PAD is associated with increased risk of cardiovascular morbidity and mortality. Medical therapy is targeted toward systemic atherosclerosis and risk factor modification. Exercise therapy is the most studied and validated therapy for symptomatic peripheral arterial disease.
Alport syndrome (AS) is a genetic disorder due to inheritance of genetic mutations which lead to production of abnormal type IV collagen. AS has been associated with renal, auditory, and ocular diseases due to the presence of abnormal alpha chains of type IV collagen in the glomerulus, cochlea, cornea, lens, and retina. The resulting disorder includes hereditary nephritis, corneal opacities, anterior lenticonus, fleck retinopathy, temporal retinal thinning, and sensorineural deafness. Aortic and aortic valve pathologies have been described as extrarenal manifestations of AS in multiple case reports. One case report described intramural hematoma of the coronary artery. We report the first case of true spontaneous coronary artery dissection (SCAD) with an intimal flap as a very rare manifestation of AS. The patient is a 36-year-old female with history of AS with chronic kidney disease, hypertension, and obesity who presented to the emergency room with acute onset of substernal chest pain radiating to her neck and arms. Troponin was elevated, and ECG showed transient 1 mm ST-segment elevation in the inferior leads. Subsequent coronary angiography revealed localized dissection of the left circumflex artery. Percutaneous coronary angioplasty was performed and her symptoms improved. This case illustrates that SCAD may be a manifestation of AS patients with chest pain.
Background: In-hospital and 30-day mortality rates of endovascular repair of thoracoabdominal aortic aneurysms shows a significant improvement over open surgery, although we are not seeing a significant difference at 1 year. We assess the hypothesis that a greater mural thrombus ratio within the aorta could function as an indicator of postoperative mortality.
Methods:The mural thrombus ratio and preoperative comorbidities of 100 consecutive patients from a single center undergoing endo-debranching between 2012 and 2019 were evaluated. Logistic regression, survival analysis, and decision tree methods were used to examine each variable's association with death at 1 year.Results: At the time of analysis, 73 subjects had 1-year outcomes and adequate imaging to assess the parameters. At 1 year, the overall survival for all subjects was 71.2% (21 died, 52 survived). For patients with a favorable mural thrombus ratio (n ¼ 36), the overall 1-year survival was 86.1% (5 died, 31 survived). The subjects with an unfavorable mural thrombus ratio (n ¼ 37), had an overall 1 year survival of 56.8% (16 died, and 21 survived). The only preoperative mortality factor that was statistically significant between the subjects with an unfavorable mural thrombus ratio was age of the patient. The survival for subjects 75 years and older with an unfavorable mural thrombus ratio was 90% (one died, nine survived) vs only 44.4% survival for subjects less than 75 years with an unfavorable mural thrombus ratio (15 died, 12 survived).Conclusions: This study examined whether a patient's mural thrombus ratio may be an indicator of 1-year survival. These findings suggest that the combination of a patient's aortic mural thrombus ratio and age can function as a preoperative indicator of their underlying cardiac reserve. Identifying patients with low cardiac reserve and fitness to handle the increased cardiac demands owing to the physiologic response to extensive aortic stent grafting before undergoing aortic repair may allow for modification of preoperative patient counseling and postoperative care guidelines to better treat this patient population.
The dextro-transposition of great arteries (d-TGA) is a rare, congenital, cyanotic heart disease and there is a paucity of data regarding long-term cardiovascular outcomes. We present a rare case of non-ST-elevation myocardial infarction (NSTEMI) in a patient with surgically repaired d-TGA. A 43-year-old male who had previously undergone a Mustard atrial switch palliative procedure presented with chest pain and diaphoresis and was diagnosed with NSTEMI. A coronary angiogram revealed a small, underdeveloped, left anterior descending and a large, left circumflex coronary artery with an acute embolic lesion. The embolic lesion was secondary to atrial fibrillation and was successfully treated with aspiration thrombectomy. This case highlights the variations in coronary anatomy in surgically repaired d-TGA and the importance of recognizing the potential for long-term complications in these cases.
Rosai-Dorfman disease is a rare, idiopathic disorder of histiocyte proliferation. We describe a case of a 59-year-old woman who presented with heart failure symptoms from a large pericardial mass causing constrictive pericarditis. Pathologic findings including immunohistochemistry suggested the diagnosis. She was treated with corticosteroids and improved clinically. (
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