Symptomatic heart disease in infants: Comparison of three studies performed during 1969–1987

We compare the data of three studies of hospitalized infants with cardiac disease performed between 1967 and 1987. The studies were from the New England Regional Infant Cardiac Program (1967–1974), the Brompton Hospital (1973–1982), and the Northern Great Plains Regional Cardiac Program (1982–1987). Considering differences in classification among the studies, the distribution of cardiac anomalies during the first year of life is similar among the three studies. Although about 30% of infants are admitted during the first week of life and nearly 40% between 3 and 6 months, the proportion of infants admitted between 6 and 12 months was larger (25%) in the latest than in the earliest study (10%). There were also differences in the distribution of the diagnoses at various ages, reflecting changes in the patterns of care during the three eras.     

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.     

Congenital heart disease in the adult

With improved medical and surgical care, more patients with congenital heart disease are now surviving to adulthood and presenting with previously unobserved problems. This review discusses the course of older operated and unoperated patients as far as physical and psychosocial problems and suggests methods of dealing with these previously unencountered clinical situations.     

Infective endocarditis in childhood

Summary A total of 37 cases of infective endocarditis, in children aged two days to 13 years, were seen from 1971 to 1983 at the Children's Hospital, Camperdown. Structural heart disease was known to preexist in 95%. Cyanotic congenital heart disease was present in 15 children, of whom 11 had been palliated by a systemic-to-pulmonary artery shunt. In five children, there was infection of a prosthetic valve. Identifiable precipitants of infection were rare. In particular, no child had had a preceding dental procedure within three months, while only 19% had had recent cardiac surgery.Organisms were identified by blood culture in 92%, the majority being streptococci (43% of cases) and staphylococci (27% of cases). While 59% of all organisms were penicillin sensitive, six of the seven early postoperative children were infected with organisms not sensitive to antibiotics, given for wound infection prophylaxis in the perioperative period.The overall death rate was 13.5%. Of the children infected withStaphylococcus aureus, 40% died, while no child withStreptococcus viridans endocarditis died. Of the survivors, four needed urgent valve replacement, and three had major central nervous system complications.Using these figures, a current profile of patients with infective endocarditis is presented.G.F.S. is the Douglas Burrows Fellow in Paediatric Cardiology. Supported by funds of the Children's Hospital, Camperdown.     

Evolution of echocardiography in neonatal diagnosis

In the late 196, Edler and Lundström introduced "ultra sound cardio graphy" for the evaluation of congenital heart disease. Initial evaluations using A- and M-mode echocardiography produced non-invasive diagnosis of many defects, including specific complex malformations such as hypoplastic left heart, Ebstein's malformation, endocardial cushion defect and transposition, all with single crystal techniques. Normal values for dimensions related to patient size and indices of function developed at that time remain as components of contemporary examinations. Two-dimensional imaging technology has evolved from 20 channels on Bom's linear array to 128-channel systems currently providing detailed imaging of structures as small as neonatal coronary arteries. The contribution of Doppler techniques for qualitative evaluation of blood flow characteristics has been greatly augmented by both the quantitative Doppler methods for accurate assessment of pressure gradients and pulmonary pressure, and by the development of color Doppler display of intracardiac and intravascular flow. These contributions have come from centers worldwide, with many initial and ongoing contributions from Lund. The evolution of instrumentation, and of application, now provides neonatal echocardio-graphic delineation of anatomic detail, function and hemodynamics of sufficient clarity and accuracy to replace the need for invasive study, or alternative technologies, in most cases.     

Present status of surgery in congenital heart disease

A brief historical review of surgery for congenital heart disease is presented. Palliative procedures designed to improve a given physiologic abnormality, such as systemic artery (or venous) to pulmonary artery shunts of various types to increase the pulmonary blood flow, banding of the pulmonary artery to decrease the pulmonary blood flow and surgical or balloon atrial septostomy to augment intracardiac mixing have been performed with success. Most of the congenital heart defects can be corrected by open heart surgical techniques; those requiring prior pallition and those that can be operated without prior palliative surgery are listed. The mortality rates for several common congenital heart defects are tabulated. Recent surgical advances include early total surgical correction for tetralogy of Fallot, Mustard and Jatene operations for transposition of the great arteries, Fontan operation and its newer modifications for tricuspid atresia, intraventricular septation or modified Fontan for single ventricle, new operations for hypoplastic left heart syndrome and newer prosthetic valves, particularly left ventricular apex to descending aorta conduit. Prostaglandin E₁ for ductal dilation in patients with severe righ ventricular outflow tract obstruction, balloon and knife enlargement of atrial defects to enhance interatrial mixing and catheter closure techniques for nonsurgical repair of atrial septal defect and patent ductsus arteriosus are also reviewed briefly.     

