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At least 1,423 records · Page 79Linked to original sources
PubMed2006

Infective endocarditis with congenital heart disease.

From 1988 to 2005, seven patients were operated at our hospital because of infectious endocarditis (IE) with congenital heart disease (CHD). Underlying CHD included ventricular septal defect (VSD) in 4 (2 previous operations with residual region), atrial septal defect (ASD) in 2 and bicuspid aortic stenosis (AS) in 1. No cases had preventive antibiotic prophylaxis for dental procedures. We could confirm bacteria origin from blood culture in all cases, but two patients had operations without gaining control of the infection. VSD or ASD closure and valve surgery were performed in four patients. One patient had a VSD closure, two patients had valve surgery. There were no operative or hospital deaths and there were no recurrences of IE during the study period. We successfully treated IE with CHD by enough debridement of the infective focus of IE, and valve surgery. It is important for patients with CHD to have preventive antibiotic prophylaxis for dental procedures.

Adolescent↗
PubMed2002

[3-D echocardiography: new developments and future prospects].

Due to limitations in transthoracic and occasionally transesophageal 2-D echocardiography with respect to volumetric analysis and morphologic and functional assessment in patients with congenital malformations and valvular heart disease, additional diagnostic tools have been established. In parallel with the rapid evolution in computer technology, 3-D echocardiography has grown into a well-developed technique, such as volume-rendered 3-D reconstruction, capable of displaying dynamic morphology depicting depth of the structures, their attachment, and spatial relation to the surrounding tissue. Nevertheless, the complexity of data acquisition and data processing required for adequate dynamic 3-D echocardiographic imaging and volumetric analysis does not allow to use this approach routinely. The commonly used dynamic 3-D echocardiography means off-line computer-assisted image reconstruction from a series of cross-sectional echocardiographic images using currently available transesophageal and transthoracic transducers. Alternatively, real-time 3-D echocardiography based on novel matrix, phased-array transducer technology has been introduced. Although this technique can be easily combined with any routine examination, its clinical use is limited because of a lower image quality in comparison with dynamic 3-D echocardiography. Up to now, there is no transesophageal approach available using real-time 3-D echocardiography. Recently, dynamic 3-D echocardiographic technique has matured noticeably. Beside the well-known sequential scanning, which is characterized by a fixed probe and patient in space and predetermined motion of the transducer, the freehand scanning using an electromagnetic location system has found its way to clinical environment. The main advantage of this technique is that the transducer can be freely moved by the examiner and, thus, the data set acquired within a routine examination. Also 3-D rendering and display have been developed further. In this respect, especially the "real-time rendering mode" allowing the reconstructed 3-D image to be animated and moved in space and to look at it from different perspectives has gained increasing acceptance. In valvular heart disease, reconstructive surgical treatment is aspired. 3-D echocardiographic imaging is the only technique providing "surgical views" prior to opening the heart. It is capable of distinguishing particular destructive substructures of the valves and the valvular apparatus. Especially in mitral valvular reconstruction, it is of clinical importance to achieve optimal surgical results. With respect to volumetric and mass analysis, 3-D echocardiography is more accurate and reproducible in comparison with conventional 2-D analysis. It provides data independent of geometric assumptions, what may considerably influence the results in the presence of wall motion abnormalities, especially in aneurysmatic ventricles. Volumetric analysis of the aneurysmal portion may also be helpful prior to surgical resection. 3-D echocardiography can also be recommended as a valuable additional approach to atrial septal defect (ASD), corrected transposition of the great arteries, cor triatriatum, and, within limits, to ventricular septal defect (VSD) as well. Especially with respect to ASD and VSD, the potential significance of 3-D echocardiography prior to device closure is emphasized. At present, its additional information in decision-making and the increasing number of clinical cases that can be addressed and answered already justify the clinical use of this technique.

Echocardiography, Three-Dimensional↗
PubMed2004

Amplatzer closure of atrial septal defect and da Vinci robot-assisted repair of vascular ring.

