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Over the past 2 decades, several categorizations have been proposed for the abnormalities of the aortic root. These schemes have mostly been devoid of input from specialists of congenital cardiac disease. The aim of this review is to provide a classification, from the perspective of these specialists, based on an understanding of normal and abnormal morphogenesis and anatomy, with emphasis placed on the features of clinical and surgical relevance. We contend that the description of the congenitally malformed aortic root is simplified when approached in a fashion that recognizes the normal root to be made up of 3 leaflets, supported by their own sinuses, with the sinuses themselves separated by the interleaflet triangles. The malformed root, usually found in the setting of 3 sinuses, can also be found with 2 sinuses, and very rarely with 4 sinuses. This permits description of trisinuate, bisinuate, and quadrisinuate variants, respectively. This feature then provides the basis for classification of the anatomical and functional number of leaflets present. By offering standardized terms and definitions, we submit that our classification will be suitable for those working in all cardiac specialties, whether pediatric or adult. It is of equal value in the settings of acquired or congenital cardiac disease. Our recommendations will serve to amend and/or add to the existing International Paediatric and Congenital Cardiac Code, along with the Eleventh iteration of the International Classification of Diseases provided by the World Health Organization.
There is variation in care and hospital length of stay following surgical repair of ventricular septal defects. The use of clinical pathways in a variety of paediatric care settings has been shown to reduce practice variability and overall length of stay without increasing the rate of adverse events.
A clinical pathway was created and used to guide care following surgical repair of ventricular septal defects. A retrospective review was done to compare patients two years prior and three years after the pathway was implemented.
There were 23 pre-pathway patients and 25 pathway patients. Demographic characteristics were similar between groups. Univariate analysis demonstrated a significantly shorter time to initiation of enteral intake in the pathway patients (median time to first enteral intake after cardiac ICU admission was 360 minutes in pre-pathway patients and 180 minutes in pathway patients, p < 0.01). Multivariate regression analyses demonstrated that the pathway use was independently associated with a decrease in time to first enteral intake (–203 minutes), hospital length of stay (–23.1 hours), and cardiac ICU length of stay (–20.5 hours). No adverse events were associated with the use of the pathway, including mortality, reintubation rate, acute kidney injury, increased bleeding from chest tube, or readmissions.
The use of the clinical pathway improved time to initiation of enteral intake and decreased length of hospital stay. Surgery-specific pathways may decrease variability in care while also improving quality metrics.
Kawasaki disease is a vasculitis that can lead to cardiac complications, including coronary artery disease and cardiogenic shock. Various scoring systems have been developed to determine those that will be refractory to routine intravenous immunoglobulin therapy or develop coronary artery disease. The objective of this study was to determine if the neutrophil–lymphocyte ratio could predict refractory disease and coronary artery lesions in patients with Kawasaki disease.
A systematic review of the literature was performed to identify manuscripts describing comparisons of neutrophil–lymphocyte ratio between those who had refractory disease and those who did not, and between those who developed coronary artery lesions and those who did not. Mean difference was compared between groups. Areas under the curve were utilised to determine the pooled area under the curve.
12 studies with 5593 patients were included in the final analyses of neutrophil–lymphocyte ratio for the prediction of refractory disease. Neutrophil–lymphocyte ratio before therapy was higher in refractory disease with a mean difference of 2.55 (p < 0.01) and pooled area under the curve of 0.724. Neutrophil–lymphocyte ratio after therapy was higher in refractory disease with a mean difference of 1.42 (p < 0.01) and pooled area under the curve for of 0.803. Five studies with 1690 patients were included in the final analyses of neutrophil–lymphocyte ratio for the prediction of coronary artery lesions. Neutrophil–lymphocyte ratio before therapy was higher in coronary artery lesions with a mean difference of 0.65 (p < 0.01).
The use of neutrophil–lymphocyte ratio may help physicians in the identification of patients at risk of refractory disease and coronary artery lesions in patients with Kawasaki disease.
