Surgical implication of aortic dissection on long-term outcome in Marfan patients

We herein review our 17-year surgical experience for the treatment of ascending aortic aneurysm in patients with Marfan syndrome to clarify the risks of increased mortality and reoperation. The subjects consisted of 15 patients who had all undergone surgery for the aortic root and ascending aorta at Niigata University Hospital between July 1978 and January 1995. Aortic valve replacement and ascending aortic wrapping were performed in 5 patients, Bentall or Cabrol operation in 6, and combined aortic arch reconstruction and Cabrol operation in 2, as the initial surgery. Patients who had an aortic dissection (Stanford type A) at initial surgery were assigned to group I (n = 7), while those with an aortic root aneurysm were assigned to group II (n = 8). In group I, 3 patients required a second operation for the remaining aortic arch aneurysm, and 1 died due to a late rupture of the distal aneurysm. In group II, no patient needed a reoperation; however, 1 died due to an intracranial hemorrhage and another due to composite valve graft failure and distal dissection. The results thus indicate that aortic dissection seems to affect long-term outcome, and therefore the combined repair of the aortic root and transverse arch is recommended in Marfan patients with aortic dissection involving the transverse aortic arch.     

Reoperations after operation on the thoracic aorta: etiology, surgical techniques, and prevention

Recurrent aortic aneurysms, persistent or new dissection, new onset of valvular and coronary artery disease, graft infection, and prosthetic endocarditis are not rare after thoracic aortic operations; they can be difficult to diagnose and represent a formidable surgical challenge. Between 1977 and 1991, 876 operations were performed on the thoracic aorta in our institution: 340 in dissections, 299 in true aneurysms, 150 for aortic remodeling and external wall support during aortic valve replacement, and 87 for miscellaneous causes. During the same period, there were 193 additional reoperations. Vascular reoperations on abdominal aorta and peripheral arteries accounted for 73 cases and are not further discussed in this study. The reasons for reoperation (n = 130) in 120 patients were: failure of biologic valves (n = 23); aneurysm recurrence in a proximal or distal aortic segment (n = 21); pseudoaneurysm formation at suture lines (n = 13); new dissection or dilatation involving ascending aorta (n = 11), aortic arch (n = 13), and descending aorta (n = 10); aneurysm after aortic remodeling (n = 13); new onset of valvular disease (n = 5); and new onset of coronary disease (n = 5). Infected aortic graft and prosthetic endocarditis accounted for 10 reoperations, and a planned two-staged procedure was performed in 6 patients. Omitting the failed biologic valves, reoperations were performed on the aortic segment previously operated on in 69.3% of the cases and on other thoracic segments in 30.7%. Overall hospital mortality rate after reoperation was 5.8%. A significant decrease in operative mortality was observed in the most recent period (3.0% between 1989 and 1991). Reoperations are technically demanding, and some of them are preventable; therefore (1) graft inclusion technique should be abandoned in ascending aortic operation due to formation of false aneurysms; (2) in patients with Marfan syndrome, complete repair of the diseased aorta should be attempted during the initial operation; (3) aortic arch dissection should be repaired definitively during the first operation in low-risk patients; (4) biological valves should be avoided in aneurysm operations; and (5) homograft replacement is the treatment of choice in prosthetic endocarditis or in infected composite graft after an aortic valve or ascending aortic operation.     

Tuberculosis: infection control measures

A 58-year-old man with acute mitral and aortic endocarditis suffered an acute ascending aortic dissection. The patient had a double valve replacement, combined with replacement of the ascending aorta with a Dacron graft and sealing and closure of the proximal and distal aortic segments with surgical glue. Six months after the operation the patient is doing well with normal function of both valve prostheses and aortic vascular graft.     

