Paediatric multisystem inflammatory syndrome associated with COVID-19: filling the gap between myocarditis and Kawasaki?

Myocarditis and Kawasaki disease are common but usually distinct diseases in children. During the coronavirus pandemic (COVID-19), reports of a new form of myocarditis with clinical features of Kawasaki appeared. We investigated the place of this new disease in the spectrum encompassing Kawasaki disease and myocarditis.Thirty two consecutive children referred to our centre for a suspicion of Kawasaki or a diagnosis of myocarditis were included and eventually divided into four groups: 11 Kawasaki diseases, 6 Kawasaki syndromes (children with another diagnosis), 7 myocarditis without Kawasaki clinical feature and 7 myocarditis with incomplete Kawasaki clinical features. All were treated with immunoglobulins except those of the myocarditis group. The survival rate was 91%. The 7 children with myocarditis and clinical features of incomplete Kawasaki were all positive for SARS-CoV-2. They had a transient myocardial failure with a favourable course and none had coronary artery disease.Conclusion: Every COVID-19 child within our population had a mild to severe myocarditis and presented with fever plus two or three Kawasaki clinical features. Short-term evolution was good for these children. This new disease seems to fill the gap between isolated myocarditis and Kawasaki disease. What is Known: • A new paediatric disease close to Kawasaki disease appeared during the COVID-19 pandemic What is New: • In our population, children presented with fever, vivid Kawasaki clinical features (although the Kawasaki syndrome was always incomplete) and a myocarditis without coronary abnormalities. • The new disease fills the gap between paediatric myocarditis and Kawasaki disease but its prognosis is much better.

Concomitant repair of tetralogy of Fallot and an aortic root aneurysm in an adult.

We reported the case of a 56-year-old male with tetralogy of Fallot associated with an aneurysm of the aortic root and severe aortic insufficiency. Repair of the aortic aneurysm along with a complete tetralogy repair was performed.

Lower circulating Sta-Liatest D-Di levels in patients with aortic intramural hematoma compared with classical aortic dissection.

OBJECTIVE: To compare the diagnostic value of circulating Sta-Liatest D-Di levels in classic acute aortic dissection (AAD) and in aortic intramural hematoma (AIH), a variant of AAD without a patent false lumen. DESIGN: Single-center retrospective case-control study. SETTING: University Hospital of Strasbourg, France. PATIENTS: Ninety-four consecutive patients with both confirmed AAD and d-dimer measurements at entry were included. d-dimer levels were assayed by the immunoturbidimetric method Sta-Liatest D-Di (Diagnostica Stago, Asnieres sur Seine, France). INTERVENTION: Patient characteristics and clinical evolution were analyzed. MEASUREMENTS AND MAIN RESULTS: Eighty-four patients (89%) presented a classic AAD with patent false lumen and ten (11%) presented an AIH. Clinical presentation did not differ between the two groups. The mortality rate was 0% in AIH and 26% in classic AAD. d-dimer levels were significantly lower in patients with AIH (median, 1230 ng/mL; interquartile range, 685-2645 ng/mL) than in patients with AAD with patent false lumen (median value, 9290 ng/mL; interquartile range, 3890-20,000 ng/mL; p = 0.008). All patients with AAD and patent false lumen had d-dimer levels above the threshold of 400 ng/mL (sensitivity 100%). However, one patient with AIH presented d-dimer levels below the threshold. Therefore, the sensitivity of the d-dimer test in detecting AIH was 90%. CONCLUSIONS: Sta-Liatest D-Di levels are lower in AIH than in AAD with patent false lumen. This test can quite possibly be negative in the case of intramural hematoma. This feature must be considered when interpreting d-dimer levels in patients with acute aortic syndrome.

Twenty-seven-year experience with composite valve graft replacement of the aortic root.