Technology for minimally invasive approaches to congenital heart disease is a rapidly evolving field. This case report reviews a novel approach to combining two of the newer technologies available to treat a pediatric patient with an atrial septal defect (ASD) and a vascular ring. This report is the first to describe the use of the da Vinci surgical system to assist in a thoracoscopic procedure for a pediatric patient. The da Vinci assisted division of the vascular ring, joined with an Amplatzer closure of the ASD, demonstrates how maximum benefit can be obtained for patients by combining emerging technologies.

Child↗
PubMedDate not supplied

Patent foramen ovale morphology and impact on percutaneous device closure.

We sought to evaluate the effects of atrial septal and patent foramen ovale (PFO) morphology on the efficacy of transcatheter closure. We performed a retrospective analysis of all patients who underwent PFO device closure from January 1997 to January 2002. Forty-seven patients underwent percutaneous closure of a PFO with a median age of 45 years (range, 8-75) and weight of 76 kg (range 28-115). The septal morphology was flat in 33 and aneurysmal in 14 patients. The PFO morphology was a simple flap in 20 and complex in 27 patients. Complex morphologies included long-tunnel PFO (n = 15), coexistent small atrial septal defect (ASD) (n = 5), and aneurysmal septum without a tunnel or ASD (n = 7). Nonstretched PFO diameters were significantly smaller than stretched (4.8 +/- 1.1 mm vs 11.6 +/- 3.8 mm, p < 0.01). Median device size: stretched diameter ratio (DS:SD) was 3.7:1 (range, 2.2-9.1). The DS:SD ratio was significantly higher in patients with complex PFO (mean, 3.9:1 vs 2.6:1; p < 0.05). Device placement was successful in all patients. Five patients required transeptal puncture of the foraminal flap in long-tunnel PFOs. Effective closure on follow-up was achieved in 45 patients (95%). Of the 2 patients with residual shunts, 1 had a complex PFO and the other a simple PFO. Two patients (5%) experienced recurrent neurologic symptoms after device closure despite having no residual shunt by echocardiography. Complex PFO did not increase risk of residual shunt or recurrent neurologic symptoms after device closure.

Adolescent↗
PubMedDate not supplied

Challenges encountered during closure of atrial septal defects.

Since the introduction of transcatheter interventional occlusion of atrial septal defects (ASDs) by King and coworkers, the device closure has become the standard treatment of selected atrial defects. Although it has become routine for standard clinical practice, increasing knowledge of intracardiac anatomy, modification of implantation techniques, and improvements in device designs are making this treatment modality increasingly applicable to a wider range of selected patients. This report summarizes the current trends in patient selection, focusing on the anatomical limits of applicability for ASD device closure, and discusses the future possibilities. Furthermore, typical complications are described with regard to the rare erosion of the atrial roof or the aortic root. Current practice is to oversize the device if the anterior rim toward the aorta tends to be diminutive, achieving a secure position of the device around the aortic root. This and the shape memory of the device may play a major role in this serious complication and should be reconsidered. Although most interventional occlusions of interatrial defects with a device have become clinically routine, some situations remain highly challenging.

Cardiac Catheterization↗
PubMedDate not supplied

Radioangioscintigraphy and Doppler echocardiography in the quantification of left-to-right shunt.

The magnitude of left-to-right shunt in 55 children with isolated congenital heart disease [atrial septal defect (ASD) or ventricular septal defect (VSD) (muscular and perimembranous)] was estimated by two methods: radionuclide quantification and Doppler echocardiography [flow (L/min) = mean velocity x area x ejection time x heart rate]. We found little difference between the magnitude of left-to-right shunt obtained with Doppler echocardiography and that with radioangioscintigraphy for a whole group of patients (N = 55, -11.42% to 12.04%) and for subgroups of ASD (n = 24, -12.49% to 12.19%) and VSD (n = 31, -10.69% to 12.23%). These results indicate that Doppler echocardiography, in comparison with radioangioscintigraphy, is sufficiently accurate for clinical estimation of the Q(p)/Q(s) ratio in isolated congenital heart disease with left-to-right shunt.

