Marfan's syndrome: combined composite valve graft replacement of the aortic root and transaortic mitral valve replacement

Echocardiographic studies in patients with Marfan's syndrome indicate that 95% eventually have aortic root dilatation, mitral valve prolapse, or a combination of these. Both lesions can progress and require operation for aortic root aneurysm, aortic valve insufficiency, and mitral valve insufficiency. This report reviews the simplified treatment of 6 patients by combined composite valve graft replacement of the aortic root and transaortic mitral valve replacement at one operation. All patients survived and are well for periods extending from 1 month to 12 months.     

Simultaneous corrections Bentall procedure, mitral valve replacement and sternoplasty for a patient with Marfan's syndrome

A successful case of simultaneous modified Bentall' procedure, MVR and sternoplasty was presented for a 42 years old man of Marfan's syndrome who had funnel chest, combined with mitral regurgitation, aortic regurgitation and annuloaortic ectasia. A few reports of one stage operation for the combination of funnel chest and heart disease in Marfan's syndrome has been published. A median sternotomy was made, with total cardiopulmonary bypass, heart was arrest and cor cooling hypothermia with crystalloid cardioplegic solution. Mitral valve was replaced with #31 B-S prosthetic valve. The ascending aorta proximal to the innominate artery and the aortic valve were replaced en-bloc with composite graft made of a vascular graft and aortic valve prostheses. Coronary artery orifices cut to the button-shaped, were sutured to the composite graft. Finally, sternoplasty was completed through modified Ravitch sterno-elevation. This is the first report in Japanese journals.     

One-staged operation for mitral regurgitation and annuloaortic ectasia without aortic regurgitation with Marfan syndrome; report of a case

A 32-year-old man with Marfan syndrome was admitted to our hospital for detail examination of congestive heart failure. Doppler echocardiography showed severe mitral regurgitation due to prolapse of posterior mitral leaflet. Annuloaortic ectasia without aortic regurgitation was also detected by aortography. Considering the future operative need for aortic root and ascending aorta, we performed mitral valve replacement with a mechanical valve and preventive concomitant aortic root replacement with a composite valve graft. His postoperative course was uneventful. Optimal surgical treatment of mitral regurgitation and annuloaortic ectasia in Marfan syndrome is controversial because the underlying connective tissue defect theoretically might compromise repair durability. Several surgical options for mitral regurgitation and annuloaortic ectasia in Marfan syndrome are discussed.     

Neochordal repair of a double orifice mitral valve

Native double-orifice mitral valve is an exceedingly rare valvular abnormality. A 77-year-old patient was referred for a severe mitral regurgitation, and a double-orifice mitral valve with a central fibrous bridge was unexpectedly found. A flail anterior leaflet with 2 ruptured chordae was observed on the anterolateral mitral valve orifice, the leaflets on the posterior valve orifice were normally thin. Mitral valve repair was successfully performed with replacement of the 2 torn chordae by two 4/0 expanded polytetrafluoroethylene neochordae. We accordingly suggest that current mitral valve repair techniques remained a valuable option, even on central bridge double-orifice mitral valve regurgitation.     

Left ventricular rupture following mitral valve replacement with preservation of posterior leaflet

Left ventricular rupture following mitral valve replacement (MVR) with preservation of the posterior leaflet is presented. A 63-year-old man underwent combined AVR, MVR with preservation of the posterior leaflet and TAP under cardiopulmonary bypass with moderate hypothermiat to 28°C and tepid blood cardioplegia. Although a 31-mm valve would fit to the mitral position, a 27 TEKNA was chosen for MVR. Beyond the cardiopulmonary bypass, a left ventricular rupture was found. Cardiopulmonary bypass was re-stated and the aortic clamp was replaced. After removal of the mitral valve prosthesis, an internal tear was detected below the anterolateral commissural area (Type I). The tear was repaired with two 3-0 monofilament buttressed sutures incorporated with felt strips and covered with a bovine pericardial patch by several interrupted pledgeted mattress sutures. Gelatin-resorcin-formaldehyd/Glutaraldehyde glue (GRF) was used to reinforce the ruptured myocardium and to fix a bovine pericardial patch to the affected ventricular wall. Then the prosthesis was re-seated. Although the aortic clamp time was 319 mins., the patient was weaned from the cardiopulmonary bypass easily with the prophylactic use of IABP. The patient was discharged and returned to his previous job.     

