Endovascular stents in congenital heart disease

Acquired or de novo vascular obstructions can adversely affect the outcomes of management algorithms for children with congenital heart lesions. Although surgical repair is frequently feasible, some acquired or congenital obstructions are difficult to address in the operating theater. Presented is the recent experience with endovascular stents to relieve such stenoses, and their impact on patient care.     

Evaluation of congenital heart disease in adults

Improvements in the diagnosis and surgical treatment of congenital heart disease during infancy and childhood have resulted in an outstanding increase in the prevalence of these entities during adulthood. Congenital heart disease in the adult represents a new diagnostic challenge to the consultant cardiologist, unfamiliar with the anatomical and functional complexities of cardiac malformations. Assessment of adult congenital heart disease with imaging techniques can be as accurate as in children. However, these techniques cannot substitute for a detailed clinical assessment. Physical examination, electrocardiography and chest x-rays remain the three main pillars of bedside diagnosis. Transthoracic echocardiography is undoubtedly the imaging technique which provides most information, and in many situations no additional studies are needed. Nevertheless, ultrasound imaging properties in adults are not as favorable as in children, and prior surgical procedures further impair image quality. Despite recent advances in ultrasound technologies such as harmonic or contrast imaging, other diagnostic procedures are sometimes required. Fortunately, transesophageal echocardiography and magnetic resonance imaging are easily performed in the adult, and do not require anaesthetic support, in contrast to pediatric patients. These techniques, together with nuclear cardiology and cardiac catheterization, complete the second tier of diagnostic techniques for congenital heart disease. To avoid unnecessary repetition of diagnostic procedures, the attending cardiologist should choose the sequence of diagnostic techniques carefully; although the information this yields is often redundant, it is also frequently complementary. This article aims to compare the diagnostic utility of different imaging techniques in adult patients with congenital heart disease, both with and without prior surgical repair.     

Echocardiography in adults with congenital heart disease

Echocardiography is a valuable tool in the investigation and follow-up of adult patients with congenital heart disease. The majority of these patients have been recognised and treated in childhood, and effective investigations in adult life depend on exact knowledge of the patient history and previous treatments/operations. The major lesions are presented and important echocardiographic features briefly discussed.     

Transplantation in adults with congenital heart disease

Considerable progress in pediatric cardiac surgery has led to more patients with congenital heart disease surviving into adulthood. However, progressive cardiopulmonary dysfunction often occurs late after palliative or corrective surgeries to the point where transplantation becomes the only treatment option. Adult congenital heart disease represents a growing population of patients being referred for heart, lung, and combined heart-lung transplantation. This group of patients presents multiple unique surgical and medical challenges to transplantation owing to their complex anatomy, multiple prior palliative and corrective procedures, frequently increased pulmonary vascular resistance, and often debilitated condition. Consequently, transplantation in adults with congenital heart disease is associated with a relatively high operative mortality secondary to increased bleeding, infection, and graft failure rates compared with noncongenital heart disease transplant recipients. However, those who survive of the first posttransplant year enjoy an excellent long-term prognosis.     

Pulmonary vascular disease in adults with congenital heart disease

Pulmonary arterial hypertension of variable degree is commonly associated with adult congenital heart disease. Depending on size and location of the underlying cardiac defect as well as on repair status, pulmonary arterial hypertension may present with or without reversed shunting and associated cyanosis (ie, Eisenmenger syndrome). We review available data on etiology, clinical presentation, prognosis, and management strategies of pulmonary arterial hypertension in adult patients with congenital heart disease. In addition, we discuss the numerous complications associated with Eisenmenger syndrome, representing a multisystem disorder. Finally, we present general management strategies and emerging disease-targeting therapies.     

