Cases reported "Dilatation, Pathologic"

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1/93. Left-ventricular inflow obstruction due to a dilated coronary sinus mimicking cor triatriatum.

    Persistence of the left superior vena cava with drainage to the coronary sinus is a common congenital anomaly. We report an infant with such a malformation associated with marked enlargement of the coronary sinus, which produced partial supramitral obstruction and consequently impairment to the left-ventricular inflow. The patient pre-sented with cardiac failure in infancy and features mimicking cor triatriatum. Surgical relief of the supramitral obstruction resulted in immediate reversal of the pulmonary hypertension, with clinical improvement. This rare entity, only once previously reported, is an unusual cause of pulmonary hypertension in infancy.
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ranking = 1
keywords = coronary
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2/93. Distensible venous malformations of the orbit: clinical and hemodynamic features and a new technique of management.

    OBJECTIVE: To investigate distensible venous malformations of the orbit (DVMO) as part of a spectrum of orbital vascular malformations, including some that involved periorbital skin, extraorbital sites (central nervous system or nasal sinuses), or combinations of these. The authors also investigated the effectiveness of a new technique of management for selected cases. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: Thirty patients had distensible venous anomalies, of which four were combined distensible venous-lymphatic vascular malformations. Distensible lesions were defined as those showing clinical or radiographic expansion with valsalva maneuver or when the head was placed in a dependent position. These lesions were then classified as superficial (anterior to the equator of the globe), deep (posterior to the globe's equator), combined (deep and superficial), or complex (with intracranial or major extraorbital involvement). INTERVENTION: Surgery was performed on 15 patients (50%), mainly for pain or for cosmetic indications. Six patients underwent this new technique, which involved intraoperative direct venography with control of outflow via pressure at the superior or inferior orbital fissure. The venous malformation was then embolized (by use of cyanoacrylate glue) and excised. RESULTS: The mean age at presentation was 28.2 years (range, 8 months to 75 years). Sixty-six percent of cases involved the left orbit. Superior and medial orbital involvement was most common. Three cases (10%) were classified as superficial, and 13 (43%) as deep. Six patients (20%) had combined superficial and deep components. Eight (27%) had major extraorbital involvement (4 intracranial, 2 facial, and 2 paranasal sinus). Direct venography demonstrated complex multichannel anomalies draining to various sites, including the face and pterygopalatine fossa, without necessarily having a direct connection to the major orbital venous circulation. CONCLUSIONS: Distensible venous malformations of the orbit are part of a spectrum of developmental venous malformations that may be localized to the orbit or involve it as part of a more extensive lesion. The authors describe their clinical and radiologic features and report a new technique of management for selected cases. This method of vascular isolation and embolization of lesions may greatly facilitate excision.
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ranking = 0.0026242895556854
keywords = circulation
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3/93. Coronary ectasia in familial hypercholesterolemia: histopathologic study regarding matrix metalloproteinases.

    A 39-year-old male heterozygous familial hypercholesterolemia patient with marked ectasia over the entire coronary artery tree had been treated with several kinds of lipid-lowering single or combined drug therapies using clofibrate, compactin, cholestyramine, probucol, and pravastatin, and LDL-apheresis. During the 19-year follow-up, he suffered myocardial infarction three times and some of the ectatic coronary segments became enlarged, others narrowed, and one of them occluded in spite of the treatment. At the age of 58, he died after a fourth cardiac attack and subsequent cardiogenic shock. The autopsy indicated that his three coronary arteries showed diffuse ectatic changes and the largest lumen diameter of the left anterior descending artery was 25 mm, of the circumflex artery 12 mm, and of the right coronary artery 13 mm. The ectatic artery wall was not thick and the major part of the lumen was occupied by organized thrombi. Microscopic examinations showed that the larger the diameter of the lumen, the more severe the structural damage of the intima and tunica media and the larger the number of infiltrated cells, including lymphocytes, macrophages, and plasma cells. Immunoreactivity against matrix metalloproteinase (MMP)-1, and MMP-2 was observed in smooth muscle cells, macrophages, lymphocytes, and endothelial cells of the vasa vasorum or neovasculature. MMP-9 immunoreactivity was also localized in intimal foamy macrophages and round cells (macrophages and lymphocytes) of the media and adventitia. MMP-1 increased with the lumen diameter of the ectatic arteries. The ratio of immunoreactivity against both MMP-2 and MMP-9 to that against tissue inhibitor of metalloproteinase (TIMP)-2 also increased with the lumen diameter, but it no longer increased when the diameter was over 10 mm. These observations suggest that the MMP-TIMP system appears to play a significant role in the development of coronary ectasia
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ranking = 0.83333333333333
keywords = coronary
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4/93. Transluminal extraction catheter atherectomy for the treatment of acute occlusion of an ectatic coronary artery.

