Pediatric Cardiology

High ECG Risk-Scores Predict Late Gadolinium Enhancement on Magnetic Resonance Imaging in HCM in the Young

High ECG Risk-Scores Predict Late Gadolinium Enhancement on Magnetic Resonance Imaging in HCM in the Young. Österberg AW, Östman-Smith I, Jablonowski R, Carlsson M, Green H, Gunnarsson C, Liuba P, Fernlund E. Pediatr Cardiol. 2021 Jan 30. doi: 10.1007/s00246-020-02506-9. PMID: 33515326   Take Home Points: An ECG risk-score has been described that predicts high risk of subsequent cardiac arrest in young patients with hypertrophic cardiomyopathy (HCM). We assessed whether an ECG risk-score could be used as an indicator of myocardial fibrosis. 12-lead ECG was used for calculating the ECG risk-score (grading 0–14p). High-risk ECG (risk-score>5p) occurred only in the HCM group. In low-risk ECG-score patients (0–2p), median percent of myocardium showing LGE were zero percent, in intermediate-risk (3–5p) were 5.4% and in high-risk (6–14p) were 10.9% ECG-score>2p had a sensitivity and specificity of 79% and 84% to detect positive LGE on CMR. ECG risk-score>2 p could be used as a cut-off for screening of myocardial fibrosis.     Commentary from Dr. Manoj Gupta (New York, USA), section editor of Pediatric & Fetal Cardiology Journal Watch: Introduction: Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disease and the leading cause of sudden cardiac death in the young. Several studies by cardiac magnetic resonance (CMR) in HCM patients have shown the gradual and progressive changes of the myocardium with findings of disturbed myocardial perfusion and perfusion defects in the surroundings of fibrotic areas. Myocardial fibrosis as measured by CMR has been proposed as a risk-factor for cardiac events. CMR with late gadolinium enhancement (LGE) is gold standard for evaluating fibrosis in HCM.   Study Details: The study groups consisted of 42 individuals: 26 HCM patients, seven individuals at risk of HCM (genotype-positive, phenotype-negative and first-degree relatives) and nine healthy volunteers, including four athletes (physical activity>10 h/week), without heredity for cardiac disease. The 14 gene standard panel for HCM in the HCM group was positive in 16 patients but the genetic mutations were unknown in 10 patients. It was possible to calculate the ECG risk-scores in all study participants (n=42). High-risk ECG-scores (6–14 p) were found in 35% of the HCM patients (9/26) but in none of the individuals at risk of HCM, nor in healthy volunteers.   LGE could be evaluated in all individuals (n=42). All LGE-positive individuals were in the HCM group. In the HCM group 54% of the individuals (14/26) had positive LGE, with median ECG risk-score of 8p [4–11p]. Among the HCM patients with a high-risk ECG-score, 89% had positive LGE (8/9). The one exception, the patient with negative LGE and a high-risk ECG-score, had HCM due to a PRKAG2 mutation and not a sarcomeric mutation.   Three HCM patients had a very low-risk ECG (0–2p): One HCM patient had a 2.3% LGE of LVM but a completely normal ECG, one HCM patient had an ECG risk-score of 1 point and 4%LGE of LVM and one HCM patient had 2 points and 2.1% LGE of LVM. The individual with normal ECG and 2.3% LGE of LVM was diagnosed with mild HCM two years before the CMR, had a known genetic predisposition with MYBPC3 where the ultrasound showed IVS and PW measurements at 14 mm. Three individuals had an intermediate-range ECG risk-score (3–5p): One HCM patient (14 years) had a score of 3 points and 13.5% LGE of LVM, one HCM patient (30 years) had 4 points and 20.3% LGE of LVM and one patient (13 years) had 5 points and 5.4% LGE of LVM.   The positive and negative predictive value (PPV and NPV) of an ECG risk-score>2 points as a screening test for LGE shows a PPV of 79% [CI 56–91%] and NPV of 84% [CI 66–94%] for LGE being present in patients.   In the HCM group 62% (13/21) had a perfusion defect (PD). The median ECG risk-score in HCM patients with a perfusion defect was 5p [1–9p] and in HCM patients without a perfusion defect 0p [0–1p], p=0.001. All individuals positive for perfusion defect were in the HCM group. An ECG risk-score>2 points was associated with positive PD present on CMR.   Discussion This study showed that conventional 12-lead ECG analyzed by the ECG risk-score method according to Östman-Smith et al. has high specificity and sensitivity for indicating myocardial fibrosis as well as perfusion defects on CMR. Thus, ECG risk-score>2p is an inexpensive and easily available method as a screening for HCM patients with likely myocardial fibrosis and/or perfusion defects. An ECG risk-score>2 points in our study predicted myocardial fibrosis on CMR by late gadolinium enhancement with a specificity of 84% and a sensitivity of 79%. An ECG risk-score>2 points also predicted myocardial perfusion defect with high sensitivity and specificity.   These findings indicate that the ECG risk score could be used as a screening tool for HCM patients for predicting who is at risk of having myocardial fibrosis and need urgent further risk stratification investigations.   Implications for the Future: This study shows the ability of the ECG risk-score in predicting fibrosis and perfusion defects on CMR. Further and larger studies are needed to validate if ECG risk-score and LGE on CMR have independent prognostic value in the clinical follow-up of HCM in the young. Conclusion: Conventional 12-lead ECG analyzed and categorized by the ECG risk-score may predict myocardial fibrosis and perfusion defects as measured by CMR in HCM in the young. An ECG risk-score>2 points could be used as a cut-off for screening of myocardial fibrosis and/or perfusion defects with high sensitivity and specificity. None of the genotype positive, phenotype-negative individuals at risk for HCM demonstrated LGE or perfusion defects on CMR or a high ECG risk-score, nor did the healthy volunteers.   

