A 63-Year-Old Male with D-Transposition of the Great Arteries Who Had an Early Form of the Arterial Switch Operation

We describe a 63-year-old male who appears to have undergone an early form of the arterial switch operation for D-transposition of the great arteries performed in the mid-1960s.We review the clinical and imaging data that support our conclusion.He had a diagnostic cardiac catheterization which demonstrated severe pulmonary hypertension responsive to epoprostenol and oxygen.Our case may represent one example of the experimental surgical work done prior to Dr.Adibe Jatene’s description of thefirst successful arterial switch performed in 1975.

Coarctation of the aorta:Management from infancy to adulthood

Coarctation of the aorta is a relatively common form of congenital heart disease, with an estimated incidence of approximately 3 cases per 10000 births. Coarctation is a heterogeneous lesion which may present across all age ranges, with varying clinical symptoms, in isolation, or in association with other cardiac defects. The first surgical repair of aortic coarctation was described in 1944, and since that time, several other surgical techniques have been developed and modified. Additionally, transcatheter balloon angioplasty and endovascular stent placement offer less invasive approaches for the treatment of coarctation of the aorta for some patients. While overall morbidity and mortality rates are low for patients undergoing intervention for coarctation, both surgical and transcatheter interventions are not free from adverse outcomes. Therefore, patients must be followed closely over their lifetime for complications such as recoarctation, aortic aneurysm, persistent hypertension, and changes in any associated cardiac defects. Considerable effort has been expended investigating the utility and outcomes of various treatment approaches for aortic coarctation, which are heavily influenced by a patient's anatomy, size, age, and clinical course. Here we review indications for intervention, describe and compare surgical and transcatheter techniques for management of coarctation, and explore the associated outcomes in both children and adults.

Effect of oral garlic on arterial oxygen pressure in children with hepatopulmonary syndrome

瞄准：与肝肺症候群在孩子在动脉的氧压力上学习口头的大蒜的效果。方法：在一种囊形式的大蒜粉末与肝肺症候群被给 15 个孩子(由对比回响心动描记法证实了) 在 1 g/1.73 m (2 ) 的剂量每天。病人们每四个星期临床上并且由动脉的血气体被评估。结果：大蒜囊与肝肺症候群予 15 个病人被以。与 9.4+/-3.9 年的吝啬的年龄有 10 个男孩和 5 个女孩。内在的问题是胆道闭锁(4 个病人) ，自体免疫的肝炎(4 个病人) ，起因不明的肝硬化(4 个病人) 和 presinusoidal 门静脉高血压(3 个病人) 。八个病人(53.3%) 在他们的吝啬的动脉的氧压力显示出 10 毫米汞柱的增加。基线 PaO (2 ) 在非应答者组是在应答者组和 47.1+/-11.2 毫米汞柱的 65.6+/-12.1 毫米汞柱。在治疗的目的，在应答者和非应答者的吝啬的 PaO (2 ) 是 92.2+/-7.75 毫米汞柱和 47.5+/-11.87 毫米汞柱，分别地(P【0.01 ) 。结论：大蒜可以增加氧化并且与肝肺症候群在孩子改进呼吸困难。

Anti-hypertensive drugs in children and adolescents

Worldwide the prevalence of essential hypertension inchildren and adolescents continues to increase. Tradi-tionally providers have used "off-label" drugs to treatpediatric hypertension, meaning that rigorous clinicaltrials of these drugs have not been specifically per-formed in pediatric patient populations. Consequentlyproviders have extrapolated dosing, safety and efficacyfrom trials in adults. This practice is sub-optimal as chil-dren demonstrate unique differences in drug metabo-lism and response. Use of unstudied or understudieddrugs increases risk of adverse events and/or can leadto sub-optimal efficacy. Recognizing these concerns,regulatory agencies have created financial incentivesfor industry to conduct pediatric clinical trials. Theseincentives, coupled with the emerging pediatric hyper-tension epidemic, have spurred over 30 clinical trialsof anti-hypertensive drugs over the past 15 years andhave resulted in labeling of 10 new drugs by the UnitedStates Food and Drug Administration for treatment ofhypertension in children and adolescents. Unfortunatelythe financial incentive structures focus on newer drugsand drug classes. Consequently there is now a relativedearth of trial data for older but sometimes commonlyprescribed pediatric antihypertensive drugs. This article reviews recent pediatric antihypertensive drug trials with a focus on trial design and endpoints, drug dosing, safety, efficacy and specific drug indications. We also review the available data and experience for some of the more commonly prescribed, but less well studied "older" pediatric antihypertensive drugs.

