Pulmonary sequestration in a newborn mimicking cardiac disease: a trap for diagnosis

A case of pulmonary sequestration in a neonate presenting with hyperdynamic circulation and a murmur is reported. Clinical, radiographic and echocardiographic data suggested the diagnosis and this was confirmed by angiography. The surgical findings were those of sequestration of the right lower lobe, in conjunction with an accessory right lung complete with bronchial and vascular supply arising from the region of the lower oesophagus. Pulmonary sequestration may mimic primary cardiovascular disease in the neonate.     

MRI技术在胎儿先天性心脏病诊断中的应用

先天性心脏病是造成新生儿死亡的主要原因之一.在产前对胎儿先天性心脏病进行诊断、评估预后是目前围生医学发展的重要内容.胎儿心脏彩超对于孕中期胎儿先天性心脏病的诊断具有相对较高的灵敏度,是胎儿先天性心脏畸形诊断的金标准.胎儿心脏核磁共振技术在过去十年内发展迅速,其视野大、可重复性好等优点在某些情况下可以弥补胎儿心脏超声的不足,但其技术上的问题也局限了其在临床上的应用,目前被认为是胎儿先天性心脏病的二线诊断方法.该文就胎儿心脏核磁共振技术在胎儿先天性心脏病诊断中应用的方法、优势以及局限性进行综述.     

临时人工心脏起搏在先天性心脏病术中及术后的应用

目的 探讨临时人工心脏起搏治疗在先天性心脏病术中及术后应用的临床意义。方法 对1999年3月～2002年12月15例因术中或术后出现各种心律失常(主要为缓慢型心律失常)的先天性心脏病患儿行临时人工心脏起搏治疗。术中及术后床旁紧急开胸右心室心外膜放置临时人工心脏起搏，全部以VVI型起搏方式，并配和药物治疗。结果 15例患儿均于术后1～14d转为稳定窦性心律，出院前全部拆除起搏导线，并康复出院未留置永久性心内膜起搏器。结论 先天性心脏病术中及术后出现各种心律失常，术中与手术操作有关，正确认识房室传导组织的解剖是防止损伤的关键。术后宜尽早放置临时起搏器，帮助患儿恢复自主心律，以期安全渡过围术期。     

Pulmonary Hypertension in Congenital Heart Disease: Irreversible Vascular Changes in Young Infants

Among 280 infants under 1 year of age with congenital heart disease autopsied at the University of Colorado Health Sciences Center between 1959 and 1978, there were six instances of grade IV¹ pulmonary artery hypertension. Five were patients with ventricular septal defect (four associated with other cardiovascular malformations). The sixth was a patient with atrioventricular canal. The youngest was 2¹/₂ months of age. Advanced degrees of pulmonary hypertensive arteriopathy (grade IV or more) have been said to be rare in infants, especially under the age of 1 year. The fact that all of these cases occurred within the last few years of the study suggests the possibility of improved supportive care leading to the prolonged survival of infants who might otherwise have died prior to developing severe disease. In addition, the role of altitude in accelerating the arteriopathy must be considered in the present series. In any case, this unexpected increase in the frequency of severe pulmonary hypertensive arteriopathy should stimulate consideration of early surgical correction of the underlying cardiovascular malformation, especially in areas of relatively high altitude.     

Fetal and post-natal diagnosis of major congenital heart disease: implications for medical and psychological care in the current era

Aim: The fetal or post‐natal diagnosis of major congenital cardiac abnormality has important medical and psychological consequences. Methods: We reviewed infants who underwent cardiac surgery in the first year of life at the Heart Centre for Children, The Children's Hospital at Westmead during 2009. The aims of this study were to: (i) examine the key features of cardiac diagnosis and clinical outcome, and (ii) consider how these data can inform priorities for the delivery of clinical services. Results: Over the 12‐month study period, a first cardiac surgical procedure was performed on 195 infants, with 85 infants (44%) diagnosed in the antenatal period. Of the total sample, a subset of 90 babies (46%) underwent their first procedure in the neonatal period, with 62% having had a fetal diagnosis. Major intracardiac lesions including truncus arteriosus (100%), single ventricular lesions (83%), pulmonary atresia with ventricular septal defect (78%) and transposition of the great arteries (53%) were diagnosed prior to birth. Improved haemodynamic stability at initial presentation was found in those with a fetal diagnosis. The overall mortality rate for all patients was 6.1% at 12 months, with a higher mortality in infants with single ventricle. Conclusions: The contemporary paradigm of care for infants with major congenital heart disease requires a multidisciplinary approach to care, with improvements in clinician–clinician and clinician–family communication, and psychological support and education for families. Changes in the allocation of resources are required to meet this model of best practice.     

