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Middle aortic syndrome is rare cause of secondary hypertension, typically manifesting in childhood or early adolescence. It involves obstructive narrowing of the aorta, often occurring in the distal thoracic and abdominal aorta and its major branches. While the exact cause of middle aortic syndrome is not fully understood, it has been linked to hereditary genetic conditions such as Williams syndrome. This case report describes a 16-year-old female with Williams syndrome who presented with abdominal pain and hematochezia. CT angiography revealed moderate narrowing of the abdominal aorta, consistent with a diagnosis of middle aortic syndrome.
Hyperlactatemia is a common and concerning finding in the paediatric cardiac ICU as it may signify tissue hypoperfusion and/or hypoxia. However, it is important to include other aetiologies for an elevated lactate in the differential, especially when the lactate is significantly elevated (> 8 mmol/L). We present the case of metabolic acidosis with severe hyperlactatemia secondary to Warburg effect and presumed thiamine deficiency in a paediatric heart transplant patient with post-transplant lymphoproliferative disorder.
We conducted an international survey of stroke physicians to assess practices and attitudes toward cardiac monitoring and early rhythm control. A 20-question survey was completed by 241 clinicians representing 61 countries. The minimum duration of actionable atrial fibrillation varied widely, and more than 90% (223/241) of respondents indicated a willingness to enroll patients in a trial assessing the ideal duration of cardiac monitoring. Only a quarter of respondents (62/241) offered early rhythm control for patients with atrial fibrillation, with the majority (209/241, 87%) expressing an opinion that there was equipoise about the benefit of rhythm control in the post-stroke population.
Understanding complex three-dimensional cardiac structures is the key to knowing CHD. Many learners have limited access to cadaveric specimens, and most alternative teaching modalities are two-dimensional. Therefore, we have developed virtual cardiac models using photogrammetry of actual heart specimens to address this educational need.
Methods:
A descriptive study was conducted at a single institution during a week-long cardiac morphology conference in October 2022 and 2023. Conference attendees viewed virtual cardiac models via laptop screen and virtual reality headset. Learners were surveyed on their opinions of the virtual models and their perceived effectiveness compared to existing educational materials.
Results:
Forty-six learners completed the survey. Participants reported the virtual cardiac models to be more effective than textbook diagrams (60%), and equally or more effective compared to didactic teaching (78%) and specimen videos (78%). Approximately half of participants (54%) found the virtual models to be less effective than hands-on cadaveric specimen inspection. Attitudes towards the virtual specimens were overall positive with most responders finding the tool engaging (87%) and enjoyable (85%). A majority reported that the models deepened their understanding of cardiac morphology (79%) and that they would recommend them to other trainees (87%).
Conclusions:
This study demonstrates that a novel teaching tool, virtual cardiac specimens, is equivalent to or more effective than many current materials for learning cardiac morphology. While they may not replace direct cadaveric specimen review, virtual models are an engaging alternative with the ability to reach a wider audience.
This study explored the prospective use of the Ages and Stages Questionnaires-3 in follow-up after cardiac surgery.
Materials and Method:
For children undergoing cardiac surgery at 5 United Kingdom centres, the Ages and Stages Questionnaires-3 were administered 6 months and 2 years later, with an outcome based on pre-defined cut-points: Red = 1 or more domain scores >2 standard deviations below the normative mean, Amber = 1 or more domain scores 1–2 standard deviations below the normal range based on the manual, Green = scores within the normal range based on the manual.
Results:
From a cohort of 554 children <60 months old at surgery, 306 participated in the postoperative assessment: 117 (38.3%) were scored as Green, 57 (18.6%) as Amber, and 132 (43.1%) as Red. Children aged 6 months at first assessment (neonatal surgery) were likely to score Red (113/124, 85.6%) compared to older age groups (n = 32/182, 17.6%). Considering risk factors of congenital heart complexity, univentricular status, congenital comorbidity, and child age in a logistic regression model for the outcome of Ages and Stages score Red, only younger age was significant (p < 0.001). 87 children had surgery in infancy and were reassessed as toddlers. Of these, 43 (49.2%) improved, 30 (34.5%) stayed the same, and 13 (16.1%) worsened. Improved scores were predominantly in those who had a first assessment at 6 months old.
Discussion:
The Ages and Stages Questionnaires results are most challenging to interpret in young babies of 6 months old who are affected by complex CHD.
Pain management for infants undergoing cardiothoracic surgery primarily utilises opioid analgesics. There is a paucity of data available for the use of non-steroidal anti-inflammatory medications such as ketorolac in this patient population.
