Adherence to clinical practice guidelines for pulmonary valve intervention after tetralogy of Fallot repair: A nationwide cohort study.

Objectives: To determine guideline adherence pertaining to pulmonary valve replacement (PVR) referral after tetralogy of Fallot (TOF) repair. Methods: Children and adults with cardiovascular magnetic resonance imaging scans and at least moderate pulmonary regurgitation were prospectively enrolled in the Comprehensive Outcomes Registry Late After TOF Repair (CORRELATE). Individuals with previous PVR were excluded. Patients were classified according to presence (+) versus absence (-) of PVR and presence (+) versus absence (-) of contemporaneous guideline satisfaction. A validated score (specific activity scale [SAS]) classified adult symptom status. Results: In total, 498 participants (57% male, mean age 32 ± 14 years) were enrolled from 14 Canadian centers (2013-2020). Mean follow-up was 3.8 ± 1.8 years. Guideline criteria for PVR were satisfied for the majority (n = 422/498, 85%), although referral for PVR occurred only in a minority (n = 167/498, 34%). At PVR referral, most were asymptomatic (75% in SAS class 1). One participant (0.6%) received PVR without meeting criteria (PVR+/indication-). The remainder (n = 75/498, 15%) did not meet criteria for and did not receive PVR (PVR-/indication-). Abnormal cardiovascular imaging was the most commonly cited indication for PVR (n = 61/123, 50%). The SAS class and ratio of right to left end-diastolic volumes were independent predictors of PVR in a multivariable analysis (hazard ratio, 3.33; 95% confidence interval, 1.92-5.8, P < .0001; hazard ratio, 2.78; 95% confidence interval, 2.18-3.55, P < .0001). Conclusions: Although a majority of patients met guideline criteria for PVR, only a minority were referred for intervention. Abnormal cardiovascular imaging was the most common indication for referral. Further research will be necessary to establish the longer-term clinical impact of varying PVR referral strategies.

Kawasaki Disease in the Time of COVID-19 and MIS-C: The International Kawasaki Disease Registry.

BACKGROUND: Patients with multisystem inflammatory syndrome in children (MIS-C) and Kawasaki disease (KD) have overlapping clinical features. We compared demographics, clinical presentation, management, and outcomes of patients according to evidence of previous SARS-CoV-2 infection. METHODS: The International Kawasaki Disease Registry (IKDR) enrolled KD and MIS-C patients from sites in North, Central, and South America, Europe, Asia, and the Middle East. Evidence of previous infection was defined as: Positive (household contact or positive polymerase chain reaction [PCR]/serology), Possible (suggestive clinical features of MIS-C and/or KD with negative PCR or serology but not both), Negative (negative PCR and serology and no known exposure), and Unknown (incomplete testing and no known exposure). RESULTS: Of 2345 enrolled patients SARS-CoV-2 status was Positive for 1541 (66%) patients, Possible for 89 (4%), Negative for 404 (17%) and Unknown for 311 (13%). Clinical outcomes varied significantly among the groups, with more patients in the Positive/Possible groups presenting with shock, having admission to intensive care, receiving inotropic support, and having longer hospital stays. Regarding cardiac abnormalities, patients in the Positive/Possible groups had a higher prevalence of left ventricular dysfunction, and patients in the Negative and Unknown groups had more severe coronary artery abnormalities. CONCLUSIONS: There appears to be a spectrum of clinical features from MIS-C to KD with a great deal of heterogeneity, and one primary differentiating factor is evidence for previous acute SARS-CoV-2 infection/exposure. SARS-CoV-2 Positive/Possible patients had more severe presentations and required more intensive management, with a greater likelihood of ventricular dysfunction but less severe coronary artery adverse outcomes, in keeping with MIS-C.

Determinants and Clinical Outcomes of Patients With Tetralogy of Fallot Lost to Cardiology Follow-up.

