Effects of low doses of inhaled nitric oxide combined with oxygen for the evaluation of pulmonary vascular reactivity in patients with pulmonary hypertension.

The purpose of this study was to evaluate the hemodynamic effects of inhaled nitric oxide in oxygen (NO + O2) in patients with pulmonary hypertension. Eighteen patients (median age 31.5 months) with pulmonary hypertension inhaled through a mask 100% O2 and 20 parts per million NO + inspired O2 fraction (FiO2) at 0.4. Hemodynamic measurements were made at baseline and after O2 and NO + O2 administration. The pulmonary vascular resistance index decreased after inhalation of O2 and NO + O2 (p = 0.0018 and p = 0.0003, respectively), the decrease being significantly greater after NO + O2 (p = 0.0311). Concerning the transpulmonary pressure gradient, a reduction occurred in values after O2 and NO + O2 inhalation when compared with baseline values (p = 0.0014 and p = 0.0008). In patients with congenital heart disease, an increase occurred in pulmonary blood flow after O2 (p = 0.0089) and NO + O2 (p = 0.0019) compared with baseline values, and an increase also occurred in the pulmonary/systemic blood flow ratio after NO + O2 (p = 0.0017). The main side effect related to NO + O2 was pulmonary congestion in 3 patients. Low doses of NO combined with O2 demonstrated a selective pulmonary vasodilator response in patients with pulmonary hypertension. Despite its use for testing pulmonary reactivity, inhalation of NO + O2 should be carefully administered because of the potential risk of pulmonary congestion.

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.

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.

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.

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.

[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.

Implante de Stent dentro de Stent recém implantado em ponte de veia safena para otimizaçäo do resultado angiográfico / Stenting a Stent in saphenous vein graft to optimize the angiogrphic result

Mulher de 60 anos, com angina progressiva e revascularizaçäo do miocárdio, há oito anos, com ponte de veia safena para coronária direita e anastomose de artéria mamaria esquerda para artéria descendente anterior. Submetida a implante de stent Gianturco-Roubin II em terço proximal da ponte de veia safena para artéria coronária direita, com resultado insatisfatório pela persistência de lesäo residual, provavelmente, decorrente de prolapso para dentro da luz de material aterosclerótico através dos coils. Foi implantado outro stent (Palmaz-Schatz biliar) dentro do stent GRII com sucesso e ótimo resultado angiográfico. Um 2§ stent Palmaz-Schatz biliar foi implantado em lesäo distal no corpo da ponte, ultrapassando os dois stents, anteriormente implantados, com sucesso. Em algumas situaçöes, implante de stent dentro de outro stent é recurso útil para otimizaçäo de resultado angiográfico do implante de um stent.

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.

[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.

[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.

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.

[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.

[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.

[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.

[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.

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.

[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.

[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.