Combined aortic root replacement and pectus excavatum correction in Marfan's syndrome

A 53-year-old man with Marfan's syndrome was admitted for repair of annulo-aortic ectasia (58 mm). He had also severe pectus excavatum. The skin was incised along the sternal midline. The pectoral muscles were detached laterally. After the perichondrium and costal cartilages were resected bilaterally. the left-sided intercostal muscles and perichondrial sheaths were divided 3 cm lateral to the sternum. To place the retractor in parasternal position, excellent exposure of the heart and aortic root was enabled. The aortic root was replaced with a Carboseal graft. Chest wall reconstructions was completed by modified Ravitch procedure with Gore-tex sheet The patient was discharged after an uneventful recovery on postoperative day 14.     

One-stage operation in a patient with Marfan's syndrome, severe pectus excavatum and annulo-aortic ectasia]

A case of Marfan's syndrome complicated by annulo-aortic ectasia with pectus excavatum (Wada's 3rd grade classification) and aortic regurgitation (Seller's 1st grade) is reported in a 36-year-old man. We performed one-stage operation combining Bentall's procedure for the annulo-aortic ectasia and sternal elevation for the pectus excavatum. Few reports of one-stage operation for pectus excavatum and annulo-aortic ectasia in Marfan's syndrome have been published. If both diseases are present and surgical indications permit, one-stage operation can be performed to ensure protection of the heart and a satisfactory postoperative course. The best approach to the heart to obtain the most favorable operating field and the best chestplasty method that also assures closure of the median sternotomy are important considerations in one-stage operation. The most appropriate combination of these two operational variables must be assessed in each case.     

An alternative surgical approach for the combined treatment of pectus excavatum and acute aortic dissection type-A in Marfan syndrome

Acute aortic dissection type-A (AADA) is a life-threatening condition especially in patients with Marfan syndrome (MFS) simultaneously suffering from severe pectus excavatum (PE). We report on emergency surgery for combined treatment of PE and AADA in a patient with MFS using an alternative approach. It leads to excellent exposure of the dislocated heart and great vessels enabling Bentall procedure followed by funnel chest repair with modified technique of Adkins and Blades. We achieved favorable functional and cosmetic results. Therefore, we conclude the surgical approach presented is feasible for standard treatment of AADA and consecutive repair of PE.     

Endoluminal and surgical treatment for the management of Stanford Type A aortic dissection.

Stanford Type A aortic dissection is a life-threatening disease. A 46-year-old female patient with Stanford Type A aortic dissection was successfully treated by placing a stent-graft into the ascending aorta via femoral artery. No complication was found immediately after the operation. Bentall operation was performed to treat the development of severe aortic insufficiency 21 months after the stent-grafting procedure. Literature review was done to discuss the possibility of using endoluminal stent placement to treat Stanford Type A aortic dissection.     

Combined severe pectus excavatum correction and aortic root replacement in Marfan's syndrome

A 23-year-old man with Marfan's syndrome was admitted for repair of annulo-aortic ectasia and severe pectus excavatum. A submammary skin incision approach followed by bilateral subperichondrial resection of abnormal costal cartilages was performed. The left intercostal muscles and perichondrial sheaths were divided 2 inches lateral to the sternum in a parasternal fashion to place the retractor. The aortic root was replaced with a 23-mm St. Jude's composite graft (St. Jude Medical, Inc, St. Paul, MN). Chest wall reconstruction was completed with a high sternal osteotomy and support of the sternum was made with Gortex strips (W.L. Gore & Associates, Inc, Flagstaff, AZ). The patient made an uneventful recovery.     

A successful treatment for myonephropathic metabolic syndrome after operation of acute aortic dissection (Stanford type A) with multiple organ ischemia

A 60-year-old man had sudden chest pain and right lower extremity pain. A diagnosis of acute aortic dissection (Stanford type A) with right lower extremity ischemia was made. An emergency ascending aortic and total arch replacement, right axillo-femoral bypass, and coronary artery bypass grafting was performed for acute aortic dissection, acute right iliac arterial occlusion, and acute myocardial infarction. Myonephropathic metabolic syndrome (MNMS) occurred 2 days after operation. The serum creatine kinase value increased over 68,000 U/l, hemodiafiltration was started on the 2nd postoperative day (POD). The patient fell into anuric state and hemodiafiltration was performed 3 times a week. Urination was obtained over 1,000 ml/day from the 25th POD and hemodiafiltration was not necessary any more. We emphasize that prompt operation including revascularization of ischemic leg should be performed in acute aortic dissection with extremity ischemia and also prompt hemodiafiltration to improve the prognosis of these disastrous lesion.     

