1/284. Ocular malformations, moyamoya disease, and midline cranial defects: a distinct syndrome.PURPOSE: To report a 10-year-old girl with developmental anomalies of both optic disks, a chorioretinal coloboma, sphenopharyngeal meningoencephalocele, and moyamoya disease. methods: A full ophthalmologic examination, cranial magnetic resonance imaging and magnetic resonance angiography, and cerebral angiography were performed. RESULTS: The patient had a morning glory disk anomaly and microphthalmos of the right eye and optic nerve hypoplasia and retinochoroidal coloboma in the left eye. She had a midfacial cleft and an episode of seizures and a stroke. magnetic resonance imaging showed a sphenopharyngeal meningoencephalocele. magnetic resonance angiography and cerebral angiography demonstrated a pattern consistent with moyamoya disease. CONCLUSIONS: This patient had a distinct syndrome of optic disk, retinochoroidal, and carotid circulation anomalies with midline cranial defects. The recognition and treatment of the vascular abnormalities and cranial defects may prevent complications such as strokes that may occur during or after general anesthesia.- - - - - - - - - - ranking = 1keywords = circulation (Clic here for more details about this article) |
2/284. Severe cutaneous cholesterol emboli syndrome after coronary angiography.cholesterol embolization syndrome is due to dislodgment of cholesterol crystals from the atherosclerotic plaques lining the walls of major arteries resulting in an occlusion of small arteries. We describe a case of severe cutaneous cholesterol emboli syndrome following repeat coronary angiography showing by our observation that this syndrome is often unrecognized or misdiagnosed and that a better evaluation of risks factors in patients undergoing invasive procedures could prevent this severe complication.- - - - - - - - - - ranking = 29.502330873282keywords = coronary (Clic here for more details about this article) |
3/284. A case of vasospastic angina presenting Brugada-type ECG abnormalities.An electrophysiological study and a provocative test of coronary artery spasm was attempted in a 68-year-old man who was having syncopal attacks and chest pain. His electrocardiogram had the characteristics of brugada syndrome and ventricular fibrillation (VF) was induced by programmed electrical stimulation. ST-segment elevation became exaggerated by procainamide, which could not prevent the induction of VF. coronary angiography revealed no stenotic lesions, and spasm in the left coronary artery was induced by intracoronary administration of acetylcholine with similar chest pain to that experienced before. Under treatment with diltiazem and flecainide, which suppressed the induction of VF, the patient experienced no recurrence of symptoms despite persistent ST-segment elevation. No previous reports have described coronary spasm associated with Brugada-type ECG abnormalities, and patients with syncope should be evaluated carefully.- - - - - - - - - - ranking = 23.601864698626keywords = coronary (Clic here for more details about this article) |
4/284. A first British case of fish-eye disease presenting at age 75 years: a double heterozygote for defined and new mutations affecting LCAT structure and expression.Fish-eye disease is a familial syndrome with corneal opacification, major high density lipoprotein (HDL) deficiency in plasma, significant cholesterol esterification in plasma on non-HDL lipoproteins, generally without premature coronary disease. This first British male case from unrelated British parents had infarcts when aged 49 and 73 years but was asymptomatic at age 81 years, with plasma cholesterol 4.3-7.1 mmol/litre, triglycerides 1.8-2.2 mmol/litre, HDL cholesterol < 0.1 mmol/litre, apolipoprotein a-i < 0.16 g/litre, lipoprotein(a) 0.61 g/litre. cholesterol esterification was impaired using HDL-3 and A-I proteoliposomes but not using VLDL/IDL/LDL. The findings are those of LCAT deficiency with the classic fish-eye disease defect. Most of the 22 reported cases were homozygous or heterozygous for a Thr-Ile mutation at codon 123 of the lecithin:cholesterol acyltransferase (LCAT) gene. This patient was a double heterozygote for this mutation and a second new incompletely defined mutation affecting LCAT expression as defined by reduced mass and activity in plasma.- - - - - - - - - - ranking = 5.9004661746564keywords = coronary (Clic here for more details about this article) |
5/284. Neurofibromatosis, stroke and basilar impression. Case report.Neurofibromatosis type 1 (NF1) can virtually affect any organ, presenting most frequently with "cafe au lait" spots and neurofibromas. Vasculopathy is a known complication of NF1, but cerebrovascular disease is rare. We report the case of a 51-year-old man admitted to the hospital with a history of stroke four months before admission. On physical examination, he presented various "cafe au lait" spots and cutaneous neurofibromas. neurologic examination demonstrated right-sided facial paralysis, right-sided hemiplegia, and aphasia. Computed tomography scan of head showed hypodense areas in the basal ganglia and centrum semiovale. Radiographs of cranium and cervical spine showed basilar impression. Angiography revealed complete occlusion of both vertebral and left internal carotid arteries, and partial stenosis of the right internal carotid artery. A large network of collateral vessels was present (moyamoya syndrome). It is an uncommon case of occlusive cerebrovascular disease associated with NF1, since most cases described in the literature are in young people, and tend to spare the posterior cerebral circulation. Basilar impression associated with this case may be considered a pure coincidence, but rare cases of basilar impression and NF1 have been described.- - - - - - - - - - ranking = 1keywords = circulation (Clic here for more details about this article) |