BACKGROUND: Various rates of loss to follow-up (LTFU) have been reported in patients with congenital heart disease, but return to follow-up is rarely considered in those analyses. Outcomes of LTFU patients are difficult to assess because the patients no longer attend cardiac care. We leveraged data from the TRIVIA cohort, which combines more than 30 years of clinical and administrative data, allowing us to study outcomes even after LTFU. METHODS: This population-based cohort included 904 patients with tetralogy of Fallot (TOF) born from 1982 to 2015 in Québec, Canada. Risk factors for LTFU and outcomes were calculated by Cox models and marginal means/rates models. Outcomes of LTFU patients were compared with propensity score-matched non-LTFU patients. RESULTS: The cumulative risk of experiencing 1 episode of LTFU was 50.3% at 30 years. However, return to follow-up was frequent and the proportion of patients actively followed was 85.9% at 10 years, 76.4% at 20 years, and 70.6% at 30 years. Factors associated with a reduced risk of LTFU were primary repair with conduit (hazard ratio [HR] 0.29, 95% confidence interval [CI] 0.15-0.58) and transannular patch (HR 0.60, 95% CI 0.46-0.79). LTFU patients had lower rates of cardiac hospitalisations (HR 0.49, 95% CI 0.42-0.56) and cardiac interventions (HR 0.32, 95% CI 0.25-0.42), but similar rates of cardiac mortality (HR 0.95, 95% CI 0.24-3.80). CONCLUSIONS: There was a lower proportion of LTFU patients compared with previous studies. Factors associated with lower rates of LTFU were conduits and non-valve-sparing surgery. LTFU patients had lower rates of cardiac procedures and cardiac hospitalisations.

Obesity and Outcomes of Kawasaki Disease and COVID-19-Related Multisystem Inflammatory Syndrome in Children.

Importance: Obesity may affect the clinical course of Kawasaki disease (KD) in children and multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19. Objective: To compare the prevalence of obesity and associations with clinical outcomes in patients with KD or MIS-C. Design, Setting, and Participants: In this cohort study, analysis of International Kawasaki Disease Registry (IKDR) data on contemporaneous patients was conducted between January 1, 2020, and July 31, 2022 (42 sites, 8 countries). Patients with MIS-C (defined by Centers for Disease Control and Prevention criteria) and patients with KD (defined by American Heart Association criteria) were included. Patients with KD who had evidence of a recent COVID-19 infection or missing or unknown COVID-19 status were excluded. Main Outcomes and Measures: Patient demographic characteristics, clinical features, disease course, and outcome variables were collected from the IKDR data set. Using body mass index (BMI)/weight z score percentile equivalents, patient weight was categorized as normal weight (BMI <85th percentile), overweight (BMI ≥85th to <95th percentile), and obese (BMI ≥95th percentile). The association between adiposity category and clinical features and outcomes was determined separately for KD and MIS-C patient groups. Results: Of 1767 children, 338 with KD (median age, 2.5 [IQR, 1.2-5.0] years; 60.4% male) and 1429 with MIS-C (median age, 8.7 [IQR, 5.3-12.4] years; 61.4% male) were contemporaneously included in the study. For patients with MIS-C vs KD, the prevalence of overweight (17.1% vs 11.5%) and obesity (23.7% vs 11.5%) was significantly higher (P < .001), with significantly higher adiposity z scores, even after adjustment for age, sex, and race and ethnicity. For patients with KD, apart from intensive care unit admission rate, adiposity category was not associated with laboratory test features or outcomes. For patients with MIS-C, higher adiposity category was associated with worse laboratory test values and outcomes, including a greater likelihood of shock, intensive care unit admission and inotrope requirement, and increased inflammatory markers, creatinine levels, and alanine aminotransferase levels. Adiposity category was not associated with coronary artery abnormalities for either MIS-C or KD. Conclusions and Relevance: In this international cohort study, obesity was more prevalent for patients with MIS-C vs KD, and associated with more severe presentation, laboratory test features, and outcomes. These findings suggest that obesity as a comorbid factor should be considered at the clinical presentation in children with MIS-C.

Cardiac Biomarkers Aid in Differentiation of Kawasaki Disease from Multisystem Inflammatory Syndrome in Children Associated with COVID-19.

