Artery In Children Research Articles

Myocardial Function Following Repair of Anomalous Origin of Left Coronary Artery from the Pulmonary Artery in Children

We aimed to assess the change in global and regional myocardial function before and after surgical revascularization and their added value when compared with conventional measures in children with anomalous left coronary artery from the pulmonary artery (ALCAPA). Advanced echocardiographic assessment was performed pre- and postoperatively in 22 children with ALCAPA (eight male; median surgery age, 0.4years; interquartile range, 0.21-1.05) and 22 healthy controls. Measurements included global and segmental longitudinal, radial, and circumferential two-dimensional speckle-tracking strain and postsystolic index. Global strains were lower in preoperative patients than in controls (longitudinal: -9% vs -21%; P<.001; circumferential: -11% vs -21%; P<.001; radial: 18% vs 60%; P<.001) and improved postoperatively when compared with preoperative findings (longitudinal: -9% pre vs -16% post; P=.002, circumferential:-11% pre vs -17% post; P=.012, radial: 18% pre vs 53% post; P=.001). Preoperatively, patients with normal global systolic function on conventional echocardiography had significantly impaired global longitudinal and radial strain compared with healthy controls. Global mechanical dyssynchrony improved significantly postoperatively (longitudinal postsystolic index 43 pre vs 6 post, P<.001; circumferential 15 pre vs 2 post, P=.001; radial 48 pre vs 5 post, P=.003). Despite overall improvement in most segments, global longitudinal and circumferential and segmental peak strain in some of the segments supplied by the ALCAPA remained postoperatively abnormal. This study shows that myocardial deformation indices were a more sensitive measure of LV dysfunction in patients before and after ALCAPA repair than conventional echocardiographic measures. We believe, therefore, they should be added to routine preoperative and serial postoperative follow-up assessment.

Analysis of four children with anomalous origin of the left coronary artery from the right sinus with interarterial course

Objective: To investigate the clinical features and improve the diagnosis and treatment of anomalous origin of the left coronary artery from the right coronary sinus with an interarterial course (ALCA-R-IAC) between the ascending aorta and main pulmonary artery in children. Methods: A retrospective analysis of the clinical manifestation, laboratory test, radiological feature, treatment and prognosis were conducted in four female children presented with ALCA-R-IAC in Beijing Children's Hospital from November 2015 to June 2018. Results: The four girls with onset age of 7.5-14.7 years were diagnosed with ALCA-R-IAC by CT coronary angiography (CTCA). Four children presented with exercise-induced syncope and clinical manifestations of acute myocardial infarction including 3 patients with acute left heart failure, 1 cardiogenic shock and 1 cardiac arrest. Nervous system involvement was found in one patient. Troponin I increased significantly to 20.65-50.00 μg/L in the four patients. Electrocardiogram (ECG) developed signs of left main coronary artery involvement. Echocardiography revealed reduced left ventricular ejection fraction (LVEF) of 25%-45% in three children and suspected anomalous origin of the left coronary artery in one child. CTCA showed an anomalous left coronary artery originating from the right coronary sinus, which had an interarterial course between the aorta and pulmonary artery leading to a slim left main coronary trunk. Two children underwent unroofing procedure and the other two children in whom physical activities were restricted received conservative managements. During a regular follow-up period of 12-43 months, all the children survived without recurrent symptoms and had good prognosis. Conclusions: ALCA-R-IAC can present as exercise-related syncope and acute myocardial infarction, even sudden death in children and adolescents. CTCA is helpful to clarify the early diagnosis of ALCA-R-IAC. Surgical intervention is the main treatment for ALCA-R-IAC and strenuous physical activities should be avoided.

Clinical analysis of rapid thrombocytopenia after percutaneous transcatheter closure of arteries in children

目的 探讨经皮动脉导管介入封堵术后血小板快速减少的临床特点、发病机制和防治措施。 方法 选择2017年1月至2018年12月青岛市妇女儿童医院心脏中心共实施经皮动脉导管介入封堵手术121例，其中6例术后发生血小板快速减少，回顾分析其相关临床资料，总结血小板快速减少发生的危险因素、临床特点、可能机制和防治措施。 结果 6例患儿血小板减少发生时间为术后第3－5天，血小板下降最低值为19×109/L，血小板恢复时间为术后7～20 d。造影测量动脉导管内径为4.0～4.5 mm，4例选用美国AGA公司生产规格9-PDA-0810堵闭器，2例选用上海形状记忆公司生产规格0810PDA堵闭器。骨髓学检查提示3例患儿骨髓产板型巨核细胞减少，免疫治疗后恢复良好。所有患儿血小板计数在出院时恢复正常。出院随访6～14个月，所有患儿血小板计数维持正常。 结论 经皮动脉导管介入封堵术后需警惕血小板快速减少，多无严重并发症发生，预后良好，免疫机制可能参与其中，需进一步研究。

