Introduction: We recently reported that luminal myofibroblastic proliferation (LMP) is an active process of smooth muscle-cell derived myofibroblasts and their matrix products that can cause progressive coronary artery (CA) stenoses in KD patients. Serial echocardiography to assess for high risk CA changes, with periodic cardiac functional assessments in selected patients, is the current standard of care for monitoring patients with CA abnormalities. Monitoring for LMP/chronic thromboses is not routinely performed. Hypothesis: We hypothesized that echocardiographic images from KD children with severe CA abnormalities could suggest the presence of LMP and/or chronic thromboses, indicating the potential for progression to CA stenoses. Methods: We reviewed echocardiographic studies from a convenience sample of 28 KD patients with multiple aneurysms/giant aneurysms, to determine whether LMP and/or thrombosis could be identified on serial imaging. Results: In 12 of the 28 patients, images were highly suggestive of LMP and/or thromboses. This could only be assessed in the proximal segments of the left main, circumflex, left anterior descending and right CAs. Detection was enhanced by optimizing the depth and sector width for the coronary artery segment of interest and use of magnification on the review station. Variations in echocardiographic techniques such as low frequency probes, harmonics, persistence, excessive compress or low gain settings made the assessment difficult. 4 patients in this cohort required CA bypass and 1 died from myocardial infarction due to progressive LMP. Distal vessel occlusion could not be visualized by echocardiography. Conclusions: Images highly suggestive of LMP/chronic thromboses were evident in at least a third of patients with severe CA disease, including one patient who died of progressive LMP stenosis documented at autopsy. Echocardiography could not distinguish between LMP and chronic thrombosis as the cause of CA stenoses. Our study highlights the need for improved serial imaging for ongoing LMP/thromboses in AHA risk group IV. Patients with these complications may require more frequent cardiac functional assessments than are routinely recommended, to avoid adverse outcomes.

Few resources exist to educate cardiac sonographers regarding proper techniques and specific issues to consider when performing pediatric coronary artery imaging. The main objective of this report is to review the echocardiographic techniques used to image the coronary arteries of children when screening for anomalous origin of the coronary arteries, as well as coronary involvement in the setting of Kawasaki disease. The authors discuss the physics and instrumentation for developing optimal coronary artery imaging system settings and present the commonly used anatomic and echocardiographic views. Use of the correct settings and a thorough understanding of the associated ultrasound physics are crucial for obtaining quality images. With this report, the authors provide guidance to sonographers and a resource for pediatric echocardiography laboratories to help ensure high-quality echocardiographic imaging of the coronary arteries.

Background: The so-called Gerbode ventriculo-atrial defect is a rare defect that permits shunting from the left ventricle to the right atrium. It takes 2 forms, either a deficiency of the atrioventricular membranous septum, or shunting initially through a ventricular septal defect, with atrial shunting occurring through a deficiency in the septal leaflet of the tricuspid valve. In this review, we describe the natural history and outcomes of surgical repair for the variant with shunting through a deficiency at the site of the atrioventricular membranous septum. Methods: From 1990 to 2008, we identified 6 patients from our departmental database who had undergone surgical closure of a congenital defect of the atrioventricular component of the membranous septum. Median age at repair was 1.6 years, with a range, from 0.4 to 19 years. All patients were symptomatic, with 3 having congestive cardiac failure, 2 failing to thrive, and 2 having intolerance to exercise. All had a dilated right atrium demonstrated by echocardiogram, with a mean preoperative gradient measured by echocardiogram to be 109 millimetres of mercury, with a range from 65 to 150 millimetres of mercury. Results: There was no operative or late mortality. The mean size of the defect was 6.2 +/- 2.0 millimetres, with a range from 4 to 8 millimetres. All were closed by insertion of a patch. The mean period of cardiopulmonary bypass was 90.5 +/- 11.3 minutes, the mean time of aortic cross-clamping 54.8 +/- 6.9 minutes, and the mean length of stay in hospital 4.3 +/- 1.0 days. No patient had a residual defect, and only trivial regurgitation of the tricuspid valve was evident by postoperative echocardiography. There were no complications or reoperations. Conclusion: The membranous ventriculo-atrial defect can be recognized echocardiographically on the basis of dilation of the right atrium in the setting of an unusually high Doppler echocardiogram gradient compared to the ventricular septal defect with shunting only at ventricular level. Since all patients in our series were symptomatic, we recommend surgical closure of all these defects.

This report describes the first case of simultaneous transcatheter closure of an atrial septal defect and radiofrequency ablation of an accessory connection. This was performed successfully on an 8-year-old boy and represents an attractive therapeutic alternative to surgical repair in this combination of relatively common cardiac conditions. Cathet. Cardiovasc. Intervent. 51:55-57, 2000. (C) 2000 Wiley-Liss, Inc.

Coronary Artery Bypass Grafting for Kawasaki Disease at Children's Memorial Hospital, Chicago. • 696

A case of coronary artery dilatation in childhood, initially attributed to Kawasaki disease, was determined by careful serial echocardiography to be the result of congenital coronary arteriovenous fistulae.

