Search PubMed⌕ Search

Biomedical subjects

F Attie

Publications and source records attributed to F Attie.

At least 55 records · Page 3Linked to original sources
PubMed1970

Ventricular inversion without transposition of the great vessels in situs inversus.

A classification of one type of congenital malformation previously reported (de la Cruz et al., 1967) is based on the integration of an embryological theory for ventricular inversions with the embryological concepts of trunco-conal malformations (de la Cruz and da Rocha, 1956). In that classification we consider that in each situs, either solitus or inversus ventricular inversions may be associated with: (a) normally arranged great vessels (not transposed); (b) transposition of the great vessels; (c) persistent truncus arteriosus. The patients had ventricular inversion without transposition of the great vessels in situs inversus.THE CORRECT ANATOMICAL DIAGNOSIS WAS NOT FORESEEN BUT THE PHYSIOPATHOLOGICAL DIAGNOSIS WAS CORRECT: pulmonary ischaemia associated with septal defects. In one case these facts were substantiated by catheterization and by angiocardiographic findings which led us to advise surgery. Haemodynamically isolated inversion of the ventricles is as severe a malformation as complete (not corrected) transposition of the great vessels. However, an operation of the anastomotic type between a systemic vessel and the narrow pulmonary artery seemed justified in these cases, as a means to convey more blood to the lungs and improve the saturation of the arterial blood. It was unsuccessfully carried out in one patient.The anatomical, radiological, and electrocardiographic features which might aid in the diagnosis are analysed.

Adult↗
PubMedDate not supplied

Ebstein's anomaly. Clinical profile in 174 patients.

The study population consisted of 148 patients who did not undergo surgical treatment and 26 who were operated, most of them diagnosed after the age of 2, with a follow-up from 6 months to 25.3 years. Patients were divided in three groups of clinical deterioration according to their functional class and cardiothoracic index (CTR) long-term follow-up in 148 nonoperated patients showed significant differences for mortality between groups I and III (p < 0.001), and between groups II and III (p < 0.02). Predictors of death included the association among functional class III or IV CTR > or = 65% with either cyanosis or arrhythmias (p < 0.05). The multivariate analysis showed that clinical deterioration (p < 0.0001), CTR (p < 0.0002) and functional class (p < 0.001), were significant for mortality. Kaplan-Meier analysis showed a survival rate of 81% in the overall patients free from surgical treatment. According to Kaplan-Meier analysis, the rate of survival was lower in patients with CTR > or = 65% (63.5%), in patients who had functional class IV (52.5%) and in patients included in group III of clinical deterioration (38.2%). Despite the fact that the association of functional class III or IV plus CTR > or = 65% with either cyanosis or arrhythmias is a good predictor for death, the mortality in patients who had only one of these variables was lower. Patients included in group II of clinical deterioration in stable condition presented long survival with medical treatment. Due to the high mortality rate found in group III, surgical treatment of Ebstein's anomaly must be done before deteriorating into group III. Surgical indication must be done considering the surgical risk of each group according to the experience of the Institution and comparing the rate of surgical mortality with the rate of survival without surgery.

Adolescent↗
PubMedDate not supplied

[Chromosome 22 (22q.11.2) deletion. Etiology of conotruncal heart abnormalities].

Important advances in the diagnosis and treatment of congenital heart disease (CHD) have been made in the past 50 years. Nowadays echocardiogram plays an important role in the diagnosis. This procedure is able to identify a wide range of malformations. Cardiac catheterization is mainly a therapeutic tool, surgery is now performed much earlier because CHDS are diagnosed sometimes before birth or very early in life. All this advances in the diagnosis and treatment of this group of patients, allows them not only a better quality of life but also the possibility of reaching adulthood and having children. The study of the etiology of CHD is a field that has not evolved as fast as the assessment and treatment. Nowadays we have a larger population of adults with CHD. The discovery of a microdeletion of chromosome 22 q11.2 associated with conotruncal cardiac defects, proves a common etiology for clinical phenotypes and conotruncal malformations. In order to identify, which of these patients share the same etiology and presented with this syndrome, we collected a group that shared not only heart defects of the conotruncal type but also specific phenotypic alterations such as broad nasal bridge, nasal dimple, high palate, and digitalization of the first finger among others. The first two patients studied with FISH technique were positive to monosomy of a locus on chromosome 22. Those patients with CHD of conotruncal type should undergo microdeletion testing so genetic counseling can be offered as well as appropriate treatment in areas such as cardiology and developmental psychology.

Child↗
PubMedDate not supplied

[Electrocardiography and echocardiography aspects of hypertrophic myocardiopathy in pediatrics].

