• Journal of Chest Surgery
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• v.12 no.3
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• pp.170-173
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• 1979

Since John Davy reported the first well-documented case of occlusive disease involving the branches of the aortic arch in 1839, many similar cases have been reported in literature, especially from oriental countries. The prognosis and symptoms depend on the degree and extent of the occlusive lesions and also on the importance of the arteries affected. The course may progress slowly or rapidly with remissions and exacerbation, and death may result from acute CVA, cardiac failure or pulmonary edema, and renal failure. No medical therapy has been able to alter conclusively the course of the disease, so various surgical procedures have been applied to relieve the obstruction and to prolong the life. We present the case of an 18 year-old female with multiple stenosis of the aorta, and performed the long bypass graft from descending aorta to common lilac artery, and the result was excellent.

• Journal of Chest Surgery
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• v.30 no.6
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• pp.625-630
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• 1997

A 56 years old male patient adklitted to our neurology department because of repeated tingling sensation in right 3, 4, 5th. (infers and weakness on grasping, which were progressively developed re ently. At this time, he had also suffered from claudication in both lower extremities. Carotid angiogram showed that right internal carotid artery was obstructed completely, and both common, both external and left internal carotid arteries had significant stenosis, Concommitantly, aortogram suggested complete obstruction just below the renal arteries. We plamled staged operation for two separated arterial lesions. Both carotid endarterectomy was performed. and we used carotid shunt for left side during operation. Abdominal aortic lesion was operated 2 weeks later We obligately clamped aorta just below the celiac artery and infuse4 kidney perservation solution to pertect kidney during ischemia. Reversed Y bypass graft and kidney perservation was successful despite of 40 minute ischemia. Postoperative courts was uneventful and patient was discharged without any specific problem.

• Clinical and Experimental Pediatrics
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• v.62 no.4
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• pp.144-147
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• 2019

The most common type of refractory hypertension found in children is secondary hypertension, which is a potentially curable disease. Reninoma, a renin-secreting juxtaglomerular cell tumor, is a rare cause of severe hypertension that is usually diagnosed in adolescents and young adults. Surgical resection of the tumor completely cures the hypertension of patients with reninoma. The typical clinical presentation of reninoma includes hypokalemia, metabolic alkalosis, and features secondary to the increased activation of the renin-angiotensin system without renal artery stenosis. We report a case of reninoma in a female adolescent with a typical clinical presentation, in which surgical removal of the tumor completely cured hypertension. We discuss here the clinical features, imaging studies, and immunohistochemical examination of the tumor used to establish the diagnosis of reninoma and for the management of the condition.

• Journal of Genetic Medicine
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• v.18 no.2
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• pp.142-146
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• 2021

Alagille syndrome (AGS) is a rare autosomal dominant inherited disorder, with major clinical manifestations of bile duct paucity, cholestasis, cardiovascular anomaly, ophthalmic abnormalities, butterfly vertebrae, and dysmorphic facial appearance. It is caused by heterozygous mutations in JAG1 or NOTCH of the Notch signaling pathway presenting with variable phenotypic penetrance and involving multiple organ systems. The following case report describes a unique case of a 16-year-old female with AGS who presented with the primary complaint of renovascular hypertension. She had a medical history of ventricular septal defect and polycystic ovary syndrome. The patient had a dysmorphic facial appearance including frontal bossing, bulbous tip of the nose, a pointed chin with prognathism, and deeply set eyes with mild hypertelorism. Stenoocclusive changes of both renal arteries, celiac artery, lower part of the abdominal aorta, and left intracranial artery, along with absence of the left internal carotid artery were found on examination. Whole exome sequencing was performed and revealed a pathologic mutation of JAG1, leading to the diagnosis of AGS. Reverse phenotyping detected butterfly vertebrae and normal structure and function of the liver and gallbladder. While the representative symptom of AGS in most scenarios is a hepatic problem, in this case, the presenting clinical features were the vascular anomalies. Clinical manifestations of AGS are diverse, and this case demonstrates that renovascular hypertension might be in some cases a presenting symptom of AGS.

• The Korean Journal of Physiology
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• v.30 no.2
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• pp.269-278
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• 1996

The mechanism of impaired endothelium-dependent relaxation in the aorta of one-kidney, one clip Goldblatt hypertensive (1K,1C-GBH) rats was investigated. 8 week-old Wistar-Kyoto (WKY) rats were made hypertensive by left renal artery stenosis with contralateral nephrectomy. Endothelium-dependent relaxation was significantly reduced in 1K,1C-GBH rats as compared with WKY rats. However, the relaxation by sodium nitroprusside in 1K,1C-GBH rats was not reduced as compared with WKY rats. The impairment of endothelium-dependent relaxation in 1K,1C-GBH rats was partially restored by the pretreatment of indomethacin or SQ29548. When the nitric oxide production was inhibited by L-nitroarginine methyl ester, acetylcholine (ACh) induced a endothelium-dependent contraction that was greater in 1K,1C-GBH rats than in WKY rats. Endothelium-dependent contraction by ACh was completely abolished by indomethacin or SQ29548. However, imidazole, tranylcypromine and superoxide dismutase did not affect the endothelium-dependent contraction in 1K,1C-GBH rats. These results suggest that impaired endothelium-dependent relaxation in the 1K,1C-GBH rats might be due to the simultaneous release of EDCF, and that prostaglandin B2 may be involved as a mediator of endothelium-dependent contraction.

• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.84-87
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• 2016

Midaortic syndrome (MAS) is a rare vascular disease that commonly causes renovascular hypertension. The lumen of the abdominal aorta narrows and the ostia of the branches show stenosis. MAS is associated with diminished pulses in the lower extremities compared with the upper extremities, severe hypertension with higher blood pressure in the upper rather than lower extremities, and an abdominal bruit. The clinical symptoms are variable, and recognition in children with hypertension can aid early diagnosis and optimal treatment. Hypertension with MAS is malignant and often refractory to several antihypertensive drugs. Recently, radiologic modalities have been developed and have led to numerous interventional procedures. We describe the case of a 3-year-old boy presenting with left ventricular hypertrophy whose severely elevated blood pressure led to the diagnosis of idiopathic MAS. This case highlights the importance of measuring blood pressure and conducting a detailed physical examination to diagnose MAS. This is the first reported case of idiopathic MAS diagnosed in childhood in Korea.

• Journal of Chest Surgery
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• v.34 no.7
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• pp.547-551
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• 2001

The effects of deep hypothermia and circulatory arrest during aortic arch reconstruction are associated with potential neurologic and myocardial injury. We describe a surgical technique that two patients underwent a modified Norwood procedure without circulatory arrest and myocardial ischemia. One was 13-day-old female patient, weighing 3.1kg, having a variant of hypoplastic left heart syndrome and another was 38-day-old male patient, weighing 3.4 kg, diagnosed Taussig-Bing anomaly with severe aortic arch hypoplasia, coarctation of the aorta, and subaortic stenosis. The arterial cannula was inserted in innominate artery directly. During Norwood reconstruction, regional high-flow perfusion into the inominate artery and coronary perfusion were maintained and there were no neurologic, cardiac, and renal complications in two patients. This technique may help protect the brain and myocardium from ischemic injury in patients with hypoplastic left heart syndrome or other arch anomalies including coarctation or interruption.

• Journal of Chest Surgery
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• v.38 no.5 s.250
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• pp.349-356
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• 2005

Background: Arterial conduits using in coronary artery bypass grafting (CABG) have been known a great long term patency rates, and improved short and long term clinical outcomes. It has been reported that Off pump CABG has better clinical results than CABG using cardiopulmonary bypass. To evaluate the advantage of arterial conduits over venous conduits and to avoid the adverse effects of cardiopulmonary bypass, we performed total arterial Off pump CABG. Material and Method: From January 2001 to October 2004, Off pump CABG using only arterial conduits was performed on 325 patients with a mean age of $59.3\pm11.9$ years ($36\~83$). Mean ejection fraction was $55.4\pm14.0\%\;(15\~86).$ Angiography showed left main disease or triple-vessel disease in $81.9\%$ of the patients. Indications of using arterial conduits was stenosis $\ge50\%$ of left anterior descending artery, stenosis $\ge80\%$ of branches of left circumflex artery, and stenosis $\ge90\%$ of right coronary artery and its branches. Multi-slice computed tomography was performed on 194 patients to evaluate the short term patency rates. Result: A total of 928 distal anastomoses were performed and the average anastomoses per a patient were $2.86\pm0.78$. There was 1 operative mortality. Postoperative complications were mediastinitis in 6 patients ($1.8\%$), renal failure in 4 patients ($1.2\%$), perioperative myocardial infarction in 3 patients ($0.9\%$), reoperation for bleeding in 3 patients ($0.9\%$). There was no postoperative stroke. Patency rate of arterial conduits was $99.3\%$ (581/585). There were 4 stenoses or competitive flows in 2 radial arteries and 2 right internal mammary arteries. Conclusion: Total arterial Off pump CABG appears to be safe, showing a low surgical mortality and morbidity and excellent short term patency rates of arterial conduits.

• Tuberculosis and Respiratory Diseases
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• v.57 no.2
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• pp.188-192
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• 2004

Takayasu's arteritis is a chronic inflammatory disease, involving mainly the aorta and its main branches, which can cause stenosis or occlusion. It involves the bracheocephalic, carotid, subclavian, vertebral, and renal, as well as the coronary and pulmonary arteries. The clinical manifestations range from asymptomatic to catastrophic, with dizziness, hypertension, claudication, cerebral infarction, chest pain and dyspnea. Takayasu's arteritis involving the pulmonary arteries, but not the aorta and its main branches, is very rare. Herein, a case of isolated pulmonary Takayasu's arteritis combined with pulmonary thromboembolism, and hyperthyroidism is reported, with a review of the literature.

• The Korean Journal of Physiology
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• v.30 no.1
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• pp.21-31
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• 1996

To investigate the mechanisms of increased endothelium-dependent contraction by acetylcholine in hypertensive rats, the relationship between endothelium-dependent contraction by acetylcholine and blood pressure was studied in spontaneously hypertensive rats (SHR), one-kidney, one clip Goldblatt hypertension (1K,1C-GBH) rats, and Wistar-Kyoto rats (WKY). SHR were treated orally with enalapril or nicardipine in order to prevent development of hypertension or suppress the developed hypertension. 1K,1C-GBH rats were made by renal artery stenosis with contralateral nephrectomy in 8 week-WKY. 1. Endothelium-dependent contractions by acetylcholine $(10^{-6}{\sim}10^{-5}\;M)$ in SHR were significantly greater than those in WKY. 2. Chronic treatment with enalapril or nicardipine reduced the endothelium-dependent contraction in SHR 3. The degree of reduction of endothelium-dependent contraction was greater in SHR which was prevented from developing hypertension than in SHR of which high blood pressure was suppressed. 4. In aortic rings from 1K,1C-GBH rats, endothelium-dependent contractions by acetylcholine were augmented as compared with WKY. 5. There is good relationship between the value of blood pressure and magnitude of endothelium-dependent contraction. Thus, it is suggested that increased endothelium-dependent contraction in hypertensive rats may he due to the high blood pressure and endothelium-dependent contraction may not be a cause of the initiation of hypertension in SHR.