Original Article
Effects of blood pressure percentile, body mass index, and race on left ventricular mass in children
- Michelle L. Udine, Jonathan R. Kaltman, Qianxi Li, Jin Liu, Deyu Sun, Man Ching Cheung, Sam Sabouni, Ahmed Al Dulaimi, Craig Sable
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- Published online by Cambridge University Press:
- 06 January 2022, pp. 855-860
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Objective:
To evaluate the association of systolic blood pressure percentile, race, and body mass index with left ventricular hypertrophy on electrocardiogram and echocardiogram to define populations at risk.
Study design:This is a retrospective cross-sectional study design utilising a data analytics tool (Tableau) combining electrocardiogram and echocardiogram databases from 2003 to 2020. Customized queries identified patients aged 2–18 years who had an outpatient electrocardiogram and echocardiogram on the same date with available systolic blood pressure and body measurements. Cases with CHD, cardiomyopathy, or arrhythmia diagnoses were excluded. Echocardiograms with left ventricle mass (indexed to height2.7) were included. The main outcome was left ventricular hypertrophy on echocardiogram defined as Left ventricle mass index greater than the 95th percentile for age.
Results:In a cohort of 13,539 patients, 6.7% of studies had left ventricular hypertrophy on echocardiogram. Systolic blood pressure percentile >90% has a sensitivity of 35% and specificity of 82% for left ventricular hypertrophy on echocardiogram. Left ventricular hypertrophy on electrocardiogram was a poor predictor of left ventricular hypertrophy on echocardiogram (9% sensitivity and 92% specificity). African American race (OR 1.31, 95% CI = 1.10, 1.56, p = 0.002), systolic blood pressure percentile >95% (OR = 1.60, 95% CI = 1.34, 1.93, p < 0.001), and higher body mass index (OR = 7.22, 95% CI = 6.23, 8.36, p < 0.001) were independently associated with left ventricular hypertrophy on echocardiogram.
Conclusions:African American race, obesity, and hypertension on outpatient blood pressure measurements are independent risk factors for left ventricular hypertrophy in children. Electrocardiogram has little utility in the screening for left ventricular hypertrophy.
Vitamin D, liver-related biomarkers, and distribution of fat and lean mass in young patients with Fontan circulation
- Lena Hansson, Camilla Sandberg, Inger Öhlund, Torbjörn Lind, Magne Sthen Bergdahl, Urban Wiklund, Eva Rylander Hedlund, Gunnar Sjöberg, Annika Rydberg
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- Published online by Cambridge University Press:
- 02 August 2021, pp. 861-868
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Introduction/aim:
Young patients with Fontan circulation may have low serum 25-hydroxyvitamin D levels, an affected liver, and unhealthy body compositions. This study aimed to explore the association between vitamin D intake/levels, liver biomarkers, and body composition in young Fontan patients.
Method:We collected prospective data in 2017 to 2018, obtained with food-frequency questionnaires, biochemical analyses of liver biomarkers, and dual-energy X-ray absorptiometry scans in 44 children with Fontan circulation. Body compositions were compared to matched controls (n = 38). Linear regression analyses were used to investigate associations of biomarkers, leg pain, and lean mass on serum levels of 25-hydroxyvitamin D. Biomarkers were converted to z scores and differences were evaluated within the Fontan patients.
Results:Our Fontan patients had a daily mean vitamin D intake of 9.9 µg and a mean serum 25-hydroxyvitamin D of 56 nmol/L. These factors were not associated with fat or lean mass, leg pain, or biomarkers of liver status. The Fontan patients had significantly less lean mass, but higher fat mass than controls. Male adolescents with Fontan circulation had a greater mean abdominal fat mass than male controls and higher cholesterol levels than females with Fontan circulation.
Conclusion:Vitamin D intake and serum levels were not associated with body composition or liver biomarkers in the Fontan group, but the Fontan group had lower lean mass and higher fat mass than controls. The more pronounced abdominal fat mass in male adolescents with Fontan circulation might increase metabolic risks later in life.
