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The main intention of this article is to report how the congenital heart diseases CHD are increasing progressively in adults and how a new disease is emerging, configured by the residual injuries, sequelae, and complications of CHDs treated surgically in childhood. Undoubtedly, the impressive progress of cardiac surgery and advances in pediatric cardiology achieved in recent decades have been responsible for this evolution.
The first achievements in this endeavor were made by Clarence Crafoort, who conceived the idea for the surgical correction of aortic coarctation, 4 Alfred Blalock, who performed the first connection of the left subclavian artery to the ipsilateral pulmonary artery in cyanotic patients with pulmonary stenosis or atresia, 5 and Richard Brock, who carried out the first pulmonary valvulotomy using a closed technique.
The main achievements of the s were the repair of ventricular septal defect VSD and tetralogy of Fallot 8 TF ; of the s, atrial physiological correction of transposition of the great vessels 9 TGV and the interposition of conduits between the right ventricle and pulmonary artery; 10 and in the s, the techniques of Fontan 11 and Jatene 12 for the reconstruction of single-ventricle heart and TGV, respectively. The progressive improvement in cardiopulmonary bypass techniques, and the use of methods for cerebral 13 and myocardial protection, 14 have made the surgical repair of most CHDs possible at a progressively earlier age, obtaining excellent short and long-term results.
The first successful heart transplantation in newborns was carried out at the Loma Linda university medical center in The initial contribution of pediatric cardiology was the morphological analysis of all the cardiac malformations.
A select group of cardiologists and anatomists -- notably Van Praagh 16 in the U. The second advance was the use of cardiac catheterization and angiocardiography techniques in the pathophysiological and anatomic assessment of CHD. The first intracardiac therapeutic procedure carried out with a catheter was atrial balloon septostomy, which was made by Rashkind in , 24 and eventually led to the development of the present therapeutic arsenal available to pediatric cardiologists 25,26 Table 2. The latest contribution of pediatric cardiology is prenatal diagnosis with fetal echocardiography 27 that, without doubt, is an extraordinary advance in the knowledge of morphological and functional disturbances of the heart from an early gestational age..
The major development experienced by pediatric cardiology and cardiovascular surgery in recent decades has made possible an impressive recovery program for children who once were condemned to die. The demographic impact of this advance has been reviewed in recent publications. In , Moller and Anderson 28 reviewed the present state of consecutive unselected children with CHD who were initially evaluated between and In , the findings of a prospective study on the long-term evolution of pulmonary stenosis, aortic stenosis, and VSD were published.
The patients who underwent surgery for uncomplicated atrial septal defect ASD in the first two decades of the life had a long-term survival similar to that of the general population.
In order to attend this increasing demand, in recent years clinical units specialized in adult CHD ACHDU have been created in major health centers in the world. This center harbors a very active nucleus of pediatric cardiology and cardiac surgery, which has been functioning for more than 35 years. According to mutual agreement between pediatric and adult cardiologists, in it was decided to unify the clinical follow-up of these patients in an ACHDU.
Between and , adults with CHD were cared for in this unit.
The analysis of this population is the main objective of this article.. The population of adult patients with CHD currently includes two large groups: a CHD with natural survival 39 and b CHD with some type of therapeutic intervention 40 surgery or a percutaneous procedure carried out in childhood.
Cases of CHD with a natural survival until adulthood can be classified in three subgroups: CHDs that require a primary therapeutic intervention in adulthood.. CHDs that do not require therapeutic intervention but do need monitoring and continuous clinical follow-up.. CHDs that are currently inoperable, can only be treated by transplantation, or in patients in which the surgical risk is greater than the risk of the natural evolution Table Without doubt, ASD continues to be the most frequent disorder in this group, followed by valvular or subvalvular aortic stenosis, and uncomplicated aortic coarctation.