Effect of intravenous gamma globulin on short- and mid-term clinical outcome in acute viral myocarditis in children

Acute viral myocarditis is an extremely diverse disease with a predictable good outcome with supportive therapy. The objective of this study was to look at the clinical outcome of patients receiving additional intravenous gamma globulin compared to those receiving conventional anti-congestive therapy alone. Patients and methods: This is a retrospective review of charts of children admitted with acute myocarditis. Group 1 was children who received intravenous gamma globulin (IVIG) along with conventional anti-congestive therapy. Group 2 were patients who received the conventional anticongestive therapy alone. Short-term outcome was survival to hospital discharge, and mid-term outcome was improvement of left ventricular ejection fraction at 6 months and one year follow-up. Results: A total of 36 patients were enrolled and 18 were males. The mean age of all patients was 2.3 ± 2.6 years and the mean duration of illness for the whole group was 6.7 ± 3.8 days. Group 1 comprised of 16 patients. There was no difference in short-term outcome with mortality of 2 patients in Group1 (12.5%) and 3(15%) in Group 2 (p = 0.2). At intermediate term follow up, recovery of ejection fraction in Group 1 was in 4/14 (28%) and in Group 2 it was 9/17 (55%) and at 12 months it was 10/13 (77%) in Group 1 and 15/16 (94%) in Group 2 (p = 0.02 and 0.19 respectively). Conclusion: Acute myocarditis has a high spontaneous cure rate. Our study did not show any significant difference in the short-term or midterm outcome between children receiving IVIG compared to those who did not.

Coronary artery occlusion after arterial switch operation in an asymptomatic 15-year-old boy

A 15-year-old boy with transposition of the great arteries(TGA) and neonatal arterial switch operation(ASO) presented with complete occlusion of the left main coronary artery(LMCA). Intra-operatively, an intramural left coronary artery was identified. Therefore, since age 7 years he had a series of screening exercise stress tests. At 13 years old, he had 3 to 4 mm ST segment depression in the infero-lateral leads without symptoms. This progressed to 4.2 mm inferior ST segment depression at 15 years old with normal stress echocardiogram. Sestamibi myocardial perfusion scan and cardiac magnetic resonance imaging was inconclusive. Therefore, a coronary angiogram was obtained which showed complete occlusion of the LMCA with ample collateralization from the right coronary artery system. This was later confirmed on a computed tomogram(CT) angiogram, obtained in preparation of coronary artery bypass grafting. The case illustrates the difficulty of detecting coronary artery stenosis and occlusion in young patients with rich collateralization. Coronary CT angiogram and conventional angiography were the best imaging modalities to detect coronary anomalies in this adolescent with surgically corrected TGA. Screening CT angiography may be warranted for TGA patients, particularly for those with known coronary anomalies.

Safety and outcome of treatment of metastatic melanoma using 3-bromopyruvate: a concise literature review and case study

3-Bromopyruvate(3BP) is a new, promising anticancer alkylating agent with several notable functions. In addition to inhibiting key glycolysis enzymes including hexokinase II and lactate dehydrogenase(LDH), 3BP also selectively inhibits mitochondrial oxidative phosphorylation, angiogenesis, and energy production in cancer cells. Moreover, 3BP induces hydrogen peroxide generation in cancer cells(oxidative stress effect) and competes with the LDH substrates pyruvate and lactate. There is only one published human clinical study showing that 3BP was effective in treating fibrolamellar hepatocellular carcinoma. LDH is a good measure for tumor evaluation and predicts the outcome of treatment better than the presence of a residual tumor mass. According to the Warburg effect, LDH is responsible for lactate synthesis, which facilitates cancer cell survival, progression, aggressiveness, metastasis, and angiogenesis. Lactate produced through LDH activity fuels aerobic cell populations inside tumors via metabolic symbiosis. In melanoma, the most deadly skin cancer, 3BP induced necrotic cell death in sensitive cells, whereas high glutathione(GSH) content made other melanoma cells resistant to 3BP. Concurrent use of a GSH depletor with 3BP killed resistant melanoma cells. Survival of melanoma patients was inversely associated with high serum LDH levels, which was reported to be highly predictive of melanoma treatment in randomized clinical trials. Here, we report a 28-year-old man presented with stage IV metastatic melanoma affecting the back, left pleura, and lung. The disease caused total destruction of the left lung and a high serum LDH level(4,283 U/L). After ethics committee approval and written patient consent, the patient received 3BP intravenous infusions(1-2.2 mg/kg), but the anticancer effect was minimal as indicated by a high serum LDH level. This may have been due to high tumor GSH content. On combining oral paracetamol, which depletes tumor GSH, with 3BP treatment, serum LDH level dropped maximally. Although a slow intravenous infusion of 3BP appeared to have minimal cytotoxicity, its anticancer efficacy via this delivery method was low. This was possibly due to high tumor GSH content, which was increased after concurrent use of the GSH depletor paracetamol. If the anticancer effectiveness of 3BP is less than expected, the combination with paracetamol may be needed to sensitize cancer cells to 3BP-induced effects.

