Cardiovascular changes following acute and chronic chemical lesions of the dorsal periaqueductal gray in conscious rats.

This study was carried out to investigate the effects of chemical lesions of dorsal periaqueductal gray (DPAG) on resting arterial pressure (AP) and heart rate (HR) as well as on cardiac baroreflex of conscious normotensive rats. Lesions were performed by bilateral microinjections of 150 mM NMDA into the DPAG (DPAG-lesion group). Controls were similarly injected with 165 mM NaCl (DPAG-sham group). Animals with chronic lesions confined only to the superior colliculus (SC-lesion group) were also used as controls of DPAG-lesion. Cardiovascular parameters were recorded 1 or 7 days after the microinjections of NMDA in acute and chronic groups, respectively. Cardiac baroreflex was assessed by measuring the HR responses to the intravenous injection of phenylephrine or sodium nitroprusside. Baroreflex was estimated by sigmoidal curve fitting of HR responses. An increased baroreflex gain was observed in chronic DPAG-lesion rats compared to both DPAG-sham (p < 0.01) and SC-lesion (p < 0.05) chronic groups. The chronic DPAG-lesion group showed also an elevation of both the tachycardia (p < 0.05) and bradycardia (p < 0.01) plateaus compared to chronic DPAG-sham rats, while the SC-lesion group showed an elevation of the bradycardia plateau only (p < 0.01). Similar results on baroreflex function were observed following acute lesion of the DPAG, i.e. an increase in baroreflex gain (p < 0.01) and the elevation of both tachycardia (p < 0.05) and bradycardia plateaus (p < 0.01) compared to the acute DPAG-sham group. Resting AP and HR did not differ among the chronic groups. In contrast, the acute lesion of the DPAG produced a reduction in AP (p < 0.01) accompanied by an increase in HR (p < 0.01). The present data suggest that the DPAG is involved in the tonic and reflex control of AP and HR in conscious rats. In addition, the SC seems to contribute to the baroreflex cardioinhibition.

[Neonatal heart failure and Marfan syndrome]. / Insuficiência cardíaca neonatal e síndrome de Marfan.

We report the case of a neonate admitted to the hospital in the 4th day of life in severe heart failure due to aortic and mitral regurgitation with a largely dilated aortic root. The associated skeletal features involving the superior and inferior limbs as well as the thorax, and joint hypermobility, allowed the clinical diagnosis of Marfan syndrome. Despite favorable initial response to medical therapy, sudden deterioration led to death two weeks after birth. Typical necroscopic findings were confirmed and the case is considered the most severe clinical manifestation possible to be found in this syndrome.

[Congenital rubella syndrome and the occurrence of congenital heart disease]. / Síndrome da rubéola congênita e a ocorrência de cardiopatias congênitas.

Aiming to document the incidence and type of associated congenital heart disease, 20 children affected with the congenital rubella syndrome have been evaluated during a 5 year period, starting 1988. Congenital heart disease was detected in 45% of the cases. Persistent ductus arteriosus was the most frequent finding, followed by ventricular and atrial septal defects. Hearing disturbances occurred in 83% of the 12 cases studied. Ophthalmic lesions appeared in 69% of the 13 cases analysed, congenital cataracts being the most frequent one. An association of 66% was found between ophthalmic and heart lesions. Regarding the nutritional index, 70% of the cases were situated between the 25th and 50th percentile. The relevant incidence of congenital defects and the documentation of an important number of cases in a short period of time point out to a significant prevalence of the syndrome with imperative need improve the vaccination programs.

[Intrapericardial teratoma. A case report and a literature review]. / Teratoma intrapericárdico. Relato de caso e revisão da literatura.

A case of newborn intrapericardial teratoma is reported. The clinical, echocardiographic, tomographic and histologic features are described, and also, the therapeutic options. The newborn was submitted to surgical excision of the intrapericardial tumor and has a clinical follow-up greater than four years.

[Congenital isolated hypoplasia of the right ventricle]. / Hipoplasia congênita isolada do ventrículo direito.

