Cardiac dysfunctions in children with drug-resistant epilepsy.

Objective: There were reports of cardiac dysfunction that led to sudden unexpected death in epilepsy (SUDEP) in patients with epilepsy. Early detection of cardiac dysfunction can lead to early management to prevent sudden cardiac death in these patients. The objective of our study is to assess cardiac functions in children with drug-resistant epilepsy (DRE) compared with the normal population by using a standard echocardiogram (SE), tissue Doppler imaging (TDI) and myocardial strain evaluations (MSE). Method: Twenty-seven children who have been diagnosed with DRE based on the International League against Epilepsy (ILAE) were included in the study, along with 27 children whose ages match those of the normal control group. Results: Seventeen children, median age 12 years old, were using more than four anti-seizure medications. Structural brain lesions were the most common cause of epilepsy, 55.6% (15). Generalized tonic-clonic seizures were the most common seizure type, 55.6% (15). Children with DRE had a lower early mitral valve E wave inflow velocity compared with the control group (p < 0.05). They also had lowered early diastolic velocities (e') and myocardial performance index (MPI) when compared with the control group (p < 0.05). There was a statistically significant difference in left ventricular myocardial strain in children with DRE, with an average of -21.1 (IQR -23.5 and -19.4) and control, -25.5 (IQR -27.3 and -24.2). Significance: Children with DRE have an impairment of left ventricular diastolic function and myocardial strain, which could indicate decreased myocardial deformation and contraction compared with controls. These cardiological assessments can be used to evaluate children with DRE for early diagnosis and management of their cardiac dysfunction.

Correlation between pressure gradient from echocardiography and peak-to-peak pressure gradient from cardiac catheterization or surgery in patients with coarctation of aorta.

The gold standard for assessing pressure gradients (PG) across coarctation involves measurements obtained through cardiac catheterization or surgical intervention. There has been ongoing discussion regarding the accuracy of non-invasive methods for estimating these gradients. This study sought to establish the correlation and agreement between the systolic blood pressure (SBP) gradient between the upper and lower extremities, as well as, the mean and maximum PG derived from echocardiography, in comparison to the peak-to-peak pressure gradient obtained from either cardiac catheterization or surgery. We conducted a retrospective study on patients < 18 years diagnosed with coarctation at Chiang Mai University Hospital from 2011 to 2022. The study involved the measurement of the SBP gradient between the upper and lower extremities, mean and maximum PG using echocardiography, peak-to-peak pressure gradient obtained from cardiac catheterization, and pressure gradient recorded during surgical procedures. The Spearman's correlation and Bland-Altman analysis were employed to assess correlation and agreement. Fifty-four patients with aortic coarctation were enrolled. The mean PG measured by echocardiography showed a significantly moderate correlation (r = 0.78, p < 0.001) and the highest level of agreement according to Bland Altman plots, in comparison to the peak-to-peak pressure gradient measured during both cardiac catheterization and surgical procedure. The max PG demonstrated a notable overestimation compared to the gold standard (mean difference + 13.14 with a slope of biases + 0.64, p < 0.001). The mean PG obtained through echocardiography has more potential to be applied in practical application in predicting pressure gradient in patients with coarctation.

Development of a clinical prediction tool for extubation failure in pediatric cardiac intensive care unit.

Introduction/objective: Extubation failure in pediatric patients with congenital or acquired heart diseases increases morbidity and mortality. This study aimed to develop a clinical risk score for predicting extubation failure to guide proper clinical decision-making and management. Methods: We conducted a retrospective study. This clinical prediction score was developed using data from the Pediatric Cardiac Intensive Care Unit (PCICU) of the Faculty of Medicine, Chiang Mai University, Thailand, from July 2016 to May 2022. Extubation failure was defined as the requirement for re-intubation within 48 h after extubation. Multivariable logistic regression was used for modeling. The score was evaluated in terms of discrimination and calibration. Results: A total of 352 extubation events from 270 patients were documented. Among these, 40 events (11.36%) were extubation failure. Factors associated with extubation failure included history of pneumonia (OR: 4.14, 95% CI: 1.83-9.37, p = 0.001), history of re-intubation (OR: 5.99, 95% CI: 2.12-16.98, p = 0.001), and high saturation in physiologic cyanosis (OR: 5.94, 95% CI: 1.87-18.84, p = 0.003). These three factors were utilized to develop the risk score. The score showed acceptable discrimination with an area under the curve (AUC) of 0.77 (95% CI: 0.69-0.86), and good calibration. Conclusion: The derived Pediatric CMU Extubation Failure Prediction Score (Ped-CMU ExFPS) could satisfactorily predict extubation failure in pediatric cardiac patients. Employing this score could promote proper personalized care. We suggest conducting further external validation studies before considering implementation in practice.

