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Macrophage migration inhibitory factor (MIF) plays an important pathophysiological role in pulmonary hypertension (PHT). Previously, we demonstrated that serum MIF is increased in pediatric PHT associated with congenital heart disease (CHD). In the present study, we determined possible associations between MIF levels, hemodynamic and histological parameters, and mitochondrial carbamyl-phosphate synthetase I (CPSI) T1405N polymorphism in a similar population. The asparagine 1405 variant (related to A alleles in the C-to-A transversion) has been shown to be advantageous in pediatric PHT compared to the threonine 1405 variant (C alleles). Forty-one patients were enrolled (aged 2-36 months) and subsequently divided into 2 groups after diagnostic evaluation: the high-pulmonary blood flow (high PBF) group (pulmonary-to-systemic blood flow ratio 2.58 (2.21-3.01), geometric mean with 95% CI) and the high-pulmonary vascular resistance (high PVR) group (pulmonary vascular resistance 6.12 (4.78-7.89) Wood units × m 2 ). Serum MIF was measured using a chemiluminescence assay. The CPSI polymorphism was analyzed by polymerase chain reaction followed by high-resolution melting analysis. Medial hypertrophy of pulmonary arteries was assessed by the histological examination of biopsy specimens. Serum MIF was elevated in patients compared to controls ( p = 0.045), particularly in the high-PVR group ( n = 16) ( p = 0.022) and in subjects with the AC CPSI T1405N genotype ( n = 16) compared to those with the CC genotype ( n = 25) ( p = 0.017). Patients with high-PVR/AC-genotype profile ( n = 9) had the highest MIF levels ( p = 0.030 compared with the high-PBF/CC-genotype subgroup, n = 18). In high-PVR/AC-genotype patients, the medial wall thickness of intra-acinar pulmonary arteries was directly related to MIF levels ( p = 0.033). There were no patients with the relatively rare AA genotype in the study population. Thus, in the advantageous scenario of the asparagine 1405 variant (AC heterozygosity in this study), heightened pulmonary vascular resistance in CHD-PHT is associated with medial hypertrophy of pulmonary arteries where MIF chemokine very likely plays a biological role.

Primary cardiac tumors are rare and approximately half of them are atrial myxomas. They rarely remain asymptomatic, especially if large. The imaging of a myxoma by contrast dye during coronary angiography is an infrequent sign, which clarifies the vascular supply of the tumor. We report herein an interesting and rare case of a left atrial myxoma hypervascularized from the right coronary artery.

Background: The present study aimed to evaluate the clinical performance, in the daily practice of a busy catheterization laboratory, of a novel drug-eluting stent (DES) built with an ultra-thin-strut metallic platform, eluting sirolimus at low doses, abluminal coated with biodegradable polymers, and mounted in a low-compliant delivery system., Methods: Prospective, single-arm study, comprising all consecutive patients undergoing percutaneous coronary intervention (PCI) with the Inspiron™ sirolimus-eluting stent (SES) (Scitech, Aparecida de Goiania, Brazil). The primary endpoint was the occurrence of major adverse cardiac events (MACE) [cardiac death, non-PCI related myocardial infarction (MI), or target vessel revascularization (TVR)]., Results: A total of 470 patients were included, from which 51.3% were diabetics, 33.8% had triple-vessel disease, 15.3% had heart failure, 38.9% had at least one bifurcation treated, 19.8% were treated for a bare metal stent restenosis, and 61.9% had at least one type C lesion; one or more of these features were found in 96.0%. At 300 days, the rate target lesion revascularization was 5.4% and the rate of MACE was 8.1%. The incidence of definite or probable stent thrombosis was 0.4%, with no cases between 30 and 300 days., Conclusions: The novel stent is associated with excellent short and mid-term clinical outcomes in patients treated with PCI in the daily practice.