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Comparison Between Currently Recommended Long-Term Medical Management of Coronary Artery Aneurysms After Kawasaki Disease and Actual Reported Management in the Last Two Decades

Comparison Between Currently Recommended Long-Term Medical Management of Coronary Artery Aneurysms After Kawasaki Disease and Actual Reported Management in the Last Two Decades. Osborne J, Friedman K, Runeckles K, Choueiter NF, Giglia TM, Dallaire F, Newburger JW, Low T, Mathew M, Mackie AS, Dahdah N, Yetman AT, Harahsheh AS, Raghuveer G, Norozi K, Burns JC, Jain S, Mondal T, Portman MA, Szmuszkovicz JR, Crean A, McCrindle BW; International Kawasaki Disease Registry Pediatr Cardiol. 2021 Jan 13. doi: 10.1007/s00246-020-02529-2. PMID: 33439285   Take Home Points: This is an observational registry study. In the 2017 American Heart Association (AHA) Kawasaki disease (KD) guidelines, risk levels (RLs) risk stratification was added for long-term management. Generally, past practice was consistent with the latest AHA guidelines. However, the important exception was that 35% of patients with persistent giant coronary artery aneurysms were not on anticoagulants. Physician education could improve guideline compliance and patient outcomes.   Commentary from Dr. Manoj Gupta (New York, USA), section editor of Pediatric & Fetal Cardiology Journal Watch: Introduction: Kawasaki disease (KD) is a self-limiting, systemic vasculitis of unclear etiology that mostly occurs in children. Coronary artery aneurysms form in approximately 25% of untreated patients and in approximately 4% of patients treated within 10 days of the onset of illness. The long-term consequences of KD may include myocardial infarction and death. In 2017, the American Heart Association (AHA) updated the approach to risk stratification and long-term management of KD patients (https://www.ahajournals.org/doi/pdf/10.1161/CIR.0000000000000484). This manuscript compared the Kawasaki disease management practice from 1999 to 2016 with the most recent AHA 2017 recommendations for thromboprophylaxis and medical therapy based on the new risk stratification. The study included the eligible patients from registry who were diagnosed with Kawasaki disease under the age of eighteen years from 1999 to 2016 and had coronary artery aneurysms (Defined as at least one coronary artery diameter with a maximum z-score ≥ 2.5 at any point in their initial work up and subsequent follow -up). Figure 1: Risk level (RL) classification of coronary artery aneurysms per the 2017 AHA guidelines using maximum z-score, luminal dimension, and amount of regression. Risk Level 1: No involvement Risk Level 2: Dilation only     Results: Prevalence of medication use by risk levels (RL) was explored across 3 eras (1999–2004, 2005–2010, and 2011–2016). Acetylsalicylic Acid (ASA): Use of ASA/aspirin ranged from 88 to 96% for RLs for which use was “indicated” (RLs 3.1, 4.1, 4.2, 5.1, 5.2, and 5.3); this represents patients with coronary artery aneurysms of any size that have not regressed to normal luminal dimensions or dilation only (z-score <2.5)   Systemic anticoagulation is only “recommended” for patients for RL 5.1 (persistent large/giant coronary artery aneurysms). Despite this, only 65% of patients in RL 5.1 were receiving anticoagulation and there was no trend toward increased usage over the different year groups.   Dual antiplatelet therapy (ASA and clopidogrel) was in use at 16% of patient visits for RL 5.2 where use was “reasonably indicated.” For visits for RLs 5.3 and 5.4, dual antiplatelet therapy was used for 11% and 9%, respectively (“not indicated”).   There was an increase in ASA use for RL 3.2 from 54% in 1999–2004 to 73% in 2011–2016 and there was increased single antiplatelet (ASA only) use from 43% in 1999–2004 to 67% in 2011–2017. Conclusions and Implications for Clinical Practice:   The management of patients with Kawasaki disease and coronary artery aneurysms from 1999 to 2016 was generally similar to 2017 AHA Kawasaki disease guidelines.   Physician education could improve guideline compliance and patient outcomes, and knowledge translation efforts are needed to further optimize practice in anticoagulation KD patients. Additionally, more research is needed to advance future recommendations, particularly for statins, beta-blockers, and direct oral anticoagulants.   

