Pediatric Cardiology最新文献

Expertise in pediatric arrhythmia management is lacking in most low- and middle- income countries (LMIC). Strategies to disseminate education in pediatric electrophysiology are essential to meet this need. Children's Heartbeat was created to meet the growing demand for pediatric electrophysiologic consultation in LMIC. Children's Heartbeat is a virtual video-conferencing program that uses the Extension for Community Healthcare Outcomes model to disseminate knowledge about pediatric arrhythmia management from pediatric electrophysiologists to clinicians in medically under-resourced regions worldwide. Monthly virtual case-based sessions are held with pediatric electrophysiologists and clinicians in medically under-resourced settings to discuss pediatric electrophysiology management. Since its inception, Children's Heartbeat viewership has grown exponentially to include 181 total registrants, 64 average monthly participants, and an additional 121 average viewers of recorded sessions. Attendees have expressed increased confidence in pediatric arrhythmia management. Children's Heartbeat has successfully provided pediatric electrophysiology consultation to medically under-resourced regions globally and have increased clinicians' confidence in caring for children with arrhythmias. Future directions include spreading the program to general pediatric and pediatric cardiology trainees and practicing pediatricians in rural parts of high-income countries and evaluating the direct impact of Children's Heartbeat on the management of arrhythmias in pediatric patients in LMIC.

As surgical options and medical care for patients with univentricular heart physiology continue to improve, leading to increased life expectancy and quality of life, a new population of Fontan patients is growing up with the desire to participate in leisure activities, including aquatic activities, high-altitude stays, and air travel. Due to significant data gaps and insufficient experience, current guidelines do not provide clear recommendations, leading to uncertainty and sometimes restrictive patient management. This review summarizes new insights and the current state of research on this subject and provides an overview of the long overdue change in policies toward less restrictive counseling for Fontan patients regarding swimming, diving, high-altitude stays, and air travel. The current review summarizes the physiologic impact of aquatic and high-altitude activities on the cardiovascular system and presents currently available data on this topic in Fontan patients. Patients with Fontan circulation in good clinical shape can tolerate activities in the water and in the mountains as well as air traveling without critical events. In order to be able to make general recommendations, further studies with larger numbers of cases must be carried out.

Individuals with Fontan circulation (FC) often have diminished exercise capacity and are at risk for psychological problems. The current study examines associations between exercise capacity and psychological functioning in children and adolescents with FC. A multidisciplinary team evaluated participants in a clinic for pediatric patients with FC. Participants completed cardiopulmonary exercise testing (CPET). Parent and child were administered a psychological questionnaire (BASC-3) to measure child depression, anxiety, and inattention symptoms. Individuals who completed CPET with adequate effort and a psychological measure were eligible for inclusion. Clinical sample (n = 51) was 55% male with a mean age of 13.6 years (SD = 2.5). A majority had hypoplastic left heart syndrome (51%). Parent-report of inattention was negatively correlated with peak VO2 (R =  - .307, 95% CI - .549/ - .018, P = 0.038). Self-report of anxiety was positively correlated with HR recovery at 3 (R = .438, 95% CI .155/.655, P = 0.004) and 8 (R = .432, 95% CI .147/.651, P = 0.004) minutes post exercise. Depression was positively correlated with HR recovery at 3 min for parent-report (R = .294, 95% CI .004/.538, P = 0.047) and 8 min for self-report (R = .410, 95% CI .122/.635, P = 0.007). Greater inattention may have negatively impacted CPET engagement, reflected in lower peak VO2. The more rapid decline from max HR to recovery for those with greater depression and anxiety symptoms was unexpected, perhaps explained by a reduction in anxiety state after exercise or possibly an age effect, as a blunted HR decline has been found in adult cardiac patients with depression/anxiety.

Fontan patients' exercise capacity and associated risks of morbidity and mortality necessitate a comprehensive study. We aim to explore maximal and submaximal exercise capacity, assessing the impact of cardiovascular and peripheral factors, including atrial strain, aortic stiffness, and muscle-related factors. Fontan patients and matched controls were examined. Clinical details were recorded, and atrial strain and aortic stiffness were measured using echocardiography. Hand grip and knee extensor strength were assessed, and muscle oxygenation was recorded. Cardiopulmonary exercise test determined exercise capacity, with peak oxygen uptake/kg (VO₂/kg) and oxygen uptake efficiency slope/kg (OUES/kg) as markers for maximal and submaximal capacity, respectively. Thirty-one Fontan patients (median age = 18, range = 7-31 years) and 30 controls (median age = 18, range = 7-32 years) were studied. No significant differences in body composition and muscle strength were observed between Fontan patients and controls (p > 0.05). Fontan patients exhibited lower peak VO₂/kg and OUES/kg (p < 0.001 for both), along with decreased atrial strain and increased aortic stiffness compared to controls (p < 0.001). Aortic pulse wave velocity (PWV), left atrial strain contractile phase (LASct), and knee extensor strength independently predicted peak VO₂/kg (R² = 0.514) and OUES/kg (R² = 0.486) in Fontan patients; age was a predictor for peak VO₂/kg. Atrial contractile strain, aortic stiffness, and knee extensor muscle strength are predictors of submaximal exercise capacity; furthermore, age, along with these variables, serves as predictors of maximal exercise capacity in Fontan patients. Evaluating hemodynamic, vascular, and muscular parameters, in conjunction with assessing both maximal and submaximal exercise capacities, is crucial for optimizing disease management in Fontan patients. Clinicaltrials.gov registration: NCT05011565.

