Listening to Patients Makes Sense: Soliciting and Purposefully Addressing Written Patient Expectations at a Provider Visit Improve Patient Satisfaction.

Patient satisfaction is an important aspect of medical care. This study aimed to assess if patient satisfaction improved when patients shared their expectations with the provider in writing before a visit, and providers purposefully addressed those expectations during the visit. We gave 2 types of questionnaires to 343 patients: Version 1 asked for written expectations before the visit and assessed the visit quality after addressing those expectations, while Version 2 only evaluated the visit without soliciting expectations. Patient satisfaction and meeting expectations were measured on a 1-10 Likert-type scale. The grouped that shared written expectations before the visit (n = 169) showed a significantly higher patient satisfaction score (9.88) compared to the group without shared expectations (n = 136, score 9.43, P < .0001). Conveying written expectations to healthcare providers before the visit improved patient satisfaction, potentially enhancing compliance and overall medical outcomes.

Self-limited myocarditis presenting with chest pain and ST segment elevation in adolescents after vaccination with the BNT162b2 mRNA vaccine.

Two adolescent males presented within 3 days after the first and second dose of the BNT162b2 vaccine with chest pain. Elevated troponin levels, ST segment elevation, and enhancement of the myocardium in cardiac MRI suggested myocarditis. Left ventricular function remained normal, symptoms resolved, and patients were discharged in 4 days. BNT162b2 vaccine may be associated with self-limited myocarditis in youth.

Spectrum of Viral Pathogens Identified in Children with Clinical Myocarditis (Pre-Coronavirus Disease-2019, 2000-2018): Etiologic Agent Versus Innocent Bystander.

OBJECTIVE: To identify the etiologies of viral myocarditis in children in the pre-coronavirus disease 2019 era. STUDY DESIGN: This was a retrospective review of all patients (age <18 years) diagnosed with myocarditis and hospitalized at Rady Children's Hospital San Diego between 2000 and 2018. RESULTS: Twenty-nine patients met inclusion criteria. Of 28 (97%) patients who underwent testing for viruses, polymerase chain reaction was used in 24 of 28 (86% of cases), and 16 of 24 (67%) detected a virus. Pathogens were rhinovirus (6), influenza A/B (4), respiratory syncytial virus (RSV) (3), coronavirus (3), parvovirus B19 (2), adenovirus (2), and coxsackie B5 virus, enterovirus, and parainfluenza virus type 2 in one case each. Six (21%) patients had no pathogen detected but imaging and other laboratory test results were compatible with myocarditis. Age 0-2 years was associated with RSV, influenza A/B, coronavirus, and enteroviruses (P < .001). Twenty-one patients (72%) experienced full clinical recovery. Three patients (10%) required venoarterial extracorporeal membrane oxygenation (VA-ECMO), and all 3 recovered. Three others (10%) required and underwent successful cardiac transplantation without complications. Two patients (7%) died 9-10 days after hospitalization (1 had RSV and 1 had influenza A/B). Two other patients presented with complete atrioventricular block; 1 case (rhinovirus) resolved spontaneously, and 1 (coronavirus) resolved after support with VA-ECMO. Age <2 years, female sex, lower ejection fraction at admission, and greater initial and peak levels of brain natriuretic peptide were significant predictors of critical outcomes (use of VA-ECMO, listing for cardiac transplantation, and death). CONCLUSIONS: Viral nucleic acid-based testing revealed a wider spectrum of viruses that could be associated with myocarditis in children than previously reported and traditionally anticipated. A predilection of certain pathogens in the very young patients was observed. Whether the observed range of viral agents reflects an undercurrent of change in viral etiology or viral detection methods is unclear, but the wider spectrum of viral pathogens found underscores the usefulness of polymerase chain reaction testing to explore possible viral etiologies of myocarditis in children.

Temporal clustering of Kawasaki disease cases around the world.

