidence of hospitalisation in infants with atrioventricular septal defect ranged from 4.5 to 7.5% of all infants hospitalised with CHD per year. A total of 33,453 (54.7%) patients were associated with Down syndrome. Double outlet right ventricle, coarctation of the aorta, and tetralogy of Fallot were the most commonly associated with CHD in 6.9, 5.7, and 4.3% of patients, respectively. Overall atrioventricular septal defect mortality was 6.3%. Multivariate analysis revealed that prematurity, low birth weight, pulmonary hypertension, and heart block were associated with mortality. Down syndrome was associated with a higher incidence of pulmonary hypertension (4.3 versus 2.8%, p < 0.001), less arrhythmia (6.6 versus 11.2%, p < 0.001), shorter duration for mechanical ventilation, shorter hospital stay, and less perioperative mortality (2.4 versus 11.1%, p < 0.001).
Conclusion:Trends in atrioventricular septal defect hospitalisation had been stable over time. Perioperative mortality in atrioventricular septal defect was associated with prematurity, low birth weight, pulmonary hypertension, heart block, acute kidney injury, and septicaemia. Down syndrome was present in more than half of atrioventricular septal defect patients and was associated with a higher incidence of pulmonary hypertension but less arrhythmia, lower mortality, shorter hospital stay, and less resource utilisation.
Coronary artery dilation in non-hospitalised children with asymptomatic or mild COVID-19
- Nicole Gerber, Christopher Lutrario, Michelle Rosenthal, Shari Platt, Ralf Holzer, Patrick Flynn
-
- Published online by Cambridge University Press:
- 31 October 2023, pp. 854-858
-
- Article
- Export citation
-
Introduction:
Infection with Sars-CoV-2 is known to cause cardiac injury and coronary artery changes in moderate to severe acute COVID-19 and post-acute multisystem inflammatory syndrome in children (MIS-C). However, little is known about the potential for cardiac involvement, in particular coronary artery dilation, in asymptomatic or mild cases of COVID-19.
Methods:A retrospective review of children ≤ 18 years of age with a history of asymptomatic or mild COVID-19 disease who underwent echocardiography after Sars-CoV-2 infection is conducted. Patients were excluded if they had been hospitalised for COVID-19/MIS-C or had a history of cardiac disease that could affect coronary artery dimension. Coronary artery dilation was defined as the Boston Z-score greater than 2.0.
Results:One hundred and fifty-seven patients met inclusion criteria with a mean age of 9.4 years (+/– 5.4 years). Eighty-four (54%) patients were identified as having COVID-19 through positive antibody testing. All patients underwent electrocardiogram and echocardiogram as part of their cardiology evaluation. One hundred and thirty-five (86%) patients had a normal evaluation or only a minor variant on electrocardiogram, while 22 patients had abnormalities on echocardiogram, 4 of which demonstrated coronary artery dilation based on the Boston Z-score.
Conclusions:Much of the literature for post-infectious screening and follow-up focuses on patients with a history of moderate to severe COVID-19 disease, emphasising the need for surveillance for the potential development of myocarditis. In this study, 4 out of 157 (2.5%) children with a history of asymptomatic or mild COVID-19 disease without MIS-C were found to have some degree of coronary artery dilation. The significance of this finding currently remains unknown.