A surgical case of acute three-channeled aortic dissection in Marfan syndrome

We surgically treated a 35-year-old male with acute 3- channeled aortic dissection in Marfan syndrome. He had acute type A aortic dissection, and underwent Bentall’s type operation, simultaneous graft replacement of the ascending aorta and total aortic arch. Pain recurred 5 years and 9 months after the first operation. CT scan showed two adjacent false lumens in the descending aorta. The morphology of the first and second dissections was Stanford type A+B. The second dissection was acute. In the second false lumen, a re-entry formation was observed in the abdominal aorta. Because severe pain was persistent, we immediately replaced the descending aorta using a femoro-femoral partial cardiopulmonary bypass. The patient was doing well and was discharged. When pain recurs in a Marfan patient with an aortic dissection, a 3-channeled aortic dissection should be suspected, and we recommend emergency surgery.     

Aortic dissection in aged siblings without Marfan's syndrome

Aortic dissection rarely occurs in 2 or more family members without Marfan's syndrome. This report describes two aged siblings who underwent emergency operations for aortic dissection. Case 1: A 71-year-old female (sister), who had Stanford type B aortic dissection, underwent replacement of the descending aorta with a Hemashield graft. Case 2: A 72-year-old male (brother of case 1), who had Stanford type A aortic dissection, underwent replacement of the ascending aorta with a UBE graft following the closure of the entry located in the proximal arch. Neither of 2 siblings nor other family members had any features of the Marfan's syndrome. It is proposed that two aortic dissections occurred coincidentally in one family without Marfan's syndrome.     

Anaesthetic management of a caesarean section in a patient with Marfan's syndrome and aortic dissection

This report describes a case of a Stanford Type B aortic dissection (originating distal to the left subclavian artery and extending to the aortic bifurcation and proximal left iliac artery) in a 31-year-old primigravid woman who was at 39 weeks gestation and had Marfan's syndrome. The dissection was managed conservatively. Caesarean section was performed under epidural anaesthesia with aggressive control of hypertension. Postoperatively, there was no extension of the dissection and no aneurysm formation. She was discharged from hospital two weeks after delivery and remained asymptomatic at six months. There are no plans for surgical intervention.     

Emergent pectus excavatum repair after aortic root replacement in Marfan patient

Chest wall deformities, including pectus excavatum, can complicate cardiac operations by impeding sternal entry and cardiac exposure and by affecting cardiac hemodynamic performance. We describe a patient with Marfan syndrome who underwent elective replacement of an aortic root aneurysm. The patient required a simultaneous, unplanned, emergent repair of her severe pectus excavatum because of hemodynamic instability after sternal closure. The success of this procedure suggests that emergent single-stage repair of pectus excavatum should be considered when median sternotomy closure precipitates hemodynamic instability in such patients.     

The challenge of simultaneous major aortic surgery and pectus excavatum repair in a patient with Ehlers-Danlos type IV syndrome

A unique case of a patient with Ehlers-Danlos type IV syndrome was referred with aortic root aneurysm and previous cosmetic primary repair of pectus excavatum (PE) through a solid silicone onlay prosthesis. The patient underwent successful removal of the silicone prosthesis and one-stage ascending aortic root replacement with concomitant Nuss procedure for repair of the PE.     

胸主动脉夹层合并腹主夹层动脉瘤的一期腔内治疗

目的：探讨胸主动脉夹层动脉瘤合并腹主动脉夹层动脉瘤病人一期腔内隔绝术治疗的可行性、手术操作技巧及并发症防治原则。方法和结果：1例Stanford B型胸主动脉夹层动脉瘤合并腹主动脉夹层动脉瘤及双侧髂动脉瘤的病人于2006年3月在本中心接受了腔内隔绝术。MRA检查提示．主动脉弓降交界处开始出现夹层．真腔受压变窄,以胸腹交界处及腹主动脉中段最明显,最扁窄处为0．5cm;假腔在腹主动脉中段明显,最大径约5．0cm,假腔再人口位于左髂总动脉近端。双侧髂总动脉迂曲并呈瘤样扩张。腹腔干、肠系膜上动脉及双侧肾动脉均发自真腔。手术在全麻下进行：降主动脉植入规格为34-34-100mm的直管型Talent移植物,封闭夹层裂口：腹主动脉植入规格为AOI26-12-170mm Talent移植物,远端连接12．12．68mmTalent移植物至一侧髂外动脉,行双侧股-股转流。瘤体隔绝完全,手术约耗时300min,失血1000ml,透视6min,使用威视派克450ml。术后21d出院。术后随访半年,病人生活质量良好,复查CTA显示：移植物通畅,瘤腔内均完全形成血栓。结论：腔内隔绝术的微创特点使一期治疗Stanford B型主动脉夹层动脉瘤合并腹主动脉瘤成为一种比较安全的手术。主动脉长段隔绝也有利于降低截瘫的发生率。     