Outcome following acute and recurrent rheumatic fever.

BACKGROUND: Rheumatic carditis is the leading cause of permanent disability caused by damage of the cardiac valve. This study aimed to determine the outcome and predictors of valve surgery in patients with acute rheumatic fever (ARF) and recurrent rheumatic fever (RRF). METHODS: This was a retrospective study of patients diagnosed with ARF and RRF between 2006 and 2021. The predictors of valve surgery were analysed using multivariable Cox proportional regression. RESULTS: The median age of patients with ARF and RRF (n=92) was 11 years (range 5-18). Seventeen patients (18%) were diagnosed with RRF. The most common presenting symptoms included clinical carditis (87%), heart failure (HF) (63%), fever (49%) and polyarthralgia (24%). Patients with moderate-to-severe rheumatic carditis (88%) were given prednisolone. After treatment, the severity of valvular regurgitation was reduced in 52 patients (59%). Twenty-three patients (25%) underwent valve surgery. The incidence of HF, RRF, severe mitral regurgitation on presentation, left ventricular enlargement and pulmonary hypertension was greater in the surgical group than in the non-surgical group. Recurrent rheumatic fever (hazard ratio 7.9, 95% CI 1.9-33.1), tricuspid regurgitation (TR) gradient ≥ 42 mmHg (HR 6.3, 95%CI 1.1-38.7) and left ventricular end-diastolic dimension (LVEDD) ≥6 cm (HR 8.7, 95% CI 2.1-35.9) were predictors of valve surgery (multivariable Cox proportional regression analysis). CONCLUSION: Clinical carditis was the most common presenting symptom in patients with ARF and RRF. The majority of patients responded positively to prednisolone. These findings highlight the predictors of valve surgery following ARF, including RRF, TR gradient ≥ 42 mmHg and LVEDD ≥ 6 cm.Abbreviations: ARF: acute rheumatic fever; CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; GAS: group A beta-haemolytic Streptococcus; HF: heart failure; HR: hazard ratio; LVEDD: left ventricular end-diastolic dimension; MR: mitral regurgitation; RHD: rheumatic heart disease; RRF: recurrent rheumatic fever; TR: tricuspid regurgitation.

Administration of Milrinone Following Tetralogy of Fallot Repair Increases Postoperative Volume Administration Without Improving Cardiac Output.

