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High take-off coronary artery anomaly is a quite rare anomaly which is usually seen in isolated form and diagnosed incidentally. Association with tetralogy of Fallot is also rare and it is not one of the well-known coronary anomalies seen in this disease. Here, we describe high take-off right coronary artery in a 10-month-old female patient with tetralogy of Fallot which was diagnosed during catheter angiography. It is very important to show this anomaly sometimes with additional imaging techniques as it alters all the surgical approach including aortic cannulation.
Left ventricular outflow tract obstruction in patients with pulmonary atresia with intact ventricular septum has been rarely reported. Data are lacking on the impact and management of systemic ventricular outflow tract obstruction that developed following the Fontan procedure. We report a case of an 8-year-old male who developed left ventricular outflow tract obstruction 6 months after the Fontan procedure.
There are a few number of case reports and small-scale case series reporting dilated cardiomyopathy due to vitamin D-deficient rickets. The present study evaluates the clinical, biochemical, and echocardiographic features of neonates with vitamin D deficiency.
Patients and methods:
In this prospective single-arm observational study, echocardiographic evaluation was performed on all patients before vitamin D3 and calcium replacement. Following remission of biochemical features of vitamin D deficiency, control echocardiography was performed. Biochemical and echocardiographic characteristics of the present cohort were compared with those of 27 previously published cases with dilated cardiomyopathy due to vitamin D deficiency.
Results:
The study included 148 cases (95 males). In the echocardiographic evaluation, none of the patients had dilated cardiomyopathy. All of the mothers were also vitamin D deficient and treated accordingly. Comparison of patients with normocalcaemia and hypocalcaemia at presentation revealed no statistically significant difference between the ejection fraction and shortening fraction, while left ventricle end-diastolic diameter and left ventricle end-systolic diameter were higher in patients with hypocalcaemia. Previously published historical cases were older and had more severe biochemical features of vitamin D deficiency.
Conclusion:
To the best of our knowledge, in this first and largest cohort of neonates with vitamin D deficiency, we did not detect dilated cardiomyopathy. Early recognition and detection before developing actual rickets and preventing prolonged hypocalcaemia are critically important to alleviate cardiac complications.
Prior to the recent release of appropriate use criteria for imaging valvulopathies in children, follow-up of valvular lesions, including isolated bicuspid aortic valve, was not standardised. We describe current follow up, treatment, and intervention strategies for isolated bicuspid aortic valve with varying degrees of stenosis, regurgitation, and dilation in children up to 18 years old and compare them with newly released appropriate use criteria.
Methods:
Online survey was sent to members of the American Academy of Pediatrics Section on Cardiology and Cardiac Surgery and PediHeartNet.
Results:
Totally, 106 responses with interpretable data were received. For asymptomatic patients with isolated BAV without stenosis, regurgitation, or dilation follow-up-intervals increased from 7+/−4 months in the newborn period to 28 +/− 14 months at 18 years of age. Respondents recommended more frequent follow-up for younger patients and those with greater disease severity. More than 80% of respondents treat aortic regurgitation or aortic dilation in the setting of bicuspid aortic valve medically. In general, intervention was recommended once stenosis or regurgitation became severe (stenosis of >4 m/s; regurgitation with LV Z score 4) regardless of age, but was not routinely recommended for younger children (newborn – age 6 years) with severe dilation. Exercise was restricted at 38+/−11 mmHg echocardiographic mean gradient.
Conclusions:
Current follow-up, treatment, and intervention strategies for isolated bicuspid aortic valve deviate from appropriate use criteria. Differences between the two highlight the need to better delineate the disease course, clarify recommendations for care, and encourage wider adoption of guidelines.
Coronary abnormalities are frequent in pulmonary atresia and intact ventricular septum, mainly in patients with a very diminutive right ventricle. They severely impact on early and late prognosis. We describe an 8-year-old girl who presented with myocardial ischaemia, late after uneventful Fontan completion. The importance of precise delineation of the coronary anatomy upon initial assessment and during follow-up is emphasised.
