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

Within the spectrum of neonatal congenital cyanotic heart disease, emergent surgery is often necessary to correct profound pulmonary hypoperfusion. An ascending aorto-to-pulmonary artery anastomosis (Waterston-Cooley) has proved to be the preferred procedure in selected patients. A new technique to correct the occasional resultant pulmonary arterial deformity is presented which is performed concomitantly with definitive intracardiac correction.
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PMID:Repair of pulmonary arterial stenosis after Waterston-Cooley anastomosis. 15 89

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

During the last 15 years, 26 children who ranged from 4 to 12 years old underwent subcoronary aortic valve replacement (AVR) at the Texas Heart Institute. The valve lesions resulted from congenital heart disease in 20 patients, 15 of whom had had a previous operation including 13 aortic valve procedures. Cystic medial necrosis necessitated AVR in 5 patients, all of whom had physical findings of Marfan's syndrome. One patient had rheumatic heart disease and required double-valve (aortic and mitral) replacement. Six different types of prosthesis were used: the Smeloff-Cutter, Starr-Edwards, Cooley-Cutter, Hancock porcine, Ionescu-Shiley, and Bjork-Shiley. The first patient in the series was the only early death (3.8%). Log-term follow-up data ranging from 1 month to 10 years were available on 20 patients. Three late deaths occurred (11.5%). All survivors are doing well and have good exercise tolerance. These data suggest that AVR performed in childhood is a safe, durable, and well-tolerated form of therapy. Later replacement, if necessary, can be accomplished with larger-sized prostheses. Our present choice is the Ionescu-Shiley bovine xenograft because of its superior hemodynamic characteristics and low incidence of embolic complications.
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PMID:Aortic valve replacement in preteenage children. 738 43

UNICEF decided to achieve the 1977 World Health Organization objective Health For All By The Year 2000 through primary health care, utilizing growth monitoring, oral rehydration therapy, breast-feeding, immunization, family planning, and education of women. Since the 1960s BCG (bacillus Calmette-Guerin) vaccination, DPT (diphtheria, pertussis, tetanus) and OPV (oral polio vaccine) have been available in Sri Lanka. The expanded program of immunization has almost eliminated diphtheria, pertussis, neonatal tetanus, and poliomyelitis. Tuberculous meningitis, bone and joint tuberculosis, measles, and miliary tuberculosis have become very rare. Among other vaccine-preventable diseases, mumps is the commonest cause of aseptic meningitis and viral encephalitis in children. Maternal rubella in the first trimester causes abortion or gross teratogenic effects including congenital heart disease. Safe vaccines may be used to prevent mumps and rubella. In recent years there has been a resurgence of measles in North America among school children, and presently a 2nd dose of vaccine is recommended for children. Japanese B encephalitis has a mortality rate of over 30% and half the survivors have residual brain damage. The Ministry of Health has immunized susceptible children in some of the prevalent areas. This vaccine also gives partial protection against dengue hemorrhagic fever. In Hong Kong, Singapore, and Taiwan hepatitis B vaccine is part of the national immunization schedule because of the common occurrence of primary hepatoma of the liver. At present this vaccine is recommended for health workers in Sri Lanka. Meningococcal meningitis occurs in some Middle East countries such as Saudi Arabia, thus Haj pilgrims are advised to be vaccinated against it before the pilgrimage. In Sri Lanka beta-thalassemia major is prevalent, and as most of these patients are subjected to splenectomy, pneumococcal vaccine should be given to them. Currently research work is being carried out for development of vaccines against rotavirus, streptococcal, and hepatitis A infection.
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PMID:Improving child survival through immunisation. 814 30

