OBJECTIVEThis review aims to provide an overview of conventional and novel indices used in clinical and research arenas for evaluation of right ventricular (RV) function in congenital heart diseases with a dual-chambered circulation.

DATA SOURCESArticles cited in this review were selected using PubMed search of publications in English with no date limits. The search terms included "echocardiography", "right ventricle", "RV function", "cardiac function", and "congenital heart disease". Key references were also searched for additional publications.

STUDY SELECTIONArticles related to description of echocardiographic techniques in the evaluation of subpulmonary or systemic RV function and their applications in congenital cardiac malformations were retrieved and reviewed.

RESULTSThree approaches have been used to evaluate subpulmonary and systemic RV function: (1) assessment of changes in RV size in the cardiac cycle, (2) determination of Doppler-derived velocities and systolic and diastolic time intervals, and (3) quantification of myocardial velocities and deformation.

CONCLUSIONSConventional and novel echocardiographic techniques enable the evaluation of subpulmonary and systemic RV function. Novel echocardiographic techniques have further allowed quantification of RV volumes and direct interrogation of myocardial deformation. These new techniques show promise in a more comprehensive evaluation beyond "eye-balling" of RV function in the growing population of adolescent and adult congenital heart patients.

Echocardiography ; methods ; Heart Defects, Congenital ; diagnosis ; physiopathology ; Humans ; Ventricular Function, Right ; physiology

Kawasaki disease (KD), an acute vasculitis that primarily affects young children, is the most common acquired paediatric cardiovascular disease in developed countries. While sequelae of arterial inflammation in the acute phase of KD are well documented, its late effects on vascular health are increasingly unveiled. Late vascular dysfunction is characterized by structural alterations and functional impairment in term of arterial stiffening and endothelial dysfunction and shown to involve both coronary and systemic arteries. Further evidence suggests that continuous low grade inflammation and ongoing active remodeling of coronary arterial lesions occur late after acute illness and may play a role in structural and functional alterations of the arteries. Potential importance of genetic modulation on vascular health late after KD is implicated by associations between mannose binding lectin and inflammatory gene polymorphisms with severity of peripheral arterial stiffening and carotid intima-media thickening. The changes in cholesterol and lipoproteins levels late after KD further appear similar to those proposed to be atherogenic. While data on adverse vascular health are less controversial in patients with persistent or regressed coronary arterial aneurysms, data appear conflicting in individuals with no coronary arterial involvements or only transient coronary ectasia. Notwithstanding, concerns have been raised with regard to predisposition of KD in childhood to accelerated atherosclerosis in adulthood. Until further evidence-based data are available, however, it remains important to assess and monitor cardiovascular risk factors and to promote cardiovascular health in children with a history of KD in the long term.
Aneurysm ; Arteries ; Arteritis ; Atherosclerosis* ; Blood Vessels ; Cardiovascular Diseases ; Child ; Cholesterol ; Developed Countries ; Dilatation, Pathologic ; Humans ; Inflammation ; Lipoproteins ; Mannose-Binding Lectin ; Mucocutaneous Lymph Node Syndrome* ; Risk Factors ; Vasculitis

Arterial stiffness describes the rigidity of the arterial wall. Its significance owes to its relationship with the pulsatile afterload presented to the left ventricle and its implications on ventricular-arterial coupling. In adults, the contention that arterial stiffness as a marker and risk factor for cardiovascular morbidity and mortality is gaining support. Noninvasive methods have increasingly been adopted in both the research and clinical arena to determine local, segmental, and systemic arterial stiffness in the young. With adoption of these noninvasive techniques for use in children and adolescents, the phenomenon and significance of arterial stiffening in the young is beginning to be unveiled. The list of childhood factors and conditions found to be associated with arterial stiffening has expanded rapidly over the last decade; these include traditional cardiovascular risk factors, prenatal growth restriction, vasculitides, vasculopathies associated with various syndromes, congenital heart disease, and several systemic diseases. The findings of arterial stiffening have functional implications on energetic efficiency, structure, and function of the left ventricle. Early identification of arterial dysfunction in childhood may provide a window for early intervention, although longitudinal studies are required to determine whether improvement of arterial function in normal and at-risk paediatric populations will be translated into clinical benefits.
Adolescent ; Adoption ; Adult ; Arteriosclerosis ; Child ; Early Intervention (Education) ; Heart Diseases ; Heart Ventricles ; Humans ; Risk Factors ; Vascular Stiffness ; Vasculitis

