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Familial hypercholesterolemia is a genetic disease with plasma total cholesterol especially low-density lipoprotein-cholesterol elevation. In this study, we aimed to examine the changes in the electrocardiographies of children with familial hypercholesterolemia.
Materials and methods:
Electrocardiography of 85 patients with a diagnosis of familial hypercholesterolemia, followed up from the Pediatric Metabolism and Pediatric Cardiology outpatient clinic was examined. Electrocardiography of 83 children from the control group who did not have hypercholesterolemia in a similar gender and age range were examined. Heart rate, P wave, PR interval, P wave dispersion, QRS wave, QT interval, corrected QT (calculated with Bazett formula), Tpeak-end interval, QT dispersion, corrected QT dispersion, JT interval, corrected JT (calculated with Bazett formula) were statistically compared.
P wave, PR interval, and P wave dispersion values were significantly higher (p < 0.05) in the children with familial hypercholesterolemia. Corrected QT, QT dispersion, corrected QT dispersion, JT interval, corrected JT, Tpeak-end interval were significantly higher than the control group (p < 0.05) in children with familial hypercholesterolemia. These statistical differences in electrocardiography parameters support the risk of atrial and/or ventricular arrhythmia in children with familial hypercholesterolemia.
We found that high total cholesterol and low-density lipoprotein-cholesterol variables are associated with an increased risk of cardiac atrial and/or ventricular arrhythmia. The findings suggest that total cholesterol and low-density lipoprotein-cholesterol variability can be used as a new marker for the risk of cardiac arrhythmia. In this case, decreasing total cholesterol and low-density lipoprotein-cholesterol variability below certain thresholds may decrease the risk of cardiac arrhythmia.
This study aimed to evaluate the early outcomes of patients who underwent a concomitant therapeutic maze procedure for congenital heart surgery.
Materials and Methods:
Between 2019 and 2020, eight patients underwent surgical cryoablation by using the same type of cryoablation probe.
Three patients had atrial flutter, two had Wolf–Parkinson–White syndrome, two intra-atrial reentrant tachycardia, and one had atrial fibrillation. Four patients underwent electrophysiological study. Preoperatively, one patient was on 3, two were on 2, five were on 1 antiarrhythmic drug. Six patients underwent right atrial maze and two underwent bilateral atrial maze. Five out of six right atrial maze patients underwent right atrial reduction. Nine different lesion sets were used. Some of the lesions were combined and applied as one lesion. In Ebstein’s anomaly patients, the lesion from coronary sinus to displaced tricuspid annulus was delicately performed. The single ventricle patient with heterotaxy had junctional rhythm at the time of discharge and was the only patient who experienced atrial extrasystoles 2 months after discharge. Seven of the eight patients were on sinus rhythm. No patient needed permanent pacemaker placement.
Cryomaze procedure can be applied in congenital heart diseases with acceptable arrhythmia-free rates by selecting the appropriate materials and suitable lesion sets. The application of cryomaze in heterotaxy patients can be challenging due to differences in the conduction system and complex anatomy. Consensus with the electrophysiology team about the choice of the right–left or biatrial maze procedure is mandatory for operational success.
We present the case of a 10-year-old boy with congenital complete atrioventricular block who had cardiac strangulation by an epicardial pacemaker lead placed during infancy. Coronary angiography and Tc99m tetrofosmin myocardial perfusion scintigraphy suggested sub-clinical myocardial ischaemia.
We present the case of a 12-year-old boy with type 2 long QT syndrome in whom torsades de pointes was induced by an acute face immersion test. This test is feasible to predict cardiac events in adolescents with long QT syndrome.
Cardio-facio-cutaneous syndrome is a genetic anomaly characterised by craniofacial dysmorphia, developmental retardation, skin lesions, mental retardation/learning disability, and cardiac malformations. Cardio-facio-cutaneous syndrome rarely causes arrhythmias and has not been previously associated with long QT in the literature. With this report, it was aimed to draw attention to a different presentation of the long QT syndrome.
