Long-Term Outcomes and Characteristics Associated With Mortality of Adult Patients Post Fontan Surgery: 27-Year Single-Center Experience.

Data on the characteristics and long-term outcomes of patients who underwent Fontan surgery and surviving into adulthood are limited. We aimed to describe our center's long-term experience with this unique patient population. Included were adult patients who had undergone Fontan surgery and were followed up at our Adult Congenital Heart Disease clinic between the years 1994 and 2021. We describe cardiac and noncardiac morbidities, medical treatment, laboratory data, echocardiographic characteristics, and all-cause mortality. The primary outcome was a composite of heart failure hospitalizations or death. A total of 107 patients who underwent Fontan surgery were followed up during the study period; 46.7% were male. The mean age at time of Fontan was 7.4 ± 6.2 years and the mean age at the last follow-up or at the time of an outcome event was 35.0 ± 8.0 years (range 21.1 to 62.8). At the last documented follow-up, 74.7% of the cohort were in New York Heart Association functional class I/II. The common morbidities included atrial arrythmias (37%) and stroke (17%). The primary outcome occurred in 17.7%. By the end of the study period, 9.3% of the patients in the cohort died. In a multivariate logistic regression analysis, controlling for gender, age, and Fontan type, worse functional class at the last follow-up (New York Heart Association III/IV vs I/II) was significantly associated with the risk of the primary outcome (odds ratio 34.57, 95% confidence interval 6.728 to 177.623, p <0.001). In conclusion, long-term outcomes of patients surviving into adulthood with a Fontan circulation is encouraging. Most of these patients achieve good functional cardiovascular status, despite the complex anatomy and a substantial burden of co-morbid conditions, specifically, atrial arrythmias and thrombotic events. Functional class was independently associated with heart failure hospitalizations and mortality.

Self-Reported Mental and Physical Measures in Adult Fontan Patients.

Introduction: The Fontan procedure is a palliative operation for patients with single functional ventricles, arising from a heterogeneous group of heart defects. There is a considerable gap in evidence regarding the self-reported physical and mental health of these patients surviving to adulthood. Methods and Results: We administered the PROMIS® Global Short Form (v 1.2) to Fontan patients during their scheduled clinic visits during 2017−2018. The raw PROMIS scores were subsequently converted to standardized T-scores, where the mean performance was 50 for the general population. We used Cronbach's alpha to assess reliability, with >0.8 considered good. A total of 42 patients were included. The median age was 30 (IQR: 24−34) years and 59% (95% CI: 43−74%) were female. The median time from birth to operation was 4.5 (IQR: 3−8) years, with 55% having an extracardiac Fontan. The questionnaire had good internal reliability with an alpha of 0.87. Seventy-one percent of respondents rated their overall health as "excellent" or "good". The mean T-score for physical health was 46.6, lower than the age-group mean (51.6, p < 0.001). The mean T-score for mental health was 53.3, higher than the age-group mean (48.5, p < 0.001). T-scores showed strong correlation with each other (r = 0.7) and weak correlation with age and time from procedure. There was no association of T-score with diagnosis or operation type. Conclusions: Adult Fontan patients report better mental health despite worse reporting physical health compared with the age group means. Patient-reported measures can provide clinically meaningful insights about the care of patients with complex congenital heart disease.

CMR Imaging 6 Months After Myocarditis Associated with the BNT162b2 mRNA COVID-19 Vaccine.

