Adult congenital heart disease: surgical advances and options

The number of adults with congenital heart disease (CHD) has steadily increased as medical and surgical treatment of congenital heart lesions—whether simple or complex—continues to improve. Over the past half century advances in surgical technique have continued with the evolution of traditional surgical repair and introduction of new surgical procedures for complex lesions previously considered to be irreparable. This article describes the rich history of surgical repair, important surgical considerations specific to the adult undergoing primary or reoperative cardiac repair or palliation, the most common types congenital heart lesions and associated cardiac procedures (including cardiac transplantation) performed in the adult population, as well as considerations regarding the optimal surgical environment and current surgical training and education.     

先天性心脏病介入治疗远期并发症评价

近年来,随着介入器材的不断更新和技术方法的日臻成熟,先天性心脏病(先心病)介入治疗技术取得了重大发展。先心病介入治疗近期疗效好、并发症少。但作为一项新的治疗技术,其疗效还需长期的临床随访结果去评价。先心病介入治疗远期并发症主要包括封堵器脱落、血栓形成、心脏穿孔、心律失常等。术后应严格随访并适当延长随访时间,及时发现并治疗远期并发症。     

非编码RNA在先天性心脏病中的研究进展

先天性心脏病是心血管结构及心电传导系统先天性畸形疾病的总称，是常见并且严重的出生畸形。随着最新分子生物学及遗传生物学的深入研究，越来越多的证据表明 非编码RNA 与先天性心脏病的发生、发展相关。本文将就目前国内、外非编码RNA 与先天性心脏病的关系的研究进展进行综述。     

Stem Cell Therapies in Patients with Single Ventricle Physiology

Single ventricle physiology, especially hypoplastic left heart syndrome, is one of the most high-risk lesions in children with congenital heart disease, and the ensuing heart failure remains as a major problem related to adverse outcomes in these patients. The field of stem cell therapy for heart failure has shown striking advances during the past 10 years, and many clinical trials using stem cell technologies have been conducted in adults, which suggest that stem cell therapy is associated with long-term improvement in cardiac function. Cardiac progenitor cells have recently been discovered, and their strong regenerative ability has been demonstrated in several studies. Although no large clinical trials have been performed in the field of congenital heart disease, recent investigations indicate that stem cell therapy may hold great potential to treat children with cardiac defects.     

Performing a myocardial biopsy in a cardiac transplant patient with a persistent left superior vena cava

The life expectancy of patients with congenital heart disease is increasing. Thus, as the prevalence of adults with congenital heart disease increases, invasive cardiologists will have more exposure to these patients and will benefit from expertise in the anatomy of congenital heart defects. We present a case of performing right heart catheterization and myocardial biopsy in a cardiac transplant patient with a history of multiple congenital defects including a persistent left superior vena cava. © 2008 Wiley‐Liss, Inc.     

Congenital Disorders of Cardiac Rhythm and Conduction

Congenital Arrhythmias and Heart Block. Electrical instability of the heart has numerous congenital origins. This review approaches the problems from a morphological standpoint, utilizing selected examples from my own studies of the human cardiac conduction system performed during the last three decades. Subjects discussed include multifocal Purkinje cell tumors, benign congenital polycystic tumors of the AV node, several types of congenital heart hlock, postnatal morphogenesis of the AV node and His bundle (including considerations of persistent fetal dispersion and crib death), the Wolff-Parkinson-White syndrome, left superior vena cava, focal fibromuscular dysplasia of small coronary arteries, hereditary neuromus-cular or musculoskeletal diseases, familial atrial fibrillation, long QT syndrome, and apoptosis of the heart. In many congenital disorders of cardiac electrical activity, both arrbythmias and conduction disturbances may occur. How the abnormal anatomy may relate to cardiac electrical instahility is discussed, including certain clinical matters to he considered.     

Congenital heart disease and pregnancy

In Europe and North America, a dramatic fall in the incidence of rheumatic fever and rheumatic heart disease has coincided with advances in medical and surgical management of congenital heart disease and has resulted in a shift in the relative incidence of these two categories of cardiac disorders in women of child-bearing age. This review deals with pregnancy and congenital heart disease—unoperated and operated. Central to this topic is the intricate interplay between maternal circulatory and respiratory physiology and maternal congenital heart disease, and the effects of this interplay upon the fetus which is exposed to risks that threaten its intrauterine viability and to risks that subsequently express themselves as developmental defects or transmitted congenital malformations of the heart or circulation.     