The Use of Trans-Esophageal Electrophysiology Study to Identify a High Risk Asymptomatic Wolff Parkinson White Syndrome Patient

Patients with a Wolff-Parkinson-White (WPW) pattern on their ECG can experience symptoms such as syncope, palpitations, supraventricular tachycardia, and atrial fibrillation, or they can be asymptomatic (aWPW). All patients with WPW, regardless of the presence or absence of symptoms, are at risk of sudden death. Therefore, it is recommended that younger patients with WPW undergo studies to determine their risk. We report a previously asymptomatic WPW patient identified as high risk for sudden death due to?rapid conduction down her accessory pathway during atrial fibrillation induced during a trans-esophageal electrophysiology study.

Congenital Coronary Artery Fistula in Children:A Review of 28 Cases with Clinical and Imaging Outcomes

Background:Congenital coronary artery fistula(CCAF)is a rare anomaly.Treatment strategies tend to close the defect with a symptomatic and significant shunt,primarily based on expert consensus and case series.Results for long-term follow-up in children are limited Methods:We conducted a retrospective study to assess clinical and imaging outcomes of children with CCAF at Siriraj Hospital,Thailand during 2000–2020.Patients with single ventricle were excluded.Treatment strategies[surgical closure(SC),and percutaneous closure(PC)]were classified and the clinical outcomes at the follow-up in 2021,including coronary thrombosis,myocardial ischemia,and the results of cardiovascular imaging were reviewed.Results:Twenty-eight children with CCAF were included in the study.The median age at diagnosis was 2.5 years(2 days–18 years).Presenting symptoms were audible murmur(82%)and heart failure(35%).Most of fistulae arose from the right coronary artery(12/28)and exited at the right atrium(11/28).In recent visits(0.5–14 years follow-up),six patients with asymptomatic small CCAF were managed by watchful follow-up without complications.PC was primarily treated in 11 children:7 underwent successful procedures;1 had a residual shunt and required re-intervention;1 had ischemic symptoms immediately after the procedure with left coronary occlusion that required device removal plus SC and 2 were technically unable to place the device,requiring SC.Four patients were waiting for interventions(1 PC and 3 SC).Cardiovascular imaging surveillance that followed closure demonstrated asymptomatic thrombus formation in three patients(1 PC and 2 SC).No mortality presented.Conclusion:CCAF with significant shunt is indicated to close either SC or PC.Ischemic events are rare but have been reported after closure.In addition,thrombus formation should be watched for post-intervention.Surveillance with cardiovascular imaging is recommended after defect closure(ideally 1–5 years post closure),or at interval follow-ups in patients with symptoms to evaluate possible recanalization,thrombus,or ischemia.Life-long clinical and echocardiographic follow-up is warranted.Watchful follow-up is acceptable for hemodynamically insignificant fistula without complication in the series.

Percutaneous Closure of Patent Foramen Ovale in a Patient with Mirror-Image Dextrocardia and Situs Inversus

A 26-year-old patient with mirror-image dextrocardia and situs inversus experienced a transient ischemic attack.We suspected that a patent foramen ovale was the reason.A Cardi-O-Fix occluder was used to close the patent foramen ovale with a mirror-reversed rotation of the radiologic views.During the 18-month follow-up,no symptoms of the transient ischemic attack appeared again.

Syncope in a 3-year-old male: A case report

Dear editor,Syncope is a common presenting complaint in pediatric emergency departments.The differential diagnosis of syncope is vast,including various neurological and cardiac conditions.Brugada syndrome(BrS)is a primary inherited arrhythmia which can present with syncope but can lead to sudden cardiac deaths due to ventricular fi brillation.BrS is a rare genetic condition,often diagnosed in the adulthood.BrS presenting in childhood,especially symptomatic BrS,is extremely rare.We present a case of a previously healthy 3-year-old male who presented to the emergency department with syncope and was later diagnosed with BrS.

