[Aortic arch interruption with restrictive ductus arteriosus]. / Interrupção do arco aórtico com canal arterial restritivo.

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.

[Transposition of the great arteries with posterior aorta]. / Transposição das grandes artérias com aorta posterior.

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 a child. Case report with accentuated coronary disease]. / Miocardiopatia isquêmica na criança. Relato de caso com acentuada coronariopatia.

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.

[Dissection in an adult with dilatation of the ascendent aorta diagnosed in childhood]. / Dissecção no adulto em dilatação de aorta ascendente diagnosticada na infância.

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.

[Endomyocardial fibrosis with massive biventricular endocardial calcification]. / Endomiocardiofibrose com Calcificação endocárdica maciça biventricular.

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.

[Neurological events in infective endocarditis]. / Eventos neurológicos na endocardite infecciosa.

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.

[Transluminal angioplasty of aortopulmonary anastomosis by the Jatene surgical technique. Report of 2 cases]. / Angioplastia transluminal da anastomose aorto-pulmonar da cirurgia de Jatene. Aplicação em dois casos.

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.

[Coronary artery perforation with rotational coronary atherectomy]. / Perfuração de artéria coronária com rotablator.

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.

[Stenting in a recently implanted stent in a saphenous vein graft for optimizing angiographic results]. / Implante de stent dentro de stent recém implantado em ponte de veia safena para otimização do resultado angiográfico.

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.

Essential thrombocythemia and acute myocardial infarction treated with rescue coronary angioplasty.

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.

[Prevalence of residual shunt after closure of patent ductus arteriosus with Rashkind umbrellas]. / Prevalência do shunt residual após fechamento de canal arterial persistente com umbrellas de Rashkind.

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.

Pulmonary vein stenosis complicating catheter ablation of focal atrial fibrillation.

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.

Balloon dilatation of the aortic valve in the fetus. A case report.

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.

Extrinsic compression of the left main coronary artery by a dilated pulmonary artery: clinical, angiographic, and hemodynamic determinants.

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.

Ductus arteriosus rupture as a balloon catheter atrioseptostomy complication.

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.

Double-outlet right ventricle with nonrelated ventricular septal defect: surgical results using the multiple patches technique.

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.