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As I have emphasized in previous supplements, Florida is the fourth largest state in the United States of America. The programme for care of children with congenital cardiac malformations at Children's Hospital of Philadelphia is one of the largest, and most prestigious and comprehensive in the world. The Congenital Heart Institute of Florida is the largest programme providing services for patients with congenital cardiac disease in Florida. “Heart Week in Florida”, the joint collaborative project sponsored by the Children's Hospital of Philadelphia together with the Congenital Heart Institute of Florida, has now become recognized as one of the major planks of continuing medical and nursing education for those working in the fields of diagnosis and treatment of heart disease in neonates, infants, children, and young adults. In 2006, however, we broke from our previous mould, since the component of our “week” organized by the group from Philadelphia was organized in Phoenix, Arizona, thanks to the support provided by our colleagues working at Children's Hospital in Phoenix. It was a huge success, diminished only slightly by the inclement weather facing those who needed to journey back from sunny Arizona and Florida to the frozen and snowy northeast coast of the United States. All institutions involved with the organization of the events of 2006, nonetheless, are very grateful to Bob Anderson, and the team at Cardiology in the Young, for their support of “Heart Week in Florida”, and for the opportunity to publish this Supplement.
Perhaps because it guards the inlet to the lesser circulation, the morphologically tricuspid valve has received less attention in terms of its anatomy than the well-explored mitral valve, which will receive attention in a subsequent review in this supplement.1 As we will show in our initial review, nonetheless, the approach to morphological analysis is the same for both valves, irrespective of whether the specific morphology is displayed in the autopsy room or the echocardiographic laboratory. It is essential that the valve be analysed so as to reveal the precise structure of each if its components – the so-called valvar complex.2 Equally important, in the current era, with the burgeoning use of three-dimensional displays that place the heart firmly within the context of the body, it is essential that the components of the valve be described as seen relative to the bodily axis,3 rather than following the present custom of describing the heart as though it is removed from the body and positioned on its own apex.
Ebstein's malformation is a congenital anomaly of the tricuspid valve and right ventricle that is characterized by several features, each of which can exhibit a spectrum of malformation. The first is adherence of the leaflets of the tricuspid valve to the underlying myocardium, this representing failure of delamination during development. This feature involves the septal and inferior leaflets, but rarely the anterior leaflet (Fig. 1). The second feature is anterior and apical rotational displacement of the functional annulus (Fig. 2). The third abnormality is dilation of the “atrialized” portion of the right ventricle, with variable degrees of hypertrophy and thinning of the wall. The fourth finding is redundancy, fenestrations, and tethering of the anterior leaflet. A fifth abnormality is dilation of the right atrioventricular junction, this being the true tricuspid valvar annulus. The final feature is variable ventricular myocardial dysfunction. Each heart with Ebstein's malformation is different, and there is an infinite variability that can occur with the above mentioned characteristics. These anatomical and functional abnormalities cause important tricuspid regurgitation, which results in right atrial and right ventricular dilation, and atrial and ventricular arrhythmias.
The outlook for patients with hypoplastic left heart syndrome has dramatically improved over the past two decades. Universally fatal only 25 years ago, since that time outcomes for staged palliation have shown consistent improvement. Recent reports show that eight to nine patients from every ten can now leave the hospital after the Norwood procedure.1 Attrition following the Norwood procedure, nonetheless, remains significant, with from five to fifteen percent of patients dying between the first and second stages of the Norwood sequence.1–4 Only three-quarters of the patients undergoing surgery for hypoplastic left heart syndrome survive after five years, even at the centres reporting the best outcomes for the Norwood procedure.1,5 In addition to the deaths, some patients are unable to progress through the three stages of reconstruction, and may require cardiac transplantation, or have no options for further therapy. There are many causes for these mortalities and morbidities following the Norwood procedure, including elevated pulmonary vascular resistance, cardiac arrhythmias, coronary arterial insufficiency, right ventricular failure, right ventricular volume overload due to shunt-dependent physiology, and tricuspid valvar regurgitation. Many of these factors are interrelated, and may form feedback loops, which serve to propagate their adverse effects on patients with hypoplastic left heart syndrome.
