Definitions and Epidemiology of Coronary Functional Abnormalities

Coronary functional abnormalities are frequent causes of angina pectoris, particularly in patients with unobstructed coronary arteries. There is a spectrum of endotypes of functional coronary abnormalities with different mechanisms of pathology including enhanced vasoconstriction (i.e. coronary artery spasm) or impaired vasodilatation, such as impaired coronary flow reserve or increased microvascular resistance. These vasomotor abnormalities can affect various compartments of the coronary circulation such as the epicardial conduit arteries and/or the coronary microcirculation. Unequivocal categorisation and nomenclature of the broad spectrum of disease endotypes is crucial both in clinical practice as well as in clinical trials. This article describes the definitions of coronary functional abnormalities with currently accepted cut-off values, as well as diagnostic methods to identify and distinguish endotypes. The authors also provide a summary of contemporary data on the prevalence of the different endotypes of coronary functional abnormalities and their coexistence.

Sinus bradycardia as the initial manifestation of multisystem inflammatory syndrome in children

Background While cardiovascular complications, including arrhythmias are now a recognized manifestation of Multisystem inflammatory syndrome in children (MIS-C), there are no reports of primary bradycardia preceding the clinical presentation. We sought to describe a case series of sinus bradycardia as an initial manifestation of MIS-C. Methods We included a series of 10 consecutive patients with confirmed COVID-19 who met WHO and CDC criteria for MIS-C, who developed sinus bradycardia with a heart rate measured in the awake state that was below the normal range for age for children, as an initial manifestation of the disease, in a prospective observational multicenter study. Patients underwent clinical, laboratory evaluation, ECG, Holter, telemetry, echocardiogram, chest X Ray, and a chest CT scan. Results Of the 10 patients included, 6 were male, with a mean age of 6.52±5.35 years, range 4 months to 14 years. All cases were Hispanic. Bradycardia was transient and did not merit treatment. Coronary abnormalities were noted in 6 cases; 4 patients had mild coronary ectasia; 9 patients had pericardial effusion with no evidence of tamponade. All patients had a mild clinical course; none had shock, heart failure, the need for mechanical ventilation, or died. All blood markers (Troponin, BNP, Platelet count, C-reactive protein, D-dimer, Ferritin) returned to normal levels by discharge/follow-up with a favorable outcome including resolution of coronary dilatation in all but 2 in which aneurysm persisted. Treatment All patients received steroids and low-weight-molecular heparin 10 patients, 8 aspirin and 8 intravenous immunoglobulins. Conclusion Sinus bradycardia may be the initial manifestation of MIS-C, usually transient and mild. Physicians should be aware of this presentation. FUNDunding Acknowledgement Type of funding sources: None. Kid, MIS-C. Bradycardia/Atrial Rhythm

Myocardial infarct late after Fontan-type surgery in pulmonary atresia and intact ventricular septum: a must-know complication! About a case report

Coronary abnormalities are frequent in pulmonary atresia and intact ventricular septum, mainly in patients with a very diminutive right ventricle. They severely impact on early and late prognosis. We describe an 8-year-old girl who presented with myocardial ischaemia, late after uneventful Fontan completion. The importance of precise delineation of the coronary anatomy upon initial assessment and during follow-up is emphasised.

Peripheral Gangerene, an Unusual Presentation of Infantile Kawasaki: A Case Report and Literature Review

Introduction. Diagnosing infantile Kawasaki disease with atypical symptoms is difficult, and it also has higher risk of coronary abnormalities which is one of the most common complications of KD. Other complications such as pericardial effusion, mitral insufficiency, congestive heart failure, myocardial systolic dysfunction, and systemic vasculitis were also reported. Peripheral gangrene and necrosis are among the rare complications of this systemic vasculitis. Case Presentation. We report an 8-month-old girl with prolonged fever, generalized petechial rash, cracked erythematous lips, edema, and coronary ectasia who received two doses of IVIG in another center, but short after her discharge, she started to develop a necrotic plaque on her knee. She was admitted in our hospital, and the repeat echocardiography showed sustained coronary ectasia. She received 3 doses of methylprednisolone pulse therapy and was discharged with aspirin and prednisolone. In the follow-up visits, the coronary ectasia was resolved and the necrotic ulcer was healing with a scar. Conclusions. The diagnosis of Kawasaki disease and echocardiographic evaluation of the coronary arteries should be considered in young infants with prolonged fever of unknown origin. Peripheral gangrene is a rare but important complication of infantile Kawasaki disease, although the exact mechanism in not fully understood.

Safe Decompression of the Right Ventricle for PAIVS in Neonates With Coronary Fistulae: Including the Selective Use of Fistula Ligation to Avoid Coronary Steal

Background: There are a number of surgical and interventional treatment options for infants with pulmonary atresia with intact ventricular septum (PAIVS). In our practice, we characterize coronary fistulae and interruptions with angiography in the newborn and have developed a strategy to safely decompress the right ventricle in association with ligation of fistulae if necessary. Methods: All infants operated for PAIVS at age < 60 days from 1999 to 2018 were retrospectively studied. Pre- and postoperative variables were collected, angiograms were reviewed, and a territory score was created to grade the severity of coronary abnormalities. This study focused on the subgroup of patients who had early surgical decompression of the right ventricle. Results: A total of 77 patients were included, with a mean follow-up of 8.6 years. Of these, 55 (71%) had coronary fistulae, including 28 (36%) with coronary artery interruption. Right ventricular decompression (RVD) was performed in 47 (60.5%) patients. There was no 30-day mortality in those who underwent RVD, whereas 6 (20%) without RVD died within 30 days ( P = .003). Ten-year survival was 97.8% and 73.3% for RVD and non-RVD, respectively. In order to prevent coronary steal, 17 patients underwent coronary fistula ligation as their RV was decompressed with 100% early and late survival. Conclusion: Early and late survival in infants with PAIVS is better if the RV can be decompressed. Coronary fistula ligation with RVD has been introduced without an adverse outcome in selected patients with large fistulae.