Kawasaki disease (KD) and Multisystem Inflammatory Syndrome in Children (MIS-C) associated with COVID-19 show clinical overlap and both lack definitive diagnostic testing, making differentiation challenging. We sought to determine how cardiac biomarkers might differentiate KD from MIS-C. The International Kawasaki Disease Registry enrolled contemporaneous KD and MIS-C pediatric patients from 42 sites from January 2020 through June 2022. The study population included 118 KD patients who met American Heart Association KD criteria and compared them to 946 MIS-C patients who met 2020 Centers for Disease Control and Prevention case definition. All included patients had at least one measurement of amino-terminal prohormone brain natriuretic peptide (NTproBNP) or cardiac troponin I (TnI), and echocardiography. Regression analyses were used to determine associations between cardiac biomarker levels, diagnosis, and cardiac involvement. Higher NTproBNP (≥ 1500 ng/L) and TnI (≥ 20 ng/L) at presentation were associated with MIS-C versus KD with specificity of 77 and 89%, respectively. Higher biomarker levels were associated with shock and intensive care unit admission; higher NTproBNP was associated with longer hospital length of stay. Lower left ventricular ejection fraction, more pronounced for MIS-C, was also associated with higher biomarker levels. Coronary artery involvement was not associated with either biomarker. Higher NTproBNP and TnI levels are suggestive of MIS-C versus KD and may be clinically useful in their differentiation. Consideration might be given to their inclusion in the routine evaluation of both conditions.

Long-Term Consequences of COVID-19 in Predominantly Immunonaive Patients: A Canadian Prospective Population-Based Study.

Background: Lingering symptoms are frequently reported after acute SARS-CoV-2 infection, a condition known as post-COVID-19 condition (PCC). The duration and severity of PCC in immunologically naïve persons remain unclear. Furthermore, the long-term consequences of these chronic symptoms on work and mental health are poorly documented. Objective: To determine the outcome, the risk factors, and the impact on work and mental health associated with post-COVID-19 symptoms. Methods: This prospective population-based study assessed acute COVID-19 symptoms and their evolution for up to nine months following infection. Individuals aged 18 years and older with COVID-19 in three Canadian regions between 1 November 2020 and 31 May 2021 were recruited. Participants completed a questionnaire that was either administered by trained student investigators over the phone or self-administered online. Results: A total of 1349 participants with a mean age of 46.6 ± 16.0 years completed the questionnaire. Participants were mostly unvaccinated at the time of their COVID-19 episode (86.9%). Six hundred and twenty-two participants (48.0%) exhibited one symptom or more, at least three months post-COVID-19. Among participants with PCC, 23.0% to 37.8% experienced fatigue at the time of survey. Moreover, 6.1% expressed psychological distress. Risk factors for PCC and fatigue included female sex (OR = 1.996), higher number of symptoms (OR = 1.292), higher severity of episode (OR = 3.831), and having a mental health condition prior to the COVID-19 episode (OR = 5.155). Conclusions: In this multicenter cohort study, almost half (47%) of the participants reported persistent symptoms >3 months after acute infection. Baseline risk factors for PCC include female sex, number and severity of symptoms during acute infection, and a previous diagnosis of mental health disorder. Having PCC negatively impacted health-related quality of life and these patients were more likely to exhibit psychological distress, as well as fatigue.

Patient-Reported Outcomes After Tetralogy of Fallot Repair.

BACKGROUND: Comprehensive assessment of tetralogy of Fallot (TOF) outcomes extends beyond morbidity and mortality to incorporate patient-reported outcomes (PROs), including quality of life (QOL) and health status (HS). OBJECTIVES: This study explored PROs in adolescents and adults with TOF and delineated variables associated with PROs. METHODS: This was a cross-sectional observational study within a larger prospective registry of adolescents and adults with repaired TOF and moderate or greater pulmonary regurgitation from North America, Europe, and Asia. Participants completed PROs, including a QOL linear analogue scale (QOL-LAS) and an HS visual analogue scale (HS-VAS). Scores were classified according to age cohorts: <18, 18 to 25, 26 to 40, and >40 years. RESULTS: The study included 607 patients (46.3% female; median age 28.5 years). Median QOL-LAS scores (0-100) were similar across age cohorts (85, 80, 80, 80; P = 0.056). Median HS-VAS scores (0-100) were lowest for the oldest cohort (77) compared with the 3 younger cohorts (85, 80, 80) (P = 0.004). With advancing age, there were increased reports of poor mobility (P < 0.001) and pain or discomfort (P = 0.004); problems in these dimensions were reported by 19.1% and 37.2% of patients aged >40 years, respectively. Of factors associated with superior PROs on multivariable regression modeling (ie, being White, being nonsyndromic, having employment, and having better left ventricular function; P < 0.05), asymptomatic status (functional class I) was the variable associated with the greatest number of QOL and HS measures (P < 0.001). CONCLUSIONS: Strategies to improve TOF outcomes should consider PROs alongside conventional clinical variables. Factors associated with poorer PROs represent opportunities to intervene to improve the lives of patients with TOF.