DiGeorge syndrome is a rare spectrum of disorder affecting structures derived from third and fourth pharyngeal pouches characterised by aplasia or hypoplasia of thymus and parathyroid glands, and conotruncal anomalies. Presentation includes infants with hypocalcemic seizures, CHD, or recurrent infection. This case report illustrates a unique combination of proximal interruption of right pulmonary artery and aberrant right subclavian artery in a 3-month-old infant who was subsequently diagnosed as DiGeorge syndrome. This constellation of vascular anomalies in an infant with DiGeorge syndrome has not been previously reported in the literature.
Aortic root dilation is a major complication of Marfan syndrome and is one of the most important criteria in establishing the diagnosis. Currently, different echocardiographic nomograms are used to calculate aortic root Z-scores. The aim of the present study was to assess the potential differences in aortic root measurements when aortic root Z-scores were obtained in a cohort of paediatric Marfan patients using several published nomograms.
Methods:
In a cohort of 100 children with Marfan syndrome, Z-scores for aortic root dimensions were calculated according to the nomograms of Pettersen et al, Gautier et al, Colan et al, and Lopez et al. Bland–Altman plots were used to estimate mean differences in Z-scores and to establish limits of agreement.
Results:
The mean Z-score of the sinus of Valsalva for Lopez et al was significantly higher compared to Gautier et al (p < 0.01) and Pettersen et al (p = 0.03). The nomogram of Lopez et al resulted in substantially higher Z-scores in patients with a large sinus of Valsalva diameter. Thirty-five percentage of the studied patients would have a Z-score ≥ 2 using Lopez et al compared to 20% for Pettersen et al, 21% for Gautier et al, and 33% for Colan et al.
Conclusion:
The currently available nomograms for calculating Z-scores of aortic dilation in children with Marfan syndrome lead to clinically relevant differences in Z-scores, especially in children with a relative large aortic root diameter. This could have impact on both the diagnosis and treatment of patients with Marfan syndrome.
Despite enormous strides in our field with respect to patient care, there has been surprisingly limited dialogue on how to train and educate the next generation of congenital cardiologists. This paper reviews the current status of training and evolving developments in medical education pertinent to congenital cardiology. The adoption of competency-based medical education has been lauded as a robust framework for contemporary medical education over the last two decades. However, inconsistencies in frameworks across different jurisdictions remain, and bridging gaps between competency frameworks and clinical practice has proved challenging. Entrustable professional activities have been proposed as a solution, but integration of such activities into busy clinical cardiology practices will present its own challenges. Consequently, this pivot towards a more structured approach to medical education necessitates the widespread availability of appropriately trained medical educationalists, a development that will better inform curriculum development, instructional design, and assessment. Differentiation between superficial and deep learning, the vital role of rich formative feedback and coaching, should guide our trainees to become self-regulated learners, capable of critical reasoning yet retaining an awareness of uncertainty and ambiguity. Furthermore, disruptive innovations such as “technology enhanced learning” may be leveraged to improve education, especially for trainees from low- and middle-income countries. Each of these initiatives will require resources, widespread advocacy and raised awareness, and publication of supporting data, and so it is especially gratifying that Cardiology in the Young has fostered a progressive approach, agreeing to publish one or two articles in each journal issue in this domain.
Platypnea–orthodeoxia syndrome is a rare clinical entity characterised by positional dyspnoea and arterial desaturation while in the upright position, the symptoms generally occurring in adults. We describe a 12-year-old girl diagnosed with platypnea–orthodeoxia syndrome associated with patent foramen ovale. The symptoms resolved following percutaneous patent foramen ovale closure.
A young woman with Turner syndrome was found to have a large coronary aneurysm in the left anterior descending coronary artery upon CT angiogram screening for aortic pathology.
We report a case of a pseudoaneurysm in the sinus of Valsalva, secondary to infective endocarditis in a child with trisomy 21. The patient had a history of subaortic stenosis, bicuspid aortic valve, and ventricular septal defect. Patch closure of the ostium of the pseudoaneurysm and aortic valve replacement was performed. The patient was discharged without severe complications.
