[Obtaining Operative Field for the Repair of Thoracic Descending and Thoracoabdominal Aortic Aneurysms].

Left thoracotomy with spiral incision is conventional approach for the repair of descending and thoracoabdominal aortic aneurysms. Because the aneurysms' locations and ranges are various, case-oriented approaches including body postures and the positions of thoracotomy are necessary. Preoperative computed tomography assessment of aneurysms is important for decision making of both operative indication and approach.

Mitral Valve Repair for Mitral Regurgitation in Patients With Marfan Syndrome.

Background: There is concern about the durability of mitral valve repair (MVr) for mitral regurgitation (MR) in Marfan patients due to limited long-term data. Furthermore, a detailed time course of changes in cardiac function after MVr in Marfan patients has not been reported. We examined repair techniques, postoperative cardiac function, and outcomes of MVr in Marfan patients.

Management of an elderly patient with nonsyndromic -related aortopathy: A case report.

Key Clinical Message: Genetic variants associated with hereditary TAAD may contribute to nonsyndromic TAAD. We present the case of a 72-year-old man with nonsyndromic TAAD undergoing prophylactic surgery after a gene panel test revealed a pathogenic variant in , but the indication for genetic testing in such elderly-onset cases still warrants further discussion.

Abstract: Hereditary thoracic aortic aneurysm and dissection (TAAD) is a clinical condition resulting in a fatal outcome.

Posttraumatic aortic regurgitation and root pseudoaneurysm following blunt chest trauma: a case report.

Background: The simultaneous diagnosis of severe aortic regurgitation and aortic root pseudoaneurysm resulting from traffic injury is extremely rare. This report presents the case of a patient with Marfan syndrome who experienced aortic root pseudoaneurysm and subacute severe aortic regurgitation following a traffic accident.

Case Presentation: A 64-year-old woman was diagnosed with Marfan syndrome 16 years ago and is undergoing ongoing follow-up at an outpatient clinic.

[Surgical Replacement of Descending Aorta for Adult Aortic Coarctation by Using Three-dimensional Computer Graphics Reconstruction].

Aortic coarctation is diagnosed in approximately 5% of adult patients with congenital heart disease and is commonly diagnosed through the close examination of hypertension. Various surgical strategies for adult coarctation have been recently reported. Generally, aortic replacement may require blood transfusion in case of injury of the well-developed collateral vessels.

Impact of prolonged cardiopulmonary resuscitation on outcomes in heart transplantation with higher risk donor heart.

Objectives: To evaluate the influence of prolonged cardiopulmonary resuscitation (CPR) on outcomes in heart transplantation with higher risk donor hearts (HRDHs).

Methods: Patients transplanted in our hospital between May 2006 and December 2019 were divided into 2 groups, HRDH recipients and non HRDH recipients. HRDH was defined as meeting at least one of the following criteria: (1) donor left ventricular ejection fraction ≤ 50%, (2) donor-recipient predicted heart mass ratio < 0.

A novel pathogenic variant located just upstream of the C-terminal Ser423-X-Ser425 phosphorylation motif in SMAD3 causing Loeys-Dietz syndrome.

Objective: Loeys-Dietz syndrome (LDS) is a heritable disorder of connective tissue closely related to Marfan syndrome (MFS). LDS is caused by loss-of-function variants of genes that encode components of transforming growth factor-β (TGF-β) signaling; nevertheless, LDS type 1/2 caused by TGFBR1/2 pathogenic variants is frequently found to have paradoxical increases in TGF-β signaling in the aneurysmal aortic wall. Here, we present a Japanese LDS family having a novel SMAD3 variant.

Recurrent aneurysm 11 years after thoracoabdominal aortic replacement due to recanalization of intercostal arteries of the wrapping native aorta in Marfan syndrome: a case report.

Background: Persistent blood flow is common in aortic aneurysms following endovascular repair. However, the recanalization of thrombosed intercostal arteries after open aortic repair is rare. We report a case of aortic aneurysm recurrence 11 years after thoracoabdominal aortic replacement resulting from recanalization of the intercostal arteries like a type II endoleak in a patient with Marfan syndrome.

Modified Ravitch procedure for pectus excavatum in Marfan syndrome with annuloaortic ectasia.

Objectives: This case series aimed to determine the feasibility of simultaneous modified Ravitch and David procedures for Marfan syndrome patients with pectus excavatum and annuloaortic ectasia.

Methods: Between March 2014 and December 2019, 7 consecutive patients underwent simultaneous surgery of modified Ravitch and David procedures for pectus excavatum and annuloaortic ectasia. The completion of cardiac surgery and sternal closure were followed by the modified Ravitch procedure.

[Aortic Root Replacement with Homograft].

Infectious endocarditis is a severe infectious disease in cardiovascular surgery fields. Appropriate antibiotics administration is the principle of treatment, while surgical intervention is required when there is intensive tissue destruction, refractory infection, or high risk of embolism. Usually, surgical risks of infectious endocarditis are rather high as preoperative general condition is often poor.

