• Journal of Chest Surgery
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• 제27권11호
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• pp.907-913
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• 1994

From October 1990 to May 1993, 19 patients underwent replacement of the transverse aortic arch. [10 men, 9 women, mean age 52.5 years] Underlying diseases were acute aortic dissection [10 cases], chronic aortic dissection [4 cases],and aortic arch aneurysm [ 5 cases]. In 19 patients, 10 underwent partial replacement and 9 underwent total arch replacement. The cerebral protection was achieved by profound hypothermia [rectal temperature,16$^{\circ}$ to 2$0^{\circ}C$] associated with total circulatory arrest [mean 35.5 minutes]. In one patient, the aortic arch distal to the left common carotid artery was resected with the distal arch being cross-clamped and in another two patients, the selective cerebral perfusion was also applied during the period of total circulatory arrest via innominate artery and left common carotid artery because of longer total circulatory arrest time. Among 14 patients of aortic dissecton, 10 presented hypertension, 1 presented Marfan syndrome, 1 presented pregnancy-induced hypertension and 2 revealed no evidence of hypertension. All of the above 14 patients complained chest pain. Among 5 patients of aortic arch aneurysm, Be het disease was suspected in only one patient and atherosclerotic aneurysm was proved in another 4 patients. The overall hospital mortality was 32% [6/19]. In aortic dissection, the mortality was 43% [Acute aortic dissection 30%, chronic aortic dissection 75%] and in aortic arch aneurysm, the mortality was 0%. Follow-up was done in all survivors for from 7 months to 36 months[mean,17.3%].

• Clinical and Experimental Pediatrics
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• 제54권6호
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• pp.272-275
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• 2011

A recently recognized connective tissue disorder, Loeys-Dietz syndrome (LDS) is a genetic aortic aneurysm syndrome caused by mutations in the transforming growth factor-receptor type I or II gene (TGFBR1 or TGFBR2). They have distinctive phenotypic abnormalities including widely spaced eyes (hypertelorism), bifid uvula or cleft palate, and arterial tortuosity with aortic aneurysm or dissection throughout the arterial tree. LDS is characterized by aggressive and rapid progression of aortic aneurysm. Therefore, the patients with distinct phenotype, marked aortic dilatation and aneurysm at early age should be suspected to be affected by LDS and rapid TGFBR gene analysis should be done. We report one child diagnosed as LDS due to typical phenotypes and two novel missense mutations of the TGFBR2 gene (c.1526G>T and c.1528A>T).

• Biomolecules & Therapeutics
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• 제28권1호
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• pp.98-103
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• 2020

Marfan syndrome (MFS), a connective tissue disorder caused by mutations in the fibrillin-1 (Fbn1) gene, has vascular manifestations including aortic aneurysm, dissection, and rupture. Its vascular pathogenesis is assumed to be attributed to increased transforming growth factor β (TGFβ) signaling and blockade of excessive TGFβ signaling has been thought to prevent dissection and aneurysm formation. Here, we investigated whether galunisertib, a potent small-molecule inhibitor of TGFβ receptor I (TβRI), attenuates aneurysmal disease in a murine model of MFS (Fbn1^C1039G/+) and compared the impact of galuninsertib on the MFS-related vascular pathogenesis with that of losartan, a prophylactic agent routinely used for patients with MFS. Fbn1^C1039G/+ mice were administered galunisertib or losartan for 8 weeks, and their ascending aortas were assessed for histopathological changes and phosphorylation of Smad2 and extracellular signal-regulated kinase 1/2 (Erk1/2). Mice treated with galunisertib or losartan barely exhibited phosphorylated Smad2, suggesting that both drugs effectively blocked overactivated canonical TGFβ signaling in Fbn1^C1039G/+ mice. However, galunisertib treatment did not attenuate disrupted medial wall architecture and only partially decreased Erk1/2 phosphorylation, whereas losartan significantly inhibited MFS-associated aortopathy and markedly decreased Erk1/2 phosphorylation in Fbn1^C1039G/+ mice. These data unexpectedly revealed that galunisertib, a TβRI inhibitor, showed no benefits in aneurysmal disease in MFS mice although it completely blocked Smad2 phosphorylation. The significant losartan-induced inhibition of both aortic vascular pathogenesis and Smad2 phosphorylation implied that canonical TGFβ signaling might not prominently drive aneurysmal diseases in MFS mice.