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.     

Adult congenital heart disease: Past,present and future

The diagnosis and management of congenital heart disease (CHD), the most common inborn defect, has been a tremendous success story of modern medicine. In the 1950s, survival of children born with CHD was only approximately 15%, whereas nowadays more than 90% of these children survive well into adulthood. Consequently, the prevalence of patients with CHD has shifted away from infancy and childhood towards adulthood. Adult CHD cardiology is now encompassing not only young or middle‐aged adults but also patients with CHD over 60 years old. Many adult patients are afflicted by residual haemodynamic lesions and also face additional opportunities and/or challenges such as pregnancy, acquired heart disease, non‐cardiac pathology etc., necessitating integrated care and all medical disciplines. We are faced with a “tsunami” in terms of adult CHD numbers, disease heterogeneity and complexity of work and interventions needed. We need to secure resources, welcome more people in our field, learn from “marching with our patients”, and educate better patients, public and ourselves so that every single patient with CHD, born anywhere in the world, may reach their full life potential.     

Flail Posterior Leaflet of the Mitral Valve in Acute Rheumatic Carditis

Pediatric Cardiology -     

先天性心脏病围手术期气管狭窄的综合治疗

目的 通过分析先天性心脏病围手术期气管狭窄患儿的治疗及转归,探讨不同原因导致气管狭窄的治疗方法.方法 回顾性分析2012年1月至2015年10月收治的60例气管狭窄合并先天性心脏病患儿的临床资料.其中,男42例,女18例;年龄1～144个月,平均4个月;体重(6.65±3.40) kg.先天性心脏病合并先天性气管狭窄39例,继发性气管狭窄21例.结果 先天性气管狭窄合并先天性心脏病39例,实施先天性心脏病矫治+气管狭窄旷置28例,先天性心脏病矫治+气管成形术5例,先天性心脏病矫治+气管支架置入1例;术后远期死亡共计4例,放弃手术治疗死亡4例,放弃治疗门诊随诊1例.继发性气管狭窄21例,实施心脏畸形矫治后气管软化狭窄减轻,不需手术治疗.结论 先天性心脏病合并先天性气管狭窄的主要致病原因在于其完全性软骨环导致气管内径增速缓慢,气道梗阻严重,即使实施心脏手术也不能完全改善其气管狭窄症状,必要时需同时实施气道成形术.继发性气管狭窄的主要致病原因在于气管临近大血管的压迫,缓解血管环畸形后气管狭窄即刻缓解,疗效满意,无需实施气管成形术.     

Fontan-type operation for correcting complex congenital defects in a criss-cross heart

Summary Three cases of criss-cross heart with severe and complex congenital defects are reported. Precise preoperative diagnosis of the morphologic abnormalities made it possible to choose an optimal method of surgical treatment: all patients successfully underwent a Fontantype surgical procedure.     

先天性心脏病的镶嵌治疗进展

小儿介入治疗与心脏外科联合即"镶嵌治疗"改善了复杂先天性心脏病的治疗方法.在食道超声引导下,结合内、外科技术治疗复杂先天性心脏病,不仅避免了外科体外循环的不利影响,缩短了手术时间,而且弥补了小婴儿、低体质量儿等内科介入治疗的盲点.目前镶嵌治疗主要集中在房间隔缺损、多发性肌部室间隔缺损、肺动脉狭窄及术后残余分流和残余梗阻的介入治疗.     