The effect of surgical technique, age, and Trisomy 21 on early outcome of surgical management of complete atrioventricular canal defect
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- Sherief Azzab, Ahmed Samy, Hamdy Singab, Mohamed Zeinah, Gledisa Musollari, Ariana Axiaq, Amer Harky, Ahmed Tarek, Mohamed El Ghanam
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- Published online by Cambridge University Press:
- 05 August 2021, pp. 869-873
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Background:
The optimal timing, surgical technique, and the influence of Trisomy 21 on the outcome of surgical repair of Complete Atrioventricular Canal Defect remains uncertain. We reviewed our experience in the repair of CAVC to identify the influence of these factors on operative outcomes.
Methods:A prospective study included 70 patients, who underwent repair of CAVC at our institute between July, 2016 and October, 2019. Primary endpoint was mortality and the secondary endpoint was a degree of left atrioventricular valve regurgitation.
Results:No significant difference was noted between patients operated on, at the first 6 months of age versus later, regarding mortality or LAVV regurgitation. Surgical repair by modified single-patch technique showed a significant reduction in bypass time (71.13 ± 13.507 min versus 99.19 ± 27.092 min, p-value = 0.001). Compared to closure of cleft only, posterior annuloplasty used for repair of LAVV resulted in significant reduction in the occurrence of post-operative valve regurgitation during the early period (LAVV 2 + 43 versus 7 %, p-value = 0.03) and at 6 months of follow-up (LAVV 2 + 35.4 versus 0 %, p-value = 0.01), respectively.
Conclusions:Early intervention, in the first 6 months in patients with CAVC by surgical repair gives comparable acceptable results to later repair; Trisomy 21 was not found to be a risk factor for early intervention. Repair of common AV valve by cleft closure with posterior LAVV annuloplasty showed better results with a significant decrease in post-operative LAVV regurgitation and early mortality in comparison to the closure of cleft only.
Quality of life and emotional vulnerability in a national cohort of adolescents living with Fontan circulation
- Inger Bygland Grosch, Brith Andresen, Lien My Diep, Trond H. Diseth, Thomas Möller
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- Published online by Cambridge University Press:
- 13 August 2021, pp. 874-882
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Introduction:
To investigate quality of life and mental health after Fontan completion, we aimed to characterise outcomes in a representative group of adolescent patients. The study was part of the pre-transition clinical work-up in adolescents with Fontan-type palliation of univentricular CHD. The programme covers the entire paediatric Fontan patient population in Norway.
Methods:Our cross-sectional study included 42 adolescents with Fontan circulation aged 15–18. We recruited a control group of 29 healthy peers. Quality of life was measured by the Pediatric Quality of Life Inventory Questionnaire, while mental health was assessed with the Strength and Difficulties Questionnaire.
Results:Fontan patients scored lower than healthy controls on the Pediatric Quality of Life Inventory total (p = 0.004), the physical (p < 0.001) and social (p = 0.001) functioning subscale, and the Strength and Difficulties Questionnaire subscale of emotional symptoms (p = 0.035). Compared to two of the healthy teens (7%), seven patients (16%) in the Fontan group scored as having impaired mental health (p = 0.224). The female/male ratio for individuals with impaired health was 7:2 (p = 0.003).
Conclusions:Compared to healthy controls, adolescents after Fontan-type palliation in Norway have good health-related quality of life and mental health, despite having slightly lower score than healthy individuals, mainly in physical domains and school functioning. Compared to healthy controls and healthy teenagers, these adolescents have somewhat more emotional problems, and compared to male patients, female patients more often have impaired mental health.
Preoperative monocytosis is predictable at diagnosis for RT-PCR negative COVİD-19 paediatric cardiac surgery patients
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- Ergin Arslanoğlu, Mehmet Emirhan Işık, Kenan Abdurrahman Kara, Fatih Yigit, Nihat Çine, Eylem Tunçer, Hakan Ceyran
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- Published online by Cambridge University Press:
- 09 August 2021, pp. 883-887
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Other markers investigated in this population have gained importance in the diagnosis of the disease since the course of COVID-19 disease is atypical in the paediatric population and PCR may be misleading. The leukocyte profile is one of these biochemical tests. Children did not have lymphopenia in hemogram count, whereas relatively neutropenia and monocytosis were detected, unlike the adult population. The reason why children do not have lymphopenia is thought to be due to the fact that the thymus is more active in the first years of life.
Two-hundred and four patients operated in our paediatric cardiac surgery clinic from 11March, 2020 to 1 April, 2021 were retrospectively examined and 11 patients with preoperative asymptomatic and PCR (−), but with PCR (+) in the post-operative period (patients with incubation period or false PCR negativity) were included in our study. Patients requiring emergency operation and operated from PCR (+) patients in the preoperative period were excluded from the study.