In a single-site study (San Diego, CA, USA), we previously showed that Kawasaki Disease (KD) cases cluster temporally in bursts of approximately 7 days. These clusters occurred more often than would be expected at random even after accounting for long-term trends and seasonality. This finding raised the question of whether other locations around the world experience similar temporal clusters of KD that might offer clues to disease etiology. Here we combine data from San Diego and nine additional sites around the world with hospitals that care for large numbers of KD patients, as well as two multi-hospital catchment regions. We found that across these sites, KD cases clustered at short time scales and there were anomalously long quiet periods with no cases. Both of these phenomena occurred more often than would be expected given local trends and seasonality. Additionally, we found unusually frequent temporal overlaps of KD clusters and quiet periods between pairs of sites. These findings suggest that regional and planetary range environmental influences create periods of higher or lower exposure to KD triggers that may offer clues to the etiology of KD.

Osteoporosis treatment rates after hip fracture 2011-2019 in Hawaii: Undertreatment of men after hip fractures.

OBJECTIVES: To investigate trends of osteoporosis treatment rates, and factors affecting osteoporosis treatment after hip fracture admission within a single health care system in Hawaii. METHODS: A retrospective chart review was conducted of patients aged 50 years or older and hospitalized for hip fractures between January 1, 2011 and December 31, 2019 at Hawaii Pacific Health, a large health care system in Hawaii. We collected data on basic demographics and osteoporosis medication prescription from electronic medical records. We evaluated trends of osteoporosis treatment rates and performed logistic regression to determine factors associated with osteoporosis treatment. RESULTS: The mean for treatment rates for osteoporosis from 2011 to 2019 was 17.2% (range 8.8%-26.0%). From 2011 to 2019 there was a small increase in treatment rates from 16.3% in 2011 to 24.1% in 2019. Men were less likely to receive osteoporosis treatment after admission for hip fracture. Patients discharged to a facility were more likely to receive osteoporosis treatment. As compared to women, men who had a hip fracture were less likely to receive dual-energy X-ray absorptiometry scan, and osteoporosis medication before hip fracture admission. CONCLUSIONS: The use of osteoporosis medication for secondary prevention after admission for hip fracture in Hawaii from 2011 to 2019 was low. However, there was a small increase in treatment rates from 2011 to 2019. Disparities in treatment of osteoporosis after hip fracture were noted in men. Significant work is needed to increase treatment rates further, and to address the disparity in osteoporosis treatment between men and women.

Acute Adult-Onset Kawasaki Disease Complicated by Coronary Artery Aneurysms, Thrombosis, and ST-Segment Elevation Myocardial Infarction.

A 21-year-old man who had an initial misdiagnosis of chest wall cellulitis and sepsis presented to the emergency department with chest pain. Electrocardiogram demonstrated ST-segment elevation in the inferior leads. Cardiac catheterization identified diffuse aneurysmal dilation and thrombosis of the distal right coronary artery. Clinical signs were consistent with acute Kawasaki disease. (Level of Difficulty: Intermediate.).

Junctional Tachycardia as a Diagnostic Criterion in Acute Rheumatic Fever.

Acute rheumatic fever (ARF) is an acute inflammatory process resulting in rheumatic carditis, one of the most common acquired heart diseases in youth. Among the clinical manifestations of carditis, pathologic valve regurgitation and atrioventricular block are included in the criteria for the diagnosis of ARF. Besides atrioventricular block, ARF may often present with other arrhythmias, such as junctional tachycardia (JT). However, JT is currently not recognized as a criterion for the diagnosis of ARF. Three adolescents presented in our hospital with JT, polyarthralgia, and laboratory signs of inflammation with evidence of preceding group A Streptococcus infection. None of the patients fulfilled the diagnostic criteria of ARF. On the basis of the presumed diagnosis of ARF, all 3 patients were treated with intravenous steroids. Steroid therapy was given, and JT converted to sinus rhythm within an average of 62 hours. Subsequent electrocardiograms revealed variable degree of atrioventricular block in all 3 patients, providing clinical evidence and fulfilling the diagnostic criteria of ARF. Patients were monitored for a total 2 to 8 days before discharge on standard antiinflammatory treatment. Follow-up electrocardiograms and Holter monitoring revealed resolution of the atrioventricular block and lack of JT recurrence in all patients. On the basis of these sentinel cases, we propose that JT should be included as a diagnostic criterion for the diagnosis of ARF.