Complete atrioventricular septal defect (CAVSD) can lead to the development of pulmonary obstructive vascular disease due to high pulmonary blood flow and pressures. This study aimed to evaluate the changes in pulmonary hemodynamics with aging and with patent ductus arteriosus (PDA) in children with CAVSD. We retrospectively evaluated 137 children (94% with trisomy 21, median age of 195 (25-2963) days, 58.4% female) with CASVD referred to cardiac catheterization from January 2000 to December 2020. Those with associated congenital heart disease, except PDA, had been excluded. They were divided into three age terciles (T1, T2, and T3). Aging was directly associated with higher mean (T1: 34.2 ± 9.1; T2: 37.1 ± 5.8; T3: 42 ± 10.6 mmHg, p < 0.001) and diastolic (T1: 19.4 ± 5.3; T2 21.6 ± 5.0; T3: 26.0 ± 9.5 mmHg, P < 0.001) pulmonary arterial pressures, and with higher pulmonary vascular resistance (T1: 3.24 ± 1.69, T2: 3.47 ± 1.19; T3: 4.49 ± 3.91 Wu.m², p = 0.023). This resulted in a loss of eligibility for anatomical correction, which became evident only after 300 days of age. PDA was associated with a higher mean (37.2 [35.9; 38.5] vs. 41.3 [37.5; 45.0] mmHg, p = 0.049) and diastolic (21.7 [20.7; 22.6] vs. 26.4 [24.1; 29.0] mmHg, p = 0.001) pulmonary pressure, and resistor-compliance time (0.28 [0.26; 0.29] vs. 0.36 [0.31; 0.40], p = 0.001) after adjusting for age and sex. In children with CAVSD, aging was associated with worsening of pulmonary vascular hemodynamics, particularly when PDA was associated, resulting in loss of eligibility for anatomical correction after 10 months of age as the first surgical option.

Patients with a single ventricle heart who had Fontan palliation (S/P Fontan) are at increased risk for acquired morbidity. Insulin resistance (IR) is a predictor of cardiac morbidity and mortality. A single-center, cross-sectional study using S/P Fontan and controls was designed to assess IR S/P Fontan. Group comparisons were made in IR via the Quantitative Insulin Index (QUICKI) and the natural log-transformed homeostasis model assessment, ln (HOMA-IR), without/with adjusting for age. A total of 89 patients (59 Fontan and 30 controls) were included. Fontan patients showed a significant decrease in QUICKI (0.34 ± 0.03 vs 0.37 ± 0.02) and an elevation of ln (HOMA-IR) (0.82 ± 0.62 vs 0.24 ± 0.44) compared to controls (both p < 0.0001); this remained significant even adjusting for age. With older age, there was a significant, progressive decrease in QUICKI (p = 0.01) and an increase in ln (HOMA-IR) (p = 0.02) S/P Fontan. Analysis excluding Fontan patients with obesity still showed a significant reduction of QUICKI and an elevation of ln (HOMA-IR) in Fontan patients compared to controls when adjusting for age (both p < 0.05). Using QUICKI, IR was present in 41 (69.5%) Fontan patients vs. 3 (10%) controls (p < 0.0001) and using HOMA-IR, IR was present in 32 (54.2%) vs 5 (16.7%) controls (p = 0.001). Fontan patients had significantly more IR compared to controls and the prevalence of IR increases with age. Since IR is known to correlate with long-term morbidity and mortality and can be ameliorated by therapies, we believe it is critical that IR be identified as early as possible in Fontan patients.