The neutrophil ratio in the lymphocytic series of 7 patients out of 11 patients was slightly below the normal range in the preoperative period, the lymphocyte ratio of 3 patients was slightly above the normal range, and the relative monocyte ratio of 10 patients was slightly above the normal range.
We think that evaluating the leukocyte profile combined with RT-PCR will give more accurate results in the diagnosis of incubation period and false RT-PCR negative patients. In addition, we believe that the algorithms for non-complex paediatric cardiac surgery procedures and timing in the paediatric population with a better course of COVID-19 disease with a positive post-operative course.
Evaluation of arterial stiffness and central blood pressure by oscillometric method in normotensive offspring of hypertensive parents
- Cansu Sivrikaya Yildirim, Pelin Kosger, Tugcem Akin, Birsen Ucar
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- Published online by Cambridge University Press:
- 13 August 2021, pp. 888-895
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Children with a family history of hypertension have higher blood pressure and hypertensive pathophysiological changes begin before clinical findings. Here, the presence of arterial stiffness was investigated using central blood pressure measurement and pulse wave analysis in normotensive children with at least one parent with essential hypertension. Twenty-four-hour ambulatory pulse wave analysis monitoring was performed by oscillometric method in a study group of 112 normotensive children of hypertensive parents aged between 7 and 18 comparing with a control group of 101 age- and gender-matched normotensive children of normotensive parents. Pulse wave velocity, central systolic and diastolic blood pressure, systolic, diastolic and mean arterial blood pressure values were higher in the study group than the control group (p < 0.001, p = 0.002, p = 0.008, p = 0.001, p = 0.005, p = 0.001, p = 0.001, respectively). In all age groups (7–10, 11–14, and 15–18 years), pulse wave velocity was higher in the study group than the control group (p < 0.001). Pulse wave velocity was higher in children whose both parents are hypertensive compared to the children whose only mothers are hypertensive (p = 0.011). Pulse wave velocity values were positively correlated with age, weight, height, and body mass index (p < 0.05). Higher pulse wave velocity, central systolic and diastolic blood pressure values detected in the study group can be considered as early signs of hypertensive vascular changes. Pulse wave analysis can be a reliable, non-invasive, and reproducible method that can allow taking necessary precautions regarding lifestyle to prevent disease and target organ damage by detecting early hypertensive changes in genetically risky children.
Parents’ decision-making for their foetus or neonate with a severe congenital heart defect
- Rebecca K. Delaney, Nelangi M. Pinto, Elissa M. Ozanne, Heather Brown, Louisa A. Stark, Melissa H. Watt, Michelle Karasawa, Angira Patel, Mary T. Donofrio, Michelle M. Steltzer, Stephen G. Miller, Susan L. Zickmund, Angela Fagerlin
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- Published online by Cambridge University Press:
- 19 August 2021, pp. 896-903
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Background:
Parents who receive a diagnosis of a severe, life-threatening CHD for their foetus or neonate face a complex and stressful decision between termination, palliative care, or surgery. Understanding how parents make this initial treatment decision is critical for developing interventions to improve counselling for these families.
Methods:We conducted focus groups in four academic medical centres across the United States of America with a purposive sample of parents who chose termination, palliative care, or surgery for their foetus or neonate diagnosed with severe CHD.
Results:Ten focus groups were conducted with 56 parents (Mage = 34 years; 80% female; 89% White). Results were constructed around three domains: decision-making approaches; values and beliefs; and decision-making challenges. Parents discussed varying approaches to making the decision, ranging from relying on their “gut feeling” to desiring statistics and probabilities. Religious and spiritual beliefs often guided the decision to not terminate the pregnancy. Quality of life was an important consideration, including how each option would impact the child (e.g., pain or discomfort, cognitive and physical abilities) and their family (e.g., care for other children, marriage, and career). Parents reported inconsistent communication of options by clinicians and challenges related to time constraints for making a decision and difficulty in processing information when distressed.
Conclusion:This study offers important insights that can be used to design interventions to improve decision support and family-centred care in clinical practice.
