Comparison of long-term outcomes of the Bentall procedure between patients with and without Marfan syndrome.

The Bentall procedure, using a composite valve graft, has become one of the standard therapies for aortic root disease. Patients with Marfan syndrome are prone to aortic annular dilatation and dissection and often undergo aortic root replacement, including the Bentall procedure. Therefore, this study aimed to compare the long-term outcomes of the Bentall procedure between Japanese patients with and without Marfan syndrome. Data from 294 patients who underwent the Bentall procedure over 37 years were retrospectively analyzed. The study compared the data of patients with Marfan syndrome (n = 94) and those without it (n = 200). Patient characteristics, surgical techniques, and postoperative outcomes were evaluated. Statistical analyses were performed to identify risk factors associated with early mortality, late mortality, reoperation, and aortic root reoperation. The early mortality rate was 4.1%, with no significant difference between patients with and without Marfan syndrome. The long-term survival rates at 10, 20, and 30 years were 81.0%, 66.5%, and 49.1%, respectively, with no significant between-group differences. Aortic reoperations were more frequent in patients with Marfan syndrome; however, the number did not differ significantly between the groups. Risk factors for late mortality included diabetes and coronary reimplantation with an inclusion technique. Aortic dissection, Marfan syndrome, and smoking were risk factors for aortic reoperation. Late mortality after the Bentall procedure was comparable between Japanese patients with and without Marfan syndrome although aortic reoperation was significantly frequent in patients with Marfan syndrome. Continuous monitoring and management, including the prevention of aortic dissection and dilation of residual aorta, are crucial for patients with Marfan syndrome undergoing the Bentall procedure.

The mid-term outcomes of aortic root replacement after surgical repair for CHD.

OBJECTIVE: The purpose of this study is to assess the mid-term outcomes of aortic root replacement after repair of CHDs. METHOD: This is a single-institutional retrospective, cohort study with consecutive patients undergoing aortic root replacement after surgical repair of CHDs between 1999 and 2022. Operative indications included aortic root dilatation with/without aortic insufficiency, sinus of Valsalva rupture, or aortic dissection involving the root. RESULTS: Forty-four patients (36 male and 8 female) were enrolled. Mean age at the root replacement was 36.6 ± 11.9 years. The most frequent primary diagnosis was congenital aortic stenosis (n = 10) and ventricular septal defect (n = 10). Mean time from the surgical repair to aortic root replacement was 26.6 ± 13.0 years. Operative indications were aortic root enlargement with or without aortic valve aetiology (n = 40), sinus of Valsalva rupture (n = 2), and aortic dissection (n = 2). Forty-two patients underwent valve-replacing aortic root replacement, and two patients underwent valve-sparing, with 40 concomitant procedures. The median follow-up was 3.5 (1.3-7.6) years. There were one early and five late mortalities and five cardiovascular-related reoperations. Actuarial survival at 5-10 years after root replacement was 81.0 ± 6.6%. The cumulative incidence of cardiovascular-related reoperation and aortic root or valve-related reoperation at 5 years after root replacement was 11.9% and 5.6%, respectively. CONCLUSION: The early and mid-term outcomes of aortic root replacement for patients with a history of repair of CHDs were favourable in terms of survival and aortic root or valve-related reoperation.

Tricuspid valve replacement provides better long-term survival and tricuspid valve function than repair in patients with systemic right ventricle.

Objective: The purpose of this study is to compare the long-term outcomes of 2 different tricuspid surgeries including valvuloplasty and replacement for significant tricuspid regurgitation in patients with systemic right ventricle. Method: This is a retrospective study of 34 patients with dextro-transposition of the great arteries or levo-transposition of the great arteries with biventricular circulation and systemic right ventricle undergoing tricuspid valve surgery between April 1979 and April 2022. Patients were divided into 2 groups based on the procedure: tricuspid valvuloplasty (n = 11) and tricuspid valve replacement (n = 23). These groups were compared in terms of survival, tricuspid valve dysfunction, and tricuspid valve-related reoperation. Results: There was no significant difference between the groups in operative age, body weight, the proportion of dextro-transposition of the great arteries, Ebstein-like tricuspid dysplasia, and preoperative right ventricular volume/function. During the median follow-up of 9.7 years, there was 1 early death (tricuspid valvuloplasty group) and 4 late deaths (3 in tricuspid valvuloplasty group and 1 in tricuspid valve replacement group). There were 7 tricuspid valve dysfunctions, including 6 significant tricuspid regurgitations in the tricuspid valvuloplasty group and 1 prosthetic valve dysfunction in the tricuspid valve replacement group, and 4 tricuspid valve-related reoperations (3 in the tricuspid valvuloplasty group and 1 in the tricuspid valve replacement group) were performed. There were significant differences between the groups in survival (tricuspid valvuloplasty vs tricuspid valve replacement: 72.7 vs 94.7% at 10 years after surgery, P = .0328) and cumulative incidence of tricuspid valve dysfunction at 10 years after tricuspid surgery (tricuspid valvuloplasty vs tricuspid valve replacement: 27.3% vs 0%, P = .0121). Conclusions: Tricuspid valve replacement provided better long-term survival and tricuspid function in patients with systemic right ventricle compared with tricuspid valvuloplasty.