Valve-in-valve replacement of primary tissue valve failure of bovine pericardial valve minor

A 73-year-old woman who underwent mitral valve replacement with a 31 mm Carpentier Edwards Pericardial Xenograft 19 years ago. She revealed sudden onset of a grade IV/VI a seagull like diastolic murmur at the apex, and severe hematuria. Echocardiography demonstrated severe mitral regurgitation. These findings were consistent with acute primary tissue valve failure. Therefore we performed emergency reoperation. At operation, valve leaflet was torn at the commissural stitch, and bioprosthesis strut was buried in the left posterior ventricular wall. The mitral prosthetic valve replaced with a 25 mm CarboMedics OptiForm using a technique of valve-in-valve replacement. This procedure would be one option for replacement of bioprosthetic mitral valve.     

Our experience with aortic root replacement using hand-sewn composite valve graft

Background The purpose of this study is to examine our experience with aortic root replacement using composite valve grafts in patients with proximal aortic disease. Methods and Results Since 1986, 16 patients underwent aortic root replacement using composite valve grafts for various indications which were Marfan's syndrome with annuloaortic ectasia (n=2), aortic regurgitation with ascending aortic aneurysm (n=3), aortic stenosis with regurgitation with poststenotic dilatation (n=9) and progressive aortic dilatation following AVR (n=2). There were 12 males and 4 females. The age range varied between 19 years and 52 years. The choice of conduit was a custom made valved graft using a monoleaflet tilting disc valve (Medtronic Hall valve=11, St Vincent's valve=3, TTK Chitra=1 and Bjork Shiley Valve=1) placed in a Dacron graft (Hemashield=13, Verisoft Cooley=3) prepared using a 4-0 polypropylene suture. The average time taken to construct a valved graft was 20 min±8 min. The coronary implantation was by isolated button technique. There was no size mismatch encountered in any patients. Postoperative bleeding necessitated a delayed sternal closure in 3 patients and re-exploration in another 2. The immediate operative mortality was 2, the cause being ventricular arrhythmia in one and cerebrovascular accident in other. Follow-up period ranged from 2 months to 16 years with a median follow-up of 12 months. One patient had an asymptomatic perigraft collection and no intervention was needed. There was no valve related complications in any of these patients. There was one late death of unknown cause. Conclusions Aortic root replacement using a custom made composite graft offers excellent long-term results, with negligible mortality rate and prosthesis related complications. Custom made grafts make this conduit easily affordable in our country and their performance is comparable to other available composite grafts.     

Bilateral pneumothorax and rupture of dissecting aortic aneurysm following a mitral valve replacement in Marfan syndrome: a case report]

A 12-year-old girl with Marfan syndrome was referred to our department because of severe mitral regurgitation. The patient was pale and in a pre-shock condition. The echocardiogram revealed a chordae rupture of the posteromedial papillary muscle at the posterior leaflet. A moderate dilatation of the aortic annulus (30 mm) and the ascending aorta (45 mm) without aortic regurgitation was also observed. Emergent mitral valve replacement was performed without replacement of the aortic valve or the ascending aorta. After two months, the patient developed bilateral pneumothorax, which was resistant to continuous suction therapy and finally required surgical treatments. Despite the relatively uneventful recovery, a lethal rupture of dissecting aortic aneurysm into the pericardial cavity, developed four months after the initial operation. We discussed the desired surgical approaches and respiratory problems in patients with connective tissue disorder.     

Mitral valve replacement through dilated aortic annulus in Marfan's syndrome.