Background: Advantages and disadvantages of ad hoc percutaneous coronary intervention have been described. However little is known about the radiation exposure of that procedure as compared with the staged intervention., Objective: To compare the radiation dose of the ad hoc percutaneous coronary intervention with that of the staged procedureMethods:The dose-area product and total Kerma were measured, and the doses of the diagnostic and therapeutic procedures were added. In addition, total fluoroscopic time and number of acquisitions were evaluated., Results: A total of 568 consecutive patients were treated with ad hoc percutaneous coronary intervention (n = 320) or staged percutaneous coronary intervention (n = 248). On admission, the ad hoc group had less hypertension (74.1% vs 81.9%; p = 0.035), dyslipidemia (57.8% vs. 67.7%; p = 0.02) and three-vessel disease (38.8% vs. 50.4%; p = 0.015). The ad hoc group was exposed to significantly lower radiation doses, even after baseline characteristic adjustment between both groups. The ad hoc group was exposed to a total dose-area product of 119.7 ± 70.7 Gycm2, while the staged group, to 139.2 ± 75.3 Gycm2 (p < 0.001)., Conclusion: Ad hoc percutaneous coronary intervention reduced radiation exposure as compared with diagnostic and therapeutic procedures performed at two separate times.

Coronary artery anomalies are congenital changes in their origin, course, and/or structure. They are the second most frequent cause of sudden death in young athletes. Dual LAD artery is a rare coronary anomaly. We present the case of a 44-year-old man with recent onset exertional angina and documented ischemia whose coronary angiogram and computed tomography (CT) showed type 4 dual LAD artery, the rarest and most interesting variant.

Coronary artery anomalies are congenital changes in their origin, course, and/or structure. Most of them are discovered as incidental findings during coronary angiographic studies or at autopsies. We present herein the case of a 70-year-old man with symptomatic severe aortic valvar stenosis whose preoperative coronary angiogram revealed a so far unreported coronary anomaly circulation pattern.

Background: The hemodynamic effects of noninvasive ventilation with positive pressure in patients with pulmonary hypertension without left ventricular dysfunction are not clearly established. Objectives: Analyze the impact of increasing airway pressure with continuous positive airway pressure on hemodynamic parameters and, in particular, on cardiac output in patients with variable degrees of pulmonary hypertension. Methods: The study included 38 patients with pulmonary hypertension caused by mitral stenosis without left ventricular dysfunction or other significant valvulopathy. The hemodynamic state of these patients was analyzed in three conditions: baseline, after continuous positive pressure of 7 cmH2O and, finally, after pressure of 14 cmH2O. Results: The population was composed of predominantly young and female individuals with significant elevation in pulmonary arterial pressure (mean systolic pressure of 57 mmHg). Of all variables analyzed, only the right atrial pressure changed across the analyzed moments (from the baseline condition to the pressure of 14 cmH2O there was a change from 8 ± 4 mmHg to 11 ± 3 mmHg, respectively, p = 0.031). Even though there was no variation in mean cardiac output, increased values in pulmonary artery pressure were associated with increased cardiac output. There was no harmful effect or other clinical instability associated with use application of airway pressure. Conclusion: In patients with venocapillary pulmonary hypertension without left ventricular dysfunction, cardiac output response was directly associated with the degree of pulmonary hypertension. The application of noninvasive ventilation did not cause complications directly related to the ventilation systems.

Introduction and Objectives: The left atrium is clinically relevant in patients with mitral valve stenosis. The objective of this study was to analyze the effects of percutaneous balloon valvuloplasty on left atrium volumes and the left atrium emptying fraction in symptomatic mitral valve stenosis patients using 2-dimensional and real-time 3 -dimensional transthoracic echocardiography., Methods: We carried out a prospective study of 28 consecutive symptomatic mitral valve stenosis patients, aged 22-72 (39 [11.5]) years, 24/28 (85.6%) women, who underwent to percutaneous balloon valvuloplasty between March 2009 and May 2011. Patients underwent 2- and 3-dimensional transthoracic and transesophageal echocardiography (atrial fibrillation) and invasive mitral valve area measurement. Echocardiographic analysis was performed before, 72 h after and 12 months after percutaneous balloon valvuloplasty. The following parameters were analyzed: a) mitral valve area (2-dimensional planimetry, pressure half-time, 3-dimensional echocardiography, invasive hemodynamic measurement); b) indexed left atrium maximum and indexed minimum volumes, and c) left atrium emptying fraction., Results: The 3-dimensional parameters of the mitral valve stenosis patients before and 72 h and 12 months after percutaneous balloon valvuloplasty were as follows: a) mitral valve area: 0.9 (0.1) cm(2); 1.8 (0.2) cm(2) (P<.001); 1.7 (0.2) cm(2) (P<.001); b) left atrium maximum volumes: 49.9 (12) mL/m(2); 42 (11.4) mL/m(2) (P<.001); 40.3 (10.2) mL/m(2) (P<.001), and c) left atrium emptying fraction: 30.1 (9.4%); 40.6 (7.4%) (P<.001); 44.1 (8%) (P<.001), respectively., Conclusions: In symptomatic mitral valve stenosis patients who underwent percutaneous balloon valvuloplasty analysis by 2- and 3-dimensional echocardiography, improvements in left atrium reverse remodeling and left atrium emptying fraction were observed 72 h and 12 months after the procedure., (Copyright © 2012 Sociedad Española de Cardiología. Published by Elsevier Espana. All rights reserved.)

