Screening for abdominal aortic aneurysms

OBJECTIVE AND METHODS: Screening for abdominal aortic aneurysms may be useful to decrease mortality related to rupture. We conducted a study to assess the prevalence of abdominal aortic aneurysms in southern Brazil and to define risk factors associated with high prevalence of this disorder. The screening was conducted using abdominal ultrasound. Three groups were studied: Group 1 - cardiology clinic patients; Group 2 - individuals with severe ischemic disease and previous coronary surgery, or important lesions on cardiac catheterism; Group 3 - individuals without cardiac disease selected from the general population. All individuals were male and older than 54 years of age. The ultrasonographic diagnosis of aneurysm was based on an anteroposterior abdominal aorta diameter of 3 cm, or on an abdominal aorta diameter 0.5 cm greater than that of the supra-renal aorta. RESULTS: A total of 2.281 people were screened for abdominal aortic aneurysms in all groups: Group 1 - 768 individuals, Group 2 - 501 individuals, and Group 3 - 1012 individuals. The prevalence of aneurysms was 4.3%, 6.8% and 1.7%, respectively. Age and cigarette smoking were significantly associated with increased prevalence of aneurysms, as was the diagnosis of peripheral artery disease. DISCUSSION: We concluded that screening may be an important tool to prevent the mortality associated with abdominal aortic aneurysms surgery. Additionally, the cost of screening can be decreased if only individuals presenting significant risk factors, such as coronary and peripheral artery disease, smokers and relatives of aneurysm patients, are examined.

Laparoscopic versus open splenectomy in the management of hematologic diseases

Splenectomy is the best available treatment for severe forms of hereditary spherocytosis, idiopathic thrombocytopenic purpura, and other hematologic conditions when these prove refractory to conservative management. It has been employed for many decades with low mortality and favorable remission rates. The use of laparoscopic splenectomy in recent years has been rapidly and even enthusiastically adopted in this field. However, the exact role of laparoscopic versus open surgery for hematologic diseases is still debated. In this study of 58 adult patients, laparoscopic procedures were compared with conventional splenectomies for similar indications. METHODS: All patients were operated on within an 8-year period. Subjects underwent similar procedures under the supervision of the same surgical school and were compared regarding age, gender, body mass index, and diagnosis. Laparoscopically managed cases (Group I, n = 30) were prospectively followed according to a written protocol, whereas the same investigation was retrospectively done with regard to traditional laparotomy (Group II, n = 28). Methods included general and demographic findings, duration and technical steps of operation, blood loss, weight of spleen, need for conversion (in minimally invasive subjects), intraoperative and postoperative complications, time until realimentation, postoperative hospitalization, mortality, and late follow-up including recurrence rate. RESULTS: Idiopathic thrombocytopenic purpura was the surgical indication in over 50% of the patients in both groups, but familial spherocytosis, thalassemia, myelodysplasia, and lymphomas were also represented in this series. Laparoscopic procedures took more time to perform (P = 0.004), and postoperative hospitalization was 2 days shorter, but this difference was not statistically significant. Postoperative hematocrit and volume of blood transfusions was equivalent, although the laparoscopic cases had a somewhat lower preoperative hematocrit (NS) and displayed better recovery for this measurement (P = 0.03). More patients in Group I were able to accept oral food on the first day than subjects undergoing conventional operations (P < 0.05). Relatively few conversions were necessary during the minimally invasive surgeries (13.3%), and postoperative early and late complications as well as recurrences occurred in similar proportions. Also, the mean weight of the spleen was not statistically different between the groups, although there was a marked numerical tendency toward larger masses in conventional procedures. No spleen in Group I exceeded 2.0 kg, whereas in Group II values up to 4.0 kg occurred, and the mean weight was 50% higher in the latter group. CONCLUSIONS: 1) Minimally invasive splenectomy was essentially comparable to open surgery with regard to safety, efficacy, and late results; 2) Advantages concerning shorter postoperative hospitalization could not be shown, despite earlier food intake and a non-significant tendency toward earlier discharge; 3) This new modality should be considered an option in cases of hematologic conditions whenever the spleen is not hugely enlarged.