Natural history of cardiac findings in mucopolysaccharidosis type I: report from an international registry
- Elizabeth Braunlin, Luisa Bay, Nathalie Guffon, Meng Yang, Nicolas Pangaud, Lorne A. Clarke
-
- Published online by Cambridge University Press:
- 18 October 2023, pp. 401-411
-
- Article
-
- You have access Access
- Open access
- HTML
- Export citation
-
Mucopolysaccharidosis type I is an inborn error of glycosaminoglycan catabolism with phenotypes ranging from severe (Hurler syndrome) to attenuated (Hurler–Scheie and Scheie syndromes). Cardiovascular involvement is common and contributes significantly to morbidity and mortality. We conducted a retrospective analysis of the prevalence and natural history of cardiac abnormalities in treatment-naïve individuals enrolled in the international Mucopolysaccharidosis Type I Registry. Interrogation of echocardiography data (presence of cardiac valve regurgitation and/or stenosis; measurements of left ventricular chamber dimensions in diastole and systole, diastolic left ventricular posterior wall and interventricular septal thicknesses and ventricular systolic function (shortening fraction)) showed that mitral regurgitation was the most common and earliest finding for individuals with both severe (58.3%, median age 1.2 years) and attenuated (74.2%, median age 8.0 years) disease. Left-sided valve stenosis was also common in individuals with attenuated disease (mitral 30.3%; aortic 25%). Abnormal ventricular wall and septal thickness (Z-scores ≥2) were observed early in both phenotypes. Z-scores for diastolic left ventricular posterior wall and interventricular septal thicknesses increased with age in the severe phenotype (annualised slopes of 0.2777 [p = 0.037] and 0.3831 [p = 0.001], respectively); a similar correlation was not observed in the attenuated phenotype (annualised slopes of −0.0401 [p = 0.069] and −0.0029 [p = 0.875], respectively). Decreased cardiac ventricular systolic function (defined as shortening fraction <28%) was uncommon but, when noted, was more frequent in infants with the severe phenotype. While cardiac abnormalities occur early in both severe and attenuated mucopolysaccharidosis type I, the pattern of valve dysfunction and progression of ventricular abnormalities vary by phenotype.
Influence of improved antenatal detection on the outcomes of complete atrioventricular block diagnosed in fetal-neonatal life and childhood periods – a single-centre experience in South Wales for 55 years
- Derya Duman, Gulhan Tunca Sahin, Graham Stuart, Mark Walsh, Massimo Caputo, Andrew Parry, Bryan Beattie, Christine Conner, Orhan Uzun
-
- Published online by Cambridge University Press:
- 27 December 2023, pp. 412-420
-
- Article
- Export citation
-
Objective:
This study aimed to analyse the influence of improved antenatal detection on the course, contemporary outcomes, and mortality risk factors of the complete atrioventricular block during fetal-neonatal and childhood periods in South Wales.
Methods:The clinical characteristics and outcomes of complete atrioventricular block in patients without structural heart disease at the University Hospital of Wales from January 1966 to April 2021 were studied. Patients were divided into two groups according to their age at diagnosis: I-fetal-neonatal and II-childhood. Contemporary outcomes during the post-2001 era were compared with historical data preceding fetal service development and hence earlier detection.
Results:There were 64 patients: 26 were identified in the fetal-neonatal period and the remaining 38 in the childhood period. Maternal antibodies/systemic lupus erythematosus disease (anti-Ro/Sjögren’s-syndrome-related Antigen A and/or anti-La/Sjögren’s-syndrome-related Antigen B) were present in 15 (57.7%) of the fetal-neonatal. Fetal/neonatal and early diagnosis increased after 2001 with an incidence of 1:25000 pregnancies. Pacemaker implantation was required in 34 patients, of whom 13 were diagnosed in the fetal-neonatal group. Survival rates in cases identified before 2001 were at 96.3% (26/27), whereas it was 83.8% (31/37) in patients diagnosed after 2001 (P > 0.05). Other mortality risk factors comprised a lower gestational week at birth, maternal antibodies, and an average ventricular heart rate of < 55 bpm.
Conclusions:Fetal diagnosis of complete atrioventricular block is still portends high fetal and neonatal mortality and morbidity despite significantly improved antenatal detection after 2001. Pacemaker intervention is needed earlier in the fetal-neonatal group. Whether routine antenatal medical treatment might alter this outcome calls for further prospective multicentre studies.
Early assessment of cardiomyopathy in Duchenne patients by means of longitudinal strain echocardiography
- Marcos Clavero-Adell, Ariadna Ayerza-Casas, Daniel Palanca-Arias, Marta López-Ramón, Lorenzo Jiménez-Montañés, Salvador Olmos
-
- Published online by Cambridge University Press:
- 05 June 2023, pp. 151-156
-
- Article
-
- You have access Access
- Open access
- HTML
- Export citation
-
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.