Other CHDs, like pulmonary stenosis, ductus arteriosus, and VSD, are now treated surgically in adults with much less frequency. In fact, except for aortic stenosis, which can continue to evolve throughout life, most patients in this group must undergo surgery before reaching adulthood, which is why the number of primary interventions of CHD in older patients is decreasing.. Main diagnostic groups of congenital heart diseases with natural survival until adulthood.
Adult congenital heart disease - Cleveland Clinic Heart & Vascular Institute, leader in the treatment of congenital heart disease in adults. Overview. Congenital heart disease (congenital heart defect) is one or more abnormalities in your heart's structure that you're born with.
A Heart diseases that require primary intervention during adulthood. B Acyanotic heart diseases that do not require intervention. C Cyanotic heart diseases not intervened.. Figure 2b shows acyanotic CHDs that have not required any intervention in our series. This group is numerous and important, and is prominently represented by patients with bicuspid aortic valve, restrictive VSD, and mild pulmonary stenosis.
They are asymptomatic patients with mild heart diseases that do not require treatment but are exposed to serious complications, such as infectious endocarditis or progressive valve degeneration stenosis or insufficiency.. In Figure 2c are shown the cyanotic CHDs in our series that have a natural survival until adulthood. In this group, Eisenmenger syndrome, single-ventricle heart, Ebstein anomaly, and pulmonary atresia with VSD are noteworthy.
These patients are symptomatic and have serious heart diseases, but cannot be undergo surgery with a reasonable risk. In some cases, cardiopulmonary transplantation, or isolated pulmonary transplantation with correction of the cardiac anomaly are the only possible options, but the risks of these therapeutic alternatives are almost always higher than those of the natural evolution.
However, these patients generally remain relatively stable, with intolerance to effort, frequent symptoms of hyperviscosity, but few signs of cardiac insufficiency. In our experience, most complications occur during pregnancy, intercurrent disease, surgical interventions, impaired hydration, excessive exercise, or medical interventions.
These patients need strict cardiological control to prevent high-risk situations and ensure that immediate treatment will be provided if complications arise: embolization of bleeding vessels, surgical drainage of cerebral abscesses, arrhythmia ablation, and others.. Currently, the most numerous group of patients with CHD who reach adulthood is formed by patients who have undergone some type of therapeutic intervention during childhood.
This fact is greatly changing the spectrum of CHDs that cardiologists see in adults, which underlines that cardiologists should be prepared to recognize this new disease. In Figure 3 are shown the main CHDs treated surgically in childhood in our series. This disease had disappeared almost completely from general cardiology clinics and now has reappeared with a much greater prevalence, but in the form of a postoperative disease. We are also observing the appearance in our clinics of diseases that are practically unknown to us, like TGV.
Complete transposition is an unviable CHD outside the first months of life and must be repaired by surgery. Nevertheless, most children with this malformation now reach adult life, although in a state modified by different surgical techniques. Patients with an anatomically or functionally unique heart constitute another large group of patients that are seen in our clinic.
Most of them arrive after undergoing surgical palliation or partial correction, which enables survival but does not modify the basic structural disturbance and adds structural changes introduced by surgery.. Main diagnostic groups of adult patients with congenital heart disease who underwent surgery in childhood. A, B, and C refer to the groups in Figure In spite of the previous intervention, the patients in this group rarely reach adulthood with a fully normal anatomy, physiology, and cardiovascular function. Few therapeutic interventions are totally curative.
The ligation of a ductus arteriosus, closure of an ostium secundum type ASD at an early age, or closure of a VSD by transatrial approach can be totally curative.
Teen Transition Program. Obstruction of the outflow tract. A fluid relation with the areas of clinical genetics genetic counseling , hematology blood dyscrasias , neurology neurological syndromes , traumatology skeletal malformations , and other specialized areas is important. Selection criteria for Fontan procedure. Three-dimensional reconstruction of the vascular magnetic resonance imaging of the pulmonary artery in the anteroposterior left and lateral right views of a patient with tetralogy of Fallot treated with a transannular patch to the pulmonary bifurcation. Dehiscence and baffle obstruction are usually located distal to the inflow tract of the superior vena cava. Publications Access a selection of valuable scientific articles and position papers.
