Acquired von Willebrand syndrome secondary to mitral and aortic regurgitation.

BACKGROUND: The association of aortic valve stenosis with gastrointestinal bleeding was first described by Edward Heyde in 1958. Since then, there have been numerous case reports of Heyde syndrome in the medical literature worldwide. AIMS: Recently, the definition of Heyde syndrome has been updated to include the combination of aortic valve stenosis, intestinal angiodysplasia, and acquired von Willebrand factor syndrome (AVWS). However, an association between aortic or mitral regurgitation and AVWS is not well established. MATERIALS & METHODS: The present case of a patient with endocarditis-associated severe aortic regurgitation and mitral regurgitation exhibited a clinically significant bleeding diathesis secondary to AVWS. RESULTS: After surgical valve repair, the von Willebrand factor (VWF) activity spontaneously normalized. DISCUSSION: AVWS secondary to cardiovascular diseases occurs from a selective loss of the largest multimers of VWF due to high shear forces in the blood circulation. Although it is established that stenotic valvular lesions are associated with AVWS, there have only been rare reports of regurgitant lesions leading to AVWS. We successfully treated this patient with perioperative supplementation of VWF and factor VIII.

Bilateral banding of malpositioned pulmonary artery branches.

This report presents two rare cases of malpositioned pulmonary artery branches managed by bilateral banding. The left-branch pulmonary artery, originating from and directly superior to the right branch, entered the posterior mediastinum before heading toward the left pulmonary hilum. The ostium of the left branch could not be visualized by midline sternotomy. Therefore, a double-subtraction technique was used to pass the banding tape around the left branch from the right side. The tape location was adjusted and confirmed by intraoperative echocardiography.

Hybrid treatment of a ruptured aneurysm in the distal aortic arch: report of a case.

An 80-year-old man was transferred to our hospital for surgical treatment of a ruptured aortic arch aneurysm. Based on a history of severe heart failure and coronary artery bypass, we considered him unsuitable for conventional open repair. He underwent a hybrid repair, in the form of supra-aortic vessel debranching followed by endoluminal aortic repair. Although the ostia of the left carotid and left subclavian arteries were occluded by the stent-graft, the left supra-aortic vessels and the left internal thoracic artery attached to the coronary artery were perfused through an extra-anatomic bypass from the right axillary artery to the left carotid artery and the left axillary artery. After additional endovascular repair for recurrent hemosputum, the patient recovered without complications. Although continued follow-up is necessary, acute hybrid arch repair seems feasible for treating ruptured aortic arch aneurysms, even in the setting of severe heart failure and a previous coronary artery bypass.

Aortic Dissection in Familial Patients with Autosomal Dominant Polycystic Kidney Disease.

Autosomal dominant polycystic kidney disease (ADPKD) is the most common congenital kidney disease. However, reports on occasional cases of aortic dissection in PKD familial patients remain scarce. Herein, we describe rare aortic dissection cases in PKD familial patients (i.e., mother and daughter) and our successful treatment experience. The mother (84 years old) and daughter (53 years old) had a referral to us to treat type A acute aortic dissection. We performed emergency surgery and successfully treated the patients with an artificial graft. For comprehensive evaluation and treatment, ADPKD patients and their families should be screened for aortic diseases.

Successful endovascular treatment of an ascending aortic pseudoaneurysm using an Amplatzer Vascular Plug II.

Pseudoaneurysms are a rare complication of cardiovascular surgery, caused by disruption of the aortic structure (adventitia, media, and intima). Some reports have observed an extremely high mortality rate associated with the open surgical repair of pseudoaneurysms. In elderly or highly frail patients, the use of less invasive procedures is preferable. In this article, we report a case of an octogenarian who had a symptomatic ascending aortic pseudoaneurysm and a history of two sternotomies and present the successful treatment strategy. We treated the patient via an endovascular procedure using an Amplatzer Vascular Plug II (AVP II). After the intervention, the symptoms of the patient resolved. A computed tomography scan performed 1 year after the procedure confirmed the exclusion of the pseudoaneurysm.

Off-pump coronary artery bypass grafting in a tracheostomy patient.

In patients who have undergone laryngectomy and have a tracheal stoma, a full median sternotomy substantially increases the risk of wound infection, osteomyelitis, mediastinitis, bleeding, tracheal injury, and poor wound healing. Several reports have been published on sternotomies and skin incisions in tracheostoma patients. Transverse bilateral thoracosternotomy, T-shaped partial sternotomy (manubrium-sparing sternotomy) with transverse skin flaps and anterolateral thoracotomy with partial sternotomy are described as successful approaches to the mediastinum for cardiac surgery. We present a successful case in which off-pump coronary artery bypass grafting (CABG) was performed in a tracheostoma patient using a low T-shaped partial sternotomy and the PAS-Port system. Good long-term results were achieved.