Prescribing in cardiology emergencies can be confusing for the new prescriber. With clear, step-by-step instructions for managing the most common presentations, including acute coronary syndromes, acute severe pulmonary oedema and arrhythmias, the authors simplify and improve the accessibility of this subject for all healthcare professionals.
Conventional ambulatory heart rhythm monitoring is limited in its ability to provide rapid diagnosis of arrhythmias in athletes participating in water or high-intensity sports. This case report is of a 17-year-old female competitive swimmer who underwent loop recorder implantation with Confirm Rx™ ICM 3500 (Abbott, Minneapolis, MN) to monitor for arrhythmias during swimming. The purpose of this case report is to describe the utility of implantable loop recorders in arrhythmia diagnosis and symptom evaluation in water sport athletes.
Emergency department (ED) patients with atrial fibrillation or flutter (AFF) with underlying occult condition such as sepsis or heart failure, and who are managed with rate or rhythm control, have poor prognoses. Such conditions may not be easy to identify early in the ED evaluation when critical treatment decisions are made. We sought to develop a simple decision aid to quickly identify undifferentiated ED AFF patients who are at high risk of acute underlying illness.
We collected consecutive ED patients with electrocardiogram-proven AFF over a 1-year period and performed a chart review to ascertain demographics, comorbidities, and investigations. The primary outcome was having an acute underlying illness according to prespecified criteria. We used logistic regression to identify factors associated with the primary outcome, and developed criteria to identify those with an underlying illness at presentation.
Of 1,083 consecutive undifferentiated ED AFF patients, 400 (36.9%) had an acute underlying illness; they were older with more comorbidities. Modeling demonstrated that three predictors (ambulance arrival; chief complaint of chest pain, dyspnea, or weakness; CHA2DS2-VASc score greater than 2) identified 93% of patients with acute underlying illness (95% confidence interval [CI], 91–96%) with 54% (95% CI, 50–58%) specificity. The decision aid missed 28 patients; (7.0%) simple blood tests and chest radiography identified all within an hour of presentation.
In ED patients with undifferentiated AFF, this simple predictive model rapidly differentiates patients at risk of acute underlying illness, who will likely merit investigations before AFF-specific therapy.
Refractory ventricular fibrillation encountered during cardiac arrest has a mortality rate of 97%.1 As per the advanced cardiac life support (ACLS) guidelines, the management algorithm of ventricular fibrillation consists of chest compressions, epinephrine, defibrillation, and anti-arrhythmics.2 There have been reports describing the use of the fast-acting selective β-blocker, esmolol, and dual-sequential defibrillation in the management of ventricular fibrillation that is refractory to standard ACLS. We present a case of a 24-year-old male who had an out-of-hospital cardiac arrest, with refractory ventricular fibrillation despite high-quality cardiopulmonary resuscitation (CPR) and ACLS management. Along with standard ACLS, triple-sequential defibrillation was used to achieve return of spontaneous circulation (ROSC) after 82 minutes of downtime. An electrocardiogram (ECG) after ROSC showed an ST-elevation myocardial infarction (MI), and the patient underwent angiography showing a 100% occlusion of his left anterior descending artery. Following management of his coronary artery disease, he was discharged from the hospital 16 days later and was neurologically intact.
The chapter opens with observations on the normal fetal heart and proceeds to a discussion of fetal hydrops and its associated cardiac causes. There follows a treatment of the syndromes associated with congenital heart disease in the fetus. Common congenital heart defects as they appear in the fetus are illustrated. Fetal cardiomyopathy, myocarditis and arrhythmia all have separate discussions. The chapter closes with a series of discussions on the pathology of twins as it affects the heart: twin–twin transfusion syndrome, conjoined twins and acardiac twins.
The initial classic Fontan utilising a direct right atrial appendage to pulmonary artery anastomosis led to numerous complications. Adults with such complications may benefit from conversion to a total cavo-pulmonary connection, the current standard palliation for children with univentricular hearts.