无先前已知心脏病儿童的电轴左偏

研究背景在儿童中,电轴左偏与一些特殊类型的心脏病理有关,许多患儿在婴儿期得到确诊;但目前尚不清楚无症状的电轴左偏儿童的心脏病发生率,也不清楚这类儿童需要做哪些其他检查。研究新知本研究确认,无先前已知心脏病的电轴左偏儿童可能存在合并心脏病的危险因素,这些危险因素可以帮助临床医师在偶然发现电轴左偏时,决定哪些患儿应做进一步的检查。

Williams-Beuren syndrome: Usual face, unusual heart

Williams-Beuren Syndrome (WB-S) occurs in approximately 1/7500 live births. It is characterized by typical facial features, congenital heart defects and mild mental retardation. Around 75% - 80% of all patients have some kind of cardiovascular disorder being supravalvular aortic stenosis and pulmonary artery stenosis the most frequent. This syndrome is due to a contiguous gene deletion (1- to 2-megabase deletion on the long arm of chromosome 7), including the entire elastin gene and 20 additional genes. We present a case of a two year old boy with WB-S and Tetralogy of Fallot, a very infrequent association. Diagnosis of WB-S could be made because of typical facial features. Characteristic WB-S deletion was present. Genetic study to rule out 22q11 deletion was also performed.

Pulmonary Perfusion Asymmetry in Patients after Repair of Tetralogy of Fallot: A 4D Flow MRI-Based Study

Background:Repaired Tetralogy of Fallot(rTOF)patients may have residual lesions such as main(MPA)and branch pulmonary artery stenosis(BPAS).While MPA stenosis is well studied,few data are available on BPAS in rTOF.We aimed to describe pulmonary perfusion in a large paediatric cohort of rTOF and its impact on right ventricular and outflow-tract hemodynamics using 4D flow CMR.Methods:130 consecutive patients(mean age at CMR 14.3±4.6 years)were retrospectively reviewed.96 patients had transannular patch without valve preservation while 34 patients had conserved annulus or valved conduit.A pulmonary blood flow ratio(right pulmonary artery(RPA)/left pulmonary artery(LPA))between 0.75 and 1.56 was considered normal.Results:Asymmetric pulmonary perfusion was present in 59/130 patients(45%),with 54/59(91%)having left lung hypoperfusion(blood flow ratio>1.56).RPA/LPA perfusion ratio in the whole cohort was independently associated with the LPA Z-score(−0.053,p=0.007),the RPA regurgitant fraction(RF)(0.013,p=0.011)and previous LPA stenting(0.648,p=0.004).Decreasing LPA%perfusion(and conversely increasing RPA%perfusion)was significantly associated with higher MPA diameter Z-score(−0.06,p=0.007).On multivariate analysis,MPA Z-score was independently associated with pulmonary RF(0.48,p<0.001)and with right ventricular indexed volumes(coefficient 3.6,p=0.023).In patients with transannular patch repair,asymmetric pulmonary flow was an independent predictor of right ventricular ejection fraction(RVEF)(−3.66,p=0.04).Conclusions:Pulmonary perfusion asymmetry is frequent in rTOF and is associated with abnormal right ventricular and outflow-tract hemodynamics,including MPA dilatation and decreased RVEF in patients after transannular patch.

Premature newborn with intracardiac fungus balls and endocarditis which is curable only with anti-fungal therapy: Case presentation

Invasive fungal infections often caused by Candida species are infections which have high morbidity and mortality rates in newborn infants. 5% of the cases have also concomitant endocarditis and this causes mortality rates over 60%. Thus it has vital importance to diagnose and treat these patients with endocarditis in early stages. Most preferred treatment option is a surgical incision in addition to systemic anti-fungal therapy. Here we presented an extremely low birth weight, premature newborn with endocarditis caused by Candida tropicalis and cured solely by systemic anti-fungal treatment without the need for surgical intervention.

Growth of the Pulmonary Valve Annulus after the Modified Blalock-Taussig Shunt in Patients with Tetralogy of Fallot

