A case of Bentall's operation for an 11-year-old patient with Marfan syndrome

An 11-year-old girl who was diagnosed to have Marfan syndrome in her infancy, visited us with complaints of easy fatigability and chest discomfort. She was pointed out to have acute development of annuloaortic ectasia with severe aortic regurgitation and mild mitral regurgitation. She underwent replacement of the ascending aorta and aortic valve using composite graft with prosthetic valve (Bentall's operation) and circular annuloplasty of the mitral valve. Bentall's operation for infants and children is remarkably rare because in this generation, acute development of aortic dilatation leading rupture and dissection is quite infrequent while main death is caused by mitral regurgitation.     

True Aneurysm of the Left Main Trunk in a Marfan Syndrome Patient at Remote Period after Bentall Operation and Total Arch Replacement: A Case Report

In 2002, a 37-year-old male with Marfan syndrome underwent the Bentall operation, total arch replacement, and aortobifemoral bypass for DeBakey type IIIb chronic aortic dissection, annuloaortic ectasia, and aortic regurgitation. In 2007, mild mitral regurgitation (MR) caused by mitral valve prolapse was identified. In April 2017, echocardiography revealed the worsening of MR and moderate tricuspid regurgitation (TR). Moreover, coronary angiography (CAG) revealed a coronary artery aneurysm in the left main trunk (LMT). In August 2017, the patient underwent mitral valve replacement (MVR), tricuspid annuloplasty (TAP), and coronary artery reconstruction. We reconstructed the LMT aneurysm using an artificial graft. True aneurysm of the coronary artery complicated with Marfan syndrome is a rare complication that has seldom been reported. This case highlights that it is essential to carefully follow-up patients with Marfan syndrome after the Bentall operation.     

Simultaneous repair of cardiovascular disorders and pectus deformity in a patient with Sprintzen-Goldberg syndrome: A case report.

We report a 12-year-old girl with Sprintzen-Goldberg syndrome (SGS) who was complicated with annuloaortic ectasia with aortic regurgitation, mitral valve prolapse with mitral regurgitation, and a severe pectus excavatum. In this patient, aortic root replacement, mitral valve replacement, and sternal elevation were simultaneously performed, and a version of Ravitch's procedure that was technically modified to support the sternum was used for sternal elevation. This modified sternal elevation technique gave excellent operative exposure, and maintained chest wall stability after the operation.     

Aortic root replacement in Marfan syndrome with hemophilia A

A 34-year-old man with Marfan syndrome was admitted to our hospital for surgical treatment of aortic regurgitation due to annuloaortic ectasia. He had no history of bleeding complications. Preoperative investigation revealed a slight prolongation of an activated partial thromboplastin time, which went unnoticed. He underwent aortic root replacement with a composite valve graft. During the operation, he had excessive bleeding due to coagulopathy after the termination of cardiopulmonary bypass, and needed a large amount of blood transfusion to obtain hemostasis. Before his discharge from our hospital, he was diagnosed as mild hemophilia A because of the decline in his factor VII level. To our knowledge, there has been no published case of cardiac operations in Marfan syndrome with hemophilia A.     

Bentall Operation for a Child with Marfan Syndrome: A Case Report

A bstract Children with Marfan syndrome rarely undergo surgery for annuloaortic ectasia and aortic regurgitation in the first decade. A 7-year-old girl presented with congestive heart failure due to severe aortic regurgitation associated with annuloaortic ectasia (6 cm). She also had funnel chest. She underwent a Bentall operation and sternal turn-over with a satisfactory result. Since the aortic valve cusps had rolled edges, the aortic valve was not spared. Histology of the aortic valve cusps showed myxoid degeneration and fragmentation of elastic fibers.     

Simultaneous operation for annulo-aortic ectasia and mitral regurgitation in a patient with non-Marfan syndrome; report of a case

A 74-year-old woman with non-Marfan syndrome underwent aortic root replacement and mitral valve replacement simultaneously for annulo-aortic ectasia and mitral regurgitation. Postoperatively, she suffered from cholecystitis and underwent percutaneous transhepatic gull bladder drainage. After this intervention, her postoperative recovery was uneventful. The purpose of this study is to suggest the possibility of mitral regurgitation due to annular enlargement of the mitral valve as an outcome of deterioration of the annulo-aortic ectasia.     