Post-operative kinetics of C-reactive protein to distinguish between bacterial infection and systemic inflammation in infants after cardiopulmonary bypass surgery: the early and the late period
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- Hanna Renk, David Grosse, Sarah Schober, Christian Schlensak, Michael Hofbeck, Felix Neunhoeffer
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- 09 August 2021, pp. 904-911
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Objectives:
Differentiation between post-operative inflammation and bacterial infection remains an important issue in infants following congenital heart surgery. We primarily assessed kinetics and predictive value of C-reactive protein for bacterial infection in the early (days 0–4) and late (days 5–28) period after cardiopulmonary bypass surgery. Secondary objectives were frequency, type, and timing of post-operative infection related to the risk adjustment for congenital heart surgery score.
Methods:This 3-year single-centre retrospective cohort study in a paediatric cardiac ICU analysed 191 infants accounting for 235 episodes of CPBP surgery. Primary outcome was kinetics of CRP in the first 28 days after CPBP surgery in infected and non-infected patients.
Results:We observed 22 infectious episodes in the early and 34 in the late post-operative period. CRP kinetics in the early post-operative period did not accurately differentiate between infected and non-infected patients. In the late post-operative period, infected infants displayed significantly higher CRP values with a median of 7.91 (1.64–22.02) and 6.92 mg/dl (1.92–19.65) on days 2 and 3 compared to 4.02 (1.99–15.9) and 3.72 mg/dl (1.08–9.72) in the non-infection group. Combining CRP on days 2 and 3 after suspicion of infection revealed a cut-off of 9.47 mg/L with an acceptable predictive accuracy of 76%.
Conclusions:In neonates and infants, CRP kinetics is not useful to predict infection in the first 72 hours after CPBP surgery due to the inflammatory response. However, in the late post-operative period, CRP is a valuable adjunctive diagnostic test in conjunction with clinical presentation and microbiological diagnostics.
Combining patient-specific, digital 3D models with tele-education for adolescents with CHD
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- David Liddle, Sheri Balsara, Karin Hamann, Adam Christopher, Laura Olivieri, Yue-Hin Loke
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- 16 August 2021, pp. 912-917
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Introduction:
Adolescents with CHD require transition to specialised adult-centred care. Previous studies have shown that adolescents’ knowledge of their medical condition is correlated with transition readiness. Three-dimensional printed models of CHD have been used to educate medical trainees and patients, although no studies have focused on adolescents with CHD. This study investigates the feasibility of combining patient-specific, digital 3D heart models with tele-education interventions to improve the medical knowledge of adolescents with CHD.
Methods:Adolescent patients with CHD, aged between 13 and 18 years old, were enrolled and scheduled for a tele-education session. Patient-specific digital 3D heart models were created using images from clinically indicated cardiac magnetic resonance studies. The tele-education session was performed using commercially available, web-conferencing software (Zoom, Zoom Video Communications Inc.) and a customised software (Cardiac Review 3D, Indicated Inc.) incorporating an interactive display of the digital 3D heart model. Medical knowledge was assessed using pre- and post-session questionnaires that were scored by independent reviewers.
Results:Twenty-two adolescents completed the study. The average age of patients was 16 years old (standard deviation 1.5 years) and 56% of patients identified as female. Patients had a variety of cardiac defects, including tetralogy of Fallot, transposition of great arteries, and coarctation of aorta. Post-intervention, adolescents’ medical knowledge of their cardiac defects and cardiac surgeries improved compared to pre-intervention (p < 0.01).
Conclusions:Combining patient-specific, digital 3D heart models with tele-education sessions can improve adolescents’ medical knowledge and may assist with transition to adult-centred care.
Incidence and predictors of epilepsy in children with congenital heart disease
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- Carlos Castillo-Pinto, Jessica L. Carpenter, Mary T. Donofrio, Anqing Zhang, Gil Wernovsky, Pranava Sinha, Dana Harrar
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- Published online by Cambridge University Press:
- 09 August 2021, pp. 918-924
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Objective:
Children with CHD may be at increased risk for epilepsy. While the incidence of perioperative seizures after surgical repair of CHD has been well-described, the incidence of epilepsy is less well-defined. We aim to determine the incidence and predictors of epilepsy in patients with CHD.
Methods:Retrospective cohort study of patients with CHD who underwent cardiopulmonary bypass at <2 years of age between January, 2012 and December, 2013 and had at least 2 years of follow-up. Clinical variables were extracted from a cardiac surgery database and hospital records. Seizures were defined as acute if they occurred within 7 days after an inciting event. Epilepsy was defined based on the International League Against Epilepsy criteria.