A 30-year-old man presented with severe aortic regurgitation due to an ascending aortic aneurysm and mitral valve insufficiency in conjunction with active endocarditis. He underwent combined aortic root replacement by a composite graft and mechanical mitral valve replacement. After excision of the aortic valve cusps the mitral valve was approached through the extremely dilated aortic annulus. This technique is seldom used. Mitral valve replacement through the aortic root can be advantageous in selected patients to minimize trauma and reduce myocardial ischemic time.     

Double orifice mitral valve associated with bicuspid aortic valve

A 63-year-old man with double orifice mitral valve (DOMV) and bicuspid aortic valve was reported. Preoperative echocardiography showed prolapse of the posterior leaflet and mitral regurgitation but was unable to show the existence of the duplication of the mitral valve. He underwent aortic and mitral valve replacement and did well after surgery. DOMV is a rare congenital malformation, and DOMV associated with bicuspid aortic valve is the first reported case in Japan.     

A case report of perforated aneurysm of mitral valve with aortic regurgitation

The patient was a 71-year-old male who complained of palpitation and tachycardia. The echocardiogram showed a bulging of the anterior mitral valve leaflet toward the left atrium that persisted throughout cardiac cycle. The cine angiogram showed deformity of the anterior mitral valve leaflet with severe mitral regurgitation and mild aortic regurgitation. At operation, a perforated aneurysm was recognized at the anterior mitral valve leaflet without thrombus and vegetation. The size of aneurysm was 40 x 25 x 25 mm. The patient underwent MVR + AVR, and the postoperative course was uneventful. Pathological examination of the anterior mitral valve leaflet revealed scar-like fibrosis and old inflammatory change. It was judged a true aneurysm of mitral valve, because the structure of endocardium was kept.     

Tricuspid valve replacement after cardiac transplantation.

Tricuspid regurgitation, a fairly common finding after cardiac transplantation, is generally mild or moderate, and is not clinically significant. The etiology of tricuspid regurgitation is not entirely understood, and experience with valve replacement after cardiac transplantation is limited. We describe a case of progressively severe tricuspid regurgitation ultimately requiring tricuspid valve replacement. At operation, the ruptured chordae of the posterior part of anterior and septal leaflet with resulting partially flail leaflets were found. Examination of the papillary muscle showed origins of several of the ruptured chordae. Damage to the tricuspid subvalvular apparatus at endomyocardial biopsy appeared to be a possible cause. A 31-mm Carpentier-Edwards porcine valve was implanted. This was because replacement with a mechanical prosthesis would prevent future right-side heart catheterization and endomyocardial biopsy and in valve repair, the patient remains exposed to the risk of the recurrence of chordal rupture. We discuss proposed causes and choices in surgical technique.     

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.     

Aortic and mitral valve replacement for calcified valvular annulus in a dialysis patient; report of a case

A 77-year-old man on hemodialysis was admitted to our hospital due to heart failure. Echocardiography showed aortic valve stenosis and regurgitation, mitral valve stenosis and regurgitaion, and tricuspid valve regurgitation. Catheter examination revealed severe calcification at aortic valve and mitral valve including their annulus. At the operation, the calcifications of the aortic and mitral valvular annulus was removed using a cavitron ultrasonic surgical aspirator (CUSA). Reconstructions of the defect of the posterior part of the mitral annulus and of the aortic annulus at the site of the left coronary cusp were achieved by patch technique using autologous pericardium. Aortic and mitral valve replacement and tricuspid valve annuloplasty were performed. The postoperative course was uneventful. Operative technique to remove calcification from valvular annulus using CUSA and reconstruct of the defect of the annulus with autologous pericardium is a very useful technique to prevent left ventricular rupture, perivalvular leakage and any other complications.     