Transaortic Mitral Valve Repair Combined with a Modified Bentall Procedure in a Patient with Marfan’s Syndrome: Report of a Case

We report a case of transaortic mitral valve repair combined with aortic root and arch replacement in a patient with Marfans syndrome. Preoperative computed tomography and echocardiography showed acute aortic dissection (DeBakey type 1), severe aortic regurgitation, annuloaortic ectasia, and mild mitral regurgitation (MR). We performed artificial chordae implantation to the anterior mitral leaflet (AML) through the aortic root, followed by insertion of an aortic composite graft and replacement of the aortic arch. The patient is well 55 months after the operation, with minimal MR. We think that the transaortic approach is a good alternative for exposure and correction of the AML and its apparatus in special circumstances.     

升主动脉根部瘤的外科治疗

目的总结升主动脉根部瘤的外科治疗经验。方法101例升主动脉根部瘤患者（年龄14～72岁，平均年龄42．7岁）的主要病因为马方综合征（Marfan syndrome，58例），主动脉瓣环扩张症（34例），主动脉瓣二叶瓣畸形（5例），大动脉炎（4例）；术前有主动脉瓣关闭不全96例，主动脉瓣狭窄4例，术前合并有A型夹层26例，急性左心衰竭（5例）。手术类型：Wheat手术4例，传统或改良Cabrol手术13例，David手术1例，Bentall手术83例。同期行主动脉半弓置换术或降主动脉腔内支架植入术16例，全弓置换术或降主动脉腔内支架植入术4例，二尖瓣置换术或成形术14例，冠状动脉旁路移植术8例。结果全组手术死亡率为6．9％（7／101），2000年后降至3．6％（3／83）；术后主要并发症为低心排血量10例，呼吸功能不全9例，肾功能不全9例。术后随访94例，随访期间死亡1例，5例马方综合征患者术后出现B型夹层。结论Bentall手术是治疗升主动脉根部瘤的首选手术方法，术前左心功能及手术技术是影响手术效果的关键因素。     

A case of mitral valve replacement and left atrial plication for infantile Marfan syndrome with giant left atrium due to mitral valve regurgitation]

In Marfan syndrome, there is a subset, called infantile Marfan syndrome, in which the disease is diagnosed during infancy and cardiac lesions including mitral regurgitation and aortic root dilatation tend to be deteriorated rapidly. In infantile Marfan syndrome, respiratory function is sometimes impaired when skeletal abnormalities such as scoliosis and pectus excavatum are severe. In this report, we describe a 2-year and 4-months old boy with infantile Marfan syndrome who presented with severe mitral regurgitation and the collapsed left lung. In addition to the impairment of respiratory function due to severe scoliosis, the left lung was collapsed because of the compression of the left bronchus by the enlarged left atrium. The patient required mitral valve replacement concomitantly with left atrial plication, resulting in the decompression of the left bronchus and the re-expansion of the left lung. Characteristics and surgical management of infantile Marfan syndrome are discussed in this report.     

Should the transverse aortic arch be replaced simultaneously with aortic root replacement for annuloaortic ectasia in Marfan syndrome?