Pulmonary vein stenosis mimicking chronic lung disease

The presence of recurrent respiratory symptoms and right heart enlargement in an ex-premature infant is suggestive of chronic lung disease. Pulmonary vein stenosis is a rare, progressive disorder that produces similar symptoms and signs. A case is reported in which pulmonary vein stenosis was revealed by Doppler echocardiography, and this application is recommended in similar cases. Pulmonary vein stenosis is an extremely rare and progressive disorder, which, if left untreated, is usually fatal. The possibility of this diagnosis may not be considered during clinical examination and may be overlooked during routine echocardiography.

Conclusion: This report describes a patient with pulmonary vein stenosis in whom the diagnosis was delayed as she had symptoms and signs more commonly associated with chronic lung disease.     

新生儿危重先天性心脏病的早期干预

目的：探讨新生儿危重先天性心脏病(先心病)的早期识别及早期干预方法。方法回顾性分析2010年1月至2014年12月阜外医院 PICU 收治的223例出生日龄≤30 d 的新生儿危重先心病病例,分析其畸形种类、临床特征及早期干预经验。结果223例先心病患儿以完全性大动脉转位及完全性肺静脉异位引流最为常见,分别占59%(131例)和17%(39例)。首诊症状以紫绀、心脏杂音和呼吸困难最为常见,分别占91%(204例)、56%(125例)和53%(118例)。术前因严重缺氧、代谢性酸中毒及心力衰竭而急诊收住 PICU 抢救59例。早期干预主要包括维持动脉导管开放、纠正内环境紊乱、治疗心力衰竭等,并尽早手术治疗。14例患儿未接受手术,其中4例术前死亡,另10例放弃治疗并均于出院后12 d 内死亡。最终接受手术209例,手术死亡9例,手术死亡率4.3%。随访3~63个月,远期死亡2例,二期解剖矫治手术2例,再次手术3例。其余术后患儿心功能正常,生长发育接近正常同龄儿童。结论必须在新生儿期进行干预的危重先心病主要包括动脉导管依赖型先心病,其中以完全性大动脉转位、完全性肺静脉异位引流最为多见,如不积极手术治疗,大多于新生儿期死亡。早期识别,适当的术前处理,及时转诊和早期手术是挽救这类先心病患儿的关键。     

Dilatation of the Left Ventricle in a Newborn: Probable in Utero Myocardial Infarction

A case study is presented of a premature infant delivered at 32 week's gestation by cesarean section. At birth the infant had massive cardiomegaly with extreme left ventricular dilatation as well as severe congestive heart failure and respiratory distress. The findings at autopsy suggest that the cardiac dysfunction was secondary to a myocardial infarct occurring in utero.     

Non-immune hydrops fetalis and bilateral pulmonary hypoplasia in a newborn infant with extralobar pulmonary sequestration

Extralobar pulmonary sequestration was found in a newborn premature infait that presented with non-immune hydrops fetalis, massive bilateral hydrothorax and polyhydramnios in utero. The baby died of severe respiratory insufficiency 15 h after birth. Postmortem examination revealed distended lymphatic vessels in the sequestered lung tissue probably due to impeded lymph drainage. We suggest that not extralobar pulmonary sequestration itself but a subsequent massive unilateral hydrothorax due to severe obstruction of lymph drainage was the cause of the non-immune hydrops fetalis, pulmonary hypoplasia and polyhydramnios. If these symptoms are diagnosed before delivery, a search for extralobar pulmonary sequestration is indicated.     