Reoperation after repair of type A and B dissecting aneurysm

BACKGROUND: In the late postoperative period after repair of an aortic dissection or dissecting aneurysm, reoperations may be required. The interval to reoperation, size and location of intimal tear, and results of reoperation were evaluated. METHODS: Between January 1982 and April 1997, 138 patients underwent surgery for Stanford type A (90 patients) or type B (48 patients) dissections of the aorta. The entire aorta was evaluated in postoperative follow-up by computed tomography and magnetic resonance imaging for 6 months to 15 years. Reoperations were performed in 14 (10.1%) patients with changes in the aneurysms at the site of the initial repair or in the distal aorta. Selective cerebral perfusion or retrograde cerebral perfusion with deep hypothermia was used in the repair of the ascending, arch, and distal arch aneurysms. Reoperations included aortic root reconstruction (n=3), resection of a pseudoaneurysm (n=1), and replacement of the ascending aorta (n=1), arch (n=5), descending aorta (n=2), thoracoabdominal aorta (n=1), or abdominal aorta (n=1). Secondary reoperations were performed in four patients (replacement of the arch [n=2], thoracoabdominal aorta and abdominal aorta). Consequently two patients had subtotal aortic replacements. The aneurysms were caused by an anastomotic leak, a new intimal tear following aortic cross-clamping, a second intimal tear in the distal arch or abdominal aorta, and Marfan syndrome. RESULTS: Two patients (2/18 11.1%) died of bleeding or low output syndrome. Two patients died of graft infection or prosthetic valve infection 3 months after surgery respectively. CONCLUSIONS: 1) The surgical results of reoperation for type A and B dissections were good. 2) Close postoperative follow-up of the patent false lumen in the entire aorta was necessary. 3) At the initial operation, total resection of the intimal tear in the aortic arch in low-risk patients reduced the risk of reoperation.     

Comparison of amyloid deposition in two lines of transgenic mouse that model familial amyloidotic polyneuropathy, type I

Aortic disease frequently requires extended and multiple resections. Occasionally, resection of the entire aorta may be indicated. At our Institution, from 1982 to 1994, 34 patients were operated upon for extended and total simultaneous aortic replacement. In seven patients, the aorta was replaced from valve to bifurcation; in 27, the aortic valve was included. Operations were performed with circulatory arrest under profound hypothermia. As the first step, the aortic valve and ascending aorta are replaced and the coronary arteries are reconnected, following which the aortic arch is reconstructed. Meanwhile, a second surgical team proceeds to open the thoracoabdominal aorta and tie up the intercostal orifices. If circulatory arrest is likely to exceed 60 minutes, the aortic graft is clamped and upper body perfusion (1000 cc/min) is begun. Finally, the thoracoabdominal aorta is fully replaced. Cardiopulmonary bypass (CPB) with rewarming is resumed only after the operation has been completed. Thirty-four patients survived operation; five died within 1 month for an overall mortality of 14.7%. No mortality occurred in the most recent nine operations. No permanent spinal neurological deficits occurred. Total simultaneous aortic replacement for treatment of extended aortic disease may be reasonable using our approach.     

Surgical repair of type A aortic dissection by the circulatory arrest-graft inclusion technique in sixty-six patients

During an 8-year period (1984 to 1991) 66 patients (mean age 59 years, range 26 to 84 years) with type A aortic dissection (60 ascending aorta tears, 6 arch tears; 35 acute, 31 chronic) had surgical repair by a continuous suture-graft inclusion technique. Hypothermic circulatory arrest (16 degrees C) was used in 58 patients (35/35 acute, 23/31 chronic; mean arrest time 26 minutes, range 10 to 55 minutes). Fifty-two patients had hemiarch repair and 6 had total arch replacement. Aortic valve disease necessitated treatment in 38 patients (1 valved conduit, 20 valve replacements, 17 valve repairs). Recently 11 patients had valve repair by reconstruction of the native aortic root, by means of techniques similar to those used for homograft valve insertion. Operative mortality was 9% (14% acute, 3% chronic). Stroke occurred in 2 patients (3%) and was fatal in both. Variables suggestive of increased operative risk by univariate analysis were acuteness (p = 0.12), visceral ischemia (p = 0.12), and preoperative shock (p = 0.13). No variable was significant by multivariate analysis. Overall actuarial survival at 48 months was 77%, with 3 late deaths from a ruptured distal aneurysm. Late computed tomography or magnetic resonance imaging scan was done in 28 patients at a mean interval of 33 months. These studies identified 1 patient with a pseudoaneurysm requiring reoperation and 3 patients with contained flow between the graft and the wrap. Three patients required late operation: 1 for pseudoaneurysm, 1 for arch dissection, and 1 for repair of a distal aneurysm.     