OBJECTIVE: The purpose of this study was to determine, on the basis of the late fate of the intact aortic arch with abnormal tissue after aortic root replacement, whether the intact aortic arch should be replaced prophylactically at the time of aortic root replacement for annuloaortic ectasia in Marfan syndrome. METHODS: A retrospective review was performed in 85 patients with Marfan syndrome who underwent aortic root replacement for annuloaortic ectasia with or without aortic dissection (mean age 37 years, range 19-61 years). These 85 patients were divided into four groups according to the postoperative condition of the residual aorta. In group I (n = 47), the patients underwent aortic root replacement for annuloaortic ectasia with or without localized dissection in the ascending aorta. In these patients the residual aorta, including the aortic arch, was therefore intact. In group II (n = 10), the aortic arch was intact, although the descending thoracic aorta was dissected because of the preoperative type B dissection. In groups III and IV, the patients had type A dissection involving the transverse arch associated with annuloaortic ectasia. In group III (n = 13), residual dissection existed in the descending thoracic aorta after concomitant total arch replacement. In group IV (n = 15), the aortic arch and the descending thoracic aorta were dissected. RESULTS: There were 5 early deaths (3 in group I, 1 in group II, and 1 in group III). Subsequent operations were required in 10, 5, 6, and 7 cases in groups I, II, III, and IV, respectively. Regarding the aortic arch, only 2 of 53 survivors of the initial hospitalization with an intact aortic arch (groups I and II) underwent subsequent total arch replacement for the onset of dissection in the aortic arch, and 4 of 14 survivors of the initial hospitalization with a residual dissecting arch (group III) needed subsequent total arch replacement. Actuarial freedom from arch repair among patients with an intact aortic arch (91% at 15 years) was significantly higher than that among patients with a residual dissecting arch (49% at 15 years, P =.0078). CONCLUSIONS: The incidence of new dissection in the residual intact arch after aortic root replacement was extremely low. Therefore prophylactic replacement of the intact arch does not appear to be necessary at aortic root replacement for annuloaortic ectasia in Marfan syndrome.     

Cardiovascular abnormalities in the relatives of patients operated upon for annulo-aortic ectasia. A clinical and echocardiographic study of 40 families

This study is based on 40 consecutive patients operated on for annuloaortic ectasia at the Turku University Central Hospital between March 1970 and April 1983. Supracoronary graft insertion was used in the first nine patients, while the remaining patients were treated by radical repair using a composite graft and reimplantation of the coronary ostia. The mean age of the patients was 40.3 years (10 to 66 years). The patients were examined postoperatively for their physical, radiological, and ophthalmological parameters. Eight had the classic Marfan syndrome. The patients had 230 first-degree relatives, and 200 of them were alive. Three of the 30 deceased relatives had died due to rupture of an ascending aortic aneurysm; 187 of the living relatives were examined as above. In addition, echocardiography was performed. Twenty-six of the surviving relatives had dilatation of the aortic root; 24 were siblings, and two were children of the operated patients. Echocardiography of the relatives revealed an increased incidence of mitral valve prolapse and enlargement of the left ventricle and left atrium. None of the relatives examined had the classic Marfan syndrome, although the incidence of some Marfan features occurred more frequently in the Marfan families. It seems clear that annuloaortic ectasia, even without the Marfan syndrome, is a hereditary disease. To detect annuloaortic ectasia at an early stage, examination of the first-degree relatives of patients operated upon is recommended.     

Acute type A aortic dissection complicated by aortic regurgitation: composite valve graft versus separate valve graft versus conservative valve repair

OBJECTIVE: To clarify the merits of various surgical approaches, we studied the outcome after composite valve graft versus separate valve and graft replacement versus conservative valve treatment with replacement of the ascending aorta in patients with acute type A aortic dissection complicated by aortic regurgitation. METHODS: Between 1967 and 1999, 123 patients (mean age 56 +/- 15 years) underwent composite valve graft replacement (n = 21), separate valve and graft replacement (n = 20), or conservative valve treatment (n = 82 [commissural resuspension in 46]); follow-up averaged 6.5 years (95% complete). RESULTS: The 30-day, 1-year, and 6-year survival estimates of 85% +/- 4%, 79% +/- 5%, and 69% +/- 5% (+/-1 standard error of mean), respectively, after conservative valve treatment were similar to 86% +/- 8%, 81% +/- 9%, and 65% +/- 16%, respectively, with composite valve graft replacement and better (but insignificantly so) than 70% +/- 10%, 70% +/- 10%, and 45% +/- 11%, respectively, with separate valve and graft replacement. The 6-year freedom from proximal reoperation was 95% +/- 3%, 89% +/- 10%, and 100% in conservative valve graft, separate valve and graft, and composite valve graft subgroups, respectively (P = not significant). Cox regression multivariable analysis identified that previous sternotomy (hazard ratio [or e(beta)] 95% confidence interval 1.4-10.9, P =.006), hypertension (0.99-2.9, P =.05), cardiac tamponade (1.1-4.0, P =.03), and stroke (1.7-7.0, P =.001) increased the hazard of death. No factors predicting a higher likelihood of late proximal reoperation were identified. CONCLUSIONS: In patients with acute type A aortic dissection and aortic regurgitation, there was no significant difference in overall survival or reoperation rates among these surgical approaches. We try to save the valve whenever possible unless the aortic root is pathologically dilated (eg, Marfan syndrome or annuloaortic ectasia) or destroyed by the dissection process, when composite valve graft or valve-sparing aortic root replacement is indicated.     