Single-drug immunosuppression is associated with noninferior medium-term survival in pediatric heart transplant recipients.

BACKGROUND: Patients are usually maintained on at least 2 immunosuppressive drugs (ISDs) after the first year post heart transplant. Anecdotally, some children are switched to single-drug monotherapy (a single ISD) for various reasons and varying durations. Outcomes associated with differences in immunosuppression after heart transplantation are unknown for children. OBJECTIVES: A priori we defined a noninferiority hypothesis for monotherapy compared to ≥2 ISDs. The primary outcome was graft failure, a composite of death and retransplantation. Secondary outcomes included rejection, infection, malignancy, cardiac allograft vasculopathy and dialysis. METHODS: This international, multicenter, retrospective, observational cohort study used data from the Pediatric Heart Transplant Society. We included patients who underwent first-time heart transplant <18 years of age between 1999 and 2020 with ≥1 year of follow-up data available. RESULTS: Our analysis included 3493 patients with a median time post-transplant of 6.7 years. There were 893 patients (25.6%) switched to monotherapy at least once with the remaining 2600 patients always on ≥2 ISDs. The median time on monotherapy after the first year post-transplant was 2.8 years (range 1.1-5.9 years). We found an adjusted hazard ratio (HR) of 0.65 (95%CI: 0.47-0.88) favoring monotherapy compared to ≥2 ISDs (p = 0.002). There were no meaningful differences in the incidence of secondary outcomes between groups, except for a lower rate of cardiac allograft vasculopathy in patients on monotherapy (HR 0.58, 95%CI: 0.45-0.74). CONCLUSIONS: For pediatric heart transplant recipients placed on monotherapy, immunosuppression with a single ISD after the first year post-transplant was noninferior to standard therapy with ≥2 ISDs in the medium term. CONDENSED ABSTRACT: Some children are switched to a single immunosuppressive drug (ISD) for various reasons after heart transplant, but outcomes associated with differences in immunosuppression are unknown for children. We assessed graft failure in children on a single ISD (monotherapy) compared to ≥2 ISDs in a cohort of 3493 children with a first heart transplant. We found an adjusted hazard ratio of 0.65 (95%CI: 0.47-0.88) favoring monotherapy. We concluded that for pediatric heart transplant recipients placed on monotherapy, immunosuppression with a single ISD after the first year post-transplant was non-inferior to standard therapy with ≥2 ISDs in the medium term.

COVID-19 Positive Versus Negative Complete Kawasaki Disease: A Study from the International Kawasaki Disease Registry.

To determine clinical differences for children with complete Kawasaki disease (KD) with and without evidence of preceding SARS-CoV-2 infection. From January 2020, contemporaneous patients with complete KD criteria were classified as either SARS-CoV-2 positive (KDCOVID+; confirmed household exposure, positive PCR and/or serology) or SARS-CoV-2 negative (KDCOVID-; negative testing and no exposure) and compared. Of 744 patients in the International Kawasaki Disease Registry, 52 were KDCOVID- and 61 were KDCOVID+. KDCOVID+ patients were older (median 5.5 vs. 3.7 years; p < 0.001), and all additionally met diagnostic criteria for multisystem inflammatory syndrome in children (MIS-C). They were more likely to have abdominal pain (60% vs. 35%; p = 0.008) and headache (38% vs. 10%; p < 0.001) and had significantly higher CRP, troponin, and BUN/creatinine, and lower hemoglobin, platelets, and lymphocytes. KDCOVID+ patients were more likely to have shock (41% vs. 6%; p < 0.001), ICU admission (62% vs. 10%; p < 0.001), lower left ventricular ejection fraction (mean lowest LVEF 53% vs. 60%; p < 0.001), and to have received inotropic support (60% vs. 10%; p < 0.001). Both groups received IVIG (2 doses in 22% vs. 18%; p = 0.63), but KDCOVID+ were more likely to have received steroids (85% vs. 35%; p < 0.001) and anakinra (60% vs. 10%; p = 0.002). KDCOVID- patients were more likely to have medium/large coronary artery aneurysms (CAA, 12% vs. 0%; p = 0.01). KDCOVID+ patients differ from KDCOVID-, have more severe disease, and greater evidence of myocardial involvement and cardiovascular dysfunction rather than CAA. These patients may be a distinct KD phenotype in the presence of a prevalent specific trigger.

