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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Paired right and left atrial indicator dilution curves performed in a series of 17 patients with D-transposition of the great arteries (D-TGA) were reviewed. Taken together, the patterns obtained from patients who had trivial mixing between the pulmonary and systemic circulations were distinctive and easily recognizable. In the presence of substantial degrees of intracardiac mixing, however, the paired curves recorded become virtually identical and cannot be distinguished from curves recorded from patients with other forms of cyanotic congenital heart disease characterized by obligatory admixture. Thus paired atrial dye curves demonstrate a high degree of diagnostic specificity for the infant with D-TGA at greatest risk, normally those with a small intracardiac shunt. Such a procedure should facilitate early balloon atrial septostomy and obviate deterioration in the infant's condition due to prolonged catheter manipulation and/or angiography.
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PMID:Indicator dilution curves in the diagnosis of D-transposition of the great arteries in infancy. 38 46

The age distribution of death in all children with congenital heart disease (CHD), who died in a 27-year period in Central Bohemia (population of 1.2 million), and the data on the incidence of CHD in children born in Bohemia (population of 6.3 million) in 1980 were used to calculate the probability of survival of a child born with CHD. Eighty-six percent of these children survived to the first month of life--mostly those with pulmonary stenosis (PS, 99%), aortic stenosis (AS, 95%), ventricular septal defect (VSD, 92%), and atrioventricular septal defect (AVSD, 91%). Seventy-one percent of patients survived the first year of life--mostly those with PS (97%), AS (91%), atrial septal defect (ASD, 89%), VSD (80%), and persistent ductus arteriosus (PDA, 78%). In total, 67% of CHD patients can be expected to survive childhood. The highest survival rates were found in PS (94%), AS and ASD (84%), VSD and PDA (70-80%), and coarctation of the aorta (COA, 68%). The survival rate for the remaining forms of CHD was less than 50%. The highest mortality rate (10% of all children born with CHD) can be expected in the first postnatal week. The lowest survival in the first week was found among those with hypoplastic left heart (HLHS, 39%), double-outlet right ventricle (DORV, 50%), truncus arteriosus (TrA, 57%), pulmonary atresia (PA, 70%), and transposition of the great arteries (TGA, 83%). In addition, total anomalous pulmonary venous connection (TAPVC) and single ventricle had the highest risk of death in the first year of life.
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PMID:Children with congenital heart disease: probability of natural survival. 160 15

A characteristic sinusoidal tcPO2 pattern was observed in 17(27%) of 62 patients with congenital heart disease manifested by cyanosis or heart failure during the neonatal period. All of these 17 patients were definitively diagnosed by cardiac catheterization, as having 8 PPA; 3 ToF; 2 TGA + VSD + PA; 2 asplenia with PA; 1 TGA + VSD + PS; and 1 TA (no TGA type). The transcutaneous oxygen pressure pattern of these patients showed a sinusoidal change with a cycle length of 6-20 min with the maximum pressure not exceeding 50 torr and the minimum pressure occasionally close to 0 torr. This sinusoidal tcPO2 pattern was associated with various types of ductus-dependent congenital heart diseases and hence may be of diagnostic value. Transcutaneous oxygen pressure monitoring is also useful in evaluating the ductal response to PG. Other possible mechanisms underlying this phenomenon including biological oscillation should be considered.
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PMID:The value of the sinusoidal tcPO2 pattern in the evaluation and management of cyanotic congenital heart disease with ductus dependent lesions. 250 23

Cine magnetic resonance imaging (MRI) was used for postoperative evaluation of eight patients who underwent intra-atrial baffle procedure for surgical repair of D-transposition of the great arteries (D-TGA). Their ages ranged from 9 months to 8 years. Younger patients were sedated with chloral hydrate (80 to 100 mg/kg) orally. MRI was performed with use of a General Electric Signa system operating at a field strength of 1.5 tesla. A body or head coil was used depending on the size of the patient. Images were obtained by use of a technique of gradient-recalled acquisition in steady state (GRASS) that utilizes a low flip angle and shorter repetition and echo times. Five patients had widely patent venae cavae and three had superior vena caval obstruction at the junction of the right atrium with a dilated azygos vein. There was no evidence of pulmonary venous obstruction in any of the patients. Right ventricular function was assessed in four patients and their ejection fractions ranged from 58% to 81%. Tricuspid and mitral regurgitation were observed in three and two patients, respectively. Both right and left ventricular outflow tracts were well visualized and showed no evidence of obstruction. Cine MRI is an entirely noninvasive, nonionizing, and safe procedure in young patients and appears to be a valuable alternative method for evaluating patients after surgical repair of D-TGA. With advancing technologies and an accumulation of experience with cine MRI, it appears that this new technique will play an important role in patient care for children with congenital heart disease.
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PMID:Cine magnetic resonance imaging after surgical repair in patients with transposition of the great arteries. 333 61