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Long-term experience with the one-and-a-half ventricle repair for simple and complex congenital heart defects

Long-term experience with the one-and-a-half ventricle repair for simple and complex congenital heart defects. Cabrelle G, Castaldi B, Vedovelli L, Gregori D, Vida VL, Padalino MA. Eur J Cardiothorac Surg. 2021 Jan 4;59(1):244-252. doi: 10.1093/ejcts/ezaa289. PMID: 32888295   Take Home Points: Long-term (median 13 years) results in patients with a single RV and a 1 ½ ventricle repair are good in this relatively small group. There was no significant difference in survival or freedom from adverse events between simple and complex patients. When planned, a 1.5V repair may be beneficial in a very specific subset of patients and possibly lead to better outcomes than single ventricle (Fontan) palliation?   Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch: One and a half ventricle (1 ½ V) repair has been proposed for those with borderline/small right ventricles with hypothetical advantages over single ventricle palliation/Fontan such as ability to increase cardiac output and adapt to exercise, maintain pulsatile pulmonary blood flow, and low venous pressure in the IVC. This single center study retrospectively evaluated 29 patients who underwent 1 ½ V staged or primary repair between 1994-2018. Inclusion criteria were hypoplastic tricuspid valve (z-score < -3) and hypoplastic RV (< 2/3 predicted normal volume). Patients were divided into simple (CHD confined to right heart lesions) and complex anatomy (including other than right heart lesions and more challenging surgical repair). Echo data, cath data (PVR), and metabolic stress test data were followed. See table 2.   Median follow up was 12.5 years. Specific surgical details were discussed in the results section. Most common post-operative complications included pericardial and pleural effusions and arrhythmias. There were 3 late deaths with 2 considered non-cardiac related. 6 required a cardiac cath after a median time of 2.8 years, most commonly to address branch pulmonary artery stenosis. 3 patients required subsequent re-operation; 1 underwent transplant and the other 2 underwent biventricular conversion due to interval RV growth. Survival at 25 years was 89%; all had normal kidney and liver function (based on lab tests). Median O2 sat was 98%. Freedom from any late adverse event was 57% (16/28) and freedom from reoperation or re-intervention was 82.1% (23/28) and 78.6% (22/28) respectively. The complex patients had a much more significant initial post-operative course as would be expected. None of the “late” deaths occurred in the first year of follow up. 5 patients had neurological AEs which possibly contributed the most to the late AE rate, but the paper does not define this well. See tables 3 and 4. Comparison between the complex and simple group found no significant differences in survival, freedom from AE, or freedom from reintervention. See figure 1. Only 10 patients had MRIs and only 12 patients had metabolic stress testing. Mean VO2 was higher than compared to a control group of Fontan patients (33 +/- 5 vs. 24 +/- 1.6 ml/kg/min).   This surgical center planned for 1.5V repair after birth and show overall good results, with a few patients even being able to undergo biventricular conversion. No patients had PLE or chronic liver congestion, known complications of the Fontan. Important limitations include low total number of patients, lack of MRI data, and limited stress test data. When planned, a 1.5V repair may be beneficial in a very specific subset of patients and possibly lead to better outcomes than single ventricle (Fontan) palliation.            

