Biomedical subjects
Publications and source records attributed to A Cabrera.
We present a case of univentricular connexion to a dominant left ventricle with double orifice tricuspid valve, left atrioventricular valvar atresia, discordant ventriculo-arterial connexion and aortic arch hypoplasia. Diagnosis was made by cross-sectional echocardiography and angiocardiography. This case is exceedingly rare.
Plasma noradrenaline (NA), adrenaline (A), dopamine (DA), platelet serotonin (pS), free serotonin (fS), cortisol (CRT), growth hormone (GH), peripheral blood lymphocytes (lymph), lymphocyte subpopulations (LSS) and CD4/CD8 ratio were serially assessed in 50 non-medicated, advanced cancer patients (spontaneous evolution) and in age- and sex-paired controls. Clonidine tests and psychiatric evaluations were also serially performed. Patients showing long symptomless periods had all normal values except for raised pS, whereas those who remained free of symptoms for only a short time had raised NA, A and CRT, plus lowered pS values. Further increases in NA, A and CRT, plus additional increases in DA and fS, occurred during exacerbation periods, during which times reductions in lymph, LSS and NK also were observed. Patients in terminal stages showed maximal decreases of all neurotransmitters and immunological parameters; only DA and fS remained raised. Psychiatric interviews performed simultaneously with the clonidine tests revealed a low incidence of moderate depression during symptomless periods and no depression during exacerbation periods. Several significant positive and negative correlations between neurotransmitters and immunological parameters were found during exacerbation periods. Pain, although not intense, and other symptoms required occasional administration of low doses of non-opiate analgesics.
A case of a previously unreported anomaly is presented in which absence of the aortic valve cusps, mitral atresia, a normal left ventricle, and an intact ventricular septum were diagnosed by cross sectional echocardiography. The development of a normal left ventricle, rather than the hypoplastic ventricle usually associated with mitral atresia, is explained by filling of the ventricular cavity via the regurgitant aortic valve.
The case of a patient with right aortic arch and retro-esophageal aorta associated with a perimembranous ventricular septal defect with a mild fibrous subaortic stenosis is presented. The existence of the retro-esophageal segment of the aorta was confirmed by left ventriculography and computerized axial tomography. This patient is the first to be diagnosed by axial tomography in our country.
We report the case of a male newborn infant with aneurysm of atrial appendage, ascending aorta and sinus of Valsalva associated to ventricular septal defect, fibromuscular subaortic stenosis and single coronary artery. The diagnosis was carried out by means of two-dimensional echocardiography and angiocardiography. This complex cardiac malformation has not been reported before.
Between 1971-1988 we have studied 46 children with dilated cardiomyopathy. Twenty were males and 18 females, with a median age of 17 months (range from 1 day to 11 years). The incidence of 3.4 cases a year during the first nine years dropped to 1.3 cases a year in the following years. Two patients were siblings. Seventeen patients were studied with Eco-Doppler, cardiac catheterization and angiocardiography. The most frequent presentation was heart failure, present in 45 cases (98%). By bidimensional echocardiography it was observed that all patients had a dilated left ventricle, with a left atrial/aorta ratio of 1.5 +/- 0.3; the ejection fraction was diminished in 12 patients (11-36%) and the length of the left ventricle in the long parasternal axis view 4.2 +/- 0.7. All the patients studied had an elevated telediastolic pressure in the left ventricle, four with pulmonary artery pressure of 96 +/- 11 and seven with right atrial pressure of 24 +/- 5. There was mitral insufficiency in 4 patients and tricuspid insufficiency in three. Clinical course was favorable in 18 patients with median age at present of 112 months (range: 11 months to 15 years). The last 6 patients, treated with vasodilators, are living. In conclusion, dilated cardiomyopathy is a moderately frequent disease, with trend to diminish in incidence prognosis improved after treatment with vasodilator was introduced, and easily assessed with non-invasive methods.
Fourteen cases (10 males, 4 females) of aortic atresia with hypoplastic left ventricle are reviewed. All were studied by two-dimensional echocardiography, treated by prostaglandin E1 and proved by necropsy. Surgery was also performed in 3 cases by Norwood technique. A good echo-anatomical correlation was found in the size of pulmonary artery (1.14 +/- 0.17 and 1.28 +/- 0.33 respectively), mitral valve (0.4 +/- 0.3 and 0.33 +/- 0.24) and aortic diameter ring with atresic valve (0.38 +/- 0.12 and 0.31 +/- 0.1). However, echocardiography overestimated the diameter of ascending aorta (p less than 0.001) and tricuspid valve (p less than 0.001) and the length of the left ventricle. These differences may be related to the greater distension of the aorta in vivo and to the difficulty of determining the exact direction of the ultrasounds. We conclude that two dimensional echocardiography is of great value not only for the diagnosis but also for the selection of patients for surgery.