Materials and Methods:
This retrospective study evaluated patients between 30 days and 6 months undergoing cardiothoracic surgery. The primary endpoint evaluates ketorolac on reducing post-operative opioid use.
Results:
Of 243 evaluated patient, 145 met inclusion. Baseline demographics were similar amongst the cohorts. Patients administered ketorolac used less cumulative opiates, in morphine milligram equivalents, for post-op days (POD) 1–3 after surgery compared to patients not receiving ketorolac (9.47 versus 12.68; p = 0.002). The no-ketorolac group required more opiates on POD 1 (10.9 versus 5; p < 0.001) and POD 2 (4.2 versus 2.5; p = 0.006) with no difference found on POD 3 (2 versus 1.6; p = 0.2). There was a mean increase from baseline to highest serum creatinine level on POD 1–3 in the no-ketorolac group compared to the ketorolac group (0.15 versus 0.09 mg/dL; p < 0.014), with no difference in stage 1 or stage 2 acute kidney injury. There were no differences in average chest tube output in mL/kg/day (0.24 versus 0.32; p = 0.569) or need for transfusion (36% versus 24%; p = 0.125), respectively.
Discussion:
Scheduled administration of ketorolac after cardiothoracic surgery resulted in a significant reduction in opioid exposure, with no difference in rates of acute kidney injury or bleeding.
Objective, evidence-based neuroprognostication of postarrest patients is crucial to avoid inappropriate withdrawal of life-sustaining therapies or prolonged, invasive, and costly therapies that could perpetuate suffering when there is no chance of an acceptable recovery. Postarrest prognostication guidelines exist; however, guideline adherence and practice variability are unknown.
Objective:
To investigate Canadian practices and opinions regarding assessment of neurological prognosis in postarrest patients.
Methods:
An anonymous electronic survey was distributed to physicians who care for adult postarrest patients.
Results:
Of the 134 physicians who responded to the survey, 63% had no institutional protocols for neuroprognostication. While the use of targeted temperature management did not affect the timing of neuroprognostication, an increasing number of clinical findings suggestive of a poor prognosis affected the timing of when physicians were comfortable concluding patients had a poor prognosis. Variability existed in what factors clinicians’ thought were confounders. Physicians identified bilaterally absent pupillary light reflexes (85%), bilaterally absent corneal reflexes (80%), and status myoclonus (75%) as useful in determining poor prognosis. Computed tomography, magnetic resonance imaging, and spot electroencephalography were the most useful and accessible tests. Somatosensory evoked potentials were useful, but logistically challenging. Serum biomarkers were unavailable at most centers. Most (79%) physicians agreed ≥2 definitive findings on neurologic exam, electrophysiologic tests, neuroimaging, and/or biomarkers are required to determine a poor prognosis with a high degree of certainty. Distress during the process of neuroprognostication was reported by 70% of physicians and 51% request a second opinion from an external expert.
Conclusion:
Significant variability exists in post-cardiac arrest neuroprognostication practices among Canadian physicians.
Dr Helen B. Taussig (1898–1986) worked a paediatric cardiologist at the Johns Hopkins University in Baltimore, Maryland from 1930 to 1963. Dr Taussig would become world-renowned for her contributions to the systemic-to-pulmonary artery shunt to treat congenital heart patients with cyanosis. This shunt would eventually be named after the surgeon/cardiologist as the Blalock–Taussig shunt. Dr Taussig’s name was also attached to the description of one form of double outlet right ventricle called the Taussig-Bing malformation. Dr Taussig ultimately received the Presidential Medal of Freedom in 1964 as a testimony to her life-long contributions to the field of congenital heart surgery.
In 1963, Dr Taussig retired from clinical practice but continued her teaching and academic pursuits at Johns Hopkins for another 14 years. Upon her “second retirement” in 1977, she moved to Kennett Square, PA. This paper will review the retirement years of Dr Helen Taussig and the curious intersection between art and medicine.
There is little known about the spectrum of cardiac injury in acute COVID-19 infection in children.
Methods:
A single-centre, retrospective chart analysis was performed. The protocol was deemed IRB exempt. All patients under the age of 21 years admitted from 20 March, 2020 to 22 June, 2021 for acute symptomatic COVID-19 infection or clinical suspicion of multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 were included. Past medical history, lab findings, echocardiogram and electrocardiogram/telemetry findings, and clinical outcomes were reviewed.
