Acalculous Cholecystitis From Kawasaki Disease in a Three-Month-Old Girl: A Rare Sign at an Uncommon Age.

We report a case of a previously healthy three-month-old girl who presented with acute fever, watery diarrhea, and right upper abdominal guarding. Abdominal ultrasonography findings were compatible with acute acalculous cholecystitis. Initially, antibiotics were administered for a total of eight days without improvement. Hence, atypical Kawasaki disease (KD) was suspected despite the absence of classical disease manifestations and her uncommon age. The diagnosis was made using alternative diagnostic criteria and echocardiography. After KD was diagnosed, high-dose intravenous immunoglobulin G and aspirin were administered on day 9 of disease onset. Her clinical condition significantly improved within 24 hours, and she recovered well without complications during the 1.5 years of follow-up.

High haemoglobin levels at the time of occlusion predict worse outcome for patients with patent ductus arteriosus and pulmonary hypertension.

INTRODUCTION: Current guidelines discourage shunt closure in patients with pulmonary vascular resistance index >8 Wood units x m2. The study examined the long-term outcome of patients over 15 years old, with pulmonary vascular resistance index >8 Wood units x m2 and patent ductus arteriosus. MATERIALS AND METHODS: This was a multi-institutional, retrospective study involving all consecutive patients (>15 years old) with patent ductus arteriosus and severe pulmonary hypertension. Patients who had patent ductus arteriosus closure were divided into the Good (no death or hospital admissions due to worsening pulmonary hypertension) and the Poor Outcome groups and these groups were compared. RESULTS: Thirty-seven patients [male: 9 (24.3%); mean age: 30.49 ± 9.56 years; median follow-up: 3 (IQR: 1.5,10) years] were included from four centers. Twenty-two patients who underwent patent ductus arteriosus closure, 15 (71.4%) had good outcomes while 7 (28.6%) had poor outcomes. Pulmonary vascular resistance index and pulmonary to systemic resistance ratio (Rp:Rs) were lower in the Good Outcome Group (14.35 ± 1.66 Wood units x m2 vs. 20.07 ± 2.44; p = 0.033 and 0.44 ± 0.16 vs. 1.08 ± 1.21; p = 0.042). Haemoglobin concentrations (<14.3 g/dL) were associated with good long-term outcomes in the Closed Group. CONCLUSIONS: Patients with patent ductus arteriosus with severe pulmonary hypertension have a dismal outcome with or without closure. High haemoglobin levels at the time of occlusion predict a worse outcome for patients with patent ductus arteriosus and pulmonary hypertension.

Verification of "Japanese Scoring Systems" to Predict IVIG Resistance and Identification of Predictors for IVIG Resistance in Thai Children with Kawasaki Disease.

The objective of this study was to assess the validity of using the Kobayashi, Sano and Egami scoring systems to predict the intravenous immunoglobulin (IVIG) resistance of Kawasaki disease (KD) and to identify the predictors of IVIG resistance in our Thai population. A retrospective study involving 130 KD patients who were admitted between January 2005 and April 2018 was performed. We found that 17 (13%) KD patients did not respond to the first IVIG dose. The three scoring systems have good specificity (80.8%, 74% and 92.1%, respectively) but low sensitivity (0%, 33.3% and 22.2%, respectively). Multivariate analysis suggested that a body temperature greater than 40.2 °C (odds ratio of 3.80, P value = 0.03), a neutrophil percentage greater than 74% (odds ratio of 3.82, P value = 0.03) and serum albumin less than 3 g/L (odds ratio of 5.09, P value = 0.01) were predictors of IVIG resistance. Our study cannot conclude that the three Japanese scoring systems are not suitable for predicting IVIG resistance in the Thai population due to study limitations. However, a high-grade fever (≥ 40.2 °C), neutrophil predominance ≥ 74% and hypoalbuminemia (serum albumin level < 3 g/L) were predictors of IVIG resistance in Thai KD patients.