Hypertrophic cardiomyopathy (HCM) is a heterogeneous disease, characterized by asymmetric hypertrophy of the left and/or right ventricle with disarray of myocardial fibers. In order to know its clinical and electrocardiographic manifestation in the pediatric age group, we made a retrospective study of 24 cases from 1986 to 1995. There were: 15 girls and 9 boys, with a mean age of 6 years (age range: 1 month to 17 years). Clinical manifestations were dyspnea (71%), syncope (42%) and palpitations (42%). Physical examination disclosed an aortic systolic murmur in all patients, a mitral regurgitation in 42% and physical signs of congestive heart failure in 54% of patients. Chest X rays showed cardiac enlargement in 71% and pulmonary capillary hypertension in 42%. The most frequent ECG abnormalities were: a prolonged time in the intrinsecoid deflection onset on leads corresponding to the affected region, more or less deep and clean Q waves on leads aVF, aVL, V5 and V6, as well as supraventricular and ventricular rhythm disturbances in 11 patients (46%) with and without congestive heart failure. Bidimensional echocardiography confirmed antero-septal hypertrophy in all patients. The mortality rate was 17%. HCM is rare disease in the pediatric age group. Mortality increases when congestive heart failure and arrhythmias are present. Treatment must be individualized in all cases.

Adolescent↗
PubMedDate not supplied

[Congenital cardiopathies in adults].

In the last 30 years, major advances have been made in the diagnosis and treatment of congenital heart disease in children. As a result, many children survive to adulthood, some of them with sequelae. In adults, the most common congenital heart disease is atrial septal defect. The most common causes of cyanotic congenital heart disease are the tetralogy of Fallot and Eisenmenger's syndrome. In this review we discuss the more common acyanotic and cyanotic congenital heart diseases that physicians who care for adults are likely to encounter.

Adult↗
PubMedDate not supplied

[Fast pathway for heart surgery].

The increasing demographic and life expectancy rates, together with the vertiginous technological development during the last two decades, have raised the number of cardiac patients requiring surgical treatment. Therefore, several institutions have been forced to give priority to advanced or more serious cases and to postpone those that do not demand an urgent surgery. This analysis was made from June 15, 1999 to June 15, 2000 and demonstrates the results obtained from maintaining a fast track at the National Institute of Cardiology "Ignacio Chávez". This has favored the practice of surgeries in cases with noncomplex pathologies, null mortality, and low morbidity rates. The analysis also compares results from patients who had been subjected to surgery for the same type of pathologies but following the usual course of admittance and surgery scheduling, proving to be cost-efficient. It also demonstrates the high incidence of inter-auricular communication and the Von Willebrand disease that exist in our environment. Based on the results, we propose to maintain and increase this type of surgeries, addressing their limitations.

Adult↗
PubMedDate not supplied

[Late results of mitral valve replacement in 155 subjects under 16 years of age. Comparative study with 4 prosthesis].

We followed 155 children aged 6 to 16 years who underwent mitral valve replacement for rheumatic heart disease. Group I included 74 patients with Starr-Edwards prostheses and the mean follow-up period was 13 years. Group II was formed by 36 patients with Bjork-Shiley prostheses and a mean follow-up of 7.5 years. Group III consisted of 31 children with dura mater bioprostheses and a mean follow-up of 5.1 years. Finally, group IV included 14 patients followed a mean period of 4.9 years with Hancock xenografts. The four groups of patients were comparable before surgery. The criteria of the New York Heart Association (NYHA) were used to evaluate all patients before and 12 months after surgery. All patients with mechanical prostheses received anticoagulant therapy with acenocumarol from the first postoperative day. The incidence of complications such as thromboembolism, infective endocarditis, prosthetic dysfunction and death were determined at the end of the follow-up. The Fisher's exact test was used for nonparametric analysis and the two-tailed Student's T test for parametric results. All but two patients improved their NYHA clinical status after surgery (p less than 0.0001). Forty eight patients with mechanical prostheses were catheterized 1 to 7 years after the operation. The mean pulmonary arterial systolic pressure was reduced from 58 mmHg to 30 mmHg (p less than 0.001) and the mean pulmonary arterial wedge pressure decreased from 22 mmHg to 11 mmHg (p less than 0.001). There were no significant differences between the groups with mechanical and tissue prostheses in the incidence of thromboembolism and infective endocarditis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMedDate not supplied

[Corrected transposition of the great arteries].