Towards defining heart failure in adults with congenital heart disease

Injury to the myocardium disrupts geometric integrity and results in changes to intracardiac pressure, wall stress and tension, and the pattern of blood flow through the heart. Significant disruption to pump function results in heart failure which is defined in terms of symptoms: breathlessness and fatigue, signs of salt and water retention, and neurohormonal activation. This syndrome most commonly occurs in the context of injury due to ischaemic heart disease and dilated cardiomyopathy but because patients with congenital heart disease (CHD) are born with sometimes gross distortions of cardiac anatomy they too are subject to the forces that drive heart failure. This paper explores the available data relating to the clinical and neurohormonal manifestations of heart failure in patients with congenital heart disease and describes how, by additionally exploring events at a cellular level, we may be able to arrive at a definition of heart failure relevant to this population.     

Muscle function in adults with congenital heart disease

Background

The aim was to assess muscle function in a sample of Swedish adult men and women with congenital heart disease (ACHD) and to compare the results with published reference values in healthy adults.

Methods and results

From April 2009 to December 2010, 762 adult outpatients were assessed for their suitability and individual need for tests of physical fitness. The patients performed five muscle function tests, two isotonic tests and three isometric tests. Of the 762 patients, 315 (41.3%) patients performed the tests. Patients with ACHD had lower isotonic muscle function compared to healthy reference values. In the heel lift test, men with ACHD performed at 63% and women at 58% of the healthy reference values and in the shoulder flexion test the corresponding performance level was 60% for men with ACHD and 85% for the women. Multiple regression analyses showed that NYHA class II–IV was a significant predictor for a lower isotonic muscle function i.e. heel lift in women (p < 0.001) and men (p = 0.05) and in shoulder flexion (p < 0.001) in women, as well as in isometric knee extension (p = 0.04) and isometric shoulder abduction (p < 0.001) in women.

Conclusion

This is the first report of muscle function in a broad and unselected group of patients with ACHD. Our data shows that patients with ACHD have lower isotonic muscle function. The impacts of low muscle function in activities of daily living and the question of whether muscle function could be improved with exercise training need further investigation.     

先天性心脏病的规范化介入治疗

自Rashkind和Miller应用球囊导管行房间隔造口术治疗大动脉转位以来,介入性导管术已成为先天性心脏病的重要治疗方法。行经导管介入治疗具有创伤小、效果好、住院时间短、并发症少等优点。20世纪70年代,甚至80年代,我国仅有少数医院可以开展先天性心脏病介入治疗。至90年代,随着封堵材料研究的进展和新型封堵装置设计的日趋合理,先天性心脏病介入治疗得到了飞速发展。2000年,国产封堵器开发成功并投入临床应用,使我国小儿先天性心脏病的介入治疗进入了飞跃发展的阶段。     

Exercise intolerance in adults with congenital heart disease

This article describes the ways to assess exercise capacity in adults with congenital heart disease (ACHD) and the impact of exercise intolerance in the population. It also discusses the likely pathogenesis of exercise intolerance in ACHD, the similarities between ACHD and acquired heart failure, and potential therapeutic options.     

Negative thoughts in adults with congenital heart disease

BACKGROUND: Many patients with congenital heart disease have persistent cardiac defects, psychosocial adjustment problems, and a poor quality of life. This study tested the relationship between negative thoughts and adaptation to congenital heart disease. METHODS: Eighty-two adult out-Patients with congenital heart disease were divided on the basis of few, moderate or many negative thoughts. Group differences were tested in medical and psychosocial adjustment variables (including negative emotions), and quality of life. RESULTS: Patients with many negative thoughts scored worse on psychosocial adjustment and quality of life, irrespective of severity of cardiac deviation, according to the cardiologist, New York Heart Association classification, number of passed and expected surgery, or use of medication. CONCLUSION: Negative affect in general, rather than negative thoughts is decisive in psychosocial adjustment and quality of life. Psychological intervention would be helpful for many patients.     

Plain chest radiography of congenital heart disease in adults

The plain chest radiograph can be an invaluable aid in the diagnosis of congenital heart disease in adults. Evaluation of the chest radiograph is made simpler and more accurate by the use of a systematic approach based on physiology. In many instances, a definitive diagnosis can be made; in others, only a differential diagnosis can be arrived at. The majority of adults with congenital heart disease will have some telltale abnormality on the chest radiograph that will occasionally furnish the first clue to the presence of a congenital anomaly.