Two cases of congenital trabecular hypoplasia of the right ventricle are reported. In the first, the neonatal diagnosis was missed and the child did well until the 13th month of life when a modified Blalock-Taussig shunt was done because of increasing cyanosis. Outcome was good until the 4th year of life when symptomatic atrioventricular block was detected in an emergency situation. A bidirectional Glenn anastomosis and pacemaker implantation were successfully carried out after clinical establization and the child is doing well up to now. The second case presents the disease with its worst features: severe cyanosis and acidosis in the first day of life. A modified Blalock-Taussig shunt was performed and death occurred soon after the operation.

[Cardiac evaluation in children with suspected heart disease. Preliminary results in 2000 patients]. / Avaliação cardiológica em crianças com suspeita de cardiopatia. Resultados preliminares em 2000 pacientes.

Two thousand consecutive children have been evaluated for suspected heart disease in a 27-month period. The main reasons for referral were: murmur (70%), precordial pain (9.5%), suspicion of arrhythmia (8.5%) and breathless (5%). Five hundred and six (25%) cases did not complete the investigation and the results were not computed. A final diagnosis was obtained based on the reason for referral and the main conclusions were: 1) a high incidence of normality was found: murmur (83%), pain (98%), arrhythmia (97%) and breathless (94%); 2) heart disease is unlikely when other referral reasons were analyzed; 3) 14% of the children were considered abnormal and the necessity of therapeutic procedures was 0.8%. A pediatric cardiology outpatient clinic in a public setting seems to be justifiable in the region, due to the high current demand.

[Management of children with heart murmurs. Diagnostic importance of noninvasive complementary tests]. / Abordagem da criança com sopro cardíaco. Importância diagnóstica dos exames complementares não-invasivos.

PURPOSE: To determine the benefits of noninvasive tests in the characterization of heart disease in children referred due to heart murmur. METHODS: Two hundred and thirty three consecutive children were fully examined with the aid of the electrocardiograma (ECG), chest X-ray and echocardiogram (ECHO). The patients were divided in 2 groups according to the initial diagnosis of innocent murmur (n = 23) and pathological murmur (n = 210). A comparison was made between the initial and final diagnosis after the noninvasive tests. Statistical analysis was employed through the qui-square test. RESULTS: The initial diagnosis did not change after ECG. The chest X-ray induced the diagnosis of idiopathic dilatation of the main pulmonary artery in one case and of cardiomyopathy in 8 cases, confirmed in only one. After ECHO, the initial diagnosis of innocent murmur was maintained in 70% of the cases, while 30% had some form of heart disease. In 80% of the pathologic murmur group, the diagnosis did not change after ECHO, while 20% were considered as having an innocent murmur. In the 2 groups together, the concordance index between initial and final diagnosis after ECHO reached 90%. Statistically, a non-significant difference was found between the 2 groups. CONCLUSION: Careful clinical examination is mandatory in the initial evaluation of children with heart murmur. ECG and chest X-ray does not change the initial clinical diagnosis. After ECHO, the diagnosis changing was low (10%), as long as clinical mistakes are eliminated. The initial diagnosis of innocent murmur excludes the necessity of noninvasive tests with a small possibility of error. EKG, chest X-ray and ECHO should be reserved to cases with obvious or persistent suspicious heart disease after clinical examination.

[Incidence of heart defects in Down syndrome]. / Incidência de cardiopatias congênitas na Síndrome de Down.

Eighty-six patients with Down Syndrome were studied with the main purpose of quantifying the incidence of congenital heart defects and the risk of occurrence according to the mothers age. Thirty-eight patients had the cariotypes determined, 35 of them having trissomy of chromosome 21 and translocation in 3 cases. Congenital heart disease was found in 44 (51%) of the patients, the most common one being ventricular septal defect. An important incidence of Fallot's tetralogy was also found (20%). These 86 children were submitted to 41 surgical procedures, most of them on the cardiovascular system. The maternal mean age was 33 -/+ 8.6 years and the estimated risk of Down Syndrome was 1/590, a lower value than the one reported in other studies.