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Six-Year Neurodevelopmental Outcomes for Children With Single-Ventricle Physiology

Six-Year Neurodevelopmental Outcomes for Children With Single-Ventricle Physiology. Sananes R, Goldberg CS, Newburger JW, Hu C, Trachtenberg F, Gaynor JW, Mahle WT, Miller T, Uzark K, Mussatto KA, Pizarro C, Jacobs JP, Cnota J, Atz AM, Lai WW, Burns KM, Milazzo A, Votava-Smith J, Brosig CL; PHN investigators. Pediatrics. 2021 Feb;147(2):e2020014589. doi: 10.1542/peds.2020-014589. Epub 2021 Jan 13. PMID: 33441486   Take Home Points: There is an increase in externalizing behavioral problems and decreased adaptive skills in school age children with HLHS. Many children with HLHS who have low adaptive skills at 6 years of age will not be identified by screening at earlier ages. Serial neurodevelopmental evaluations will be necessary to diagnose deficits as these single ventricle patients get older and advance in school.   Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch: Neurodevelopmental deficits are one of the most common long term issues that face children with HLHS. Early identification could theoretically allow for implementation of therapies that could improve long-term educational and behavioral outcomes. This extension study to the Single Ventricle Reconstruction (SVR) Trial studied the original cohort who had a 14-month mental development index (MDI) and psychomotor development index (PDI) via annual developmental (ASQ) and behavioral (externalizing and internalizing)/adaptive assessments (BASC-2) based on parental questionnaires from ages 3-6. The ASQ measures the child’s development in communication, gross motor, fine motor, problem solving and personal social skills, and the BASC-2 measures adaptive skills and problem behaviors in the community and home settings. Adaptive functioning refers to how a child copes with demands of everyday life and their personal independence. Scores were compared to population norms and classified as at least average (< 1 SD), at risk (< 1-2 SC) and impaired (< 2 SD). 249 patients completed the assessments.   The greatest change in proportion of children at being at risk or impaired occurred between ages 3 and 4, but this was largely due to a lower rate of reported problem behaviors at age 3 than the population norms. However, by age 6, many more had externalizing behavior challenges (e.g. hyperactivity, aggression, and rule breaking). There was not an increase in internalizing behaviors or differences compared to population norms. The most significant deviations were noted on the adaptive skills portion of BASC-2, with most differences occurring between ages 5 and 6. At age 3, 87% were age-appropriate, but by age 6, this dropped to only 71%. 22% (vs. 14% expected) were classified as at risk and 7% (vs. 2%) as impaired. See figure 1 and table 2. The authors note that this finding may not be a regression, but rather the parents now having increased opportunities for social comparison now that the children are in school and begin to act more independently. Parent ratings of deficits in problem solving skills and communication were strongly related to future poor adaptive skills, with ~44% of children at risk/impaired on the 14 month MDI and ~ 36% of children at risk/impaired on the 14 month PDI having impaired adaptive skills at age 6. As a “half glass full” observation, the authors highlight that a significant proportion (77-85%) who do not show early impairments remain unimpaired at age 6.   Limitations of the study were that only parental assessments were made, a selection bias was possibly present given that participants had fewer risk factors for impaired neurodevelopment and higher socioeconomic status than non-participants, and the inability to measure impact of access to early intervention services on outcome scores. A follow up study at 10-12 years is already underway to allow for further understanding of a relationship with later school age outcomes and standardized testing.       

