Association of pulmonary atresia with intact ventricular septum and aortic valve stenosis. Prenatal diagnosis

A rare association of pulmonary atresia with an intact septum was diagnosed through echocardiography in a fetus 32 weeks of gestational age. The diagnosis was later confirmed by echocardiography of the newborn infant and further on autopsy. The aortic valve was bicuspid with a pressure gradient of 81mmHg, and the right ventricle was hypoplastic, as were the pulmonary trunk and arteries, and the blood flow was totally dependent on the ductus arteriosus.

Influence of the echocardiographic score and not of the previous surgical mitral commissurotomy on the outcome of percutaneous mitral balloon valvuloplasty

OBJECTIVE: To evaluate prior mitral surgical commissurotomy and echocardiographic score influence on the outcomes and complications of percutaneous mitral balloon valvuloplasty. METHODS: We performed 459 complete mitral valvuloplasty procedures. Four hundred thirteen were primary valvuloplasty and 46 were in patients who had undergone prior surgical commissurotomy. The prior commissurotomy group was older, had higher echo scores, and a tendency toward a higher percentage of atrial fibrillation. RESULTS: When the groups were compared with each other, no differences were found in pre- and postprocedure mean pulmonary artery pressure, mean mitral gradient, mitral valve area, and mitral regurgitation . Because we found no significant differences, we subdivided the entire group based on echo scores, those with echo scores <=8 and those with echo scores >8 the mitral valve area being higher in the <=8 echo score group 2.06±0.42 versus 1.90±0.40cm² (p=0.0090) in the >8 echo score group. CONCLUSION: Dividing the groups based on echo score revealed that the higher echo score group had smaller mitral valve areas postvalvuloplasty.

Some predictors of cardiovascular mortality among the elderly population of Botucatu (SP)

OBJECTIVE: To detect factors associated with cardiovascular mortality in the elderly of Botucatu. METHODS: We evaluated 29 variables of interest in a cohort of patients aged ³60 using data from a survey conducted between 1983/84. The elderly cohort was analyzed in 1992 to detect the occurrence of cardiovascular deaths. Survival analysis was performed using the Kaplan-Meier method, the log-rank test, and Cox regression analysis. Three models were adapted for each group of variables, and a final model was chosen from those variables selected from each group. RESULTS: We identified predictor for cardiovascular death according to age for elderly males not supporting the family, not possessing a vehicle, and previous cardiovascular disease. In elderly females, the predictor variables were previous cardiovascular disease and diabetes mellitus. CONCLUSION: Socioeconomic indicators (family heading and vehicle ownerrship) may be added to well stabilished medical factors (diabete mellitus and hypertension to select target groups for programs intended to reduce deaths due to cardiovascular diseases in elderly people.

Retrospective Study of the Survival of Patients who Underwent Cardiopulmonary Resuscitation in an Intensive Care Unit

OBJECTIVE: To evaluate clinical and evolutive characteristics of patients admitted in an intensive care unit after cardiopulmonary resuscitation, identifying prognostic survival factors.METHODS: A retrospective study of 136 patients admitted between 1995 and 1999 to an intensive care unit, evaluating clinical conditions, mechanisms and causes of cardiopulmonary arrest, and their relation to hospital mortality.RESULTS: A 76% mortality rate independent of age and sex was observed. Asystole was the most frequent mechanism of death, and seen in isolation pulmonary arrest was the least frequent. Cardiac failure, need for mechanical ventilation, cirrhosis and previous stroke were clinically significant (p<0.01) death factors.CONCLUSION: Prognostic factors supplement the doctor's decision as to whether or not a patient will benefit from cardiopulmonary resuscitation.

Fibrinolytic therapy for thrombosis in cardiac valvular prosthesis short and long term results

