Multisystem Inflammatory Syndrome in Children Unmasking Brugada Type 1 Pattern.

We describe the case of a 14-year-old boy hospitalized for multisystem inflammatory syndrome in children who developed atrial fibrillation during the acute phase and a transient Brugada type 1 pattern in the subacute phase. Eight months later, a provocative test with ajmaline confirmed the suspicion of Brugada syndrome. (Level of Difficulty: Intermediate.).

Cardiac dysfunction in Multisystem Inflammatory Syndrome in Children: An Italian single-center study.

BACKGROUND: Multisystem inflammatory syndrome in children (MIS-C) is a novel condition temporally associated with SARS-CoV2 infection. Cardiovascular involvement is mainly evident as acute myocardial dysfunction in MIS-C. The aim of this study was to describe the cardiac dysfunction in patients with MIS-C, defining the role of severity in the clinical presentations and outcomes in a single cohort of pediatric patients. METHODS: A single-center retrospective study on patients diagnosed with MIS-C, according to the Center for Disease Control and Prevention (CDC) definition, and referred to Vittore Buzzi Children's Hospital in Milan from November 2020 to February 2021. Patients were managed according to a local approved protocol. According to the admission cardiac left ventricular ejection fraction (LVEF), the patients were divided into group A (LVEF < 45%) and group B (LVEF ≥45%). Pre-existing, clinical, and laboratory factors were assessed for evaluating outcomes at discharge. RESULTS: Thirty-two patients were considered. Cardiac manifestations of MIS-C were reported in 26 patients (81%). Group A included 10 patients (9 M/1F, aged 13 years [IQR 5-15]), and group B included 22 patients (15 M/7 M, aged 9 years [IQR 7-13]). Significant differences were noted among clinical presentations (shock, diarrhea, intensive care unit admission), laboratory markers (leucocytes, neutrophils, and protein C-reactive), and cardiac markers (troponin T and N-terminal pro B-type Natriuretic Peptide) between the groups, with higher compromission in Group A. We found electrocardiogram anomalies in 14 patients (44%) and rhythm alterations in 3 patients (9%), without differences between groups. Mitral regurgitation and coronary involvement were more prevalent in group A. Total length of hospital stay and cardiac recovery time were not statistically different between groups. A recovery of cardiac functioning was reached in all patients. CONCLUSION: Despite significant differences in clinical presentations and need for intensive care, all of the MIS-C patients with significant cardiac involvement in this study completely recovered. This suggests that the heart is an involved organ and did not influence prognosis if properly treated and supported in the acute phase.

Improved survival among patients with Eisenmenger syndrome receiving advanced therapy for pulmonary arterial hypertension.

BACKGROUND: Advanced therapy (AT) for pulmonary arterial hypertension in the context of congenital heart disease (Eisenmenger syndrome) improves pulmonary hemodynamics, functional class, and the 6-minute walk test. We examined the potential effect of AT on survival in this population. METHODS AND RESULTS: Data on all Eisenmenger patients attending our center over the past decade were collected. Survival rates were compared between patients on and off AT with the use of a modified version of the Cox model, which treats AT as a time-varying covariate. Baseline differences were adjusted for the use of propensity scores. A total of 229 patients (aged 34.5 + or - 12.6 years; 35.4% male) were included. The majority had complex anatomy, and 53.7% were in New York Heart Association class > or = III at baseline assessment. Mean resting saturations were 84.3%. Sixty-eight patients (29.7%) either were on AT or had AT initiated during follow-up. During a median follow-up of 4.0 years, 52 patients died, only 2 of them while on AT. Patients on AT were at a significantly lower risk of death, both unadjusted and after adjustment for baseline clinical differences by propensity score regression adjustment (C statistic=0.80; hazard ratio, 0.16; 95% confidence interval, 0.04 to 0.71; P=0.015) and propensity score matching (hazard ratio, 0.10; 95% confidence interval, 0.01 to 0.78; P=0.028). CONCLUSIONS: AT for pulmonary arterial hypertension in a contemporary cohort of adults with Eisenmenger syndrome was associated with a lower risk of death. Survival benefits should be considered together with improved hemodynamics and functional class when decisions are made about AT in this population.