Simultaneous repair of severe pectus excavatum and aortic valve replacement following previous open-heart surgery

A 60-year-old man with a severe degree of pectus excavatum and previous coronary artery surgery required aortic valve replacement. At operation the sternal wires were found to be densely adherent to the aortic wall. We describe the surgical technique, which was carried out uneventfully.     

Swinging motion of intimal flap through the aortic valve in acute aortic dissection

The purpose of this article is to present a very rare case of Stanford type A acute aortic dissection featuring a swinging motion of the cylinder-shaped intimal flap through the aortic valve. The patient was a 62-year-old male suffering from severe cardiogenic shock. A transthoracic echocardiogram revealed aortic dissection and severe aortic regurgitation. A transesophageal echocardiogram demonstrated that the aortic dissection in the ascending aorta was circumferential and the proximal portion of the intimal flap was swinging through the aortic valve, ie., falling into the left ventricle during the diastolic phase and being ejected back into the ascending aorta during the systolic phase. An emergency graft replacement of the ascending aorta was performed. During ventricular fibrillation under total cardiopulmonary bypass, we performed cardiac massage to prevent myocardial ischemia, because blood flow from a heart lung machine inverted the intimal flap, which might have disturbed the coronary circulation. The patient's postoperative course was uneventful, and his postoperative echocardiogram revealed only a trace of regurgitant flow through the aortic valve. Back-and-forth movement of the cylinder-shaped intima requires coexistence of the following three conditions: severe aortic regurgitation, circumferential dissection, and complete transection of the intimal flap. We conclude that this movement of the intimal flap should be regarded as one of the most serious complications leading rapidly to cardiogenic shock. From a surgical point of view, it is most important to prevent myocardial ischemia during cardiopulmonary bypass especially in cases in which ventricular fibrillation has occurred. We describe the ways to prevent myocardial ischemia in this rare situation.     

A successful emergency operation of acute aortic dissection (DeBakey I) with aortic root rupture and right coronary avulsion

We report a successful emergency operation for a patient of acute aortic dissection with cardiogenic shock. A 73-year-old man was admitted to our hospital with a complaint of severe chest pain. The emergency aortography and echocardiography demonstrated aortic dissection (DeBakey I) with aortic valve regurgitation and massive pericardial effusion. The emergency operation started 2 hours after the onset. We performed reconstruction of ascending aorta and aortic valve using composite graft, since the patient had aortic root rupture and right coronary avulsion. Subsequently, the right coronary bypass grafting of saphenous vein was performed in addition to the reimplantation of left coronary artery by carrel patch method. The postoperative course was uneventful and the patient remains asymptomatic.     

Familial aortic dissection; cases involving a father,mother, and son

The occurrence of familial aortic dissection is rare in cases that are not linked to Marfan syndrome. We report the 3 cases of acute aortic dissection, involving both parents and their son. Case 1 (father case): 79-year-old male visited a nearby doctor complaining of severe chest and back pain. He was diagnosed as acute aortic dissection (Stanford type A). An emergency operation was carried out and he had an artificial vascular replacement of ascending aorta and arch portion. Case 2 (mother case): 73-year-old female was treated for hypertension. Suddenly, she had a terrible epigastralgia and back pain. The contrast enhanced chest computed tomography (CT) revealed an acute aortic dissection (Stanford type A). The ascending aortic aneurysm ruptured in the ambulance while she was transferred to our hospital. She arrived at our hospital after cardiac and respiratory arrest. We attempted to perform cardiac massage, but we could not bring her back to life. Case 3 (son case): 48-year-old male was transferred to our hospital diagnosed with acute aortic dissection (Stanford type A). The ascending aorta replacement was performed on the same day. We were able to save cases 1 and 3 by performing surgery. Aortic dissection is thought to be the disease of medial degeneration. Hereditary links to aortic dissection are not well understood, with the exception of Marfan syndrome cases. The investigation of genetic mechanisms related to aortic dissection will be expected in the future.     

