1/57. Treatment of the bradycardia-tachycardia syndrome with permanent demand pacing.The bradycardia-tachycardia syndrome (paroxysmal supraventricular tachycardia alternating with sinus bradycardia and episodes of sinus node arrest) has previously presented a complicated therapeutic dilemma when excitatory and suppressive drugs have been utilized. A patient with this syndrome successfully treated with a permanent ventricular transvenous demand pacemaker is presented. Various aspects of this syndrome as well as facets of diagnosis and treatment have been reviewed and discussed. Significant underlying cardiac disease was ruled out in this patient by the usual diagnostic methods including left heart catheterization and coronary angiography. An interesting possibility of the relationship of vagal stimulation secondary to hiatus hernia as an etiologic factor in this syndrome has been discussed. The opinion is expressed that the currently preferred method of treatment is the insertion of a permanent transvenous pacemaker alone or in conjunction with antiarrhythmic drugs, preferably digitalis and propranolol.- - - - - - - - - - ranking = 1keywords = catheterization (Clic here for more details about this article) |
2/57. lemierre syndrome in an immunocompromised patient.lemierre syndrome or septic thrombophlebitis of the internal jugular vein is a potentially life-threatening complication. This condition may result from oropharyngeal infection, central venous catheterization, and intravenous drug abuse. Immunocompromised patients and individuals with systemic disease are at higher risk of developing the syndrome. We present here a case of septic jugular vein thrombosis in an adolescent with systemic lupus erythematosus. The etiological factors, pathogenesis, and diagnostic and therapeutic measures are discussed.- - - - - - - - - - ranking = 1keywords = catheterization (Clic here for more details about this article) |
3/57. Atypical Bland-White-Garland syndrome with stenosis of the origin of the left coronary artery: catheter intervention after mammary artery bypass stenosis and residual fistula to the pulmonary trunk.A 16-year-old boy with anomalous origin of the left coronary artery from the pulmonary artery, Bland-White-Garland syndrome, underwent a mammary artery bypass grafting to the left coronary artery (LCA) together with closure of the stenosed origin of the left coronary artery. A residual LCA to pulmonary artery fistula and stenosis of the shunt at the implantation site developed that resulted in a dominant perfusion of the LCA from the right coronary artery. Interventional catheterization was performed with stenting of the LCA mammary artery anastomosis and subsequent coil occlusion of the residual fistula. After this intervention the LCA was exclusively perfused by the mammarian bypass with no residual leak to the pulmonary artery.- - - - - - - - - - ranking = 1keywords = catheterization (Clic here for more details about this article) |
4/57. adult type anomalous origin of the left coronary artery from the main pulmonary artery: report of one case.Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA syndrome) is a rare disease. Most of the cases suffer from myocardial infarction, congestive heart failure or even death during the early infantile period, and thus are called "infantile type". We present one five-year-old girl with ALCAPA syndrome, adult type, who was initially suspected to be a case of atypical Kawasaki disease (KD) complicated with coronary arterial aneurysms. Due to persistent dilatation of the coronary artery after medical treatment for one year and a turbulent flow noticed between the left coronary artery (LCA) and the main pulmonary artery (MPA), cardiac catheterization was arranged. The diagnosis was established from the coronary cineangiography in spite of a direct connection between the aorta and LCA revealed from the echocardiogram. Thereafter open heart surgery was undertaken. diagnosis of ALCAPA syndrome can be made by echocardiogram, but this is not fully satisfactory. Selective coronary cineangiography remains the definitive diagnosis. Although the patients of adult type are more asymptomatic, surgical correction should be undertaken as soon as the diagnosis is established since a substantial risk is still present.- - - - - - - - - - ranking = 1keywords = catheterization (Clic here for more details about this article) |
5/57. Multiple intrapulmonary arteriovenous fistulas in childhood.Pulmonary arteriovenous fistulas (AVFs) are a rare but recognized cause of cyanosis in childhood. Lesions may be acquired as in hepatopulmonary syndrome or they may be congenital, particularly in association with certain multisystem disorders. Large fistulas are more common than multiple small connections. Two cases. both boys, presenting in the first decade of life are described. "Bubble" echocardiography was the most telling investigation and strongly suggested the presence of AVFs in both cases. Each patient then underwent cardiac catheterization, which demonstrated normal pulmonary artery pressure and diffuse pulmonary telangiectasis. Both patients were treated effectively with nifedipine and continue with this mode of therapy.- - - - - - - - - - ranking = 1keywords = catheterization (Clic here for more details about this article) |