Decrease of left ventricular function measured by speckle-tracking echocardiography based on systemic lupus erythematosus severity in children
- Masita I. Rustam, Reni Ghrahani, Dzulfikar D.L. Hakim, Rahmat B. Kuswiyanto, Mia M. Dewi, Sri E. Rahayuningsih
-
- Published online by Cambridge University Press:
- 08 September 2023, pp. 624-627
-
- Article
-
- You have access Access
- Open access
- HTML
- Export citation
-
Introduction:
Systemic lupus erythematosus in children generally manifests more severely with a more aggressive disease course. Cardiac involvement in systemic lupus erythematosus often does not show specific signs and symptoms, but speckle-tracking echocardiography can detect cardiac dysfunction. This study aimed to determine the differences in left ventricular function as measured by speckle-tracking echocardiography in children with various severity of systemic lupus erythematosus activity.
Methods:A cross-sectional study of 49 children diagnosed with systemic lupus erythematosus are currently undergoing outpatient or inpatient care at Dr Hasan Sadikin General Hospital, Bandung, from May 2023 to June 2023. Disease activity was assessed by Mexican Version of the Systemic Lupus Erythematosus Disease Activity Index (MEX-SLEDAI) with a score of 2–5 classified as mild activity, 6–9 as moderate, and ≥10 as severe. Each subject underwent conventional echocardiography and speckle-tracking echocardiography with a Philips EPIQ machine performed by a Pediatric Cardiologist Consultant 10 days after inclusion.
Results:Fifteen (30.6%) subjects had mild disease activity, and 34 (69.4%) subjects had moderate disease activity. Most subjects (81.96%) were female with an average age of 15 years. The mean ejection fraction and fractional shortening as well as the median E/A ratio in the mild and moderate disease activity groups were not significantly different (65.76 versus 67.38%, 35.73 versus 37.11%, 1.6 versus 1.5%, respectively, p > 0.005). The global longitudinal strain in the moderate activity group was reduced more significantly than in the mild activity group (−16.58 versus −19.65, p = 0.008).
Conclusion:Left ventricular function as measured by speckle-tracking echocardiography was lower in children with moderate systemic lupus erythematosus activity than those with mild disease activity.
Adult congenital heart disease care in a municipal public health system
- Maria Andrea Francesca M. Tinsay, Dan G. Halpern, Jodi L. Feinberg, Matthew Vorsanger, Norma Keller, Adam J. Small
-
- Published online by Cambridge University Press:
- 01 November 2023, pp. 859-864
-
- Article
-
- You have access Access
- Open access
- HTML
- Export citation
-
Specialty care is associated with improved outcomes for adults with adult CHD and must be extended to the underserved. A retrospective cohort study was performed to describe the provision of care to adult CHD patients in America’s largest municipal public health system including patient demographics, diagnostic and therapeutic procedures, and adherence to guideline-recommended surveillance. We identified 229 adult CHD patients aged >18 years through electronic medical records. The most common diagnoses were atrial septal defect, ventricular septal defect, patent ductus arteriosus, and valvular pulmonary stenosis. In total, 65% had moderate or greater anatomic complexity. A large number of patients were uninsured (45%), non-white (96%), and non-English speaking (44%). One hundred forty-six patients (64%) presented with unrepaired primary defects. Fifty eight patients underwent primary repair during the study period; 48 of those repairs were surgical and 10 were transcatheter. Collaboration with an affiliated Comprehensive Care Center was utilised for 28% of patients. A high proportion of patients received adult CHD speciality visits (78%), echocardiograms (66%), and electrocardiograms (56%) at the guideline-recommended frequency throughout the study period. There was no significant difference in the rate of adherence to guideline-recommended surveillance based on insurance status, race/ethnicity, or primary language status. The proportion of patients who had guideline-recommended adult CHD visits, echocardiograms, and electrocardiograms was significantly lower for those with more advanced physiological stages. These results can inform the provision of adult CHD care in other public health system settings.