Junctional tachycardia due to multisystem inflammatory syndrome in children with SARS-CoV-2 infection in a 12-year-old female.

A 12-year-old girl presented with fever and signs of systemic inflammation, and was found to have junctional tachycardia. She was subsequently diagnosed with Multisystem Inflammatory Syndrome in Children and treated with intravenous immunoglobulin and steroids, which led to resolution of the arrhythmia.

Elevated Levels of Pentraxin 3 Correlate With Neutrophilia and Coronary Artery Dilation During Acute Kawasaki Disease.

Kawasaki disease (KD) is the leading cause of acquired pediatric heart disease in the developed world as 25-30% of untreated patients and at least 5% of treated patients will develop irreversible coronary artery lesions (CAL). Pentraxin-3 (PTX-3) has been well-studied in inflammatory diseases, particularly in cardiovascular diseases associated with vascular endothelial dysfunction. We hypothesized that PTX-3 plays an important role in the development of KD-associated CAL and investigated the circulating levels of PTX-3 in the serum of KD patients. Children with acute KD were followed from diagnosis through normalization of the clinical parameters of inflammation (convalescent phase). Serum samples were obtained and echocardiograms were conducted at several phases of the illness: acute [prior to intravenous immunoglobulin (IVIG) treatment], sub-acute (5-10 days after IVIG treatment), and convalescent (1-4 months after KD diagnosis). Seventy children were included in the final cohort of the study, of whom 26 (37%) presented with CAL and 18 (26%) developed IVIG resistance. The patients included in this study came from diverse ethnic backgrounds, mostly with mixed ancestry/ ethnicity. Significantly increased PTX-3 levels were observed during the acute phase of KD compared to the sub-acute and the convalescent phases. The PTX-3 levels during acute KD were significantly higher among KD patients with CAL compared to patients with normal coronary arteries (NCA). Also, the PTX-3 levels were significantly higher in patients with IVIG resistance. Furthermore, the PTX-3 levels were significantly higher in IVIG-resistant KD patients with CAL as compared to the NCA group. Moreover, the PTX-3 levels were significantly correlated to coronary artery z-score during acute KD and to neutrophil counts throughout KD progression regardless of coronary artery z-score. Elevated PTX-3 levels correlated to elevated neutrophil counts, a known source of PTX-3 in acute inflammation and an important player in the development of KD vasculitis. We, therefore, suggest PTX-3 as a novel factor in the development of KD-associated CAL and propose neutrophil-derived PTX-3 as contributing to KD vascular dysfunction.

Electrocardiogram Standards for Children and Young Adults Using Z-Scores.

BACKGROUND: Normative ECG values for children are based on relatively few subjects and are not standardized, resulting in interpersonal variability of interpretation. Recent advances in digital technology allow a more quantitative, reproducible assessment of ECG variables. Our objective was to create the foundation of normative ECG standards in the young utilizing Z-scores. METHODS: One hundred two ECG variables were collected from a retrospective cohort of 27 085 study subjects with no known heart condition, ages 0 to 39 years. The cohort was divided into 16 age groups by sex. Median, interquartile range, and range were calculated for each variable adjusted to body surface area. RESULTS: Normative standards were developed for all 102 ECG variables including heart rate; P, R, and T axis; R-T axis deviation; PR interval, QRS duration, QT, and QTc interval; P, Q, R, S, and T amplitudes in 12 leads; as well as QRS and T wave integrals. Incremental Z-score values between -2.5 and 2.5 were calculated to establish upper and lower limits of normal. Historical ECG interpretative concepts were reassessed and new concepts observed. CONCLUSIONS: Electronically acquired ECG values based on the largest pediatric and young adult cohort ever compiled provide the first detailed, standardized, quantitative foundation of traditional and novel ECG variables. Expression of ECG variables by Z-scores lends an objective and reproducible evaluation without interpreter bias that can lead to more confident establishment of ECG-disease correlations and improved automated ECG readings in high-volume cardiac screening efforts in the young. Graphic Abstract: A graphic abstract is available for this article.

Mapping the Trends of Kawasaki Disease in Hawai'i from 1996 to 2018.