6/284. A young man with recurrent syncopes, right bundle branch block and ST segment elevation.We report on the case of a 33-year-old man with recurrent syncopes appearing suddenly due to sustained monomorphic ventricular tachycardias. The electrocardiogram (ECG) showed a right bundle branch block pattern and ST segment elevation in the precordial leads V1 to V2, not explained by ischemia, electrolyte disturbances, toxic ingestion, or structural heart disease (coronary and right ventricle angiograms as well as biopsies of the right ventricle were normal). ECG image was compatible with the so-called brugada syndrome, first described in 1992. This entity is very rare. Missed diagnosis can be disastrous because life-threatening ventricular arrhythmias often develop in patients.- - - - - - - - - - ranking = 5.9004661746564keywords = coronary (Clic here for more details about this article) |
7/284. 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 = 5.9004661746564keywords = coronary (Clic here for more details about this article) |
8/284. Acute thrombotic-ischemic coronary syndromes: the usefulness of TEC.Transluminal extraction catheter (TEC) is a percutaneous device that performs simultaneous thrombus aspiration and plaque excision. Clinical indications for its application are acute myocardial infarction, unstable angina, and stable angina caused by atherosclerotic, thrombotic lesions located within native coronary arteries and degenerated saphenous vein grafts. The device is useful in management of ischemic patients with contraindications to either pharmacologic thrombolytics or platelet GPIIb/IIIa receptor inhibitors, and can also effectively be used in combination with these agents. A successful TEC procedure requires careful patient selection, strict adherence to recommended indications, optimal equipment selection, familiarity with mechanical components of the device, full understanding of safe and efficacious techniques for deployment and activation, as well as recognition of unique associated angiographic manifestations such as the "empty-pouch phenomenon." As with other debulking devices, the incidence of restenosis post-TEC appears to be directly related to acute luminal gain at the time of procedure and therefore requires the need for adjunct stenting. This communication describes and illustrates various clinical, technical, and angiographic aspects of TEC procedure in patients with acute ischemic-thrombotic coronary syndromes. Cathet. Cardiovasc. Intervent. 48:406-420, 1999.- - - - - - - - - - ranking = 35.402797047939keywords = coronary (Clic here for more details about this article) |
9/284. Gordon Holmes spinocerebellar ataxia: a gonadotrophin deficiency syndrome resistant to treatment with pulsatile gonadotrophin-releasing hormone.The Gordon Holmes spinocerebellar ataxia syndrome (GHS) is associated with idiopathic hypogonadotrophic hypogonadism (IHH). There are conflicting reports in the literature as to whether the primary neuroendocrine defect is of hypothalamic GnRH secretion, as with most causes of IHH, or of pituitary resistance to GnRH action. Because of the anatomical inaccessibility of the hypophyseal portal circulation, direct measurement of GnRH levels in human subjects is not possible. Previous investigators have attempted to unravel this problem through the use of GnRH stimulation tests and the limitations of this approach may explain the differing results obtained. We used the more physiological approach of treating a male GHS patient for four weeks with GnRH, 7-10 microg/pulse, delivered subcutaneously at 90 minute frequency via a portable minipump. This therapy failed to induce any rise in plasma gonadotrophin and testosterone concentrations. By contrast, eight weeks treatment with exogenous gonadotrophins maintained physiological plasma testosterone concentrations and induced testicular enlargement with induction of spermatogenesis. The data indicate that the primary endocrinopathy in GHS is of pituitary gonadotrophin secretion and not of hypothalamic GnRH. Moreover, the patient did not harbour any mutation of the GnRH receptor gene. Two clinical observations are consistent with progressive involution of gonadotrophic function, rather than a congenital gonadotrophin deficiency. First, the patient's development was arrested at early mid-puberty at the time of original presentation and, second, effective spermatogenesis was induced extremely rapidly during gonadotrophin treatment, suggesting prior exposure of the testes to FSH. Both spinocerebellar ataxia and pituitary dysfunction might thus have been in evolution since late childhood.- - - - - - - - - - ranking = 1keywords = circulation (Clic here for more details about this article) |
10/284. An adult case of bland white garland syndrome with huge right coronary aneurysm.We report the case of a 62-year-old male patient with left main coronary artery originating from the pulmonary trunk, severe mitral insufficiency, and huge right coronary artery aneurysm. He is the oldest such patient among those reported in the literature, surviving to the sixth decade without any anginal symptoms. He is also the first such case with such a huge and calcified right coronary artery aneurysm and a prominent collateral from the noncoronary circulation.- - - - - - - - - - ranking = 48.203729397251keywords = coronary, circulation (Clic here for more details about this article) |
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