    Thrombotic occlusion of an ectatic coronary artery may not respond to thrombolytic therapy or balloon angioplasty, since the infarct-related vessel contains a significant amount of thrombus. A patient with acute myocardial infarction of an ectatic right coronary artery that was occluded by a heavy clot burden is described. The patient was treated successfully with transluminal extraction catheter atherectomy and results were confirmed by intravascular ultrasound.
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ranking = 1
keywords = coronary
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5/93. Atresia of the proximal right coronary artery in association with coronary artery fistula.

    A 2-year-old boy presented for evaluation of a systolic murmur. Echocardiogram demonstrated dilated left main and right coronary arteries. Selective injection of the left coronary artery confirmed dilation of the left main and anterior descending coronary arteries. The left anterior descending coronary artery subsequently filled the mid portion of the right coronary artery retrograde, which emptied into a blind fistula. The orifice of the right coronary artery was atretic, with no filling from the aortic root or the fistula.
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ranking = 2.3333333333333
keywords = coronary
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6/93. Clinical significance of coronary arterial ectasia.

    In a study group of 2,457 consecutive patients undergoing cardiac catheterization, 30 patients had coronary arterial ectasia, an irregular dilatation of major vessels up to seven times the diameter of branch vessels. The frequency of hypertension, abnormal electrocardiogram and history of myocardial infarction was greater than that in a control group with obstructive coronary artery disease. patients with ectasia did not differ from patients with obstructive disease in sex, age, prevalence of angina or presence of metabolic abnormalities. Six deaths occurred in the group with ectasia during a mean follow-up period of 24 months (annual rate of 15 percent). Extensive destruction of the musculoelastic elements was evident, resulting in marked attenuation of the vessel wall. The short-term prognosis in this group is the same as in medically treated patients with three vessel obstructive coronary artery disease.
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ranking = 1.1666666666667
keywords = coronary
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7/93. Multiple coronary artery aneurysms combined with abdominal aortic aneurysm.

    Coronary artery aneurysm (CAA) is defined as coronary dilatation which exceeds the diameter of a normal adjacent segment or the diameter of the patients's largest coronary vessel by as much as 1.5 times. It is an uncommon pathology with a frequency of 1-4% in routine autopsies or coronary angiographies. atherosclerosis plays an important role in the development of CAA, and it may be a predominant cause in the majority of patients. However, the timing of surgical intervention and the treatment options for CAA are still controversial. In this report, we present a patient who had multiple CAAs of all main coronary arteries and abdominal aortic aneurysm. Different treatment modalities and indications are also discussed.
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ranking = 1.3333333333333
keywords = coronary
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8/93. Effectiveness of an antiplatelet agent for coronary artery ectasia associated with silent myocardial ischemia.

    A 74-year-old Japanese male was referred to our hospital because of an abnormal electrocardiogram. The electrocardiogram revealed tall P waves in leads II, III, and aV(F). echocardiography disclosed hypokinesis extending from the anteroseptal region to the apex. iodine-123 15-(p-iodophenyl)-3-(R,S)-methylpentadecanoic acid (123-BMIPP) scintigraphy revealed reduced uptake from the anteroseptal region to the apex. Coronary arteriography demonstrated diffuse dilatation of the right and left coronary arteries without organic stenosis, and left ventriculography showed hypokinesis at the same area. Furthermore, the coronary flow reserve in the left anterior descending artery was decreased. He was treated with an antiplatelet agent. Ten months later, the left ventriculography, 123I-BMIPP scintigraphy findings and coronary flow reserve were normalized. These findings demonstrate that antiplatelet therapy may be useful in the preservation of left ventricular function in patients with coronary artery ectasia.
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ranking = 1.3333333333333
keywords = coronary
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9/93. Dilated coronary sinus mimicking posterior left ventricular aneurysm.

    Dilation of the coronary sinus is mostly a congenital lesion. As acquired lesion it was found to be associated with poor left ventricular function. In the present case an angiographic diagnosed posterior left ventricular aneurysm was during surgery found to be a dilated coronary sinus. Preoperative left ventricular function was normal.
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ranking = 1
keywords = coronary
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10/93. Aortic regurgitation with dilation of ascending aorta and right coronary artery occlusion by a rudimentary aortic cusp.

    Occlusion of a coronary artery ostium, especially that of the right by an aortic cusp, is a rare congenital anomaly. We had the experience of dealing with an adult patient with aortic regurgitation due to a rudimentary aortic cusp that also occluded the right coronary ostium. We performed composite graft replacement because the patient also had coexistent dilation of the ascending aorta. Postoperative course was uneventful.
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ranking = 1
keywords = coronary
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