Kawasaki disease is a systemic vasculitis of unknown etiology and is the leading cause of acquired heart disease in children in the developed world. Historically, Hawai'i has had the highest incidence of Kawasaki disease in the United States, likely due to the population's unique ancestral composition. To analyze the epidemiology, demographics and spatiotemporal distribution of Kawasaki disease in Hawai'i, a retrospective chart review was conducted utilizing data from Kapi'olani Medical Center for Women and Children encompassing the period of 1996-2018. A total of 858 patients were analyzed with 877 episodes of Kawasaki disease. On average, 37 episodes of Kawasaki disease were diagnosed annually over the 23-year period. The annual incidence was 32 per 100 000 children <5 years of age. Asian children (66.1%) accounted for the majority of cases, followed by Native Hawaiians and Other Pacific Islanders (16.6%). Unlike Japan and the continental United States, there was no characteristic seasonal pattern in the distribution of Kawasaki disease in Hawai'i, which may be attributed to its tropical climate or the recent changes in global weather patterns. Local geographical differences in the incidence of Kawasaki disease demonstrated that the Windward (Eastern) coast of O'ahu had a higher rate, while the Leeward (Western) coast displayed a lower incidence rate. This could be explained by variations in ethnic composition and weather patterns of certain areas. Future studies could provide geographical weather data and statistical analysis to determine what environmental triggers are correlated with Kawasaki disease trends in the State of Hawai'i.

The Importance of Echocardiogram during the Second Week of Illness in Children with Kawasaki Disease.

OBJECTIVE: To determine the timing of peak coronary artery dilation and the characteristics of patients who present with new-onset coronary artery dilation during the acute phase of Kawasaki disease with an initial normal echocardiogram. STUDY DESIGN: This retrospective study analyzed 231 children hospitalized for Kawasaki disease in Hawai'i over a period of 7 years. Clinical and echocardiographic data were collected to calculate the timing of peak z score, and study subjects were compared based on the timing of coronary dilation. RESULTS: Peak coronary artery dilation was observed on average at 11.5 days from the onset of fever (median 8, IQR 7-13 days). Among study subjects with normal z scores in both coronary arteries during the initial encounter and echocardiogram (n = 164), 16 (10%) developed coronary artery dilation or aneurysm at the second echocardiogram, and 5 (3%) continued to have coronary artery dilation or aneurysm at the convalescent phase. CONCLUSIONS: A repeat echocardiogram during the second week of illness (day 7-14 from fever onset) in patients with normal initial echocardiogram could identify new-onset coronary artery dilation or aneurysm and could be useful in the timely adjustment of antithrombotic or anti-inflammatory therapies.

Clinical Outcomes and Modes of Death in Timothy Syndrome: A Multicenter International Study of a Rare Disorder.

OBJECTIVES: The objective of this study was to evaluate contemporary clinical outcomes and identify triggers for arrhythmias or sudden death in an international cohort of Timothy Syndrome (TS) patients including those with novel TS-associated CACNA1C mutations. BACKGROUND: TS is an extremely rare genetic disorder of the L-type cardiac channel Cav1.2 encoded by CACNA1C. The syndrome is characterized by multisystem abnormalities consisting of QT prolongation, congenital heart defects, syndactyly, facial dysmorphism, and neurological symptoms. METHODS: Patients diagnosed with TS between January 1, 1994, and April 1, 2016, from 12 international tertiary care pediatric centers were included in this retrospective study. Data were gathered via survey from the patients' electrophysiologists. RESULTS: Seventeen patients diagnosed with TS were identified. Length of follow-up was 4.9 years (range 3.0 to 19.0 years). Mean QTc was 640 ms (range 500 to 976 ms). All patients were treated with beta-blockers; 13 patients (76%) were also treated with an implantable defibrillator. Eleven patients experienced an episode of aborted cardiac arrest, 6 associated with general anesthesia and 2 with hypoglycemia. Four patients died suddenly due to ventricular fibrillation, 2 of whom had associated hypoglycemia. CONCLUSIONS: This study shows that mortality in TS patients is due to multifactorial mechanisms, which include ventricular arrhythmias, pulseless electrical activity, and hypoglycemia. A simple nomenclature for ongoing studies of TS and related syndromes is described. A worldwide prospective registry is needed for continued exploration of this syndrome.

