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

Thallium-201 planar and SPECT studies were performed in 38 children with congenital heart disease using high-resolution three-headed SPECT. The 201Tl total or average right ventricular-to-left ventricular (RV/LV) count ratios were measured by summed short-axis images. In the planar study, the RV/LV systolic pressure ratio correlated with 201Tl RV/LV average count ratio (r = 0.91) better than that in the SPECT study (r = 0.78). However, in the SPECT study, patients with atrial septal defect (n = 9), ventricular septal defect (n = 13), tetralogy of Fallot and double outlet right ventricle (n = 6) showed a characteristic distribution in the plot of RV/LV pressure ratio versus 201Tl RV/LV total count ratio. A prospective study showed that the RV/LV average count ratio was a good predictor of RV/LV pressure ratio (n = 19, r = 0.91). Three-headed SPECT is useful for pediatric cardiac studies, and the overload types found in congenital heart disease seemed to be differentiated by the SPECT study.
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PMID:Assessment of right ventricular overload by a thallium-201 SPECT study in children with congenital heart disease. 174 6

Double-chambered right ventricle (DCRV), a form of right ventricular outflow obstruction that sometimes accompanies a ventricular septal defect (VSD), is associated with superior and rightward displacement of the septal insertion of the moderator band. It was hypothesized that this superior displacement is present and identifiable by echocardiography in patients with a VSD even before right ventricular outflow tract obstruction develops. Eight patients who had a previous echocardiographic study showing a VSD alone were echocardiographically diagnosed as having DCRV. Their initial echocardiographic studies were reviewed, and superior displacement of the moderator band was quantified by measuring the distance between the pulmonary valve and moderator band, normalized to tricuspid anulus diameter. These measurements were compared with those from the initial studies of the following 3 other groups: (1) an age-matched group of 10 patients with no structural heart disease; (2) an age-matched group of 10 patients with a VSD who did not develop DCRV; and (3) a group (not age-matched) of 10 patients with VSD and DCRV in whom subpulmonary obstruction was present on the initial study. The 8 patients who eventually developed subpulmonary obstruction had significant superior displacement of the moderator band at the time of their initial echocardiogram compared with that of the 2 age-matched control groups (p less than 0.01). In contrast, there was no significant difference in moderator band displacement between these patients and the 10 with DCRV who already had right ventricular outflow obstruction at their initial study (p = not significant).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Pulmonary valve-moderator band distance and association with development of double-chambered right ventricle. 174 72

Tetralogy of Fallot, the most common cyanotic heart defect, has not been closely associated with a specific chromosome defect. The San Luis Valley Recombinant Chromosome 8 [SLV Rec(8)] syndrome is strongly associated with congenital heart disease, particularly tetralogy of Fallot. This article reviews SLV Rec(8) syndrome and other chromosome 8 aberrations to suggest locations for cardiogenic genes. SLV Rec(8) [rec(8),dup q,inv(8)(p23q22)] syndrome has been found in Hispanic families in the southwestern United States. Congenital heart disease is found in 93.3% of SLV Rec(8) individuals (n = 45), with tetralogy of Fallot constituting 40.5% of all lesions and conotruncal defects, 55.6%. These frequencies exceed the incidence of tetralogy of Fallot (10%) and conotruncal defects (20%) among all children with heart defects (P less than 0.003 for both). Review of patients with deletion 8p (n = 13) showed heart defects in 84.6% with 27.3% being conotruncal defects. Among duplication 8q patients (n = 20), 45% had heart defects with conotruncal defects constituting 44%. Neither group differed significantly from expected in its incidence of conotruncal defects. Among patients with mosaic trisomy 8 (n = 47), 12 had heart abnormalities including one conotruncal defect. Among 3 patients with other rec(8) chromosomes, one had a ventricular septal defect. The cause of heart defects in SLV Rec(8) cannot be assigned to either the deletion of 8p or the duplication of 8q. The lack of an association between other chromosome 8 abnormalities and tetralogy of Fallot suggests that genes at the SLV Rec(8) breakpoints or an interaction between genes on both arms of chromosome 8 are important.
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PMID:San Luis Valley recombinant chromosome 8 and tetralogy of Fallot: a review of chromosome 8 anomalies and congenital heart disease. 174 13