The analysis of blood pressure (BP) and heart rate (HR) variability is currently used to investigate the mechanisms responsible for cardiovascular control; therefore, we assessed whether an impairment of 24-h BP and HR profiles and sympathovagal interaction modulating cardiovascular function was present in patients with thalassemia major (TM) in preclinical phase of heart disease. Nine beta-thalassemic patients 18 years old without clinical signs of cardiac failure and 9 age- and sex-matched controls were studied. Twenty-four-hour-ambulatory BP and HR were measured using the SpaceLabs 90207 device. A truncated Fourier series with four harmonics was used to describe the diurnal blood pressure profile. Mean 24-h ambulatory systolic BP, diastolic BP, and mean arterial pressure were significantly lower in TM patients than in normal subjects (P < .05). A significantly higher nighttime HR value was found in TM patients (P < .05). More than 40% of the TM patients did not show a significant diurnal BP and HR rhythm. In TM patients, the overall amplitude of systolic BP, diastolic BP, and HR was significantly lower than in controls (P < .01). The night/day differences of systolic BP, diastolic BP, and HR were significantly lower in TM patients than in normals (P < .01). Furthermore, we performed power spectral analysis on short-term continuous finger BP and HR data in supine position and during passive head-up tilt. Total spectral power of systolic BP was significantly lower in patients than controls (P < .05). Low-frequency (LF) power of systolic BP and diastolic BP and LF/high-frequency (HF) ratio of HR were significantly lower during tilt in TM patients compared to controls (P < .05). High-frequency power of HR was significantly higher in patients than controls (P < .05). The baroreflex gain assessed by alpha-index was the same in supine position but was higher in TM patients during passive tilt (P < .05). An inverse relationship between LF/HF ratio of HR and hemoglobin levels in TM patients was found. Finally, plasma norepinephrine levels were significantly lower in thalassemics (P < .005). In young TM patients in a preclinical stage of heart disease, these findings demonstrated abnormal 24-h BP and HR rhythms and a decreased short-term variability of BP and HR, in particular in the LF range, showing a diminished sympathetic activity.
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PMID:Blood pressure and heart rate in young thalassemia major patients. 963 89

Treatment of patients with beta-thalassemia major has improved dramatically during the past 40 years; however, the current clinical status of these patients remains poorly characterized. We performed a cross-sectional study of 342 patients in the Registry of the National Institutes of Health-sponsored Thalassemia Clinical Research Network. Evidence of hepatitis C exposure was present in 35% of tested patients, was associated with age, and had a rate of spontaneous viral clearance of 33%. Ferritin levels ranged from 147 to 11 010 ng/mL (median, 1696 ng/mL). Median hepatic iron content was 7.8 mg/g dry weight and 23% of patients had values of 15 mg/g dry weight or higher. No patients 15 years or younger and 5% of patients aged 16 to 24 years had heart disease requiring medication. Ten percent had cirrhosis on biopsy. Endocrinologic complications were common among adults. Seventy-four (22%) patients had recent implantable central venous access devices (CVADs) placed. Among 80 episodes of bacteremia in 38 patients, 90% were attributable to the CVAD. Among 330 patients who had received deferoxamine chelation therapy, 224 (68%) reported no complications. We conclude that hepatitis C, iron-related organ dysfunction, and complications of iron chelation therapy are strongly age-dependent in North American patients with beta-thalassemia.
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PMID:Complications of beta-thalassemia major in North America. 1498 52

Regular monitoring of left ventricular ejection fraction (LVEF) for thalassemia major is widely practiced, but its informativeness for iron chelation treatment is unclear. Eighty-one patients with thalassemia major but no history of cardiac disease underwent quantitative annual LVEF monitoring by radionuclide ventriculography for a median of 6.0 years (interquartile range, 2-12 years). Intraobserver and interobserver reproducibility for LVEF determination were both less than 3%. LVEF values before and after transfusion did not differ, and exercise stress testing did not reliably expose underlying cardiomyopathy. An absolute LVEF of less than 45% or a decrease of more than 10 percentage units was significantly associated with subsequent development of symptomatic cardiac disease (P <.001) and death (P =.001), with a median interval between the first abnormal LVEF findings and the development of symptomatic heart disease of 3.5 years, allowing time for intervention. In 34 patients in whom LVEF was less than 45% or decreased by more than 10 percentage units, intensified chelation therapy was recommended (21 with subcutaneous and 13 with intravenous deferoxamine). All 27 patients who complied with intensification survived, whereas the 7 who did not comply died (P <.0001). The Kaplan-Meier estimate of survival beyond 40 years of age for all 81 patients is 83%. Sequential quantitative monitoring of LVEF is valuable for assessing cardiac risk and for identifying patients with thalassemia major who require intensified chelation therapy.
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PMID:Value of sequential monitoring of left ventricular ejection fraction in the management of thalassemia major. 1500 68