Fetal echocardiography in the diagnosis of congenital heart disease

Abstract Fetal echocardiography provides an opportunity to diagnose congenital heart disease as early as the midtrimester of pregnancy, allowing for proper planning of perinatal care and counselling of the parents. This paper reviews the accuracy and outcomes of fetal cardiac ultrasound studies at The Prince Charles Hospital over the past 4 years.
A total of 43 fetuses, of gestational ages 16–39 weeks, were studied. The indications for these were: previous sibling with cardiac anomaly (16 cases); abnormal heart on obstetric ultrasound scan (USS) (13 cases); abnormalities found on obstetric USS with normal appearing heart (one case); fetal bradycardia (seven cases); fetal tachycardia (four cases); irregular fetal heart beat (two cases).
At birth all fetuses assessed because of a previously affected sibling were normal. Of the 13 referred because of structural heart lesions suspected on obstetric USS, eight were abnormal, four were normal and one was terminated without autopsy. Important rhythm disturbances occurred in nine of the 11 referred because of abnormal heart rates. One false positive diagnosis of a possible coarctation was made, and in four cases an abnormality was noted on USS but the diagnosis was not completely correct. Nine of the 19 infants with abnormalities detected have died.
Fetal echocardiography is an accurate and useful method of diagnosing congenital heart disease in utero . Although the mortality of affected fetuses is high, antenatal diagnosis allows planning of medical care and offers the greatest chance of a successful outcome.     

Current techniques and procedures in paediatric and congenital cardiac catheter intervention

There has been marked innovation and progress in the techniques, technologies and outcomes in the field of paediatric and congenital cardiac catheter intervention over the past 4 decades. In the modern era, it is now possible to treat many forms of congenital heart disease through minimally invasive percutaneous therapies, with associated reduction in morbidity for this group of patients. This article provides an overview of current practice and developments in this field, with particular focus on the interventional procedures commonly performed in the paediatric population.     

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??Objective To investigate the effect and safety of single dose of dezocine for anesthesia in children with cyanotic complex congenital heart disease on cardiac catheterization??and compare it with ketamine. Methods Sixty-four children with cyanotic complex congenital heart disease undergoing cardiac catheterization??under 6 years old?? between January 2014 and April 2015 in the First Hospital of Tsinghua University were randomly divided into intravenous dezocine??0.2 mg/kg?? group ??group D??n = 32?? and intravenous ketamine??2.0 mg/kg?? group ??group L??n = 32?? as soon as arriving at OR. All patients were inserted the laryngeal mask??LMA??after the same intravenous induction and the initial end-tidal concentration of sevoflurane was 2.0% to 2.5%. The time to lose consciousness??T1????mask assisted ventilation time??T2????anesthesia recovery time??T3????and such adverse reactions as airway spasm??respiratory depression??dysphoria??pain??nausea and vomiting were recorded. Results T1 of group D was significantly longer than that of group L??P??0.05??. T3 of group D was significantly shorter than that of group L??P??0.05??. The cases whose drop of SPO2 during induction and after extubation was greater than 20% and who needed suction more than twice in group D were fewer than in group L??P??0.05??. Conclusion Single dose dezocine of 0.2 mg/kg can be safely used in children with cyanotic complex congenital heart disease on cardiac catheterization??providing satisfactory sedation and analgesia??and the incidence of airway adverse reaction is lower than that of ketamine.     

Pulmonary hypertension in newborn infants: pathophysiology,clinical assessment and management

Clinically significant pulmonary hypertension (PH) affects up to 1 in 2000 infants after birth and is a major contributor to morbidity and mortality globally in newborn populations. Pulmonary hypertension is a component of many different neonatal diseases and can present a significant treatment challenge in both acute and chronic stages. PH is most commonly due to elevated pulmonary vascular resistance (PVR). This may be caused by structural and functional changes in the pulmonary vasculature, which may be combined with left, right or bi-ventricular dysfunction. Elevated PVR is frequently present from birth due to failure of the normal transition from fetal to extra-uterine environments, giving rise to the classic presentation of persistent pulmonary hypertension of the newborn (PPHN). Regardless of aetiology, the hallmarks of clinically significant PH are hypoxaemic right-to-left shunting, and cardiac dysfunction resulting in systemic cyanosis, hypotension and acidosis. Assessment of these informs targeted treatment aimed at optimising PVR and a supporting cardiac function. In this review we aim to provide an overview of the underlying pathophysiological concepts in PH, and use this to guide practical clinical management in all infants with PH. Based on the latest available evidence we discuss assessment of the severity of PH, and a structured, targeted approach to clinical management, acknowledging areas of uncertainty and controversy.     