Results:
Sixty-six patients with MIS-C and 178 with acute COVID-19 were reviewed. Patients with MIS-C had more cardiac testing than those with acute COVID-19. Inflammatory markers were more likely elevated, and function was more likely abnormal on echocardiogram in those with MIS-C with testing performed. Among patients with MIS-C, 17% had evidence of coronary dilation versus 0% in the acute COVID-19 group. One (0.6%) patient with acute COVID-19 had clinically significant electrocardiogram or telemetry findings, and this was in the setting of prior arrhythmias and CHD. Four (6%) patients with MIS-C had clinically significant findings on electrocardiogram or telemetry. Among patients with acute COVID-19, extracorporeal membrane oxygenation support was required in 0.6% of patients with acute COVID-19, and there was a 2.8% mortality. There were no deaths in the setting of MIS-C.
Conclusions:
Patients with acute COVID-19 and clinical suspicion of cardiac injury had a lower incidence of abnormal laboratory findings, ventricular dysfunction, or significant arrhythmia than those with MIS-C.
Familial hypercholesterolemia (FH) is vastly underdiagnosed and causes an increased risk for sudden cardiac death. Cardiology providers (CHCPs) are in an ideal position to care for FH patients. This research aimed to understand the practice behaviors of CHCPs in the screening, diagnosis, and management of FH.
Methods:
Adaptation of an existing FH knowledge tool guided survey development. The results of the quantitative survey guided development of the interview protocol. CHCPs were recruited in the Division of Cardiology at Columbia University Irving Medical Center (CUIMC). A review of the educational materials offered by the Division of Cardiology was conducted to identify FH knowledge domains presented.
Results:
CHCPs with MDs, at CUIMC for 6–10 years, in clinical practice for 1–5 years, and in inpatient services had the highest average total knowledge scores. CHCPs with RNs, at CUIMC for less than 1 year, in clinical practice for 6–10 years, and in Cath Lab had the lowest average knowledge scores. Four themes emerged – variability in FH care; issues related to addressing FH at institutional, practice setting, and individual levels; importance of identifying FH early; and intervention approaches to overcome barriers to caring for FH patients in cardiology. CHCPs with MDs or with experiential FH knowledge described FH care beyond screening. The document review revealed that only MDs were provided lectures pertaining to FH.
Conclusions:
Future interventions should increase didactic and experiential FH knowledge incorporating institutional, local, and national FH resources. Improving FH care can reduce FH-related morbidity and mortality, as well as improve FH health outcomes.
During the COVID-19 pandemic, Kentucky prohibited elective medical procedures from 3/18/2020-4/27/2020. We sought to determine if cessation of elective procedures in Kentucky during the COVID-19 pandemic resulted in a decrease in the proportion of rarely appropriate outpatient transthoracic echocardiograms interpreted at the open echocardiography lab at Norton Children’s Hospital.
Methods:
A retrospective chart review was conducted comparing proportions of rarely appropriate outpatient paediatric transthoracic echocardiograms performed pre-COVID (3/21/2019-4/28/2019) and during COVID (3/19/2020-4/27/2020). Transthoracic echocardiogram indication was determined by chart review and echocardiogram reports. Indication appropriateness was evaluated using paediatric appropriate use criteria for initial outpatient transthoracic echocardiogram or CHD follow-up as applicable.
Results:
Of transthoracic echocardiograms pre-COVID, 100 (37.7%) were rarely appropriate versus 18 (20.2%) during COVID. Pre-COVID, paediatric cardiologists tended to order fewer rarely appropriate transthoracic echocardiograms than paediatricians (35.9% versus 46.4%), although this difference was not statistically significant. Cardiologists ordered the majority of outpatient transthoracic echocardiograms during COVID (77/89, 86.5%), limiting the ability to compare transthoracic echocardiogram indications by provider type. There was no significant difference in diagnostic yield of initial outpatient transthoracic echocardiograms with (13.0%) abnormal studies pre-COVID versus 7 (15.5%) during COVID.
Conclusion:
While elective procedures were prohibited in Kentucky during the COVID-19 pandemic, a decrease in the proportion of rarely appropriate outpatient paediatric transthoracic echocardiograms was observed. There was no significant difference in diagnostic yield of initial outpatient transthoracic echocardiograms between time periods, suggesting that clinically significant echocardiogram findings were still detected despite more prudent utilisation of echocardiography during this time.
To study the impact of out-of-hours delivery on outcome for neonates with antenatally diagnosed transposition of the great arteries.
Setting
Tertiary paediatric cardiology centre (Yorkshire, United Kingdom), with co-located tertiary neonatal unit.
Patients
Neonates with antenatally diagnosed simple transposition of the great arteries delivered out-of-hours (Monday to Friday 17:00–08:00 and weekends) versus in-hours between 2015 and 2020.