A single institution, retrospective chart review was conducted for all Fontan conversion procedures performed from July, 1999 through January, 2017. Variables analysed included age, sex, reason for Fontan conversion, age at Fontan conversion, and early mortality or heart transplant within 1 year after Fontan conversion.
A total of 41 Fontan conversion patients were identified. Average age at Fontan conversion was 24.5 ± 9.2 years. Dominant left ventricular physiology was present in 37/41 (90.2%) patients. Right-sided heart failure occurred in 39/41 (95.1%) patients and right atrial dilation was present in 33/41 (80.5%) patients. The most common causes for Fontan conversion included atrial arrhythmia in 37/41 (90.2%), NYHA class II HF or greater in 31/41 (75.6%), ventricular dysfunction in 23/41 (56.1%), and cirrhosis or fibrosis in 7/41 (17.1%) patients. Median post-surgical follow-up was 6.2 ± 4.9 years. Survival rates at 30 days, 1 year, and greater than 1-year post-Fontan conversion were 95.1, 92.7, and 87.8%, respectively. Two patients underwent heart transplant: the first within 1 year of Fontan conversion for heart failure and the second at 5.3 years for liver failure.
Fontan conversion should be considered early when atrial arrhythmias become common rather than waiting for severe heart failure to ensue, and Fontan conversion can be accomplished with an acceptable risk profile.
Aneurysms of the right atrium are rare in the paediatric population. We report a case of a foetal diagnosis of right atrial aneurysm with associated atrial tachycardia in foetal and postnatal life. Unique to our case are the findings of isolated pericardial effusion without hydrops fetalis and the development of aortic coarctation in postnatal life.
Outcome analyses in large administrative databases are ideal for rare diseases such as Becker and Duchenne muscular dystrophy. Unfortunately, Becker and Duchenne do not yet have specific International Classification of Disease-9/-10 codes. We hypothesised that an algorithm could accurately identify these patients within administrative data and improve assessment of cardiovascular morbidity.
Hospital discharges (n=13,189) for patients with muscular dystrophy classified by International Classification of Disease-9 code: 359.1 were identified from the Pediatric Health Information System database. An identification algorithm was created and then validated at three institutions. Multi-variable generalised linear mixed-effects models were used to estimate the associations of length of stay, hospitalisation cost, and 14-day readmission with age, encounter severity, and respiratory disease accounting for clustering within the hospital.
The identification algorithm improved identification of patients with Becker and Duchenne from 55% (code 359.1 alone) to 77%. On bi-variate analysis, left ventricular dysfunction and arrhythmia were associated with increased cost of hospitalisation, length of stay, and mortality (p<0.001). After adjustment, Becker and Duchenne patients with left ventricular dysfunction and arrhythmia had increased length of stay with rate ratio 1.4 and 1.2 (p<0.001 and p=0.004) and increased cost of hospitalization with rate ratio 1.4 and 1.4 (both p<0.001).
Our algorithm accurately identifies patients with Becker and Duchenne and can be used for future analysis of administrative data. Our analysis demonstrates the significant effects of cardiovascular disease on length of stay and hospitalisation cost in patients with Becker and Duchenne. Better recognition of the contribution of cardiovascular disease during hospitalisation with earlier more intensive evaluation and therapy may help improve outcomes in this patient population.
Acute rheumatic fever is the most commonly acquired heart disease in developing countries. The most common cardiac presentation is valvular disease. Although some rhythm disturbances may occur during the acute stages of the disease, ventricular tachycardia is extremely rare. Here, a case of acute rheumatic fever with severe endocarditis involving four valves and ventricular tachycardia is presented.
Steroids are used in the treatment of acute rheumatic fever with moderate-to-severe carditis. Corticosteroids have several cardiovascular side affects that are more common in adults than in children. Corticosteroid-related bradycardia is a rarely seen side effect. Children with bradycardia following oral corticosteroid use are rarely reported previously. We present a child who developed bradycardia after oral corticosteroid treatment and concurrent Wolff–Parkinson–White pattern.