BACKGROUND: Phosphodiesterase inhibitors are known to relieve symptoms in the setting of heart failure, although their effects in restrictive ventricular physiology have been poorly characterized. We explored the association between the use of milrinone and volume administration during the first 72 hours following surgical repair of tetralogy of Fallot (TOF). METHODS: We reviewed all cases of primary surgical repair of TOF with pulmonary stenosis or atresia at Boston Children's Hospital between 2011 and 2020. To adjust for baseline differences between patients who did and did not receive milrinone, we matched patients with similar propensity scores in a 1:1 ratio (use of milrinone versus not). We then compared the need for volume administration during the first 72 hours postoperatively, vital signs, and measures of cardiac output between the matched cohorts. Additionally, in the group of patients receiving milrinone, linear regression modeling was used to explore the relationship between total dose of milrinone and total volume administration. RESULTS: Among 351 included patients, 134 received perioperative milrinone. A total of 212 patients (106 per group) were matched based on anatomic and surgical risks using a propensity score. After propensity matching, compared with nonmilrinone-treated patients, milrinone-treated patients were given postoperative volume more frequently (66% vs 52%; difference 14% [95% confidence interval, CI, 1%-27%]; P = .036). Milrinone-treated patients had a slower recovery of tachycardia during the first 12 hours (difference in slope 0.30 [95% CI, 0.14-0.47] beats per minute [BPM]/h; P < .001), and the intergroup difference peaked at 12 hours postoperatively (8 [95% CI, 5-12] BPM). Milrinone administration was not associated with improved cardiac output, including arteriovenous oxyhemoglobin saturation difference. In propensity-matched patients receiving milrinone, the total volume administered during the first 72 postoperative hours was significantly associated with the cumulative dose of postoperative milrinone ( r = 0.20; 95% CI, 0.01-0.38; P = .036). Based on the slope of the regression line, for every 1000 µg/kg of milrinone (equivalent to ~0.25 µg/kg/min for 72 hours) administered in the first 72 postoperative hours, an estimated 11.0 (95% CI, 0.6-21.4) mL/kg additional volume was administered. CONCLUSIONS: The use of milrinone within the first 72 hours following TOF repair is associated with more frequent administration of volume, a positive association between a higher total dose of postoperative milrinone and the amount of postoperative volume administered, a higher heart rate, and a lower blood pressure, but is not associated with improved cardiac output.

Pheochromocytoma presenting with QT prolongation and catecholamine-induced myocarditis in a child.

Pheochromocytomas are catecholamine-producing tumors derived from the adrenomedullary chromaffin cells. The presentation is a classic triad of episodic headaches, sweating, and tachycardia. Hypertensive crisis can occur due to profuse catecholamine excess. Unusual manifestations mimicking cardiogenic shock, arrhythmia, and myocarditis have been rarely reported in children. We present a case with uncommon manifestations of pheochromocytoma in a child, including the episodes of exercised-induced presyncope with QT prolongation, and subsequently cardiogenic shock due to fulminant myocarditis. He later developed hypertensive crisis. The adrenal mass on abdominal computed tomography with an increased chromogranin A level and elevated plasma normetanephrine, and the histological study confirmed the diagnosis of pheochromocytoma. Cardiac functions completely recovered after adrenalectomy. Genetic testing was positive for von Hippel-Lindau syndrome. We describe pheochromocytoma crisis presenting with prolonged QT and catecholamine-induced myocarditis. We discuss the clues to assist in the diagnosis of this condition and its appropriate treatment.

Comparison of aortic stiffness and hypertension in repaired coarctation patients with a bicuspid versus a tricuspid aortic valve.

BACKGROUND: Coarctation of the aorta (COA) is associated with reduced aortic distensibility and systemic hypertension (HTN). 60-85% of COA patients have a bicuspid aortic valve (BAV). It is not known if the presence of a BAV accentuates the aortopathy and HTN in CoA patients. We examined whether patients with COA and a BAV had lower aortic distensibility by CMR, and a higher prevalence of systemic HTN compared with COA patients with a tricuspid aortic valve (TAV). METHODS: In successfully repaired COA patients excluding those with residual COA, ascending aorta (AAO) and descending aorta (DAO) distensibility was calculated by CMR. HTN was assessed using standard pediatric and adult criteria. RESULTS: Among 215 COA patients (median age 25.3 years), 67% had a BAV, and 33% had a TAV. Median AAO distensibility z-score was lower in the BAV group (- 1.2 versus - 0.7; p = 0.014) but DAO distensibility was similar in BAV and TAV patients. HTN prevalence was similar in BAV (32%) and TAV groups (36%, p = 0.56). On multivariable analysis controlling for confounders, HTN was not associated with BAV but was associated with male gender (p = 0.003) and older age at follow-up (p = 0.004). CONCLUSIONS: In young adults with treated COA, those with a BAV had stiffer AAO compared to those with a TAV, but DAO stiffness was similar. HTN was not related to BAV. These results suggest that although the presence of a BAV in COA exacerbates the AAO aortopathy, it does not exacerbate the more generalized vascular dysfunction and associated HTN.