P1280 Vascular and left ventricular mechanics after surgical correction of aortic coarctation of children

Abstract Background The main cause of the suboptimal long-term outcome after surgical correction of aortic coarctation(CoA) is late correction, residual CoA or reCoA. Cardiovascular remodeling after correction CoA in neonatal period without residual CoA is not well established. Purpose Evaluation of global longitudinal strain of the left ventricle (LV) and circumferential mechanics common carotid artery in children 5 years after surgical correction of the CoA in the neonatal period. Methods We examined 58 children aged 5,2 ± 0,39 years after successful repair CoA in neonate period. We excluded children with residual CoA or reCoA. Echo was performed with using vector velocity imaging. We studied LV longitudinal strain (LS) and strain rate (LSR). In the left common carotid artery we estimated: carotid strain (CC S) and strain rate (CC SR), fraction area change (FAC CC), carotid β2-stiffness index (CC β2), and intima-media thickness (IMT). The obtained data were compared with the results of 25 healthy children. Results LS LV and SR LV are worse of children after CoA correction compare with healthy, even LV ejection fraction is similar to healthy children. We found correlation LV mass index (LV MI) with CC S and CC SR. CC β2 of children after Co A repair was higher than of control group. CC β2 correlated with LS LV, SR LV and with LV MI. IMT and FAC didn’t differ of operated and healthy children. Conclusion Parameters of the LS of the LV and CC β2 of children 5 years after CoA repair significantly differ from healthy children and reflect the ongoing process of cardiovascular remodeling. Assessment of the CC S allows timely confirmation of the presence of increased arterial stiffening.

Prospective evaluation of obstructive sleep apnea syndrome, nasal flow and systolic pressure of the pulmonary artery in children with tonsillar hypertrophy

Prospective evaluation of obstructive sleep apnea syndrome, nasal flow and systolic pressure of the pulmonary artery in children with tonsillar hypertrophy

Transcranial Doppler Screening in Children with Sickle Cell Anemia Is Feasible in Central India and Reveals High Risk of Stroke