Temporal association between BNT162b2 mRNA COVID-19 vaccine and myocarditis (PCVM) has been reported. We herein present early and 6-month clinical follow-up and cardiac magnetic resonance imaging (CMR) of patients with PVCM. A retrospective collection of data from 15 patients with PCVM and abnormal CMR was performed. Clinical manifestation, laboratory data, hospitalizations, treatment protocols, and imaging studies were collected early (up to 2 months) and later. In nine patients, an additional CMR evaluation was performed 6 months after diagnosis. PCVM was diagnosed in 15 patients, mean age 17 ± 1 (median 17.2, range 14.9-19 years) years, predominantly in males. Mean time from vaccination to onset of symptoms was 4.4 ± 6.7 (median 3, range 0-28) days. All patients had CMR post diagnosis at 4 ± 3 (median 3, range 1-9) weeks, 4/5 patients had hyper enhancement on the T2 sequences representing edemaQuery, and 12 pathological Late glandolinium enhancement. A repeat scan performed after 5-6 months was positive for scar formation in 7/9 patients. PCVM is a rare complication, affecting predominantly males and appearing usually within the first week after administration of the second dose of the vaccine. It usually is a mild disease, with clinical resolution with anti-inflammatory treatment. Late CMR follow up demonstrated resolution of the edema in all patients, while some had evidence of residual myocardial scarring.

Health Service Utilization Patterns Among Adults With Congenital Heart Disease: A Population-Based Study.

Background Several studies have examined hospitalizations among patients with adult congenital heart disease (ACHD). Few investigated other services or utilization patterns. Our aim was to study service utilization patterns and predictors among patients with ACHD. Methods and Results We identified 11 653 patients with ACHD aged ≥18 years (median, 47 years), through electronic records of 2 large Israeli healthcare providers (2007-2011). The association between patient, disease, and sociogeographic characteristics and healthcare resource utilization were modeled as recurrent events accounting for the competing death risk. Patients with ACHD had high healthcare utilization rates compared with the general population. The highest standardized service utilization ratios (SSRs) were found among patients with complex congenital heart disease including primary care visits (SSR, 1.53; 95% CI, 1.47-1.58), cardiology outpatient visits (SSR, 5.17; 95% CI, 4.69-5.64), hospitalizations (SSR, 6.68; 95% CI, 5.82-7.54), and days in hospital (SSR, 15.37; 95% CI, 14.61-16.12). Adjusted resource utilization hazard increased with increasing lesion complexity. Hazard ratios (HRs) for complex versus simple disease were: primary care (HR, 1.14; 95% CI, 1.06-1.23); cardiology outpatient visits (HR, 1.40; 95% CI, 1.24-1.59); emergency department visits (HR, 1.19; 95% CI, 1.02-1.39); and hospitalizations (HR, 1.75; 95% CI, 1.49-2.05). Effects attenuated with age for cardiology outpatient visits and hospitalizations and increased for emergency department visits. Female sex, geographic periphery, and ethnic minority were associated with more primary care visits, and female sex (HR versus men, 0.89 [95% CI, 0.84-0.94]) and periphery (HR, 0.72 [95% CI, 0.58-0.90] for very peripheral versus very central) were associated with fewer cardiology visits. Arab minority patients also had high hospitalization rates compared with the majority group of Jewish or other patients. Conclusions Healthcare utilization rates were high among patients with ACHD. Female sex, geographic periphery, and ethnicity were associated with less optimal service utilization patterns. Further research should examine strategies to optimize service utilization in these groups.

Depression and anxiety are associated with high health care utilization and mortality among adults with congenital heart disease.

BACKGROUND: The significance of depression/anxiety among ACHD patients in terms of health care utilization is unknown and data on the association with mortality are scarce. METHODS: Analyses comprised 8334 ACHD patients, age ≥ 18 years, insured by a large healthcare organization (2007-2011). Depression/anxiety were determined by diagnoses and treatments recorded in the organization database. Adjusted utilization relative rates (RRs) were estimated with negative binomial models and mortality hazard ratios (HRs) with the Cox proportional hazard model. RESULTS: ACHD patients with depression/anxiety (N = 2950, 35%) were more likely to be older (mean ±â€¯SD: 54 ±â€¯17 vs. 45 ±â€¯18 years), women (61% vs. 45%), and have comorbidities than counterparts without depression/anxiety. Following multivariable adjustment, patients with depression/anxiety had more primary care and cardiology clinic visits, more emergency department visits and more hospitalizations. RRs (95% confidence interval) were: 1.31 (1.27-1.35); 1.07 (1.01-1.13); 1.60 (1.46-1.77); and 1.18 (1.08-1.29) respectively, for diagnosis before the study period, and 1.36 (1.31-1.42); 1.22 (1.14-1.30); 1.43 (1.24-1.60) and 1.47 (1.33-1.64), respectively, for diagnosis during the study. Stratifying by age, the highest adjusted primary care and cardiology visit RRs were found among 18-24 years old patients and the lowest among patients ≥65 years. Between 2007 and 2017, 905 patients died. Depression/anxiety were associated with increased mortality risk with adjusted HRs: 1.10 (95% CI: 0.94-1.29) for past diagnosis and 1.40 (1.17-1.67) for study period depression/anxiety diagnosis. CONCLUSIONS: Depression/anxiety in ACHD patients is associated with increased health-care utilization and a higher risk of death. The efficacy of addressing patients' psychosocial needs in optimizing health-care utilization and improving prognosis needs further evaluation.

