Sunil J. Ghelani

Demographics, Trends, and Outcomes in Pediatric Acute Myocarditis in the United States, 2006 to 2011

Background—There is a lack of clear diagnostic and management guidelines for acute myocarditis in the pediatric population. We used a multi-institutional database to characterize demographics, practice variability, and outcomes in this population. Methods and Results—Patients with acute myocarditis (n=514) were identified from April 2006 to March 2011 using the Pediatric Health Information System database, and regional variations in management and outcomes were analyzed. Ninety-seven patients (18.9%) received extracorporeal membrane oxygenation, 22 (4.3%) received ventricular assist device, 21 (4.1%) received heart transplantation, and 37 (7.2%) died. Of the 104 patients who received extracorporeal membrane oxygenation or ventricular assist device, 17 (16.3%) had heart transplantation, 25 (24%) died, and 62 (59.6%) showed recovery of myocardial function. There was a decrease in the use of endomyocardial biopsy (P=0.03) and an increase in the use of magnetic resonance imaging (P<0.01) over the study period. Although the use of medications and procedures varied between different regions, the occurrence of death or heart transplantation showed no significant regional associations. The use of extracorporeal membrane oxygenation (odds ratio, 5.8; 95% confidence interval, 2.9–11.4; P<0.01), ventricular assist device (odds ratio, 8.2; 95% confidence interval, 2.7–24.9; P<0.01), and vasoactive medications (odds ratio, 5.7; 95% confidence interval, 1.2–26.1; P=0.03) was independently associated with death/transplantation. Conclusions—There is significant temporal and regional variation in the diagnostic modalities and management used for pediatric myocarditis, which continues to have high morbidity and mortality. Extracorporeal membrane oxygenation, ventricular assist device, and vasoactive medications are independently associated with increased mortality/transplantation.

Endothelial dysfunction in a cohort of North Indian children with Kawasaki disease without overt coronary artery involvement

OBJECTIVE Kawasaki disease (KD) is a diffuse necrotizing vasculitis with predominant involvement of coronary arteries. Endothelial dysfunction has been implicated as an important event in the pathogenesis of arteriosclerosis, coronary vasoconstriction, hypertension, and myocardial ischemia. We examined the presence of endothelial dysfunction in North Indian children (of Caucasoid ethnicity) with KD without overt coronary artery involvement. METHODS Twenty children (mean age 8.4+/-2.3 years; range 4.5-12.1 years) in the convalescent phase of KD were studied. All had received intravenous immunoglobulin during the acute phase of the disease. The interval between acute episode and enrolment ranged from 3 to 78 months (mean 25.3+/-20.1 months). High-resolution ultrasonography was used to analyze brachial artery responses to reactive hyperemia (with increased flow causing endothelium-dependent dilatation) and sublingual nitroglycerine (causing endothelium-independent dilatation). Flow-mediated dilatation was also studied in an equal number of healthy age- and sex-matched controls. Carotid artery stiffness index (SI) was calculated and compared in all subjects using previously published equations. RESULTS Significant differences were observed between the percent flow mediated dilatation in children with KD (5.7+/-9.2%) compared with controls (12.2+/-8.9%, p=0.017). Sublingual nitroglycerine-mediated dilatation in children with KD was 28.5+/-12.3%. Carotid artery SI was higher in children with KD (2.81+/-0.77 U) when compared to controls (2.32+/-0.80 U), but it failed to meet statistical significance (p=0.058). CONCLUSION Brachial arteries of children with KD show evidence of endothelial dysfunction. This may point towards possibility of preclinical arteriosclerosis in children with KD even in the absence of coronary artery abnormalities.

Impact of pharmacotherapy on interstage mortality and weight gain in children with single ventricle.

OBJECTIVE.: Infants with single ventricle physiology have a high mortality and poor somatic growth during the interstage period. We retrospectively assessed the impact of pharmacotherapy in this population using a multicenter database. DESIGN AND RESULTS.: Records for 395 patients (63.5% boys) with single ventricle were obtained from the National Pediatric Cardiology Quality Improvement Collaborative registry. Median of five medications were prescribed per patient at discharge after stage 1 palliation (interquartile range 3 to 6); the most common medications being aspirin (95.7%), diuretics (90.4%), angiotensin convertase enzyme inhibitors (37.7%), proton pump inhibitors (33.4%), H2 receptor blockers (30.6%), and digoxin (27.6%). Interstage mortality was 9.4%. Digoxin use was associated with lower risk of death (P =.03) on univariable analysis, however no single medication was an independent predictor on regression analysis. Change in weight-for-age z-score was studied as outcome of somatic growth with 36.3% patients showing a decrease during the interstage period. Total number of medications prescribed to a patient showed a negative correlation with the interstage change in z-score (r = -0.19, P =.002). On univariable comparisons, use of metoclopramide and lansoprazole were associated with decreased z-score (P =.004 and.041, respectively) although linear regression failed to identify any agent as independent predictor. CONCLUSIONS.: Children with single ventricle have high mortality and a profound medication burden. No individual medication is independently associated with better survival or weight gain during interstage period. Despite widespread use, proton pump inhibitors and prokinetic agents are not associated with better outcomes and may be associated with poor growth.