Treatment of the aortic valve and ascending aorta. The significance of reconstructive methods on the aortic root

BACKGROUND AND OBJECTIVE: The standard surgical repair of disease of the aortic valve and the ascending aorta has been combined replacement, which includes the disadvantage of inserting a mechanical valve. We have investigated an individualized approach which preserves the native valve. PATIENTS AND METHODS: Between October 1995 and October 1997, a consecutive total of 101 patients (72 men, 29 women, aged 21-83 years) underwent operations for disease of the ascending aorta: aortic dissection type A in 34 patients, aneurysmal dilatation in 67. Dilatation of the aortic arch was associated with aortic regurgitation in 58 patients. There were 11 patients with aortic valve stenosis or previously implanted aortic valve prosthesis among a total of 46 whose aortic valve was replaced (group II). Supracommissural aortic replacement with a Dacron tube was performed in 16 patients (group I) with normal valve cusps and an aortic root diameter < 3.5 cm. In 28 patients with an aortic root diameter of 3.5-5.0 cm the aortic root was remodelled (group III). Resuspension of the native aortic valve was undertaken in 11 patients with aortic root dilatation of > 5.0 cm (group IV). RESULTS: Operative intervention was electively performed in 72 patients, without any death. Of 29 patients operated as an emergency for acute type A dissection four died (14%). In 55 of the 58 patients with aortic regurgitation in proved possible to preserve native aortic valve (95%). In the early postoperative phase and after an average follow-up time of 11.8 months, transthoracic echocardiography demonstrated good aortic valve function, except in one patient each of groups III and IV who developed aortic regurgitation grades I or II. CONCLUSION: The described individualized approach makes it possible to preserve the native aortic valve in most patients with aortic regurgitation, at a low risk. Follow-up observations so far indicate good results of the reconstruction.     

Composite valve graft replacement in patients with type A aortic dissection--a modified cabrol procedure

Composite valve graft replacement of the ascending aorta and aortic valve is indicated for a variety of conditions affecting the aortic root. However, a major drawback in this operation is bleeding from the proximal suture line and coronary anastomosis especially in patient with friable root tissue involved by aortic dissection. We describe here a modified technique to take advantage of the aortic button and cabrol techniques to reattach the coronary artery ostia. We have experienced seven patients with the aortic root replacements for type A dissection using the described technique over the past two years. In view of our favorable experience, we recommend this technique especially for patient with acute dissection involving nondilated aortic annulus, in addition to the patients with Marfan syndrome or annulo-aortic ectasia.     

False aneurysm of the ascending aorta after surgery for Marfan's syndrome. Report of a case and review of the literature

A case is described in which false aneurysm of the ascending aorta developed following corrective surgery for Marfan's syndrome. During the initial procedure, a dissecting aortic aneurysm and incompetent aortic valve were managed by replacing the valve with a Starr-Edwards prosthesis and the ascending aorta with a woven Dacron aortic prosthesis. Two years later the operation was repeated with the use of antibiotic cover, because the patient developed a paraprosthetic leak following subacute bacterial endocarditis with Staphylococcus aureus. The woman remained well for 6 months, after which she developed a false aneurysm of the ascending aorta and aortic incompetence which necessitated repeating the operation for a third time. The patient remains well one year later. Relevant literature available on false aneurysm of the ascending aorta is also reviewed.     

A case report of acute aortic dissection with aortic regurgitation and cardiac tamponade: intimal tear just above commissure of right coronary cusp and left coronary cusp

A 57-year-old man with acute dissecting aneurysm of the ascending aorta underwent immediate operation. Preoperative study showed aortic regurgitation and cardiac tamponade. The intimal tear originated just above commissure of right coronary cusp and left coronary cusp. The procedure was a combination of direct closure of the entry and dissecting space and resuspension of native aortic valve. It was preferential of simple intervention limited to the ascending aorta without using prosthesis. There was no complication such as aortic valve regurgitation, enlargement of the ascending aorta, or persistent of dissection during follow-up period of 12 months.     

Incidentaloma due to abdominal splenosis

Three patients underwent pedicle transposition of the greater omentum for sternal osteomyelitis and mediastinitis after cardiac operation. These patients were previously operated on with coronary artery bypass grafting, aortic valve replacement and redo Fontan operation in each. This procedure was carried out on 4, 6, 8, and 14th postoperative day. The complications cured within about one month after the procedure in all cases. It is suggested that this maneuver is effective for obtaining healing osteomyelitis and mediastinitis following cardiac operation.     