Prenatal diagnosis and outcome of right aortic arch without significant intracardiac anomaly.

BACKGROUND: Right aortic arch (RAA) is usually associated with the presence of a significant congenital heart disease, usually a conotruncal defect, which determines the postnatal outcome. In the absence of such cardiac defects, the significance of RAA has not been determined. The aims of this study were to evaluate the significance of recognizing RAA in fetuses with normal or near normal intracardiac anatomy and to determine which associations may be present. METHODS: A retrospective study was completed of all fetuses diagnosed with RAA with normal or near normal intracardiac anatomy between 1999 and 2011. The aim was to evaluate the presence of RAA with complete ultrasonic evaluation using two-dimensional imaging complemented by the Doppler color flow technique, paying particular attention to the three-vessel and tracheal view. We compared the prenatal findings with the postnatal outcomes and management of this cohort of fetuses. RESULTS: Among 16,450 fetal echocardiograms, 58 fetuses (0.35%) were diagnosed with RAA with normal or near normal intracardiac anatomy. Gestational age at diagnosis ranged from 19 to 34 weeks (mean, 23 weeks). Isolated RAAs were found in 50 fetuses, and double aortic arches (DAAs) were recognized in eight other cases. The postnatal cohort consisted of 44 newborns with RAAs and eight with DAAs (two were lost to follow-up, and four pregnancies were terminated). Postnatal echocardiography confirmed the prenatal diagnosis of RAA in 41 of 45 children, and four were found to have DAAs. Three of seven fetuses diagnosed prenatally as having DAAs were found to have only RAAs. Fourteen fetuses underwent karyotyping; two had 22q11 deletion and two had 47xxy. Eleven infants (21%) had respiratory symptoms, eight with DAAs, one with RAA, mirror-image head and neck vessels, and two with RAAs and aberrant left subclavian arteries. Surgery was indicated in all symptomatic patients except one, whose symptoms resolved. One asymptomatic patient underwent operation for significant compression of the trachea. CONCLUSIONS: RAA on fetal ultrasonography may indicate vascular and chromosomal abnormalities that may complicate postnatal management. When RAA is identified, fetal karyotype analysis (including the integrity of chromosome 22) is warranted. RAA may herald an occult DAA and may be a clue to a tight vascular ring. Hence, it seems essential to conduct a careful postnatal evaluation of fetuses with RAAs on prenatal ultrasound.

Cerebrospinal fluid lactate dehydrogenase isoenzymes in children with bacterial and aseptic meningitis.