OBJECTIVE: To assess the short- and long-term results of the use of streptokinase (SK) for the treatment of thromboses in cardiac valvular prostheses. METHODS: Seventeen patients with cardiac prosthetic thrombosis diagnosed by clinical, echocardiographic, and radioscopic findings underwent fibrinolytic treatment with a streptokinase bolus of 250,000 U followed by 100.000 U/hour. Short- and long-term results were assessed by radioscopy and echocardiography. RESULTS: Of the 17 patients, 12 had mechanical double-disk prostheses (4 aortic, 6 mitral, 2 tricuspid), 4 had single-disk prostheses (2 aortic, 1 mitral, and 1 tricuspid), and 1 had a tricuspid bioprosthesis. The success rate was 64.8%, the partial success rate was 17.6%, and the nonsuccess rate was 17.6%. All patients with a double-disk prosthesis responded, completely or partially, to the treatment. None of the patients with a single-disk prosthesis had complete resolution of the thrombosis. The time of streptokinase infusion ranged from 6 to 80 hours (mean of 56 h). The mortality rate due to the use of streptokinase was 5.8% and was secondary to cerebral bleeding. During streptokinase infusion, 3 (17.6%) embolic episodes occurred as follows: 1 cerebral, 1 peripheral, and 1 coronary. The rethrombosis index was 33% in a mean follow-up of 42 months. CONCLUSION: The use of fibrinolytic agents was effective and relatively safe in patients with primary thrombosis of a double-disk prosthesis. A fatal hemorrhagic complication occurred in 1 (5.8%) patient, and embolic complications occurred in 3 (17.6%) patients. In a mean 42-month follow-up, 67% of the patients were free from rethrombosis.

Validation of the Killip-Kimball Classification and Late Mortality after Acute Myocardial Infarction

Background: The classification or index of heart failure severity in patients with acute myocardial infarction (AMI) was proposed by Killip and Kimball aiming at assessing the risk of in-hospital death and the potential benefit of specific management of care provided in Coronary Care Units (CCU) during the decade of 60. Objective: To validate the risk stratification of Killip classification in the long-term mortality and compare the prognostic value in patients with non-ST-segment elevation MI (NSTEMI) relative to patients with ST-segment elevation MI (STEMI), in the era of reperfusion and modern antithrombotic therapies. Methods: We evaluated 1906 patients with documented AMI and admitted to the CCU, from 1995 to 2011, with a mean follow-up of 05 years to assess total mortality. Kaplan-Meier (KM) curves were developed for comparison between survival distributions according to Killip class and NSTEMI versus STEMI. Cox proportional regression models were developed to determine the independent association between Killip class and mortality, with sensitivity analyses based on type of AMI. Results: The proportions of deaths and the KM survival distributions were significantly different across Killip class >1 (p <0.001) and with a similar pattern between patients with NSTEMI and STEMI. Cox models identified the Killip classification as a significant, sustained, consistent predictor and independent of relevant covariables (Wald χ2 16.5 [p = 0.001], NSTEMI) and (Wald χ2 11.9 [p = 0.008], STEMI). Conclusion: The Killip and Kimball classification performs relevant prognostic role in mortality at mean follow-up of 05 years post-AMI, with a similar pattern between NSTEMI and STEMI patients.

Debranching Solutions in Endografting for Complex Thoracic Aortic Dissections

Background: Conventional surgical repair of thoracic aortic dissections is a challenge due to mortality and morbidity risks. Objectives: We analyzed our experience in hybrid aortic arch repair for complex dissections of the aortic arch. Methods: Between 2009 and 2013, 18 patients (the mean age of 67 ± 8 years-old) underwent hybrid aortic arch repair. The procedural strategy was determined on the individual patient. Results: Thirteen patients had type I repair using trifurcation and another patient with bifurcation graft. Two patients had type II repair with replacement of the ascending aorta. Two patients received extra-anatomic bypass grafting to left carotid artery allowing covering of zone 1. Stent graft deployment rate was 100%. No patients experienced stroke. One patient with total debranching of the aortic arch following an acute dissection of the proximal arch expired 3 months after TEVAR due to heart failure. There were no early to midterm endoleaks. The median follow-up was 20 ± 8 months with patency rate of 100%. Conclusion: Various debranching solutions for different complex scenarios of the aortic arch serve as less invasive procedures than conventional open surgery enabling safe and effective treatment of this highly selected subgroup of patients with complex aortic pathologies.