Aortic disruption after operation for pectus excavatum in a infant with Marfanoid hypermobility syndrome

An elective correction of pectus excavatum was carried out in a 5-year-old boy who subsequently died on the sixth postoperative day from rupture of descending aorta. The rupture which was longitudinal disruption 5 cm in length occurred 3.2 cm distal to the orifice of the left subclavian artery. Histological examination revealed that cystic medial necrosis was present in the thoracic and abdominal aorta. This boy had a slightly tall frame, arachnodactylia, hyperextensibility of the joint, but no ocular symptoms. His skin was excessively elastic and friable. Although he had been identified with characteristics to neither Marfan syndrome nor Ehlers-Danlos syndrome, he was thought to be with Marfanoid hypermobility syndrome. Since aortic dilatation was 4.6 cm in diameter and no valvular involvement was detected preoperatively, only the cosmetic surgery for the pectus excavatum was made. The correction of pectus excavatum might be a trigger of aortic disruption. We must mention that aortic disruption may be occur besides the dilated portion in case of connective tissue disease.     

Emergency operation and hypothermic therapy for Stanford type A acute aortic dissection in the state of coma

We report of a 77-year-old woman who was admitted to our hospital in coma by emergency. A computed tomography scan revealed acute aortic dissection (Stanford type A). We established selective antegrade cerebral perfusion within 3 hours of the onset and then performed ascending aortic replacement. In the state of hypothermia (35 degrees C), the patient was weaned from cardiopulmonary bypass. The patient was kept hypothermic until the operation was completed. We kept mild hypothermia (34.5 degrees C) in intensive care unit (ICU) for 40 hours. The patient was extubated at 94 hours after the operation. The patient was discharged from the hospital on foot on postoperative day 21.     

Stanford B型主动脉夹层术前发生全身炎症反应综合征的多因素Logistic回归分析

目的 探讨并分析Stanford B型主动脉夹层术前发生全身炎症反应综合征的危险因素.方法 回顾性分析2004年1月-2013年9月151例Stanford B型主动脉夹层患者术前资料,以术前是否发生全身炎症反应综合征为因变量,对术前发生全身炎症反应综合征的影响因素分别进行单因素分析,具有统计学差异的变量继续进行逐步Logistic回归分析.结果 Stanford B型主动脉夹层组术前发生全身炎症反应综合征45例,发病率为29.80％.单因素分析显示,年龄、夹层分期、高血压分级、脏器灌注不良、主动脉瓣关闭不全程度是Stanford B型主动脉夹层术前发生全身炎症反应综合征的危险因素,均P＜0.10.多因素分析显示,主动脉夹层分期(OR:0.927,95％ CI:0.887 ～0.969),年龄(OR:0.171,95％CI:0.070 ～0.417)是Stanford B型主动脉夹层术前发生全身炎症反应综合征的独立危险因素.结论 Stanford B型主动脉夹层术前发生全身炎症反应综合征比例高,主动脉夹层进入慢性期预示Stanford B型主动脉夹层术前发生全身炎症反应综合征减弱或消退.     

Eyes wide shut - unusual two stage repair of pectus excavatum and annuloaortic ectasia in a 37 year old marfan patient: case report

We report about a 37 year old male patient with a pectus excavatum. The patient was in NYHA functional class III. After performed computed tomography the symptoms were thought to be related to the severity of chest deformation. A Ravitch-procedure had been accomplished in a district hospital in 2009. The crack of a metal bar led to a reevaluation 2010, in which surprisingly the presence of an annuloaortic ectasia (root 73 × 74 mm) in direct neighborhood of the formerly implanted metal-bars was diagnosed. Echocardiography revealed a severe aortic valve regurgitation, the left ventricle was massively dilated presenting a reduced ejection fraction of 45%. A marfan syndrome was suspected and the patient underwent a valve sparing aortic root replacement (David procedure) in our institution with an uneventful postoperative course. A review of the literature in combination with discussion of our case suggests the application of stronger recommendations towards preoperative cardiovascular assessment in patients with pectus excavatum.