Introduction: India has been identified as having the second largest number of births with sickle cell anemia (SCA) in the world after Africa, with estimated 44,400 new-borns affected per year. SCD was previously reported to have a milder course in children from India, with less severe disease among aboriginal tribal populations than in non-tribal populations. Recent reports indicate the occurrence of severe manifestations of SCD in both tribal and non-tribal populations in India. Stroke is one of the serious complications of SCD, but there are no data on transcranial Doppler (TCD) screening for evaluating children with SCD in India who may be at high risk for strokes. The objective of this study was to assess the feasibility of using TCD to measure time averaged maximum of the mean velocities (TAMMV) in the intracranial arteries in children attending a tertiary centre in central India. Methods: STUDY DESIGN: A cross sectional study was conducted in consecutively recruited stable children of either sex with homozygous SCA proven by electrophoresis and high performance liquid chromatography in the age group of 1-26 years. Patients who were febrile, acutely ill, hypoxic or asleep were not included in the study as these conditions can falsely elevate the intracranial blood flow velocities. Patients with hemoglobinopathies other than HbSS or S/b0 Thalassemia and those with a history of congenital neurological illness were excluded. DETERMINATION OF TCD VELOCITY: TCD was performed in a tertiary care center in Nagpur using either an imaging machine (Lasiq s8) in the department of radiology or a portable non-imaging TCD (Compumedics); for both a probe of frequency 2Mhz was used. Maximum values for TAMMV in the Middle (MCA) and Anterior (ACA) cerebral arteries were measured in all; for the non-imaging TCD values for posterior cerebral artery (PCA) and basilar artery were also obtained. The results of the first scan performed on these individuals were included in this study. Using values similar to the STOP trial, TAMMV of each of these vessels were categorized as follows: Normal &lt;= 170cm/s; Conditional - between 170 and 199 cm/s; Abnormal &gt;= 200 cm/s; Low &lt;50 cm/s and unobtainable. MEASUREMENT OF HAEMATOLOGICAL VALUES: Laboratory parameters such as Hemoglobin, white blood cell count (WBC), Mean corpuscular volume (MCV) and hemoglobin F (HbF) levels of the patients in the study were also included if the parameters were available on the day of TCD or within 90 days of TCD study. MEASUREMENT OF HEIGHT, WEIGHT AND BMI: The height and weight of each of the patients on the day of TCD or within a period of 60 days from the TCD were measured and the body mass index (BMI) was calculated. Results One hundred and twenty children and youth aged 1-26 (median 7) years, 67 male (56%), were recruited. Of the 120 patients, 106 (88.5%) belonged to the Scheduled Caste category, 3 (2.5%) to the Scheduled Tribe category and 11 (9.1%) to the Other Classes category. Three (2.5%) had had a clinical stroke and 8 (7%) had had seizures, one of whom also had a stroke. Twenty-seven (23%) children had TAMMV outside the normal range. Five had abnormal TAMMV in the MCA (n=4) and/or ACA (n=1), 8 had conditional TAMMV in the MCA (n=7) and/or ACA (n=1) while 14 patients had low (n=12) or unobtainable (n=2) TAMMV in the MCA. One child with stroke had low TAMMV and one had conditional TAMMV while the third had normal TAMMV. Of the 7 with isolated seizures, one had low TAMMV and one had conditional TAMMV while the remaining 5 were normal. BMI was 8.6-25.3 (median 14.1), height/weight was 3.4-10.3 (median 6.5), hemoglobin was 43-134 (median 81) g/L, oxygen saturation 87-100 (median 99)%, HbF was 1.9-60 (median 21) g/dL, MCV was 59.1-96.7 (median 83.2) fl, WBC was 2.3-35.9 (median 10.1)*109. Those with TAMMV outside the normal range were not different from those with normal TAMMV in terms of age, BMI, Height/weight, or recent hemoglobin, oxygen saturation, HbF, MCV, or WBC. Discussion This study demonstrates the feasibility and importance of TCD screening in Indian SCD population. TAMMV on TCD was outside the normal range in nearly a quarter of children with SCD, as has been reported in studies in other populations. The findings of this study may not be representative of stroke risk in tribal populations since they were underrepresented in this study. These data provide the rationale for implementing systematic screening with TCD to reduce the risk of stroke in children affected by SCD in India. Disclosures Darbari: Novartis: Membership on an entity's Board of Directors or advisory committees; Hilton Publishing: Membership on an entity's Board of Directors or advisory committees; Global Blood Therapeutics: Other: one day advisory board meeting .

P4692Pronostic factors after arterial switch operation for transposition of the great arteries in children

Abstract The objective of this study was to assess long-term outcome and factors associated wih mortality in children after arterial switch operation (ASO) for transposition of the great arteries (TGA). Material and methods Retrospective analysis of all ASO performed from 2000 to 2016 in patients with TGA. Demographics, clinical and biological data, surgical techniques, echocardiographic measurements, and outcomes were collected. Pronostic factors for mortality were assessed. Results 364 patients were included (263 males). Antenatal diagnosis was made in 60,4%. Birth weight was 3207,5±511.2g, gestational age was 39.1±1.6weeks. Intramural coronary artery was present in 24 cases (6.6%). Long term follow-up was 9.2±4.8 years. Rashkind procedure was performed in 88.7% of the cases. Ventilatory support was needed in 56% and prostaglandins in 85% preoperatively. ASO occurred at the age of 7.44±6.26 days (1 to 56) and 5.6±4.6 days after admission. Bypass duration was 123.1±33.3mn. Overall and postoperative hospital stay were respectively 19.9±8.6 and 14.4±7.3 days. Survival rates were 95.3% at 1 month, 94.8% at 3, 6 and 12 months and 94.5% at 2.5 years and up to 18 years after surgery. Twenty patients died (5.5%): 17 early postoperatively (4.7%). Time from surgery to death was 64.2±207.9 days (0 to 919). Long-term events occurred in 49.6% of the patients: pulmonary stenosis, aortic or mitral regurgitation, residual shunt, coronary lesions (1.7%), arrhythmias (5.5%), cardiac failure. Freedom from late reintervention was 99.7%, 97.4%, 96.2% at 1, 3 and 6 months, 95.9%, 95%, 92.3% and 90.3% at respectively 1, 2, 5 and beyond 8 years after ASO. No Rashkind, postoperative ECMO support, delayed chest closure, bypass duration, troponine level and small birth weight were significant predictive factors of mortality (respectively p=0.006, p&lt;0.0001, p=0.0007, p&lt;0.0001, p=0.001 and p=0.044). Antenatal diagnosis, gender, gestational age, preoperative ventilatory support, prostaglandin infusion, and SpO2, coronary arteries anatomy or age at surgery were not associated with death. Conclusion This study showed that Rashkind procedure may have a favourable impact on prognosis of neonates with TGA. Postoperative ECMO support and troponine level, and small birth weight were associated with worse outcome.