Results:Two-hundred and twenty-one patients were identified, 157 of whom were included in our analysis. Five patients (3.2%) developed epilepsy. Acute seizures occurred in 12 (7.7%) patients, only one of whom developed epilepsy. Predictors of epilepsy included an earlier gestational age, a lower birth weight, a greater number of cardiac surgeries, a need for extracorporeal membrane oxygenation or a left ventricular assist device, arterial ischaemic stroke, and a longer hospital length of stay.
Conclusions:Epilepsy in children with CHD is rare. The mechanism of epileptogenesis in these patients may be the result of a complex interaction of patient-specific factors, some of which may be present even before surgery. Larger long-term follow-up studies are needed to identify risk factors associated with epilepsy in these patients.
Comparison of myocardial T1 mapping during breath-holding and free-breathing
- Hideharu Oka, Kouichi Nakau, Sadahiro Nakagawa, Yuki Kobayashi, Rina Imanishi, Kunihiro Iwata, Hiroshi Azuma
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- 09 August 2021, pp. 925-929
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Background:
T1 mapping is a recently developed imaging analysis method that allows quantitative assessment of myocardial T1 values obtained using MRI. In children, MRI is performed under free-breathing. Thus, it is important to know the changes in T1 values between free-breathing and breath-holding. This study aimed to compare the myocardial T1 mapping during breath-holding and free-breathing.
Methods:Thirteen patients and eight healthy volunteers underwent cardiac MRI, and T1 values obtained during breath-holding and free-breathing were examined and compared. Statistical differences were determined using the paired t-test.
Results:The mean T1 values during breath-holding were 1211.1 ± 39.0 ms, 1209.7 ± 37.4 ms, and 1228.9 ± 52.5 ms in the basal, mid, and apical regions, respectively, while the mean T1 values during free-breathing were 1165.1 ± 69.0 ms, 1103.7 ± 55.8 ms, and 1112.0 ± 81.5 ms in the basal, mid, and apical regions, respectively. The T1 values were lower during free-breathing than during breath-holding in almost all segments (basal: p = 0.008, mid: p < 0.001, apical: p < 0.001). The mean T1 values in each cross section were 3.1, 7.8, and 7.7% lower during free-breathing than during breath-holding in the basal, mid, and apical regions, respectively.
Conclusions:We found that myocardial T1 values during free-breathing were about 3–8% lower in all cross sections than those during breath-holding. In free-breathing, it may be difficult to assess myocardial T1 values, except in the basal region, because of underestimation; thus, the findings should be interpreted with caution, especially in children.
Liver nodules after the Fontan operation: role of the computerised tomography scan
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- Saviga Sethasathien, Suchaya Silvilairat, Rekwan Sittiwangkul, Krit Makonkawkeyoon, Yupada Pongprot, Surin Woragidpoonpol
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- Published online by Cambridge University Press:
- 09 August 2021, pp. 930-935
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Currently, there is an increasing prevalence of liver nodules in patients following the Fontan operation. The appropriate non-invasive modalities have been applied to assess a diagnosis of Fontan-associated liver disease. The aims of this study were to determine the prevalence and associated factors for the presence of liver nodules using CT scan. A cross-sectional study of 34 patients older than 15 years of age was recruited. Ultrasound upper abdomen, ultrasound liver elastography, and CT scan of the upper abdomen were performed after the Fontan operation. The median age of patients was 20 years (range 14–36 years). The median age at the Fontan operation was 7 years (range 5–17 years) and the duration after the Fontan operation was 12 years (range 4–22 years). The prevalence of liver nodules was 62% as detected by CT scans. Hepatic vein pressure in patients with liver nodules was significantly higher than in those without liver nodules. Hepatic vein pressure above 13 mmHg was a factor associated with liver nodules. There was little agreement between the ultrasound of the upper abdomen and CT scan of the upper abdomen in the evaluation of liver nodules. Hepatic pressure was the only associated factor for the occurrence of liver nodules in patients following the Fontan operation. The prevalence of liver nodules was very high after the Fontan operation. The upper abdomen CT scan should be performed for the surveillance of liver nodules in every Fontan patient over the age of 15 years.