Cardiac disease is the primary cause of mortality in beta-thalassemia patients. Except for ventricular dysfunction and pulmonary hypertension that represent the main forms of heart disease in these patients, valvular abnormalities including valvular regurgitation, endocardial thickening and calcification and mitral valve prolapse have also been described. Here we present two patients with thalassemia major and mitral chordal rupture, a previously undescribed abnormality in this population. Pathogenesis of this finding may involve thalassemia-related pseudoxanthoma elasticum-like syndrome, a diffuse elastic tissue defect, which is observed with a notable frequency in these patients and has been associated with numerous cardiovascular complications, including valvular ones.
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PMID:Rupture of chordae tendineae in patients with beta-thalassemia. 1508 70

Splenomegaly is common in beta-thalassemia, bearing some particular hemodynamic features, while splenectomy is an established therapeutic intervention in these patients. Their effects, however, on systemic hemodynamics and thalassemia heart disease have not yet been assessed. The study included 32 consecutive patients, 13 with thalassemia major (TM) and 19 with thalassemia intermedia (TI), aged 23.4+/-4.2 years, requiring splenectomy. Assessment was performed before and 6 months after splenectomy and included hematological profile and resting echocardiography; total blood volume was also measured in 10 of 32 cases. Preoperative echocardiographic data were compared with those of 34 controls. Preoperative left ventricular diameters and mass, cardiac index, and systolic pulmonary artery pressure were all significantly higher in patients compared to controls (p<0.001), but did not differ significantly between TM and TI patients. Postoperatively, the mean hemoglobin level increased from 8.1+/-0.6 to 9.1+/-0.4 g/dl (p<0.001), total blood volume index declined from 2847+/-332 to 2310+/-276 ml/m(2) (p<0.001), blood transfusions were discontinued in 80% of TI patients and mean 6-monthly transfusion requirements in TM patients were reduced from 28+/-5 to 22+/-4 units (p<0.001). However, cardiac parameters were not significantly modified. It seems that left ventricular remodeling, high output state, and increased pulmonary artery pressure characterize both TM and TI patients who require splenectomy. Although these abnormalities remain unchanged after splenectomy, the removal of the spleen may contribute to the prevention of further cardiac damage by ameliorating the patients' hematological status and reducing their transfusion needs.
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PMID:Cardiovascular effects of splenomegaly and splenectomy in beta-thalassemia. 1571 2

The Cardioseal/Starflex septal occluder is usually deployed through a long transvenous sheath for closure of atrial septal defects, persistent foramen ovale, and, less commonly, ventricular septal defects. We describe two patients with residual left-to-right shunting after surgical palliation of congenital heart defects and illustrate the successful use of the double-umbrella device in these situations using a retrograde transarterial approach. This is exemplified by two previously not described interventions: retrograde closure of a large muscular ventricular septal defect in a patient after a palliative Mustard operation and a transcatheter closure of a Waterston-Cooley anastomosis. Effective and safe closure of left-to-right shunting can be achieved by retrograde use of the Cardioseal/Starflex device in selected adolescents and grown-ups with congenital heart disease.
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PMID:Retrograde use of the Cardioseal/Starflex occluder device. 1592 74


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