A child in shock: carotid blowout syndrome

Paediatricians commonly encounter neck lumps during their routine clinical practice; vascular abnormalities, such as (pseudo)aneurysms, are a rare cause of these. Pseudoaneurysms of the carotid artery in children are usually...

Evolution of obstructive sleep apnea syndrome, nasal flow and systolic pressure of the pulmonary artery in children with indication for adenoidectomy and/or tonsillectomy over 18 months

BackgroundObstructive sleep apnea syndrome in childhood has aroused great interest due to its cardiovascular repercussions and its adverse effects on the quality of life of the affected individuals. However, fundamental aspects of the syndrome remain unknown. ObjectiveHerein we prospectively assessed pulmonary artery systolic pressure (PASP) and nasal flow in children with obstructive oral breathing with an indication for adenoidectomy and/or tonsillectomy and their relationship to the obstructive apnea and hypopnea index (OAHI). MethodsTwenty-one children were evaluated at the time of the surgical indication (T0) and 18 months later (T1). Polysomnography, and rhinomanometry data were collected when we evaluated PASP. ResultsAmong the 21 children, 13 (61.9%) presented an altered OAHI at T0. Fourteen children (66.7%) underwent surgery. Of these, nine (64.3%) had an altered OAHI at T0 and seven (50.0%) at T1. Of the seven non-operated children, four (57.1%) had an altered OAHI at T0 and two (33.3%) at T1. Mean nasal flow increased in both groups independently of surgery (p- ≤ 0.001). PASP exhibited a significant reduction between T0 and T1 in the operated group (p ≤ 0.001). OAHI of the operated group did not show a significant decrease over time (p = 0.074). In the non-operated children, the average nasal flow increased (p < 0.001), the PASP values did not reduce (p = 0.99), and the OAHI increased and then decreased over time (p = 0.025). ConclusionPASP decreased significantly and OAHI did not normalize in the operated group. Mean nasal airflow increased in the operated and non-operated groups.

ESTIMATION OF THE THICKNESS OF THE INTIMA-MEDIA COMPLEX IN CHILDREN WITH FAMILIAL HYPERCHOLESTEROLEMIA

Objective:to evaluate the diagnostic significance of measuring the thickness of the intima-media complex (IMC) in children with autosomal dominant familial hypercholesterolemia for early diagnostics and prompt treatment of atherosclerosis.Materials and methods.The study included 109 children – 64 children with familial hypercholesterolemia and 45 healthy children. Both groups were divided into 2 subgroups according to the age – from 3 to 8 years and from 9 to 18 years. We measured the intimamedia thickness (IMT) of the common carotid artery in all the children. To evaluate IMT we used an ultrasound scanner HD11XE (Philips, USA) with a linear (3–12MHz) sensor IMT.Results.We found a statistically significant difference (p=0.012) of the IMT of the common carotid artery in children with familial hypercholesterolemia (0.61 ± 0.02 mm) in comparison with the control group (0.49±0.02 mm), starting from the age of 9 years. There were no sex differences of IMT in patients older than 9 years.Conclusion.We found that children with familial hypercholesterolemia have higher values of IMT already from the age of 9 as compared with healthy children; the increase in TCIM is an additional criterion for the early diagnostics of atherosclerosis and evaluation of cardiovascular risk. These results emphasize the relevance of lipid-lowering therapy for patients with familial hypercholesterolemia in childhood, before the first signs of atherosclerosis appear.