The added value of the electrocardiogram in Noonan syndrome
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- Eefke Vos, Erika Leenders, Sterre R. Werkman, Floris E. A. Udink ten Cate, Jos M. T. Draaisma
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- Published online by Cambridge University Press:
- 12 August 2021, pp. 936-943
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Noonan syndrome is a genetic disorder characteried by short stature, typical facial features, developmental delay, and CHD. In this single-centre retrospective study, we analysed typical Noonan syndrome-related electrocardiographic features in 95 patients with clinically and molecularly confirmed Noonan syndrome. Typical Noonan syndrome-related electrocardiographic features are left axis deviation, small left precordial R-waves, large right precordial S-waves, abnormal Q-wave, and abnormal wide QRS complex. In this representative cohort, CHD was found in 59 patients (62.1%) and typical Noonan syndrome-related electrographic features in 60 patients (63.2%). The typical Noonan syndrome-related electrographic features were also increased over baseline in patients without CHD (41.7%). Of all 95 patients, left axis deviation was seen in 46.3%, small left precordial R-waves in 30.5%, large right precordial S-waves in 5.3%, and abnormal Q-wave and wide QRS complex in 2.1%. There was no significant difference in the frequency of the individual-specific electrographic features between the group with CHD and the group without CHD. However, there were significantly more patients with a small left precordial R-wave in the subgroup with pulmonary stenosis compared to patients without pulmonary stenosis. Conclusion: Specific Noonan syndrome-related electrographic features are frequently present in patients with Noonan syndrome, also in the absence of CHD. These results suggest that there may be a continuum of cardiac anomalies from overt CHD to milder abnormalities that are only seen on electrocardiogram.
Paediatric cardiac rapid response systems: a survey of multicentre practices
- Aarti C. Bavare, Natasha S. Afonso, Kerry A. Sembera, Jason R. Buckley, Tia T. Raymond, Angela McKeta, John M. Costello
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- Published online by Cambridge University Press:
- 19 August 2021, pp. 944-951
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Introduction:
While the efficacy and guidelines for implementation of rapid response systems are well established, limited information exists about rapid response paradigms for paediatric cardiac patients despite their unique pathophysiology.
Methods:With endorsement from the Paediatric Cardiac Intensive Care Society, we designed and implemented a web-based survey of paediatric cardiac and multidisciplinary ICU medical directors in the United States of America and Canada to better understand paediatric cardiac rapid response practices.
Results:Sixty-five (52%) of 125 centres responded. Seventy-one per cent of centres had ∼300 non-ICU beds and 71% had dedicated cardiac ICUs. To respond to cardiac patients, dedicated cardiac rapid response teams were utilised in 29% of all centres (39% and 5% in centres with and without dedicated cardiac ICUs, respectively) [p = 0.006]. Early warning scores were utilised in 62% of centres. Only 31% reported that rapid response teams received specialised training. Transfers to ICU were higher for cardiac (73%) compared to generalised rapid response events (54%). The monitoring and reassessment of patients not transferred to ICU after the rapid response was variable. Cardiac and respiratory arrests outside the ICU were infrequent. Only 29% of centres formally appraise critical deterioration events (need for ventilation and/or inotropes post-rapid response) and 34% perform post-event debriefs.
Conclusion:Paediatric cardiac rapid response practices are variable and dedicated paediatric cardiac rapid response systems are infrequent in the United States of America and Canada. Opportunity exists to delineate best practices for paediatric cardiac rapid response and standardise practices for activation, training, patient monitoring post-rapid response events, and outcomes evaluation.
Impact of Z score system on the management of coronary artery lesions in Kawasaki disease
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- Raymond P. Lorenzoni, Noah Elkins, Morgan Quezada, Ellen J. Silver, Joseph Mahgerefteh, Daphne T. Hsu, Nadine F. Choueiter
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- 08 September 2021, pp. 952-959
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Background:
Coronary artery aneurysms are well-described in Kawasaki disease and the Multisystem Inflammatory Syndrome in Children and are graded using Z scores. Three Z score systems (Boston, Montreal, and DC) are widely used in North America. The recent Pediatric Heart Network Z score system is derived from the largest diverse sample to-date. The impact of Z score system on the rate of coronary dilation and management was assessed in a large real-world dataset.