Pectus excavatum, mitral valve prolapse (MVP), and dilated aortic root occur frequently in patients with Marfan's syndrome (MS). Patients with isolated pectus excavatum (IPE) have a high prevalence of MVP, but it is not known whether aortic root dilatation is a risk in those patients. To test the hypothesis that IPE and MS represent a spectrum of connective tissue dystrophy with MV and aortic root involvement, two-dimensional (2D) echocardiography was used to measure the aortic root diameter and assess for MVP in IPE (n=31), MS (n=14), and normal (n=16) gender- and age-matched patients. Aortic root was measured in parasternal long- and short-axis views, just above the aortic sinuses, at end systole, in six cardiac cycles, and averaged. Parasternal long-axis view was used to assess for MVP.

Cryoprecipitates are postulated to play a role in the pathogenesis of several vasculitic illnesses and infectious diseases. To investigate the presence of cryoprecipitates in Kawasaki syndrome, we studied sera from 25 children with acute Kawasaki syndrome. None of the subjects was treated with intravenous gamma-globulin. Cryoprecipitates were detectable in sera of 11 of 25 (44%) children studied. The mean (±SE protein concentration of the cryoprecipitates was 88.0 (±20.2) Mg/ml serum. Cryoprecipitates consisted primarily of IgG and IgM; no complement components were detected but highly sensitive methods were not used. The presence of cryoprecipitates in the serum of children with acute Kawasaki syndrome was associated with the subsequent development of coronary artery aneurysms detected by echocardiogram (P <0.05). There was no association between detectable cryoprecipitates and either peak platelet count or erythrocyte sedimentation rate. In one patient, measurement of cryoprecipitates in serial samples showed a reduction in concentrations that paralleled subsidence of disease activity. We speculate that cryoprecipitates may be a marker for increased risk of coronary aneurysm formation and may play a role in the pathogenesis of the cardiac disease in Kawasaki syndrome.

EchocardiographyVolume 5, Issue 1 p. 43-52 Diagnosis of Anomalous Origin of Left Coronary Artery from Pulmonary Artery by Comprehensive Echocardiography MOHAMED SELIEM M.D., MOHAMED SELIEM M.D. Division of Cardiology, The Children's Memorial Hospital, Chicago, Illinois.Search for more papers by this authorC. ELISE DUFFY M.B.B.S., F.R.A.C.P., Corresponding Author C. ELISE DUFFY M.B.B.S., F.R.A.C.P. Division of Cardiology, The Children's Memorial Hospital, Chicago, Illinois.Address for correspondence: C. Elise Duffy, M.D., Division of Cardiology, The Children's Memorial Hospital, 2300 Children's Plaza, Chicago, IL 60614.Search for more papers by this authorMICHEL ILBAWI M.D., MICHEL ILBAWI M.D. Division of Cardiology, The Children's Memorial Hospital, Chicago, Illinois.Search for more papers by this authorSERAFIN DELEON M.D., SERAFIN DELEON M.D. Division of Cardiology, The Children's Memorial Hospital, Chicago, Illinois.Search for more papers by this author MOHAMED SELIEM M.D., MOHAMED SELIEM M.D. Division of Cardiology, The Children's Memorial Hospital, Chicago, Illinois.Search for more papers by this authorC. ELISE DUFFY M.B.B.S., F.R.A.C.P., Corresponding Author C. ELISE DUFFY M.B.B.S., F.R.A.C.P. Division of Cardiology, The Children's Memorial Hospital, Chicago, Illinois.Address for correspondence: C. Elise Duffy, M.D., Division of Cardiology, The Children's Memorial Hospital, 2300 Children's Plaza, Chicago, IL 60614.Search for more papers by this authorMICHEL ILBAWI M.D., MICHEL ILBAWI M.D. Division of Cardiology, The Children's Memorial Hospital, Chicago, Illinois.Search for more papers by this authorSERAFIN DELEON M.D., SERAFIN DELEON M.D. Division of Cardiology, The Children's Memorial Hospital, Chicago, Illinois.Search for more papers by this author First published: January 1988 https://doi.org/10.1111/j.1540-8175.1988.tb00233.xCitations: 4AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat Citing Literature Volume5, Issue1January 1988Pages 43-52 RelatedInformation

Thirty-six children aged 6 months to 18 years, underwent insertion of 37 St. Jude Medical cardiac prostheses. In 20, the valve was placed in the aortic or mitral position, and in 16 in the pulmonary or tricuspid position. There was one (2.8%) hospital death. All patients received maintenance doses of salicylates and dipyridamole after the operation. Follow-up data are available for all patients for 12 to 24 postoperative months. There was no incidence of valve dysfunction or thromboembolic complication in any of the 20 patients with valves in the systemic (left) side of the circulation, and all manifested improvement in their functional class. In contrast, six (37%) of the 16 patients with valves in the pulmonary (right) side of the circulation developed dysfunction of the prosthesis 1 to 6 months after insertion. Prosthesis failure was associated with fibrous tissue growing into the struts, leading to leaflet immobilization. At 2 years, the actuarial functional life was 100% for mitral and aortic valves and 70% for pulmonary and tricuspid valves. The data illustrate the excellent hemodynamic function of the St Jude Medical valve in children. The absence of thromboembolic complications warrant continued implantation of the prothesis in the left side without warfarin anticoagulation therapy, but the high incidence of valve dysfunction in the pulmonary position does not justify its continued use in the right side.