In hypertensive patients with indication of renal arteriography to investigate renal artery stenosis (RAS) there are no recommendations regarding when to investigate coronary artery disease (CAD). Moreover, the predictors of CAD in patients with RAS are not clear. We aimed to evaluate the frequency and the determinants of CAD in hypertensive patients referred to renal angiography. Eighty-two consecutive patients with high clinical risk suggesting the presence of RAS systematically underwent renal angiography and coronary angiography during the same procedure. Significant arterial stenosis was defined by an obstruction ≥ 70% to both renal and coronary territories. Significant CAD was present in 32/82 (39%) and significant RAS in 32/82 (39%) patients. Both CAD and RAS were present in 25.6% from the 82 patients. Patients with severe CAD were older (63 ± 12 vs. 56 ± 13 years; p = 0.03) and had more angina (41 vs. 16%; p = 0.013) compared to patients without severe CAD. Significant RAS was associated with an increased frequency of severe CAD compared to patients without significant RAS (66% vs. 22%, respectively; p<0.001). Myocardial scintigraphy showed ischemia in 21.8% of the patients with CAD. Binary logistic regression analysis showed that RAS ≥ 70% was independently associated with CAD ≥ 70% (OR: 11.48; 95% CI 3.2-40.2; p<0.001), even in patients without angina (OR: 13.48; 95%CI 2.6-12.1; p<0.001). Even considering a small number of patients with significant RAS, we conclude that in hypertensive patients referred to renal angiography, RAS ≥ 70% may be a strong predictor of severe CAD, independently of angina, and dual investigation should be considered.

Aims: To evaluate the risk and predictors of death in a large population of patients with stable coronary disease treated with percutaneous intervention., Methods and Results: The study population comprised 1,276 patients with chronic angina or silent ischaemia who underwent elective coronary angioplasty. Baseline and in-hospital mortality data were prospectively collected for all patients during the index hospitalisation. Post-discharge outcome was assessed at out-patient clinic, by review of the patients' records, or direct phone contact. Deaths were classified as cardiac and non-cardiac. Age, peripheral arterial disease, congestive heart failure with NYHA class >or= III, triple-vessel disease, and procedural success (i.e. angiographic success for all lesions in the absence of peri-procedural infarction) remained as multivariate independent predictors of death. For the entire population 4-year cumulative all-cause and cardiac mortality were respectively 5.4% and 4.1%. Four-year mortality for patients without any multivariate predictor was 2.4%, while for patients with two or more predictors the death rate was 16.3% after four years., Conclusions: Patients with stable coronary disease undergoing percutaneous treatment have an overall low mortality rate after four years. Nevertheless, stable patients comprise a heterogeneous population in terms of risk profile, ranging from patients at very low risk of late death to individuals with a poor long-term prognosis.

Background: Factors influencing the size of target vessels of patients referred for coronary intervention are poorly defined. We aimed to investigate in a large series of patients undergoing percutaneous intervention the relation of constitutional, anatomical, and clinical features with the reference diameter of coronary vessels treated with stenting., Methods: A total of 4,850 de novo coronary lesions, non-ostial and non-bifurcational, located in native vessels were analyzed. The following pre-specified characteristics were analyzed to reflect the relation between constitutional, anatomical, and clinical features on reference vessel diameter: age, gender, height, weight, proximal location, vessel, diabetes, hypertension, multivessel disease, and clinical presentation., Results: The average reference diameter was 2.66+/-0.50 mm. All pre-specified markers had a significant relation with the vessel reference diameter at univariate analysis, except by hypertension which showed a strong tendency. However, at multivariate analysis, only diabetes, proximal location, multivessel disease, clinical presentation, vessel, weight, and height were identified as independent predictors of reference vessel diameter., Conclusion: Reference diameter of coronary vessels at the site of lesions treated by stenting is significantly influenced by a variety of characteristics. We hypothesize that the treated segment size of patients undergoing stenting ultimately reflects the conjoint effect of several different factors, including constitutional, anatomical, and clinical features.