To establish foetal cardiovascular parameters as predictors of perinatal outcome in pregnancy, M-Mode, 2-D echocardiography and pulse Doppler study was performed at 24-32 weeks of gestation in 65 pregnancies. These pregnancies were followed up for perinatal outcome. The studied population included 24 normal pregnancies, 21 pregnant women with heart disease (14 rheumatic and 7 congenital heart disease) and 20 high risk pregnancies (bad obstetric history in 7, suspected intrauterine growth retardation in 4, hypertensive disease of pregnancy in 6 and diabetes mellitus in 3). There was no perinatal mortality. Two foetuses were born with complete heart block and one with a small ventricular septal defect; 6 neonates had intrauterine growth retardation and two of these had neonatal asphyxia with APGAR score less than 6 at one minute. Anatomically normal heart was correctly diagnosed in all 64 foetuses and ventricular septal defect was detected antenatally in one. Antenatal diagnosis of complete heart block was correctly made in two foetuses. One new born with complete heart block required a permanent pacemaker, which was implanted. The ratio of peak velocity across mitral valve during atrial systole (A) to peak velocity during early diastolic ventricular filling (E) was chosen to correlate with perinatal outcome. The ratio was less than 1.0 in 6 foetuses, all of whom were subsequently confirmed to have intrauterine growth retardation. In normal pregnancies A/E ratio was more than 1.0. We conclude that foetal echocardiography is a useful tool for predicting perinatal outcome and may be helpful in screening patients who require specific perinatal management.
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PMID:Role of foetal echocardiography in predicting perinatal outcome. 175 17

Ventricular emptying was evaluated in patients with congenital heart disease (CHD) with left-to-right (L-R) shunt by factor analysis of gated equilibrium radionuclide angiography. In 36 (95%) of 38 ventricular septal defect patients and 20 (95%) of 21 atrial septal defect patients with small L-R shunt (pulmonary to systemic blood flow, Qp/Qs less than or equal to 2.5), as well as all patent ductus arteriosus patients, two significant cardiac factors corresponding to the ventricles (ventricular factor) and the atria plus large vessels (atrial factor) were extracted. However, in all of nine ventricular septal defect patients with large L-R shunt (Qp/Qs greater than 2.5), two different ventricular factors were determined which corresponded to the right and left ventricles (RV and LV). The RV factor showed a delay of ejection phase compared with the LV factor, and the delay was correlated with the value of Qp/Qs (r = 0.82, P less than 0.01). In eight (80%) of 10 ASD patients with large L-R shunt (Qp/Qs greater than 2.5), RV was described by the two different ventricular factors located in the septal and free-wall regions. The LV was extracted in the same factor as that located in the septal region of RV. This study demonstrates the capability of factor analysis in the pathophysiological investigation of CDH with L-R shunt.
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PMID:Factor analysis of gated cardiac blood-pool data: application to patients with congenital heart disease. 179 21

The coronary artery lesions in Kawasaki disease (KD) are thought to be a possible risk factor for atherosclerosis in the long term from the pathological point of view. Here we report results of a comparative control study to investigate the distensibility of the coronary artery in KD. A total of 146 patients were divided into 5 groups as follows. (I) Normal group (N); consisting of 44 patients with a known history of KD but with no coronary artery lesion. Among this group of patients those who each showed a regression in their coronary artery aneurysms were divided into 2 subgroups according to the time since onset of KD. (II) A group in the early stage after regression (ER); consisting of 18 patients with a known history of KD followed for 1.8 +/- 0.6 years. (III) A group in the later stage after regression (LR); consisting of 22 patients with a known history of KD followed for 9.4 +/- 2.8 years. (IV) an abnormal group (A); consisting of 21 patients followed for 7.9 +/- 4.0 years with a persistent aneurysm and/or stenosis in a coronary artery at the time of this study. And (V) a control group (C); consisting of 41 patients with a congenital heart disease such as a small ventricular septal defect or mild pulmonary stenosis or patent ductus arteriosus and no history of KD. In each case, the coronary artery diameter was measured by angiography before and after intracoronary infusion of isosorbide dinitrate (ISDN).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Coronary artery distensibility in Kawasaki disease--evaluation by intracoronary infusion of isosorbide dinitrate in long-term follow-up. 184 Nov 75