Adolescent↗
PubMedDate not supplied

Effects of electrocardiography and chest radiography on the accuracy of preliminary diagnosis of common congenital cardiac defects.

The objective of this study was to compare the accuracy of the expert clinical examination for certain common cardiac defects with and without electrocardiogram (EKG) and chest radiogram (x-ray). The design of the study was a prospective, blinded comparison of diagnostic accuracy of the expert examination with and without EKG and x-ray, using echocardiography as the diagnostic standard. The setting of the study was the pediatric cardiology outpatient department. There were 749 outpatients with heart murmur under 21 years of age without prior echocardiography or pediatric cardiology consultation. The intervention was echocardiography as clinically indicated for evaluation of heart murmur of uncertain cause. Measurements were carried out using the incorporation of EKG and x-ray into multiple linear regression models to assess independent associations, if any, with the accuracy of clinical examination. Results were reported as the presence or absence of independent significant impact of availability of EKG and x-ray on examiner's diagnostic accuracy for innocent murmur, ventricular septal defect (VSD), pulmonary stenosis (PS), aortic valve disease, atrial septal defect (ASD), and patent ductus arteriosus. EKG enhanced detection of ASD and may have helped detect PS. X-ray enhanced detection of intermediate to large VSD. X-ray and EKG were otherwise without demonstrable independent advantage for defect-specific diagnosis. Routine use of one or both of these tests in the initial evaluation of heart murmur in the pediatric cardiology clinic should remain an option.

Child↗
PubMedDate not supplied

Long-term outcome of children with complete heart block diagnosed after the newborn period.

The aims of this study were to assess morbidity, mortality, and long-term cardiac outcome of children with congenital complete heart block (CHB) diagnosed between the ages of 3 months and 15 years. The study population consists of 61 children with CHB diagnosed in five tertiary referral centers in Finland. There were two study groups: CHB diagnosed 3 months to 2 years of age (group 1; n = 29) and CHB diagnosed between the age of 2 and 15 years (group 2; n = 32). Neonatal morbidity was significantly higher in group 1 than in group 2 (p = 0.047). In the whole study population, permanent pacemaker implantation with the main indications of low ventricular rate and syncope (52%) was performed in 80% of cases, with a median age of 11.2 years. Structural heart defect not causally associated with CHB was evident in 9 of 61 patients (15%). Of 8 patients with atrial septal defect (ASD) secundum, 6 (75%) were operated on and 7 (88%) were paced. One patient had small ventricular septal defect. The incidence of dilated cardiomyopathy was 7%. The mortality with cardiomyopathy was very high (75%). Of the 61 children, 3 (5%) died due to cardiomyopathy at the ages of 2, 26, and 31 years. In the long-term follow-up of 17 years (median, range 2.9-46 years) 17% of patients who survived had cardiac problems: arrhythmias in 5, mitral valve insufficiency in 3, ASD secundum in 1, and cardiomyopathy in 1. The long-term outcome did not depend on the age at which CHB was diagnosed. In conclusion, CHB diagnosed after the newborn period carries relatively low mortality and morbidity. Pacemaker application was indicated in most children. The possibility of an associated heart defect or dilated cardiomyopathy indicates regular echocardiographic monitoring in all children and young adults with CHB.

Adolescent↗
PubMedDate not supplied

Surgical therapy of a coronary artery fistula draining into the left atrium.

Coronary artery fistulas (CAFs) are rare vascular anomalies. We describe a case of CAF draining into the left atrium, combined with an atrial septum defect (ASD) and azygos continuity in a 3-year-old girl. Surgical therapy included closure of the ASD combined with closure of the CAF through a left atrial approach. Even though CAF can be treated by interventional means, surgical correction may be required in the presence of associated cardiac abnormalities.