A clinical case of a 10-year-old male patient is reported. His dilated and prevailing right ventricular myocardiopathy shows diagnostic difficulties between previous myocarditis etiology and arrhythmogenic dysplasia. As the elements are not pathognomonic of one or other cause, the increase of cardiac enzymes in subacute stage maybe tends to the supposition of previous myocarditis. Hence, the questioning that many cases labeled as arrhythmogenic dysplasia can truly correspond to the possibility of evolutional myocarditis. The controversial clinic management is disputable.

Aims: Coil protrusion into the left pulmonary artery (LPA) has been described after transcatheter closure of the patent ductus arteriosus (PDA). The possible impact of such a finding in lung perfusion has not been completely clarified. We evaluated Doppler flow velocities and lung perfusion in patients submitted to that procedure., Methods: After transcatheter closure of PDA with coils, 70 patients (mean age 8.6+/-3.4 years) were followed for a period of 3.6+/-0.9 years (range 2.1-5.9) and compared to 22 controls. Peak flow velocities and coil protrusion were assessed by Doppler echocardiography. A Doppler velocity index (DVI) was calculated by the difference between the LPA and right pulmonary artery (RPA) peak flow velocities relative to the pulmonary trunk (PT) expressed in percentage, as follows: DVI=(LPA velocity - RPA velocity)/PT velocity x 100. Lung scintigraphy was performed using (99m)Tc-labelled macro-aggregated albumin., Results: Device protrusion was observed in 94% of the patients, 10% of whom presented abnormal left lung perfusion. Peak LPA velocity and DVI were significantly greater in patients (p=0.001) and correlated negatively with left lung perfusion values (R(2)=0.21 and R(2)=0.65, respectively). A cut-off value of 50% for the DVI showed high sensitivity and specificity for reduced lung perfusion., Conclusion: Impaired left lung perfusion may appear following transcatheter closure of PDA with coils and the determination of DVI may anticipate such alteration.

A 64-year-old man with left main coronary artery disease underwent myocardial revascularization. The left internal thoracic artery (LITA) was sutured to the left anterior descending artery, and the right internal thoracic artery (RITA) was sutured to the obtuse marginal artery. Eighteen years later, angina reoccurred. Catheterization revealed that both the coronary and the left subclavian arteries were occluded and that a patent RITA graft was maintaining the cardiac blood supply. The RITA graft evaluation revealed increased lumen diameters, suggestive of remodeling. The LITA was subsequently disconnected and sutured to the aorta as a free graft in order to restore appropriate myocardial blood flow. This case emphasizes the benefits of using a live graft for left coronary system grafting, which include long-term patency and flow-dependent remodeling.

A consecutive series of interventions in vessels with reference diameter < or = 2.75 mm was retrospectively analyzed according to preprocedure strategy: balloon angioplasty with provisional stenting (PTCA group, 73 patients) and primary stenting (PS group, 122 patients). In the PS group, there were more patients with single-vessel disease (54.1% vs. 37.0%; P = 0.021), less patients with three-vessel disease (9.0% vs. 24.7%; P = 0.003), more LAD interventions (54.9% vs. 31.5; P = 0.002), and less left circumflex interventions (22.1% vs. 45.2%; P < 0.001). Reference diameter was larger in the PS group (2.28 +/- 0.35 mm vs. 2.11 +/- 0.36 mm; P = 0.001). Provisional stenting was performed in 39.7% of PTCA group. At long-term outcome, the incidence of composite major events was similar between the PTCA and the PS groups (20.5% vs. 17.2%, respectively; P = NS). Treatment of small vessels with balloon dilatation and provisional stenting or with primary stenting yielded similar late outcomes. Operators' choice of treatment strategy was based on particular angiographic characteristics., (Copyright 2002 Wiley-Liss, Inc.)