Generation of functional human T cell development in NOD/SCID/IL2rγnull humanized mice without using fetal tissue: Application as a model of HIV infection and persistence.

Humanization of mice with functional T cells currently relies on co-implantation of hematopoietic stem cells from fetal liver and autologous fetal thymic tissue (so-called BLT mouse model). Here, we show that NOD/SCID/IL2rγnull mice humanized with cord blood- derived CD34+ cells and implanted with allogeneic pediatric thymic tissues excised during cardiac surgeries (CCST) represent an alternative to BLT mice. CCST mice displayed a strong immune reconstitution, with functional T cells originating from CD34+ progenitor cells. They were equally susceptible to mucosal or intraperitoneal HIV infection and had significantly higher HIV-specific T cell responses. Antiretroviral therapy (ART) robustly suppressed viremia and reduced the frequencies of cells carrying integrated HIV DNA. As in BLT mice, we observed a complete viral rebound following ART interruption, suggesting the presence of HIV reservoirs. In conclusion, CCST mice represent a practical alternative to BLT mice, broadening the use of humanized mice for research.

Incremental Detection of Severe Congenital Heart Disease by Fetal Echocardiography Following a Normal Second Trimester Ultrasound Scan in Québec, Canada.

BACKGROUND: The benefit of fetal echocardiograms (FE) to detect severe congenital heart diseases (SCHD) in the setting of a normal second-trimester ultrasound is unclear. We aimed to assess whether the increase in SCHD detection rates when FE are performed for risk factors in the setting of a normal ultrasound was clinically significant to justify the resources needed. METHODS: This is a multicenter, population-based, retrospective cohort study, including all singleton pregnancies and offspring in Quebec (Canada) between 2007 and 2015. Administrative health care data were linked with FE clinical data to gather information on prenatal diagnosis of CHD, indications for FE, outcomes of pregnancy and offspring, postnatal diagnosis of CHD, cardiac interventions, and causes of death. The difference between the sensitivity to detect SCHD with and without FE for risk factors was calculated using generalized estimating equations with a noninferiority margin of 5 percentage points. RESULTS: A total of 688 247 singleton pregnancies were included, of which 30 263 had at least one FE. There were 1564 SCHD, including 1071 that were detected prenatally (68.5%). There were 12 210 FE performed for risk factors in the setting of a normal second-trimester ultrasound, which led to the detection of 49 additional cases of SCHD over 8 years. FE referrals for risk factors increased sensitivity by 3.1 percentage points (95% CI, 2.3-4.0; P<0.0001 for noninferiority). CONCLUSIONS: In the setting of a normal second-trimester ultrasound, adding a FE for risk factors offered low incremental value to the detection rate of SCHD in singleton pregnancies. The current ratio of clinical gains versus the FE resources needed to screen for SCHD in singleton pregnancies with isolated risk factors does not seem favorable. Further studies should evaluate whether these resources could be better allocated to increase SCHD sensitivity at the ultrasound level, and to help decrease heterogeneity between regions, institutions and operators.

Distinctive Roles of Furin and TMPRSS2 in SARS-CoV-2 Infectivity.