Mechanical circulatory support for end-stage heart failure in repaired and palliated congenital heart disease

Approximately one in one hundred children is born with congenital heart disease. Most can be managed with corrective or palliative surgery but a small group will develop severe heart failure, leaving cardiac transplantation as the ultimate treatment option. Unfortunately, due to the inadequate number of available donor organs, only a small number of patients can benefit from this therapy, and mortality remains high for pediatric patients awaiting heart transplantation, especially compared to adults. The purpose of this review is to describe the potential role of mechanical circulatory support in this context and to review current experience. For patients with congenital heart disease, ventricular assist devices are most commonly used as a bridge to cardiac transplantation, an application which has been shown to have several important advantages over medical therapy alone or support with extracorporeal membrane oxygenation, including improved survival to transplant, less exposure to blood products with less immune sensitization, and improved organ function. While these devices may improve wait list mortality, the chronic shortage of donor organs for children is likely to remain a problem into the foreseeable future. Therefore, there is great interest in the development of mechanical ventricular assist devices as potential destination therapy for congenital heart disease patients with end-stage heart failure. This review first discusses the experience with the currently available ventricular assist devices in children with congenital heart disease, and then follows to discuss what devices are under development and may reach the bedside soon.     

Transesophageal echocardiography for congenital heart disease

Surface echocardiographic imaging of small children is routinely successful in defining anatomical details and Doppler flow patterns with even the most complex congenital cardiac malformations. However, in larger children or adults, imaging is frequently limited. A recent expansion of the role of echocardiography is intraoperative epicardial imaging. Epicardial and postoperative imaging, however, have significant limitations. To avoid some of these limitations, transesophageal echocardiography has increasingly been used in the arena of congenital heart disease. The more recent development of small sized gastroscopic probes has allowed transesophageal echocardiographic assessment of congenital heart disease in children down to newborn size. As detailed studies of individual lesions are reported, it has become clear that the mere presence of a congenital heart defect is not an indication for transesophageal echocardiography in most children if imaging can be accomplished by surface examination. However, transesophageal echocardiography may be indicated for the intraoperative or postoperative assessment of that defect, particularly when repair has been difficult or is known to be associated with significant residual abnormalities. Cardiac structures encountered with horizontal and vertical imaging plane transducers have been described and should be completely familiar to the examining echocardiographer.     

Techniques and applications of transcatheter embolization procedures in pediatric cardiology

Transcatheter embolization of congenital or acquired superfluous vascular structure has become routine procedures performed by interventional pediatric cardiologists. Embolization procedure is often part of a collaborative effort with cardiac surgeons to palliate complex congenital heart defect, such as in embolizing aortopulmonary collateral arteries in patient with single ventricle physiology. In other cases, the procedure is the definitive treatment as in embolizing coronary artery fistula. Pediatric cardiologists performing embolization procedures should be familiar with available technologies as well as understand the underlying cardiac anatomy and pathophysiology. This article provides a comprehensive review of presently available embolization agents and technologies. Some of the technologies are used only by interventional radiologists but may be useful to pediatric cardiologists. Specific clinical applications in pediatric cardiology are also discussed with summary of current literature. With continue advancement in transcatheter technology and operator expertise, all unwanted vascular communication should be amenable to transcatheter embolization.     

The Adult Congenital and Pediatric Cardiology Section: increasing the opportunities for the congenital heart disease community within the American College of Cardiology

The Adult Congenital and Pediatric Cardiology (AC/PC) Section was established to develop a clear voice within the American College of Cardiology and address the myriad issues facing the congenital heart disease profession. The Section is governed by the AC/PC Council, which includes pediatric cardiologists, adult congenital cardiologists, a cardiac care associate, and a fellow-in-training member. The Council is responsible for bidirectional communication between the College's Board of Trustees and the AC/PC Section members. Since its founding in 2004, Section objectives have been defined by the College's mission: to advocate for quality cardiovascular care through education, research promotion, and the development and application of standards and guidelines and to influence health care policy. The pillars of the College-advocacy, quality, education, and member engagement-serve as the defining template for the Section's strategy. The Section has developed work groups in advocacy, clinical practice, education and training, quality, and publications. A separate leadership group has been developed for adult congenital heart disease. Work groups are open to all Section members. Recognition of the importance of lifelong care in congenital heart disease led Section leaders to incorporate pediatric cardiology and adult congenital heart disease content into each of the work groups. There are more than 1,200 Section members, with nearly 400 members actively contributing to Section activities. This article outlines Section efforts to date and highlights significant successes to date.     

右前胸小切口治疗先天性心脏病65例

目的总结应用右前胸小切口进行心内直视手术的治疗体会.方法1999年3月-2003年6月,我们采用右前胸小切口行心内直视下修补先天性房、室间隔缺损65例,其中房间隔缺损37例,室间隔缺损28例.结果所有患者无手术死亡及手术并发症.结论经右前胸小切口进行心内直视手术是一种安全可靠的微创手术,对一些简单的先天性心脏病有良好的手术和美容效果,但不适合比较复杂的心脏病手术.     