There are a variety of approaches to biventricular repair in neonates and infants with adequately sized ventricles and left-sided obstruction in the presence of a ventricular septal defect. Those who undergo this in a staged manner initially undergo a Norwood procedure followed by a ventricular septal defect closure such that the neo-aorta is entirely committed to the left ventricle and placement of a right ventricular to pulmonary artery conduit (Yasui operation). This study aimed to determine clinical and haemodynamic factors upon paediatric cardiac ICU admission immediately after the two-stage Yasui operation that was associated with post-operative length of stay.
This was a retrospective review of patients who underwent the Yasui procedure after the initial Norwood operation between 1 January 2011 and 31 December 2020. Patients with complete data on admission were identified and analysed using Bayesian regression analysis.
A total of 15 patients were included. The median age was 9.0 months and post-operative length of stay was 6days. Bayesian regression analysis demonstrated that age, weight, heart rate, mean arterial blood pressure, central venous pressure, pulse oximetry, cerebral near infrared spectroscopy, renal near infrared spectroscopy, pH, pCO2, ionised calcium, and serum lactate were all associated with post-operative length of stay.
Discrete clinical and haemodynamic factors upon paediatric cardiac ICU admission after staged Yasui completion are associated with post-operative length of stay. Clinical target ranges can be developed and seem consistent with the notion that greater systemic oxygen delivery is associated with lower post-operative length of stay.
Transcatheter stenting of the arterial duct is an alternative to surgical systemic to pulmonary artery shunt in neonates with parallel circulation. The current study compares haemodynamic and laboratory values in these patients for the first 48 hours after either intervention.
Neonates with ductal dependent pulmonary blood flow who underwent surgical shunt placement or catheter-based arterial ductal stent placement between January 2013 and January 2022 were identified. Haemodynamic variables included heart rate, blood pressure, near infrared spectroscopy, central venous pressure, vasoactive inotropic score, and arterial saturation. Laboratory variables collected included blood urea nitrogen, serum creatinine, and serum lactate. Variables were collected at baseline, upon post-procedural admission, 6 hours after admission, 12 hours after admission, and 48 hours after admission. Secondary outcomes included post-procedural mechanical ventilation duration, post-procedural hospital length of stay, need for reintervention, need for extracorporeal membrane oxygenation, cardiac arrest, and inpatient mortality.
Of the 52 patients included, 38 (73%) underwent shunt placement while 14 (27%) underwent a stent placement. Heart rates, renal oxygen extraction ratio, and cerebral oxygen extraction ratio were significantly lower in the stent group (p = <0.01, 0.01, and < 0.01, respectively).
Haemoglobin and vasoactive inotropic scores were significantly lower in the stent group (p = <0.01, <0.01, respectively). The stent group had increased risk for cardiac arrest (p = 0.04).
Patients who undergo arterial ductal stent placement have lower heart rates, haemoglobin, renal oxygen extraction ratio, cerebral oxygen extraction ratio, and vasoactive inotropic score in the first 48 hours post-procedure compared to patients with shunt placement.
Tricuspid atresia with common arterial trunk is a very rare association in complex CHD. This association has even more infrequently been documented concomitantly with interrupted aortic arch. We present the diagnosis and initial surgical management of an infant with a fetal diagnosis of tricuspid atresia and common arterial trunk, with additional postnatal finding of interrupted aortic arch with interruption between the left common carotid and left subclavian artery. Due to the infant’s small size, she was initially palliated with bilateral pulmonary artery bands and a ductal stent. This was followed by septation of the common arterial trunk and interrupted aortic arch repair and 4 mm right subclavian artery to main pulmonary artery shunt placement at two months of age. She was discharged home on day of life 81.
The primary objective of this study was to determine whether Altmetric score, number of reads, and citations for paediatric cardiology manuscripts correlate with one another. A secondary objective was to determine the extent to which factors mediated citation number for paediatric cardiology manuscripts.
Data for this study came from manuscripts published in Cardiology in the Young (2010–2021). Data were extracted by using data shared on the journal website. Spearman’s correlation analyses were conducted between manuscript reads, citations, and Altmetric score. Regression analyses were conducted with number of citations as the dependent variable and year of publication, publication type, number of reads, and Altmetric score as independent variables.