Replacement of the mitral valve, aortic valve, and ascending aorta with coronary transplantation in a child with the Marfan syndrome

A girl with the Marfan syndrome is described who developed mitral regurgitation, an aneurysm of the ascending aorta, and severe aortic regurgitation. Complete surgical correction required mitral valve, aortic valve, and ascending aorta replacement with transplantation of the origin of the coronary arteries.     

Coil embolization of a gluteal false aneurysm in a patient with Marfan syndrome

The classic cardiovascular complications of Marfan syndrome are annuloaortic ectasia, mitral valve disease, and aortic dissection. These conditions can be explained by a progressive loss of connective tissue elastic fibers of the inner arterial wall that lead to its fragility and distensibility. Other aneurysmal locations have been reported in relation to this condition, though clearly less frequently. We report here the case of a patient with Marfan syndrome who successively had annuloaortic ectasia and Barlow's disease, which were successfully treated in 1987, a false aneurysm on the reimplantation of the right coronary artery that necessitated reintervention in 1992, and a gluteal false aneurysm diagnosed and treated in 1993.     

An aortic valve-sparing operation for patients with aortic incompetence and aneurysm of the ascending aorta.

A number of patients who require an operation for complications of annuloaortic ectasia, such as aortic incompetence or aneurysm of the aortic root (or both), have normal aortic valve leaflets. We have treated these patients by excising the aneurysmal portion of the ascending aorta and sinuses of Valsalva but by leaving the aortic valve leaflets and some arterial wall attached to the left ventricular outflow tract. The aortic valve is reimplanted inside a collagen-impregnated tubular Dacron graft, similar to what is done for implantation of an aortic valve homograft. The coronary arteries are also reimplanted. This operation was performed in 10 patients. All patients had annuloaortic ectasia and five had the stigmata of Marfan syndrome. Four patients had acute aortic dissection. There were no operative deaths, but one patient required composite replacement of the aortic valve and ascending aorta because of persistent aortic incompetence. Postoperative Doppler echocardiography revealed normal aortic valve function in six patients and mild incompetence in three. The preliminary results of this new operation are encouraging. Further investigation is necessary to establish the best size, shape, and type of material that should be used to replace the aortic root while preserving the aortic valve.     

Marfan's syndrome with annulo-aortic ectasia and ruptured mitral chorda--a case report of combined composite valve graft replacement of the aortic root and mitral valve replacement]

We report a case of Marfan's syndrome with acute heart failure caused by a ruptured mitral chorda that was successfully treated by one operation of combined composite valve graft replacement of aortic root and mitral valve replacement (MVR). A 23-year-old man was admitted to our hospital presenting severe dyspnea and chest pain. Echocardiography and cardiac catheterization studies demonstrated marked annulo-aortic ectasia, aortic regurgitation and significant mitral regurgitation due to a ruptured chorda. In operation, it was found that a chorda of the mitral posterior leaflet had been torn, with the leaflet completely prolapsed to the left atrium, and that the aortic root was dilated to 90 mm in diameter. The ascending aorta was extensively resected leaving those areas of aortic tissue involving the coronary ostia. Then the mobilized coronary arteries were reattached to the composite graft. MVR was performed with preservation of the whole anterior and posterior mitral valve apparatus except for that small part with the torn chorda. Histopathological findings of the aortic wall and mitral valve were compatible with those of Marfan's syndrome.     

Extensive aortic reconstruction for aortic aneurysms in Marfan syndrome

Background. Marfan syndrome patients frequently develop aneurysms or dissections involving multiple segments of the aorta, and occasionally require staged replacement of the entire aorta. This study reviews the surgical outcome of patients with Marfan syndrome who underwent extensive aortic reconstruction. Extensive reconstruction is defined as reconstruction of more than two segments of the ascending, arch, descending thoracic, or abdominal aorta.