6/57. Esophageal varices in congenital heart disease with total anomalous pulmonary venous connection.Total anomalous pulmonary venous connection (TAPVC) is an uncommon congenital anomaly in which the anatomical presentations vary widely among patients. We hereby present two newborns with TAPVC associated with asplenia syndrome; both had severe esophageal varices due to infradiaphragmatic pulmonary venous drainage. Ultrafast computed tomography (CT) scanning was superior to color Doppler echocardiography and cardiac catheterization as it provided a detailed portrait of the pulmonary drainage. The remarkable radiographic manifestations are presented.- - - - - - - - - - ranking = 1keywords = catheterization (Clic here for more details about this article) |
7/57. The bronchial cast syndrome after the fontan procedure: further evidence of its etiology.We describe a three-year-old boy who presented with recurrent expectoration of bronchial casts six months following creation of a fenestrated lateral tunnel Fontan circulation for pulmonary atresia with intact ventricular septum. Cardiac catheterization demonstrated elevated central venous pressure with two areas of stenosis within the Fontan circuit, specifically at the junction of the right superior caval vein and the right pulmonary artery, and between the atrial baffle and the right superior caval vein. Insertion of Palmaz stents in these areas resulted in a reduction in central venous pressure, and a transient reduction in production of casts. Eight weeks after catheterization, however, he produced further casts, which resulted in hypoxia, respiratory arrest and death. We reviewed the autopsied specimens obtained from patients with the Fontan circulation over an eleven-year period at our institution in order to ascertain the prevalence of subclinical production of bronchial casts. We found no casts in the thirteen patients examined. Hemodynamic assessment is vital in all patients who develop this syndrome, and should be the primary focus of investigation, rather than solely directing efforts at lysis of casts.- - - - - - - - - - ranking = 2keywords = catheterization (Clic here for more details about this article) |
8/57. Echocardiographic findings in Uhl's anomaly. Demonstration of diastolic pulmonary valve opening.The echocardiographic findings in a case of Uhl's anomaly, or congenital hypoplasia of the right ventricular myocardium, are reported. Diastolic opening of the pulmonary valve is described. Comparison is made with echocardiograms in Ebstein's disease of the tricuspid valve, and it is suggested that echocardiography can help in differentiating these two entities. In addition to the pulmonary valve finding, increased right ventricular dimension, delayed tricuspid closure and abnormality (prolapse) of the mitral valve were noted. The echocardiographic findings are compared with cardiac catheterization data.- - - - - - - - - - ranking = 1keywords = catheterization (Clic here for more details about this article) |
9/57. gyrate atrophy of the choroid and retina with hyperornithinemia.A case of gyrate atrophy of the choroid and retina and hyperornithinemia in a 28-year-old man was subjected to extensive clinical and biochemical investigation. The familial occurrence of the ocular disease and of abnormality of amino acids was unique to this patient, being absent in parents and siblings. He presented with progressive visual loss, and had cataracts and large areas of peripheral lacumar atrophy. Clinically there was no other abnormality. However, he was hyperuricemic and had an abnormal electroencephalogram. Despite otherwise normal biochemical indices of hepatic, renal, and muscle function, selective catheterization of an artery, the hepatic vein, the renal vein, and a deep forearm vein showed all of these circulatory beds to be producing ornithine according to arteriovenous difference measurements. cerebrospinal fluid and urine contained increased amounts of ornithine. Though electromyography was normal a muscle biopsy specimen was abnormal. Clinical tests including arginine loading, glucose tolerance testing, and other measurements of blood variables provided inferences as to the metabolic locus of the abnormality. The syndrome is a systemic multiorgan disorder in which the choriod and retina would appear to be target organs and the hyperornithinemia to be of as yet undetermined cause and pathogenic significance.- - - - - - - - - - ranking = 1keywords = catheterization (Clic here for more details about this article) |
10/57. Horseshoe lung with pseudo-ring-sling complex.We report on a 1-month-old boy who presented with a "horseshoe" lung, complete "O" rings of the trachea, and an unusual course of the left pulmonary artery mimicking a left pulmonary artery sling. Computed tomography, cardiac catheterization, and bronchoscopy were performed to confirm the rare coexistence of these anomalies. To our knowledge, such a combination of anomalies has not been reported in the English-language literature.- - - - - - - - - - ranking = 1keywords = catheterization (Clic here for more details about this article) |
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