A total of 2642 manuscripts were included in the final analyses. Reads and citations had poor correlation (r-value 0.32); reads and Altmetric score had negligible correlation (r-value 0.26); and Altmetric score and citations had negligible correlation (r-value 0.07). Year of publication was independently associated with number of citations (β –0.95, p-value <0.01). Manuscript type was independently associated with number of citations (β 1.04, p-value <0.01). Number of reads was independently associated with citations (β 0.01, p-value <0.01). Altmetric score was independently associated with number of citations (β 0.05, p-value <0.01).
This study describes the correlation of reads, citations, and Altmetric score in manuscripts published in Cardiology in the Young, demonstrating poor correlation, at best, between these metrics. Each bibliometric index seems to represent a different phenomenon of manuscript consumption. No single bibliometric index in isolation offers ample representation of manuscript consumption.
Paediatric residents are often taught cardiac anatomy with two-dimensional images of heart specimens, or via imaging such as echocardiography or computed tomography. This study aimed to determine if the use of a structured, interactive, teaching session using heart specimens with CHD would be effective in teaching the concepts of cardiac anatomy.
The interest amongst paediatric residents of a cardiac anatomy session using heart specimens was assessed initially by circulating a survey. Next, four major cardiac lesions were identified to be of interest: atrial septal defect, ventricular septal defect, tetralogy of Fallot, and transposition. A list of key structures and anatomic concepts for these lesions was developed, and appropriate specimens demonstrating these features were identified by a cardiac morphologist. A structured, interactive, teaching session was then held with the paediatric residents using the cardiac specimens. The same 10-question assessment was administered at the beginning and end of the session.
The initial survey demonstrated that all the paediatric residents had an interest in a cardiac anatomy teaching session. A total of 24 participated in the 2-hour session. The median pre-test score was 45%, compared to a median post-test score of 90% (p < 0.01). All paediatric residents who completed a post-session survey indicated that the session was a good use of educational time and contributed to increasing their knowledge base. They expressed great interest in future sessions.
A 2-hour hands-on cardiac anatomy teaching session using cardiac specimens can successfully highlight key anatomic concepts for paediatric residents.
The effects of alpha-blockade on haemodynamics during and following congenital heart surgery are well documented, but data on patient outcomes, mortality, and hospital charges are limited. The purpose of this study was to characterise the use of alpha-blockade during congenital heart surgery admissions and to determine its association with common clinical outcomes.
Materials and Methods:
A cross-sectional study was conducted using the Pediatric Health Information System database. De-identified data for patients under 18 years of age with a cardiac diagnosis who underwent congenital heart surgery were obtained from 2004 to 2015. Patients were subdivided on the basis of receiving alpha-blockade with either phenoxybenzamine or phentolamine during admission or not. Continuous and categorical variables were analysed using Mann−Whitney U-tests and Fisher exact tests, respectively. Characteristics between subgroups were compared using univariate analysis. Regression analyses were conducted to determine the impact of alpha-blockade on ICU length of stay, hospital length of stay, billed charges, and mortality.
Of the 81,313 admissions, 4309 (5.3%) utilised alpha-blockade. Phentolamine was utilised in 4290 admissions. In univariate analysis, ICU length of stay, total length of stay, inpatient mortality, and billed charges were all significantly higher in the alpha-blockade admissions. However, regression analyses demonstrated that other factors were behind these increased. Alpha-blockade was significantly, independently associated with a 1.5 days reduction in ICU length of stay (p < 0.01) and a 3.5 days reduction in total length of stay (p < 0.01). Alpha-blockade was significantly, independently associated with a reduction in mortality (odds ratio 0.8, 95% confidence interval 0.7−0.9). Alpha-blockade was not independently associated with any significant change in billed charges.
Alpha-blockade is used in a subset of paediatric cardiac surgeries and is independently associated with significant reductions in ICU length of stay, hospital length of stay, and mortality without significantly altering billed charges.
Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code (IPCCC) is now utilized worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11). The most recent publication of the IPCCC was in 2017. This manuscript provides an updated 2021 version of the IPCCC.