Piccolo in transcatheter PDA closure multi-centre study from premature to adolescent children
- Osman Baspinar, Nazmi Narin, Ali Baykan, Kaan Yildiz, Ozge Pamukcu, Abdullah Ozyurt, Mehmet Kervancioglu, Yunus Emre Kum, Munevver Tugba Temel, Alper Dogan, Sedat Bagli, Tulay Demircan, Derya Aydin Sahin, Ferhan Elmali, Cem Karadeniz
-
- Published online by Cambridge University Press:
- 07 June 2023, pp. 157-162
-
- Article
- Export citation
-
In this multi-centre study, the mid- to long-term efficacy and safety of the Amplatzer Piccolo Occluder in patent ductus arteriosus closure in premature and term infants as well as children were discussed. Methods. Between 2016 and 2021, 645 patients, 152 of whom were less than 1 month old, underwent ductus closure with the Piccolo device from five different centres in Turkey. The median age of the patients was 2.2 years, and the mean narrowest point of duct diameter was 1.8 mm. Sixty-two patients weighed ≤ 1.5 kg, 90 patients 1.5–3 kg, and the mean follow-up was 20.4 months. In 396, the duct was closed by the retrograde route. Ductal anatomy was Type A in 285, C in 72, E in 171, and F in 64 patients. Fluoroscopy duration was 6.2 min. The procedure success rate was 99.1%. Device embolisation occurred in 13 patients (2%), and 11 were retrieved with a snare. Cardiac perforation and death developed in one premature baby. The left pulmonary artery and the descending aorta stenosis were observed in 3 (0.4%) and in 5 patients (0.5%). Results. Piccolo device is safe and effective in closing ductus in all age groups. It has low profile for use in premature and newborn babies, a small embolisation risk, and a low residual shunt rate after closure. Conclusion. The Piccolo device can be considered as close an ideal occluder. The lower profile, smaller delivery catheter size, and symmetry of this device allow for a venous or arterial approach.
Pulmonary valve replacement in tetralogy of Fallot – who and how?
- Catarina M. Costa, Cristina Cruz, Teresa Pinho, Sofia Torres, João C. Silva, José A. Madureira, Elson Salgueiro, Jorge Casanova, Paulo Pinho, Filipe Macedo
-
- Published online by Cambridge University Press:
- 03 November 2023, pp. 865-869
-
- Article
- Export citation
-
Background and Aim:
Pulmonary regurgitation is the most common complication in repaired tetralogy of Fallot patients. Severe chronic pulmonary regurgitation can be tolerated for decades, but if not treated, it can progress to symptomatic, irreversible right ventricular dilatation and dysfunction. We investigated clinical associations with pulmonary valve replacement among patients with significative pulmonary regurgitation and how interventional developments can change their management.
Methods:All adult patients with repaired tetralogy of Fallot who were followed at an adult CHD Clinic at a single centre from 1980 to 2022 were included on their first outpatient visit. Follow-up was estimated from the time of correction surgery until one of the following events occurred first: pulmonary valve replacement, death, loss to follow-up or conclusion of the study.
Results:We included 221 patients (116 males) with a median age of 19 (18–25). At a median age of 33 (10) years old, 114 (51%) patients presented significant pulmonary regurgitation. Among patients with significant pulmonary regurgitation, pulmonary valve replacement was associated with male gender, older age at surgical repair, and longer QRS duration in adulthood. Pulmonary valve replacement was performed in 50 patients, including four transcatheter pulmonary valve implantations, at a median age of 34 (14) years.
Conclusion:Pulmonary regurgitation affects a large percentage of tetralogy of Fallot adult patients, requiring a long-term clinical and imaging follow-up. Sex, age at surgical repair and longer QRS are associated with the need of PVR among patients with significative pulmonary regurgitation. Clinical practice and current literature support TPVI as the future gold standard intervention.