Unlike the tricuspid valve, the mitral valve has frequently received the attention of anatomists. Indeed, the drawings made by Leonardo da Vinci still retain their currency,1 whilst it was no less a personage than Andreas Vesalius who, as far as we know, first likened the bifoliate appearance of the valve to the Episcopal mitre. It was also Vesalius who recommended that the two leaflets be described as aortic and mural, reflecting their respective relationships to the aortic valve and the parietal atrioventricular junction. It was Roberts and Perloff,2 however, who emphasized the necessity, for clinical purposes, of analyzing not only the valvar leaflets, but also the overall valvar complex. As we will demonstrate in our review, this approach to analysis also proves its worth for the echocardiographic recognition of the congenitally malformed valve.
In contrast to older patients, children and young adults rarely have isolated disease of the systemic atrioventricular valve. Stenosis and/or regurgitation of the systemic atrioventricular valve, however, frequently coexist with complex congenital cardiac disease. In addition, most patients undergoing surgery on the systemic atrioventricular valve have had previous intracardiac repairs.
It is now well recognized that patients fulfilling the diagnostic criterions for the group of hearts usually described as atrioventricular canal malformations, or atrioventricular septal defects, can present with shunting at atrial level, at both atrial and ventricular levels, and on occasion, with shunting only at ventricular level.1,2 It is also well recognized that, in most instances, the patients with shunting exclusively at atrial level have separate atrioventricular valvar orifices for the right and left ventricles, this arrangement often described as the “ostium primum” variant of atrial septal defect.3 Morphological and echocardiographic studies, however, have shown that, in this variant presumed to represent deficient atrial septation, it is the atrioventricular septal structures, rather than the atrial septum, which are deficient, the phenotypic feature being the presence of a common atrioventricular junction.4,5 In this review, we will show how, using modern day echocardiographic techniques, particularly the newly developed potential for three-dimensional display, it is an easy matter to identify the presence or absence of the common atrioventricular junction, and then to demonstrate the various relationships between the valvar leaflets, the septal structures, and the common junction itself which determine the options for clinical presentation within the group.
The repair of atrioventricular septal defect with a common atrioventricular valve is reconstructive surgery at its best, and hence one of the favourite operations performed by paediatric cardiac surgeons. In the past, the post-operative course from such patients was dominated by the occurrence of pulmonary hypertension crises, which were responsible for significant morbidity and mortality. Nowadays, repair is generally undertaken early in infancy, and this approach has mitigated the problems emanating from pulmonary hypertension. Coupled with a better understanding of the anatomy, and adaptation of the surgical techniques, repair can now be achieved safely at around 2 to 4 months of life, without increasing the risk of postoperative regurgitation across the reconstructed left atrioventricular valve. In this review, we discuss the surgical techniques required for, and clinical results of, such early repair.
The association of atrioventricular septal defect with common atrioventricular junction and malformations of the ventricular outflow tracts presents a significant challenge for the surgeon. In the most common of these, the association with tetralogy of Fallot, several surgical techniques have been described, and shown to deliver excellent results.1–10 On the other hand, in the setting of more extreme malformations, such as double-outlet right ventricle, discordant ventriculo-arterial connections, or common arterial trunk, albeit rare lesions, the combination presents a more formidable surgical challenge, as evidenced by the few reports of successful repair of these lesions. This challenge is both physiological, when dealing with a very sick neonate or infant, as well as anatomical in terms of the complexity of the malformation and the ability to achieve a successful biventricular repair. Our goal in this review is to discuss the surgical treatment in the setting of tetralogy of Fallot and double outlet right ventricle, with emphasis on biventricular repair.