Favorable long-term outcomes in hospital survivors of a neonatal arterial switch operation.

Objectives: To assess long-term survival and reoperation-free survival after the arterial switch operation (ASO) and analyze the outcomes of reoperations after hospital discharge. Methods: This was a single-institution retrospective study of 476 hospital survivors of ASO since August 1982. Preoperative diagnoses included 286 transpositions of the great arteries with intact septum, 143 transpositions with ventricular septal defect, and 47 double outlet right ventricles. There were 236 neonatal ASOs, 30 aortic arch repairs, 22 concomitant left ventricular outflow tract obstruction reliefs, 16 preoperative mild pulmonary regurgitations, and 13 intramural coronary arteries. Results: During a median follow-up of 21.1 years (range, 0.1-39.2 years), 25 late deaths (5.3%) and 91 reoperations in 69 patients (14.5%) were noted. The reoperations included 44 left-sided reoperations in 34 patients (7.1%), 35 right-sided reoperations in 30 patients (6.3%), and 12 other reoperations in 12 patients (2.5%). Among the 69 patients who underwent reoperation, those with left-sided reoperations had lower survival at 15 years after reoperation compared to those with non-left-sided reoperations (91.2% vs 100%; P = .015; log-rank, 5.9). Multivariable analysis identified non-neonatal ASO, preoperative pulmonary regurgitation, intramural coronary artery, aortic arch repair, and concomitant left ventricular outflow tract obstruction relief as risk factors for left-sided reoperations. Reoperation-free survival was significantly higher in neonatal ASO compared with non-neonatal ASO (89.2% vs 75.2% at 20 years; P < .001; log-rank, 13.4). Conclusions: Hospital survivors of neonatal ASO had favorable long-term outcomes.

Endovascular treatment after the fenestrated frozen elephant trunk technique.

Recently, several centers have performed total arch replacement using the fenestrated frozen elephant trunk technique for acute Stanford type-A aortic dissection. However, the long-term results and need for additional treatment following this procedure are unclear. We report a case of a 54-year-old man who underwent endovascular therapy for endoleaks after total arch replacement using the fenestrated frozen elephant trunk technique for acute type-A aortic dissection with an isolated left vertebral artery. After the surgery, the endoleak was resolved, and the patient was asymptomatic with no neurological deficits. This strategy might be effective in similar cases.

[Early Structure Valve Deterioration after Aortic Valve Replacement with the Externally Mounted Bioprosthetic Valve( Trifecta):Report of Two Cases].

Trifecta valve is made from single bovine pericardial sheet, which is externally mounted on a titanium stent. This valve design provides good hemodynamic performance and wide effective orifice area. However, there have been some reports of early structural valve dysfunction which caused acute heart failure. Case 1:An 80-year-old man who had undergone aortic valve replacement (AVR) using Trifecta 21 mm five years ago. He presented with fever and diastolic murmur. Echocardiography showed prosthetic valve regurgitation, and the patient later developed prosthetic valve endocarditis. Case 2:An 80-year-old woman who had undergone AVR using Trifecta GT 21 mm two years ago developed sudden chest pain and dyspnea, prosthetic valve regurgitation. Both patients underwent re-AVR with a new bioprosthetic valve via re-sternotomy. Large tear was found in the right coronary cusp of the Trifecta in both cases. During Trifecta accomodation, we should avoid oversizing, and check the position of coronary ostia and sinus of Valsalva. Secure late outcome of Trifecta GT remains unproven and its use still needs caution.

Tetralogy of Fallot with severe ascending aortic enlargement in early childhood.

The Rastelli procedure is indicated for repair of tetralogy of Fallot (TOF) with pulmonary atresia. However, currently, there are no criteria available for concomitant surgical intervention for an enlarged ascending aorta in infancy. We report a rare case of TOF with pulmonary atresia complicated by severe enlargement of the ascending aorta in a girl aged 2 years and 5 months. We performed the Rastelli procedure with ascending aorta replacement. Pathological findings of the ascending aorta included fragmentation of elastic fibers and loss of smooth muscle cells. Postoperatively, the patient remained healthy and asymptomatic. This case indicates that ascending aortic enlargement in conotruncal anomalies may be associated with pathological lesion even in early childhood. Replacement of the diseased aorta could be considered as a concomitant procedure with definitive repair for conotruncal anomalies presenting with severe ascending aortic enlargement.