Multisystem Inflammatory Syndrome in Children associated with COVID-19: Report of four cases in Mexico across the Mexico-US Border

Multisystem Inflammatory Syndrome in Children (MIS-C) is a newly described autoimmune disease mostly occurring in older children, adolescents, and young adults associated with Severe Acute Respiratory Syndrome-Coronavirus type 2 (SARS-CoV-2) infection. Several MIS-C publications from Europe and North America are available, with only a few from Latin America. This is the first Mexican publication of four case reports of MIS-C. Median age at admission was of 8.2 years. All cases manifested with fever, cutaneous rash, conjunctivitis, abdominal pain, and nausea/vomiting. All were admitted with shock, developed coronary abnormalities in the echocardiogram, and had lung abnormalities in the computerized tomography scan. Three needed medical care at the Pediatric Intensive Care Unit, all were resistant to intravenous immunoglobulin, and one patient died of severe myocarditis, shock and acute myocardial infarction. All four cases had a positive SARS-CoV-2 RT-PCR from nasopharynx.

COVID-19 and Multisystem Inflammatory Syndrome in Children: A Systematic Review and Meta-analysis

Background: Multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 has been increasingly recognized. However, the clinical features of MIS-C and the differences from Kawasaki disease remain unknown. The study aims to investigate the epidemiology and clinical course of MIS-C. Methods: PubMed and EMBASE were searched through August 30, 2020. Observational studies describing MIS-C were included. Data regarding demographic features, clinical symptoms, laboratory, echocardiography and radiology findings, treatments, and outcomes were extracted. Study-specific estimates were combined using one-group meta-analysis in a random-effects model. Results: A total of 27 studies were identified including 917 MIS-C patients. The mean age was 9.3 (95% confidence interval [CI], 8.4-10.1). The pooled proportions of Hispanic and Black cases were 34.6% (95% CI, 28.3-40.9) and 31.5% (95% CI, 24.8-38.1), respectively. The common manifestations were gastrointestinal symptoms (87.3%; 95% CI, 82.9-91.6) and cardiovascular involvement such as myocardial dysfunction (55.3%; 95% CI, 42.4-68.2), coronary artery aneurysms (21.7%; 95% CI, 12.8-30.1) and shock (65.8%; 95% CI, 51.1-80.4), with marked elevated inflammatory and cardiac markers. The majority of patients received intravenous immunoglobulin (81.0%; 95% CI, 75.0-86.9), aspirin (67.3%; 95% CI, 48.8-85.7), and corticosteroids (63.6%; 95% CI, 53.4-73.8) with a variety of anti-inflammatory agents. Although myocardial dysfunction improved in 55.1% (95% CI, 33.4-76.8) at discharge, the rate of extracorporeal membrane oxygenation use was 6.3% (95% CI, 2.8-9.8) and the mortality was 1.9% (95% CI, 1.0-2.8). Conclusion: Our findings suggest that MIS-C leads to multiple organ failure, including gastrointestinal manifestations, myocardial dysfunction and coronary abnormalities, and has distinct features from Kawasaki disease.

Abstract 17385: Soluble CD163 Predicts Myocardial Fibrosis in People Living With Human Immunodeficiency Virus Infection: Myocardial and Coronary Abnormalities in HIV Infection (MACHIN) Study

Background: Soluble CD163 (sCD163) is a scavenger receptor that is shed during inflammation and monocyte/macrophage activation and appears to be useful in resolution of the inflammation. This marker has been associated with increased mortality and morbidity in several inflammatory conditions. Hypothesis: sCD163 levels are related to myocardial fibrosis (MF) in people living with HIV infection (PLWH) and controls. Methods: sCD163 levels were measured in serum samples of 92 participants (54 PLWH and 38 HIV- controls, mean age 47±14 years, 28% female).. Myocardial structure and function were assessed using CMR (GE 3.0T; acquisition: RT-Hawk, Heart Vista Inc; analysis: cvi42, Circle Inc, v5.10). Our protocol included cine, pre-contrast, 12-minute post contrast T1 mapping and LGE after infusion of 0.1mmol/kg of gadolinium (Multihance). 12-minute ECV (extra cellular volume) maps were generated from T1 maps. Results: We studied 40 PLWH and 28 HIV- controls (mean age 45±14 years, 46% men). HIV-positive individuals had significantly higher levels of sCD163, compared to controls ( mean±SD: 78±6 versus 54±5 ng/dL, p-value<0.01). The association of sCD163 with diffuse fibrosis detected by ECV was modified by HIV infection (p-value for interaction term: 0.05). log-sCD163 levels were significantly associated with ECV in PLWH (regression coefficient: 9, 95% CI:2-15, p:0.01), but not in controls (p:0.74). The significant association of sCD163 with ECV remained significant even after adjusting for age, gender, race, and LVEF (p:0.02) and even after adding history of protease inhibitor and abacavir use to the models (p:0.02). But after adding both nadir CD4 and highest viral load to the above model, the association was not significant (p:0.08). LGE was not significantly associated with sCD163 levels. Conclusions: Soluble CD163, as a marker of monocyte/macrophage activation, is associated with increased diffuse subclinical MF, estimated by postcontrast ECV, in virologically-suppressed PLWH. Importantly, this association is independent of demographic factors, LV function, and ART medication use. These findings highlight the importance of chronic sustained inflammation in development of MF in PLWH.