The spike protein (S) of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) directs infection of the lungs and other tissues following its binding to the angiotensin-converting enzyme 2 (ACE2) receptor. For effective infection, the S protein is cleaved at two sites: S1/S2 and S2'. The "priming" of the surface S protein at S1/S2 (PRRAR685↓) [the underlined basic amino acids refer to critical residues needed for the furin recognition] by furin has been shown to be important for SARS-CoV-2 infectivity in cells and small-animal models. In this study, for the first time we unambiguously identified by proteomics the fusion activation site S2' as KPSKR815↓ (the underlined basic amino acids refer to critical residues needed for the furin recognition) and demonstrated that this cleavage was strongly enhanced by ACE2 engagement with the S protein. Novel pharmacological furin inhibitors (BOS inhibitors) effectively blocked endogenous S protein processing at both sites in HeLa cells, and SARS-CoV-2 infection of lung-derived Calu-3 cells was completely prevented by combined inhibitors of furin (BOS) and type II transmembrane serine protease 2 (TMPRSS2) (camostat). Quantitative analyses of cell-to-cell fusion and S protein processing revealed that ACE2 shedding by TMPRSS2 was required for TMPRSS2-mediated enhancement of fusion in the absence of S1/S2 priming. We further demonstrated that the collectrin dimerization domain of ACE2 was essential for the effect of TMPRSS2 on cell-to-cell fusion. Overall, our results indicate that furin and TMPRSS2 act synergistically in viral entry and infectivity, supporting the combination of furin and TMPRSS2 inhibitors as potent antivirals against SARS-CoV-2. IMPORTANCE SARS-CoV-2, the etiological agent of COVID-19, has so far resulted in >6.1 million deaths worldwide. The spike protein (S) of the virus directs infection of the lungs and other tissues by binding the angiotensin-converting enzyme 2 (ACE2) receptor. For effective infection, the S protein is cleaved at two sites: S1/S2 and S2'. Cleavage at S1/S2 induces a conformational change favoring the S protein recognition by ACE2. The S2' cleavage is critical for triggering membrane fusion and virus entry into host cells. Our study highlights the complex dynamics of interaction between the S protein, ACE2, and the host proteases furin and TMPRSS2 during SARS-CoV-2 entry and suggests that the combination of a nontoxic furin inhibitor with a TMPRSS2 inhibitor significantly reduces viral entry in lung cells, as evidenced by an average synergistic ∼95% reduction of viral infection. This represents a powerful novel antiviral approach to reduce viral spread in individuals infected by SARS-CoV-2 or future related coronaviruses.

Diagnostic Efficacy of a Single-Lead Ambulatory 14-Day ECG Monitor in Symptomatic Children.

BACKGROUND: The CardioSTAT is a single-lead ambulatory electrocardiography monitor that has been validated for use in adult patients. Recording is made through 2 electrodes positioned in a lead-I configuration, and the device allows monitoring for 2, 7, or 14 days. We sought to investigate the efficacy of this device in children with paroxysmal palpitations. METHODS: In phase I, the quality of tracings from simultaneous CardioSTAT recordings and D1-lead recordings of a standard 12-lead electrocardiography machine in 23 children were compared. Phase II was a prospective observational cohort study comparing arrhythmia detection using the CardioSTAT vs currently used devices (24-hour Holter monitor and the Cardiomemo loop recorder) in 52 children complaining of palpitations. RESULTS: In Phase I, all but 3 rhythm strips were correctly identified. The pacing spikes on 3 strips were not adequately identified by the observers for the CardioSTAT recording. In Phase II, symptomatic episodes were reported in 42%, 73%, and 100% of subjects during monitoring with the Holter, Cardiomemo, and CardioSTATdevices, respectively. An abnormal rhythm was detected in 13%, 23%, and 35% of subjects by the Holter, Cardiomemo, and CardioSTAT monitors, respectively. The underlying rhythm during symptomatic events was determined in 90% of cases with the CardioSTAT monitor, whereas it was determined in only 19% and 29% of cases using the Holter and Cardiomemo monitors, respectively. CONCLUSIONS: The CardioSTAT monitor provided good-quality tracings and was superior to the 24-hour Holter monitor and the Cardiomemo loop recorder in determining the presence or absence of pathologic arrhythmia in the study cohort.

CONTEXTE: Le CardioSTAT est un moniteur d'électrocardiographie ambulatoire à dérivation unique dont l'utilisation a été validée chez les patients adultes. L'enregistrement se fait au moyen de 2 électrodes positionnées dans une configuration de type « dérivation I ¼, et l'appareil permet un suivi pendant 2, 7 ou 14 jours. Nous avons cherché à étudier l'efficacité de cet appareil chez les enfants atteints de palpitations paroxystiques. MÉTHODOLOGIE: Dans la phase I, la qualité des tracés provenant d'enregistrements simultanés de CardioSTAT et d'enregistrements de la dérivation I d'un appareil d'électrocardiographie standard à 12 dérivations a été comparée chez 23 enfants. La phase II était une étude de cohorte observationnelle prospective comparant la détection de l'arythmie à l'aide du moniteur CardioSTAT par rapport aux appareils utilisés actuellement (moniteur Holter à surveillance sur 24 heures et enregistreur d'événements portatif Cardiomémo) chez 52 enfants se plaignant de palpitations. RÉSULTATS: Dans la phase I, toutes les bandes d'enregistrement sauf trois ont été correctement identifiées. Les spicules de stimulation n'ont pas été correctement détectés par les observateurs sur trois bandes d'enregistrement du moniteur CardioSTAT. Dans la phase II, des épisodes symptomatiques ont été signalés chez 42 %, 73 % et 100 % des sujets pendant la surveillance avec les appareils Holter, Cardiomémo et CardioSTAT, respectivement. Un rythme anormal a été détecté chez 13 %, 23 % et 35 % des sujets par les moniteurs Holter, Cardiomémo et CardioSTAT, respectivement. Le rythme sous-jacent pendant les événements symptomatiques a été déterminé dans 90 % des cas avec le moniteur CardioSTAT, alors qu'il n'a été déterminé que dans 19 % et 29 % des cas, respectivement, avec les moniteurs Holter et Cardiomémo. CONCLUSIONS: Le moniteur CardioSTAT a fourni des tracés de bonne qualité et s'est révélé supérieur à l'appareil Holter à surveillance sur 24 heures et à l'enregistreur d'événements portatif Cardiomémo pour déterminer la présence ou l'absence d'arythmie pathologique dans la cohorte étudiée.