Ryff's psychological well-being inventory: factorial structure and life history correlates among Japanese university students.

The theoretical model of psychological well-being that encompasses six domains (self-acceptance, positive relations with others, autonomy, environmental mastery, purpose in life, and personal growth) was tested with a Japanese university student population (N = 574) using a Japanese translation of Ryff's 1989 Psychological Well-being Inventory. A factor structure similar to Ryff's original model emerged. Both depression and anxiety correlated only moderately with scores on some subscales of the inventory, suggesting the relative independence of these dimensions of psychological well-being and negative affectivity. With negative affectivity controlled, some early life experiences were significantly linked with psychological well-being: relationships with romantic partners were linked with greater autonomy and experiences which enhance self-esteem were liked with greater personal growth. Careful psychometric work on the Japanese version is required to use the scale; then a replication and extension of the present study would be feasible.

Successful surgical treatment for a thrombus straddling a patent foramen ovale: a case report.

Paradoxical embolism (PDE) occurs after embolic material passes from the venous to the arterial circulation through a right-to-left shunt, which is frequently a patent foramen ovale (PFO). We describe the case of a patient with deep venous thrombosis and an intracardiac thrombus straddling a PFO and who was successfully treated with an emergency surgery.

Malignant solitary fibrous tumor of the left ventricular epicardium.

Reports describing solitary fibrous tumors of the pericardium are rare. Surgical treatment was performed on a 49-year-old woman with a large pericardial mass. The mass was attached to the left ventricular wall with a broad stalk and was free of the parietal pericardium. It was apparent macroscopically that the tumor had invaded the left ventricular muscle. On histopathology, the tumor was diagnosed as a solitary fibrous tumor with low-grade malignancy.

Endovascular aortic repair of patent ductus arteriosus in an adult patient.

We describe the case of a large patent ductus arteriosus in a 52-year old man, which was deemed unsuitable for coil occlusion or Amplatzer duct occluder. His ductus was successfully closed using Talent prostheses (Medtronic AVE, Santa Rosa, CA, USA). The postoperative course was uneventful.

Can early aortic root surgery prevent further aortic dissection in Marfan syndrome?

We reviewed 50 patients with Marfan syndrome who underwent surgery for aortic root pathologies comprising a root aneurysm without (n = 25; group A) and with (n = 25; group B) dissection. Aortic root repair included Bentall (n = 37) and valve-sparing (n = 13) procedures. Hospital mortality was 4.0%. Twenty-two patients required 36 repeat surgeries on the distal aorta. The main indication for re-intervention was the dilation of the false lumen. In group A, the distal aorta was stable for up to 7 years, but new dissection developed in 5 (33.3%) of the 15 patients who were followed up for >7 years after the root repair. Actuarial survival including operative mortality was 88.1 and 65.0% at 10 and 20 years, respectively; groups A and B did not significantly differ. Rates of freedom from all-cause death, new dissection or repeated aortic surgery were 60.1, 44.5 and 26.0% at 5, 10 and 15 years, respectively. Group A was significantly better than group B. Prophylactic aortic root repair apparently reduces the likelihood of overall adverse events, but it cannot guarantee the prevention of further aortic dissection. A multidisciplinary approach is needed for patients with Marfan syndrome.

Reconstruction of the free margin of the anterior mitral leaflet with autologous pericardium in active infected endocarditis.

In patients with active infective endocarditis mitral valve repair is better than mitral valve replacement, but it remains a challenge when there has been massive destruction of the rough zone of the anterior leaflet. We report a technical modification of mitral valve repair for advanced active infective endocarditis in which a widely infected rough zone and the chordae were successfully replaced with autologous pericardium and multiple artificial chordae. The procedure described here is capable of improving the prospects of mitral valve repair in advanced infective endocarditis.

Hybrid arch repair including supra-aortic debranching on the descending aorta.

A 49-year-old patient with an anastomotic pseudoaneurysm in the aortic arch was considered at high risk for conventional surgery through a median sternotomy because he had previously undergone several operations to treat aortic dissection and had a deep sternal infection after one procedure. Therefore, a hybrid repair was performed. Stent grafts were placed bridging two previously implanted aortic prostheses, which were in the ascending aorta and descending aorta, respectively. The supra-arch vessels were perfused by means of an extra-anatomic bypass from the descending aorta. The aneurysm was completely excluded from the blood flow, and the patient had no serious complications.