Background:The surgical outcomes of tetralogy of Fallot(TOF)have evolved dramatically and have resulted in lower mortality rate.Currently,the many cardiac centers have a trend to early single-stage complete repair more than a staged repair.However,the patients who have an early primary repair were required transannular patch augmentation of a pulmonary valve frequently.This effect has been developed a chronic pulmonary insufficiency may lead to right ventricular dilation,dysfunction.In this era,the aim of treatment of TOF is attempted to preserve pulmonary valve annulus for prevent right ventricular dysfunction in the future.The systemic to pulmonary artery shunt is a palliative procedure or known as staged repair for symptomatic patients with TOF.The modified Blalock-Taussig shunt(mBTS)is the most useful systemic to pulmonary shunt and perform as an initial procedure before complete repair.The mBTS can provide increase pulmonary blood flow as well as improve oxygenation and also promote pulmonary artery(PA)growth.However,the effect of this procedure to promote growth of a pulmonary valve annulus is still debate.Objectives:To compare a growth of pulmonary valve annulus between after staged repair and primary repair in patients with TOF(without pulmonary atresia).Methods:A retrospective case-control study,review of patients with TOF underwent total repair at our hospitals from January 2005 and December 2017 was performed,a total number of 112 patients underwent TOF repair.Twenty-nine patients(26%)underwent a staged repair(mBTS group)and 83(74%)underwent total repair only or primary repair(PR group).We evaluated diameter of pulmonary valve annulus by using echocardiography at the time of first diagnosis and before complete repair on both groups.Results:The age of diagnosis of mBTS group were younger than PR group(p=0.011).Therefore,pulmonary valve annuls were smaller in mBTS group.(Z-score,−2.93±1.42 vs.−1.89±0.97;p=0.001).However,the growth potential of pulmonary valve annulus was increase more than PR group significantly(Z-score,−1.46±1.02 vs.−2.11±1.19;p=0.009)Even though a patent ductus arteriosus was found commonly in PR group(p=0.018).Conclusions:Our results suggest the systemic to pulmonary shunt or mBTS can promote growth of pulmonary valve annulus in patients with TOF.

儿童输注蒽环类药物后心脏功能和形态的急性改变

本研究旨在利用不同的超声心动图方法观察蒽环类药物对左室收缩和舒张功能的急性影响。对13例计划接受蒽环类药物治疗的儿童进行前瞻性观察。在蒽环类药物(剂量为30～75m g/m2)首次给药前及前三次给药后2h内进行全面的二维和多普勒超声心动图检查,其中包括组织多普勒成像。给予首剂后,可观察到舒张期末室壁厚度增加、收缩期室壁增厚降低以及心肌作功指数增加。舒张期功能参数发生显著改变:二尖瓣E波降低、E/A比值降低和等容舒张时间延长。另外,还观察到舒张早期纵向心肌速率和等容收缩期心肌速率加速的降低,以及纵向和径向收缩期应变率和应变峰值的降低。在随后的给药过程中,这些参数仍维续显著降低。

年轻患者中伴和不伴主动脉缩窄的二叶型主动脉瓣的形态和功能

该研究旨在确定年轻人群中伴和不伴主动脉缩窄(CoA)的二叶型主动脉瓣(BAV)的形态和功能。分别回顾分析117例BAV患者和62例CoA患者的经胸超声心动图结果并与正常结果相比较。

马方综合征患者中主动脉根部扩张与年龄的关系

目的：此研究的主要目的是描述马方综合征（MFS）患者发生病理性主动脉根部扩张时的年龄。方法和结果：共对160例在地区心脏中心接受治疗的MFS患者进行了回顾。通过将最大的主动脉窦测量值与文献中的对照资料进行比较来诊断升主动脉扩张。采用Kaplan-Meier生存曲线估计扩张发生时的年龄。所有患者就诊时的平均年龄为15．5岁（极差1．5～40岁）。有95％的患者出现了骨骼畸形。18％的患者累及了眼睛。在160例患者中，有115例发生主动脉根部畸形，其中78例（68％）在19岁之前就发生了主动脉根部扩张。

Significant exercise limitations after recovery from MIS-C related myocarditis

Background Myocarditis is one of the presentations of multisystemic infammatory syndrome in children(MIS-C)following coronavirus disease 2019(COVID-19).Although the reported short-term prognosis is good,data regarding medium-term functional capacity and limitations are scarce.This study aimed to evaluate exercise capacity as well as possible cardiac and respiratory limitations in children recovered from MIS-C related myocarditis.Methods Fourteen patients who recovered from MIS-C related myocarditis underwent spirometry and cardiopulmonary exercise testing(CPET),and their results were compared with an age-,sex-,weight-and activity level-matched healthy control group(n=14).Results All participants completed the CPET with peak oxygen uptake(peak.V O2),and the results were within the normal range(MIS-C 89.3%±8.9%and Control 87.9%±13.7%predicted.V O2).Five post-MIS-C patients(35%)had exerciserelated cardio-respiratory abnormalities,including oxygen desaturation and oxygen-pulse fattening,compared to none in the control group.The MIS-C group also had lower peak exercise saturation(95.6±3.5 vs.97.6±1.1)and lower breathing reserve(17.4%±7.5%vs.27.4%±14.0%of MVV).Conclusions Patients who recovered from MIS-C related myocarditis may present exercise limitations.Functional assessment(e.g.,CPET)should be included in routine examinations before allowing a return to physical activity in post-MIS-C myocarditis.Larger,longer term studies assessing functional capacity and focusing on physiological mechanisms are needed.