We present a rare and challenging case of left ventricular aneurysm in an African child with no history of previous infection or trauma, admitted for surgical treatment, who presented non reversible cardiorespiratory arrest with cardiorespiratory resuscitation before surgery.
There are three cusps in a normally developed aortic valve. Abnormal excavation or fusion, during the embryological development of the aortic valve, results in a varying number of cusps. Bicuspid aortic valve is the most common, but more rarely, unicuspid and quadricuspid aortic valves can be seen.
Here, a case of a 16-year-old male with a unicommissural unicuspid aortic valve and a case of a 13-year-old female with a quadricuspid aortic valve were reported.
Transcatheter closure of ventricular septal defects is considered first-line therapy when anatomically appropriate but is often challenged by proximity to the conduction system in perimembranous defects, or irregular defect shape, especially residual defects that may remain post-operatively. Advancements in device design, however, have allowed for significant improvements in deployment techniques and overall safety. Here we describe the first use of the Lifetech Konar-multifunction™ occluder device in North America, and our specific use of this device to close complex post-operative muscular and a perimembranous-ventricular septal defects in the same patient.
An 11-year-old male was admitted with cough and fever for the last 4 days and also complained of pain in the right lung for some weeks. The boy did not show any other symptoms and his past medical history was unremarkable as well. The radiologist findings showed an aspect that suggested for echinococcosis.
At first, it was realised the heart intervention. About a 2-month period later, the child underwent another cyst removal in lung. He had begun taking albendazole 5 days before the heart intervention. The therapy was continued until the lung intervention and for 12 weeks post-operatively. The patient had an uneventful recovery and after about 4 years.
SARS-CoV-2, which causes the disease COVID-19, generally has a mild disease course in children. However, a severe post-infectious inflammatory process known as multisystem inflammatory syndrome in children has been observed in association with COVID-19. This inflammatory process is a result of an abnormal immune response with similar clinical features to Kawasaki disease. It is well established that multisystem inflammatory syndrome in children is associated with myocardial dysfunction, coronary artery dilation or aneurysms, and occasionally arrhythmias. The most common electrocardiographic abnormalities seen include premature atrial or ventricular ectopy, variable degrees of atrioventricular block, and QTc prolongation, and rarely, haemodynamically significant arrhythmias necessitating extracorporeal membrane oxygenation support. However, presentation with fever, hypotension, and relative bradycardia with a left axis idioventricular rhythm has not been previously reported. We present a case of a young adolescent with multisystem inflammatory syndrome in children with myocarditis and a profoundly inappropriate sinus node response to shock with complete resolution following intravenous immunoglobulin.
The relationship between different surrogates of insulin resistance and left ventricular geometry in obese children is still unclear.
Objective:
We sought to explore the relationship between commonly used measures of insulin sensitivity/resistance (homeostatic model assessment index, serum uric acid, and triglycerides to high-density lipoprotein cholesterol ratio) and left ventricular geometry in normotensive obese children.
Methods:
In this cross-sectional study, 32 normotensive obese children were examined. Transthoracic echocardiography was used to measure left ventricular mass index and relative wall thickness. Homeostasis model assessment index, serum uric acid level, and a ratio of triglycerides to high-density lipoprotein cholesterol were used as markers of the insulin resistance. Simple and partial correlation analyses (to control for the effects of body mass index) were conducted to explore relationship between studied variables and left ventricular mass index or relative wall thickness as outcome variables.
Results:
We found positive correlations between homeostasis model assessment index and relative wall thickness (r = 0.47, p = 0.03) which remained significant after controlling for the effect of body mass index, z-score (r = 0.48, p = 0.03). The cutoff level of homeostasis model assessment index with the optimum sensitivity (Sn) and specificity (Sp) derived from the receiver operating characteristic (ROC) curves for predicting concentric remodelling was ≥5.51 with Sn = 83.33 and Sp = 68.75.
Conclusion:
There is a positive relationship between homeostasis model assessment index and relative wall thickness of obese normotensive children which may help to distinguish at risk obese normotensive children for the development of concentric left ventricular remodelling.