Hydrogen therapy as a potential therapeutic intervention in heart disease: from the past evidence to future application.

Cardiovascular disease is the leading cause of mortality worldwide. Excessive oxidative stress and inflammation play an important role in the development and progression of cardiovascular disease. Molecular hydrogen, a small colorless and odorless molecule, is considered harmless in daily life when its concentration is below 4% at room temperature. Owing to the small size of the hydrogen molecule, it can easily penetrate the cell membrane and can be metabolized without residue. Molecular hydrogen can be administered through inhalation, the drinking of hydrogen-rich water, injection with hydrogen-rich-saline, and bathing of an organ in a preservative solution. The utilization of molecular hydrogen has shown many benefits and can be effective for a wide range of purposes, from prevention to the treatment of diseases. It has been demonstrated that molecular hydrogen exerts antioxidant, anti-inflammatory, and antiapoptotic effects, leading to cardioprotective benefits. Nevertheless, the exact intracellular mechanisms of its action are still unclear. In this review, evidence of the potential benefits of hydrogen molecules obtained from in vitro, in vivo, and clinical investigations are comprehensively summarized and discussed with a focus on the cardiovascular aspects. The potential mechanisms involved in the protective effects of molecular hydrogen are also presented. These findings suggest that molecular hydrogen could be used as a novel treatment in various cardiovascular pathologies, including ischemic-reperfusion injury, cardiac injury from radiation, atherosclerosis, chemotherapy-induced cardiotoxicity, and cardiac hypertrophy.

Predictive factors of extubation failure in pediatric cardiac intensive care unit: A single-center retrospective study from Thailand.

Introduction/objective: Extubation failure increases morbidity and mortality in pediatric cardiac patients, a unique population including those with congenital heart disease or acquired heart disease. This study aimed to evaluate the predictive factors of extubation failure in pediatric cardiac patients and to determine the association between extubation failure and clinical outcomes. Methods: We conducted a retrospective study in the pediatric cardiac intensive care unit (PCICU) of the Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand, from July 2016 to June 2021. Extubation failure was defined as the re-insertion of the endotracheal tube within 48 hours after extubation. Multivariable log-binomial regression with generalized estimating equations (GEE) was performed to explore the predictive factors associated with extubation failure. Results: We collected 318 extubation events from 246 patients. Of these, 35 (11%) events were extubation failures. In physiologic cyanosis, the extubation failure group had significantly higher SpO2 than the extubation success group (P < 0.001). The predictive factors associated with extubation failure included a history of pneumonia before extubation (RR 3.09, 95% CI 1.54-6.23, P = 0.002), stridor after extubation (RR 2.57, 95% CI 1.44-4.56, P = 0.001), history of re-intubation (RR 2.24, 95% CI 1.21-4.12, P = 0.009), and palliative surgery (RR 1.87, 95% CI 1.02-3.43, P = 0.043). Conclusion: Extubation failure was identified in 11% of extubation attempts in pediatric cardiac patients. The extubation failure was associated with a longer duration of PCICU stay but not with mortality. Patients with a history of pneumonia before extubation, history of re-intubation, post-operative palliative surgery, and post-extubation stridor should receive careful consideration before extubation and close monitoring afterward. Additionally, patients with physiologic cyanosis may require balanced circulation via regulated SpO2.

Utility of cardiac CT in infants with congenital heart disease: Diagnostic performance and impact on management.

