[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.

[Origin of left coronary artery from right and left pulmonary arteries--clinical, anatomico surgical and follow-up evaluation of 3 cases]. / Origem da artéria coronária esquerda das artérias pulmonares, direita e esquerda--avalia ção clínica, anatomocirúrgica e evolutiva de três casos.

Three infants with anomalous origin of left coronary artery, being two from the right pulmonary artery and one from the left, without other congenital defects, ages of 5, 15 and 3 months old, presented with early congestive heart failure due to anterolateral myocardial infarction. Anatomical characteristic was peculiar in two cases since the left coronary artery, before the anastomosis with the right pulmonary artery, presented a wy, 15 mm extension path within the aortic wall which has facilitated the surgical correction by the proximity of these structures. In the third case, the left coronary artery was anastomosed with the left pulmonary artery, 2 cm from its origin and anterior to the left atrium being the surgical correction by direct implant at the ascending aorta. Post-operative follow-up, 36, 30 and 33 months respectively, showed clinical and laboratorial normalization except for the second case in which ST segment remained upward due to ventricular aneurysm. Myocardial recovery, even after extensive infarction, in this anomaly, emphasizes the need for earlier diagnosis and management.

[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.

[Agenesis of the right pulmonary artery with severe pulmonary hypertension, attenuated by surgical correction]. / Agenesia da artéria pulmonar direita com acentuada hipertensão pulmonar, atenuada por correção cirúrgica.

A 22 months old infant with agenesis of right pulmonary artery, without other associated congenital heart defects, is reported. Reconstruction of pulmonary arteries by connecting them through a 7mm Goretex tube was performed, with clear clinical improvement in view of disappearance of signs of cardiac insufficiency and of hypoxemia. Contralateral pulmonary hypertension, at systemic level, decreased to 77% in the immediate postoperative period. So, this technique becomes the first option, before considering these cases for cardiopulmonary transplantation.

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 INCORFMUSP. 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.

[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.

[Treatment using balloon catheterization of pulmonary valve stenosis]. / Tratamento por cateter-balão da estenose pulmonar valvar.

From October 84 to September 88 we performed pulmonary valvuloplasty with balloon catheter (PVBC) in 90 patients (pt) with pulmonary valve stenosis (PVS). The patients were divided in 3 groups (Gr) by their ages: group I (Gr. I) 7 pt (8%) aged 4 days to 11 months, Gr. II 66 pt (73%) aged 1 year to 12 years and Gr. III 17 pt aged 13 to 34 years. This study consists of clinical and echocardiographic evaluation adopting the following parameters of indication: a) transvalvar pulmonary gradient (G); b) absence of dysplasia or hypoplasia of the pulmonary annulus. There were associated lesions which were found in each group: Gr. I atrial septal defect (ASD) in 2 pt and tricuspid insufficiency (TI) in 1 pt, in Gr. II supra valvar stenosis (SVS) in 1 pt and patent arterial ductus (PAD) in 1 pt and in the Gr. III (ASD) in 1 pt. The results after pulmonary valvuloplasty with balloon catheter (PVBC) were: (Gr.I) in 5 pt the G were reduced below 50 mmHg and the average of right ventricular systolic pressure (RVSP) changed from 92 to 62 mmHg and the average of G from 76 to 44 mmHg. One of these pt was submitted to surgery 1 month after the procedure and 2 developed restenosis in 2 and 3 years respectively, being submitted to another dilatation with good results. The 2 pt in which the procedure was not effective were submitted to surgery. In the second group the PVBC was effective in 61 pt (92%) showing the RVSP changed from 118 to 64 mmHg and G changed from 86 to 23 mmHg.(ABSTRACT TRUNCATED AT 250 WORDS)

[Transluminal angioplasty in total anomalous drainage of obstructed pulmonary veins. A case report]. / Angioplastia transluminal na drenagem anômala total de veias pulmonares na forma obstrutiva. Relato de caso.

Transluminal balloon angioplasty has been successfully used in the treatment of various congenital heart diseases, specially in pulmonary valve stenosis. Dilation angioplasty has also been used in venous obstructions in children, but the results have been contradictory. This report describes the case of a three-month-old female infant with obstructed total anomalous pulmonary venous return into the right superior vena cava. Percutaneous angioplasty was performed with two 4 mm diameter balloon catheters (Simpson Ultra Low Profile), with success. Right ventricular systolic pressure decreased from 68 to 49 mmHg and mean pulmonary vein pressure decreased from 41 to 17 mmHg. There was significant clinical improvement. Although it would be an unique experience in our Department, authors suggest that transluminal balloon angioplasty could be an initial therapeutic alternative in infants with obstructive total anomalous pulmonary venous return, a high-risk group for total surgical repair.

[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.

[Hemodynamic evaluation and clinical outcome of children with severe dilated cardiomyopathy eligible for heart transplantation]. / Avaliação hemodinâmica e evolução clínica de crianças portadoras de cardiomiopatia dilatada grave candidatas a transplante cardíaco.