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Low-dose prostaglandin E1 is safe and effective for critical congenital heart disease: is it time to revisit the dosing guidelines?

Low-dose prostaglandin E1 is safe and effective for critical congenital heart disease: is it time to revisit the dosing guidelines? Daniel Vari 1, Wendi Xiao 2, Shashank Behere 3, Ellen Spurrier 3, Takeshi Tsuda 3, Jeanne M Baffa 3 Cardiol Young. 2021 Jan;31(1):63-70. doi: 10.1017/S1047951120003297. Epub 2020 Nov 3. PMID: 33140712; DOI: 10.1017/S1047951120003297   Take Home Points: Prostaglandin E1 at an initial and maintenance dose of 0.01 μg/kg/minute was sufficient to maintain ductal patency in 83% in this study, instead of the standard starting dose of prostaglandin E1 is 0.05 μg/kg/minute. Starting low-dose prostaglandin E1 at 0.01 μg/kg/minute is a safe and effective therapy for critical CHD. Patients with pulmonary obstruction were more likely to require higher doses than patients with systemic obstruction. Postnatally diagnosed patients with systemic obstruction are also at a higher risk of dose escalation than prenatally diagnosed infants. The incidence of respiratory depression requiring mechanical ventilation was low and was mostly seen in premature infants.      Commentary from Dr. Manoj Gupta (New York, USA), section editor of Pediatric & Fetal Cardiology Journal Watch: Of the 153 eligible patients, 127 (83%) were started and maintained on a prostaglandin E1 dose of 0.01 μg/kg/minute until the end- point. Of the 26 patients who had their doses increased, the final dose was less than 0.05 μg/kg/minute in 15, 0.05 μg/kg/minute in five, and greater than 0.05 μg/kg/minute in two patients. 15 patients had their dose increased due to both echocardiographic findings and clinical factors suggesting ductal constriction. In systemic obstruction patients, these factors included blood pressure gradients, pulse abnormalities between upper and lower extremities, and elevated serum lactate levels. In pulmonary obstruction and inadequate mixing patients, the primary clinical factor driving dose increase was hypoxemia. In six patients, there was an echocardiographic finding of ductal constriction without corresponding clinical signs.   Of the 137 patients analyzed for respiratory depression, 38 (28%) had documented respiratory depression at a dose of 0.01μg/kg/ minute. In 10 of these patients, the respiratory depression was transient and did not merit initiation of respiratory support although four were started on caffeine. Fourteen patients (10%) were started on nasal cannula or high-flow nasal cannula, three patients (2%) were placed on continuous positive airway pressure, and 11 patients (8%) were mechanically ventilated via endotracheal intubation as a result of respiratory depression. Premature infants were more likely to experience respiratory depression (12/18, 67%) than term infants (26/117, 22%, p < 0.001). Mechanical ventilation was also more frequent in premature infants (6/18, 33%) than in term infants (5/117, 4%, p = 0.001).   

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Comparison Between Currently Recommended Long-Term Medical Management of Coronary Artery Aneurysms After Kawasaki Disease and Actual Reported Management in the Last Two Decades

Comparison Between Currently Recommended Long-Term Medical Management of Coronary Artery Aneurysms After Kawasaki Disease and Actual Reported Management in the Last Two Decades. Osborne J, Friedman K, Runeckles K, Choueiter NF, Giglia TM, Dallaire F, Newburger JW, Low...

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Identification of patients at risk of sudden cardiac death in congenital heart disease. The prospective study on implantable cardIoverter defibrillator therapy and sudden cardiac death in adults with congenital heart disease: Prevention-ACHD

Identification of patients at risk of sudden cardiac death in congenital heart disease. The prospective study on implantable cardIoverter defibrillator therapy and sudden cardiac death in adults with congenital heart disease: Prevention-ACHD. Vehmeijer JT, Koyak Z,...

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