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Biomedical subjects

J S Carvalho

Publications and source records attributed to J S Carvalho.

At least 19 recordsLinked to original sources

First-trimester transabdominal fetal echocardiography.

BACKGROUND: Fetal echocardiography is usually done during the second trimester of pregnancy, but waiting until that time can lead to anxiety for the parents if the fetus is at high risk of having cardiac abnormalities. We report the feasibility of transabdominal first-trimester fetal echocardiography for early reassurance of normality or a prenatal diagnosis of a cardiac malformation. METHODS: We did first-trimester fetal echocardiography and colour-flow mapping with a 5 MHz curvilinear transducer at 13 weeks' gestation (range 12-13+6 days) in 15 fetuses at risk of cardiac defects. The fetus was judged to be normal if the investigation showed normal visceral situs, four-chamber view, and two normally related great arteries of similar size. We did second-trimester scans and neonatal follow-up for the women who continued with their pregnancies; necropsy was done in two cases of pregnancy termination. FINDINGS: Ten fetuses had normal hearts at the time of the first scan, which was confirmed during second-trimester assessment. In one fetus, the four-chamber view was asymmetrical and a moderate-sized apical muscular ventricular septal defect (VSD) was diagnosed after birth. In another two fetuses we diagnosed small muscular VSD on the second-trimester scans. A complex cardiac malformation was correctly diagnosed in one fetus at 12 weeks of gestation. Thus, in 11 fetuses, the imaging was of sufficient quality to reassure the family or to diagnose an abnormality. In the other four fetuses, the investigation was judged to be incomplete, but no definite cardiac abnormality was identified. INTERPRETATION: Transabdominal fetal echocardiography in the first trimester of pregnancy is feasible. In most patients the resolution of images is sufficient to allow assessment of basic cardiac anatomy, when normal, or detection of complex malformations, when present.

Echocardiography, Doppler, Color

The cobweb syndrome: first trimester sonographic diagnosis of multiple amniotic bands confirmed by fetoscopy and pathological examination.

Amniotic band syndrome is a well described clinical entity presenting with deformities of the limbs, thorax, craniofacial skeleton, soft tissues and umbilical cord, but it still lacks a precise definition and a coherent hypothesis for its pathogenesis. We report on a case of first trimester diagnosis of amniotic band syndrome by sonography and fetoscopy. This revealed multiple abnormalities including facial cleft, brain and limb deformities; the appearance of the amniotic cavity was that of a cobweb containing the fetus. Post-mortem examination and histopathological studies confirmed the diagnosis of amniotic band syndrome. These results may enhance the knowledge of its natural course. In addition, based on histological and newly identified ultrastructural features, we present a hypothesis which could help to explain the aetiopathogenesis of the amniotic band syndrome.

Adult

Coronary arterial anatomy in tetralogy of Fallot: morphological and clinical correlations.

OBJECTIVE: To clarify the problems in angiographic diagnosis of major coronary arteries crossing the right ventricular outflow tract. DESIGN: A retrospective study with clinicomorphological correlations to ascertain any aberrant coronary arteries and variations in distribution of the normal right coronary arterial branches. SETTING: Tertiary referral centre. SUBJECTS: 36 necropsy specimens together with the aortograms and surgical reports from 130 patients with tetralogy of Fallot. RESULTS: A preventricular branch was found in 19% of cases with tetralogy of Fallot, but in none of 13 normal hearts. Aberrant origin of the anterior interventricular coronary artery was found in 14% of the specimens. The combination of "laid back" and straight lateral views, when reviewed retrospectively, identified this anomaly correctly in nine of 16 patients, with these findings confirmed at surgery in seven patients. A major branch initially thought to cross the outflow tract was shown retrospectively to be an infundibular artery in six, with surgical confirmation in four. It was a preventricular branch in another patient. CONCLUSIONS: Using the laid back view alone, infundibular and preventricular branches may be mistaken for a major aberrant artery. A combination of laid back and straight lateral views is needed to avoid false positive diagnosis.

Child

Prognostic value of posterior wall thickness in childhood dilated cardiomyopathy and myocarditis.