The International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD), in collaboration with the World Health Organization (WHO), developed the paediatric and congenital cardiac nomenclature that is now within the eleventh version of the International Classification of Diseases (ICD-11). This unification of IPCCC and ICD-11 is the IPCCC ICD-11 Nomenclature and is the first time that the clinical nomenclature for paediatric and congenital cardiac care and the administrative nomenclature for paediatric and congenital cardiac care are harmonized. The resultant congenital cardiac component of ICD-11 was increased from 29 congenital cardiac codes in ICD-9 and 73 congenital cardiac codes in ICD-10 to 318 codes submitted by ISNPCHD through 2018 for incorporation into ICD-11. After these 318 terms were incorporated into ICD-11 in 2018, the WHO ICD-11 team added an additional 49 terms, some of which are acceptable legacy terms from ICD-10, while others provide greater granularity than the ISNPCHD thought was originally acceptable. Thus, the total number of paediatric and congenital cardiac terms in ICD-11 is 367. In this manuscript, we describe and review the terminology, hierarchy, and definitions of the IPCCC ICD-11 Nomenclature. This article, therefore, presents a global system of nomenclature for paediatric and congenital cardiac care that unifies clinical and administrative nomenclature.
The members of ISNPCHD realize that the nomenclature published in this manuscript will continue to evolve. The version of the IPCCC that was published in 2017 has evolved and changed, and it is now replaced by this 2021 version. In the future, ISNPCHD will again publish updated versions of IPCCC, as IPCCC continues to evolve.
In patients with right ventricular diastolic dysfunction after complete repair of tetralogy of Fallot, some employ the use of beta-blockade. The theoretical benefit of this therapy is felt to be one of the two: 1) reduction in heart rate with subsequent increase in diastolic filling time and stroke volume; 2) halting or reversal of right ventricular remodelling. This study aimed to characterise the use of beta-blockade in paediatric admissions with complete repair of tetralogy of Fallot and characterise the effects of beta-blockade on admission characteristics.
Admissions from 2004 to 2015 in the Pediatric Health Information System database with complete repair of tetralogy of Fallot were identified. Characteristics between admissions with and without beta-blockade were compared by univariate analysis. Next, regression analyses were conducted to determine the independent association of beta-blockade on length of admission, billed charges, cardiac arrest, and inpatient mortality while controlling for demographic variables and comorbidities.
A total of 3594 admissions were included in the final analyses. Of these, 371 employed beta-blockade. Admissions with beta-blockade were more likely to have heart failure and tachyarrhythmias. These admissions also tended to be longer by univariate analysis. Regression analyses demonstrated that beta-blockade was independently associated with a 2.8-day increase in length of stay and no statistically significant change in billed charges, cardiac arrest, or inpatient mortality.
Beta-blockade after complete repair of tetralogy of Fallot is associated with a longer length of stay but did not statistically significantly impact billed charges, cardiac arrest, or inpatient mortality.
Manuscripts pertaining to paediatric cardiology and CHD have been published in a variety of different journals. Some of these journals are journals dedicated to paediatric cardiology, while others are focused on adult cardiology. Historically, it has been considered that manuscripts published in journals devoted to adult cardiology have greater citation potential. Our objective was to compare citation performance between manuscripts related to paediatric cardiology and CHD published in paediatric as opposed to adult cardiology journals.
We identified manuscripts related to paediatric cardiology and CHD published in five journals of interest during 2014. Of these journals, two were primarily concerned with adult cardiology, while the other three focused on paediatric cardiology. The number of citations for these identified manuscripts was gathered from Google Scholar. We compared the number of citations (median, mean, and 25th, 75th, 90th, and 95th percentiles), the potential for citation, and the h-index for the identified manuscripts.
We identified a total of 828 manuscripts related to paediatric cardiology and congenital heart as published in the 5 journals during 2014. Of these, 783 (95%) were published in journals focused on paediatric cardiology, and the remaining 45 (5%) were published in journals focused on adult cardiology. The median number of citations was 41 in the manuscripts published in the journals focused on adult cardiology, as opposed to 7 in journals focused on paediatric cardiology (p < 0.001). The h-index, however, was greater for the journals dedicated to paediatric cardiology (36 versus 27).