BACKGROUND: Advances in cardiac CT (CCT) scanner technology allow imaging without anesthesia, and with low radiation dose, making it an attractive technique in infants with congenital heart disease. However, the utility of CCT using a dual-source scanner with respect to diagnostic performance and impact on management has not been systematically studied in this population. METHODS: Retrospective review of infants who underwent CCT to determine the utility of CCT with respect to the following: answering the primary diagnostic question, providing new diagnostic information, prompting a change in management, and concordance with catheterization or surgical inspection. RESULTS: A total of 156 infants underwent 172 scans at a median age of 64 days, (IQR 4-188) from Jan 2016-Dec 2019. The most frequent diagnostic question was related to the pulmonary arteries (43%), followed by the aortic arch (30%), pulmonary veins (26%), coronary arteries (17%), patent ductus arteriosus (10%) and others (9%). A high-pitch spiral scan was frequently used (90%). The median effective radiation dose was low (0.66 â€‹mSv) and general anesthesia was used infrequently (23%). CCT answered the primary diagnostic question in 168/172 (98%) and added to the diagnostic information already available by echocardiography in 161/172 (96%) scans. CCT led to a change in management following 78/172 (53%) scans and had an impact on management following 167/172 (97%) scans. On follow-up, after 107/172 (62%) scans, subjects underwent cardiac surgery, and after 55/172 (32%) scans, they had cardiac catheterization. CCT findings were concordant with catheterization and/or surgical inspection in 156/159 (98%) scans. CONCLUSIONS: In infants with complex congenital heart disease, CCT was accurate, answered the diagnostic questions in nearly all cases, and frequently added diagnostic information that impacted management. Radiation exposure was low, and anesthesia was needed infrequently.

Modeling severe functional impairment or death following ECPR in pediatric cardiac patients: Planning for an interventional trial.

AIM: We aimed to characterize extracorporeal CPR (ECPR) outcomes in our center and to model prediction of severe functional impairment or death at discharge. METHODS: All ECPR events between 2011 and 2019 were reviewed. The primary outcome measure was severe functional impairment or death at discharge (Functional Status Score [FSS] ≥ 16). Organ dysfunction was graded using the Pediatric Logistic Organ Dysfunction Score-2, neuroimaging using the modified Alberta Stroke Program Early Computed Tomography Score. Multivariable logistic regression was used to model FSS ≥ 16 at discharge. RESULTS: Of the 214 patients who underwent ECPR, 182 (median age 148 days, IQR 14-827) had an in-hospital cardiac arrest and congenital heart disease and were included in the analysis. Of the 110 patients who underwent neuroimaging, 52 (47%) had hypoxic-ischemic injury and 45 (41%) had hemorrhage. In-hospital mortality was 52% at discharge. Of these, 87% died from the withdrawal of life-sustaining therapies; severe neurologic injury was a contributing factor in the decision to withdraw life-sustaining therapies in 50%. The median FSS among survivors was 8 (IQR 6-8), and only one survivor had severe functional impairment. At 6 months, mortality was 57%, and the median FSS among survivors was 6 (IQR 6-8, n = 79). Predictive models identified FSS at admission, single ventricle physiology, extracorporeal membrane oxygenation (ECMO) duration, mean PELOD-2, and worst mASPECTS (or DWI-ASPECTS) as independent predictors of FSS ≥ 16 (AUC = 0.931) and at 6 months (AUC = 0.924). CONCLUSION: Mortality and functional impairment following ECPR in children remain high. It is possible to model severe functional impairment or death at discharge with high accuracy using daily post-ECPR data up to 28 days. This represents a prognostically valuable tool and may identify endpoints for future interventional trials.

Longitudinal changes in extent of late gadolinium enhancement in repaired Tetralogy of Fallot: a retrospective analysis of serial CMRs.

BACKGROUND: Right ventricular (RV) late gadolinium enhancement (LGE) occurs due to surgical scarring and RV remodeling, and has been shown to be associated with clinical outcomes in Tetralogy of Fallot (TOF). However, it is not known if cardiovascular magnetic resonance (CMR) LGE extent progresses over time, and therefore, it is not known if serial reassessment of LGE is necessary. We determined the rate of progression in the extent of RV LGE on serial CMR examinations in repaired TOF. METHODS: Retrospective review of 127 patients after TOF repair (49% male, median age at first CMR 18.9 years (Interquartile range (IQR) 13.3,27.0) who had at least two CMRs (median follow-up duration of 4.0 years (IQR 2.1,5.9)) was performed. 84/127 patients had no interventions between serial CMRs (Group 1) while 43/127 patients had transcatheter or surgical intervention between CMRs (Group 2). The extent of RV LGE was assessed using 2 methods: a semiquantitative RV LGE score and a quantitative RV LGE extent expressed as % of RV mass. Mixed effects linear regression modeling to estimate changes in LGE over time. RESULTS: RV LGE was present in all patients on the first CMR. % RV LGE extent and LGE score did not increase over time in either patient group. The mean 5 year rates of change were small and negative for both % RV LGE extent [- 2.3 (95% CI - 2.9, - 1.8, p < 0.001) in Group 1, and - 1.9 (95% CI - 3.2, - 0.7, p = 0.004) in Group 2], and RV LGE score [- 0.9 (95% CI - 1.1, - 0.6, p < 0.001) in Group 1, and - 0.5 (95% CI - 1.1, - 0.0, p = 0.047) in Group 2]. CONCLUSIONS: In this serial CMR evaluation of children and adults with repaired TOF, no significant progression in the extent of RV LGE was seen on intermediate term follow-up. Given recent concerns regarding the safety of gadolinium-based contrast agents, frequent assessment of LGE may not be necessary in follow-up.