The wedding cake solution: A percutaneous correction of a form fruste superior sinus venosus atrial septal defect

We present the case of a patient with a superior sinus venosus defect (SSVD), with the atypical finding of an intact atrial septum and posterior atrial wall, which underwent percutaneous closure in the cardiac catheterization laboratory for repair. SSVDs are rare congenital cardiac anomalies, but when the anatomy is amenable to percutaneous strategies, a collaborative and creative approach can result in a safe and effective result. © 2015 Wiley Periodicals, Inc.     

Radiofrequency perforation in the treatment of congenital heart disease.

Catheter-directed perforation of cardiac tissue with radiofrequency (RF) energy has expanded the horizon of the interventional cardiologist dealing with congenital heart disorders. The focus of the following discussion will be to detail the biophysical basis behind RF perforation and review its application in the management of congenital heart lesions.     

Managing the pregnant patient with heart disease

Pregnancy and its associated hemodynamic challenge presents potential risks to the patient with cardiac disease, whether acquired or congenital. The hematologic changes which occur during pregnancy include alterations in blood volume, hematocrit, and blood viscosity. There are also alterations in cardiac output and regional blood flow patterns. These hemodynamic alterations are associated with signs and symptoms which can be impressive yet benign, or can suggest poor outcome, depending on the underlying cardiovascular disorder. The clinician is faced with the sometimes difficult task of differentiating the benign from the dangerous while making appropriate management decisions. In this article, diagnosis and current management of many of the more common acquired and congenital cardiac abnormalities are discussed as they pertain to the gravid female. The effects of common cardiovascular medications in pregnancy are also briefly reviewed.     

Developing Tools to Measure Quality in Congenital Catheterization and Interventions: The Congenital Cardiac Catheterization Project on Outcomes (C3PO)

The broad range of relatively rare procedures performed in pediatric cardiac catheterization laboratories has made the standardization of care and risk assessment in the field statistically quite problematic. However, with the growing number of patients who undergo cardiac catheterization, it has become imperative that the cardiology community overcomes these challenges to study patient outcomes. The Congenital Cardiac Catheterization Project on Outcomes was able to develop benchmarks, tools for measurement, and risk adjustment methods while exploring procedural efficacy. Based on the success of these efforts, the collaborative is pursuing a follow-up project, the Congenital Cardiac Catheterization Project on Outcomes—Quality Improvement, aimed at improving the outcomes for all patients undergoing catheterization for congenital heart disease by reducing radiation exposure.     

New associations of primary ciliary dyskinesia syndrome

Ten of 32 patients with primary ciliary dyskinesia syndrome (PCDS) also had other conditions. Five esophageal problems, 4 congenital heart disease, 2 scoliosis, and 4 miscellaneous and probably coincidental conditions were discovered. Additionally, a patient in this series, who has a normal heart, had a brother who died after surgery for complex congenital heart disease. In retrospect, he too probably had PCDS. The association of severe esophageal and cardiac disease with primary ciliary dyskinesia has not been described before. The diagnosis of PCDS may carry more implications than previously recognized. Pediatr Pulmonol. 1993; 16:9–12. © Wiley-Liss, Inc.     

Initial experience with high frequency ultrasound for the newborn C57BL mouse

The mouse has become a powerful genetic tool for studying genes involved in cardiac development and congenital heart disease. Many of the most severe congenital heart defects are ductal-dependent, resulting in neonatal lethality. Recent advances in ultrasound technology provide an opportunity for the use of high-frequency transducers to characterize the cardiac anatomy and physiology of the newborn mouse. In this study, we define limited normative values for cardiac structure and function in the C57BL newborn mouse. Specifically, we define normal values for 19 indices derived from standard echocardiographic views. This study demonstrates that transthoracic echocardiography using a 40-MHz high-frequency transducer is a safe and reliable noninvasive modality for the delineation of cardiac anatomy and physiology in the newborn mouse.     

The usefulness of echocardiography in the surgical management of infants with congenital heart disease.

Between January 1988 and August 1991, at The Children's Heart Center, Atlanta, 83 infants with congenital heart defects were diagnosed by echocardiography and underwent surgery without cardiac catheterization. The diagnostic categories included 46 infants with left heart obstructive lesions, 19 infants with cyanotic heart lesions, and 18 infants with miscellaneous lesions. Forty-five infants (55%) underwent surgery under cardiopulmonary bypass. There were three errors in diagnosis, yielding a diagnostic accuracy of 95%. Many infants with congenital heart disease can be accurately and completely diagnosed by echocardiography and can safely undergo surgery without cardiac catheterization.