Extrinsic compression of the left main coronary artery (LMC) by the pulmonary artery (PA) is a very unusual and poorly understood entity, usually associated with the presence of adult congenital heart disease. We identified 12 patients (age range, 6 months to 55 years) with LMC stenosis (> or = 50%) presumably secondary to compression by a dilated main PA and related to various forms of heart disease (11 congenital, 1 pulmonary hypertension). In all cases, the main PA was dilated with the main PA/aortic root diameter increased (mean, 2.0; normal value, < or = 1.0), and in all but two, PA pressures were increased (> 30 mm Hg systolic). Left coronary trunk stenosis was usually visualized in only one angiographic view (best seen in 45 degrees left anterior oblique, 30 degrees cranial projection). The LMC also appeared to be inferiorly displaced and in close contact with the left aortic sinus (mean angle between sinus and LMC was 23 degrees +/- 13 degrees, a control group was 70 degrees +/- 15 degrees ). In one patient, surgical correction of the dilated PA was associated with a reduction in LMC stenosis from 85% to < 50% and less inferior left main displacement (from 25 degrees to 50 degrees ). Patients with a dilated main PA may exhibit extrinsic LMC compression leading to significant eccentric narrowing and downward displacement of the LMC. In the presence of significant dilatation of the main PA from any etiology, functional and/or anatomic studies should be performed to exclude significant LM obstruction.

Introduction: A recently described focal origin of atrial fibrillation, mainly inside pulmonary veins, is creating new perspectives for radiofrequency catheter ablation. However, pulmonary venous stenosis may occur with uncertain clinical consequences. This report describes a veno-occlusive syndrome secondary to left pulmonary vein stenosis after radiofrequency catheter ablation., Methods and Results: A 36-year-old man who experienced daily episodes of atrial fibrillation that was refractory to antiarrhythmic medication, including amiodarone, was enrolled in our focal atrial fibrillation radiofrequency catheter ablation protocol. The left superior pulmonary vein was the earliest site mapped, and radiofrequency ablation was performed. Atrial fibrillation was interrupted and sinus rhythm restored after one radiofrequency pulse inside the left superior pulmonary vein. Atrial fibrillation recurred and a new procedure was performed in an attempt to isolate (26 radiofrequency pulses around the ostium) the left superior pulmonary vein. Ten days later, the patient developed chest pain and hemoptysis related to severe left superior and inferior pulmonary veins stenosis. Balloon angioplasty of both veins was followed by complete relief of symptoms after 2 months of recurrent pulmonary symptoms. The patient has been asymptomatic for 12 months, without antiarrhythmic drugs., Conclusion: Multiple radiofrequency pulses applied inside the pulmonary veins ostia can induce severe pulmonary venous stenosis and veno-occlusive pulmonary syndrome.

A 48-year-old man with essential thrombocythemia suffered an extensive anterior acute myocardial infarction; this is a rare association. A pharmacological thrombolysis was performed, without success. He subsequently underwent successful rescue coronary angioplasty. To our knowledge, there is no other report in the literature relating the triad of essential thrombocythemia, acute myocardial infarction and rescue coronary angioplasty.

A 60 year-old woman with progressive angina who had been submitted to saphenous bypass-graft to right coronary artery and a left mammary artery graft to anterior descending artery eight years previously, underwent implantation of a Gianturco Roubin II stent in the proximal third of the saphenous vein graft. The result was suboptimal by persistence of a residual stenosis probably due to prolapse of atherosclerotic material through the coil spaces. Another stent (Palmaz-Schatz biliar stent) was implanted at the previously stented site with no residual stenosis. Another Palmaz-Schatz biliar stent was successfully implanted in the distal body of the graft to treat another lesion (passing through the previously stents without difficulty). Stenting a stent, in selected situations, is a useful tool to optimize the angiographic result of stent implantation.