The most appropriate way of describing the congenital cardiac malformations unified because the atrial chambers are joined across the atrioventricular junctions to morphologically inappropriate ventricles has long been contentious. In the past, the lesions have been described in such arcane terms as mixed levocardia,1 while “ventricular inversion” still retains it currency in some circles. As we will show in this review, the abnormal arrangements at the atrioventricular junctions can be found with various patterns, but most frequently the patients also have the arterial trunks arising from morphologically inappropriate ventricles. This combination is best described as congenitally corrected transposition, and will form the focus of our review. It is salutary to note that, when von Rokitansky gave the first description of this combination,2 one of his illustrations was ideally suited to aid the understanding of modern-day echocardiographers (Fig. 1). We hope to emulate von Rokitansky in our own review.
Congenitally corrected transposition is a complex cardiac lesion that is often associated with ventricular septal defect, obstruction of the outflow tract of the morphologically left ventricle, and abnormalities of the morphologically tricuspid valve.1,2 Nomenclature for this lesion has been variable and confusing.1 In this review, we define, and hopefully clarify this terminology. The lesion is a combination of discordant union of the atrial chambers with the ventricles, and the ventricles with the arterial trunks.1,2 In rare circumstances, discordant atrioventricular connections can be associated with concordant ventriculo-arterial connections. This malformation has been called “isolated ventricular inversion”. The term is less than precise, and the descriptive approach using the phrase “discordant atrioventricular connections with concordant ventriculo-arterial connections” is preferred, as discussed below.
The congenital cardiac malformation characterized by discordant connections between the atriums and ventricles, as well as those between the ventricles and the arterial trunks, has been given many names. The terms atrioventricular discordance, l-transposition of the great arteries, ventricular inversion, and congenitally corrected transposition have all been used. Regardless of terminology, this complex congenital anomaly has only recently been studied to analyze the long-term effects of its natural history and outcomes following traditional surgical repair of the associated malformations which serve to uncorrect the circulatory pathways. As more patients survive into adulthood, the effects of this condition are now better understood, and the surgical approaches used in the past are being re-examined in light of longer-term follow up.
The classical option for surgical repair in patients with congenitally corrected transposition takes advantage of the physiologic correction provided by nature. At the end of the surgical procedures, however, the morphologically right ventricle remains as the systemic ventricle. Surgical intervention is essentially the correction of associated lesions, including closure of ventricular septal defects, pulmonary valvotomy, placement of a conduit from the morphologically left ventricle to the pulmonary arteries, replacement of the morphologically tricuspid valve, and placement of pacemakers for third degree atrioventricular block. For many years, the classical approach was the “standard” surgical approach.1–4 More recently, newer alternatives have become available, including forms of anatomic repair, the “one-and-a half” ventricular option, and conversion to the Fontan circulation. The goal of anatomic repair is to craft connections such that the morphologically left ventricle becomes the systemic ventricle. Surgical techniques that accomplish this are a Rastelli procedure combined with an atrial baffle,5 and the combination of an arterial switch with an atrial baffle, be it a Mustard or Senning procedure.6
A variety of surgical strategies have been utilized in attempts to accomplish long-term haemodynamic stability in patients with cardiac anomalies characterized by discordant atrioventricular connections, ventricular septal defect, and severe sub-pulmonary obstruction. The majority of these patients have what is commonly referred to as congenitally corrected transposition, together with a ventricular septal defect and pulmonary stenosis or atresia, in the setting of either usual or mirror imaged arrangement of the atrial chambers and the other organs of the body. A smaller sub-group, with discordant atrioventricular connections and double outlet right ventricle, with severe sub-pulmonary obstruction or pulmonary atresia, present similar physiology, and a comparable surgical challenge.