Real-time two-dimensional Doppler echocardiography (2-D Doppler) is established as an effective diagnostic tool for cardiovascular diseases. However, in determining indications for surgery of congenital heart disease (CHD), the possibility of avoiding preoperative cardiac catheterization including radiographic angiography, has not been fully discussed since the clinical introduction of 2-D Doppler. We evaluated 278 patients with CHD by 2-D Doppler including ASDs (75 patients), VSD (76 patients), TOF (23 patients), TGA (12 patients), PDA (23 patients), ECD (13 patients), and 60 miscellaneous types of CHD during 27 months. The diagnosis of the 278 patients were confirmed by catheterization and/or surgery. Of these, 114 patients underwent corrective surgery (83 patients) or palliative procedures (31 patients). Preoperative cardiac catheterization was performed for 88 patients (Group A), and omitted in 26 patients (Group B). The 2-D Doppler diagnosis was correct in 79 patients of Group A (89.8%) and in 26 patients of Group B (100%). The surgical procedures scheduled according to the preoperative 2-D Doppler diagnosis were performed for these 105 patients (92.7%) without any corrections by cardiac catheterization diagnosis or by surgical findings. However, 2-D Doppler failed to establish the preoperative diagnosis for six patients in Group A (two patients with ASD, three with PDA, and one with TOF) and failed to detect associated cardiac abnormalities for three patients in Group A (two with PAPVC, one with Azygos connection). Results of this study suggest that preoperative cardiac catheterization could be omitted in a maximum of about 90% of surgical candidates with CHD. However, to omit cardiac catheterization 2-D Doppler echocardiographic criteria for indications for surgery in each group of CHD must be established, and any associated coronary artery abnormalities must be ruled out.
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PMID:[Color flow mapping 2-D Doppler in determining surgical indication for congenital heart disease]. 350 16

Figures of incidence and risk factors of neurological complications (NC) endangering children with cyanotic congenital heart disease (CCHD) are very different. We examined 308 children with CCHD over an 8 years' period finding 39 cases (= 12,7) of NC. There were 5 hypoxic attacks and 3 brain abscesses. In 31 patients a cerebro-vascular accident was proven or probable. 56% of NC occured during the first year of life, most frequently in children with simple TGA. Mortality was not influenced by NC. Children without NC, surviving the first year of life represent the control group for comparison of the following hematologic parameters: erythrocytes, hematocrit, MCHC, hemoglobin, O2-saturation, platelets count. Only the last two were significantly lower in patients with NC, but no parameter allows to predict NC. The hypothesis of hypochromic, microcytic anemia as essential pathogenetic factor for cerebro-vascular accidents has no base in our material.
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PMID:[Neurological complications in children with congenital cyanotic heart disease (author's transl)]. 610 46

This study demonstrated the diagnostic usefulness of the newly-developed real-time two-dimensional Doppler echocardiography (2-D Doppler) in congenital heart disease. Among fifty-four patients with congenital heart disease, 18 had ASD; 16, VSD; 6, T/F; 3, PDA; 3, d-TGA; 3, ECD; and 1 each, DORV, PA, PS, Ebstein's anomaly, and ruptured aneurysm of the sinus of Valsalva. Each diagnosis was confirmed by cardiac catheterization and/or surgery. Forty normal cases were subjected as the control. The study cases included 26 adults and 28 children under 15 years old, 13 infants and seven newborns. In 52 cases (96.3%) 2-D Doppler provided diagnostic abnormal intracardiac blood flow images which were compatible with the data of cardiac catheterization and cardiac angiography and/or patients' cardiac anatomy observed during surgery. The blood flow data obtained by 2-D Doppler facilitated determining the appropriate timing of palliative surgery such as the Blalock-Taussig shunt procedure. 2-D Doppler was effective in evaluating medical (pharmaco-echocardiography) and surgical therapy including radical and palliative procedures for congenital heart disease. Thus, 2-D Doppler proved a non-invasive and useful diagnostic method for congenital heart heart disease. Our data suggest that with this technique cardiac surgery can be performed without cardiac catheterization or cardiac angiography in some cases of congenital heart disease.
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PMID:[Real-time two-dimensional Doppler echocardiography in congenital heart disease: its clinical significance]. 654 77