Pimozide was compared with carbamazepine in a double-blind crossover trial in 48 patients with trigeminal neuralgia who were refractory to medical therapy. Pimozide treatment produced greater reduction in trigeminal neuralgia symptoms than carbamazepine treatment. All of the pimozide-treated patients improved, while only 56% of carbamazepine-treated patients were relieved of their pain. Although both drugs provoked some adverse effects, it was not necessary to interrupt the trial in any case. After this 24-week trial, all patients began receiving pimozide and were followed up according to an open-label study design. In all cases, the pimozide dosage was progressively reduced until the minimal effective dose was reached. Central and peripheral mechanisms that may underlie pimozide-induced improvement are discussed.
The authors review the literature on subarachnoid haemorrhage of unknown aetiology (SAHUE) and analyze a personal series of 212 patients diagnosed as SAHUE. These patients represent 30% of all cases of primary SAH admitted over a 14.5 year period. The age, sex, antecedents and initial clinical presentation of patients with SAHUE were indistinguishable from those of patients with subarachnoid haemorrhage due to ruptured aneurysm (SAHRA). However, the present series of SAHUE compare favourably with both a personal and a previously reported series of SAHRA insofar as clinical grade on admission (94% of patients in grades I-II of Botterell), presence of blood on CT (51%), vasospasm (5%), ischaemic deficits (3.3%), persistent hydrocephalus (3.5%), rebleeding (6%) and fatal result (3.9%) are concerned. The amount of blood on CT scan in our patients with SAHUE was associated with a significantly higher incidence of brain ischaemia and hydrocephalus but did not correlate with the Botterell grade on admission or final outcome, which were good in the majority of cases regardless of the presence or not of visible cisternal haemorrhage. The results of the present series confirm that the final prognosis of patients with primary SAH showing normal four-vessel cerebral angiography is essentially favourable.
We present a study using cross-sectional echocardiography in 39 patients (29 male and 10 female) with discrete subaortic stenosis. Five parameters were evaluated in the study: the morphology of the obstruction, the distance of the stenosis from the aortic valve, the type of insertion, the base of implantation, and any associated anomalies. The lesion could be divided into groups with either fibrous or fibromuscular shelves. In the group of 14 patients with fibrous shelves, the distances from the stenotic lesion to the aortic valve was less than 15% of the length of the left ventricle. The obstructive fibrous tissue was inserted on the septum and extended onto the aortic leaflet of the mitral valve (circumferential lesion) in 12 cases. The base of implantation was narrow in all 14 of them. In the 25 patients having fibromuscular lesions, the distance between obstruction and valve was greater than 18.4% of the length of the left ventricle. The insertion of the obstructive lesion was circumferential in 18 cases and its base of implantation was wide in 20 of the patients. Nineteen patients, 16 of whom had fibromuscular lesions, showed associated anomalies. Statistical analysis showed a good correlation between the type of stenosis and the base of implantation of the stenotic lesion (P less than 0.001) or associated anomalies (less than 0.01), but there was no distinction between the groups with regard to the type of insertion (less than 0.38). Twenty-five patients underwent surgical correction which was carried out between the ages of 4-14 years. Prosthetic valves (3 aortic and 1 mitral) were inserted in 4 patients.(ABSTRACT TRUNCATED AT 400 WORDS)
A 57-year-old white man presenting frequent recurrent chest and precordial pain, heartburn (pyrosis) and post-prandial vomiting for the previous 33 years (one to two years after Bilroth II gastrectomy) was submitted to cardiovascular, endoscopic, radiologic and biochemical studies with negative results. Doctors recommended surgical operation because of an excessively long afferent loop, Several biologic markers were performed at our hospital (intestinal pharmacomanometry, i.m. clonidine test, plasma neurotransmitters plus hormones, oral glucose tolerance test, plasma insulin, etc.), revealing an autonomic nervous system (ANS) imbalance characterized by hyperactivity of the cholinergic plus hypoactivity of the noradrenergic central system. Psychiatric evaluation demonstrated Dysthymic Depression. Treatment with a small daily dose of amitriptyline (a drug which enhances central noradrenergic activity and exerts powerful anticholinergic effects) suppressed symptoms, normalized physiological plus hormonal plus neurochemical parameters and made depressive manifestations disappear. The results suggest that the ANS imbalance was related to depressive syndrome and potentiated by neurohumoral disorders depending on duodenal and jejunal exclusion, and on intestinal post-prandial hyper-osmolarity.