Traditional ambulatory rhythm monitoring in children can have limitations, including cumbersome leads and limited monitoring duration. The ZioTM patch ambulatory monitor is a small, adhesive, single-channel rhythm monitor that can be worn up to 2 weeks. In this study, we present a retrospective cross-sectional analysis of the ZioTM monitor’s impact in clinical practice. Patients aged 0–18 years were included in the study. A total of 373 studies were reviewed in 332 patients. In all, 28.4% had structural heart disease, and 16.9% had a prior surgical, catheterisation, or electrophysiology procedure. The most common indication for monitoring was tachypalpitations (41%); 93.5% of these patients had their symptoms captured during the study window. The median duration of monitoring was 5 days. Overall, 5.1% of ZioTM monitoring identified arrhythmias requiring new intervention or increased medical management; 4.0% identified arrhythmias requiring increased clinical surveillance. The remainder had either normal-variant rhythm or minor rhythm findings requiring no change in management. For patients with tachypalpitations and no structural heart disease, 13.2% had pathological arrhythmias, but 72.9% had normal-variant rhythm during symptoms, allowing discharge from cardiology care. Notably, for patients with findings requiring intervention or increased surveillance, 56% had findings first identified beyond 24 hours, and only 62% were patient-triggered findings. Seven studies (1.9%) were associated with complications or patient intolerance. The ZioTM is a well-tolerated device that may improve what traditional Holter and event monitoring would detect in paediatric cardiology patients. This study shows a positive clinical impact on the management of patients within a paediatric cardiology practice.
SCN5A encodes sodium-channel α-subunit Nav1.5. The mutations of SCN5A can lead to hereditary cardiac arrhythmias such as the long-QT syndrome type 3 and Brugada syndrome. Here we sought to identify novel mutations in a family with arrhythmia.
Genomic DNA was isolated from blood of the proband, who was diagnosed with atrial flutter. Illumina Hiseq 2000 whole-exome sequencing was performed and an arrhythmia-related gene-filtering strategy was used to analyse the pathogenic genes. Sanger sequencing was applied to verify the mutation co-segregated in the family.
Results and conclusions
A novel missense mutation in SCN5A (C335R) was identified, and this mutation co-segregated within the affected family members. This missense mutation was predicted to result in amplitude reduction in peak Na+ current, further leading to channel protein dysfunction. Our study expands the spectrum of SCN5A mutations and contributes to genetic counselling of families with arrhythmia.
Potentially fatal arrhythmias add to the mental health challenges of adolescence. This systematic review sought to summarise current knowledge regarding the mental health of adolescents and pre-adolescents diagnosed with inherited arrhythmia syndromes. Searches combining psychological problems with inherited cardiac arrhythmia diagnoses identified 16 studies with paediatric (<18 years) inherited arrhythmia patients. All studies were cross-sectional; 8/16 required an implantable cardioverter defibrillator. Methods were quantitative (n=11), qualitative (n=4), or mixed (n=1), with 14–100% of participants having an inherited arrhythmia syndrome. Mean/median age in 13/16 studies was 12–16 years. Patients and parents reported lower quality of life, particularly in relation to physical function, social relationships, restriction of peer activities, bodily pain, and mental and emotional health. Self-perceptions and behaviour were similar to healthy populations. Rates of anxiety and depression (15–33% of these patients) were not increased in these studies where patients were assessed 2+ years after diagnosis. Higher mental health risk occurred among patients who have a diagnosed sibling, those with cardiomyopathy, and those who report decreased quality of life. Mental health research among youth with inherited arrhythmias is extremely limited and of low quality. Data, primarily from patients 2–4 years after diagnosis or treatment with an implantable cardioverter defibrillator, indicate that quality of life may be decreased and 15–33% experience mental health issues. Future research is required to examine the mental health and quality of life of paediatric patients with inherited arrhythmia syndromes, whether or not they have an implantable cardioverter defibrillator, from time of diagnosis.