Surgical treatment of congenital heart defects in adults

Of 73 patients with congenital heart diseases (CHD), aged from 16 to 82 years, operated on in 1977-1998 yrs., there were 41 women and 32 men. Fourteen (19.6%) of patients were 60 years old or older. Complex CHD were revealed in 27 (37%) of patients, an ischemic heart disease-in 8 (11%). There were no intraoperative mortality. Complications have occurred in 2 patients. Late follow-up results (in terms from 4 months till 20.5 years) were studied in 71 patient. One patient died in 7 years because of another cause, the state of the rest of the patients is good or satisfactory.     

Catheter treatment of congenital heart defects. Arterial septal defects and open ductus arteriosus

The results of new methods for catheter treatment of congenital heart defects are presented. Between 1989 and 1996 closure of a patent ductus arteriosus was performed in 66 instances on 63 patients, eight of which were with coils. Three patients were treated twice, one with an additional umbrella, two with coils. The overall complete closure rate for umbrellas was 75%, after two ducts, which were initially totally occluded, recanalized. In six more patients the procedure was either aborted or indication was not present. All six ducts treated with coils as the first procedure were completely closed. One of two patients who had residual leak after previous umbrella treatment achieved complete closure after subsequent coil implantation. Closure of atrial septal defects in the oval fossa was performed using the Amplatzer septal occluder in seven children. Complete closure was achieved in all of them. There have been no complications, in particular there have been no cases of embolization in any of the groups. The results seem to indicate that coil occlusion of a persistently patent duct may be at least as good as the umbrella in terms of complete closure. So far both methods have been safe, but experience with coils is limited. The closure of atrial septal defects shows encouraging results. We will continue to offer this treatment as an alternative to open heart surgery in carefully selected patients.     

Infectious endocarditis and congenital heart defects in adults

BACKGROUND: Infective endocarditis still remains a cardiological menace. However, the type of predisposing diseases has changed: the incidence of rheumatic heart disease in advanced countries has declined, advances made in the surgical and medicamentous treatment of inborn heart disease are the reasons why we are encountering, with increasing frequency, infective endocarditis which develops on their background. METHODS AND RESULTS: The objective of the investigation was to assess the frequency of infective endocarditis and predisposing diseases. During the time interval between 1987 and 1991 16 patients (50% younger than 50 years) were hospitalized with the diagnosis of infective endocarditis. Rheumatic valvular damage and inborn heart disease were the predisposing factor in 25%. All patients were younger than 30 years (mean 24, range 18-30), and half the patients suffered from defects of the ventricular septum. Other defects were tetralogy of Fallot and inborn aortic stenosis. In 10% of the patients infective endocarditis developed on the background of a mitral valve prolapse with regurgitation. Echocardiographic examination confirmed the diagnosis in almost 70% by revealing vegetation. Bacteriological examination revealed the agent in 60%, most frequently it was Streptococcus viridans. The mortality rate in the group was 13%. CONCLUSIONS: The recorded incidence of infective endocarditis, 1.5 pro mille, is consistent with data in the literature. Corrected and not corrected heart disease plays an important role as predisposing disease. Despite the opportunity of intensive antibiotic treatment, the mortality remains high--13%.     

Quality of life in children with congenital heart defects

PURPOSE: The antiepileptic effects of zonisamide (ZNS) have been well documented experimentally and clinically. The purpose of this study was to examine whether ZNS reduces cerebral damage after transient focal ischemia in rats. METHODS: Ischemia was induced by a transient occlusion of the left middle cerebral artery (MCA) with a 3-0 nylon monofilament for 90 min. Neurological evaluation was performed by measuring the event of neurological deficit of the contralateral forepaw and hindpaw at 10 min and 1 day after MCA occlusion (MCAo). Brain infarct size was determined by measuring triphenyltetrazonium chloride-negative stained area of the serial brain sections 1 day after MCAo. RESULTS: The pre- or postischemic treatment with ZNS [(10-100 mg/kg p.o.), 30 min before and 4 h after or 15 min and 4 h after the occlusion] markedly reduced cerebral damage in the ipsilateral hemisphere and the neurological deficit induced by transient ischemia. The reducing effect on the damage was observed in the cortical and subcortical regions. Preischemic treatment with carbamazepine (CBZ 60 mg/kg p.o. twice 30 min before and 4 h after MCAo) tended to reduce the cerebral damage and neurological deficit, but the lower dose (20 mg/kg p.o. twice) did not. Valproate (VPA 1,000 mg/kg p.o. twice) also had no effect. CONCLUSIONS: ZNS at the anticonvulsant dose, unlike CBZ and VPA, ameliorated the brain infarction and the event of neurological deficit after transient focal cerebral ischemia. These data suggest that ZNS has therapeutic potential in protecting against ischemic cerebral damage, such as stroke.     