Estimation of the intima-media thickness of the common carotid artery in children with familial hypercholesterolemia

Estimation of the intima-media thickness of the common carotid artery in children with familial hypercholesterolemia

Role of Circulatory Hypoxia in the Development of Retinopathy of Prematurity

Purpose: to study the pathogenesis of retinopathy of prematurity.Patients and methods.642 preterm infants born before 30 weeks gestation with the weight less 1500 grams were examined. The study included: indirect ophthalmoscopy; digital retinoscopy; calibrometry of retinal vessels; dopplerography; analysis of partial pressure of oxygen and carbon dioxide in the capillary blood. Results. The first phase of retinopathy of prematurity (ROP) occurred due to the immaturity of the mechanisms of autoregulation of the blood flow and the inadequate reaction of immature retinal vessels to oxygen. It was established: the less gestational age of a child, so there are less mechanisms of autoregulation of hemodynamics. The perverse reaction of autoregulation leads to pronounced arteriospasm, increasing the zone of hypoxia, and triggering pathological angiogenesis. In morphologically immature children, arteriospasm of retina vessels is more often detected, and narrowing degree of vessels is higher. Arteriospasm is defined in 82 % of children born at the age of gestation from 25 to 27 weeks, in 67 % — at a period from 28 to 29 weeks, in 54 % — from 30 to 32 weeks. In children with ROP in the preclinical period of the disease, retinal angiospasm is noted at values of pO2 in capillary blood less then 40 mm Hg or exceeding the upper limit of the norm values of pCO2 (the norm values of pO2 are 40–60 mmHg, pCO2 are 35–45 mm Hg). Autoregulation of blood flow is immature and inadequate. In the group before 30 weeks of post-conceptual gestational age without development of ROP, a spastic reaction of retinal arteries was revealed at the background of subnormal pO2 values in capillary blood. Biochemical autoregulation of blood flow is still immature. But from the 30 week of post-conceptual gestational age arteriospasm of retina is formed at values of pO2 of 45 mm Hg or higher (p &lt; 0.05). The normal caliber of retinal arteries in children without development of ROP is revealed at the background of mean values of pO2 below 45 mm Hg and values of pCO2 below 42 mm Hg before 30 weeks of post-conceptual gestational age, and above 45 mm Hg from the 30th week of post-conceptual gestational age. In children without ROP from 30 weeks of gestational age biochemical autoregulation of blood flow functions. It is important the degree of an angiospasm for the development of ROP. Dopplerographic critical index of blood flow disturbance in the ophthalmic and anterior cerebral arteries has been established — the index of resistance (IR) exceeding 0.8. It is a precursor of the development of ROP and pathological angiogenesis. In the group of children with ROP, the IR of the anterior cerebral artery is 0.84 ± 0.02 (in av.). The IR of the ocular artery was 0.83 ± 0.03. In higher index (up to 0.9–1), the process in the eye was more active.

Normal abdominal aorta diameter in infants, children and adolescents

The normal limits of arterial diameters must be known to diagnose pathologies such as dilatation, stenosis and hypoplasia. Thoracic aorta diameter in childhood has been measured in previous studies, but the number of studies in which normal aortic diameter is determined is very small. The aim of this study was therefore to determine the normal standard diameter of the aorta and iliac arteries in children according to gender and level. Pediatric abdominal computed tomography carried out for various reasons was retrospectively re-evaluated between January 2016 and February 2017. Patients were classified according to age into six groups. Vessel diameter, age, gender and vertebra diameter were evaluated. Mean proximal aorta diameter was 8.84mm for 12-36months, 10.02mm for 37-84months, 12.32mm for 85-132months, 14.45mm for 133-180months, and 16.51mm for 181-204months. Mean distal aorta diameter was 6.67mm for 12-36months, 8.07mm for 37-84months, 10.79mm for 85-132months, 12.14mm for 133-180months, and 14.01mm for 181-204months. The lumen diameter of the abdominal aorta, both proximally and distally, correlated linearly with age as an independent predictor. Similarly, the lumen diameter of the right and left common iliac artery correlated linearly with age as an independent predictor. There was no statistically significant difference in vascular diameter according to gender in any age groups. Optimal evaluation of aortic diameter is possible on computed tomography. Measurement outside the normal range is a sign of aneurysm or hypoplasia.

Cardiac Magnetic Resonance to Evaluate Percutaneous Pulmonary Valve Implantation in Children and Young Adults.

