Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Fifty children with established congenital heart disease (CHD) were surveyed for the immune profile. Ventricular septal defect (VSD) was the commonest lesion (56%) followed by Tetralogy of Fallot (ToF; 16%), atrial septal defect (ASD; 8%), patent ductus arteriosus (PDA; 4%), transposition of great arteries (TGA; 4%), aortic stenosis (AS; 4%), and pulmonic stenosis (PS), tricuspid atresia (TA), single ventricle with pulmonic stenosis (SV with PS) and dextrocardia with ToF (2% each). Immunoglobulins (IgG, IgA and IgM) were estimated. IgG and IgA levels were significantly reduced in all children with congenital heart disease, whereas IgM levels were increased in cyanotic but unaffected in the acyanotic group. Complement C3 and C4 levels were reduced in all, more so in cyanotics. T-helper cells were decreased and T-suppressor cells were increased in all groups with congenital heart disease as compared to controls. B-cell percentage was increased in cyanotics but not affected in the acyanotics.
 ...
PMID:Immunological profile in congenital heart disease. 789 64

The clinical effectiveness and safety of balloon atrioseptostomy (BAS) under color flow mapping Doppler echocardiography (CFM) guidance was evaluated in 21 BAS procedures performed on 19 patients with cyanotic heart disease (d-TGA 15, PA 1, TA 2, Ebstein's anomaly PA + supramitral ring 1) in the cardiac catheterization laboratory (16 patients) or in the intensive care unit (ICU 3 patients). The indication for BAS was established based on CFM diagnosis. BAS was performed with combined CFM and X-ray guidance on 16 patients prior to cardiac catheterization, and with CFM guidance only on 3 patients in the ICU. A series of BAS was performed until satisfactory interatrial opening was obtained and a complete hemodynamic study was performed by CFM and catheterization after BAS, if possible. In all 19 patients BAS was performed very safely with positioning of balloon in the left atrium, selection of appropriate size of balloon, and the confirmation of the effect of BAS provided by CFM guidance at each step. The size of the interatrial opening was significantly increased from 2.6 to 8.1 mm (p < 0.01) after a series of BAS and the arterial oxygen saturation was significantly increased from 44 to 75% (p < 0.01). CFM provides effective diagnosis and guidance for BAS in severely cyanotic neonates with congenital heart disease, and should significantly contribute to the speed, efficacy, and safety of the procedure.
 ...
PMID:[Balloon atrioseptostomy under color flow Doppler echocardiography guidance]. 806 84

A case is presented of complete transposition of great vessels with atrial and ventricular septum defect and coarctation of the pulmonary artery in Cantrell syndrome. The Cantrell syndrome consists of: congenital heart disease, defect of pericardium, diaphragm, sternum, and anterior abdomen wall. In all cases of Cantrell syndrome described as yet ventricular septum defect was present alone or in combination with other intracardiac defects. The presented case is the first report of congenital abnormality in the from of d-TGA in Cantrell syndrome.
 ...
PMID:[Transposition of great vessels in Cantrell syndrome]. 824 20

Thirteen infants under three months old were repaired aortic coarctation between 1980 and 1988. The mean age at operation was 2 +/- 0.8 months, the mean weight was 3.7 +/- 0.6 kg. Patients were simple coarctation in 1, coarctation with VSD in 7, and coarctation with complex heart disease in 5. As the operative method, the prosthetic patch plasty was done in 11 patients, end to end anastomosis in 1 patient and remaining one was primary VSD closure and secondary coarctation repair (patch plasty). Three patients died perioperatively, the overall early mortality rate was 23%. Since the introduction of PGE1 therapy in 1985, operative mortality rate of the operation (patch aortoplasty) was improved dramatically. Two of 10 patients died from LOS after the secondary operation (one was TGA and another was congenital MS with VSD). Eight patients are alive and clinical course after operation had been good. The measurement of arm-leg pressure difference didn't reveal significant residual gradient. Postoperative isthmus index was 90 +/- 13% in follow up measurement. Recoarctation has not been occurred in patients undergoing operations with patch plasty. Postoperative hypoplastic arch index was 93 +/- 19% in 1 to 2 years after operation compared with 70 +/- 16% of the preoperation. Therefore, it is concluded that prosthetic patch aortoplasty and end to end anastomosis of the aorta are both available as reasonable and/or adequate operation for the early infants with coarctation.
 ...
PMID:[Surgical management of coarctation of the aorta in early infants]. 930 98