Atrial Appendage↗
PubMed2001

Flow pattern of the superior caval vein in children after closure of atrial septal defect: a comparison of catheter therapy with open-heart surgery.

To compare the flow pattern of the superior caval vein (superior vena cava; SVC) after catheter therapy with that after open-heart surgery, we examined the flow velocity curves of the SVC in 10 pediatric patients who underwent catheter closure of atrial septal defect (ASD) and in 20 pediatric patients who underwent open-heart surgery. We used pulsed Doppler examinations to record the velocity curves. On the velocity curve of the SVC in children after open-heart surgery, the velocity in systole was lower than that in diastole, which is contrary to the normal pattern. The velocity in systole increased more than that in diastole during inspiration, which also differs from the normal pattern. In contrast, the velocity curve of the SVC in patients after catheter therapy showed a normal pattern. The change of flow velocity during respiration became smaller after treatment in the surgery group, although it did not change after treatment in the catheter group. These results might indicate less reserve ability in the right atrium in patients who undergo open-heart surgery. ASD closure by catheter is useful not only for its economy and noninvasiveness but also for its potential to contribute conservation of right atrial function.

Blood Flow Velocity↗
PubMed2000

Pulmonary arterial compliance in children with atrial and ventricular septal defect.

Previous studies have documented the normal values of pulmonary arterial compliance (Cp) in animals and adult humans. In the past, variations in Cp and its measurement in children with septal defects has been unknown. In the present study, we found the Windkessel model, which uses compliance and resistance as parameters, to be a useful tool in understanding Cp in pediatric patients. Calculations of Cp were based on the pulmonary arterial diastolic pressure waveform as an exponential function of time. First, Cp was estimated by studying pressure tracings of the main pulmonary artery (MPA) obtained from both routine cardiac catheterization and pressure measured by a catheter-tip manometer, which was performed during a catheterization study of seven children with various congenital heart diseases. Second, 124 children with atrial and ventricular septal defects aged between 45 days and 12 years were studied using the data obtained from routine catheterization. Hemodynamic data were used to calculate pulmonary vascular resistance (Rp), pulmonary arterial time constant (Tp) and Cp. A strong correlation (r = 0.954) was found in the Cp value estimated by data obtained from routine catheterization and from the catheter-tip manometer study. The present study shows an estimated mean (SEM) Cp in normal children of 1.53 (0.17) ml/mmHg per m2. The estimated mean (SEM) Cp was 1.91 (0.10) and 1.70 (0.11) ml/mmHg per m2 in children with atrial septal defect (ASD) and ventricular septal defect (VSD), respectively. It was found that Cp was significantly (P = 0.04) higher in female patients with a VSD. Also, a significantly low Cp (0.95 +/- 0.06 ml/mmHg per m2) was observed in patients with a VSD and pulmonary hypertension (VSDPH). In conclusion, Cp was calculated by the exponential decay portion of the MPA diastolic pressure waveform. A normal Cp value was observed in ASD and VSD patients and a significantly low Cp was observed in children with a VSDPH. Cp was higher in female VSD patients than in male VSD patients.

Child↗
PubMed2002

[Interventional occlusion of foramen ovale and atrial septal defects after paradoxical embolism incidents].