A case of double-chambered right ventricle due to cap-like fibrous tissue and aberrant chordal insertion of tricuspid valve in adult.

BACKGROUND: A double-chambered right ventricle is a rare congenital heart disease where an anomalous structure divides the right ventricle into two cavities. CASE: A 78-year-old man with dyspnea was referred to our institution for a double-chambered right ventricle (DCRV) and ventricular septal defect (VSD). Echocardiography showed normal left ventricular function, right ventricular hypertrophy, and mild tricuspid regurgitation. On parasternal short-axis views, color-flow Doppler studies showed a mosaic pattern through the stenotic right ventricular outflow tract (RVOT). Cardiac catheterization showed a 122 mmHg pressure gradient between the high-pressure chamber and the low-pressure chamber of the RVOT. Computed tomography showed a cap-like structure consisting of fibrous tissue, with moderate stenosis, which divided the right ventricle into two cavities. The aberrant tendinous chords supporting the anterior leaflet of the tricuspid valve were found inserted into the anterior wall of the RV (Figure 1). Resection of the cap-like fibrous tissue and abnormal muscle (Figure 2), detachment of aberrant tendinous chords of the tricuspid valve, closure of VSD by direct suture, and tricuspid valve plasty (TVP) were performed. TVP was achieved using an artificial chordae replacement with expanded polytetrafluoroethylene (CV-5) suture and a 32-mm Physio Tricuspid annuloplasty ring (Edwards Lifeline). Postoperative echocardiography revealed no RVOT pressure gradient and a normal right ventricular pressure of 21 mmHg. DISCUSSION: In conclusion, we report a rare case of DCRV secondary to a cap-like structure fibrous tissue with anomalous chordal insertion of a tricuspid valve leaflet.

[Closure of Multiple Ventricular Septal Defects by the Sandwich Technique and Pulmonary Artery Plasty after Arterial Switch Operation].

A 14-year-old girl had undergone a Jatene operation for double-outlet right ventricle with multiple muscular ventricular septal defects (VSD) at 12-day-old. During follow up periods, she was diagnosed with bilateral pulmonary artery stenosis and multiple muscular VSDs by echocardiography. Cardiac catheterization revealed elevated right ventricular pressure up to 93% of left ventricle. Multiple times balloon angioplasty failed to improve pulmonary artery stenosis. She underwent closure of multiple VSDs by the sandwich technique and pulmonary artery plasty with translocation of superior vena cava. We present operative technique for the complicated lesions in these operation.

Repair of a unique sinus of Valsalva defect in an infant.

Sinus of Valsalva aneurysm is a rare disease characterized by the partial elevation of the aortic root. Few reports are available on the surgical treatment for infants. We report the repair of an extremely rare case of a sinus of Valsalva defect with a ventricular septal defect and right ventricular outflow tract stenosis in an infant. It was not a sinus of Valsalva aneurysm, but it exhibited abnormal partial bulging of the aortic root and forming an aneurysm-like cavity within the right ventricular myocardium. We performed direct closure of the sinus of Valsalva aneurysm-like cavities and intracardiac repair in two stages. Three years after total repair, the patient remained healthy and asymptomatic.

[Aortic Regurgitation and Stenosis Associated with Ventricular Septal Defect in the Elderly;Report of a Case].

A 63-year-old man had ventricular septal defect (VSD) and had been followed up without heart failure. Recently, he had palpitation caused by atrial fibrillation and the echocardiography revealed moderate aortic valve regurgitation and stenosis with right coronary cusp prolapse due to subpulmonary ventricular septal defect. He underwent patch closure of VSD, aortic valve replacement with mechanical valve, and maze procedure. In recent years, advanced case like this patient is rare because most of patients with subpulmonary VSD and right coronary cusp prolapse are operated in childhood.

Late Period Rastelli Take-Down and Arterial Switch Operation After Rastelli Operation.

The Rastelli operation is a useful technique for treating the transposition of the great arteries. However, conduit stenosis of the right ventricular outflow tract is a late complication of the procedure. We report the case of a 35-year-old man for whom an arterial switch operation was performed to treat conduit stenosis and improve ventricular arterial alignment 28 years after he underwent a Rastelli operation to treat transposition of the great arteries with a ventricular septal defect. In the 8 years that followed the operation, he was asymptomatic and was treated with warfarin.