[Congenital cerebral arteriovenous fistula. Diagnostic peculiarity and surgical repair in an infant with heart failure]. / Fístula arteriovenosa cerebral congênita. Peculiaridade diagnóstica e correção cirúrgica em lactente com insuficiência cardíaca.

A case of 3 month-old-boy with Down's syndrome and heart failure due to a localized arteriovenous fistula of the cranial vessels is reported. Clinical diagnosis was made after cranial bruit detection based on a retroauricular thrill incidentally felt by the mother. The diagnosis was confirmed by digital subtraction angiography and a good result was achieved by means of embolization and surgical ligation of the fistula. Three years after the operation the patient is asymptomatic, on no medication and with normal magnetic resonance image of the cranial vessels.

[Delayed follow-up after aortic coarctation correction. Subsequent surgical reoperation]. / Seguimento tardio após correção de coarctação da aorta. Reintervenção cirúrgica subseqüente.

PURPOSE: To determine the frequency and main features of subsequent cardiovascular surgery in patients operated on for coarctation of the thoracic aorta. METHODS: One hundred and five patients operated on for coarctation of the aorta with a mean follow-up period of 14 years had their notes analysed. The patients were divided in 4 groups according to age at correction of the coarctation. The incidence of recoarctation repair and other cardiovascular operations were noted. RESULTS: Surgical morbidity was important: 33%. Recoarctation occurred in 14% of the cases, mainly in those who had the coarctation resected during the first year of life. Operation for other cardiovascular defects was necessary in 24.5% of the cases. Among these, correction of left-to-right shunts was done in 50% of the patients who had the coarctation resected in the first year of life. Relief of aortic stenosis was the most frequent procedure (73%), tends to be more frequent the other is the patient at coarctation repair and more than one procedure may be necessary in some cases. CONCLUSION: Reoperation is frequent in the long term of patients operated on for coarctation of the aorta. The elective coarctation should be repaired after the first year of life hoping to avoid recoarctation. Routine follow-up is advisable for all patients aiming to detect residual left-to-right shunts and left ventricular outflow tract obstruction. Family counseling regarding prognosis after coarctation resection is recommended.

Congenital coarctation of the lower thoracic aorta. A rare surgically correctable cause of hypertension in the young--case report.

We report a case of congenital coarctation of the lower thoracic aorta. The patient, a 15-year-old man, presenting with the signs of classical coarctation, had the diagnosis confirmed by an aortography. A good surgical result was achieved by means of resection of the internal shelf and aortoplasty using a bovine pericardium patch. One year after the operation the patient has normal blood pressure with good femoral pulses.

[Aortic coarctation associated with a large aneurysm]. / Coarctação da aorta associada a grande aneurisma.

The case of a symptomatic 16-year-old girl, referred with the diagnosis of coarctation of the aorta is presented. Aortography showed a severe coarctation and a huge aneurysm distal to the coarctation. Successful operation was performed with resection of the coarctation and aneurysm and interposition of a tube of bovine pericardium for the anastomosis. Two months after the operation the blood pressure was normal and a 10 mm/Hg gradient was detected by doppler-echocardiography.

[A congenital arteriovenous fistula between the subclavian artery and vein. Its successful surgical correction in an infant]. / Fístula artério-venosa congênita entre a artéria e veia subclávia. Correção cirúrgica favorável em um lactente.

The case of an asymptomatic 6-month-old boy, first seen due to a right arm edema is described. A continuous murmur heard at the right infraclavicular area, cardiomegaly plus increased flow to the lungs on the chest X-ray were associated with an isolated right subclavian artery-to-vein fistula diagnosed at angiography. Successful ligation of the fistula was done at operation with normalization of the arm circumference and heart size on the chest X-ray at 4 years follow up.

[Transient atrioventricular conduction disorder: a potential cause of sudden death in myocarditis due to varicella. A case report]. / Distúrbio transitório da condução atrioventricular: uma causa potencial de morte súbita na miocardite por varicela. Relato de caso.