Sixty-nine patients with Corrected Transposition of the Great Arteries were studied, with a mean age of 8.3 years. Forty-nine had atrial situs solitus (SS) and 20 atrial situs inversus (SI). The diagnosis was proved by echocardiography in 28 cases and in all by angiocardiography. In cases with SS, 29 presented ventricular septal defect (VSD), 20 had subvalvular pulmonary stenosis (SVPS), 13 tricuspid regurgitation (TR), 13 atrio-ventricular block (AVB), and seven without associated defects. In cases with SI, 13 had VSD, 14 SVPS, five IT, three AVB, and two without associated defects. The long-term follow-up of the atrioventricular (AV) conduction in 53 cases, ranging between 2 and 37 years (mean 8.3) showed at the beginning of the evaluation 37 cases with normal AV conduction, 14 with first degree AVB, and two with complete AVB. At the end of the follow-up 24 cases had normal AV conduction, 13 first degree, two second degree, four intermittent and 10 complete AVB. The contingency table showed that the possibility of developing changes of the AV conduction was greater in those cases who had an abnormality at the beginning with a relative risk of 2.27 (p = 0.03). Twenty-three cases had SVPS associated with VSD without significant symptoms. During a follow-up ranging from 1 to 20 years (mean 6.5 +/- 5.9), five of these patients were surgically corrected at ages from 6 to 11 years. The remaining cases have had a stable course. At the beginning of the study, 18 cases had TR, and it developed in five more patients during the same follow-up period.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMedDate not supplied

[Recessive autosomal inheritance in Marfan syndrome].

Three brothers 9, 12, and 14 years of age were studied. The three of them fulfilled the diagnostic criteria of Marfan Syndrome that was made based on physical and ophthalmologic examination, these criteria included musculoskeletal abnormalities, subluxation of lens, myopia and aortic aneurysm. The three siblings died due to complications of the aortic aneurysm, which had an early onset as well as the other clinical manifestations of the disease. They were 2 brothers and one sister. There was neither mental or psychomotor retardation, nor metabolic disease in any of the patients. Family history did not show any clinical manifestation of the disease in parents or grandparents. We propose that the hereditary pattern is recessive autosomic instead of dominant autosomic given the features of these cases and their history.

Child↗
PubMedDate not supplied

[Primary pulmonary arterial hypertension in children under 2 years of age].

A retrospective analysis of 18 patients under two years of age who suffered from primary pulmonary hypertension was made between july 1982 november 1988 in order to define their clinical course and prognosis. Perinatal history was irrelevant. In 13 patients diagnosis was established before the first year of life and in 8 of them in the neonatal period. All patients presented the same symptomatology: Cyanosis, dyspnea and/or repeated respiratory infections. The electrocardiogram showed various degrees of enlargement of the right atrium and right ventricle as well as an uneven ST segment in the precordial leads; these could not be related to the systolic or diastolic pressures of the right ventricle. In the chest roentgenogram the most important and constant feature was the heart enlargement. Cardiac catheterization showed a mean pulmonary systolic arterial pressure of 64.46 mmHg and a mean pulmonary arterial resistance of 6.4 U/m.2 These parameters could not be related to a bad prognosis. Three patients were given vasodilators (hydralazine) with positive clinical and echocardiographic results. Five patients died (27%) two months after the diagnosis was made and three months after the onset of symptomatology, four of them died due to congestive heart failure and one had sudden death. This study shows the early onset of the disease as well as its short term high mortality.

Age Factors↗
PubMedDate not supplied

[Color doppler-echocardiography study of the triatrial heart].

Two-dimensional echocardiography has proved its utility in the diagnosis of cor triatriatum sinister by visualizing the defect and its relation to the other structures. Recently pulsed and continuous wave Doppler have been used to determine the degree of obstruction of the membrane. In this study we present three patients with ages six, eight and fourteen months respectively, all with a clinical history of growth retardation, respiratory infections and dyspnea. The patients were studied with two-dimensional and color flow Doppler echocardiography. In all, a membrane was visualized, which divided the left atrium in two chambers, a superior one which received the pulmonary veins and an inferior in continuity with the mitral valve and the left atrial appendage and with color flow Doppler the absence of obstruction at this level was demonstrated. In one patient besides the anomaly of cor triatriatum complete transposition of great arteries with pulmonary stenosis was diagnosed and in another one, patent ductus arteriosus and ventricular septal defect. Diagnosis were confirmed by hemodynamic studies. It is concluded that the color flow Doppler is a diagnostic method of great utility because it permits to obtain more information which complements conventional echocardiography.

Cor Triatriatum↗
PubMedDate not supplied

[Surgical alternatives in the treatment of double-outlet right ventricle].