Half-dose thrombolysis to begin with, when immediate coronary angioplasty in acute myocardial infarction is not possible.

BACKGROUND: Low-dose lytic drugs are sometimes administered to patients with ST-elevation acute myocardial infarction (AMI) as a bridge to coronary angioplasty (facilitated PTCA). Reports are scarce. The characteristics and outcomes of a recent series of consecutive patients treated in our Center are presented. METHODS: In August 2000 facilitated PTCA with half-dose reteplase was started in our Center in all cases when the cath lab was not immediately (< 30 min) available, or the patient had to be transferred to us. Since August 2000, 153 patients were admitted to our cath lab to undergo facilitated (n = 80) or primary (n = 73) PTCA. The data of all patients were prospectively collected, and were analyzed on an "intention-to-treat" basis. RESULTS: No significant differences were found between facilitated and primary PTCA patients with regard to: gender, diabetes, hypertension, previous PTCA/bypass surgery, heart rate at admission, systolic blood pressure, anterior AMI, number of leads with ST-segment elevation, total ST-segment deviation, collateral flow to the infarct-related artery, and three-vessel disease. In our series, facilitated vs primary PTCA patients had a better risk profile: they were younger (61 +/- 13 vs 66 +/- 11 years, p = 0.016), less frequently had a previous AMI (7 vs 24%, p = 0.01), had a shorter time from pain onset to first emergency room admission (122 +/- 104 vs 168 +/- 162 min, p = 0.045), and a trend to a shorter total time to the cath lab (209 +/- 121 vs 255 +/- 183 min, p = 0.073) despite a similar emergency room-to-cath lab component (89 +/- 50 vs 98 +/- 92 min, median 74 vs 65 min, p = NS). Moreover, they presented with a lower Killip class on admission (1.1 +/- 0.4 vs 1.5 +/- 0.98, p = 0.01), with more patients in Killip class 1 (95 vs 74%, p = 0.001). One vs 8% of patients were in shock. Facilitated vs primary PTCA patients had an initial TIMI 2-3 flow in 42 vs 25% of cases (p = 0.031), a final TIMI 3 flow in 82 vs 71% (p = NS), > or = 50% ST-segment resolution in 73 vs 58% (p = NS), and both of the latter in 62 vs 45% (p = 0.099); distal coronary embolization occurred in 9 vs 14% of cases (p = NS); intra-aortic balloon counterpulsation was used in 5 vs 12% and glycoprotein IIb/IIIa inhibitors in 10% of the whole population. The overall in-hospital mortality was 3.7 vs 9.6% (p = NS), and 2.5 vs 4.5% (p = NS) when patients in shock at admission were not considered. Reinfarction occurred in 2 patients submitted to facilitated PTCA (who had had no immediate PTCA, due to full reperfusion) and in none of the patients submitted to primary PTCA; no patient presented with stroke or major bleeding. CONCLUSIONS: Pre-treatment with thrombolysis often provides a patent vessel before PTCA, appears to be safe, and may improve reperfusion after PTCA. In this setting, the additional use of glycoprotein IIb/IIIa inhibitors before PTCA only in non-reperfused patients may be significantly risk- and cost-effective.

Management and outcomes of patients transferred for rescue coronary angioplasty in acute myocardial infarction.