Epicardial Intraoperative Three-Dimensional Mapping of Wolff-Parkinson-White Syndrome in a Child With Ebstein's Anomaly.

Catheter ablation of arrhythmias in small patients has inherent significant risk because of the patient, catheter, and lesion size. Heart block, perforation, and coronary damage may occur. This report shows adaptation of a three-dimensional electroanatomic mapping system for intraoperative use in a small patient with Ebstein's anomaly who was undergoing a cone procedure and a bidirectional Glenn procedure. Use of this technique gave clear identification of the epicardial insertion points of two accessory pathways causing Wolff-Parkinson-White syndrome and resistant episodes of supraventricular tachycardia. Successful three-dimensional mapping and cryoablation were performed with a short procedure time and no complications. The patient remains without preexcitation or arrhythmia 6 months postoperatively.

Study design and rationale to assess Doxycycline Efficacy in preventing coronary Artery Lesions in children with Kawasaki disease (DEAL trial) - A phase II clinical trial.

BACKGROUND: Kawasaki disease (KD) is the most common acquired heart disease in children of the developed world, and triggers progressive coronary artery lesions (CAL) in 30% of cases if left untreated. Despite standard anti-inflammatory treatment for KD, CAL (dilation or aneurysm) still occurs in 5-10% of children, increasing their risk for fatal coronary artery complications. CAL is mediated by enhanced matrix metalloproteinase activity and elastin breakdown induced by the inflammatory process in the coronary artery wall. Doxycycline is an effective inhibitor of matrix metalloproteinases, and has been shown to reduce elastin breakdown and CAL in a mouse model of KD, but has not been evaluated in patients. OBJECTIVE: We aim to evaluate the efficacy of doxycycline in the prevention of CAL in children during the acute phase of KD. DESIGN: This is a phase II prospective, randomized, double-blinded, clinical trial in two steps. In Step 1, any child older than 1month with the diagnosis of KD will be included. Children with KD will be included in Step 2 if they develop coronary artery dilation (z-score≥2.5) within 20days from the onset of fever. Study subjects in Step 2 will be randomized to receive a 3-week course of doxycycline or placebo. EVALUATION: The efficacy of a 3-week doxycycline course during the acute phase of KD will be evaluated by measuring the decline in coronary artery z-scores from baseline with doxycycline treatment compared to placebo. CLINICAL TRIAL REGISTRATION: This study was registered on clinicaltrials.gov (NCT01917721).

Intraoperative Three-Dimensional Mapping of Supraventricular Tachycardia in a Young Fontan Patient.

Preoperative tachycardia is uncommon before an initial Fontan procedure. Catheter intervention can risk complications in small patients. A patient with fetal tachycardia and double inlet left ventricle underwent Norwood and bidirectional Glenn procedures. She had persistent tachycardia. A three-dimensional electroanatomic mapping system was modified for the operative procedure; it was used to identify the arrhythmia substrate and cryoablate an atrioventricular nodal tachycardia. Mapping added 20 minutes to the operation. She is arrhythmia-free and off medication after 4 months. This three-dimensional system was successfully adapted for an intraoperative SVT ablation in this small patient, and it may be useful in other settings.

Underdiagnosis of Conditions Associated with Sudden Cardiac Death in Children--Is it the Absence of a Comprehensive Screening Program or a True Low Prevalence?

This study aimed to assess the prevalence of conditions associated with sudden cardiac death (SCD) among all children and children with sudden infant death syndrome (SIDS) in the State of Hawai'i, where no comprehensive screening program is conducted for such conditions. A retrospective chart review was conducted from the single tertiary pediatric hospital in Hawai'i, from offices of all pediatric cardiologists in Hawai'i, and the Hawai'i State Department of Health from 1/1/2000 to 12/31/2013. Children aged 0-18 years were included in the study. A subset of the study analyzed records of infants aged 0-12 months. SIDS rate was calculated and compared to national data. Prevalence was calculated for known conditions associated with SCD. The identified prevalence was compared to the established prevalence of conditions associated with SCD. In Hawai'i, the infant SIDS rate (66.4/100,000) was similar to the national rate (54.4/100,000). Over 14 years, only 51 children were diagnosed with a condition associated with SCD; 28 with a cardiomyopathy and 21 with a channelopathy. A 14-year retrospective analysis in the State of Hawai'i revealed that less than 1 in 30 children, who are expected to harbor a SCD-associated condition, had been appropriately diagnosed. The underdiagnosis of conditions associated with SCD reflects that in the absence of a comprehensive screening program, conditions without obvious signs and symptoms are difficult to diagnose. Many children with these conditions will remain at risk of SCD.