Risk of pulmonary tuberculosis in children with congenital heart disease

Children with low-flow congenital heart lesions are reported to have an increased incidence of pulmonary tuberculosis. The aim of this study was to investigate if children with congenital heart disease have an increased incidence of pulmonary tuberculosis and to determine if patients with certain heart conditions are more susceptible to pulmonary tuberculosis than others. This retrospective study over a 6-year period showed that pulmonary tuberculosis was 2.5-fold more common in children with congenital heart disease than in normal children from the same community. Children with congenital pulmonary stenosis had a prevalence equal to those with acyanotic (ventricular and atrial septal defects) and cyanotic (transposition of the great arteries) high-flow heart lesions, whereas there were no cases of tuberculosis in children with low-flow cyanotic heart lesions such as tetralogy of Fallot. Cardiac surgery had to be postponed as a result of pulmonary tuberculosis in 7.2% of all patients in whom it was required. Over the 6-year period of the study, cardiac surgery had to be delayed in 60% of cases with pulmonary tuberculosis and congenital heart lesions so antituberculosis therapy could be completed. Physicians treating children with congenital heart lesions should maintain a high index of suspicion for the development of pulmonary tuberculosis, especially in those with acyanotic and cyanotic high-flow lesions and pulmonary stenosis.     

Safety and Efficacy of Intravenous Contrast Imaging in Pediatric Echocardiography

This study was performed to determine the safety and efficacy of intravenous contrast echocardiography in children attending a tertiary cardiac center. This was a prospective study to evaluate the use of Optison contrast agent in children with severely limited transthoracic echocardiographic windows. Twenty children (median age, 15 years; range, 9–18) underwent fundamental imaging (FI), harmonic imaging (HI), and HI with intravenous contrast (Optison FS-069). Endocardial border delineation was determined based on a visual qualitative scoring system (0, none: 4, excellent). Endocardial border definition was significantly improved in all patients using contrast echocardiography (FI vs Optison, p < 0.001 for each). Improved border definition was most dramatic in the apical and left ventricular (LV) free wall regions. Left ventricular ejection fraction (LVEF) was measurable in 20 patients (100%) using contrast compared to 11 (55%) with FI or HI (p < 0.05). The echocardiographic diagnosis was correctly delineated in 1 patient with a severely dyskinetic LV segment only with use of intravenous contrast and HI. No patients suffered adverse hemodynamic effects, changes in taste, or flushing episodes. Three patients experienced transient headaches. Intravenous contrast echocardiography offers an additional tool in evaluating children with very poor transthoracic echocardiographic windows. Such a strategy increases diagnostic accuracy and allows accurate LVEF determination. Adverse hemodynamic effects related to intravenous contrast are exceedingly rare.     

Idiopathic dilatation of the right atrium revealed in childhood by dysrhythmias

Summary A 7-year-old male presented with apparently banal but repetitive episodes of paroxysmal supraventricular tachycardia, along with other manifestations of atrial hyperexcitability. Isolated dilatation of the right atrium was discovered. This disorder is usually latent or benign, but some-times it is serious because of conduction defects. Lack of relevant studies means the basis of the disorder is not understood.     

Hypoplastic left heart syndrome in context

Hypoplastic left heart syndrome is a rare condition requiring major cardiac surgery during the neonatal period to sustain life, with subsequent procedures culminating in completion of the Fontan circulation - the common pathway for all 'single ventricle' conditions. Algorithms for care of these children are now well defined with predictable medium-term outcomes with the majority achieving a Fontan circulation. Hypoplastic left heart syndrome is one of a group of conditions that require complex surgery as a neonate and require a similar perioperative approach. Antenatal diagnosis is common in this patient subgroup, and there is a significant body of work that can be drawn on to inform parental choice.     

结构性心脏病介入治疗相关瓣膜损伤的防治

结构性心脏病是指以心脏和大血管结构异常为主要表现的一大类心脏病,既包括先天性疾病也包括后天获得性疾病,主要包括先天性心脏病、获得性心脏瓣膜病和心肌病。近年来,结构性心脏病的介入手术治疗已逐渐成熟并广泛应用于临床。由于患者心脏瓣膜及瓣膜相关组织较为脆弱或先天性发育不良,加之手术复杂,介入手术操作可引起相关瓣膜损伤并发症,该文就结构性心脏病介入治疗相关的瓣膜损伤原因及其防治措施进行介绍。     

Opportunities and challenges in linking information across databases in pediatric cardiovascular medicine

Multicenter databases are increasingly utilized in pediatric cardiovascular research. In this review, we discuss the rational for using these types of data sources, provide several examples of how large datasets have been utilized in clinical research, and describe different mechanisms for linking databases to enable studies not possible with individual datasets alone.