In most cases, surgical or interventional procedures are only reparative and in other cases are just palliative in nature. This means that almost all patients with CHD who were operated during childhood present residual injuries, sequelae, or complications that can have a progressive character during adulthood.. Table 4 offers a classification of residual lesions, sequelae, and complications of CHD in adults, modified from the Perlof and Warnes classification. In general, these problems can be caused by intrinsic malformations, the effects of therapeutic procedures, secondary hemodynamic changes, or unexpected complications.
Residual lesions are anomalies that are deliberately left uncorrected during surgical repair. Examples of residual lesions are a restrictive VSD associated with a repaired aortic coarctation, or mitral insufficiency due to a cleft anterior leaflet that was not repaired during the surgical closure of an ostium primum type ASD. Neurological or systemic abnormalities that remain after the intervention are also residual lesions.
Sequelae are new cardiovascular disturbances that occur as a necessary consequence of repair. Pulmonary failure secondary to the surgical correction of TF with a transannular patch, or persistent electrophysiological anomalies after right ventriculotomy are common examples. In contrast, complications are undesired cardiovascular or systemic disturbances that may be related to therapeutic procedures or appear spontaneously in the course of the natural evolution. Postoperative AV block, myocardial dysfunction due to insufficient protection of the cardiac muscle during ischemia, or the consequences of infectious endocarditis are complications that can have a major impact on survival and morbidity in adulthood.
This review does not allow an exhaustive analysis of the residual lesions, sequelae, and complications of CHDs operated in childhood. TF is the most frequent cyanotic CHD in children. For this reason, the surgeon often must use a transannular patch that can reach the pulmonary bifurcation and, sometimes, a prosthetic conduit between the right ventricle and pulmonary artery. It is not uncommon that the pulmonary branches have to be enlarged if there is congenital stenosis or stenosis induced by previous surgical fistulas..
Obstruction of the outflow tract. When they reach adulthood, most patients maintain an effective correction. This raises serious diagnostic problems in adults because the pulmonary branches are poorly evaluated by conventional or transesophageal Doppler echocardiography. Nevertheless, vascular magnetic resonance imaging is a high-resolution non-invasive technique for the anatomic assessment of the main pulmonary branches Figure 4. The surgical treatment of stenosis of the pulmonary branches also has serious difficulties, but percutaneous procedures using stents are very effective 46,47 Figure 5.
In patients who have undergone correction with a conduit or valve prosthesis, restenosis is the rule and many patients need to be reoperated several time in the course of their lives. Dilation with stent placement 48 and later percutaneous implantation of a valve prosthesis is a new procedure that shows great promise in these patients. Three-dimensional reconstruction of the vascular magnetic resonance imaging of the pulmonary artery in the anteroposterior left and lateral right views of a patient with tetralogy of Fallot treated with a transannular patch to the pulmonary bifurcation.
It can be seen that the left pulmonary branch is straddling and there are stenoses at the distal insertion of the patch and right pulmonary branch.. Angiography of the left pulmonary artery in a patient with operated tetralogy of Fallot and a pulmonary prosthesis.
The stenosis of the left pulmonary branch before left and after right insertion of an intravascular stent.. Residual shunts are not infrequent. Thirty percent of the patients in our series have a persistent shunt, in most cases small and of scant clinical importance.
Some of our patients have a permeable foramen ovale or an ASD not closed in the correction. This has been cause of paradoxical embolism in some cases, which could have been avoided if more attention had been given to the interatrial septum during surgery. Most residual interatrial or aortopulmonary shunts can be treated with percutaneous procedures..
Valvular and myocardial dysfunction.