Objective: Introduce a new surgical technique for biventricular correction of double-outlet right ventricle with noncommitted ventricular septal defect., Methods: From April 1987 to February 1996, 15 patients with double-outlet right ventricle with noncommitted ventricular septal defect were operated on using a new technique for biventricular repair with multiple bovine pericardial patches to create a tunnel between the left ventricle and the aorta. Ages ranged from two months to 13 years (mean age 4.8 years). Thirteen patients had situs solitus and levocardia, one patient had situs inversus and dextrocardia, and one patient had situs solitus and dextrocardia. Construction of the tunnel began at the right atrium. The ventricular septal defect (VSD) was enlarged anteriorly, if restrictive or small, and the first patch was sutured in the infero-posterior edge of the VSD. The second, third and sometimes the fourth patches were sutured in sequence, through the right ventriculotomy, directing the tunnel to the aortic annulus., Results: Overall mortality was 20%, with two early and one late death. The surviving patients were followed-up for a period ranging from ten months to nine years (mean 33 months), and all were in functional class I (NYHA). Minimal residual ventricular septal defect was observed in one patient, stenosis in two patients and moderate pulmonary insufficiency in one. There was no obstruction of the intraventricular tunnel between the LV and the aorta., Conclusion: Based on these data, we conclude that this technical modification for the biventricular repair of the double-outlet right ventricle with noncommitted VSD allows for the construction of a tunnel with adequate internal diameter, respecting the spatial changes between the VSD and aorta. In addition, the intraventricular bovine pericardial tunnel takes up less space, thus reducing the incidence of right ventricle outlet obstruction.

Endomyocardial fibrosis is an endemic cardiac disease, characterized by the presence of fibrous tissue in the endocardium, eventually extending to the myocardium. Massive endocardial calcification of the left ventricle is a rare finding, with only a few cases reported in the literature. We reported a first case of biventricular massive endocardial calcification associated with endomyocardial fibrosis in a 22 year old woman.

Fetal echocardiography has recently caused an impact on the treatment of congenital heart disease and in the field of therapeutic, cardiological intervention. The present study reports on a case of critical aortic stenosis, diagnosed in utero at 27 weeks' gestation, and in which balloon dilatation was attempted to improve the poor prognosis associated with this condition. Since the endocardium at this stage of development was apparently normal, this therapeutic intervention was attempted to avoid irreversible damage to the left ventricle. Although hydrops disappeared and the myocardium hypertrophied, endocardial fibroelastosis progressed and the neonate died within the first day of life, after surgical aortic valvotomy. More data are necessary to clarify whether endocardial fibroelastosis is really a consequence of high pressure in the left ventricle resulting from stenosis of the aortic valve or whether it is a disease, the progression of which is unavoidable once it takes hold.

The authors describe a rare case of circumflex coronary artery perforation during rotational coronary atherectomy complicated with cardiac tamponade and good outcome. The possible causes of perforation are discussed and the burr oversize (burr/artery ratio was 0.58) was refused. Shortening and artery plicature (accordeon effect) might have been the cause of this event. Quantitative measurement was made in order to strengthen this hypothesis. It is emphasized the importance of selecting lesions that should be submitted to rotational coronary atherectomy.

Purpose: To determine the prevalence of residual left-to-right shunt in patients submitted to closure of patent ductus arteriosus with use of Rashkind double-disc ductal occluding device, analyzing predictive factors that determine short and long-term prevalence of residual shunt., Methods: Thirteen patients were submitted to percutaneous closure of patent ductus arteriosus with use of Rashkind double-disc device. Ten patients were male with mean age of 5.7 years. A 12mm diameter device was used in 7 cases and a 17mm device in the remaining six patients. All patients had clinical, radiological and echocardiographic follow up, after 24h, 1 month, 6 months and one year after the procedure. Morphology and length of the ductus arteriosus and the presence of residual shunt after 15 min, 24h and one year after the procedure, were correlated., Results: In one case, embolization of the device to the pulmonary artery determined the in success of the procedure. Residual shunt was present in 75% of the patients after 15 min of the procedure, in 33.3% after 24h, in 25% after 1 month and 6 months and in 16.6% after 1 year. The most important and isolated predictive factor leading to a high prevalence of residual shunt after 24h and after 1 year of the procedure was the presence of ductus arteriosus diameter > or = 4.5mm at the site of its insertion in the pulmonary artery., Conclusion: Prevalence of residual left-to-right shunt decreases over the time, with a low incidence after one year follow-up. A higher incidence of residual shunt at 24h and 1 year after the procedure occurred in the cases where the diameter of the ductus arteriosus was > or = 4.5mm, at the site of its insertion in the pulmonary artery.

A newborn with transposition of the great arteries presented with rupture of the ductus arteriosus after balloon catheter atrioseptostomy. The necropsy study demonstrated persistent ductus patency, and a 0.5-cm-long horizontal fissure could be observed. On microscopy, there was laceration of the intimal layer, with wall dissection and focal hemorrhage extending to the adventitia. Ductus rupture was attributed to the wall weakness, as a consequence of prostaglandin E1 administration.