Outcome
The primary outcome was survival to hospital discharge. Secondary outcomes included neurological morbidity, length of stay, and time to balloon atrial septostomy.
Results
Of 51 neonates, 38 (75%) were delivered out-of-hours. All neonates born in the tertiary centre survived to discharge. Time to balloon atrial septostomy was slightly longer for out-of-hours deliveries compared to in-hours (median 130 versus 93 mins, p = 0.33). Neurological morbidity occurred for nine (24%) patients in the out-of-hours group and one (8%) in-hours (OR 3.72, 95% CI: 0.42–32.71, p = 0.24). Length of stay was also similar (18.5 versus 17.3 days, p = 0.59). Antenatal diagnosis of a restrictive atrial septum was associated with a lower initial pH (7.03 versus 7.13; CI: 0.03–0.17, p = 0.01), longer length of stay (22.6 versus 17.3 days; CI: 0.37–10.17, p = 0.04), and increased neurological morbidity (44% versus 14%; OR 4.80, CI 1.00–23.15, p = 0.05). A further three neonates were delivered in surrounding hospitals, with a mortality of 67% (versus 0 in tertiary centre; OR 172, CI 5-5371, p = 0.003).
Conclusion
Neonates with antenatally diagnosed transposition of the great arteries have similar outcomes when delivered out-of-hours versus in-hours. Antenatal diagnosis of restrictive atrial septum is a significant predictor of worse outcomes. In our region, delivery outside the tertiary cardiac centre had a significantly higher risk of mortality.
Antidepressants and antipsychotics have a wide range of cardiac side effects. Although the absolute risk is considered low, some are potentially life-threatening.
Objectives
We aim to review the main cardiological complications of antidepressants and antipsychotics and their management. We will consider 1) QTc prolongation and arrhythmia 2) heart rate 3) blood pressure 4) myocarditis.
Methods
Review of cardiological complications of antidepressants and antipsychotics.
Results
Qtc prolongation is correlated with arrhythmia risk. QTc is obtained with Bazett’s formula, which has limitations. All inpatients and some outpatients starting antipsychotic should undergo ECG. Increased QTc can result in different approaches, depending on severity. Most antidepressants do not significantly affect QTc, except for escitalopram and tricyclics, mostly in overdose. Sinus tachycardia can occur with most antipsychotics. Tricyclics can also produce this effect. Other causes should be excluded, and management can be achieved with bisoprolol. Other antidepressants most commonly produce a slight decrease in heart rate or have a minimal to no effect. Antipsychotics can cause hypertension or hypotension depending on the degree of affinity to specific adrenergic receptors. Tricyclics can lead to postural hypotension. Antidepressants interfering with noradrenaline can cause hypertension. Myocarditis is mostly associated with clozapine. Patients should be screened for clinical signs and laboratory findings - especially in the presence of risk factors. Suspicion should prompt echocardiological examination and confirmation leads to cardiology referral.
Conclusions
Weighing the risks and benefits of these medications is a continuous process. Management of cardiological complications is possible and may involve a multidisciplinary approach.
The aim of this study was to compare the sociodemographic and clinical characteristics of delirium in patients treated in a clinical cardiology unit (CCU) and an oncological palliative care unit (OPCU) at a high-complexity institution.
Context
Delirium is a neuropsychiatric syndrome with multicausal etiology, associated with increased morbidity and mortality.
Method
This was a cross-sectional, analytical observational study. CCU and OPCU patients were evaluated for 480 days. The diagnosis was made according to DSM-V. Sociodemographic characteristics, the Karnofsky index, and the Charlson index were evaluated. Possible etiologies were verified. Severity was assessed with the Delirium Severity Scale (DRS-R98).
Results
A total of 1,986 patients were evaluated, 205 were eligible, and 110 were included in the study (CCU: 61, OPCU: 49). Delirium prevalence was 11.35% in the CCU and 9.87% in the OPCU. CCU patients were 12 years older (p < 0.03) and a history of dementia (41 vs. 8.2%; p < 0.001). Organ failure was the most frequent etiology of delirium in the CCU (41.0%), and in the OPCU, the etiologies were neoplasms (28.6%), side effect of medication (22.4%), and infections (2.5%). Differences were found in the clinical characteristics of delirium evaluated by DRS-R98, with the condition being more severe and with a higher frequency of psychotic symptoms in OPCU patients.