Experience with cardiac magnetic resonance to evaluate coronary arteries in children and young adult patients is limited. Because noninvasive imaging has advantages over coronary angiography, we compared the effectiveness of these techniques in patients who were being considered for percutaneous pulmonary valve implantation. We retrospectively reviewed the cases of 26 patients (mean age, 12.53 ± 4.85 yr; range, 5-25 yr), all of whom had previous right ventricular-to-pulmonary artery homografts. We studied T2-prepared whole-heart images for coronary anatomy, velocity-encoded cine images for ventricular morphology, and function- and time-resolved magnetic resonance angiographic findings. Cardiac catheterization studies included coronary angiography, balloon compression testing, right ventricular outflow tract, and pulmonary artery anatomy. Diagnostic-quality images were obtained in 24 patients (92%), 13 of whom were considered suitable candidates for valve implantation. Two patients (8%) had abnormal coronary artery anatomy that placed them at high risk of coronary artery compression during surgery. Twelve patients underwent successful valve implantation after cardiac magnetic resonance images and catheterization showed no increased risk of compression. We attempted valve implantation in one patient with unsuitable anatomy but ultimately placed a stent in the homograft. Magnetic resonance imaging of coronary arteries is an important noninvasive study that may identify patients who are at high risk of coronary artery compression during percutaneous pulmonary valve implantation, and it may reveal high-risk anatomic variants that can be missed during cardiac catheterization.

Evaluation of Cardiac Coronary Artery in Children with Kawasaki Disease by Echocardiography

&lt;p&gt;Kawasaki disease is an acute, unknown etiology of vascular inflammatory response syndrome, heart coronary artery changes as one of the main clinical manifestations of Kawasaki disease. How to determine the effective changes of coronary heart disease in children has become a lot of research scholars concern. With the continuous development of medical technology in recent years, echocardiography is widely used in the clinical diagnosis of various diseases and for the effective development of clinical treatment had a positive impact. A large number of research scholars found that by echocardiography, it can effectively achieve the diagnosis of coronary heart disease in children with Kawasaki disease, in order to lay a good foundation for the development of clinical treatment.&lt;/p&gt;

Tissue expander-stimulated lengthening of arteries for the treatment of midaortic syndrome in children

BackgroundMidaortic syndrome (MAS) is a rare condition characterized by stenosis of the abdominal aorta. Patients with disease refractory to medical management will usually require either endovascular therapy or surgery with use of prosthetic graft material for bypass or patch angioplasty. We report our early experience with a novel approach using a tissue expander (TE) to lengthen the normal native arteries in children with MAS, allowing primary aortic repair without the need for prosthetic graft material. MethodsWe conducted a retrospective review of patients with MAS undergoing the TE-stimulated lengthening of arteries (TESLA) procedure at our institution from 2010 to 2014. Data are presented as mean (range). ResultsFive patients aged 4.8 years (3-8 years) underwent the TESLA procedure. Stages of this procedure include the following: stage I, insertion of retroaortic TE; stage II, serial TE injections; and stage III, final repair with excision of aortic stenosis and primary end-to-end aortic anastomosis. Stage II was completed in 4 months (1-9 months) with 12 (7-20) TE injections. Goal lengthening was achieved in all patients. Stage III could not be completed in one patient because of extreme aortic inflammation, which precluded safe excision of the aortic stenosis and required use of a prosthetic bypass graft. The other four patients completed stage III with two (one to three) additional vessels also requiring reconstruction (renal or mesenteric arteries). At 3.2 years (1-6 years) of follow-up, all patients are doing well. ConclusionsThe TESLA procedure allows surgical correction of MAS without the need for prosthetic grafts in young children who are still growing.

The role of echocardiography in Kawasaki disease.

Kawasaki disease (KD) is an acute, self-limited vasculitis affecting young children. It can result in coronary artery abnormalities in a significant proportion of patients, especially if the diagnosis is missed or treatment gets delayed. Echocardiography is the imaging modality of choice for detection of coronary artery abnormalities and assessment of myocardial function. It is also useful for characterization and risk stratification of patients with KD. Echocardiography should be performed at the time of diagnosis and then again at 1-2 weeks and 4-6 weeks after treatment, for uncomplicated cases who do not have significant coronary artery involvement. Use of a standardized imaging protocol is necessary to detect and characterize coronary artery abnormalities, including standardization of measurements (Z scores). For patients with evolving abnormalities, more frequent assessment is necessary in order to detect thromboses in aneurysms. Long-term prognosis and management is dependent on both the maximal and current Z scores of aneurysms. Patients with large or giant aneurysms (i.e., Z score ≥ 10) are at the highest risk of both thrombosis and stenosis. Such patients need careful follow-up for subsequent cardiovascular events. Many of them would be candidates for advanced cardiovascular imaging and may require revascularization therapy. Serial echocardiography plays a key role in surveillance. In addition, stress echocardiography has proven useful as a modality to assess for inducible myocardial ischemia. Intravascular ultrasound has been recommended for functional and structural assessment of coronary arteries in children with KD.