Methods:Using a combined dataset of patients with acute Kawasaki disease from the Children’s Hospital at Montefiore and the National Heart, Lung, and Blood Institute Kawasaki Disease Study, coronary Z scores and the rate of coronary lesions (Z ≥ 2.0) and aneurysms (Z ≥ 2.5) were determined using four Z score systems. Agreement among Z scores and the effect on Kawasaki management were assessed.
Results:Of 333 patients analysed, 136 were from Montefiore and 197 from the Kawasaki Disease Study. Age, sex, body surface area, and rate of coronary lesions did not differ between the samples. Among the four Z score systems, the rate of acute coronary lesions varied from 24 to 55%. The mean left anterior descending Z scores from Pediatric Heart Network and Boston had a large uniform discrepancy of 1.3. Differences in Z scores among the four systems may change anticoagulation management in up to 22% of a Kawasaki population.
Conclusions:Choice of Z score system alone may impact Kawasaki disease diagnosis and management. Further research is necessary to determine the ideal coronary Z score system.
Peri-operative myocardial performance in infants with Down syndrome undergoing CHD repair
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- Lyudmyla Zakharchenko, Afif EL-Khuffash, Eleanor J. Molloy, Colm Breatnach, Orla Franklin
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- 31 August 2021, pp. 960-968
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Background:
We aimed to characterise the impact of Down syndrome on myocardial performance and loading conditions in infants with Down syndrome and CHD over the peri-operative period by comparing them with infants matched for cardiac lesion with a normal microarray.
Methods:Left ventricular global longitudinal strain, right ventricular free wall longitudinal strain, left ventricular end-systolic wall stress, and right ventricular systolic pressure were measured in the two groups over the peri-operative period.
Results:Fifty-five infants had a diagnosis of Down syndrome and these were compared with 29 control infants. Left ventricular global longitudinal strain decreased in both groups post-operatively with the Down syndrome group demonstrating some recovery pre-discharge (18 ± 3 versus 16 ± 3 %, p = 0.01). Right ventricular longitudinal strain significantly decreased in both groups post-operatively with the control group demonstrating better recovery by hospital discharge (14 ± 4 versus 18 ± 6 %, p < 0.01). End-systolic wall stress was lower and right ventricular systolic pressure was higher in the Down syndrome group throughout the study period (all p < 0.05). Down syndrome was an independent predictor of the duration of ventilation, post-operative use of inotropes, and intensive care stay. Right ventricular longitudinal strain was an independent predictor of duration of intensive care stay.
Conclusion:This study demonstrates the difference between the two groups in relation to left and right ventricular function, particularly prior to discharge, and outlines the additional impact a diagnosis of Down syndrome has on myocardial performance during the peri-operative period.
Risk factors for prolonged ventilation after the modified Fontan procedure
- Masahiro Tsubura, Masaki Osaki, Kensaku Motono, Nao Hamamoto
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- Published online by Cambridge University Press:
- 25 August 2021, pp. 969-974
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Objective:
To investigate the risk factors associated with prolonged ventilation after Fontan surgery.
Design:Retrospective case series.
Setting:Tertiary childrens hospital.
Patients:We included 123 children who underwent Fontan surgery without delayed sternal closure or extracorporeal membrane oxygenation between 2011 and 2017.
Intervention:Fontan surgery.
Measurements and main results:Prolonged ventilation was defined as intubation for more than 24 hours after surgery. Preoperative, intraoperative, and perioperative data were collected retrospectively from medical records. Multivariate logistic regression analysis was used to identify risk factors for prolonged ventilation. The median age and weight of patients were 2.2 years and 10.0 kg, respectively. Seventeen per cent of the patients (n = 21) received prolonged mechanical ventilation, and the median intubation period was 2.9 days. There were no 90-day or in-hospital deaths. The independent predictors of prolonged ventilation identified were fenestration (p < 0.01), low pulmonary artery index (p = 0.02), and advanced atrioventricular regurgitation (p < 0.01). The duration of ICU stay was significantly longer in the prolonged ventilation group than in the early extubation group (10 days versus 6 days, p < 0.01).
Conclusion:Fenestration, low pulmonary artery index, and significant atrioventricular regurgitation are risk factors for prolonged ventilation after Fontan surgery. Careful preoperative and perioperative management that considers the risk factors for prolonged ventilation in each individual is important.