Conclusion
Delirium was a common condition in hospitalized patients in the CCU and the OPCU. The clinical characteristics were similar in both groups; however, significant differences were found in OPCU patients in terms of age, personal history of dementia, and opioid use, as well as the severity of delirium and a greater association with psychotic symptoms. These findings have implications for the early implementation of diagnostic and therapeutic strategies.
To assess the variables associated with incomplete and unscheduled cardiology clinic visits among referred children with a focus on equity gaps.
Study design:
We conducted a retrospective chart review for patients less than 18 years of age who were referred to cardiology clinics at a single quaternary referral centre from 2017 to 2019. We collected patient demographic data including race, an index of neighbourhood socio-economic deprivation linked to a patient’s geocoded address, referral information, and cardiology clinic information. The primary outcome was an incomplete clinic visit. The secondary outcome was an unscheduled appointment. Independent associations were identified using multivariable logistic regression.
Results:
There were 10,610 new referrals; 6954 (66%) completed new cardiology clinic visits. Black race (OR 1.41; 95% CI 1.22–1.63), public insurance (OR 1.29; 95% CI 1.14–1.46), and a higher deprivation index (OR 1.32; 95% CI 1.08–1.61) were associated with higher odds of incomplete visit compared to the respective reference groups of White race, private insurance, and a lower deprivation index. The findings for unscheduled visit were similar. A shorter time elapsed from the initial referral to when the appointment was made was associated with lower odds of incomplete visit (OR 0.62; 95% CI 0.52–0.74).
Conclusion:
Race, insurance type, neighbourhood deprivation, and time from referral date to appointment made were each associated with incomplete referrals to paediatric cardiology. Interventions directed to understand such associations and respond accordingly could help to equitably improve referral completion.
Anomalous right coronary artery from pulmonary artery (ARCAPA) is a rare congenital heart disease that can lead to abnormal coronary perfusion and a need for surgical repair. Here, we report the outcomes of patients who underwent ARCAPA surgery within the Pediatric Cardiac Care Consortium (PCCC), a North American registry of interventions for paediatric heart diseases. We queried the PCCC for patients undergoing surgical repair for ARCAPA at <18 years of age between 1982 and 2003. Outcomes were obtained from the PCCC and after linkage with the National Death Index (NDI) and the Organ Procurement and Transplantation Network (OPTN) through 2019. Twenty-four patients (males: 15) were identified having surgery for ARCAPA at a median age of 5.8 (IQR 2.7–10.3) years. Of them, 23 cases were considered “simple” (without major intracardiac disease) and one “complex” (co-existing with tetralogy of Fallot). Five patients presented with symptoms [chest pain (1), dyspnoea on exertion (2) or history of syncope (2)]; while the remaining 19 patients were referred for evaluation of either murmur or co-existing CHD. There was no in-hospital mortality after the surgical repair. Fourteen patients had sufficient identifiers for NDI/OPTN linkage; among them, only one death occurred from unrelated non-cardiac causes within a median period of 19.4 years of follow-up (IQR: 18–24.6). Outcomes were excellent after reimplantation up to 25 years later and further longitudinal monitoring is important to understand the interaction of pre-existing coronary pathology with the effects of ageing.
The study aimed to show the chromosomal copy number variations responsible for the aetiology in patients with isolated conotruncal heart anomaly by array comparative genomic hybridisation and identify candidate genes causing conotruncal heart disease. A total of 37 patients, 17 male, and 20 female, with isolated conotruncal heart anomalies, were included in the study. No findings indicated any syndrome in terms of dysmorphology in the patients.
Results:
Copy number variations were detected in the array comparative genomic hybridisation analysis of five (13.5%) of 37 patients included in the study. Three candidate genes (PRDM16, HIST1H1E, GJA5) found in these deletion and duplication regions may be associated with the conotruncal cardiac anomaly.
Conclusion:
CHDs can be encountered as the first and sometimes the single finding of many genetic disorders in children. It is thought that genetic tests, especially array comparative genomic hybridisation, may be beneficial for children with CHD since the diagnosis of genetic diseases in these patients as early as possible will help to prevent or reduce complications that may develop in the future. Also, it would be possible to detect candidate genes responsible for conotruncal cardiac anomalies with array comparative genomic hybridisation.
Congenital junctional ectopic tachycardia is a rare but serious cardiac arrhythmia seen in neonates and young infants. It is frequently resistant and refractory to first-line treatment options such as cardioversion with adenosine and direct current shock, and it carries a high morbidity and mortality rate. The aim of this article is to present the case of congenital junctional ectopic tachycardia observed in a 14-day-old neonate, highlighting the role of ivabradine in the management, followed by a discussion about current approaches to treatment.