We reviewed autopsies in 50 consecutive cases of congenital heart disease between 1980 and 1988. Autopsy results were compared to premortem clinical diagnosis. The patients ranged in age from two days to 66.5 years with a mean age of 6.42 years; 68% were less than one year of age. The most common diagnoses were hypoplastic left heart syndrome in 16 (32%) and ventricular septal defect in five (10%). Other common diagnoses included coarctation of the aorta, hypoplastic right heart syndrome, tetralogy of Fallot, transposition of the great vessels and tricuspid atresia. No patient had a missed diagnosis that would have changed survival (Class 1). Four (8%) of the patients had one missed major diagnosis that did not change their survival because they were already being symptomatically treated or because no clinical treatment was available (Class 2). The majority of autopsy derived data were missed minor diagnoses that were related [14 (28%)-Class 3] or unrelated [12 (24%) - Class 4] to the terminal disease process. However, despite the small percentage of autopsy findings that would have directly affected survival, the autopsy provided important additional clinical information in 30 (60%) patients. This information could be used to correct management errors and to develop protocols for prophylaxis against known complications. The pediatric autopsy can provide useful clinical data that may be a guide to altering clinical therapy and is essential in providing genetic counseling.
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PMID:The value of the autopsy in congenital heart disease. 187 3

Sixty patients with congenital heart disease (CHD), age range 3 months-45 years (median 2 years), underwent lung biopsy to exclude pulmonary vascular disease (PVD): 25 had an atrioventricular (AV) septal defect, 14 a ventricular septal defect (VSD), 7 a complete transposition of the great arteries (TGA) + VSD, 3 an atrial septal defect, and 2 a patent ductus arteriosus; 9 had other malformations. Scoring of histological section (0-4) according to a modified Heath-Edwards classification disclosed that 30 patients had severe "irreversible" PVD (greater than or equal to grade 3) (11 AV septal defect, 8 VSD, 6 TGA + VSD and 5 others); 8 patients were younger than 1 year. Pulmonary vascular resistance (PVR) was calculated in 51 patients and exceeded 7 U/m2 in 1 of 2 patients with grade 0, in 9 of 18 with grade 1, in 1 of 4 with grade 2, in 11 of 18 with grade 3, and in 8 of 9 with grade 4. PVR was also calculated after 100% oxygen administration in 19 patients, 14 of whom had a resting PVR greater than or equal to 7 U/m2. PVR persisted greater than or equal to 7 U/m2 in 5 patients: 4 had PVD greater than or equal to grade 3 and died after surgery. PVR fell to under 7 U/m2 in 14 patients, 3 of whom had PVD greater than or equal to grade 3:1 patient with grade 4 was not operated upon and 2 with grade 3 were operated upon: all are alive. Follow-up in discharged living patients was 100%, with a mean of 50 months. Fifty-five patients underwent surgery with 10 early and 2 late deaths. Among the 12 subjects with a fatal outcome, 10 had irreversible lesions. The 5 non-operated patients all had irreversible lesions at biopsy, and are still alive.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Is lung biopsy useful for surgical decision making in congenital heart disease? 202 37

Klippel-Feil syndrome is characterized by congenital fusion of the cervical vertebrae. Anomalies in other organs sometimes combine. Many kinds of congenital cardiac anomalies, most frequently ventricular septal defect, considerably associate with this syndrome. Few cases of surgical corrections of cardiac anomalies with Klippel-Feil syndrome have been reported up to today. Therefore surgical problems with this syndrome have not been solved yet, completely. We have experienced a very rare case of a 18-year-old female of total anomalous pulmonary venous connection (Darling Ia type) associated with Klippel-Feil syndrome, that was repaired successfully. No difficulty in the surgical and anesthesiological management referring to the fusion of cervical vertebrae was encountered. The result of surgical correction of congenital cardiac anomalies in Klippel-Feil syndrome mainly depends on the severity of the heart disease and not on Klippel-Feil syndrome itself, as literature and the present case suggest.
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PMID:[Total anomalous pulmonary venous connection associated with Klippel-Feil syndrome: a case report]. 205 86

The congenital heart disease incidence study was designed to collect valid data with respect to new diagnostic methods and to an improved system of preventive health care in order to compare the results with former studies. In the years 1979-1983 there were 41,725 live births in the Tyrol. Among these, 341 cases of congenital heart disease were recognised giving an incidence of 8.2 per 1000. This does not imply a real increase in congenital heart disease, but can be explained by differences in methodological bias. As in previous investigations as well in our study, ventricular septal defect has been noted as the most common cardiac lesion. A remarkable result is a 56% incidence of spontaneous closures. Within five years, 81 children died; 89% of them died by the age of one year.
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PMID:Incidence of congenital heart disease in Tyrol, Austria 1979-1983. 205 12


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