Role of vitamin A in the formation of congenital heart defects

Retinoic acid, the biologically active form of vitamin A, is a critical player in normal development. The concentration of retinoic acid is highly regulated by the embryo to prevent either a deficit or an excess of this molecule, conditions that have been shown to produce cardiac defects that vary depending on the severity and the timing of the insult. The vast majority of these defects are associated with the valves or the membranous septa of the heart, suggesting a problem with the formation of the cardiac mesenchyme from both within and outside the heart. While the exact role of retinoic acid in cardiac development is not known, it is believed that retinoic acid influences development by up- or down-regulating cardiac specific genes. This review briefly discusses the role of cardiac mesenchyme and cardiac neural crest in septation of the heart. This is followed by a discussion of vitamin A metabolism and the cardiac defects associated with abnormal levels of retinoic acid. Finally, a mechanism is proposed concerning the ways abnormal levels of retinoic acid lead to similar cardiac defects by disrupting the production of the extracellular matrix.     

Plasma endothelin 1 concentrations in children with congenital heart defects

A therapy refractory brain edema is causally responsible for the death of approximately 50% of patients following severe craniocerebral trauma. The development of a brain edema which cannot be controlled by conservative means is also the most frequent cause of death with cerebral emergencies not caused by trauma. The cerebral perfusion pressure (CPP), which is the decisive factor for sufficient cerebral oxygenation, can be calculated on condition that the mean arterial pressure (MAP) and the intracranial pressure (ICP) are continually monitored: (CPP = MAP-ICP). On the basis of neurological observations, the computer tomographical results and the jugular vein oxymetry, an incipient cerebral decompensation and consequently the failure of the ongoing conservative treatment becomes apparent at an early stage. At this point at the latest, a bitemporal craniectomy should be considered for treatment. A drop in CPP to below 70 mmHg for adults and 50 mmHg for children is regarded as the intervention limits. Our experience shows that the outcome can be improved if the time of the bitemporal craniectomy lies before that of the cerebral decompensation.     

High frequency current ablation of supraventricular tachyarrhythmias in congenital heart defects

Supraventricular tachycardia is a frequent cause of disease in patients with congenital heart defects and has a potentially high impact on quality of life, morbidity and mortality of this patient cohort. Conventional treatment often fails to avoid recurrences of tachycardia in a long-term perspective. Potential side effects of antiarrhythmic drugs include aggravation of heart disease related disturbances of impulse generation and conduction properties or negative inotropic effects on haemodynamically impaired ventricular chambers. For these reasons, interventional electrophysiology is increasingly used for the treatment of supraventricular tachycardias in patients with congenital heart disease. Until March 1998 a total of 83 patients with congenital heart defects underwent an attempt for radiofrequency current treatment of supraventricular tachycardias. Among these were 36 children with an age of 5 months to 15 years (8.2 +/- 4.6 years) and 4.7 grown ups with an age of 17 to 76 years (39.3 +/- 14.3 years). In a natural course or preoperative status of the congenital heart disease were 35 patients, while palliative or corrective surgery was performed in 48 patients. Supraventricular tachycardia was based on a total of 63 congenital arrhythmogenic substrates, among them were 53 accessory pathways, 4 Mahaim fibres, 5 functionally dissociated AV-nodes and an anatomically doubled specific conduction system including 2 distinct AV-nodes in one case. In the remaining patients with tachycardia based on acquired arrhythmogenic substrates there were 45 incisional atrial reentrant tachycardias, 15 atrial flutters of the common type and 6 ectopic atrial tachycardias. In a total of 105 sessions 78 of the 83 patients were successfully treated with the use of radiofrequency current ablation. There were no significant procedure related complications. Radiofrequency current ablation can be carried out safely and successfully for the treatment of supraventricular tachycardia in young and adult patients with congenital heart disease. As such therapeutic strategy meets the specific requirements of this patient cohort, early consideration for this therapy is recommended.     