Effects of ghrelin on growth and cardiac function in infants with congenital heart disease

Background: Ghrelin has effects on appetite and growth. Recent reports suggest effects on cardiac function, but no study has evaluated the ghrelin levels of congenital heart disease (CHD) infants with heart failure. The purpose of the present study was therefore to investigate the relationship between ghrelin level and growth and cardiac function in CHD infants. Methods: Twenty‐eight infants with CHD were eligible for the study. Blood samples were obtained at the time of insertion of intracardiac catheter and correlation was examined between ghrelin plasma level and anthropometric parameters, including z score of height and weight, body mass index (BMI), and %bodyweight gain rate, severity of heart failure, and the levels of leptin and insulin‐like growth factor‐1. Results: In the CHD group, active ghrelin (A‐Ghr) had a significant negative correlation with z score of bodyweight, and a significant positive correlation with cardiac function. There were no correlations, however, with height and BMI. A‐Ghr levels were significantly higher in the high heart failure index score group. Significant correlation between A‐Ghr and desacyl‐ghrelin in the CHD group was observed. Conclusions: A‐Ghr is involved in cardiac function and has little effect on their physique in infants with CHD.     

先天性食管闭锁合并简单先天性心脏病新生儿预后危险因素分析

目的探讨先天性食管闭锁(CEA)合并简单先天性心脏病(CHD)新生儿预后不良的危险因素。方法回顾性分析1998年—2013年收治并手术的CEA合并简单CHD患儿的临床资料,包括一般情况、实验室检查、术后并发症等,并比较存活和死亡患儿的临床资料。结果 75例患儿纳入最终研究,存活67例,死亡8例,病死率10.67%。死亡及存活患儿的出生胎龄,出生体质量,入院日龄,手术日龄,手术持续时间,手术前、手术当天、痊愈出院/死亡前最近的一次血常规、肝肾功能、电解质、血气分析的差异均无统计学意义(P均0.05);死亡患儿的呼吸衰竭和心力衰竭的发生比例均高于存活患儿,差异有统计学意义(75%、9.0%,P=0.000;50%、1.5%,P=0.000)。结论 CEA合并简单CDH患儿死亡可能与并发呼吸衰竭和心力衰竭有关。     

复杂性先天性心脏病的超声诊断

超声心动图诊断复杂性先天性心脏病应采用顺序节段分析法,按照心房、心室和大动脉等三个节段进行检测,以及分析心房-心室连接和心室-大动脉连接,才能对复杂性先心病作出全面的诊断.     

先天性心脏病肺高压患儿血浆肾上腺髓质素浓度的测定及其临床意义:附33例报告

用特异性放射免疫非平衡法测定左向右分流型先天性心脏病 (CHD)患儿血浆肾上腺髓质素 (AM )水平 ,并观察心脏手术后AM的动态变化。结果显示 :CHD肺高压组AM明显高于无肺高压组及正常对照组 (P<0.05 ,P<0.01)。且AM随着肺高压病情加重而升高 ;中、重度肺高压组AM高于轻度肺高压组 ,差异有显著性 (P<0.01)。肺高压组术后AM明显下降 ,与术前比较差异有显著性 (P<0.05 ,P<0.01)。CHD组患儿AM与肺动脉收缩压 (PASP)呈显著正相关 (r=0.77,P<0.05)。本资料表明 ,AM参与了左向右分流型CHD合并肺高压的病理生理过程 ,AM的升高是机体的一种防御性反应 ,对维持肺循环稳定 ,减缓肺高压的发展有积极作用