Innovation in surgical and medical management of cardiac disease has generated a dramatic improvement in operative survival. Along with these favourable results in terms of survival is the heightened awareness of neurologic complications, which often become evident beyond the early postoperative period. A large, multicentre prospective study found serious neurologic injury occurs in about one-twentieth of patients after myocardial revascularization in adults.1 More subtle evidence of persistent cognitive decline and functional impairment has been shown to occur in over two-fifths of such patients.2 Acute neurologic abnormalities are reported in up to one-fifth of infants and children who undergo cardiac surgery.3–6 Lasting impairments in cognitive, motor, and expressive functioning have been reported in up to three-fifths of children who have undergone complex cardiac surgery during infancy.7 Specifically, gross and fine motor delays, visual-spatial problems, language deficits and long-term emotional and behavioural problems have been found.8–13
Chronic illness in a child produces stress for both the child with the illness and the family of which he or she is a part.1 Today, it is estimated that greater than one-tenth of children are living with some form of chronic illness or condition.2–3 Faced with this stress, children and families are required to adapt to potential physical, emotional, social, and financial challenges. Professionals providing health care have an opportunity to influence how children and families interpret and adapt to these challenges. Guidance can be drawn from the multiple theoretical perspectives that have explored the process of adaptation to chronic illness.
Surgical intervention for hearts with transposition, defined as concordant atrioventricular and discordant ventriculo-arterial connections, has been one of the landmark achievements in the field of paediatric cardiac surgery. In the early 1950s, pioneer surgeons attempted to palliate patients with transposed arterial trunks with an early form of the arterial switch operation. As a result of initially dismal outcomes secondary to difficulties with coronary arterial transfer, the unprepared nature of the morphologically left ventricle, and primitive methods for cardiopulmonary bypass, the arterial switch was abandoned in favour of several procedures achieving correction at atrial and venous levels, culminating in the Mustard and Senning operations.1,2 These innovative procedures produced the earliest surviving children with transposition. Although the atrial switch procedures achieved widespread acceptance and success during the mid-1960s through the mid-1980s, the search for an operation to return the great arteries to their normal anatomic positions continued. This pursuit was stimulated primarily by the accumulating observations in mid-to-late term follow up studies of: an increasing frequency of important arrhythmic complications, including sinus nodal dysfunction, atrial arrhythmias, and sudden, unexplained death, by the development of late right ventricular dysfunction and significant tricuspid regurgitation in a ventricle potentially unsuited for a lifetime of systemic function by a small but important prevalence of obstruction of the systemic and/or pulmonary venous pathways, and by dissatisfaction with the operative mortality in the subgroup of infants complicated by additional presence of a large ventricular septal defect.3–6 As we have already discussed, a number of novel procedures to achieve anatomic correction had been described as early as 1954, but clinical success was not accomplished until 1975, when Jatene and co-workers7 astounded the world of paediatric cardiology with their initial description.
For patients requiring intervention because of progressive disease of the aortic valve, the perfect palliation will provide a valve that produces normal dynamics of flow, will not require anti-coagulation, will grow with the patient, and have long term durability. Current surgical interventions include aortic valvoplasty, or replacement with either a mechanical or tissue prosthesis. Options for tissue valves include insertion of a pulmonary autograft in the Ross procedure, a cadaveric homograft, or porcine or bovine xenograft valves. The optimal option is still debated.
At this time, the current practice for treatment of patients with isolated ventricular septal defect is infrequently studied. With this in mind, it was our intent to assess the current management of ventricular septal defect at a single center, The Children's Hospital, Denver. We reviewed the practice at this institution to determine if there is an evidence base for when or if a patient with an isolated ventricular septal defect requires surgical repair. With approval from the Colorado Multiple Institutional Review Board (protocol # 06-0097), we reviewed the data on patients with isolated ventricular septal defect seen during the calendar years of 2004 and 2005, determining the state of the patients, and the level of intervention through December 31, 2005.
A long with prematurity and chronic lung disease, the presence of congenital cardiac disease in infants and young children is a significant risk for the clinical consequences of an illness produced by infection with the respiratory syncytial virus.1 In this review, we present a current understanding of such illnesses, their prevention, and their treatment.