We have carried out an epidemiological-statistical study about congenital heart disease on 33.245 live-born infants in Parma from 1972 to 1980. 327 newborns with congenital heart disease were found; the incidence was 9,8/1000 live-born. The lowest value was 5,8/1000 live-born in 1972, the highest was 14,5/1000 live-born in 1977. In the majority of cases diagnosis was only clinical VSD, PDA, ecc.), whereas in the more complex cardiac malformations and in patients with severe congestive heart failure diagnosis was confirmed at cardiac catheterization and at surgery. In 60 cases diagnosis was confirmed also at autopsy. The congenital heart disease most frequently observed was VSD (33%), followed by PDA (13,4%) and TGA (8,8%), ecc. Clinical status at 1 year of age in all patients was evaluated. The Authors think that infant cardiac program is necessary to improve the results on congenital heart disease control.
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PMID:[Epidemiological study of congenital heart diseases in the province of Parma during the years 1972-80 (author's transl)]. 734 42

A total of 38 patients aged under 3 months with congenital heart disease (CHD) underwent palliative surgery between April, 1988 and March, 1993. The mean age at operation was 28.0 (range 1 to 87) days. Palliative procedures were: pulmonary artery banding (PAB) in 14 patients (IAA complex: 4, CoA complex: 6, AVSD: 2, TA: 2), Blalock-Taussig shunt (BTS) in 12 (TOF: 2, TGA: 1, AVSD: 1, PA-IVS: 3, PA-VSD: 3, PA-SV: 1, PA-AVSD: 1), Brock operation in 6 (PPS: 2, PA-IVS: 3, PA-SV: 1), Blalock-Hanlon operation (BH) with PAB in 2 (MA-SV: 2) and Norwood operation (NRD) in 4 (HLHS: 4). PAB of IAA or CoA complex was performed just after the repair of IAA or CoA. Overall operative mortality was 23% (PAB: 14.3, BTS: 8.3, Brock: 33.3, NRD: 100%). One week after PAB, pulmonary artery pressure (PAP) decreased significantly compared to the intraoperative PAP value after PAB (43.1 +/- 16.2, 32.3 +/- 9.0 mmHg, p < 0.05, respectively). Pulmonary artery index (PAI), which is an index of pulmonary artery growth, after BTS increased significantly compared to the preoperative value (mean follow-up interval: 22.1 months) (379.5 +/- 101.4, 159.3 +/- 51.2, p < 0.001, respectively). During Brock operation, balloon catheter was used in order to dilate pulmonary valve. One year after Brock operation, mean pressure gradient through the pulmonary valve 22.6 mmHg. Two-staged corrections of CHD will be performed both safely and successfully by effective palliations at the first stages in early infancy.
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PMID:[Palliative surgery of congenital heart disease in early infancy]. 751 26

The so-called "conotruncal anomaly face syndrome" (CTAFS) is characterized by a peculiar facial appearance associated with congenital heart disease (CHD), especially cardiac outflow tract defects such as tetralogy of Fallot (TOF), double outlet right ventricle (DORV), and truncus arteriosus (TAC). CTAFS and the DiGeorge anomaly (DGA) have many similar phenotypic characteristics, suggesting that they share a common cause. In many cases DGA is known to be associated with monosomy for a region of chromosome 22q11.2. Fifty CTAFS patients and 10 DGA patients, 11 parents couples and 10 mothers of CTAFS patients, and 3 parents couples and 2 mothers of DGA patients were examined by fluorescent in situ hybridization (FISH) using the N25 (D22S75) DGCR probe (Oncor). Monosomy for a region of 22q11.2 was found in 42 CTAFS, 9 DGA, 4 mothers, and 1 father who had CTAF without CHD. The remaining 8 CTAFS patients 1 DGA patient and 1 mother who had questionable CTAF without CHD, showed no such chromosome abnormality. For the control, 60 patients who had CHD without CTAF or other known malformation syndromes were examined and had no deletion of 22q11.2. Therefore, we conclude that CTAFS is a part of the CATCH 22 syndrome; cardiac defects, abnormal faces, thymic hypoplasia, cleft palate, and hypocalcemia (CATCH) resulting from 22q11.2 deletions.
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PMID:Confirmation that the conotruncal anomaly face syndrome is associated with a deletion within 22q11.2. 785 65


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