Differentiation of bacterial from aseptic meningitis may be difficult. Our aim was to determine the pattern of distribution of lactate dehydrogenase (LDH) isoenzymes in the cerebrospinal fluid (CSF) of patients with bacterial and aseptic meningitis. One hundred and fifty-seven patients with suspected meningitis were enrolled in the study. They were divided into 3 groups according to the culture- or bacterial antigen assay-proven diagnosis and CSF findings: bacterial meningitis (n = 31), aseptic meningitis (n = 65), and non-meningitis (n = 61). Total LDH level and percentages of LDH isoenzymes in the CSF were measured in each patient. Each group showed a distinct LDH isoenzyme distribution pattern, with a statistically significant difference among the groups in the percentages of the various isoenzymes. Compared with the non-meningitis group, total LDH activity in the CSF was high in the aseptic meningitis group (49.82+/-35.59 U/L, P < 0.001) and exaggerated in the bacterial meningitis group (944.53+/-112.3 U/L, P < 0.001). Low LDH-2 levels were unique to bacterial meningitis (P < 0.01), whereas high LDH-3 levels were characteristic of aseptic meningitis (P < 0.05). Both groups had low levels of LDH-1 and high levels of LDH-4 and LDH-5. In conclusion, the LDH isoenzyme pattern may be of clinical diagnostic value in meningitis, particularly when culture results are pending.

Effect of educational intervention on antibiotic prescription practices for upper respiratory infections in children: a multicentre study.

OBJECTIVES: To evaluate the impact of an educational intervention on judicious antibiotic prescription for upper respiratory diseases in children. METHODS: A multicentre before-and-after study was conducted in five major community child healthcentres in Israel. Antibiotic prescription data were collected for all visits of patients aged 3 months to 18 years with a diagnosis of acute otitis media, tonsillopharyngitis, sinusitis or upper respiratory tract infection from November 1999 through February 2000 (pre-intervention period) and from November 2000 through February 2001 (post-intervention period). The intervention consisted of a 1 day seminar on the diagnosis and judicious treatment of respiratory tract infections in children according to the recommendations of the Centers of Disease Control and Prevention. The patient files were reviewed for patient characteristics, specific respiratory disease, and specific antibiotics prescribed. The main outcome measures were the rates and appropriateness of antibiotic prescribing for the different respiratory diseases before and after an educational intervention for practising paediatricians. RESULTS: A total of 4580 clinic visits were eligible for analysis in the pre-intervention period and 4364 in the post-intervention period. From the pre- to the post-intervention period, the odds ratio for appropriate antibiotic treatment was 1.8 for acute otitis media (95% CI 1.52-2.11, P < 0.01) and 1.35 for pharyngitis (95% CI 1.13-1.61, P < 0.01). Overall, use of antibiotics for acute otitis media decreased from 93% to 87.4% (P < 0.05), and for upper respiratory tract infection, from 13.8% to 11.5% (P < 0.05). There were no significant changes in these factors for sinusitis. CONCLUSIONS: A targeted educational intervention can improve antibiotic prescription practices for respiratory infections in children and decrease unnecessary antibiotic use. Such studies can also pinpoint areas that require further attention.

Recombinant factor VIIa (NovoSeven) as a hemostatic agent after surgery for congenital heart disease.

BACKGROUND: Postoperative bleeding and blood product requirements can be substantial in children undergoing open-heart surgery, and reexploration is required in 1% of cases. Recombinant activated factor VII (rFVIIa, NovoSeven, NovoNordisk, Denmark) is a hemostatic agent approved for the treatment of hemophilic patients with inhibitors to factor VIII or factor IX. It has also been used with success in other conditions. We present our experience with rFVIIa treatment for uncontrolled bleeding after open-heart surgery in five pediatric patients. METHODS: The study group consisted of five patients after open-heart surgery with excessive blood loss. The patients were treated with rFVIIa after failure of conventional treatment to control the bleeding. Blood loss, blood product consumption, and coagulation test results were recorded before and after rFVIIa administration. RESULTS: In all cases, blood loss decreased considerably after rFVIIa administration (mean 7.8 ml x kg(-1) x h(-1)), almost eliminating the need for additional blood products, and the prolonged prothrombin time normalized. In two patients with thrombocytopathy, rFVIIa helped to discriminate surgical bleeding from bleeding caused by a defect in hemostasis. No side effects of rFVIIa treatment were noted. CONCLUSIONS: These cases support the impression that RFVIIa is efficient and safe in correcting hemostasis in children after cardiopulmonary bypass when other means fail. However, the data are still limited, and more extensive research is needed.