Pulmonary vascular damage may be associated with oxidative stress in congenital heart diseases. We investigated whether small ventricular septal defects have an effect on the pulmonary bed.
Methods:
This prospective cohort study included 100 patients with small ventricular septal defects and 75 healthy controls. Ischemia-modified albumin, high-sensitivity C-reactive protein, and various cardiovascular parameters were assessed in both groups.
Results:
The mean ischemia-modified albumin level was significantly higher in patients with small ventricular septal defects (0.62 ± 0.17 absorbance units) than in the control group (0.51 ± 0.09 absorbance units; p < 0.001). The mean high-sensitivity C-reactive protein level was significantly higher in the ventricular septal defects group (3.72 ± 1.57) than in the control group (2.45 ± 0.89; p < 0.001). The ischemia-modified albumin levels in patients with left ventricular internal diameter end diastole and end sistole and main pulmonary artery z-scores ≥ 2 were significantly higher than patients whose z-scores were <2. The ischemia-modified albumin and high-sensitivity C-reactive protein levels were positively correlated in the small ventricular septal defects group (rho = 0.742, p < 0.001). Receiver operating characteristic analyses showed that at the optimal cut-off value of ischemia-modified albumin for the prediction of pulmonary involvement was 0.55 absorbance units with a sensitivity of 60%, specificity of 62% (area under the curve = 0.690, p < 0.001).
Conclusions:
We demonstrated the presence of oxidative stress and higher ischemia-modified albumin levels in small ventricular septal defects, suggesting that ischemia-modified albumin might be a useful biomarker for evaluating the effects of small ventricular septal defects on the pulmonary bed.
Assess the acute and short-term haemodynamic impact of transcatheter pulmonary valve implantation on left ventricular systolic and diastolic function stratified by pre-transcatheter pulmonary valve implantation physiology.
Background:
Transcatheter pulmonary valve implantation is a widely available option to treat residual or recurrent pulmonary stenosis and pulmonary insufficiency. Transcatheter pulmonary valve implantation acutely increases pulmonary artery size and diastolic pressure in patients with pulmonary insufficiency and acute pulmonary edema has been reported after transcatheter pulmonary valve implantation, possibly related to acute left ventricular volume loading. However, the impact of transcatheter pulmonary valve implantation on left ventricular diastolic function has not been established.
Methods:
Patients who underwent transcatheter pulmonary valve implantation from 2010 to 2017 at our centre were grouped by indication for transcatheter pulmonary valve implantation as pulmonary stenosis, pulmonary insufficiency, or mixed disease. Separate analysis was performed on those who underwent transcatheter pulmonary valve implantation for pulmonary stenosis versus pulmonary insufficiency or mixed disease. Intracardiac haemodynamics immediately before and after transcatheter pulmonary valve implantation and echocardiographic assessment of left ventricular systolic and diastolic function at baseline, 1-day post transcatheter pulmonary valve implantation, and 1-year post transcatheter pulmonary valve implantation were compared between groups.
Results:
In 102 patients who underwent transcatheter pulmonary valve implantation, the indication was pulmonary stenosis in 29 (28%), pulmonary insufficiency in 28 (29%), and mixed disease in 44 (43%). There were no significant differences in left ventricular systolic or diastolic function between groups at baseline, immediately after transcatheter pulmonary valve implantation, or 1-year post implantation. The mean pulmonary artery wedge pressure increased equally across groups.
Conclusions:
While patients with pulmonary insufficiency likely have acute left ventricular volume loading following transcatheter pulmonary valve implantation, this does not appear to be haemodynamically significant as transcatheter pulmonary valve implantation was not associated with measurable changes in left ventricular systolic or diastolic function acutely or 1-year post implantation.
We presented a rare case of traumatic mitral annular avulsion and interventricular septum dissection after an unintentional falling injury in a 5-year-old female child. A successful surgical repair of mitral annulus and interventricular septum was performed to restore the haemodynamic stability.