M-mode indices of left ventricular dimension and posterior wall thickness were derived from echocardiograms of children presenting with dilated cardiomyopathy/myocarditis and were related to outcome. Echocardiograms from 16 of 18 children were manually digitized to obtain changes of left ventricular dimension and posterior wall thickness throughout the cardiac cycle. Indices of ventricular function and the ratio of end-diastolic posterior wall thickness to cavity dimensions were obtained. Patients were divided into group I (alive, n = 7), and group II (died, n = 6 or heart transplantation, n = 3) at median follow-up of 25 months. No significant difference was seen for the shortening fraction, the percentage of posterior wall thickening or the normalized peak rate of left ventricular filling. The normalized peak rate of posterior wall thinning was greater in group II. The posterior wall thickness to cavity dimension ratio was higher in group I (median = 0.19) than group II (median = 0.13) (P < 0.005). Five of six survivors, whose ventricular function improved, had ratios > 0.17. All but one with a ratio < or = 0.16 remained with a dilated heart, died or required transplantation (P = < 0.01). A relatively thicker posterior wall (ratio > 0.17) was associated with better prognosis and recovery. This index should be taken into account in decision-making regarding timing for cardiac transplantation.

Cardiomyopathy, Dilated

Fetal telemedicine: six month pilot of real-time ultrasound and video consultation between the Isle of Wight and London.

DESIGN: Prospective audit of first six months. SETTING: Referral from a district general hospital on the Isle of Wight to a comprehensive tertiary referral service, the Centre for Fetal Care at Queen Charlotte's Hospital 120 km away in London. PARTICIPANTS: Women whose pregnancy was suspected, or at risk, of fetal abnormality. INTERVENTIONS: Remote consultation by transmitting ultrasound and video in real-time over ISDN 30 telephone lines. Contemporaneous questionnaire to referring practitioner and patient. MAIN OUTCOME MEASURES: Frequency, indication, technical success and duration of consultation. Qualitative and semi-quantitative image quality. Effect of teleconsultation on need for physical referral. RESULTS: Twenty-nine women underwent 39 teleconsultations, and image quality was sufficient for diagnosis in all but one. Fetal abnormalities were present in 76%. Referral in person was required for only four women, significantly fewer than the 13 the referring hospital indicated would have been physically referred in the absence of this service (P < 0.001). Most mothers were counselled by the specialist "face-to-face' over the link, and 80% felt teleconsultation reduced their anxiety. CONCLUSIONS: A fetal telemedicine service is technically and clinically feasible. This demonstration suggests that such a service reduces the need for physical referral while increasing the rate of consultation, allowing better selection of patients who might benefit from referral. Further evaluation in a variety of clinical settings is now indicated, along with cost-benefit analysis.

England

Granulocyte-macrophage colony-stimulating factor in combination with pentavalent antimony for the treatment of visceral Leishmaniasis.

The efficacy of GM-CSF was investigated in 20 neutropenic patients (< 1500 neutrophils/microliters) with acute visceral leishmaniasis due to Leishmania chagasi. Patients were randomized to receive either GM-CSF, 5 micrograms/kg daily (intravenously or subcutaneously), or placebo for ten days, in combination with pentavalent antimony, 10-20 mg/kg daily for 20 days. Neutrophil counts were significantly greater on days 5 and 10 of treatment in the GM-CSF group compared with the placebo group (p < 0.02). Eosinophil and monocyte counts were also significantly increased in the GM-CSF group at day 10 (p < or = 0.03). Interestingly, at day 30, platelet counts were significantly increased in the GM-CSF group on days 5 and 10 (p = 0.04 and 0.02, respectively). Patients in the GM-CSF group experienced fewer secondary bacterial or viral infections than placebo patients. Infections occurred in only three patients given GM-CSF compared with eight patients given placebo (p < 0.04). All patients had complete resolution of disease symptoms at three months. Few adverse events were recorded. GM-CSF given subcutaneously at a dose of 5 micrograms/kg daily for ten days was well tolerated, reversed neutropenia rapidly and reduced the number of secondary infections in patients with leishmaniasis.

Adolescent

Anatomic correction for post-mustard pulmonary venous obstruction.

Pulmonary artery banding and arterial switch operation as a two-stage approach have offered a surgical solution to the failing right ventricle after intraatrial repair of the transposition of the great arteries. Banding of the pulmonary artery increases the resistance to the left ventricular output and, therefore, "prepares" the left ventricle for supporting the systemic circulation and arterial switch operation. We report a case of anatomic correction for transposition of the great arteries after intraatrial repair (Mustard's operation) in which the left ventricle was retrained serendipitously as a consequence of pulmonary venous obstruction complicating Mustard's procedure.

Child

Recombinant human granulocyte-macrophage colony-stimulating factor reverses neutropenia and reduces secondary infections in visceral leishmaniasis.