Combined surgery for coronary artery disease and thoracic aortic disease

Combined surgery in 6 cases who had coronary artery disease and thoracic aortic disease simultaneously was analyzed. Case # 1 had ascending aortic replacement under deep hypothermic circulatory arrest because of iatrogenic aortic dissection caused by aortic clamp during the routine coronary artery bypass grafting (CABG). Case # 2 had DeBakey type II chronic dissection. Case # 3 had type I aortic dissection 4 years after the initial CABG. Both case # 2 and # 3 had ascending aortic replacement under retrograde cerebral perfusion along with CABG. Transverse aortic replacement was performed in case # 4, # 5 and # 6 under selective cerebral perfusion along with CABG. Case # 4 was associated with ascending-transverse aortic aneurysm. Case # 5 had aortitis syndrome complicated with severe coronary ostial stenosis and cervical branch stenosis. Case # 6 also had aortitis syndrome, severe coronary ostial stenosis, heavily calcified ascending-transverse aorta, and mitral and aortic regurgitation. This case had mitral and aortic valve replacement additionally. Case # 2 died of low cardiac output syndrome and multi-organ failure postoperatively. Case # 4 did not recover from profound shock that followed the preoperative acute myocardial infarction. The problems of low cardiac output syndrome caused by long interval of ischemic cardiac arrest, and also the problems of proximal anastomotic site of saphenous vein grafts were discussed.     

Preliminary clinical results of surgery for type A acute aortic dissections using gelatin-resorcin-formaldehyde glue

We used gelatin-resorcin-form-aldehyde (GRF) glue to fuse the false lumen of type A acute aortic dissection in four patients. All were operated on within 3-24 hours after onset, and gluing of the two cylinders of the dissecting aorta could be done safely in a short time. Initial intimal tears were located in the transverse aorta in three patients and in the proximal descending aorta in one. Simple transection and end-to-end anastomosis of the ascending aorta was done for the first two cases. But in the last two patients, we resected the intimal tear in the transverse aorta and applied GRF glue to the stump of the aortic arch and to that of the aortic root, followed by graft replacement of the ascending aorta. There were no hospital deaths. But we had to reoperate on one patient five months after the first operation due to potentially residual dissection in the aortic root. GRF glue is a very useful adhesive for acute aortic dissection operations, but further refinement of the operative technique using it is necessary.     

Early and late outcome of operated and non-operated acute dissection of the descending aorta

OBJECTIVE: At present debate continues concerning the optimal mode of treatment for type B dissections. Controversies are mainly due to discordant results regarding survival following medical or surgical treatment. We assessed early and long-term outcome of acute dissection of the descending aorta treated by emergency aortic replacement, medical treatment or delayed surgery. METHODS: Between 1980 and 1995, 225 patients were hospitalized in the medical or surgical department of our institution with the diagnosis of acute type B aortic dissection. A total of 38 patients (16.8%) underwent replacement of the descending aorta within the first week after hospital admission. Primary indications for immediate surgery were: rupturing aneurysm (n = 15), diameter of the descending aorta (n = 13), malperfusion of the thoracoabdominal aorta (n = 8) and pseudocoarctation syndrome with uncontrollable hypertension (n = 2). All other patients (n = 187) underwent primary conservative treatment on the intensive care unit, including appropriate anti-hypertensive medication. In 12 of them, surgery was denied because of age or significant concomitant diseases. RESULTS: Hospital mortality after urgent or emergency surgery was 21% (8/38 patients) for the overall time period. There has been a significant decrease in hospital mortality during the last 5 year-period (12% versus 30% between 1980 and 1994). Causes of death were: cardiac failure in 3, bleeding complications in 2, postoperative mesenteric ischemia in 2 and septicemia in one patient. From the 30 operative survivors, 9 (30%) patients required further surgery on the native aorta after a mean follow-up of 48 +/- 13 months. Hospital mortality during conservative treatment was 17.6% (33/187 patients). Main causes of death were rupture in 14, thoraco-abdominal malperfusion in 13 and cardiac failure in 3 patients, whereas in 3 patients, the cause of death could not be evaluated. In this group, 9 patients had to be shifted to early surgery during the initial hospitalization because of impending rupture (n = 4), rapidly increasing diameter (n = 2) and suspicion of intestinal ischemia (n = 3). After hospital discharge, surgery for chronic dissection was performed in 47 patients, mainly because of expanding descending aortic aneurysm. Hospital mortality was 8% (4/47 patients). Actuarial survival rates after surgery during the first admission were 85 +/- 6% at 5 years and 61 +/- 8% at 10 years, versus 76 +/- 5 and 50 +/- 7% respectively, following conservative treatment (P < 0.001). CONCLUSION: Nowadays, acute type B dissection can be treated surgically with a reasonable perioperative risk. Despite aggressive anti-hypertensive treatment, hospital mortality of primary conservative treatment is still high and a substantial percentage of patients requires surgery during initial hospitalization. Main causes of death in both groups are rupture and abdominal malperfusion: therefore, closed clinical and radiologic assessment of the whole thoraco-abdominal aorta is of utmost importance. Long-term results are satisfying; unlimited radiographic follow-up allows for detection of potential severe complications and for proper planning of elective reoperations when indicated.     