BACKGROUND AND AIM OF THE STUDY: The study aim was to assess early and late outcome in patients undergoing composite valve graft replacement (CVGR) of the aortic root by means of the Bentall procedure, and to identify predictors of early and late death associated with this surgical approach. METHODS: Between August 1975 and July 2002, 162 consecutive patients underwent a Bentall procedure for CVGR. Demographic, treatment and clinical outcome data from these patients were gathered, reviewed, and analyzed. Potential predictors of early and late mortality were analyzed. RESULTS: The study population was predominantly male (n = 132; 81.5%) and middle-aged (mean age 51.3 +/- 15.8 years; range: 10-79 years). The main indications for surgery were annuloaortic ectasia (n = 75; 46.3%), aortic dissection (n = 44; 27.2%) and Marfan syndrome (n = 34; 21%). Reoperation was required in 37 cases (22.8%). The mean follow up was 74 months. Early (in-hospital) mortality was 1.9% (n = 3). The only independent determinant of early mortality was cardiopulmonary bypass time (p = 0.025). Late mortality was 27.7% (n = 44). On multivariate analysis, the only independent risk factors for late mortality were age >60 years (p = 0.044) and left ventricular ejection fraction <50% (p = 0.037). Actuarial survival rates were 92.9%, 77%, 56.2%, and 47.1% at one, five, 10, and 15 years, respectively. Rates of freedom from reoperation on the aortic root and ascending aorta were 90.6% and 72.5% at five and 15 years, respectively. No false aneurysms were observed at any coronary reimplantation sites. CONCLUSION: In this series, the Bentall procedure was associated with low operative mortality and good early and late results. This suggests that the procedure may be considered as a reference to other operations on the aortic root, at least in adult patients.

Trends in isolated coronary artery bypass grafting over the last decade.

OBJECTIVES: The purpose of this study was to assess the impact on hospital mortality and morbidity of extensive myocardial revascularization, using arterial grafts in patients undergoing isolated coronary artery bypass grafting (CABG). METHODS: Our prospective perioperative database was used to define two groups of patients who underwent isolated CABG with cardiopulmonary bypass, based on the years in which the operation was performed: Group A (2000-2003; 898 patients) and Group B (2009-2012; 1249 patients). The baseline and operative characteristics and outcomes were compared. RESULTS: Several significant changes in perioperative variables were observed. Group B included higher percentages of patients aged over 80 years (+58.1%), with diabetes (+32.0%) and with a history of percutaneous coronary intervention (+24.9%). The mean EuroSCORE II was significantly increased from 2.5 ± 4.4% in Group A to 3.2 ± 5.7% in Group B (P= 0.001). The mean number of distal anastomoses was significantly increased over time (total: 2.6 ± 0.8 vs 3.1 ± 1.0, P< 0.0001 and with arterial grafts: 1.6 ± 0.8 vs 2.6 ± 0.9, P< 0.0001). In-hospital mortality was low and did not significantly differ between Groups A and B (1.3 vs 2.4%; P= 0.08). Significant increases of new-onset atrial fibrillation (11.7 vs 21.9%, P= 0.017) and deep sternal infection (0.2 vs 1.1%, P= 0.017) were observed in Group B, compared with Group A. In multivariate analysis, extensive use of arterial grafts was not a risk factor of hospital mortality or sternal morbidity. CONCLUSIONS: Despite the increasing risk profiles of patients undergoing CABG, extensive myocardial revascularization using arterial grafts is associated with good early results.

Predictors of Atrial Fibrillation After Coronary Artery Bypass Grafting: A Bayesian Analysis.

BACKGROUND: This study was conducted to identify preoperative predictors of postoperative atrial fibrillation (POAF) after isolated coronary artery bypass grafting (CABG) by using a Bayesian analysis that included information from prior studies. METHODS: We performed a prospective observational study from October 2008 to December 2013 of 1,481 patients who underwent isolated CABG with cardiopulmonary bypass and had no history of AF. Bayesian analysis was used to study the preoperative risks factors for POAF. RESULTS: The POAF incidence was 21%. Multivariate analysis identified the following independent predictors of POAF after CABG: high CHA2DS2-VASc (Congestive heart failure, Hypertension [blood pressure >140/90 mm Hg or treated hypertension on medication], Age ≥75 years, Diabetes mellitus, prior Stroke or transient ischemic attack or thromboembolism, vascular disease, Age 65 to 74 years, Sex category [female sex]) score (odds ratio [OR], 1.23; 95% credible interval [CI], 1.14 to 1.33 per 1-point increment, probability (Pr) [OR > 1] = 1), severe obesity with a body mass index of 35 kg/m2 or higher (OR, 1.28; 95% CI, 1.12 to 1.45; Pr [OR > 1] = 1), preoperative ß-blocker use (OR, 1.12; 95% CI, 1.06 to 1.20; Pr [OR > 1] = 1), preoperative antiplatelet therapy (OR, 1.75; 95% CI, 1.14 to 2.79, Pr [OR > 1] = 1), and renal insufficiency with a creatinine clearance of less than 60 mL/min (OR, 1.34; 95% CI, 1.03 to 1.74; Pr [OR > 1] = 1). CONCLUSIONS: This prospective Bayesian analysis identified five independent preoperative predictors of POAF after isolated CABG with cardiopulmonary bypass: CHA2DS2-VASc score, severe obesity, preoperative ß-blocker use, preoperative antiplatelet therapy, and renal failure. The main interest in the CHA2DS2-VASc score as a predictor of POAF is that it is a simple and widely used bedside tool. Patients with these independent predictors of POAF may constitute a target population to test preventive strategies, such as non-antiarrhythmic and antiarrhythmic drugs.

