Acute valve malfunction with thrombosed bioprosthetic valve after surgical aortic valve replacement.

Acute valve thrombosis after bioprosthetic aortic valve replacement even under anticoagulation therapy is extremely rare. Cardiac computed tomography is a powerful imaging tool to detect valve thrombosis, and surgery is necessary in case of unstable hemodynamics.

Surgical, endovascular, and hybrid treatment of deep femoral artery aneurysm: Three case reports.

Key Clinical Message: Deep femoral artery aneurysms (DFAA) are extremely rare. We treated four DFAAs with different procedures including surgical, endovascular, and hybrid surgery. The best treatment should be selected for each individual case. Abstract: We report three cases of deep femoral artery aneurysms treated with different techniques. Case 1: A 69-year-old man with a huge deep femoral artery aneurysm underwent open reconstruction using a 6 mm expanded polytetrafluoroethylen graft. Case 2: A 67-year-old man presented with bilateral deep femoral artery aneurysms. The right-sided rupture was treated with hybrid embolization, while the left aneurysm was treated by endovascular stent-grafts deployment. Case 3: A 87-year-old man with a large deep femoral artery aneurysm underwent simply surgical aneurysmectomy. As there are many treatment options for deep femoral artery aneurysms, a comprehensive preoperative assessment is essential, encompassing an evaluation of symptoms, anatomy, and comorbidities.

Emergency Transcatheter Aortic Valve Implantation in a Surgical Aortic Bioprosthetic Valve for Acute Decompensated Heart Failure Caused by Sudden Progression of Structural Valve Deterioration.

Bioprosthetic aortic valves have limited durability. We herein report sudden progression of structural valve deterioration (SVD) and a successful case of emergency transcatheter aortic valve (TAV) implantation for acute decompensated heart failure (ADHF) caused by SVD. A 79-year-old man who had undergone a Bentall operation 11 years prior was diagnosed with ADHF due to suddenly progressive SVD. Emergency TAV implantation in the surgical bioprosthetic valve was selected based on the surgical risk. Ours and previous case reports suggest that SVD can progress suddenly, even after months of stability, and that emergency TAV implantation is effective.

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.

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.

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.

[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.

Acute aortic dissection with highly compressed true lumen: unanticipated pitfall of point-of-care ultrasonography.

A 46-year-old man presented with sudden onset of chest pain. He was in cardiogenic shock at arrival. Based on the results of ECG and echocardiogram, he was diagnosed with ST-segment elevation myocardial infarction. Point-of-care ultrasonography (POCUS) did not reveal acute aortic dissection (AAD). During an emergency coronary angiography, aortic dissection was detected and computed tomographic angiography (CTA) revealed Stanford type A AAD with a highly compressed true lumen. Because of this form of aortic dissection, the enlarged false lumen could be potentially misidentified as a normal aorta in POCUS. Although POCUS is useful when AAD is suspected, we should not overestimate its findings and lower the threshold for CTA.

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.

A Patient with 22q11.2 Deletion Syndrome Presenting with Systemic Skin Rash and Dermatopathic Lymphadenitis of Unusual Histology.

BACKGROUND Chromosome 22q11.2 deletion syndrome (22q11.2 DS) currently includes DiGeorge syndrome, conotruncal anomaly face syndrome, and velocardiofacial syndrome. We present the case of a male infant with 22q11.2 DS exhibiting generalized skin rash and dermatopathic lymphadenitis. CASE REPORT The patient was born at 40 weeks of gestation with interruption of aortic arch, ventricular septal defect, and thymic defect. Fluorescence in situ hybridization method performed on buccal smears detected the deletion of 22q11.2. On day of life 33, diffuse erythema appeared on the entire body. A skin biopsy detected vacuolar interface dermatitis with superficial perivascular infiltration. Laboratory examinations revealed eosinophilia and hypocalcemia. Clinically, cutaneous inflammation was correlated with the abnormal immune response in 22q11.2 DS. On day of life 210, the patient died due to sepsis caused by Pseudomonas aeruginosa. An autopsy revealed lymph nodes swellings in the bilateral axillar and subclavicular areas and around the bilateral iliac arteries. Histology of the lymph nodes demonstrated sparse distribution of atrophic germinal centers surrounded by wide zones of proliferating spindle cells, as well as macrophages, Langerhans cells, and interdigitating dendritic cells. Fontana-Masson staining revealed abundant melanin particles in the macrophages. Accordingly, we diagnosed this case as dermatopathic lymphadenitis. Interestingly, CD123 and CD56 double-positive spindle cells also proliferated around the germinal center. CONCLUSIONS This case had an unusual histological feature of dermatopathic lymphadenitis. Considering the wide variety of unusual immune conditions in 22q11.2 DS, the lymph nodes in the systemic skin inflammation may exhibit an extraordinary histology of spindle cells proliferation.

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.

[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.