Status and after-care of young adults with congenital heart defects

INTRODUCTION: We studied patients with congenital heart defects born in 1975 and followed by our institution. We were interested in the outcome of these patients at the age of 20 years with regard to professional status, physical activity and psychosocial aspects. Furthermore, we investigated the handover from the paediatric to the adult cardiologist as well as the information level concerning antibiotic prophylaxis against bacterial endocarditis. METHODS: From a previous follow-up study to the age of 16 years, we knew 224 patients with congenital heart disease born in 1975, 119 were considered to need further cardiological follow-up. In 112 (94%) we have a complete follow-up to the age of 20 years with a structured interview by phone. RESULTS: Mortality and medical care during the period between 16 and 20 years of age were at a lower level than in the previous periods. Except in patients suffering from complex or surgically treated cyanotic heart disease, the results, compared to the standard population at the age of 20 years, are better with regard to professional status, and equal for physical activity and psychosocial integration. Only 60% of the patients were followed by an adult cardiologist and only 45% of the patients needing antibiotic prophylaxis against bacterial endocarditis are aware of this. CONCLUSION: Except in patients with complex congenital or surgically treated cyanotic heart diseases, the outcome at the age of 20 years with regard to professional status, physical activity and psychosocial integration is very good compared with the standard population at the age of 20 years. The handover to the adult cardiologist is inadequate and knowledge and awareness of antibiotic prophylaxis against bacterial endocarditis is also insufficient-both aspects need improvement.     

Catheter treatment of congenital heart disease

Over the last 10 years, several advances have been made in paediatric cardiology and cardiac surgery. However, the major developments have been in non-surgical attempts at transcatheter treatment of congenital heart disease. Initially these concerned some simple defects such as pulmonary valve stenosis but lately much more high-risk and complex defects have been treated.     

Echocardiographic diagnosis alone for the complete repair of major congenital heart defects

OBJECTIVES: The study was done to determine the diagnostic accuracy of echocardiography alone in the preoperative diagnosis of children with major congenital heart defects undergoing primary complete repair. BACKGROUND: Although echocardiography is well established as the first-line imaging technique for the diagnosis of all forms of congenital heart disease, most institutions continue to perform cardiac catheterization prior to complete repair of more complex defects. METHODS: To determine the diagnostic accuracy of echocardiography alone and echocardiography plus catheterization, we reviewed the records of 503 children with major congenital heart defects who underwent primary complete repair at our institution between July 1992 and June 1997. We included children with transposition of the great arteries, tetralogy of Fallot, double-chamber right ventricle, interrupted aortic arch, aortic coarctation, atrioventricular septal defect, truncus arteriosus, aortopulmonary septal defect, and totally anomalous pulmonary venous return. We excluded children with less complex defects such as isolated shunt lesions, as well as those with the most complex defects that would require surgical palliation (e.g., functional univentricular heart). We defined major errors as those that increased the surgical risk and minor errors as those that did not. Errors in diagnosis were determined at surgery. RESULTS: Eighty-two percent of children (412 of 503) underwent surgery after preoperative diagnosis by echocardiography alone. There were 9 major (2%) and 10 minor errors in the echocardiography alone group and 7 major and 5 minor errors in the echocardiography plus catheterization group. The most common type of error was misidentification of coronary artery anatomy in patients with transposition of the great arteries. No error in either group resulted in surgical morbidity or mortality. CONCLUSIONS: This study suggests that echocardiography alone is an accurate tool for the preoperative diagnosis of major congenital heart defects in most children undergoing primary complete repair, and may obviate the need for routine diagnostic catheterization.     