Mitral valve replacement after percutaneous transvenous mitral commissurotomy

Percutaneous transvenous mitral commissurotomy is widely performed as the first choice of the non-pharmacological treatments for mitral stenosis. Five patients have been identified who required mitral valve replacement after percutaneous transvenous mitral commissurotomy. The causes leading to mitral valve replacement were mitral regurgitation in three cases and insufficient commissurotomy in two. Massive mitral regurgitation is one of the most serious complications of percutaneous transvenous mitral commissurotomy. This report aims to elucidate the mechanism of massive mitral regurgitation occurring during percutaneous transvenous mitral commissurotomy. In every such case, there was a large tear in the posterior leaflet without any split in the commissures. The Japanese literature reports that 16 patients have undergone mitral valve replacement for massive regurgitation after percutaneous transvenous mitral commissurotomy; 14 of these cases had a tear in one of the leaflets and no evidence of splitting of the posterior commissure. These facts indicate that relative fragility of the leaflets as compared with rigidity of commissural fusion, especially in the posterior commissure, is an important factor of massive mitral regurgitation during percutaneous transvenous mitral commissurotomy. Copyright © 1996 Published by Elsevier Science Ltd.     

Emergent David-V operation for a ruptured aortic root aneurysm in a 9-year-old child.

Ruptured aortic root aneurysm is very rare in children less than 10 years of age. Isolated dilatation of the ascending aorta and/or aortic root in a child is mostly associated with Marfan's syndrome, and the standard surgical treatment is aortic root replacement with a composite valve graft or homograft. We report here a successful emergent T. David-V operation using two grafts of different sizes for a ruptured aortic root aneurysm in a 9-year-old child with Marfan's syndrome.     

Mitral valve replacement with posterior transposition of the anterior mitral leaflet which covers and buttresses partially decalcified posterior mitral annular bed

Mitral valve replacement (MVR) in the presence of the extensive calcification of the mitral annulus is a technical challenge. The heavily calcified annulus can cause great difficulty in the insertion of a prosthetic valve and periprosthetic leakage later on. Vigorous annular decalcification may cause circumflex coronary artery injury, atrioventricular rupture and thromboembolic events. We herein describe a surgical technique for MVR in such cases while focusing on partial decalcification of the posterior mitral annulus and its reinforcement and buttressing with the transferred anterior mitral leaflet (AML). At the same time, the transferred AML supports the posterior annular region and maintains ventricular-annular continuity, thus preserving the left ventricular function.     

Kay's mitral valve repair for mitral regurgitation due to ruptured chordae tendineae--clinical and hemodynamic observations

Kay's mitral valve repair was performed in six consecutive patients with symptomatic mitral regurgitation due to ruptured chordae tendineae of the posterior leaflet. All patients including one with mild residual murmur showed a marked decrease in the heart size and significant clinical improvement. Postoperative hemodynamic studies, performed in four patients, showed restoration of normal or near-normal dynamics. The medium-term follow-up, 15 to 30 months after the operation, revealed continuing asymptomatic states in all. The function of the repaired valves was significantly better than that of the Hancock mitral bioprostheses. The advantages of this procedure compared with mitral valve replacement for the same condition were stressed.     

Mitral valve repair with anterior leaflet augmentation for rheumatic mitral valve disease

A 74-year-old male with congestive heart failure was referred to our hospital, and massive mitral regurgitation as well as aortic stenosis and regurgitation were detected by echocardiography. His mitral valve was successfully repaired with anterior leaflet augmentation with the equine pericardial patch followed by aortic valve replacement. Postoperative transthoracic Doppler echocardiography revealed no mitral regurgitation. The patient recovered uneventfully and was discharged on the 19th postoperative day. At 2 years and 2nd month after the operation, he is well without limitation of daily activities and any evidence of mitral regurgitation.     

Kay's mitral valve repair for mitral regurgitation due to ruptured chordae tendineae clinical and hemodynamic observations

Kay's mitral valve repair was performed in six consecutive patients with symptomatic mitral regurgitation due to ruptured chordae tendineae of the posterior leaflet. All patients including one with mild residual murmur showed a marked decrease in the heart size and significant clinical improvement. Postoperative hemodynamic studies, performed in four patients, showed restoration of normal or near-normal dynamics. The medium-term follow-up, 15 to 30 months after the operation, revealed continuing asymptomatic states in all. The function of the repaired valves was significantly better than that of the Hancock mitral bioprostheses. The advantages of this procedure compared with mitral valve replacement for the same condition were stressed.