Endovascular stent graft repair for aneurysms on the descending thoracic aorta

BACKGROUND: The traditional treatment of aneurysms of the descending thoracic aorta includes posterolateral thoracotomy and aortic replacement with a prosthetic graft. In this study, we report our experiences and results in endovascular stent graft placement as an alternative to surgical repair. METHODS: Between January 1989 and July 1997, a total of 68 patients (24 women) underwent replacement of the thoracic aorta. Mean age at operation was 51 years. Fifty-eight patients underwent conventional surgical treatment. All of these patients were suitable candidates for endovascular stenting; however, no stent graft material was available at the time of operation. Ten patients (1 chronic dissection, 9 atherosclerotic aneurysm) received in the past 8 months the first commercially manufactured endovascular stent graft. The mean diameter of the aneurysms in this group was 7 cm (range, 6 to 8 cm). Two stent patients were operated on using only spinal cord analgesia. All stent grafts were custom designed for each of the 10 patients. RESULTS: The 30-day mortality in the conventional group was 31% versus 10% in the stent group. Mean length of intervention was 320 minutes in the conventional group versus 150 minutes in the endovascular group. Spinal cord injury occurred in 5 patients (12%) in the surgical group, whereas none of the stented patients developed any neurologic sequelae. Mean intensive care unit stay was 13 days, followed by a mean of 10 days on a ward in the first group compared to 4 days in the intensive care unit and 6 days on the ward in the stent group. One stent was required in 2 patients, two stents were required in 3 patients, and four stents were deployed in 5 patients of our series. Five patients required transposition of the left subclavian artery to achieve a sufficient neck for the proximal placement of the stent. There was complete thrombosis of the thoracic aortic aneurysm surrounding the stent graft in 8 patients (80%). Two patients required restenting as a result of leakage (20%). Stent graft placing was performed through the femoral artery in 8 patients, whereas access was only achieved through the abdominal aorta in 2 patients. CONCLUSIONS: These preliminary results demonstrate that endovascular stent graft replacement might be a promising, cheaper, and safe alternative method in selected patients with descending thoracic aneurysms.     

Repair of ruptured sinus of valsalva aneurysm: determinants of long-term survival

BACKGROUND: Ruptured sinus of Valsalva aneurysm is a rare cardiac anomaly and long-term survival after surgical treatment is not well established. This study was designed to investigate the determinants of long-term survival after repair of ruptured sinus of Valsalva aneurysm. METHODS: From April 1978 to April 1996, 53 patients underwent operation for ruptured sinus of Valsalva aneurysm. The incidence among our cardiac surgical population was 0.56%. Long-term survival was investigated in 46 patients (13 to 65 years) who survived the operation, with 96.2% follow-up completeness (mean+/-standard deviation, 6.5+/-4.9 years; maximum, 17.2 years), by univariate and multivariate analyses. RESULTS: There was no early operative death and no recurrence after the initial repair. Actuarial survival was 83.8%+/-8.4% at 15 years. Reoperation, aneurysm draining into the left ventricle, aortic prosthetic dehiscence, bacterial endocarditis, and aortic cross-clamp time (<70 minutes) were significant factors in long-term survival (p < 0.05). Multivariate analysis revealed that only aortic prosthesis dehiscence was the significant factor influencing late survival (p = 0.0001). CONCLUSIONS: Surgical treatment for ruptured sinus of Valsalva aneurysm is safe and has satisfactory results. Aortic prosthesis dehiscence is the independent determinant for long-term survival. Other factors including bacterial endocarditis, concomitant ventricular septal defect repair, and aortic valve replacement did not independently influence long-term survival.     