• Journal of Chest Surgery
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• 제25권5호
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• pp.511-517
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• 1992

From January 1988 to December 1990, 18 adult patients with aortic disease underwent surgical repair using hypothermia and total circulatory arrest. The age at operation ranged from 17 years to 64 years[mean 45.2$\pm$10.7 years]. We disease entities included aortic dissection in 12, aortoannuloectasia in 3 and thoracic aortic aneurysm in 3 cases. Partial cardiopulmonary bypass via femoral vessels along with surface cooling was used upon the induction of deep hypothermia[18~20oC]. Modified Bentall operation was performed in 7 cases, ascending aorta replacement in 6, graft interposition in descending thoracic aorta in 3 and others in 2 cases. The circulatory arrest was maintained for periods of 2 minutes to 86 mimutes[mean 34.7$\pm$5.0 minutes]. Overall hospital mortality was 27.8%[5/18]: brain damage was responsible for the death of 2 patients. 4 patients out of 13 survivors experienced postoperative neurologic dysfunction, which was proved to be self-limited except one case showing left hemiparesis. 12 patients were followed up postoperatively with the mean follow-up period 22.7$\pm$10.1 months. There was no death. No new neurologic problems were observed during follow-up period. All but one patient showing recurrent dissection and aortic regurgitation are in exellent clinical condition. These clinical data suggests that the principle of deep hypothermia and total circulatory arrest can be applied rather safely in adult patients, especially in the treatment of patients with aortic disease, it can be a valuable adjunct with better clinical results.

• Journal of Chest Surgery
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• 제28권10호
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• pp.906-911
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• 1995

From January 1991 to January 1995, 11 patients with aortic diseases underwent various surgical repairs. The age at operation ranged from 26 years to 63 years[ mean=50.9 years . The disease entities included 8 aortic dissections[ type I in 4, type II in 2 and type III in 2 cases , 2 Marfan`s syndrome with annuloaortic ectasia and 1 desecending thoracic aortic aneurysm The operative procedures we tried were 3 Bentall`s operation, 5 graft replacement of ascending aorta, and 3 graft interposition in descending thoracic aorta.Overall hospital mortality rate is 36.3%[4/11 . And causes of death are pump weaning failure in 2 cases and multiorgan failure in 2 cases. It was that 2 sternal dehiscence & mediastinitis, 1 acute renal failure, 2 hypoxic brain damages and 2 postoperative psychosis were complicated. Recently we tried surgical repair of aortic dissection five out of 6 cases using total circulatory arrest with deep hypothermia at 14$^{\circ}C$. Total circulatory arrest time ranged from 18 to 26 minutes[ mean 22.2 minutes , and mean aortic cross-clamping time was 48.2 minutes. One of 5 patient died on the 7th postoperative day due to multiorgan failure. Mortality of patients with TCA was 20%[1/5 , and it of remainders was 50%[3/6 . Our result for surgical repair using total circulatory arrest with deep hypothermia is satisfactory on the basis of our clinical data.

• Journal of Chest Surgery
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• 제30권8호
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• pp.752-759
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• 1997

복합 심장질환이나 대동맥궁 수술시 뇌손상을 보호하기 위하여 오래전부터 초저체온하 완전순환정지법 을 이용하고 있다. 수술후 뇌손상을 예방하기 위하여 여러 방법들을 이용하여 왔으며, 1994년이후 저자들은 뇌대사를 감시하기 위한 방법으로 수술중 뇌파감시를 하고 있다. 저자들은 심장수술실에 사용이 편리한 뇌 파 압축 스펙트럼 정렬(compressed spectral array; CSA) 시스템을 개발하여 이를 대동맥수술시 초저체온 및 설 전순환차단하에 뇌파감시에 적용하여 이의 유용성을 검증하고, 초저체온하 뇌파를 연속감시하여 뇌파가 소 실되고 다시 나타나는 온도를 확인하여 순환정지의 안전한 온도와 시간을 연구하였다. 급성 대동맥박리증 3 례와 대동맥궁 대동맥류 3례(2례는 가성)의 대동맥질환 환자를 연구대상으로 하였다. 직장과 식도체온을 연 속 감시하였으며,마취후 뇌파를 연속감시하였다. 대상환자들중 3례는 초저체온하 완전순환차단하에 수술을 하였으며, 3례는 초저체온하 선택적 뇌관류하에(500 700Ml분) 수술을 하였다. 환자의 체온을 하강하여 뇌 파가 소실된 후 약 3분 후에 순환정지나 선택적 뇌관류를 하였다. 뇌파가 소실된 체온은 직장\ulcorner온이 $16.1^{\circ}C-22.1^{\circ}C(평균:$ $18.4\pm2.0),$ 식도체온이 $12.7^{\circ}C~16.4^{\circ}C(평균:$ $14.7\pm1.6)였다.$ 완전순환차단 환자의 경우 뇌 허혈 시간은 각각 30, 36, 및 56분이었으며, 뇌관류 환자의 경우 각각 41, 56, 92분이었다. 수술중 뇌파가 다시 나 타난 시간 및 체온은(증례 3~6) 5~23분후에 직장체온이 $14.1^{\circ}C~20.3^{\circ}C,$ 식도체온이 $11.7^{\circ}C~15.4^{\circ}C에서$ 나타났다. 수술후 뇌손상의 증상은 없었으며,수술과 관련된 사망은 없었다. 이상의 결과로 식도체온이 $16^{\circ}C이하에서도$ 뇌파가 23분이내에 다시 나타난 것으로 보아 식도체온이 $15^{\circ}C이하는$ 되어야 약 30분간의 완전순환 차단에 안전하리라 생각되었으며, 대동맥 수술시 CSA를 이용한 뇌파감시는 전기뇌활동을 감시하는데 유용 한 방법으로 생각되었다.