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)

30 patients with tetralogy of Fallot were examined before and after correction. 10 of whom had previous procedures including 13 Blalock-Taussig shunts, 1 Cooley anastomosis and 6 pulmonary valvulotomies (Brock) with a dilator. Hemoglobin and blood gases were measured in 22 patients pre- and postoperatively on the 7th respectively 14th day and finally after 12 months. In 8 children the concentration of 2,3-DPG was accessed (pre-, postoperatively, immediately in ICU, on the 1st, 7th, 14th day and after 21 months). Hypoxia of various degrees was found at any time of the investigation, verified by a low venous oxygen saturation, high 2,3-DPG concentration and an erythrocytosis. The 2,3-DPG concentration was always elevated (preoperatively 18.2 +/- 1.8 muMol/g Hb; postoperatively 1st till 14th day 19.0 +/- 2.2; after 21 months 16.3 +/- 1.2 muMol/g Hb). Preoperatively hypoxia was correlated to the degree of the heart disease expressed by the hight of the Hb-concentration. In contrary after the correction signs of hypoxia (decreased venous oxygen saturation, increased 2,3 DPG-concentration) appeared with a low Hb as found in patients with anemia. The long term check-ups are indicative for slight cardiac residual disorders as there are hypoxic myocardial damage, residual gradients over the right ventricular outlet, reopened VSD's, and ventriculotomy scar tissue. Though the elevated 2,3-DPG-concentration and the consecutive rightward shift of the oxygen saturation curve obviously compensate these cardiac handi-caps as the excellent physical condition of the children shows.
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PMID:[Correction of tetralogy of Fallot and its influence to oxygen transport and lung changes. Part I. Oxygen transport (author's transl)]. 84 77

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 number of pediatric heart transplantations for complex congenital heart disease has increased over the last years, but little experience has been reported in adolescent and adult populations. Between 1987 and 1992, 14 patients (mean age 33.2 years, range 14 to 51 years) were transplanted in our institution because of structural congenital heart disease (n = 9) or other rare disorders of the endomyocardial morphogenesis (n = 5). The main diagnosis included transposition of the great arteries, congenitally corrected transposition of the great vessels, left superior vena cava, tricuspid atresia with right ventricular hypoplasia, double outlet right ventricle with transposition, left ventricular sinusoidal malformation and right ventricular dysplasia. In several cases there were additional intracardiac malformations, including ventricular septum defect, atrial septum defect as well as different forms of pulmonary stenosis. Seven patients had undergone one or more palliative repairs that consisted of modified Blalock-Taussig shunts, Glenn's cavopulmonary anastomosis, Waterstone shunt, Blalock-Hanlon atrioseptectomy and Brock pulmonary valvotomy. Two patients had undergone Senning procedure for transposition of the great arteries. The donor cardiectomy was modified in order to include complete inflow and outflow tissue in the explant and transplantation could be performed without prosthetic material in all patients; deep hypothermic cardiac arrest was never necessary in this series. There was no early or late mortality after a mean follow-up of 37 months (range 4 to 74 months); postoperative echocardiography and cardiac catheterization demonstrated perfect anatomical and functional results in all patients. Adolescent and adult patients with complex congenital cardiac diseases can be transplanted with a very low perioperative risk, even after several prior operative procedures.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Should cardiac transplantation for congenital heart disease be delayed until adult age? 781 78

The number of pediatric heart transplants for complex congenital heart disease has increased in recent years, but little experience has been reported in the adolescent and adult population. Between 1987 and 1992, 6 patients (mean age 24 years, range 14 to 42) underwent transplant in our institution because of structural congenital heart disease with or without prior palliative operation or definitive repair. The diagnose covered: congenitally corrected transposition of the great vessels, late systemic ventricular failure after surgically corrected transposition of the great arteries, left superior vena cava, and tricuspid atresia with right ventricular hypoplasia. The palliative repairs included modified Blalock-Taussig shunt, cavo-pulmonary Glenn shunt, two aorto-pulmonary Waterstone shunts, two Blalock-Hanlon atrioseptectomies and one pulmonary valvotomy by the method of Brock. One patient had undergone pulmonary artery banding, in two patients atrial repair by Senning's technique had been performed for definitive repair of transposition of the great arteries. Donor cardiectomy was modified to remove complete inflow and outflow tissue and transplantation was performed without prosthetic material in all patients, in no case was deep hypothermic arrest necessary. There was no early or late mortality after a mean follow-up of 28 months. Postoperative echocardiography and cardiac catheterization demonstrated perfect anatomical and functional results. There was no early or late mortality after a mean follow-up of 28 months. Postoperative echocardiography and cardiac catheterization demonstrated perfect anatomical and functional results. Adult patients with complex congenital cardiac diseases can be transplanted with a very low perioperative risk, even after several prior operative procedures.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Role of heart transplantation in the treatment of complex congenital malformations in adolescents and adults]. 821 Oct 34

Maurice Campbell was the first editor of the British Heart Journal, now Heart, and was called the journal's progenitor and formative influence, an untiring editor who virtually produced each issue single handedly. Campbell was also valued for his scholarly opinions, organisational and editorial skills, and for his careful statistical work which was advanced for its time and of great importance in the early understanding and treatment of congenital heart disease and arrhythmias. In addition, his collaboration with Russell Brock was instrumental in developing surgery for rheumatic and congenital heart disease at Guy's Hospital.
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PMID:Maurice Campbell: first editor of Heart. 1461 35