Correlation Between Fragmented QRS and Ventricular Function from Cardiac Magnetic Resonance in Patients with Repaired Tetralogy of Fallot.

Fragmented QRS is an abnormal sign in an ECG resulting from ventricular dyssynchrony. The presence of fragmented QRS (fQRS) is related to mortality in patients with repaired tetralogy of Fallot (rTOF). This study aimed to analyze the correlations between fQRS and ventricular function parameters from cardiac magnetic resonance (CMR) images. A total of 54 patients with rTOF or repaired DORV, TOF type who had been investigated by CMR at Songklanagarind Hospital from January 2012 to August 2019 were retrospectively reviewed. Most of the patients (47 patients, 87%) were diagnosed with TOF and most of these (57.4%) were treated with a transannular patch (TAP) on the right ventricular outflow tract (RVOT). Forty-four patients (81%) had fQRS. The median time interval between EKG and CMR was 4.2 months (IQR 1.1, 6.9). Patients with fQRS had significantly higher RVEDVi (145.6 [IQR 121.1, 173.1) vs. 115.9 [IQR 96.2, 146.9] mL/m2, p = 0.037), higher RVESVi (82.5 [IQR 62.9, 100.8] vs. 58.6 [IQR 46, 77.2] mL/m2, p = 0.032) and higher RVEDV/LVEDV ratio [1.6 (IQR 1.4, 1.9) vs. 1.4 (IQR 1.3, 1.5), p = 0.026]. The extent of fQRS was significantly associated with an increase in RVEDVi (r = 0.39, p = 0.004) and RVESVi (r = 0.45, p < 0.001) but a decrease in RVEF (r = - 0.37, p = 0.006). Fragmented QRS had higher sensitivity and lower specificity than prolonged QRS duration (≥ 160 ms) to predict all of the parameters indicating pulmonary valve replacement (PVR). Patients with rTOF who had fQRS were significantly associated with RV dilatation. The extent of fQRS had only low to moderate correlation with right ventricular parameters (RVEDVi, RVESVi and RVEF). Fragmented QRS could be used for early detection of RV dilatation and the need for PVR in patients with rTOF.

Main pulmonary artery-to-descending aorta ratio in computed tomography: cut-off value to diagnose pulmonary hypertension in children.

PURPOSE: To establish a cut-off value of main pulmonary artery (MPA)/descending aorta (DA) ratio and MPA/ascending aorta (AA) ratio by computed tomography (CT) to identify pulmonary hypertension (PHT) in children. MATERIAL AND METHODS: A total of 45 children diagnosed with PHT, who underwent both right heart catheterization (RHC) and CT, were enrolled as a case group (PHT), and their CT findings were compared with those from a control group (non-PHT). The widest short-axis diameters of DA at the level of the diaphragmatic outlet, AA, and MPA at its bifurcated level were measured. RESULTS: The most common indication to order a CT in the PHT group was congenital heart disease (CHD) (n = 30, 66.7%), and in the non-PHT group it was metastatic workup (n = 31, 68.9%). The median (IQR) diameters of MPA in the PHT and the non-PHT groups were 18.7 mm (15.5, 26.7) and 16.4 mm (13.7, 19.5) (p = 0.005). The MPA/DA ratios were 2.2 and 1.5 in the PHT and non-PHT groups (p < 0.001). The MPA/AA ratios were 1.2 and 1.1 in the PHT and non-PHT groups (p = 0.042). An MPA/DA ratio of 1.8 carried a positive likelihood ratio (LR+) of 7.5 with a sensitivity of 66.67%, specificity of 91%, positive predictive value (PPV) of 88%, and negative predictive value (NPV) of 73.21%. CONCLUSIONS: The MPA/DA ratio > 1.8 suggests PHT in children and may lead to the avoidance of invasive cardiac catheterization particularly in non-CHD patients.

Predictors of death after receiving a modified Blalock-Taussig shunt in cyanotic heart children: A competing risk analysis.