Involvement of C4 allotypes in the pathogenesis of human diseases

The complement system is an important humoral defense mechanism that plays a relevant role against microbial agents, inflammatory response control, and immunocomplex clearance. Classical complement pathway activation is antibody-dependent. The C4 component participates in the initial step of activation, and C4 expression is determined by 2 pairs of allotypes: C4A and C4B. Deficiencies in C4 allotypes have been associated with several diseases. The aim of the present review is evaluate the reported data in the literature regarding specific C4A and C4B deficiencies and characterize their clinical relevance. We searched the MEDLINE and LILACS databases. Papers referring to total C4 deficiency without allotype evaluation and case reports of primary C4 deficiency were not included. Deficiencies in C4 allotypes have been associated with Mycobacterium leprae infection, erythema nodosum, systemic sclerosis with anti-topoisomerase I antibodies, intermediate congenital adrenal hyperplasia with DR5 genotype, diabetes mellitus type 1 with DR3,4 genotype, and diabetes mellitus with antibodies against islet cells. C4 allotype deficiency is also related to C4B deficiency and autoimmune-associated diseases, such as systemic lupus erythematosus, or diseases with an autoimmune component, such as autism. Some reports associate C4A with thyroiditis after delivery as well as limited and systemic sclerosis without anti-topoisomerase I antibodies. However, the studies with C4A and C4B have been concentrated in isolated populations, and some of the studies could not be reproduced by other authors.

A questionnaire to evaluate the impact of chronic diseases: validated translation and Illness Effects Questionnaire (IEQ) reliability study

INTRODUCTION: Patients' perception about their health condition, mainly involving chronic diseases, has been investigated in many studies and it has been associated to depression, compliance with the treatment, quality of life and prognosis. The Illness Effects Questionnaire (IEQ) is a tool which makes the standardized evaluation of patients' perception about their illness possible, so that it is brief and accessible to the different clinical settings. This work aims to begin the transcultural adaptation of the IEQ to Brazil through the validated translation and the reliability study. METHODS: The back-translation method and the test-retest reliability study were used in a sample of 30 adult patients under chronic hemodialysis. The reliability indexes were estimated using the Pearson, Spearman, Weighted Kappa and Cronbach's alpha coefficients. RESULTS: The semantic equivalence was reached through the validated translation. In this study, the reliability indexes obtained were respectively: 0.85 and 0.75 (p < 0.001); 0.68 and 0.92 (p < 0.0001). DISCUSSION: The reliability indexes obtained attest to the stability of responses in both evaluations. Additional procedures are necessary for the transcultural adaptation of the IEQ to be complete. CONCLUSION: The results indicate the translation validity and the reliability of the Brazilian version of the IEQ for the sample studied.

Aortic stenosis. Gender influence on left ventricular geometry and function in patients under 70 years of age

OBJECTIVE: To verify if adaptive left ventricle (LV) characteristics are also present in individuals under 70 years of age with severe aortic stenosis (AS). METHODS: The study comprised 40 consecutive patients under 70 years of age with AS and no associated coronary artery disease, referred for valve surgery. Out of the 40 patients, 22 were men and 18 women, and the mean age was 49.8±14.3 years. Cardiac symptoms, presence of systemic hypertension (SH), functional class according to the New York Heart Association (NYHA), and valve lesion etiology were considered. LV cavity dimensions, ejection fraction (EF), fractional shortening (FS), mass (MS), and relative diastolic thickness (RDT) were examined by Doppler echocardiography. RESULTS: Fourteen (63.6%) men and 11 (61.6%) women were classified as NYHA class III/IV (p=0.70). There was no difference in the frequency of angina, syncope or dyspnea between genders. The incidence of SH was greater in women than in men (10 versus 2, p=0.0044). Women had a smaller LV end-diastolic diameter index (32.1±6.5 x 36.5±5.3mm/m², p=0.027), LV end-systolic diameter index (19.9±5.9 x 26.5±6.4mm/m², p=0.0022) and LV mass index (MS) (211.4±71.1 x 270.9±74.9g/m², p=0.017) when compared with men. EF (66.2±13.4 x 52.0±14.6%, p=0.0032), FS (37.6±10.7 x 27.9±9.6%, p=0.0046) and RDT (0.58±0.22 x 0.44±0.09, p=0.0095) were significantly greater in women than in men. CONCLUSION: It is the patient gender rather than age that influences left ventricular adaptive response to AS.