Next-generation sequencing (NGS) as a fast molecular diagnosis tool for left ventricular noncompaction in an infant with compound mutations in the MYBPC3 gene.

Left ventricular noncompaction (LVNC) is a clinically heterogeneous disorder characterized by a trabecular meshwork and deep intertrabecular myocardial recesses that communicate with the left ventricular cavity. LVNC is classified as a rare genetic cardiomyopathy. Molecular diagnosis is a challenge for the medical community as the condition shares morphologic features of hypertrophic and dilated cardiomyopathies. Several genetic causes of LVNC have been reported, with variable modes of inheritance, including autosomal dominant and X-linked inheritance, but relatively few responsible genes have been identified. In this report, we describe a case of a severe form of LVNC leading to death at 6 months of life. NGS sequencing using a custom design for hypertrophic cardiomyopathy panel allowed us to identify compound heterozygosity in the MYBPC3 gene (p.Lys505del, p.Pro955fs) in 3 days, confirming NGS sequencing as a fast molecular diagnosis tool. Other studies have reported neonatal presentation of cardiomyopathies associated with compound heterozygous or homozygous MYBPC3 mutations. In this family and in families in which parental truncating MYBPC3 mutations are identified, preimplantation or prenatal genetic screening should be considered as these genotypes leads to neonatal mortality and morbidity.

The prothrombotic paradox of severe obesity after cardiac surgery under cardiopulmonary bypass.

BACKGROUND: Obesity is suggested to reduce postoperative bleeding in patients undergoing cardiac surgery with cardiopulmonary bypass (CPB) but perioperative hemostasis variations have not been studied. Therefore, we investigated the effects of severe obesity (body mass index [BMI] ≥35kg/m(2)) on chest tube output (CTO) and hemostasis in patients undergoing cardiac surgery with CPB. MATERIALS AND METHODS: We prospectively investigated 2799 consecutive patients who underwent coronary and/or valve surgery using CPB between 2008 and 2012. 204 patients (7.3%) presented a severe obesity. RESULTS: In the severe obesity group, the 6-h and 24-h CTO were significantly reduced by -21.8% and -14.8% respectively (P<0.0001) compared with the control group. A significant reduction of the mean number of red blood cell units transfused at 24h was observed in the severe obesity groups (P=0.01). On admission to the intensive care unit, a significant increase of platelet count (+9.2%; P<0.0001), fibrinogen level (+12.2%; P<0.0001) and prothrombin time (+4.1%; P<0.01) and a significant decrease of the activated partial thromboplastin time (-4.2%; P<0.01) were observed in the severe obesity group compared with the control group. In multivariate analysis, severe obesity was significantly associated to a decreased risk of excessive bleeding (24-h CTO >90th percentile; Odds ratio: 0.37, 95% CI: 0.17 to 0.82). No significant differences were observed regarding postoperative thromboembolic events between the two groups. CONCLUSIONS: Severe obesity is associated with a prothrombotic postoperative state that leads to a reduction of postoperative blood loss in patients undergoing cardiac surgery with CPB.

Exclusive low-molecular-weight heparin as bridging anticoagulant after mechanical valve replacement.

BACKGROUND: Unfractionated heparin has been the standard anticoagulant used immediately after mechanical heart valve replacement (MHVR). The purpose of this study was to assess a postoperative anticoagulation protocol with low-molecular-weight heparin (LMWH) immediately after MHVR without the use of unfractionated heparin or anti-factor Xa monitoring. METHODS: We performed a prospective, single-center, observational study of 1,063 consecutive patients undergoing elective MHVR with postoperative LMWH anticoagulation treatment. The exclusion criteria were as follows: renal failure, intraaortic balloon counterpulsation, critical perioperative state, or a recent neurologic event. The postoperative anticoagulation protocol used subcutaneous enoxaparin as a bridging anticoagulant treatment beginning on the first postoperative day and continuing until vitamin K antagonist treatment was fully effective. Patients were followed for 6 weeks. The primary endpoints were the incidence of thromboembolic or major bleeding events. RESULTS: Eleven (1%) thromboembolic events occurred. Ten of these events were transient or permanent strokes. Major bleeding events occurred in 44 patients (4.1%), 7 of which were observed before the enoxaparin treatment period. At the time of discharge, 570 patients (53.6%) were no longer receiving LMWH treatment due to achieving the target international normalized ratio. The mean length of hospital stay was 8.5 ± 2.9 days. There were no deaths during the 6-week follow-up period. CONCLUSIONS: In our highly selected population, after MHVR, postoperative anticoagulation using LMWH is associated with a low rate of thromboembolic and major bleeding events. This large observational study demonstrates that the use of LMWH as an anticoagulant is effective and safe after MHVR.