Twenty-four patients with acute visceral leishmaniasis and leukopenia (< 1500 neutrophils/mm3) due to Leishmania chagasi were studied, 4 in an open-label pilot study and 20 in a double-blind, placebo-controlled trial. Patients received granulocyte-macrophage colony-stimulating factor (GM-CSF), 5 micrograms/kg daily, or placebo for 10 days, plus 10-20 mg/kg pentavalent antimony daily for 20 days. In GM-CSF recipients, neutrophil counts increased threefold and fourfold over baseline at 5 and 10 days, respectively, and were significantly higher than those in placebo recipients (P < .02). Eosinophil and monocyte counts were significantly increase in GM-CSF recipients at 10 days (P < or = .03). Secondary infections occurred in 3 GM-CSF and in 8 placebo recipients (P = .04). All patients had complete resolution of their leishmaniasis at 3 months. Few adverse events were recorded. GM-CSF, 5 micrograms/kg daily for 10 days, was safe, rapidly reversed neutropenia, and reduced the number of secondary infections in patients with leishmaniasis.

Adolescent

Cardiomyopathy of glycogen storage disease type III.

To identify the severity of cardiac involvement in glycogen storage disease type III (GSDIII), and its relation to skeletal muscle involvement and age, 23 patients were studied. The median age was 10 years. Echocardiography, electrocardiography, and creatine phosphokinase (CK) levels were used to assess cardiac and skeletal muscle involvement. Septal and left ventricular posterior wall measurements were compared with normal data. Shortening fraction was derived from left ventricular cavity dimensions. In some patients the echocardiogram resembled that of hypertrophic cardiomyopathy. Thirteen of 20 electrocardiograms (ECG) were abnormal. Eleven patients had septal and/or posterior wall thickness > 95% confidence limits (CL). Despite this, cardiac symptoms were uncommon. The CK levels were not directly associated with cardiac abnormalities. Older patients (> 20 years) had more abnormal measurements of posterior wall thickness than did younger ones (< 20 years). This finding, albeit in a cross-sectional series, suggests progressive myocardial involvement with age despite the absence of symptoms.

Adolescent

Angiographic diagnosis of anomalous coronary artery in tetralogy of Fallot.

OBJECTIVE: To obtain angiographic views in tetralogy of Fallot that can show whether or not an anomalous coronary artery passes anterior to the right ventricular outflow tract. DESIGN: (a) A 10 year retrospective review of all patients who underwent repair of tetralogy of Fallot up to December 1990; (b) a prospective study of 30 children undergoing routine cardiac catheterisation. PATIENTS AND METHODS: 295 cases in whom standard angiographic views had been used were reviewed retrospectively. Thirty non-consecutive children with tetralogy of Fallot were studied prospectively, including one child previously studied in whom diagnosis of an unsuspected anomalous coronary artery was made only at operation. The aortogram was performed with > or = 45 degrees caudocranial and 20 degrees-30 degrees left anterior oblique angles. SETTING: Tertiary referral centre. RESULTS: Ten of the 295 cases reviewed were shown to have a coronary vessel traversing the right ventricular outflow tract. In one case the diagnosis was suspected before operation but it was missed in the others. Even in retrospect we could not be certain of the precise anatomy with the use of standard angiographic views. In the prospective study the caudocranial aortogram showed the aortic valve face on in all the patients. The right ventricular outflow tract lay in a left and anterior (seen as superior) position in relation to the aortic root. Thus any vessel crossing the outflow tract could be identified. Identification of the aortic cusps allowed precise definition of the origin of the coronary arteries. All but four had normal origin and course of the coronary arteries. Four had paired left anterior descending arteries (including the restudied patient), in all cases with a large vessel originating from the right coronary artery passing across the right ventricular outflow tract. CONCLUSIONS: Important anomalies of the coronary arteries in tetralogy of Fallot may remain undiagnosed if standard angiographic projections are used. Aortography with > or = 45 degrees caudocranial and 20 degrees-30 degrees left anterior oblique angles allows precise definition of the anatomy and certainty as to whether any major vessel crosses the right ventricular outflow tract. Interpretation, however, can only be correct if the projection is technically adequate with a view of the aortic valve face on. Furthermore, a normal bifurcation of the left main stem does not exclude a second left anterior descending artery crossing the pulmonary outflow tract.

Child

Exercise capacity after complete repair of tetralogy of Fallot: deleterious effects of residual pulmonary regurgitation.