Utility of a pharmacist-managed Anticoagulation Program in patients with congenital heart disease
- Meredith J. O’Neil, BreAnn N. Garr, Jenna M. Faircloth, Julie A. Ciambarella, Adam M. Lubert, Nicole L. Nelson, David S. Cooper
-
- Published online by Cambridge University Press:
- 08 September 2023, pp. 628-633
-
- Article
- Export citation
-
Background:
Warfarin remains the preferred anticoagulant for many patients with CHD. The complexity of management led our centre to shift from a nurse-physician-managed model with many providers to a pharmacist-managed model with a centralized anticoagulation team. We aim to describe the patient cohort managed by our Anticoagulation Program and evaluate the impact of implementation of this consistent, pharmacist-managed model on time in therapeutic range, an evidence-based marker for clinical outcomes.
Methods:A single-centre retrospective cohort study was conducted to evaluate the impact of the transition to a pharmacist-managed model to improve anticoagulation management at a tertiary pediatric heart centre. The percent time in therapeutic range for a cohort managed by both models was compared using a paired t-test. Patient characteristics and time in therapeutic range of the program were also described.
Results:After implementing the pharmacist-managed model, the time in therapeutic range for a cohort of 58 patients increased from 65.7 to 80.2% (p < .001), and our Anticoagulation Program consistently maintained this improvement from 2013 to 2022. The cohort of patients managed by the Anticoagulation Program in 2022 included 119 patients with a median age of 24 years (range 19 months–69 years) with the most common indication for warfarin being mechanical valve replacement (n = 81, 68%).
Conclusions:Through a practice change incorporating a collaborative, centralized, pharmacist-managed model, this cohort of CHD patients on warfarin had a fifteen percent increase in time in therapeutic range, which was sustained for nine years.
Assessing autonomic activity and prognostic risk factors comparing multisystem inflammatory syndrome and isolated viral myocarditis/myopericarditis
- Derya Duman, Derya Karpuz, Bahar Taşdelen, Necdet Kuyucu
-
- Published online by Cambridge University Press:
- 02 February 2024, pp. 421-427
-
- Article
- Export citation
-
Introduction and aim:
In pediatric multisystem inflammatory syndrome and isolated viral myocarditis/myopericarditis, autonomic nervous system function can be evaluated by a non-invasive method called heart rate variability. This study aims to evaluate heart rate variability in these two groups by comparing them with each other. This is the first study assessing these values in these two groups of patients.
Method:Patients who are diagnosed with multisystem inflammatory syndrome in children and isolated viral myocarditis/myopericarditis at a university hospital from September 2021 to February 2023 are screened by electrocardiography, echocardiography, and 24-hour Holter monitoring. A healthy control group, compatible in age and gender with the patient groups, was selected from healthy subjects that applied to the hospital for palpitation, murmur, and/or chest pain. Heart rate variability parameters and related laboratory markers were analyzed and compared among the three groups.
Results:There were 30 patients with multisystem inflammatory syndrome in children, 43 patients with isolated viral myocarditis/myopericarditis, and 109 participants in the healthy control group. Statistically significant differences were found in most of the heart rate variability parameters: standard deviation of normal to normal intervals (SDNN), the mean of the 5- minute RR interval standard deviations (SDNNİ), the standard deviation of 5-minute R wave to R wave(RR) interval means (SDANN), the root mean square of successive RR interval differences (RMSSD), and the percentage of the beats with a consecutive RR interval difference of more than 50 ms (pNN50%), very low frequency, high frequency, low frequency, triangular index, and low frequency/high-frequency ratio. Multisystem inflammatory syndrome in children patients had impaired and declined heart rate variability values compared to the other two groups. In patients with myocarditis/myopericarditis, we couldn’t find a significant difference in these parameters with the control group.
Conclusion:Heart rate variability can be used as an important non-invasive autonomic function parameter in determining prognosis and treatment plans, especially in patients diagnosed with multisystem inflammatory syndrome in children. This impairment of autonomic activity could be more prominent in patients with decreased left ventricular systolic functions.