Dynamic three-dimensional reconstruction of the heart by transesophageal echocardiography

OBJECTIVE: To evaluate echocardiography accuracy in performing and obtaining images for dynamical three-dimensional (3D) reconstruction. METHODS: Three-dimensional (3D) image reconstruction was obtained in 20 consecutive patients who underwent transesophageal echocardiography. A multiplanar 5 MHz transducer was used for 3D reconstruction. RESULTS: Twenty patients were studied consecutively. The following cardiac diseases were present: valvar prostheses-6 (2 mitral, 2 aortic and 2 mitral and aortic); mitral valve prolapse- 3; mitral and aortic disease - 2; aortic valve disease- 5; congenital heart disease- 3 (2 atrial septal defect- ASD - and 1 transposition of the great arteries -TGA); arteriovenous fistula- 1. In 7 patients, color Doppler was also obtained and used for 3D flow reconstruction. Twenty five cardiac structures were acquired and 60 reconstructions generated (28 of mitral valves, 14 of aortic valves, 4 of mitral prostheses, 7 of aortic prostheses and 7 of the ASD). Fifty five of 60 (91.6%) reconstructions were considered of good quality by 2 independent observers. The 11 reconstructed mitral valves/prostheses and the 2 reconstructed ASDs provided more anatomical information than two dimensional echocardiography (2DE) alone. CONCLUSION: 3D echocardiography using a transesophageal transducer is a feasible technique, which improves detection of anatomical details of cardiac structures, particularly of the mitral valve and atrial septum.

Aortic valve assessment. Anatomical study of 100 healthy human hearts

PURPOSE: To assess anatomical characteristics of the aortic valve, so that they may be useful in diagnostic situations and surgical treatment. METHODS: The study analyzed 100 healthy fixed human hearts; 84% of them obtained from males, 61% of them from Caucasian individuals. The ages of the individuals ranged from 9 to 86 years (mean 30±15.5 years). The characteristics assessed related to age, sex, and race were the following: number and height of the cusps, size of the lunulae, internal and external intercommissural distance, position of the coronary ostium in relation to the aortic valve, position of the ventricular septum in relation to the aortic valve, thickness of the cusps. RESULTS: All hearts assessed had a tricuspidal aortic valve. In regard to the height of the cusps and size of the lunula, the left coronary cusp was larger, followed by the right coronary cusp and the noncoronary cusp. The internal and external intercommissural distances had mean values of 24.6±5.7mm and 19.7±7mm, respectively. In regard to the position of the coronary ostia, in one heart two ostia emerged from the left coronary sinus, and in another, the ostium was supracommissural. The mean diameter of the aorta was 21.8±3.6mm, and there were no significant sexual or racial differences, but the diameter increased progressively with the increase in age. The thickness of the cusps did not show any significant difference in the 3 points assessed. CONCLUSION: The aortic valve annulus did not show a perfect circumference, with some variations in the measurements of the annulus, in the cusps and in the relation with the ventricular septum.