Two hundred and fifty-one patients with a persistent foramen ovale (PFO), mean age 43.3 +/- 12.4 years, underwent catheter closure between 6/1995 and 6/2001. One hundred and forty-one had an ischemic stroke, 99 a transient ischemic attack (TIA) or prolonged reversible ischemic neurologic deficit, 5 peripheral arterial embolism, 4 suffered from decompression sickness after diving and 2 had transient global amnesia. Fifty-nine of them had multiple events in spite of antiplatelet or anticoagulant therapy. The patients received five different devices: 13 Rashkind Occluders, 20 Amplatzer septal Occluders, 109 Amplatzer PFO-Occluders, 73 CardioSEAL and 36 STAR-Flex devices. Time of fluoroscopy was 8.3 +/- 4.5 min. In three patients a device embolized and had to be removed from the groin vessels. We saw five inguinal or retroperitoneal venous hematomas with the need for operation in one patient. One early and one late perforation of the left atrium caused by a guide wire and a left-atrial disc, respectively, also needed surgery. Fourteen patients had documented late arrhythmias. Six patients with atrial fibrillation needed drugs or cardioversion while the other patients with runs of supraventricular tachycardia, atrial flutter and multiple extrasystoles needed no therapy. On transesophageal echocardiography (TEE) 6 months after implantation we found four significant residual leaks. These patients had the defect closed with a second device. In addition a secundum atrial septal defect (ASD) was closed in 17 patients (mean age 38 +/- 10.5 years) with Amplatzer septal Occluders (12) and CardioSEAL devices (5). These patients had experienced eight strokes and nine TIAs, 3 of them had had multiple events. Two of these patients had a significant residual defect and one had atrial flutter following the procedure. Two hundred and two PFO-patients and 12 ASD patients were followed for 6-62 (24.6 +/- 14.2) months; 2 died due to a traffic accident and a myocardial infarction, respectively. Four patients had another neurologic event following PFO-closure. We now overlook 210 patients with 348.6 symptom-free patient years and have a 1-year recurrence rate of neurologic events of 1.9%. Catheter closure of the PFO and atrial septal defect is a simple, effective and quick method which ensures a high closure rate, avoids life-long anticoagulation and has a low recurrence rate of neurologic events.

Adolescent↗
PubMed2002

[Pulmonary plasma catecholamine levels and pulmonary hypertension in congenital heart disease].

Plasma catecholamines may play a role in the pathogenesis of pulmonary hypertension in congenital heart disease with increased pulmonary blood flow. At cardiac catheterization, blood samples were obtained before and after passage of the lung in patients with congenital heart disease with normal pulmonary blood flow (n=39), elevated pulmonary blood flow with normal pulmonary pressure and normal pulmonary vascular resistance (PVR) in patients with atrial septal defect (ASD) (n=57) or ventricular septal defect (VSD) (n=12), and increased pulmonary blood flow, pressure and vascular resistance in patients with ASD, VSD or both (n=22), or in patients with primary pulmonary hypertension (n=4). Plasma catecholamines were determined by reversed phase high performance liquid chromatography and electrochemical detection (HPLC-ECD). In patients with elevated PVR elevated norepinephrine levels (NE) were observed. In contrast, epinephrine levels (E) were not associated with the development of pulmonary hypertension. Patients with primary pulmonary hypertension had the highest levels of NE while patients with associated Down's syndrome had significantly lower epinephrine levels. Pulmonary plasma norepinephrine levels are increased in patients with elevated pulmonary vascular resistance. Whether this phenomena is the cause or sequalae of pulmonary hypertension needs further examination. In the future, plasma catecholamines may serve as a diagnostic feature or may result in further therapeutic options.

Adolescent↗
PubMed2002

Transcatheter closure of intracardiac shunts.

In the last decade percutaneous transcatheter techniques and devices for closure of intracardiac defects have considerably improved. Transcatheter ASD closure has become a routine procedure in many centers. It is technically feasible in about 80% of the patients with a secundum type defect. The success rate today is higher than 99% and the rate of any complication below 0.5%. The advantages compared to surgery are the avoidance of a thoracotomy, postoperative pain and morbidity. The length of hospital stay is much shorter and most often general anesthesia is not necessary. Patent foramen ovale has been recognized as a potential cause of paradoxical embolism and embolic stroke particularly in younger patients. Transcatheter closure has recently gained popularity since several studies demonstrated a reduced rate of recurrent strokes after device closure. The occlusion is technically relatively simple and can be performed with ASD closure devices or with devices modified according to the anatomy of the foramen ovale. Catheter closure has certain advantages compared with life-long anticoagulation. For some patients this is the only therapeutic option. Nevertheless, there is still a need for randomized studies. Catheter closure of ventricular septal defects is possible today in selected patients. There are devices available specifically designed for muscular defects, for perimembraneous defects and subaortic ventricular septal defects.