UNLABELLED: From 1983 to 1987 we have studied by two-dimensional echocardiography 11 pediatric patients with Marfan's syndrome. All of them presented cardiovascular lesions localized mainly in the aortic and mitral valves and in the ascending aorta. Nine patients had a dilated aortic ring in both transversal and longitudinal diameters 2.03 and 2.29 cm/m2 of body surface (bs), respectively. The average value of the aortic valvular area was 4.71 cm2/m2 bs. The 9 patients presented also dilatation of the ascending aorta (2.01 cm/cm2 bs). There was thickening of the mitral valve in 5 cases and a prolapse was present in 8 (73%). In 4 cases the prolapse was localized in anterior leaflet valve in 2 in the posterior and in 2 in both. The maximal mitral valve diastolic area was 3.25 cm2/m2 bs. None of them has needed during follow up valvular replacement. Considering the importance of this cardiovascular disease and knowing its unpredictable evolution we recommend, in the absence of valvular insufficiency, and annual echocardiographic reevaluation, and more often if valvular insufficiency is already present. IN CONCLUSION: two-dimensional echocardiographic is useful and necessary for the diagnosis and follow up of patients with Marfan's syndrome.
We present 10 symptomatic patients with scimitar syndrome. Their age was below 1 year and four were newborn babies. In 5 patients there was secundum atrial septal defect and in one there was a patent ductus arteriosus. Sixty per cent of the patients had pulmonary sequestration. The right lung was moderately or severely hypoplasic in 8 cases and seven had frequent respiratory infections. The anomalous venous drainage produced a left-to right shunt at atrium level which in our series had a value of 2.87 +/- 0.5. Five patients had pulmonary hypertension either moderate or severe. The severity of this disease in early infancy is related to the pulmonary hypertension (40%), requiring a prompt surgical correction. Eight patients were operated upon, the drainage being connected to the left atrium. The six surviving patients are asymptomatic at 12 years of follow-up. Our series is the biggest published in our country and the second in the world literature in infancy.
Double orifice mitral valve is a rare congenital anomaly which is frequently associated with atrioventricular defects. According to its structure it is classified as central, commissural or valvar. Diagnosis by cross-sectional echocardiography was verified in 4 patients with a double orifice mitral valve of the central type. Age of the patients at the time of diagnosis ranged from 3 months to 17 years. Three of them had an associated ostium primum atrial septal defect. The defect was observed in parasternal long-short axis views and subcostal 4 chamber axis views. The parasternal short axis gave information on the central type but subvalvular structures of the double orifice and insertion of the chords in papillary muscle were best observed in parasternal long and subcostal four chamber axis views.
The clinical course and the intracranial pressure (ICP) changes in 66 severe head injury patients presenting bulk enlargement of one cerebral hemisphere within a few hours of trauma have been analyzed. These patients represent 11% of a series of 589 severe head injury cases studied with computerized tomography (CT). Cerebral hemisphere swelling, which was associated with an ipsilateral subdural haematoma of variable extent in 58 patients (88%), or a large epidural haematoma in 5 patients (7%), and occurred as an isolated lesion in 3 patients (4%), carried the highest incidence of uncontrollable intracranial hypertension, the highest mortality rate and the shortest survival period after trauma in the authors' severe head injury series. The high incidence of arterial hypotension and/or hypoxaemia at admission (48% of cases), and the severity of clinical presentation (82%) of patients scored 5 patients or less in the Glasgow Coma Scale, 77% had uni- or bilateral mydriasis and 82% initial ICP above normal limits) correlated with the very poor final outcome (85% mortality). Only one of the 12 patients with normal initial ICP continued to have low pressure throughout the course. High dose thiopental failed to control severe intracranial hypertension in 29 patients (44%) who had a fulminant, malignant course. A transient decrease in ICP elevation was achieved in 17 patients (26%) and a definitive control in 12 patients (18%), among them the 10 survivors in this series. In the authors experience once ICP is controlled, and unless haemodynamic instability compells action to the contrary, barbiturate should not be discontinued until a control CT scan shows complete disappearance of the mass effect.