Prenatal Diagnosis and Management of Berry Syndrome, a Rare Conotruncal Anatomy.

Berry syndrome is a rare cardiac malformation made up of aortopulmonary window, interrupted aortic arch, aortic origin of right pulmonary artery, and intact interventricular septum.1 Prenatal diagnosis has the potential to improve outcomes with prompt initiation of prostaglandin E1 infusion and early surgery, thereby avoiding pulmonary overcirculation and heart failure. A 26-year-old primigravida presented to the fetal cardiology clinic at 23 weeks’ gestation with a diagnosis of vasa previa, suspected congenital heart disease, and inability to visualize the aortic arch clearly on the obstetric ultrasound. A fetal echocardiogram at 25 weeks’ gestation demonstrated the constellation of Berry syndrome (Figure, A and Movie I in the online-only Data Supplement). The mother delivered a 2-kg female infant at 35 weeks as a result of premature rupture of membranes …

Demographic and Treatment Variability of Refractory Kawasaki Disease: A Multicenter Analysis From 2005 to 2009

OBJECTIVE Approximately 10% to 15% of Kawasaki disease (KD) is refractory to treatment with initial intravenous immunoglobulin (IVIG). However, there is no consensus on pharmacologic treatment of refractory KD (rKD). Demographic characteristics of patients with rKD and regional variability in their treatment in the United States have not been reported on a large scale. The goal of this study was to describe the demographic and treatment variability in rKD by using a large multi-institutional database. METHODS Data were obtained for patients with KD from January 2005 to June 2009 by using the Pediatric Health Information System. Patients who received a single dose of IVIG were labeled as having standard KD (sKD) and those who required additional medications were labeled as having rKD. RESULTS Of the 5633 patients studied, 4818 (85.5%) received 1 dose of IVIG (sKD) and 815 (14.5%) received >1 medication (rKD). Median age was 30 months (interquartile range: 14-53) and 30 months (interquartile range: 15-54) for rKD and sKD patients, respectively (P= .438). No significant change was noted in the gender or ethnic distribution of patients between rKD and sKD groups. Seasonal distribution of rKD was comparable to sKD. IVIG was the most common (64.5%) initial medication chosen to treat rKD, followed by methylprednisolone (27.1%) and infliximab (8.3%); however, there was significant regional variability. Of patients with rKD, 81% required only 1 additional medication (after the initial IVIG) for treatment. CONCLUSIONS Patients with rKD have similar age, gender, ethnic, and seasonal distribution as sKD patients. IVIG is the most common initial medication chosen to treat rKD; however, there is regional variation.

Three-Dimensional Mitral Valve Morphology and Age-Related Trends in Children and Young Adults with Structurally Normal Hearts Using Transthoracic Echocardiography

Background: The mitral valve has a complex three‐dimensional (3D) morphology that is incompletely described by two‐dimensional echocardiography (echo). Three‐dimensional echo provides a more robust tool to analyze the mitral valve. The shape of the mitral annulus and leaflets, and differences with age, have not been described by 3D echo in normal children. Our objective was to characterize and quantify the 3D mitral valve morphology in children with normal transthoracic echocardiograms over a broad spectrum of age and to identify differences in valve shape with age. Methods: Three‐dimensional midsystolic mitral valve models were constructed in 100 children and young adults with normal echocardiograms using 3D transthoracic images. Annular and leaflet metrics were quantified and regression equations were prepared. Interuser and intrauser variability was measured. Results: Two hundred fifty patients, from neonate to young adult, were retrospectively reviewed to obtain 100 evaluable patients (40% evaluable). The annular height to commissural width ratio of the mitral valve (“saddle shape”) was preserved across age (median 24.3, IQR 21.8–28.1). Three‐dimensional mitral valve area, length, and volume parameters were linearly related to body surface area (P < .001). The ratio of anterior to posterior leaflet length and posterior leaflet angle increased with body surface area (P = .0004 and .002, respectively) suggesting posterior movement of the coaptation line. Two‐dimensional lateral annular diameter underestimated 3D lateral annular metrics (P < .001, mean difference 20–22%) but was highly correlated (R > 0.87, P < .001). Interuser and intrauser variability were acceptable. Conclusions: Assessment of 3D mitral valve morphology in children is possible in a modern clinical pediatric echocardiography laboratory using transthoracic images, although further optimization of imaging is needed. The saddle shape of the mitral annulus was preserved across age and size. Most mitral valve parameters increased linearly with patient size. Further investigation is warranted to explore changes in valve morphology in the pediatric population in health and with disease. HighlightsAssessment of three‐dimensional mitral valve morphology in children is possible in a modern clinical pediatric echocardiography laboratory using transthoracic images, although further optimization of imaging is needed.The saddle shape of the mitral annulus was preserved across age and size.Most mitral valve parameters increased linearly with patient size, however, the ratio of anterior to posterior leaflet length and posterior leaflet angle increased with BSA suggesting posterior movement of the coaptation line.Two‐dimensional lateral annular diameter systematically underestimated three‐dimensional lateral annular metrics, but was highly correlated.