Left Ventricular Mechanical Property Changes During Acute AV Synchronous Right Ventricular Pacing in Children.

Prolonged RV pacing is recognized as a cause of LV dysfunction due to dyssynchronous activation. There are no specific longitudinal parameters known to help predict RV pacing-induced LV dysfunction. The aim of the study was to assess the acute effects of AV synchronous RV pacing on LV mechanics using echocardiographic speckle tracking. Nineteen children, aged 6-23 years, underwent echocardiographic evaluation prior to and following elective electrophysiology and ablation studies. The subjects were evaluated in sinus rhythm and later with AV synchronous RV pacing at a cycle length of 550 ms with a short AV delay of 80 ms. The echocardiographic clips were analyzed using speckle tracking methods to calculate LV circumferential and longitudinal strain, rotation and twist in all conditions. Acute RV apical pacing decreased LV longitudinal strain from 16.1 ± 3.7% in sinus rhythm to 14.4 ± 3.3% (p = 0.03) and LV base rotation from -8.4° ± 3.6° to -6.4° ± 4.0° (p = 0.04). The circumferential strain, apical rotation and LV twist were not affected. Separate analysis of subjects with no prior preexcitation showed that acute RV pacing caused significant twist reduction, from 15.9° ± 7.6° to 12.1° ± 7.0° (p = 0.02), and decreased longitudinal strain and base rotation. Patients with preexcitation had abnormalities that persisted acutely after ablation. Acute RV apical pacing causes reductions in LV base rotation, longitudinal strain and twist. The recognition of abnormal LV activation patterns may provide longitudinal clues to LV dysfunction in chronically paced patients and potential novel indices of effective CRT interventions to reverse these abnormalities.

The Electrocardiogram Is a Poor Diagnostic Tool to Detect Left Ventricular Hypertrophy in Children: A Comparison with Echocardiographic Assessment of Left Ventricular Mass.

OBJECTIVE: The electrocardiogram (ECG) is used to detect left ventricular hypertrophy (LVH) in children and young adults, and it is considered an important screening tool for the most common causes of sudden cardiac arrest in the United States. However, the reliability and accuracy of the ECG in the detection of LVH are poorly understood. This study's objective was to assess the reliability and accuracy of ECG parameters predicting LVH in comparison with echocardiographic two-dimensional left ventricular mass (2D-LVM) assessment. DESIGN: Two hundred fifty patients met study criteria with complete 2D-LVM measurements, temporally matched ECGs and no confounding structural heart defects. The echocardiographic diagnosis of LVH was made by 2D-LVM z-score > 2. The electrocardiographic diagnosis of LVH was fulfilled if specific Q, R, or S wave voltages exceeded the normal limits for the respective age groups established by the Davignon, Macfarlane and Rijnbeek datasets, or fulfilled the LVH criteria set forth by adult indices (Sokolow-Lyon, Cornell, Gubner, Romhilt-Estes). RESULTS: None of the ECG parameters showed significant correlation with 2D-LVM z-scores. In the detection of LVH, the most commonly used R in V6 voltages showed the following results: sensitivity 13-29%, specificity 77-96%, positive predictive value (PPV) 29-50%, and negative predictive value (NPV) 77%. The overall accuracy ranged 65-75%. The accuracy of adult indices for LVH ranged from 49% to 78%. CONCLUSIONS: The ECG's prediction of LVH is unreliable with dismal sensitivity, variable specificity, poor PPV, and barely acceptable NPV. With such overall poor reliability, the use of current ECG standards in screening for LVH warrants reassessment.