Perloff JK, deLeon AC, and Ronan JA: Ventricular septal defect with the Perloff JK: Pediatric congenital cardiac becomes a post-operative adult: the changing. congenital heart disease, so that many survive into adult life. This success story has resulted in a growing population of adults and young adults with congential. Request PDF on ResearchGate | Perloff's Clinical Recognition of Congenital Heart Disease | The first edition of Joseph Perloff's now-classic textbook on.
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download Clinical Recognition of Congenital Heart Disease 4 by Joseph K. Perloff MD Congenital Heart Disease ebook PDF or Read Online books in PDF, EPUB. Dr. Joseph Perloff is joined by Dr. Ariane Marelli to bring you a new edition of Clinical Recognition of Congenital Heart Disease. This medical. Congenital heart disease in adults is a recognized cardio- Correspondence to Joseph K. Perloff, MD, UCLA Division of Cardiology, Room.
Even with treatment, the lifespan of individuals with congenital heart disease is limited compared with their peers; Survival in individuals with congenital heart disease who reach adulthood is reduced Verheugt et al.
Death is commonly due to heart failure or sudden death, and risk is increased by arrhythmia, endocarditis, myocardial infarction, and pulmonary hypertension. In , 1 million adults in the United States were living with congenital heart disease. An estimated half of them had relatively simple residual disease, one quarter had moderately complex residual disease, and one quarter had severe residual disease Warnes et al.
The ICD-9 diagnosis codes indicating congenital heart disease fall primarily between Distinguishing among the various congenital heart disease defects can be difficult, and accurate diagnosis can require special expertise and training.
Children with congenital heart disease are best evaluated, when possible, in pediatric cardiology centers and by echocardiographic centers certified in congenital heart disease. Adults with congenital heart disease are best evaluated by specialized centers devoted to the evaluation and treatment of such adults. These centers are recommended for their ongoing care Warnes et al.
Individuals with congenital heart disease also have a higher prevalence of neuropsychiatric disorders, including learning disabilities, cognitive impairment, depression, and anxiety. The latest contribution of pediatric cardiology is prenatal diagnosis with fetal echocardiography27 that, without doubt, is an extraordinary advance in the knowledge of morphological and functional disturbances of the heart from an early gestational age.
The demographic impact of this advance has been reviewed in recent publications. In , Moller and Anderson28 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. Cases of CHD with a natural survival until adulthood can be classified in three subgroups 1. 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 3.
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.
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.
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 2.
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.
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.
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.
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. Cycle lengths, duration of menstrual cycle, regularity of menses, amount of flow, and breakthrough bleeding were compared in these two groups and against agematched controls.
The mean age of menarche for the sample With the exception of breakthrough bleeding, statistical differences were not found between the study population and the controls. When the menstrual histories of cyanotic women were compared with those of acyanotic women, differences were found for all categories of menstrual patterns with the exception of the amount of menstrual flow.
Menstrual patterns of cyanotic women compared to controls were found to be significantly different for all categories with the exception of amount of flow. In general, women with CHD have menstrual patterns similar to those in the general population.