Methods. From March 1973 to December 1997, 101 patients with Marfan syndrome underwent aortic operation. Twenty-six patients (25.7%) had extensive aortic reconstruction. All 26 patients suffered from aortic dissection: 13 patients had Stanford type A and 13 had type B dissection. Twenty-three patients (88.4%) had annuloaortic ectasia and aortic regurgitation. Surgical procedures included composite valve graft replacement (n = 23, 88.4%), aortic arch reconstruction (n = 15, 57.7%), graft replacement of the descending thoracic aorta (n = 6, 23.1%), and graft replacement of the thoracoabdominal aorta (n = 16, 61.5%). Five patients (19.2%) had total thoracoabdominal aortic replacement, and three patients (11.5%) had replacement of the entire aorta. Twenty-one patients (80.8%) required multiple operations.

Results. Follow-up was complete in all patients. The 30-day survival rate was 88.5%. None of the survivors had paraplegia or paraparesis. The overall long-term survival rate was 88.5 ± 6% at 1 year, and 81.7 ± 9% at 9 years.

Conclusions. Aortic surgery prolongs survival in patients with Marfan syndrome, and currently there is a relatively low associated morbidity and mortality even for aggressive surgical treatment.     

Cardiac surgery for annuloaortic ectasia and mitral regurgitation in an adult patient with dextrocardia

We report a surgical case of dextrocardia complicated with annuloaortic ectasia (AAE) and mitral regurgitation, which induced congestive heart failure. Preoperative electrocardiography-gated multidetector-row computed tomography (MDCT) showed the following complex cardiovascular abnormalities without motion artifacts: dextrocardia, situs inversus, polysplenia, AAE, absence of the inferior vena cava, azygos vein continuation, drainage of the hepatic vein into the right atrium, and bilateral superior venae cavae. On the basis of the MDCT data, we established a cardiopulmonary bypass; and a modified Bentall procedure (Piehler method) and mitral valve replacement were performed without complications.     

Successful consecutive seven time's surgical repair of Marfan syndrome

We report a patient with Marfan syndrome who received successful 7 consecutive operations during 11 years. She underwent descending aortic replacement for chronic type B dissection at the age of 24. Mitral valve replacement (MVR) was performed for mitral regurgitation (MR) at the age of 30, and abdominal aortic replacement was done for persistent dissection at the age of 31, aortic root and arch replacement was done at the age of 34. The 9 months later, she received re-MVR for dysfunction of bioprosthesis and tricuspid valve anuloplasty (TAP) for tricuspid regurgitation (TR). But severe paravalvuler leakage of mitral valve necessitated direct closure of detachment. Thoracoabdominal replacement was performed for rupture of persistent dissection at the age of 35. She was discharged on the 54th day after the 7th surgery.     

Surgical Management of Marfan Syndrome in Children

Between August 1983 and January 1991, seven patients with Marfan syndrome underwent surgery for severe cardiovascular complications. The mean age at presentation was 5.7 months (range 4 to 9 months) in the infant group (n = 3), and 13.3 years (range 10 to 16 years) in a group of older children (n = 4). The primary indications for surgery in the infant group (performed at a mean of 3 years after diagnosis) were ascending aortic aneurysm with valvar regurgitation in one patient, and severe mitral valve prolapse with regurgitation in two. In the older group, surgical indications (performed at a mean of 2.8 years after diagnosis) were ascending aortic aneurysm with valvar regurgitation in three patients and acute aortic dissection in one. For aortic surgery, a composite valved conduit was used in four patients, and an aortic homograft in one. For mitral valve surgery, mechanical prostheses were used. Ail patients survived the primary operation. Over a mean follow-up of 17.5 patient-years (range 1 to 9 years), two patients in the infant Marfan group went on to further successful surgery (prosthetic mitral valve replacement and aortic root repair with aortic homograft) at a mean interval of 4.3 years after the Initial surgery. Our results suggest that the major cardiovascular risk factors of Marfan syndrome in the young, even in those diagnosed during infancy, have been favorably changed by surgery with an encouraging medium-term outlook. The correct timing of surgery is aided by echocardiography. (J Card Surg 1994;9:50–54)