OBJECTIVE: To determine risk factors affecting time-to-death ≤90 and >90 days in children who underwent a modified Blalock-Taussig shunt (MBTS). METHODS: Data from a retrospective cohort study were obtained from children aged 0-3 years who experienced MBTS between 2005 and 2016. Time-to-death (prior to Glenn/repair), time-to-alive up until December 2017 without repair, and time-to-progression to Glenn/repair following MBTS were presented using competing risks survival analysis. Demographic, surgical and anesthesia-related factors were recorded. Time-to-death ≤90 days and >90 days was analyzed using multivariate time-dependent Cox regression models to identify independent predictors and presented by adjusted hazard ratios (HR) and 95% confidence intervals (CI). RESULTS: Of 380 children, 119 died, 122 survived and 139 progressed to Glenn/repair. Time-to-death probability (95% CI) within 90 days was 0.18 (0.14-0.22). Predictors of time-to-death ≤90 days (n = 63) were low weight (<3 kg) (HR 7.6, 95% CI:2.8-20.4), preoperative ventilator support (HR 2.7, 95% CI:1.3-5.6), postoperative shunt thrombosis (HR 5.0, 95% CI:2.4-10.4), bleeding (HR 4.5, 95% CI:2.1-9.4) and renal failure (HR 4.1, 95% CI:1.5-10.9). Predictors of time-to-death >90 days (n = 56) were children diagnosed with pulmonary atresia with ventricular septal defect and single ventricle (compared to tetralogy of fallot) (HR 3.2, 95% CI:1.2-7.7 and HR 3.1, 95% CI:1.3-7.6, respectively), shunt size/weight ratio >1.1 vs <0.65 (HR 6.8, 95% CI:1.4-32.6) and longer duration of mechanical ventilator (HR 1.002, 95% CI:1.001-1.004). Shunt size/weight ratio ≥1.0 (vs <1.0) and ≥0.65 (vs <0.65) were predictors for overall time-to-death in neonates and toddlers, respectively (HR 13.1, 95% CI:2.8-61.4 and HR 7.8, 95% CI:1.7-34.8, respectively). CONCLUSIONS: Perioperative factors were associated with time-to-death ≤90 days, whereas particular cardiac defect, larger shunt size/weight ratio, and longer mechanical ventilation were associated with time-to-death >90 days after receiving MBTS. Larger shunt size/weight ratio should be reevaluated within 90 days to minimize the risk of shunt over flow.

Feasibility and early outcomes of aortic coarctation treatments with BeGraft Aortic stent.

OBJECTIVES: To assess feasibility and early outcomes of using BeGraft Aortic stent in the treatment of aortic coarctation (CoA). BACKGROUND: BeGraft Aortic stent (Bentley InnoMed, Hechingen, Germany) allows large postdilation diameter up to 30 mm. With availability of lengths of 19-59 mm and lower stent profile, they can be used in native and recurrent CoA in adults and in pediatric patients. MATERIALS AND METHODS: This is a multicentre retrospective analysis of 12 implanted BeGraft Aortic stents in CoA between May 2017 and April 2019. RESULTS: Twelve patients aged 7.7-38 years (median 18.3 years) with body weight of 19.9-56 kg (median 45.5 kg). Eight patients (66%) had native juxtaductal CoA while four had recurrent CoA after previous surgical or transcatheter treatments. The stents were implanted successfully in all the patients with no serious adverse events. The length of the stents ranged from 27 to 59 mm and the implanted stent diameter varied from 12 to 18 mm. The median intraprocedural CoA pressure gradient decreased from 25 mmHg (range 16-66 mmHg) to 2 mmHg (range 0-13 mmHg). The mean follow-up duration was 10.2 months. Two patient (16.6%) had residual stent narrowing requiring staged redilation. One patient (8%) had pseudoaneurysm formation at 1 year cardiac CT follow-up. CONCLUSIONS: The BeGraft Aortic stent may be considered to be safe and effective in the short term in treatment of CoA from childhood to adulthood. Long-term follow-up is needed.