Survival and Predictive Factors of Lethality in Hemodyalisis: D/I Polymorphism of The Angiotensin I-Converting Enzyme and of the Angiotensinogen M235T Genes

Background: End-stage kidney disease patients continue to have markedly increased cardiovascular disease morbidity and mortality. Analysis of genetic factors connected with the renin-angiotensin system that influences the survival of the patients with end-stage kidney disease supports the ongoing search for improved outcomes. Objective: To assess survival and its association with the polymorphism of renin-angiotensin system genes: angiotensin I-converting enzyme insertion/deletion and angiotensinogen M235T in patients undergoing hemodialysis. Methods: Our study was designed to examine the role of renin-angiotensin system genes. It was an observational study. We analyzed 473 chronic hemodialysis patients in four dialysis units in the state of Rio de Janeiro. Survival rates were calculated by the Kaplan-Meier method and the differences between the curves were evaluated by Tarone-Ware, Peto-Prentice, and log rank tests. We also used logistic regression analysis and the multinomial model. A p value ≤ 0.05 was considered to be statistically significant. The local medical ethics committee gave their approval to this study. Results: The mean age of patients was 45.8 years old. The overall survival rate was 48% at 11 years. The major causes of death were cardiovascular diseases (34%) and infections (15%). Logistic regression analysis found statistical significance for the following variables: age (p = 0.000038), TT angiotensinogen (p = 0.08261), and family income greater than five times the minimum wage (p = 0.03089), the latter being a protective factor. Conclusions: The survival of hemodialysis patients is likely to be influenced by the TT of the angiotensinogen M235T gene.

Assessment of Myocardial Infarction by Cardiac Magnetic Resonance Imaging and Long-Term Mortality

Background: Cardiac magnetic resonance imaging provides detailed anatomical information on infarction. However, few studies have investigated the association of these data with mortality after acute myocardial infarction. Objective: To study the association between data regarding infarct size and anatomy, as obtained from cardiac magnetic resonance imaging after acute myocardial infarction, and long-term mortality. Methods: A total of 1959 reports of “infarct size” were identified in 7119 cardiac magnetic resonance imaging studies, of which 420 had clinical and laboratory confirmation of previous myocardial infarction. The variables studied were the classic risk factors – left ventricular ejection fraction, categorized ventricular function, and location of acute myocardial infarction. Infarct size and acute myocardial infarction extent and transmurality were analyzed alone and together, using the variable named “MET-AMI”. The statistical analysis was carried out using the elastic net regularization, with the Cox model and survival trees. Results: The mean age was 62.3 ± 12 years, and 77.3% were males. During the mean follow-up of 6.4 ± 2.9 years, there were 76 deaths (18.1%). Serum creatinine, diabetes mellitus and previous myocardial infarction were independently associated with mortality. Age was the main explanatory factor. The cardiac magnetic resonance imaging variables independently associated with mortality were transmurality of acute myocardial infarction (p = 0.047), ventricular dysfunction (p = 0.0005) and infarcted size (p = 0.0005); the latter was the main explanatory variable for ischemic heart disease death. The MET-AMI variable was the most strongly associated with risk of ischemic heart disease death (HR: 16.04; 95%CI: 2.64-97.5; p = 0.003). Conclusion: The anatomical data of infarction, obtained from cardiac magnetic resonance imaging after acute myocardial infarction, were independently associated with long-term mortality, especially for ischemic heart disease death.

One-year Mortality after an Acute Coronary Event and its Clinical Predictors: The ERICO Study

Background:Information about post-acute coronary syndrome (ACS) survival have been mostly short-term findings or based on specialized, cardiology referral centers.Objectives:To describe one-year case-fatality rates in the Strategy of Registry of Acute Coronary Syndrome (ERICO) cohort, and to study baseline characteristics as predictors.Methods:We analyzed data from 964 ERICO participants enrolled from February 2009 to December 2012. We assessed vital status by telephone contact and official death certificate searches. The cause of death was determined according to the official death certificates. We used log-rank tests to compare the probabilities of survival across subgroups. We built crude and adjusted (for age, sex and ACS subtype) Cox regression models to study if the ACS subtype or baseline characteristics were independent predictors of all-cause or cardiovascular mortality.Results:We identified 110 deaths in the cohort (case-fatality rate, 12.0%). Age [Hazard ratio (HR) = 2.04 per 10 year increase; 95% confidence interval (95%CI) = 1.75–2.38], non-ST elevation myocardial infarction (HR = 3.82 ; 95%CI = 2.21–6.60) or ST elevation myocardial infarction (HR = 2.59; 95%CI = 1.38–4.89) diagnoses, and diabetes (HR = 1.78; 95%CI = 1.20‑2.63) were significant risk factors for all-cause mortality in the adjusted models. We found similar results for cardiovascular mortality. A previous coronary artery disease diagnosis was also an independent predictor of all-cause mortality (HR = 1.61; 95%CI = 1.04–2.50), but not for cardiovascular mortality.Conclusion:We found an overall one-year mortality rate of 12.0% in a sample of post-ACS patients in a community, non-specialized hospital in São Paulo, Brazil. Age, ACS subtype, and diabetes were independent predictors of poor one‑year survival for overall and cardiovascular-related causes.