Maternal mental distress and cortisol levels in pregnancies with congenital heart disease
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- Yao Wu, Nickie Andescavage, Catherine Lopez, Jessica Lynn Quistorff, Mary T. Donofrio, Adré J. du Plessis, Catherine Limperopoulos
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- Published online by Cambridge University Press:
- 31 August 2021, pp. 975-979
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Objectives:
Prenatal maternal stress is associated with adverse offspring outcomes, which may be mediated by maternal stress hormones. However, evidence supporting the association between maternal stress and cortisol levels in high-risk pregnancies is limited. This study aims to determine the relationship between self-reported maternal mental distress and maternal salivary cortisol levels in pregnancies complicated by foetal CHD compared with healthy pregnancies.
Methods:We recruited women with pregnancies complicated by foetal CHD and healthy pregnancies. Maternal saliva was collected between 22 and 40 gestational weeks. Standardized questionnaires measuring stress, depression, and anxiety were completed by patients. Generalized estimating equation was used to evaluate associations between maternal mental distress scales and cortisol levels.
Results:We studied 165 women (55 CHD, 110 controls) and collected 504 cortisol samples (160 CHD, 344 controls). Women carrying CHD foetuses had higher stress, anxiety, and depression scores compared to women carrying healthy foetuses. However, maternal cortisol levels did not significantly differ in CHD and controls. Cortisol levels were higher in women carrying foetuses with functionally single-ventricle versus two-ventricle CHD. In both CHD and controls, there was no significant association between maternal stress, depression or anxiety scores and cortisol levels.
Conclusion:Our data suggest that self-reported maternal stress, anxiety, and depression are not associated with maternal salivary cortisol levels in CHD and healthy pregnancies. The impact of maternal mental distress on foetal health may be through other mediating pathways other than maternal cortisol concentrations.
Long-term follow-up of subvalvular aortic stenosis in children: a single-centre experience
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- Mehmet G. Ramoğlu, Selen Karagözlü, Tayfun Uçar, Zeynep Eyileten, Adnan Uysalel, Semra Atalay, Ercan Tutar
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- Published online by Cambridge University Press:
- 29 November 2021, pp. 980-987
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Objective:
The aim of this study is to evaluate clinical and surgical outcomes of children with subaortic stenosis, to determine the risk factors for surgery and reoperation and to compare isolated subaortic stenosis and those concomitant with CHDs.
Methods:The study involved 80 children with subaortic stenosis. The patients were first classified as isolated and CHD group, and the isolated group was further classified as membranous/fibromuscular group. The initial, pre-operative, post-operative and the most recent echocardiographic data, demographic properties and follow-up results of the groups were analysed and compared. The correlation of echocardiographic parameters with surgery and reoperation was evaluated.
Results:There was a significant male predominance in all groups. The frequency of the membranous type was higher than the fibromuscular type in the whole and the CHD group. The median time to the first operation was 4.6 years. Thirty-five (43.7%) patients underwent surgery, 5 of 35 (14%) patients required reoperation. The rate of surgery was similar between groups, but reoperation was significantly higher in the isolated group. The gradient was the most important factor for surgery and reoperation in both groups. In the isolated group besides gradient, mitral-aortic separation was the only echocardiographic parameter correlated with surgery and reoperation.
Conclusion:Reoperation is higher in isolated subaortic stenosis but similar in membranous and fibromuscular types. Early surgery may be beneficial in preventing aortic insufficiency but does not affect the rate of reoperation. Higher initial gradients are associated with adverse outcomes, recurrence and reoperation.
Brief Report
SARS-CoV-2 infection induced thyroid storm and heart failure in an adolescent girl
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- Bibhuti B. Das, Divya Shakti, Jyothsna Akam-Venkata, Obiageli Obi, Michael D. Weiland, William Moskowitz
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- Published online by Cambridge University Press:
- 18 October 2021, pp. 988-992
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We report a case of thyroid storm precipitated by SARS-CoV-2 infection in an adolescent girl with a history of Graves disease and dilated cardiomyopathy. This case highlights that SARS-CoV-2 infection can potentially trigger a thyrotoxicosis crisis and acute decompensated heart failure in a patient with underlying thyroid disease and myocardial dysfunction even in the absence of multi-system inflammatory syndrome in children. We systematically reviewed the thyrotoxicosis cases with SARS-CoV-2 infection and described its impact on pre-existing dilated cardiomyopathy.