PURPOSE: To evaluate hemodynamic parameters and clinical outcome of children with severe cardiomyopathy who are candidates for heart transplantation. METHODS: Twenty four children aged from 4 months to 10 years and 8 months (mean 3.7 +/- 2.5 years) from February 1992 to May 1996, were submitted to hemodynamic study and the following parameters were measured: cardiac output, mean pulmonary artery pressure (MPAP) and capillary wedge pressure. The pulmonary vascular resistance index (PVRI) and transpulmonary pressure gradient (TGP) were calculated. RESULTS: Ten (41.6%) children were transplanted (group A), 5 (20.8%) are still waiting heart transplantation (group B) and 9 (37.6%) died (group C). The mean age of the B and C group were significantly different. The PVRI, MPAP and TPG were significant lower in group A than group C (p = 0.01; p = 0.044 and p = 0.023 respectively). CONCLUSION: The profile of the hemodynamic parameters of children with severe dilated cardiomyopathy was compatible with the clinical findings. The age was the only difference comparing groups B and C. PVRI, MPAP and TPG were significant factors between group A and C. The older the patient, the worse was the prognosis.

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.

[Percutaneous angioplasty and surgical revascularization in renovascular hypertension: experience in treatment and long-term follow up in 124 patients]. / Angioplastia percutânea e revascularização cirúrgica em hipertensão renovascular. Experiência no tratamento e seguimento de longo prazo em 124 pacientes.

PURPOSE: To report the experience in the treatment and long-term follow-up of patients with renovascular hypertension submitted to angioplasty and/or surgical revascularization, and to compare blood pressure and renal function responses to both treatment. METHODS: One hundred and twenty-four patients diagnosed with renovascular hypertension, divided, according to the etiology, as atherosclerosis (76 patients), fibromuscular dysplasia (32) and arteritis (16), were submitted to isolated or associated treatment of renal revascularization by percutaneous transluminal angioplasty (71 patients) or surgery (84). The patients were followed-up during 2.5 +/- 1.5 years and we evaluate blood pressure response and renal function preservation. In relation to blood pressure control, the patients were considered as cured when normotensives without antihypertensives, improved when normotensives with fewer drugs than previously, and unchanged, when out of these criteria. RESULTS: From 124 patients, 20% were considered cured, 54% improved and 26% unchanged, after a mean follow-up of 2.5 +/- 1.5 years. Patients with atherosclerosis and fibromuscular dysplasia presented a higher rate of care and improvement after surgery. The blood pressure and the number of antihypertensive drugs decreased significantly during the follow-up among patients that cured or improved arterial hypertension, either after angioplasty (190 +/- 26/115 +/- 14 mmHg vs 130 +/- 34/85 +/- 7 mmHg. 2.70 vs 1.60 drugs) or after surgery (194 +/- 17/115 +/- 16 mmHg vs 143 +/- 18 mmHg vs 88 +/- 8 mmHg, 1.88 vs 1.51 drugs). It was also observed a significant decrease of serum creatinine among these patients. CONCLUSION: Renal revascularization in patients with renovascular hypertension, either by angioplasty or surgery, is beneficial to control blood pressure and to preserve renal function in the majority of patients.

[Atrial septal defect percutaneous transvenous occlusion with the buttoned device]. / Oclusão percutânea transvenosa de comunicação interatrial mediante utilização do buttoned device.

PURPOSE: To analyse the experience with the use of Sideris' device for percutaneous transvenous occlusion of atrial septal defect (ASD). METHODS: Thirty six procedures of ASD occlusion were performed in 32 patients; 27 (84.4%) female and five (15.6%) male, mean age of 16 years (4-61). All patients had secundum atrial septal defects with clinical and hemodynamic compromise. The ASD diameter measured < 31 mm and occupied less than 50% of the septal length in all cases. The basic device was used in 28 (87.5%) patients and the self centered model in four (12.5%). The direct implant technique was employed in 15 (41.7%) procedures while in the other 21 (58.3%) an over a wire technique was used. The size of the device was selected according with the Sideris' normogram. RESULTS: Occlusion of the defect was achieved in 29 patients (90.63%). In two (6.25%) the position of the device was not adequate and in one (3.12%) the device embolized to the pulmonary artery. In these three cases the device was removed surgically during the defect closure. There was neither morbidity nor mortality in this series. Trivial or small residual shunt was detected with color flow mapping in three (9.4%) patients immediately after the procedure. In 22 patients with 12 months follow-up, trivial residual shunt was present in only one (4.5%). Structural modification of the device was detected in one patient, however with complete ASD occlusion and neither clinical nor hemodynamic disturbance. CONCLUSION: The Sideris' device is safe and efficient for ASD occlusion in selected patients. Although there is a relatively high incidence of residual shunt immediately after the procedure, the shunt itself tends to become smaller or disappear during the follow-up and it does not preclude clinical and hemodynamic improvement.

[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.