OBJECTIVE: To determine the effects of residual pulmonary regurgitation on exercise tolerance after complete repair of tetralogy of Fallot. DESIGN: Prospective study of symptom free patients more than five years after complete repair. Graded exercise performance was measured with standard Bruce protocol. Maximal oxygen uptake and ventilatory anaerobic threshold were measured by respiratory mass spectrometry. Measurement of pulmonary regurgitant fraction was from pressure-volume loops constructed from measurements of right ventricular volume obtained from biplane angiograms and simultaneous pressures measured with a micromanometer. SETTING: Tertiary referral centre. PATIENTS: 16 patients were studied. Two patients had been excluded because of residual cardiac lesions or inadequate data from cardiac catheterisation. Four were later excluded because they failed to reach a respiratory quotient of greater than 1.0 during graded exercise. RESULTS: There was a significant negative correlation between the degree of residual regurgitation and both total duration of exercise and maximal heart rate achieved. Maximal heart rate and total duration of exercise were significantly lower in the patients than in normal controls. Patients with an abnormal maximal oxygen uptake (less than 85% of the predicted normal value) had significantly greater residual pulmonary regurgitation than those in whom oxygen uptake was normal. CONCLUSIONS: Impaired exercise capacity after complete repair of tetralogy of Fallot is directly related to the degree of residual pulmonary regurgitation. These data should be taken into account when deciding the optimal timing and nature of corrective surgery.

Adolescent

Analysis of left ventricular wall movement before and after reimplantation of anomalous left coronary artery in infancy.

Five infants who underwent direct reimplantation or redirection of an anomalous left coronary artery from the pulmonary artery to the aorta were studied. There were no deaths from the procedure. Anastomoses were patent at a mean follow up of 33 months. Left ventricular angiograms before and after operation were digitised frame by frame. Analysis of wall motion showed considerable generalised hypokinesia with no change in cavity shape before operation. After reimplantation there was a significant improvement of global ventricular function as assessed by ejection fraction and end diastolic shape index. There was also a tendency for the shape index change to increase; this was compatible with better global systolic function. Three of the four patients who were restudied showed normal isometric and contour wall motion plots. One child, the oldest at the time of operation, still showed asynchronous onset of contraction and isovolumic relaxation. The procedure offers a low risk of mortality and a high rate of patency of the anastomosis. Improvement of ventricular function can be remarkable and its extent cannot be assessed simply by ejection fraction measurements.

Anastomosis, Surgical

Treatment of visceral leishmaniasis with pentavalent antimony and interferon gamma.

Acute visceral leishmaniasis is associated with an antigen-specific immunosuppression of mononuclear cells as evidenced by defective in vitro production of interferon gamma. We evaluated treatment with recombinant human interferon gamma in combination with conventional pentavalent antimony therapy in patients with visceral leishmaniasis. Six of eight patients with visceral leishmaniasis (mean duration, 17 months) that had been unresponsive to multiple courses of pentavalent antimony responded to treatment with recombinant human interferon gamma (100 to 400 micrograms per square meter of body-surface area per day) in addition to pentavalent antimony (20 mg per kilogram of body weight per day) for 10 to 40 days. The other two patients improved initially but then relapsed and required treatment with amphotericin B. Eight of nine additional patients with previously untreated severe visceral leishmaniasis were also successfully treated with the combination of interferon gamma and pentavalent antimony. The 14 patients who responded to this regimen had marked improvement in symptoms and in measures of anemia and leukopenia, as well as weight gain, a decrease in spleen size, and an absence or reduction of leishmanias in splenic aspirates. These patients had no recurrence of illness after a mean (+/- SE) follow-up of 8 +/- 1 months. Fever was the only major side effect of interferon gamma. We conclude that the combination of interferon gamma and pentavalent antimony is effective in treating seriously ill patients with refractory or previously untreated visceral leishmaniasis.

Adolescent

Do asymptomatic school children have normal haemodynamics 6-13 years after Mustard's operation?

Twenty asymptomatic school children who had undergone Mustard's operation for simple complete transposition (concordant atrioventricular and discordant ventriculo-arterial connexions) were catheterised electively 6-13 years later. The studies were carried out under general anaesthesia in air and in 100% O2. Oxygen consumption was measured and end-tidal gases were monitored using respiratory mass spectrometry. There was significant left ventricular outflow tract obstruction in 2 patients. Cardiac output in air was normal in 15 and decreased in 5 patients. The pulmonary vascular resistance was normal in 18 of 19 cases, but grossly elevated in one patient. Baffle dysfunction was present in 11 patients: 10 with important gradients between the venous pathways and the systemic venous atrium, and 5 with a leak identified either by a left-to-right shunt or by the course of the catheter. Balloon dilatation was attempted in the inferior caval venous channel in 6 and in the superior caval venous channel in 2. Mean gradient before the dilatation fell after the procedure. No pulmonary venous obstruction was identified. Even in this group of children selected as asymptomatic, approximately half had a detectable haemodynamic abnormality.

Adolescent