Subclinical hyperthyroidism and the risk of coronary heart disease and mortality.

BACKGROUND: Data from prospective cohort studies regarding the association between subclinical hyperthyroidism and cardiovascular outcomes are conflicting.We aimed to assess the risks of total and coronary heart disease (CHD) mortality, CHD events, and atrial fibrillation (AF) associated with endogenous subclinical hyperthyroidism among all available large prospective cohorts. METHODS: Individual data on 52 674 participants were pooled from 10 cohorts. Coronary heart disease events were analyzed in 22 437 participants from 6 cohorts with available data, and incident AF was analyzed in 8711 participants from 5 cohorts. Euthyroidism was defined as thyrotropin level between 0.45 and 4.49 mIU/L and endogenous subclinical hyperthyroidism as thyrotropin level lower than 0.45 mIU/L with normal free thyroxine levels, after excluding those receiving thyroid-altering medications. RESULTS: Of 52 674 participants, 2188 (4.2%) had subclinical hyperthyroidism. During follow-up, 8527 participants died (including 1896 from CHD), 3653 of 22 437 had CHD events, and 785 of 8711 developed AF. In age- and sex-adjusted analyses, subclinical hyperthyroidism was associated with increased total mortality (hazard ratio[HR], 1.24, 95% CI, 1.06-1.46), CHD mortality (HR,1.29; 95% CI, 1.02-1.62), CHD events (HR, 1.21; 95%CI, 0.99-1.46), and AF (HR, 1.68; 95% CI, 1.16-2.43).Risks did not differ significantly by age, sex, or preexisting cardiovascular disease and were similar after further adjustment for cardiovascular risk factors, with attributable risk of 14.5% for total mortality to 41.5% forAF in those with subclinical hyperthyroidism. Risks for CHD mortality and AF (but not other outcomes) were higher for thyrotropin level lower than 0.10 mIU/L compared with thyrotropin level between 0.10 and 0.44 mIU/L(for both, P value for trend, .03). CONCLUSION: Endogenous subclinical hyperthyroidism is associated with increased risks of total, CHD mortality, and incident AF, with highest risks of CHD mortality and AF when thyrotropin level is lower than 0.10 mIU/L.

Endovascular thoracic aortic aneurysm repair without angiography.

OBJECTIVE: : Intravascular ultrasound (IVUS) generates high definition circumferential cross-sectional images and provides real-time readout of vascular dimensions, including visualization of vessel branches. We have used it as an alternative to angiography in the endovascular thoracic aneurysm repair work-up. METHODS: : Out of consecutive 203 patients with descending thoracic aortic aneurysm, 89 (43.8%) received endovascular treatment [mean age, 68 ± 8 years; range, 29-82; male, 79 (88.7%); female, 10 (11.3%)] without using angiography during the endovascular procedure. IVUS (6 F, 12.5 MHz probe or 10 F 9 MHz) coupled with fluoroscopy for the placement of radiopaque markers was used for target site identification, landing zone measurement, device positioning, and assessment of endovascular repair. RESULTS: : Hospital mortality was 4/89 (4.5%). Number of devices implanted in each patient was 1.2 (range, 1-3). X-ray exposure time was 12 ± 8 minutes. Median procedure time was 63 ± 20 minutes. Conversion to open surgery was necessary in one patient (1.1%) because of aortic dissection. In nine patients (10.1%) left subclavian artery was covered because of a short neck. Two patients (2.2%) had vascular access lesions and required surgical repair. One patient developed paraplegia (1.1%). Early endoleak was observed in eight patients (8.9%) and 4 (4.5%) required additional procedures (proximal or distal extensions). Late conversion was necessary in one patient (1.1%). CONCLUSIONS: : IVUS provides all information necessary for device selection, target site identification as well as safe and correct deployment of thoracic endoprostheses and makes periprocedural angiography unnecessary, thus avoiding the risk of renal failure because of contrast medium.

Transplantation rénale pédiatrique: expérience lausannoise de 1971 à 1998 [Pediatric renal transplantation: Experience in Lausanne 1971 to 1998].