Cardiac self-gating using blind source separation for 2D cine cardiovascular magnetic resonance imaging.

PURPOSE: To develop and validate a new cardiac self-gating algorithm using blind source separation for 2D cine steady-state free precession (SSFP) imaging. METHODS: A standard cine SSFP sequence was modified so that the center point of k-space was sampled with each excitation. The center points of k-space were processed by 4 blind source separation methods, and used to detect heartbeats and assign k-space data to appropriate time points in the cardiac cycle. The proposed self-gating technique was prospectively validated in 8 patients against the standard electrocardiogram (ECG)-gating method by comparing the cardiac cycle lengths, image quality metrics, and ventricular volume measurements. RESULTS: There was close agreement between the cardiac cycle length using the ECG- and self-gating methods (bias 0.0 bpm, 95% limits of agreement ±2.1 bpm). The image quality metrics were not significantly different between the ECG- and self-gated images. The ventricular volumes, stroke volumes, and mass measured from self-gated images were all comparable with those from ECG-gated images (all biases <5%). CONCLUSION: The self-gating method yielded comparable cardiac cycle length, image quality, and ventricular measurements compared with standard ECG-gated cine imaging. It may simplify patient preparation, be more robust when there is arrhythmia, and allow cardiac gating at higher field strengths.

Wilms tumor with dilated cardiomyopathy: A case report.

BACKGROUND: Wilms tumor is the most common renal malignancy in childhood. It occurs primarily between the ages of 2 and 5 years. The usual manifestations are abdominal mass, hypertension, and hematuria. The case presented here had an unusual presentation, with dilated cardiomyopathy and hypertension secondary to the Wilms tumor. CASE SUMMARY: A 3-year-old boy presented with a 5-d history of irritability, poor appetite, and respiratory distress. His presenting clinical symptoms were dyspnea, tachycardia, hypertension, and a palpable abdominal mass at the left upper quadrant. His troponin T and pro-B-type natriuretic peptide levels were elevated. Echocardiography demonstrated a dilated hypokinetic left ventricle with an ejection fraction of 29%, and a suspected left renal mass. Computed tomography scan revealed a left renal mass and multiple lung nodules. The definitive diagnosis of Wilms tumor was confirmed histologically. The patient was administered neoadjuvant chemotherapy and underwent radical nephrectomy. After surgery, radiotherapy was administered, and the adjuvant chemotherapy was continued. The blood pressure and left ventricular function normalized after the treatments. CONCLUSION: Abdominal mass, dilated cardiomyopathy and hypertension can indicate Wilms tumor in pediatric patients. Chemotherapy and tumor removal achieve successful treatment.

Neonatal Central Cyanosis Caused by Anomalous Drainage of the Right Superior Vena Cava.

The draining of the right superior vena cava (SVC) into the left atrium is a very rare anomaly of systemic vein drainage. This case report describes a term male infant presenting with central cyanosis diagnosed with right SVC drainage into the left atrium. The diagnosis was performed using conventional echocardiography and computed tomography angiography. Surgical correction with translocation of the right SVC to the right atrium was necessary to treat the symptom of cyanosis and prevent further complications, including brain abscesses and paradoxical embolization.