Double-outlet right ventricle (DORV) is a type of ventriculo-arterial connection, in which both great arteries are connected to the morphologically right ventricle in at least 50% of their diameter. The relation of the great arteries with the ventricular septal defect (VSD) and the pulmonary stenosis, are important anatomical aspects in the election of the surgical technique. In this study we describe four patients with DORV, in whom different surgical techniques were used according to the specific anatomy of each case. Two patients had subaortic restrictive VSD, with subpulmonary stenosis in one of them. In the other two patients the VSD was subpulmonary with the aorta anterior and to the left, in one of these existed moreover a severe mixed pulmonary stenosis. It is concluded, that with different surgical techniques according to the specific anatomy of each patient, good results can be obtained in the surgical treatment of these complex congenital malformations.

Child↗
PubMedDate not supplied

[Tetralogy of Fallot with abnormal coronary arteries].

Between 1980 and 1989 nine patients with Tetralogy of Fallot with abnormal coronary artery underwent surgical treatment. In all but one, the abnormal coronary artery was identified at surgery. Seven (group I) had the anterior descending coronary artery arising from the right coronary artery; one (group II) with had the right coronary artery arising from the left coronary artery and finally, the only case of the group III had left coronary artery arising from the main pulmonary artery. Systemic-pulmonary anastomosis was done in two cases of the Group I, in one due to severe hypoplasia of the pulmonary ring and main pulmonary artery and in the other due to the anomaly of the coronary artery. In the case of the Group II the right coronary artery was accidentally cut and the patient developed right ventricular infarction and died. All surviving are in NYHA functional class I. Nowadays, the preoperative evaluation of Tetralogy of Fallot, can be done by echocardiography despite the possibility of its association with abnormal coronary artery. In special cases aortography or arterial coronarography should be considered.

Anastomosis, Surgical↗
PubMedDate not supplied

Results from an international survey of Kawasaki disease in 1979-82.

Kawasaki disease is a febrile illness of unknown etiology affecting mainly children younger than five years, 20% of whom develop coronary artery aneurysms. The disease was first reported in Japan, but case reports have come from several countries; epidemics apparently occur every two to three years. A committee of the International Society and Federation of Cardiology (ISFC) collaborated on an international, retrospective survey in mid-1983 through 1984, asking about Kawasaki disease cases seen in 1979-82 and their outcomes. Responses were received from 53 countries; reports from 20 countries indicated that no cases were seen in the years surveyed. Kawasaki disease did not always follow the Japanese pattern. An indication of 'epidemic' increases was found in some years in some countries. Recognition of Kawasaki disease varies considerably, but further educational efforts are likely to uncover additional cases, and intensive epidemiological efforts would be possible in a number of countries.

Child, Preschool↗
PubMedDate not supplied

[Malposition of the great arteries. Is this term a valid one?].

We present four cases of a congenital heart malformation classically known as malposition of the great arteries. This term means there are concordant ventriculo-arterial connections with abnormal spatial relations of the aortic and the pulmonary arteries, in which the former is anterior relative to the latter, and either to the right or to the left, according to the position of the corresponding ventricle. This anomaly can be found in any type of atrial situs, notwithstanding the type or mode of atrioventricular connection, as in the cases we studied. If we consider that this anomaly depends on the associated lesions in order to produce physiological heart alterations, we think that the term of malposition of the great arteries is not valid, since in the anatomic diagnosis of congenital heart disease it is important to consider the connections between atria, ventricles and arteries, the spatial relations between them being of second importance.

Adolescent↗
PubMedDate not supplied

[Scimitar syndrome].

We studied 15 cases of the scimitar syndrome. The diagnosis was suspected in 87% of the patients by x-rays studies. Bronchography is useful in order to detect bronchial anomalies, such as hypoplasia or agenesis of the medial lobe which is responsible for the left isomerism. The hemodynamic study is important in symptomatic patients in order to demonstrate associated congenital heart disease as well as the degree of pulmonary hypertension. Angiography is fundamental in determining the anomalous venous drainage of the right lung, whether complete or partial, and in demonstrating the exact site of the connection which may be to the right atrium or more frequently to the inferior vena cava above or below the diaphragm. There are variant forms in so far as the pathway, extension and connection of the anomalous venous trunk which gives rise to the scimitar sign. This syndrome is due to a persistent connection of the right pulmonary veins to the proximal portion of the right vitelin vein which normally forms the suprahepatic segment of the inferior vena cava. Asymptomatic patients should have medical treatment symptomatic ones with an associated congenital heart disease and moderate pulmonary hypertension must be subject to surgery.

Adolescent↗