AIMS OF THE STUDY: Analysis of indications and results of paediatric renal transplantation in a single centre, before and after the introduction of cyclosporine A (CSA). METHODS: Historical retrospective study. RESULTS: 19 transplantations were performed in 14 patients (5 second grafts) between 1971 and 1987 (group I). 13 patients were transplanted between 1988 and 1998 (no second transplant) (group II). In group II, all the patients had immunosuppression with CSA, but none in group I. Group II, with CSA, showed better renal survival than patients without CSA. In group I, obstructive uropathies (posterior urethral valves, pyelo-ureteral junction stenosis, vesico-ureteral reflux) represent a common cause (35%) of terminal chronic renal failure (TCRF), whereas in group II they represent only 15% of the causes and chronic glomerulonephritis is the most common cause (69%) of TCRF. Acute and chronic graft rejections were the cause of 9 and 1 graft losses in group I and II respectively. Living related donors account for 14% of all renal transplantations in group I and 46% in group II. CONCLUSIONS: The incidence of paediatric patients referred to Lausanne for TCRF is stable. We have observed a constant and steady decrease in obstructive uropathies leading to TCRF and renal transplantations, whereas glomerulonephritis are increasingly frequent. Graft survival has much improved since the introduction of cyclosporine A, without an increase in morbidity. In carefully selected cases, intrafamilial renal transplantation provides good results and helps to shorten the time spent on dialysis.

Surgery for ruptured thoracic and thoraco-abdominal aortic aneurysms.

OBJECTIVE: To assess the outcome of patients with ruptured descending thoracic and thoracoabdominal aortic aneurysms undergoing emergency repair, in comparison to elective surgery for chronic lesions. METHODS: A prospective study of 100 consecutive patients operated upon the descending aorta (1-8 segments) using proximal unloading and distal protection with partial cardiopulmonary bypass, heparin surface-coated perfusion equipment and low systemic heparinization (loading dose 100 IU/kg, activated coagulation time > 180 s), staged cross-clamping, sealed grafts and graft inclusion. RESULTS: Arteriosclerotic lesions were present in 53/100 patients (53%) for all, 30/53 (56%) for chronic, and 21/33 (63%) for ruptured, aneurysms (NS). Dissecting lesions were found in 38/100 patients (38%) for all, 20/53 (38%) for chronic, and 8/33 (24%) for ruptured aneurysms (NS). Preoperative hematocrit was 38 +/- 6% for all, 40 +/- 5% for chronic, and 33 +/- 5% for ruptured aneurysmal patients (P < 0.001 ruptured versus chronic). The extent of aortic repair (1-8 segments) was 3.3 +/- 1.6 for all, 3.5 +/- 1.5 for chronic, and 3.2 +/- 1.4 for ruptured, aneurysms (NS). Transdiaphragmatic repair was performed in 51/100 (51%) of all, 28/53 (53%) of chronic, and 17/33 (51%) of ruptured aneurysms (NS). Aortic cross-clamp time was 38 +/- 21 min for all, 39 +/- 24 min for chronic, and 38 +/- 17 min for ruptured, aneurysmal patients (NS). The amount of red cells washed and autotransfused was 2792 +/- 2239 ml in all, 3143 +/- 2531 ml in chronic, and 2074 +/- 1350 ml in ruptured, aneurysmal patients (P < 0.025). The amount of packed red cells required was 2181 +/- 1830 ml for all, 1736 +/- 1333 ml for chronic, and 2947 +/- 2395 ml for ruptured aneurysmal patients (P < 0.010). Thirty-day mortality was 9/100 (9%) for all, 3/53 (6%) for chronic, and 5/33 (15%) for ruptured aneurysmal patients (NS). Parapareses/plegias occurred in 9/100 (9%) of all, 6/53 (11%) of chronic, and 3/33 (9%) of ruptured, aneurysmal patients (NS). Stepwise regression analysis identified aortic cross-clamp time as a predictor of early mortality (P = 0.002) and parapareses and paraplegias (P = 0.001). Age (P = 0.001), extent of repair (P = 0.008) and preoperative hematocrit (P = 0.001) were predictors for homologous transfusion requirements. CONCLUSION: Emergency repair of ruptured descending thoracic and thoracoabdominal aortic aneurysms can be achieved with acceptable results.