Significant improvement in survival of children with congenital cardiac malformations has resulted in an increasing population of adolescent and adult patients with congenital heart disease. Of the long-term cardiac problems, ventricular dysfunction remains an important issue of concern. Despite corrective or palliative repair of congenital heart lesions, the right ventricle, which may be the subpulmonary or systemic ventricular chamber, and the functional single ventricle are particularly vulnerable to functional impairment. Regular assessment of cardiac function constitutes an important aspect in the long-term follow up of patients with congenital heart disease. Echocardiography remains the most useful imaging modality for longitudinal monitoring of cardiac function. Conventional echocardiographic assessment has focused primarily on quantification of changes in ventricular size and blood flow velocities during the cardiac cycles. Advances in echocardiographic technologies including tissue Doppler imaging and speckle tracking echocardiography have enabled direct interrogation of myocardial deformation. In this review, the issues of ventricular dysfunction in congenital heart disease, conventional echocardiographic and novel myocardial deformation imaging techniques, and clinical applications of these techniques in the functional assessment of congenital heart disease are discussed.
Adolescent ; Adult ; Blood Flow Velocity ; Child ; Echocardiography ; Echocardiography, Three-Dimensional ; Heart ; Heart Defects, Congenital* ; Heart Ventricles ; Humans ; Ventricular Dysfunction ; Ventricular Function

OBJECTIVEThe investigators compared the outcome of infants and children having right atrial isomerism with normal pulmonary venous drainage to those with anomalous drainage and determined factors associated with poor outcome. They further determined the prevalence of symptomatic cardiac arrhythmia in these patients and its relation to long-term morbidity and mortality.

METHODSThe authors made a retrospective review of management and outcome of 116 infants and children diagnosed to have right atrial isomerism between January 1980 and December 2000. The type, timing and precipitating factors of symptomatic cardiac arrhythmia that occurred in patients, among a cohort of 85 who had or are awaiting surgical interventions, were noted.

RESULTSThe 116 patients presented at a median of 1 day (range 1 day to 3.7 years) with cyanosis in the majority (96%). No interventions were planned in 31 (27%) patients who all died. The early surgical mortality for pulmonary venous repair was 25% (2/8), Fontan procedure 26% (5/19), cavopulmonary shunting 8% (1/13) and systemic-pulmonary arterial shunt insertion 2% (1/53). Late mortality was related to infection (n = 10), sudden death of unknown aetiology (n = 7) and documented arrhythmia (n = 1). Patients with obstructed anomalous pulmonary venous drainage had poor survival (P < 0.001). The mean (SEM) survival estimates for those with normal pulmonary venous drainage at 1, 5, 10 and 15 years were 81 (5)%, 67 (7)%, 60 (8)% and 43 (12)%, respectively, similar to those of patients with non-obstructed anomalous drainage (P = 0.06). Independent risk factors for mortality included pulmonary venous obstruction (relative risk RR 3.8, P = 0.001) and a single ventricle (RR 2.9, P = 0.016). Symptomatic cardiac arrhythmia occurred in 15/85 (18%) patients; 11 of whom had supraventricular tachycardia, and 1 atrial tachycardia, 1 atrial flutter, 1 ventricular tachycardia and 1 congenital complete heart block. The arrhythmias occurred before surgery in 4, early after surgery in 5, and late after surgery in 6 patients. Freedom from arrhythmia at 1, 5, 10, 15 and 20 years was (93 +/- 3)%, (86 +/- 4)%, (80 +/- 6)%, (73 +/- 9)% and (48 +/- 15)%, respectively. Logistic regression failed to identify any risk factors for symptomatic arrhythmia.

CONCLUSIONThe long-term outcome of infants and children with right atrial isomerism, whether associated with normal or anomalous pulmonary venous drainage, remains unfavourable. Sepsis and sudden death are major causes of late mortality. While symptomatic cardiac arrhythmias are not uncommon. They do not seem to relate to the overall high mortality and occurrence of sudden death in this patient group. Nonetheless, detailed assessment and aggressive management of cardiac arrhythmias once they occur are warranted in light of the precarious single ventricular haemodynamics.

Arrhythmias, Cardiac ; etiology ; mortality ; Child, Preschool ; Heart Atria ; abnormalities ; Heart Defects, Congenital ; complications ; surgery ; Humans ; Infant ; Infant, Newborn ; Prognosis ; Survival Analysis ; Survival Rate