Purpose: The study of frequency, modalities and course of neurological complications of infective endocarditis (IE), as well as the current indication and value of supplementary examinations., Methods: Sixty-three patients with IE, 39 with native valve and 24 with valvar prosthesis, were prospectively studied; the mean age was 42 years and 45 (71.4%) were males. Two groups were formed: A) 41 patients without neurological events and B) 22 patients who presented 28 neurological events before or during hospitalization: ischemic cerebrovascular accident 20, hemorrhagic cerebrovascular accident 2, meningeal hemorrhage 2, meningitis 2, brain abscess 1 and seizure 1. All patients were submitted to neurological clinical examination; 57 computerized tomographies of the cranium, 28 arteriographies and 32 cerebrospinal fluid analysis were performed., Results: The incidence of neurological events corresponded to 34.92% of IE patients, with a clear predominance (85.71%) of vascular as compared to infectious manifestations. Mortality was 2.32 times higher in group B patients (22.73% x 9.76%), albeit p = 0.256, and was not related to staphylococcal etiology. The neurological events were not related to sex, age and presence of valvar prosthesis. The presence of neurological complications was greater (p = 0.047) in patients with simultaneous infections in two valves (mitral and aortic) and also (p = 0.00884) in those with IE in prosthesis implanted for less than three months. All supplementary neurological examinations in group A were normal., Conclusion: 1) Occurrence of neurological events is a factor which influences the prognosis of IE; 2) supplementary neurological examinations did not reveal subclinical neurological complications; 3) neurological complications were significantly more frequent in patients with simultaneous mitral and aortic valve IE; 4) IE in prosthesis implanted for less than 3 months has a greater probability to develop a neurological picture as compared to IE in prosthesis implanted for more than 3 months.

Interruption of the aortic arch is an uncommon cardiovascular anomaly. It is usually associated with a large ductus arteriosus (DA) and interventricular septal defects. Two cases of one and two month old girls with restrictive DA are reported. The clinical, anatomical and surgical findings are discussed.

Two cases of transposition of the great arteries (TGA) with posterior aorta are reported. The first was submitted to the Senning procedure with good outcome; the other had the diagnosis of double outlet right ventricle with severe pulmonary stenosis, and a Blalock-Taussig shunt was accomplished for hypoxic crisis. The patient died 11 days after surgery and the necropsy demonstrated TGA with posterior aorta. In both cases there was a sub-pulmonary infundibulum. The presence of ventricular septal defects allowed aortic-mitral fibrous continuity, a finding previously described in anatomical reports. Clinical and angiographic aspects are discussed.

Ischemic cardiomyopathy in childhood is related with congenital and acquired pathologies which could lead to serious cardiac sequelae as myocardial infarction and sudden death. Unfortunately, when coronary artery malformations are excluded, it is difficult in some cases to be completely certain on the etiology of the arterial coronary disease, due the lack of pathognomonic diagnostic tests. Case report of 6 year old child, with severe coronary artery disease: aneurysm of main branch of left coronary artery; left anterior descending branch and right coronary artery occlusions with accentuated myocardial dysfunction. We believe that the diagnosis of Kawasaki disease is presumptive in this patient. The principal aspects to elucidate the etiology of coronary artery disease are analysed, as well as the importance of identifying patients without known previous illness, but with severe coronary artery lesions, as in this reported case.

Dilatation of the ascending aorta is relatively infrequent during childhood. Besides the Marfan syndrome, the congenital origin should be considered. We report a patient with dilatation of the ascending aorta diagnosed at the age of 10 who presented acute aortic dissection and rupture after a 13-year period of follow-up. Several aspects of the proper diagnosis are discussed.

Two patients with transposition of the great arteries submitted to Jatene surgery at ages of 6 days and 6 months respectively, presented in the postoperative period stenosis of the aorto-pulmonary anastomosis. The diagnosis was made three and six years after the surgery. In both patients successful percutaneous transluminal angioplasty (PTA) was performed, respectively six months and immediately after the diagnosis. Pressure gradients decreased from 83 mmHg to 24 mmHg in the first case, and from 76 mmHg to 13 mmHg in the other case, with clear improvement of the angiographic image. Complications of the procedure did not occur in any case. This application for PTA was not previously reported and should be considered as an alternative to the surgical correction of the "new" pulmonary trunk stenosis in the Jatene surgery.