The clinical and computed tomographic (CT) findings in a series of 161 consecutive patients operated upon for postraumatic extradural hematoma are analyzed. Thirteen (8%) patients had delayed epidural hematoma formation. The overall mortality for the series was 12%, significantly lower than that observed during the prior "angiographic" period at the same unit (30%). Because all but 1 of the deaths occurred among the 66 patients unconscious at the time of operation (27% mortality in this subgroup), the authors sought differential factors between comatose and noncomatose patients at operation. There were no significant differences between these groups in age, sex, mechanism of injury, preoperative course of consciousness (lucid interval or not), or epidural hematoma location and shape. In contrast, significant differences were seen between the two subgroups in trauma-to-operation interval, hematoma volume, CT hematoma density (mixed low-high CT density vs. homogeneous hyperdensity), midline displacement, severity of associated intracranial lesions, and postoperative intracranial pressure (ICP). Patients comatose at operation usually evidenced a more rapid clinical deterioration (a shorter trauma-to-operation interval) and tended to have a large hematoma volume, a higher incidence of mixed CT density clot (hyperacute bleeding), more marked shift of midline structures, more severe associated lesions, and higher postoperative ICP levels.
Mortality due to epidural hematoma is virtually restricted to patients who undergo surgery for that condition while in coma. The authors have analyzed the factors influencing the outcome of 64 patients who underwent epidural hematoma evacuation while in coma. These patients represented 41% of the 156 patients operated on for epidural hematoma at their centers after the introduction of computerized tomography (CT). Eighteen patients (28.1%) died, two (3.1%) became severely disabled, and 44 (68.8%) made a functional recovery. The mortality rate for the entire series was 12%, significantly lower than the 30% rate observed when only angiographic studies were available. A significant correlation was found between the final result and the mechanism of injury, the interval between trauma and surgery, the motor score at operation, the hematoma CT density (homogeneous vs. heterogeneous), and the hematoma volume. The patient's age, the course of consciousness before operation (whether there was a lucid interval), and the clot location did not correlate with the final outcome. The mortality rate was significantly higher in patients operated on within 6 hours or between 6 and 12 hours after injury than in those undergoing surgery 12 to 48 hours after injury. Compared with the patients operated on later, the patients undergoing surgery in the early period were, on the average, older and had more rapidly developing symptoms, more pupillary changes, lower motor scores at surgery, larger hematomas, a higher incidence of mixed CT density clots, more severe associated intracranial lesions, and higher postoperative intracranial pressure (ICP). The mechanism of trauma seems to influence the course of consciousness before and after surgery. Passengers injured in traffic accidents had a lower incidence of a lucid interval and longer postoperative coma than patients with low-speed trauma, suggesting more frequent association of diffuse white matter-shearing injury. The duration of postoperative coma correlated with the morbidity rate in survivors. Forty-eight patients (75%) had one or more associated intracranial lesions, and 70% of these required treatment for elevation of ICP after hematoma evacuation. An ICP of over 35 mm Hg strongly correlated with poor outcome; administration of high-dose barbiturates was the only effective means for lowering ICP in nine of 15 patients who developed severe intracranial hypertension after surgery. This study attempts to identify patients at greater risk for presenting postoperative complications and to define a strategy for control CT scanning and ICP monitoring.
The authors have analyzed the clinical course and intracranial pressure (ICP) changes in 55 severely head-injured patients presenting with bulk enlargement of one cerebral hemisphere within a few hours after trauma. These patients represent 10.5% of a series of 520 patients with severe head injury studied with computerized tomography (CT). Cerebral hemispheric swelling has the highest mortality rate and the shortest survival period after trauma in all series of severe head injury. In this series, it was associated with an ipsilateral subdural hematoma of variable size in 47 patients (85%) or with a large epidural hematoma in five patients (9%); in three patients (5.4%) it occurred as an isolated lesion. Evacuation of an associated extracerebral hematoma, which was performed within 4 hours after injury in only 20% of cases, scarcely changed the patients' preoperative neurological status. The high incidence of arterial hypotension and/or hypoxemia at admission (47% of cases) and the severity of the clinical presentation (82% of patients scored 5 points or less on the Glasgow Coma Scale, 74% had unilateral or bilateral mydriasis, and 80% had an initial ICP above normal) correlated with a very poor final outcome (87% mortality). Only one of the 11 patients with normal initial ICP continued to have normal pressure throughout the course. High-dose thiopental failed to control severe intracranial hypertension in 24 patients (51%) who had a fulminant, malignant course. A transient decrease in ICP elevation was achieved in 15 patients (31.4%) and definitive control in eight patients (17%), among whom were the seven survivors in this series. In the authors' experience, once ICP is controlled, barbiturate administration should not be discontinued until a control CT scan shows complete disappearance of the mass effect.