Anomalous origin of the left coronary artery from the right pulmonary artery with intramural aortic trajectory. Clinicosurgical diagnostic implications

OBJECTIVE: Anomalous origin of the left coronary artery from the right pulmonary artery (AOLCARPA), is a rare entity that is usually associated with other defects. Of the 20 cases of AOLCARPA reported in the literature, 14 (70%) had associations. We describe four patients with AOLCARPA without associated defects, but with a peculiar intramural aortic trajectory. METHODS: Fifty-five patients with anomalous origin of the left coronary artery were operated upon at INCOR-FMUSP. Four of the patients had the anomalous origin from the right pulmonary artery (RPA) without associated defects but with intramural aortic trajectory. Clinical and laboratory examinations were analyzed, as well as surgical findings. RESULTS: All patients had congestive heart failure (CHF) and 3 also had angina pectoris. Two patients had a murmur of mitral regurgitation, signs of myocardial infarction on the ECG and cardiomegaly. The shortening fraction varied from 9% to 23%. The hemodynamic study confirmed the diagnosis of anomalous origin of the coronary artery, but the intramural trajectory and the origin from the RPA were established only at surgery. In 3 patients, the technique of side-to-side anastomosis was performed with a good outcome. One patient, who underwent end-to-side anastomosis, died 6 months after the surgery. CONCLUSION: Association with other defects usually occurs in the AOLCARPA, and the intramural aortic trajectory is difficult to clinically diagnose but easy to surgically correct.

Critical aortic stenosis in the neonate. Clinical assessment and surgical outcome in 18 patients

OBJECTIVE - To assess neonates with aortic stenosis with early decompensation operated upon. (LCO) (CHF). METHODS - A and retrospective study analyzing 6 neonates with LCO, group I (GI), and 12 neonates with CHF, group II (GII). Clinical radiographic, electrocardiographic and echocardiographic findings also provided comparative bases for the study, as did surgical and evolutional findings. RESULTS - The mean ages at hospitalization and surgery (p = 0.0031) were 14.3 and 14.8 days in GI and 35.4 and 42.8 days in GII, respectively. Cardiac murmurs were more intense in GII (p = 0.0220). The aortic ring was smaller in GI (8.0 ± 2.5mm) as compared to GII (11.4±1.4mm) (p = 0.2882). Ventricular function was reduced to 18±5.5% and 33.3±7.6% in GI and GII, respectively (p = 0.0162). Aortic atresia, however, was present only in 2 neonates in GI. Five of 6 patients in GI died but all patients in GII survived (p=0.0007). In the latter group, 84.6% of the patients were in functional class I (FC-I) in the long-term follow-up, with moderate residual lesions in 6 neonates, discrete residual lesions in 4, and reoperation in 2. CONCLUSION - Aortic stenosis is a severe anomaly of the neonate, whose immediate evolution depends on the pre-operative anatomic and functional findings, and the late evolution essentially depends on the anatomic features of the valve.

Epidemiological characteristics of congenital heart diseases in Londrina, Paraná South Brazil

OBJECTIVE: To determine the prevalence and other epidemiological characteristics of congenital heart diseases. METHODS: A retrospective population based study of children who were born in Londrina, from January '89 to December '98 (80,262 live births). Diagnoses were confirmed through autopsy, surgery, catheterization, or echocardiography. RESULTS: A total of 441 patients was as certained what corresponds to a prevalence of 5.494:1,000 live births. Ventricular septal defect was the commonest lesion. A small number of transpositions of the great vessels and of left ventricular hypoplasia was observed. A high propation of ventricular septal defect (28.3%) and atrioventricular septal defects (8.1%) occurred. Fifty-one (11.35%) affected children had syndromic diseases and 52 (12.01%) children had nonsyndromic anomalies. CONCLUSION: The prevalence of congenital heart diseases in Londrina is in accordance with that of other regions of the globe. This prevalence also may reflect the reality in the southern region of Brazil, because population characteristics are very similar in the 3 southernmost Brazilian states.