The DiGeorge syndrome (DGS) is a developmental defect of the third and fourth pharyngeal pouches, which is associated with congenital heart defects, hypoparathyroidism, cell-mediated immunodeficiency, velo-pharyngeal insufficiency and craniofacial dysmorphism. The aetiological factor in a great majority of DGS cases is monosomy for the chromosomal region 22q11. To analyze DGS at the molecular level, a new molecular probe (DGCR680) encompassing the ADU balanced translocation breakpoint was prepared. When 13 Korean patients with DGS-type congenital heart disease were analyzed with this probe, 9 turned out to have a deletion at this locus, and all of them except one exhibited a typical facial dysmorphism associated DGS. Though only 9 independent patients were detected to have a deletion at the locus using the commercial probe N25 (D22S75), which maps at about 160 kb from the ADU breakpoint to the telomeric end, results from fluorescence in situ hybridization revealed a deletion in all cases tested at this locus. Two patients who had a deletion at the locus D22S75 but not at DGCR680 did not exhibit any DGS-type facial abnormalities. This result implies that the 680 bp probe covering the ADU translocation breakpoint might be a candidate for a molecular marker that can distinguish a specific phenotype, such as facial features associated with the DiGeorge syndrome. This study also suggested that systematic approaches with several small DNA probes along the DGCR could help to dissect the complex phenotypes associated with the DiGeorge syndrome, such as cardiac defects, abnormal faces, thymic hypoplasia, cleft palate, and hypocalcemia, etc.
 ...
PMID:Molecular genetic analysis of the DiGeorge syndrome among Korean patients with congenital heart disease. 1010 75

Of the "five T's" of cyanotic congenital heart disease--tetralogy of Fallot, TGA, TAPVC, truncus, and tricuspid valve abnormalities (tricuspid atresia, stenosis, and displacement)--the first and last are commonly associated with diminished PBF. The four features that comprise tetralogy of Fallot--right ventricular hypertrophy, VSD, overriding aorta, and subpulmonary stenosis--are all secondary to a single morphogenetic defect: failure of expansion of the subpulmonary conus. This also explains the variability in clinical presentation. When neonates need intervention, shunts are usually performed. Coronary arterial anatomy must be defined before repair, which is usually done after these infants are 3 months of age. Although children with repaired tetralogy of Fallot are not completely "normal," markedly increased longevity and improvement in quality of life can be achieved. When major associated defects are present, such as atrioventricular canal defect, diminutive pulmonary arteries or collateral vessels, or left heart lesions, the prognosis changes from excellent to merely good. Tetralogy of Fallot with absent pulmonary valve syndrome is physiologically different from other tetralogy of Fallot conditions and characterized primarily by airway obstruction from massive dilatation of the central and perihilar pulmonary arteries; repair with pulmonary artery reduction is necessary. Tricuspid valve abnormalities include atresia, hypoplasia (i.e., pulmonary atresia with intact ventricular septum), and displacement (i.e., Ebstein anomaly). The pathophysiology that dictates these children's clinical condition (and prognosis) relates to three factors: (1) status of the tricuspid valve, (2) presence and size of a VSD, and (3) TGA or normally related great arteries. Virtually all children with tricuspid valve abnormalities can be palliated; reparative options include repair using two-ventricle, one-ventricle, or 1-1/2 ventricle repair. Children with critical pulmonary stenosis generally have a normal tricuspid valve and right ventricle. Balloon dilation is usually the only therapy necessary.
 ...
PMID:Cyanotic congenital heart disease with decreased pulmonary blood flow in children. 1021 82