The Mathematical Limitations of Fetal Echocardiography as a Screening Tool in the Setting of a Normal Second-Trimester Ultrasound.

BACKGROUND: The effectiveness of screening strategies targeting pregnancies at higher risk of congenital heart disease (CHD) is reduced by the low prevalence of severe CHD, the increase in CHD detection rates by second-trimester ultrasound (U/S), and the high proportion of severe CHD in low-risk pregnancies. We aimed to determine situations in which additional screening by fetal echocardiography (FE) would result in a significant increase in sensitivity and a sizable decrease in the false-negative rate of detection of severe CHD. METHODS: We simulated the change in the numbers of detected severe CHD cases when FE is offered to women with a normal second-trimester U/S who have a higher risk of bearing a child with CHD, compared to U/S alone. The primary outcome was the increase in sensitivity. Secondary outcomes were the number needed to screen and the reduction in the rate of missed cases. RESULTS: For an U/S sensitivity of 60%, the addition of FE in pregnancies at high risk of CHD (risk ratio 3.5; range: 2 to 5) increased sensitivity by 2.4 percentage points (1.1 to 7.9). The number needed to screen to detect one additional case of severe CHD was 436 (156 to 952). The rate of additional severe CHD cases detected by FE was 4 per 100,000 pregnancies (2 to 32). CONCLUSIONS: The addition of FE to U/S for severe CHD prenatal screening in pregnancies at high risk of CHD yielded marginal benefits in terms of increased sensitivity and decreased rates of false negatives, at the expense of significant resource utilization.

CONTEXTE: L'efficacité des stratégies de dépistage ciblant les grossesses à haut risque de cardiopathie congénitale (CC) se trouve réduite par la faible prévalence des CC sévères, par l'augmentation des taux de détection des CC par l'échographie (E/G) du deuxième trimestre, et par la proportion élevée de CC sévères dans les grossesses à faible risque. Dans le cadre de la détection prénatale des CC sévères, nous avons cherché à identifier les situations pour lesquelles un dépistage supplémentaire par échocardiographie fœtale (EF) entraînerait une augmentation significative de la sensibilité et une diminution notable du taux de faux négatifs. MÉTHODES: Nous avons simulé le changement du nombre de cas de CC sévère détectées lorsque l'EF est proposée aux femmes dont l'E/G du deuxième trimestre est normal et qui présentent un risque plus élevé de porter un enfant atteint de CC, par rapport à l'examen du deuxième trimestre seul. L'issue primaire était l'augmentation de la sensibilité. Les issues secondaires étaient le nombre de dépistages nécessaires et la réduction du taux de cas manqués. RÉSULTATS: Pour une sensibilité de l'E/G de 60 %, l'ajout de l'EF pour les grossesses à haut risque de CC (rapport de risque de 3,5; intervalle: 2 à 5) a accru la sensibilité de 2,4 points de pourcentage (1,1 à 7,9). Le nombre de dépistages nécessaires pour détecter un cas supplémentaire de CC sévères était de 436 (156 à 952). La proportion de cas supplémentaires de CC sévères détectées par EF était de 4 pour 100 000 grossesses (2 à 32). CONCLUSIONS: L'ajout de l'EF à l'E/G pour le dépistage prénatal des CC sévères pour les grossesses à risque élevé de CC n'a apporté que des avantages marginaux en termes d'augmentation de la sensibilité et de diminution des taux de faux négatifs, cela au prix d'une utilisation importante des ressources.