The case of a patient with varicella skin lesions presenting with heart failure at 4 years of age is described. A transitory atrioventricular conduction disturbance due to myocardial impairment was documented during hospital stay. Despite the clinical improvement with conventional therapy, the patient died from respiratory failure due to a lung infection but with an almost normal electrocardiogram. Necropsy showed myocardial lesions compatible with acute myocarditis. A possible viral specific affinity for the conduction system of the heart is suggested and judged to be a potential cause of death in this disease. Since the actual incidence of varicella myocarditis associated to an atrioventricular conduction disturbance is unknown it seems prudent that every patient with varicella infection should have a cardiac examination during the acute phase of the disease. As the diagnosis of subclinical myocarditis depends essentially on an electrocardiogram, we believe this method should be employed routinely during the acute phase in order to assess myocardial involvement in this disease, which may be a cause of sudden death.

[Fatal hypertrophic cardiomyopathy in a newborn infant. A case report]. / Miocardiopatia hipertrófica fatal no recém-nascido. Relato de caso.

A patient with severe hypertrophic cardiomyopathy and heart failure at six weeks of age is reported. It was believed that myocardial infarction had occurred based on the striking ECG changes and the histologic appearance of the myocardium at necropsy.

[Surgical approach in bacterial endocarditis of the tricuspid valve in children. Report of 2 cases]. / Abordagem cirúrgica na endocardite bacteriana da valva tricúspide em crianças. Relato de dois casos.

The two techniques usually employed for the surgical treatment of tricuspid valve endocarditis are valve replacement or simple valve excision without valve replacement. A third more conservative procedure consists of resection of the vegetation ("vegetectomy") and leaflet repair, if necessary. Valve excision without valve replacement is described in adult patients especially in addicts of intravenous drugs, with normal ventricular function. Two cases of tricuspid valve endocarditis in children with interventricular communication successfully treated by "vegetectomy" and valve excision without valve replacement are reported. In the first case, surgery was performed during acute infection. Contrarily in the other case there was evidence that cure had been obtained, but still the prosthesis was not implanted due to the excellent hemodynamic status at the moment, and to abbreviate bypass time due to the preoperative conditions of the child. The uncommon approach to these two cases of tricuspid valve endocarditis in childhood motivated this presentation.

Myocardial reperfusion by thrombolysis after acute total left main artery occlusion--a case report.

Coronary recanalization with thrombolytic agents is a new therapeutic approach to the treatment of acute myocardial infarction that can be beneficial even to patients in cardiogenic shock. Although few cases have been reported in the literature, treatment of acute occlusion of the left main coronary artery (LMCA) has been made possible by myocardial reperfusion. This communication concerns a patient with acute LMCA occlusion who was successfully treated by thrombolytic therapy with streptokinase followed by revascularization of the myocardium seventy-two hours after reperfusion was achieved.

Myocardial bridging of coronary arteries associated with an impending acute myocardial infarction.

A 57-year-old woman developed severe substernal chest pain radiating to the left arm accompanied by pallor and marked diaphoresis. These symptoms appeared at rest, lasted 45 minutes, and terminated spontaneously. The patient had been treated for mild hypertension during the last 6 months. An ECG tracing obtained at the beginning of treatment was unremarkable. However, an ECG tracing recorded shortly after the end of the symptoms showed T-wave inversion in all anterior leads. Coronary arteriography was then performed and showed no fixed obstructive coronary artery disease. Nonetheless, a lengthened and constricted myocardial bridging of both the left anterior descending coronary artery and its major diagonal branch was detected. Also, the left anterior descending coronary artery was observed to be very short, terminating before the cardiac apex. The left ventricle was hypertrophied. The patient was treated with a beta-blocking agent which eliminated all symptoms. An ECG tracing obtained about three months after the onset of the clinical picture was normal. Our findings suggest that marked myocardial ischemia at rest does occur in patients having myocardial bridges under special circumstances, such as lengthened and constricted myocardial bridging of a short coronary artery which supplies a hypertrophied ventricle. This anomaly should be taken into account as a possible cause of a threatened myocardial infarction, which may be successfully treated with a beta-blocking agent.