BACKGROUND: Rescue coronary angioplasty (PTCA), though recommended by the guidelines, is not regularly performed after failed lysis in patients with ST-elevation acute myocardial infarction (AMI), and data from large contemporary studies are not available. The outcomes of a recent series of consecutive patients in our Center are presented. METHODS: Between August 2000 and November 2003, 270 patients with AMI < 12 hours were referred to our cath lab for emergency PTCA: 117 (43%) for rescue PTCA after failed lysis, and 153 for primary or facilitated PTCA. The baseline, procedural and outcome data of all patients were prospectively collected, analyzed on an "intention-to-treat" basis and compared. Cineangiographic data were reviewed by three angiographers who were unaware of the clinical data. RESULTS: No significant differences were found between rescue PTCA and primary/facilitated PTCA patients as to: age, female gender, diabetes, hypertension, previous AMI, time from pain onset to the first emergency room admission, heart rate at admission, systolic blood pressure, number of leads with ST-segment elevation, total ST-segment deviation, collateral flow to the infarct-related artery, initial TIMI 2-3 flow, and three-vessel disease. Patients with rescue PTCA, as compared to primary/facilitated PTCA, had a longer time from pain onset to the cath lab (336 +/- 196 vs 229 +/- 155 min, p = 0.0001) and more frequently had an anterior AMI (52 vs 38%, p = 0.027), a higher Killip class (1.5 +/- 0.98 vs 1.26 +/- 0.7, p = 0.02), shock (11 vs 5%, p = 0.073), and intra-aortic balloon pump use (17 vs 8%, p = 0.048); fewer patients were in Killip class 1 (74 vs 85%, p = 0.043). PTCA was performed immediately in 78 vs 95% of patients (p = 0.0001); 8 vs 3 patients had PTCA of the infarct-related artery and 8 vs 1 had bypass surgery later during hospitalization. Patients with rescue PTCA, as compared to primary/facilitated PTCA, had a final TIMI 3 flow in 62 vs 76% of cases (p = 0.017), > or = 70% ST-segment resolution in 36 vs 50% (p = 0.086), and both of the latter in 24 vs 45% (p = 0.006); the overall hospital mortality was 12 vs 6.5%, and 5.8 vs 3.4% when patients in shock on admission were not considered; reinfarction and stroke occurred in 0.9 vs 1.3% and in 2.6 vs 0% of the patients respectively. CONCLUSIONS: Due to referral, rescue PTCA patients were admitted to the cath lab later after the onset of infarction, and had a higher risk profile, as compared to primary/facilitated PTCA patients; both recanalization and reperfusion were less satisfactory, as were the outcomes. Thrombolysis is often ineffective but, as long as it remains a widespread treatment, efforts should be made to improve reperfusion and survival in these patients, possibly by an earlier referral for rescue PTCA.

Replacement therapy for iron deficiency improves exercise capacity and quality of life in patients with cyanotic congenital heart disease and/or the Eisenmenger syndrome.

INTRODUCTION: Iron deficiency is common in cyanotic congenital heart disease (CHD) and results in reduced exercise tolerance. Currently, iron replacement is advocated with limited evidence in cyanotic CHD. We investigated the safety and efficacy of iron replacement therapy in this population. METHODS: Twenty-five iron-deficient cyanotic CHD patients were prospectively studied between August 2008 and January 2009. Oral ferrous fumarate was titrated to a maximum dose of 200mg thrice-daily. The CAMPHOR QoL questionnaire, 6 minute walk test (6MWT) and cardiopulmonary exercise testing were conducted at baseline and after 3 months of treatment. RESULTS: Mean age was 39.9 ± 10.9 years, 80% females. Fourteen had Eisenmenger syndrome, 6 complex cyanotic disease and 5 Fontan circulation. There were no adverse effects necessitating termination of treatment. After 3 months of treatment, hemoglobin (19.0 ± 2.9 g/dL to 20.4 ± 2.7 g/dL, p<0.001), ferritin (13.3 ± 4.7 µg/L to 54.1 ± 24.2 µg/L, p<0.001) and transferrin saturation (17.8 ± 9.6% to 34.8 ± 23.4%, p<0.001) significantly increased. Significant improvements were also detected in the total CAMPHOR score (20.7 ± 10.9 to 16.2 ± 10.4, p=0.001) and 6MWT distance (371.7 ± 84.7 m to 402.8.0±74.9m, p=0.001). Peak VO(2) remained unchanged (40.7 ± 9.2% to 43.8 ± 12.4% of predicted, p=0.15). CONCLUSION: Three months of iron replacement therapy in iron-deficient cyanotic CHD patients was safe and resulted in significant improvement in exercise tolerance and quality of life. Identification of iron deficiency and appropriate replacement should be advocated in these patients.