Between August 1995 and July 1999, we have experienced 14 donors for allografts (mean age: 39.8 +/- 15.8, M/F = 10/4, mean warm ischemic time: 359 minutes). Donated tissues were included 12 aortic valves and 12 pulmonary valves, respectively. Since February 1994, clinical diagnoses of 14 patients included 7 congenital heart disease, 5 infective heart disease, 1 artificial graft infection, and 1 thrombosed valve. There was no graft-transmitted disease. In congenital heart disease, 3 patients (HLHS: 1, Truncus: 1, TOF + PA: 1) died (early mortality, 42%) and 1 with TGA had residual conduit stenosis. However, in infective heart disease, all patients survived without recurrent infection and did not need reoperation (early mortality, 0%). Our clinical results of homograft implantation for infective heart disease were excellent, but more careful consideration will be needed for congenital heart disease in neonates and/or patients with poor preoperative condition.
 ...
PMID:[Recent donation and clinical results of homograft tissue]. 1077 53

Quantitative assessment of ventricular function in patients with congenital heart disease is often challenging due to distorted ventricular geometry. A myocardial performance index (MPI) has been reported in adults and children that is a Doppler-derived nongeometric measure of ventricular function. The MPI measures the ratio of isovolumic time intervals (isovolumic contraction time and isovolumic relaxation time) to ventricular ejection time. The effects of altered ventricular preload or afterload on the MPI have yet to be determined. This study assesses the impact of altered preload or afterload on right ventricular (RV) function and the RV MPI in the clinical setting of congenital heart disease. Patient groups were compared with normal pediatric and adult populations before and after repair of their congenital heart lesion. Patients with large atrial septal defects (ASDs) represented the clinical setting of increased ventricular preload, whereas patients with isolated pulmonary valve stenosis represented increased RV afterload. Patients with congenitally corrected transposition of the great arteries (CC-TGA) with severe left atrioventricular valve regurgitation represented a combined increase in RV preload and afterload. The RV MPI in 152 normal children (ages 3 to 18 years) and 37 adults (ages 18 to 51 years) was 0.32 +/- 0.03 and 0.28 +/- 0.04, respectively. In pediatric patients (n = 45) and adult patients (n = 40) with ASD, the RV MPI was 0.35 +/- 0.09 (p = NS) and 0.38 +/- 0.04 (p < 0.01 compared with normal adults), respectively. Patients with pulmonary stenosis (n = 21, ages 1 day to 19 years) had a RV MPI of 0.32 +/- 0.06 (p = NS). CC-TGA patients had a RV MPI of 0.72 +/- 0.17 (p < 0.001). No significant change in the RV MPI was seen in any postoperative patient group despite relief of RV volume or pressure overload. Thus, the MPI is a quantitative measure of RV performance that is appears to be relatively independent of changes in preload or afterload in the clinical setting.
 ...
PMID:Usefulness of the myocardial performance index for assessing right ventricular function in congenital heart disease. 1098 Feb 18

An 18-year old female with mental retardation and unexamined complex congenital heart disease received dental care under general anesthesia. Anesthesia was induced and maintained successfully without any significant hemodynamic changes with inhalation of nitrous oxide, oxygen (FIO2 0.25-0.3) and sevoflurane after a heavy premedication (morphine 10 mg, scopolamine 0.3 mg and midazolam 5 mg i.m.). After induction of anesthesia, cardiac anomaly was diagnosed by transesophageal echocardiography as TGA, VSD, PS, and operation was completed without any problem. Two points are considered important in this case; first, to appropriately estimate preoperative cardiac function and second, to adequately manage anesthesia to avoid any hemodynamic fluctuation.
 ...
PMID:[Anesthetic management for a patient with mental retardation and unexamined complex congenital heart disease]. 1107 67

We report a patient with fetal hydantoin syndrome (FHS) with associated d-transposition of the great arteries (d-TGA) and persistent hypocalcemia. d-TGA and hypocalcemia have each been individually reported once in association with FHS, but these patients were also prenatally exposed to phenobarbital. To our knowledge, this is the first report of these problems occurring after prenatal exposure to hydantoin alone. The combination of congenital heart disease and hypocalcemia in our patient raises the possibility of a hydantoin effect on neural crest migration.
 ...
PMID:Transposition of the great arteries and hypocalcemia in a patient with fetal hydantoin syndrome. 1184 Feb 51


<< Previous 1 2 3 4 5 6 Next >>