PROLONGED QRS, MECHANICAL DYSSYNCHRONY AND VENTRICULAR DILATION ARE ASSOCIATED WITH VENTRICULAR ARRHYTHMIAS LATE AFTER THE FONTAN OPERATION

Ventricular tachycardia (VT) and sudden cardiac death are not uncommon after the Fontan operation; however their association with ventricular function, dyssynchrony, and QRS duration has not been explored. Subjects with the Fontan circulation and a CMR were retrospectively identified. Primary

Angiographically detectable Thebesian veins are a dynamic and reversible finding in the setting of congenital heart disease: Tannous et al.

OBJECTIVE Angiographically detectable Thebesian veins (ThVs) are a rare finding sometimes associated with coronary steal and myocardial ischemia in adults, but there are limited data regarding prominent ThVs in the setting of complex congenital heart disease (CHD). This study represents the largest series to date describing the presence and temporal changes of angiographically detectable ThVs in children with CHD. METHODS This is a single center case series describing the clinical characteristics and coronary anatomy in children with CHD and angiographicall detectable ThVs. After identification of the index case, additional patients were identified in a prospective manner during the course of routine clinical care. We developed a qualitative scale to grade ThV burden, with changes tracked over time in the subset of patients who underwent serial cardiac catheterizations. RESULTS A total of 10 patients are included in this report. There was a predominance of female gender (8 of 10 patients), right-dominant single ventricle physiology (7 of 10 patients), and heterotaxy syndrome (6 of 10 patients). The degree and location of epicardial coronary artery tortuosity was qualitatively related to ThV burden. The subset of patients who underwent serial cardiac catheterizations demonstrate that ThV dilation can progress or regress over time. CONCLUSIONS Angiographically detectable Thebesian veins are a rare finding in patients with congenital heart disease and may represent global changes in the coronary circulation. This is the first report, adult or pediatric, to demonstrate that ThV dilation is a dynamic process capable of both progression and regression. The physiologic impact of these findings remains to be elucidated.

Pulmonary arteriovenous malformations: The consequences of bypassing the capillary bed

completely resected after the removal of hemorrhage and blood clots in the thorax. Postoperative recovery was uneventful. No recurrence of symptoms was observed during a 5-year follow-up. PAVFs are rare vascular malformations of the lung. The cause may be congenital or acquired. Although more than half of patients with PAVFs are asymptomatic, the typical symptoms of fatigability, exertional dyspnea, and palpitations are frequently found in some patients. Hemothorax

Can Coronary Artery Involvement in Kawasaki Disease be Predicted

Background: Coronary artery involvement is seen in approximately 15–20% of children with Kawasaki disease. There is conflicting literature regarding the clinical and laboratory findings associated with coronary artery involvement. In this retrospective study, we attempt identification of predictive factors for coronary artery involvement at our institute and review the existing literature. Methods and results: A review of 203 patients (65% males) with Kawasaki disease was performed, of whom 33 (16.3%) had coronary artery involvement. High erythrocyte sedimentation rate, high platelet count, low hematocrit, low albumin levels, and refractory Kawasaki disease showed significant association with coronary artery involvement. High erythrocyte sedimentation rate and refractory Kawasaki disease were found to be independent predictors of coronary artery involvement. Review of literature suggested a wide range of coronary involvement (<5% to >60%), and highly conflicting clinical and laboratory associations. Conclusion: It remains difficult to accurately determine risk of coronary artery involvement, although some laboratory markers may provide information that is helpful for parental counseling and clinical follow up. Future identification of novel biomarkers and host predispositions may further our understanding of coronary artery risks and help personalize therapy for Kawasaki disease.