Most epidemiological studies in pediatric heart failure (HF) use administrative database sources, defining patient cohorts by presence of a single HF ICD code. However, the ability of ICD codes to identify true HF patients is unknown in pediatrics. Here we describe the accuracy of HF ICD-10-CM code search algorithms, in identifying pediatric patients with HF from electronic data sources. Based on the adult HF literature, search algorithms were designed to incorporate HF ICD codes, imaging, and medications. Sensitivity, specificity, positive and negative predictive value and accuracy of the algorithms were tested among children in an advanced HF clinic ("Clinic cohort"). Top-performing algorithms were then tested in a large-scale regional electronic data warehouse (EDW), 01/2017 to 01/2020, generating the "EDW Cohort". False positive cases were identified and characterized by chart review. Within the Clinic Cohort, 78/378 patients (21%) had gold standard HF diagnoses. A search algorithm with one HF ICD coded visit was more sensitive but less specific than > 1 HF ICD coded visit, (sensitivity 94% and specificity 89% versus 69% and 97%, respectively). Correspondingly, > 1 ICD coded visit had a higher PPV than one ICD coded visit; 84% vs. 69%. Accuracy was similar (90% vs 91%). Presence of 1 HF ICD code combined with HF medication had high sensitivity, specificity, PPV, NPV and accuracy, all higher than the single ICD code algorithm. The "1 HF coded visit + any medication" algorithm resulted in highest accuracy (93%). Top-performing algorithms were tested in the EDW: the algorithm with > 1 HF ICD coded visit, and the algorithm with one HF ICD coded visit combined with HF medication. In the EDW Cohort, 133/248 (53.6%) patients had gold standard HF diagnoses though 115/248 (46.3%) were false positive cases; 41% of those had pulmonary over-circulation from congenital heart disease. Excluding children < 30 days old and those with a history of an isolated VSD repair, complete AVSD repair, or PDA closure further reduced the proportion of false positives to 50/248 (20%). A search algorithm using a single HF ICD code can have acceptable sensitivity, specificity, PPV, NPV and accuracy in identifying children with HF from within electronic medical records. Similar to adult HF literature, specificity improves by including HF medication. When applied to large data sources, however, the search algorithms result in a high proportion of patients with pulmonary overcirculation related to congenital heart disease. To narrow the population to those with myocardial dysfunction, case identification may require use of ICD codes with linked of administrative, surgical, and/or imaging databases.

Children with cardiac disease suffer higher rates of in-hospital cardiac arrest. Cardiac arrest outside of the intensive care unit (ICU) is an infrequent event, which is a threat for suboptimal code team performance. Shorter time to the first epinephrine dose during cardiac arrest has been associated with improved survival and neurologic outcomes. Moderate fidelity in situ simulation training was implemented in the pediatric cardiac acute care unit (PCACU) to improve code team performance in August 2015. A small interprofessional team of simulation facilitators was developed that included nurses, physicians, and advanced practice providers. The primary outcome was time to first epinephrine dose in pulseless electrical activity (PEA) scenarios. Time to epinephrine of all simulation exercises, and actual cardiac arrests, that occurred in the PCACU were reviewed through May 2022. A total of 72 simulations were performed and 42 (58%) were PEA scenarios. A center line shift was observed for time to epinephrine for simulated PEA cardiac arrests (from 5 to 3 min). After implementation of simulation exercises, a center line shift was observed for time to epinephrine for actual cardiac arrests in the PCACU (from 8 to 2 min). Survival to hospital discharge after cardiac arrest improved after implementation of the training (0% vs. 64%, p = 0.02). Code team performance, as measured by time to epinephrine, can be improved following implementation of in situ simulation exercises in a pediatric cardiac acute care unit. Optimizing code team performance may contribute to improved patient outcomes.

Evidence suggests that pediatric palliative care involvement (PPC) is beneficial to medically complex patients. Historically, PPC involvement has been overlooked or delayed and varies by institution but PPC awareness has increased in cardiovascular ICUs (CVICU) and so we investigated frequency and timeliness of PPC referral for patients dying in ICU. Retrospective study of pediatric cardiac patients experiencing death in ICU to review PPC presence and timing of initial PPC, most recent PPC, and interventions, therapies, CPR, and presence of do-not-resuscitate DNR discussion. Fifty-four patients died during a 5-year period aged 11d-17y (54% male). PPC involvement occurred in 40/54 (74%). Of those patients without PPC, the Center to Advance Palliative Care (CAPC) guidelines would have supported PPC in 11/14 (79%). DNR discussion was more likely in PPC patients (63% vs 14%; p = 0.0011), though often only on DOD. Comparing prior to DOD, PPC patients were still more likely to have DNR discussion (55% vs 0%; p = 0.0003). PPC patients were no less likely to have CPR on DOD (28% vs 43%, p = 0.29). PPC occurred frequently in patients experiencing death in CVICU. However, frequently the initial PPC occurred within a week or day of death. Patients without PPC would often qualify under published guidelines. Standardization, timing, and patient identification for PPC will expand efficacy in CVICU.