Plasma fibrinogen level on admission to the intensive care unit is a powerful predictor of postoperative bleeding after cardiac surgery with cardiopulmonary bypass.

INTRODUCTION: Evidence regarding the behavior of fibrinogen levels and the relation between fibrinogen levels and postoperative bleeding is limited in cardiac surgery under cardiopulmonary bypass (CPB). To evaluate perioperative fibrinogen levels as a predictor of postoperative bleeding in patients undergoing cardiac surgery with CPB. MATERIALS AND METHODS: In this prospective, single-center, observational cohort study of 1956 patients following cardiac surgery with CPB, fibrinogen level was measured perioperatively. Excessive bleeding group was defined as patients with a 24-h chest tube output (CTO) exceeded the 90th percentile of distribution. RESULTS: The median 24-h CTO was 728.6±431.1ml. A total of 189 patients (9.7%) were identified as having excessive bleeding. At admission to the intensive care (Day 0), the fibrinogen levels were 2.5±0.8g/l and 2.1±0.8g/l in the control and excessive bleeding groups, respectively (P<0.0001). The fibrinogen level on Day 0 was significantly correlated with the 24-h CTO (rho=-0.237; P<0.0001). Multivariate analysis demonstrated that the fibrinogen level at Day 0 was the best perioperative standard laboratory test to predict excessive bleeding (P=0.0001; odds ratio, 0.5), whereas preoperative fibrinogen level was not a predictor. Using receiver operating characteristics curve analyses, the best Day 0 fibrinogen level cutoff to predict postoperative bleeding was 2.2g/l. CONCLUSIONS: In this large prospective study, the fibrinogen level upon admission to the intensive care unit after CPB predicted the risk of postoperative bleeding. Our data add to the concern regarding the fibrinogen level threshold that might require fibrinogen concentrate infusion to reduce postoperative blood loss.

Flash pulmonary edema in an orthotopic heart transplant recipient.

Flash pulmonary edema (FPE) is a severe renovascular disease that leads to acute recurring pulmonary edema and acute systemic hypertension. Though rarely reported in the literature, its incidence is probably underestimated secondary to misdiagnosis, especially in patients with normal left ventricular function. We report the case of an orthotopic heart transplant recipient who presented with FPE despite having normal left ventricular function and no signs or symptoms of transplant rejection. Discovery of severe bilateral atherosclerotic renal artery stenosis in this patient led to emergency hepatorenal bypass surgery and a favorable postoperative course.

Splenic injury during biventricular assist device support as bridge to transplantation.

Biventricular assist device (BVAD) has been shown to be effective for bridge to heart transplantation with an acceptability low incidence of adverse effects in critically ill heart failure patients. We report the case of a 44-year-old man with severe acute ischemic heart failure who was supported with the Thoratec paracorporeal biventricular assist system. After an initial uneventful postoperative course the patient experienced a splenic rupture which required a splenectomy. The pathological mechanism of this outcome remains unclear. Two months after discharge, the patient underwent heart transplantation and had no postsplenectomy sepsis or thrombotic complications at the last follow-up.

Right atrial metastasis from hepatocellular carcinoma.

A 65-year-old patient with a past medical history of hypertension, alcoholism, micronodular cirrhosis, and coronary artery bypass grafting 10 years ago developed a hepatocellular carcinoma, treated by chemoembolization. One month after treatment, thoracoabdominal CT scan showed no residual hepatic tumor, but tumoral aspect in the right atrium with extension into the inferior vena cava. The patient being asymptomatic, cardiac ultrasound confirmed the presence of a free, mobile, pediculated tumor in the right atrium. Surgical exploration found a well-circumscribed mass, attached to the atrial wall by a 1.5-cm diameter pedicle implanted near the inferior vena cava ostium, moving freely in the right atrial cavity. The tumor was easily resected by section of the pedicle and its surrounding parietal implantation zone. No complications occurred postoperatively, and the patient was discharged on the 10th postoperative day. Three years after, the patient is in good health and is asymptomatic; cardiac ultrasound showed no tumor recurrence.