Pediatric Cardiology

Kawasaki Disease and Clinical Outcome Disparities Among Black Children View Article Luz A Padilla 1, Jacqueline L Collins 2, Adeniyi J Idigo 3, Yung Lau 2, Michael A Portman 4, Sadeep Shrestha 3 J Pediatr. 2020 Sep 24;S0022-3476(20)31244-0.   doi: 10.1016/j.jpeds.2020.09.052. Online ahead of print.  PMID: 32980379.  PMCID: PMC7513890. DOI: 10.1016/j.jpeds.2020.09.052 Take-Home Points : Black children with Kawasaki disease have a higher prevalence of Intravenous Immunoglobulin therapy refractoriness when compared to white children. Black children present with increased inflammatory markers but lower albumin, sodium when compared to white children even though no difference exists in time from fever to admission in between both the groups. Black children receive more ancillary drugs and stay longer in the hospital when compared to white children with Kawasaki disease and have higher persistence of coronary abnormalities on follow up echocardiograms. The disparate outcomes seem to be more related to biologic/genetic variation than health care access and delivery in this cohort of patients admitted to a tertiary level children’s hospital in the South Eastern United States. Commentary from Dr. Venu Amula (Salt Lake City, USA), section editor of Pediatric & Fetal Cardiology Journal Watch:  Race and ethnic variation in the incidence and outcomes of Kawasaki disease have been well reported worldwide. Though racial differences exist in health care access and delivery, it is unknown whether the Black race poses a significant risk for poor outcomes in Kawasaki Disease. Data show that Black children may be disproportionately affected by the Kawasaki disease and manifest altered response to treatment. The authors conducted a retrospective observational cohort study of Black and White children who met the American Heart Association Criteria for Kawasaki Disease and admitted to a tertiary level children’s hospital between January 2000 to 2015. The hospital serves proportionate Black and White populations and gave the investigators a unique opportunity to evaluate any differences in Kawasaki disease characteristics and clinical outcomes between the two communities. The groups were classified by race as assigned by the parent report.  Children who failed to meet AHA criteria, presented 36 hrs. after Intravenous Immunoglobulin treatment rendered at an outside hospital, and those with inadequate documentation at admission were excluded. Patients were identified using ICD codes from the hospital database. The baseline characteristics, hospital presentation, treatment, and echocardiography findings during Kawasaki disease hospitalization were compared between the two racial groups. The study resulted in a final cohort of 369 patients, comprising 192 Whites and 177 Blacks with no significant differences in age at admission or sex between the two racial groups. Mean hemoglobin and hematocrit levels were significantly lower in Black children at admission. Inflammatory markers at admission were higher, particularly C-reactive protein (CRP) and erythrocyte sedimentation rate. There was no difference in time to treatment (initiation of IVIG infusion) or healthcare delivery in both racial groups.  Ninety-four percent of the Black children and 89.7% of the White children received IVIG within ten days of fever onset, per the AHA guidelines. Among those treated, Black children had a lower IVIG response rate compared with whites (86.6% vs. 95.6%; P = .007). IVIG refractoriness was defined as persistent or recurrent fever within 36 hrs. of completing the treatment. More Black children than White children received alternative therapies (9.6% vs. 2.6%; P = .003. And also had a longer average length of hospital stay.  Black children are also noted to have a higher proportion of persistent coronary abnormalities upon follow-up echocardiograms. This critical study evaluates whether disparate outcomes exist in black children with Kawasaki disease than white children in a tertiary level children’s hospital.  Even though racial differences exist in response to the disease and IVIG therapy refractoriness, there is no difference in time to intervention suggesting uniform health care provision. The outcomes and sequelae of Kawasaki disease may be related to underlying biological and genetic variation. This study is limited by being a single-center study with a high likelihood of selection bias. Nevertheless, genetic differences in response to treatment cannot be underestimated.  