Approximately one-twentieth of the work relating to paediatric cardiology and CHD is published in journals that focus predominantly on adult cardiology. The median number of citations is greater when manuscripts concerning paediatric cardiology and CHD are published in these journals focused on adult cardiology. The h-index, however, is higher when the manuscripts are published in journals dedicated to paediatric cardiology. While such publications in journals that focus on adult cardiology tend to generate a greater number of citations than those achieved for works published in specialised paediatric cardiology journals, the potential for citation is no different between the journals. Due to the drastically lower number of manuscripts published in journals dedicated to adult cardiology, however, median performance is different.
We report a case of two-month old with a functionally univentricular heart and parallel circulation who presented to the emergency department with Covid-19 and subsequently developed acute respiratory distress syndrome. The course of illness, clinical values, and laboratory markers are characterized in this report.
Children with congenital heart disease and cardiomyopathy are a unique patient population. Different therapies continue to be introduced with large practice variability and questionable outcomes. The purpose of this study is to determine the impact of various medications on intensive care unit length of stay, total length of stay, billed charges, and mortality for admissions with congenital heart disease and cardiomyopathy.
Materials and methods:
We identified admissions of paediatric patients with cardiomyopathy using the Pediatric Health Information System database. The admissions were then separated into two groups: those with and without inpatient mortality. Univariate analyses were conducted between the groups and the significant variables were entered as independent variables into the regression analyses.
A total of 10,376 admissions were included these analyses. Of these, 904 (8.7%) experienced mortality. Comparing patients who experienced mortality with those who did not, there was increased rate of acute kidney injury with an odds ratio (OR) of 5.0 [95% confidence interval (CI) 4.3 to 5.8, p < 0.01], cardiac arrest with an OR 7.5 (95% CI 6.3 to 9.0, p < 0.01), and heart transplant with an OR 0.3 (95% CI 0.2 to 0.4, p < 0.01). The medical interventions with benefit for all endpoints after multivariate regression analyses in this cohort are methylprednisolone, captopril, enalapril, furosemide, and amlodipine.
Diuretics, steroids, angiotensin-converting enzyme inhibitors, calcium channel blockers, and beta blockers all appear to offer beneficial effects in paediatric cardiomyopathy admission outcomes. Specific agents within each group have varying effects.
A hyperinflammatory response to COVID-19 is being described in children. While this presents, and responds to management, similar to that of Kawasaki Disease it is being coined a new entity. But is it really? We explore how this phenomenon may be Kawasaki Disease with a new trigger.
For CHD patients undergoing corrective surgery utilising cardiopulmonary bypass, post-operative inhaled nitric oxide has been administered to alleviate pulmonary hypertension. We performed a systematic review and meta-analyses to determine the effect of inhaled nitric oxide on haemodynamics, gas exchange, and hospitalisation characteristics in children immediately after cardiopulmonary bypass.
Materials and methods:
A systematic review of the literature was performed to identify full-text manuscripts in English. PubMed, EMBASE, and the Cochrane databases were queried. Once manuscripts were identified for inclusion, a list of all the endpoints in each manuscript was created. Endpoints with data present from two or more studies were then kept for pooled analyses. All endpoints included were continuous variables and so mean and standard deviation were utilised as the effect data for comparison.
A total of eight studies were deemed appropriate for inclusion. There were significant differences with decreases in mean pulmonary artery pressure of −6.82 mmHg, left atrial pressure of −1.16 mmHg, arteriovenous oxygen difference of −1.63, arterial carbon dioxide concentration of −2.41 mmHg, mechanical ventilation duration of −8.56 hours, and length of cardiac ICU stay duration of −0.91 days. All significant variables achieved p < 0.001.
Inhaled nitric oxide in children immediately after cardiopulmonary bypass decreases mean pulmonary artery pressure significantly and decreases the arterial carbon dioxide concentration significantly without significantly altering other haemodynamic parameters. This results in a statistically shorter duration of mechanical ventilation and cardiac ICU length of stay without altering overall hospital length of stay.