Computed Tomography Coronary Angiography for Evaluation of Children With Kawasaki Disease

We sought to assess the feasibility of computed tomography coronary angiography (CTCA) on a 128-slice, dual source scanner in children with acute and convalescent phase Kawasaki disease (KD). A prospective study of 49 children with KD (12 at presentation and 37 in the convalescent phase) was conducted between November 2013 and April 2015. CTCA was performed with either prospective (n = 37) or retrospective (n = 12) electrocardiographic gating. A radiologist blinded to clinical profile and echocardiogram evaluated each scan. Median age (36 boys and 13 girls) was 7 years. Median dose-length product value and median effective CT radiation dose was 32mGycm (interquartile range [IQR]: 21-74) and 0.54 miliSieverts (mSv) (IQR: 0.77-3.2) for all scans, and 27mGycm (IQR: 18.5-33.75) and 0.48mSv (IQR: 0.18-1.17) for prospectively triggered scans (n = 37). Fourteen subjects (30 coronary segments) showed abnormalities by CTCA including aneurysms (n = 27) and stenoses (n = 3). In the acute phase (n = 12), aneurysms were detected in 5 children (18 segments). CTCA allows comprehensive evaluation of coronary arteries in children with KD.

Surgical Outcomes of Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery in Children: An Echocardiography Follow-up.

Background:Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare but potentially life-threatening congenital heart defect. A retrospective analysis was carried out to elucidate the surgical outcomes of ALCAPA in infants and children using follow-up echocardiography.Methods:From September 2008 to March 2017, 26 children diagnosed with ALCAPA underwent left coronary re-implantation. All surviving patients received echocardiography during follow-up.Results:The mortality rate after the operation was 11.5%. Before repair, twenty patients (76.9%) presented with left ventricular (LV) dysfunction. The mean Z-score of the preoperative LV end-diastolic diameter was 4.42 ± 2.09. Mitral regurgitation (MR) was present in all patients. Two patients (7.7%), both with mitral valve prolapse, underwent mitral valve repair at the time of ALCAPA repair. Two children required postoperative extracorporeal membrane oxygenation. LV function normalized at a median time of 5.3 months (range: 0.5–36.0 months). The Z-score of the LV end-diastolic diameter decreased simultaneously. The degree of MR gradually decreased in all surviving patients. All patients had patency of the proximal left coronary artery confirmed by echocardiography at the most recent follow-up. Six patients (26.1%) showed supravalvar pulmonary stenosis and seven patients (30.4%) showed right pulmonary stenosis during follow-up.Conclusions:Coronary re-implantation was effective for rebuilding a dual coronary system in patients with ALCAPA and resulted in progressive improved LV function and reduced functional MR. Echocardiography was valuable for evaluating the outcomes. LV function, the degree of MR, and possible complications could be detected with follow-up echocardiography.

Abnormalities of the Coronary Arteries in Children: Looking beyond the Origins.

Coronary arterial abnormalities are uncommon findings in children that have profound clinical implications. Although anomalies of the coronary origins are well described, there are many other disease processes that affect the coronary arteries. Immune system-mediated diseases (eg, Kawasaki disease, polyarteritis nodosa, and other vasculiditides) can result in coronary arterial aneurysms, strictures, and abnormal tapering of the vessels. Because findings at imaging are an important component of diagnosis in these diseases, the radiologist's understanding of them is essential. Congenital anomalies may present at varying ages, and findings in hemodynamically significant anomalies, such as fistulas, are key for both diagnosis and preoperative planning. Pediatric heart surgery can result in wide-ranging postoperative imaging appearances of the coronary arteries and also predisposes patients to a multitude of complications affecting the heart and coronary arteries. In addition, although rare, accidental trauma can lead to injury of the coronary arteries, and awareness and detection of these conditions are important for diagnosis in the acute setting. Patients with coronary arterial conditions at presentation may range from being asymptomatic to having findings of myocardial infarction. Recognition of the imaging findings is essential to direct appropriate treatment. ©RSNA, 2017.