[et_pb_section][et_pb_row][et_pb_column type="4_4"][et_pb_text] Ascending Aortoplasty in Pediatric Patients Undergoing Aortic Valve Procedures. View Article Tan CW, Marathe SP, Kwon MH, Chavez M, Friedman KG, Staffa S, Del Nido P, Baird CW. Ann Thorac Surg. 2020 Sep 15:S0003-4975(20)31477-6. doi: 10.1016/j.athoracsur.2020.06.115. Online ahead of print. PMID: 32946842 Take Home Points: Ascending aortoplasty at the time of aortic valve surgery is safe and effective in reducing ascending aortic dimensions and recurrent aortic regurgitation in the short/intermediate term. Longer term follow up will be necessary to determine continued rate of growth. Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch:  It is well known that hemodynamic and genetic factors can result in aortic root and ascending aorta dilation in patients with aortic valve disease. There are relatively clear guidelines in the adult population for when to intervene on the aorta during or independent of aortic valve surgery. However, the morbidity and mortality, the risk of dissection, and the impact on progressive aortic regurgitation in pediatric patients with ascending aorta dilation based on z-score is not well known. This results in a lack of guidelines within the pediatric population for intervention. Reduction aortoplasty can immediately reduce the size of the aorta, but it is unclear if this will prevent future growth and decrease risk of dissection/rupture or prevent future aortic regurgitation. The current general trend has been to accept higher aortic dimensions, but this may be result in the significant mortality. This was a retrospective study of patients between 2010-2018 who had an ascending aorta z-score of > +2 at the time of aortic valve surgery. Exclusions included patients with prior ascending aorta repair, single ventricle pathology, s/p ASO, heart transplant or known connective tissue disorder. 47 patients underwent aortoplasty with 39 having complete data points. They were compared with 39 matched controls. Echocardiograms pre-operatively, immediate post-operatively, and at latest follow up were reviewed for lateral ascending aorta dimensions in the PLAX and z-scores obtained based on BSA. The surgical technique was described in the paper and the goal was to reduce the ascending aorta size to a z-score between 0 and +2 or a decrease of at least 2 z-scores. A total of 39 subjects with a median age of 11 years and weight of ~41 kg were compared to 39 controls, with only BSA being dissimilar. No patients required a 2nd cross clamp due to inappropriate reduction of the aorta. Ozaki type reconstruction of the aortic valve was more common in the aortoplasty group. Table 2 shows the operative and post-operative details of each group. Within the study group, pre-operative mean ascending aorta z-score was 5.35+/-1.52 and reduced to 1.22+/-1.63 post-operatively. 29/47 patients had a z-score < +2. For the 39 patients with all-time points, median follow up was 12.5 months and ascending aorta z-score remained similar at 1.37+/-1.72 (Figure 2). Of the 12 patients with a post-operative z-score > +2, all remained with unchanged z-score (average change 0.14) at latest follow up. No differences in z-scores could be identified by aortic valve morphology, pathology or intervention, patient weight or BSA. In the control group, median follow up was 40.8 months. Pre-operative mean ascending z-score was 4.15+/-1.65 and was reduced to 3.26+/-2 at latest follow up that was deemed to be from greater somatic growth than aortic growth over time, resulting in a lower z-score. While the control group also had a significant reduction in average z-score, the reduction was significantly larger in the aortoplasty group (see Figure 3). The control group had 6.84 times the odds of moderate or greater aortic regurgitation (see Figure 4), and this was confirmed to not be related to type of aortic valve repair on secondary analysis. No patients in the aortoplasty group had any early post-operative complications. The authors note that rapid aneurysmal growth did not occur after aortoplasty. Additionally, there may be even greater benefit in those patients with residual aortic valve disease and persistent hemodynamic effect on the ascending aorta. Limitations of this study include the small sample size, retrospective design, and relatively short follow up duration. Additionally, it would have been nice if the authors would have included aortic root z-scores (sinuses and STJ) as well as the ascending aorta, and it is unclear why they were not. Hopefully, there will be longer term studies (greater than 10 years) and possibly including other imaging modalities (CT/MRI) looking at the absolute aortic dimensions and rate of change after aortoplasty. However, given the likely relatively low short and intermediate risk, as well as what seems to be a quite significant decrease in risk of progressive aortic regurgitation, it may be reasonable to consider aortoplasty in more patients.   [/et_pb_text][/et_pb_column][/et_pb_row][/et_pb_section]