The Myocardial Accumulation of Aggregated Desmin Protein in a Case of Desminopathy with a de novo DES p.R406W Mutation.

Desminopathy is a cardiac and skeletal myopathy caused by disease-causing variants in the desmin (DES) gene and represents a subgroup of myofibrillar myopathies, where cytoplasmic desmin-postive immunoreactivity is the pathological hallmark. We herein report a 28-year-old Japanese man who was initially diagnosed with sporadic hypertrophic cardiomyopathy with atrioventricular block at 9 years old and developed weakness in the soft palate and extremities. The myocardial tissue dissected during implantation of the ventricular-assisted device showed a dilated phase of hypertrophic cardiomyopathy and intracellular accumulation of proteinase K-resistant desmin aggregates.

Prophylactic negative pressure wound therapy is not effective for preventing driveline infection following left ventricular assist device implantation.

Background: Driveline infection (DLI) following left ventricular assist device (LVAD) implantation remains an unresolved problem. Negative pressure wound therapy (NPWT) promotes wound healing by applying negative pressure on the surface of the wound. Recently, the prophylactic application of NPWT to closed surgical incisions has decreased surgical site infections in various postsurgical settings.

[Aortic Root Pseudoaneurysm].

Aortic root pseudoaneurysm is often derived from suture sites after aortic root replacement on the basis of connective tissue disease, aortitis, or prosthetic valve endocarditis. Preoperative computed tomography (CT) and echocardiography are useful not only for diagnosing a pseudoaneurysm but also for planning to repair it. Redo aortic root replacement is necessary to repair it in most cases.

Giant internal carotid artery aneurysms and porcelain aorta in an elderly patient with Marfan syndrome.

Marfan syndrome (MFS) an inherited disorder caused by gene variants, is well known to cause lethal aortic aneurysm and dissections at a relatively young age. Here, we report giant internal carotid artery aneurysms (ICAAs) and porcelain aorta in an elderly patient with MFS.

Aortic valve replacement of a quadricuspid aortic valve with right coronary artery ostium adjacent to one of the commissures.

Background: Quadricuspid aortic valve is a rare congenital heart disease that may be associated with a different anatomical relationship between the coronary artery ostium and the commissure.

Case Presentation: Herein, we report a case of a 59-year-old woman who underwent aortic valve replacement for a quadricuspid aortic valve with severe aortic regurgitation. Intraoperatively, the aortic valve had four cusps of almost equal size and the right coronary artery arose adjacent to the commissure between the right coronary cusp and one of the two non-coronary cusps.

Perigraft seroma after total aortic arch replacement using Triplex graft.

A 42-year-old gentleman underwent total aortic arch replacement with Triplex graft (Terumo corporation, Tokyo, Japan) for acute type A dissection. Sixteen months later, a computed tomography revealed a rapidly enlarging low-density area around the graft, with no contrast enhancement. The area was compressing the mediastinal structures, such as the superior vena cava and right pulmonary artery.

Sarcopenia and risk of infection in adult heart transplant recipients in Japan.

Aims: Heart transplantation (HT) is an effective therapeutic option for end-stage heart failure. Infection is a major cause of morbidity and mortality after HT. Sarcopenia, defined as the loss of muscle mass and strength, is a common comorbidity in HT candidates with end-stage heart failure.

Association between left ventricular ejection fraction and mortality after Bentall procedure.

Root infection or dissection involving coronary artery frequently necessitates an emergent Bentall procedure, with low left ventricular ejection fraction (LVEF). In contrast, concerning an elective Bentall for aneurysm, surgeons should balance the risk and benefit of surgery, especially in low LVEF cases. We investigated the association between preoperative LVEF and outcomes after Bentall.

Proposed Management Policy for Pregnant Women with Loeys-Dietz Syndrome Following Prophylactic Aortic Root Replacement Based on Experience from a Tertiary Care Center.

Loeys-Dietz syndrome (LDS) is a connective tissue disorder with a high incidence of aortic dissection (AD). After treating two previously reported cases of postpartum AD in women with LDS following prophylactic aortic root replacement (ARR), we succeeded in managing a 30-year-old primigravida with no AD during her peripartum period. On the basis of the patient's stated desire to conceive during preconception counseling, a multidisciplinary team was assembled.

A Novel Knitted Polytetrafluoroethylene Patch for Cardiovascular Surgery.

Polytetrafluoroethylene (PTFE) is widely used in cardiovascular surgeries; however, postoperative complications such as thrombosis, calcification, and neointimal hyperplasia are yet to be resolved in patients. We developed two types of novel knitted PTFE patches and evaluated them using a swine model. Both patches were composed of knitted PTFE impregnated with micro-PTFE particles, and one of them was pressed after PTFE impregnation.