[Open Aortic Arch Surgery for Type â Endoleak after Thoracic Endovascular Aortic Repair Using Fenestrated Stent Graft;Report of a Case].

Thoracic endovascular aortic repair (TEVAR) has become a major procedure for thoracic aortic aneurysm and its indication is expanding. On the other hand, TEVAR specific complication is rather critical and its treatment is of increasing interest. Especially, open repair after TEVAR is sometimes demanding and case based strategy is mandatory. We experienced a case of open repair for aneurysm infection and endoleak after fenestrated TEVAR in 76-year-old man. He underwent initial aneurysmal repair using fenestrated graft 2 years ago. Five months later, debridment of infected tissue was performed because of aneurysmal infection. Type Ⅰ endoleak appeared after the surgery and expansion of the aneurysm made us decide extensive open repair. The operation was done under hypothermic circulatory arrest and selective cerebral perfusion. Partial removal of stent-graft and insertion of the open stent-graft, replacement of ascending aorta and reconstruction of neck vessels were done. Postoperative course was smooth. Open repair after TEVAR is often demanding. Sophisticated strategy for each case has to be planned.

Surgical and endovascular treatment of a bilateral deep femoral artery aneurysm.

We report the rare case of a 68-year-old man with a bilateral deep femoral artery aneurysm. Right-sided rupture was treated via plug embolization of the right deep femoral artery and ligation. In the following year, Viabahn® stent grafts were placed in the left superficial femoral artery to relieve occlusion and in the left deep femoral artery to treat the left aneurysm. The postoperative course of the patient was uneventful.

Therapeutic Strategies for Infectious Multiple Aortic Aneurysms: Thoracoabdominal Aortic Aneurysm Treatment Using a Fenestrated Stent-Graft.

Recently, it has been reported that a fenestrated stent graft is an effective option in the treatment of pararenal artery abdominal aortic aneurysm. We report the case of a 72-year-old male patient with multiple aortic aneurysms in the distal arch, thoracoabdominal aorta, right common iliac artery, as well as a pararenal abdominal aortic aneurysm. The patient was found to have a mass with a tendency of rapid expansion within a month from its discovery. Because it was a saccular aneurysm with a tendency of rapid expansion and wide spread, the risk of rupture was judged to be high, and surgical treatment became necessary. One-stage treatment was desirable; therefore, endovascular treatment with a fenestrated stent graft was selected. Four fenestrations were made to a stent graft for the celiac artery, superior mesenteric artery, and bilateral renal arteries. The postoperative computed tomography (CT) showed no branch occlusion or endoleak, and the 2-year postoperative CT showed the shrinkage and subsequent disappearance of the aortic aneurysm at the treatment site. For extensive aortic aneurysm, including pararenal artery abdominal aortic aneurysms, one-stage treatment with fenestrated stent graft was considered to be effective as a treatment strategy. (This is a translation of Jpn J Vasc Surg 2020; 29: 9-13.).

[Mitral Valve Repair in a Jehovah's Witnesses Patient with Post Myocardial Infarction Papillary Muscle Rupture;Report of a Case].

A 70-years old man was referred to our department for acute heart failure due to post myocardial infarction papillary muscle rupture. We planned emergent surgery, but he refused blood transfusion because of religious reason( Jehovah's Witness). Therefore, we chose medical therapy using intra-aortic balloon pumping and catecholamine. He was also treated with subcutaneous erythropoietin and intravenous iron supplement to increase preoperative hemoglobin. One month later, we decided to undergo mitral valve repair because he was stabilized with medical treatment. The patient underwent mitral valve repair with artificial chordae through median sternotomy. The mitral valve A3 prolapse was caused by posterior papillary muscle rupture. No blood transfusion was given and postoperative course was uneventful. We experienced successful mitral repair for post infarction papillary muscle rupture in a Jehova's Witnessess patient.

Surgical treatment of cardiac fibroma in a child with left ventricular noncompaction.

Surgical treatment of cardiac fibroma is rare in patients with left ventricular noncompaction (LVNC). Although several case reports regarding cardiac fibroma have been published, resection in a patient with LVNC has not been described. Here, we describe the surgical treatment of left ventricular fibroma in a child with LVNC. We resected a cardiac fibroma in a 10-year-old boy with LVNC to control ventricular arrhythmia. Partial resection with careful tumor dissection was performed to avoid endocardial damage and entering the ventricular cavity. The postoperative course was uneventful, and the patient remains asymptomatic without heart failure or arrhythmia. Surgical excision of cardiac fibroma can be performed safely with excellent results, even in a child with LVNC.