Left ventricular dysfunction after open repair of simple congenital heart defects

A new triterpenoid saponin, coumoside A, has been isolated from the whole plant of Cyclamen coum and the structure of this novel saponin (C58H92O27) has been deduced by NMR methods based on 1H, 13C, DEPT, 1H-1H COSY, HETCOR, NOESY-NMR experiments and the FAB-mass spectrum. It has the structure 3 beta-O-[beta-D-glucopyranosyl-(1-6)-[alpha-L-arabinopyranosyl- (1-2)]-beta-D-glucopyranosyl-(1-4)-[beta-D-glucopyranosyl-(1-2)]-alpha-L -arabinopyranosyl]-16 alpha-hydroxy-30,28 beta-lactone-olean-12-ene and is called coumoside.     

Diagnosis and surgical treatment of traumatic heart defects

The authors suggest a method of aldosterone determination in the urine. Aldosterone is extracted from the sample and purified with the aid of columnar and thin-layer chromatography on silicagel. Aldosterone is distinctly identified in ultraviolet light on the chromatogram by its green fluorescence developing after sprinkling the plate with phosphoric acid and its subsequent heating. Quantitative determination is carried out by the measurement of the intensity of aldosterone fluorescence in sulfuric acid.     

Surgery in congenital heart defects: current developments and a few case examples

Congenital heart surgery includes the palliative treatment and surgical complete repair of cardiac malformations in newborns, children and adolescents. Palliative surgery allows early or long-term survival, depending on the primary malformation and the condition of the patient. Confection of a systemic-to-pulmonary shunt (the modified Blalock-Taussig shunt) allows in general recovery from severe cyanosis and leads to development of the hypoplastic pulmonary vascular tree in newborns with severe pulmonary stenosis. Longterm palliation can be applied to all patients in whom establishment of a biventricular heart can not be realized. The total cavo-pulmonary connection represents one possibility to bypass a single ventricle in these complex cases. Total repair allows the restitution of a completely normal anatomy and physiology after surgery; usually, this type of surgery is followed by a normalization of life expectancy with minimal pharmacotherapy. Preoperative diagnosis of congenital heart disease is reasonably performed by transthoracic echocardiography in the majority of cases. Cardiac catheterism is reserved for complex cases and those in which full hemodynamic evaluation is required for proper planning of surgery. Continuous improvement has been realized in the fields of cardiac anesthesiology and pediatric intensive care during the last decade; hence more and more complex cases have been accepted without any negative effect on the operative mortality. Additionally there has been a number of improvements in surgical and perfusion techniques, thus allowing open heart surgery in newborns with a minimal weight of 2000 g. Intraoperative transesophageal echocardiography is performed routinely and allows the proper control of surgical repair; furthermore this examination may be helpful during the weaning period from the extracorporeal circulation. There is a number of interesting topics that will take importance or will be developed in the near future: the role of interventional cardiology in pediatric patients gains more and more importance. There is a potential for minimally-invasive surgery and the number of potential candidates for heart transplantation may increase, due to the fate of long-term survivors after palliative surgery.     

The use of autogenous periosteal grafts as barriers for the treatment of intrabony defects in humans

The purpose of this study was to evaluate the clinical effectiveness of a connective tissue graft including periosteum used as a barrier for guided periodontal tissue regeneration in interproximal bony defects. An open flap debridement of a comparable interproximal bony defect in the same patient was used as a control. This study was performed at 2 different clinical centers. Six paired defects were treated at one center, and 16 paired defects at the other. Reentry surgeries were performed at 6 months. Preoperative comparisons of control and experimental sites with respect to clinical parameters and osseous measurements were similar. Post-surgical experimental sites produced more gain in clinical attachment (1.25 mm on buccal and 1.25 mm on lingual sites at center A and 1.26 mm on buccal and 1.18 mm on lingual sites at center B) and osseous defect fill (1.84 mm on buccal and 2.00 mm on lingual sites at center A and 1.66 mm on buccal and 2.04 mm on lingual sites at center B) when compared to control sites. The results of this trial indicate that clinical resolution of interproximal periodontal defects can be obtained with periosteal grafts used as barriers.