Adolescent↗
PubMed2006

Bland-White-Garland syndrome and atrial septal defect - rare association and diagnostic challenge.

We report on a 40-year-old woman referred for evaluation of a cardiac murmur and dyspnea on exertion. The electrocardiogram (ECG) showed incomplete right bundle branch block, and echocardiography revealed a large atrial septal defect (ASD, ostium secundum type) with dilated right-sided heart chambers. At cardiac catheterization, a large left-to-right shunt (78% of the pulmonary blood flow) was found, and surprisingly, the additional diagnosis of anomalous origin of the left coronary artery from pulmonary artery (ALCAPA) was established. After ASD closure and left coronary artery ligation with implantation of a vein graft to the left anterior descending artery, she had an uneventful 18-years follow-up. We discuss the interaction of the two associated conditions, and based on the herein reported unusual combination, we highlight typical features of non-invasive examinations including auscultation, ECG, and echocardiography in adult patients with ALCAPA.

Adult↗
PubMed1997

Identification and genetic characterization of the Pseudomonas aeruginosa leuB gene encoding 3-isopropylmalate dehydrogenase.

The Pseudomonas aeruginosa leuB gene, encoding 3-isopropylmalate dehydrogenase, was identified upstream of asd, encoding aspartate-beta-semialdehyde dehydrogenase. Genetic analysis indicated that leuB is identical to the previously mapped gene defined by the leu-10 allele. The chromosomal leuB locus was inactivated by gene replacement. The insertions had no adverse effect on expression of the downstream asd gene but resulted in leucine auxotrophy. The leuB gene encodes a protein containing 360 amino acids (with a molecular weight of 39153), which was expressed in Escherichia coli as a M, 42000 protein. The results suggested that, in contrast to the situation in other bacteria (E. coli, Salmonella typhimurium and Bacillus subtilis) the P. aeruginosa leuB gene is physically separated from the genes encoding the other enzymes of the isopropylmalate pathway.

3-Isopropylmalate Dehydrogenase↗
PubMedDate not supplied

Prognostic value of pre- and postoperative cardiac troponin I measurement in children having cardiac surgery.

The objective of this study was to determine if perioperative elevation of cardiac troponin I (cTnI) predicts mortality in infants and children after surgical correction of congenital heart defects. One hundred infants and children having open heart surgery were studied. Blood samples for cTnI analysis were collected before cardiopulmonary bypass (CPB) and at 4, 8, 12, and 24 h after initiation of CPB. Demographic information, cardiac defect, repair performed, duration of CPB, complications, and outcome were recorded. Cardiac defects were categorized as atrial septal defect (ASD), ventricular septal defect (VSD), hypoplastic left heart syndrome (HLHS), complex, and "other." Baseline cTnI was significantly lower in survivors (mean 0.42 ng/ml, median 0.35 ng/ml) than in nonsurvivors (mean 1.89, median 1.30), p = 0.0001. Baseline cTnI was significantly higher in the HLHS group (mean 1.47, median 1.10) than in all other subgroups (mean 0.62, median 0.35), p </= 0.009. There were no significant differences between survivors and nonsurvivors at the remaining sampling times. Children who died from cardiac failure (n = 2) were more likely to have 4 h cTnI >125 ng/ml compared to survivors (2 of 90). Within cardiac defect subgroups, 4 h cTnI was significantly higher in the complex group (mean = 53.51, median = 32.30) than in the ASD (mean = 23.84, median = 19.85) and other (mean = 21.59, median 21.50) subgroups. Perioperative measurement of cTnI identifies children within specific cardiac defect subgroups at risk of mortality after cardiac surgery. We speculate that detection of myocardial injury may decrease mortality and morbidity in children with complicated congenital cardiac lesions by leading to improvements in perioperative management.

Female↗