Experience of reoperations followed by repairs of abdominal aortic aneurysms

We experienced 8 cases who required reoperations, including 2 re-redo operations, after repairs of infrarenal abdominal aortic aneurysms. Of 8 patients, one patient developed a new aneurysm due to atherosclerosis in thoraco-abdominal aorta involving all visceral arteries and other 7 patients had aneurysmal formations at proximal anastomotic sites, including 3 suprarenal, 2 juxtarenal and 2 infrarenal aortic lesions. Etiology at initial operation in patients who subsequently developed anastomotic aneurysms included vasculo-Beh?et disease in 4, atherosclerosis in 2 and dissecting aortic aneurysms type III due to Marfan syndrome in 1. At reoperation, all who had vasculo-Beh?et disease had ruptures of anastomotic sites and 2 patients underwent repairs of dehiscent patch, 1 extra-anatomic bypass between ascending and abdominal aorta and 1 interposition of graft. One patient who had graft infection after repair of abdominal aortic aneurysm required axillo-femoral bypass with removal of infected graft. A patient who had dehiscence of proximal anastomosis after repair of aortoiliac occlusive disease required interposition of graft. Two patients, Marfan syndrome and aneurysm in thoraco-abdominal aorta, underwent graft replacement of thoraco-abdominal aorta concomitant with reconstruction of all visceral arteries. There were 8 patients who required reoperations for aneurysms at distal anastomotic sites after repairs of abdominal aortic aneurysms. Five patients underwent repairs of new aneurysms, including replacement of total arch in 3, descending aorta in 1 and iliac artery in 1. In all cases, no hospital death was noted, however, late deaths were occurred in vasculo-Beh?et disease, Marfan syndrome and graft infection. Thus, late result depends on etiology of disease. Although patients who requires reoperation after repair of abdominal aortic aneurysms have higher operative risk factors, early and late results are satisfactory compared to initial operations.     

Successful graft replacement of the descending aorta after an extended reconstruction of the ascending and transverse aorta in a patient with Marfan's syndrome

A 27-year-old man with Marfan's syndrome underwent a total aortic graft replacement in three separate stages. Initially the abdominal aorta was replaced, followed by the ascending aorta and aortic arch, and finally the residual portion. The extensive reconstruction of both the ascending and transverse aorta at the second operation, even though no dissection was present in the aortic arch, reduced the risk of the subsequent operation since the same surgical approach did not have to be used.     

Studying electric field effects on embryonic myocytes

OBJECTIVE: Little attention has been paid to the occurrence of aortic regurgitation after complete repair in patients with pulmonary atresia and ventricular septal defect or tetralogy of Fallot. To highlight the development of aortic regurgitation or aortic root dilation severe enough to necessitate aortic valve replacement with or without aortic aneurysmorrhaphy or aortic root replacement, we retrospectively reviewed the records of patients who underwent aortic valve operation at our institution subsequent to repair of pulmonary atresia and ventricular septal defect or tetralogy of Fallot. METHODS: We searched the Mayo Clinic database for patients with pulmonary atresia and ventricular septal defect or tetralogy of Fallot who subsequently had aortic valve or aortic root operations. The degree of aortic regurgitation before operation was noted. Aortic sinus and root dimensions were measured. RESULTS: Sixteen patients underwent complete repair at a median age of 17 years, followed by an aortic operation a median of 13.5 years later. All 16 patients had dilated aortic sinuses at the time of the aortic valve operation. These 16 patients had aortic valve replacement: 11 with mechanical prostheses and 5 with bioprostheses. Five of the 16 also had reduction of aortic dilation by lateral aneurysmorrhaphy, and 1 had graft replacement of the ascending aorta. Five patients had associated conditions (evidence of valvular damage, recurrent ventricular septal defect, or history of endocarditis) discovered at the aortic valve operation that have been reported to be related to the development of aortic regurgitation. The remaining 11 patients had progressive aortic regurgitation despite complete, uncomplicated repair. CONCLUSIONS: Progressive aortic regurgitation and aortic root dilation can occur despite complete repair of pulmonary atresia and ventricular septal defect or tetralogy of Fallot.     

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.