Successful management of HeartMate 3 in a patient with arrhythmogenic right ventricular cardiomyopathy.

The management of right heart failure during durable left ventricular assist device (LVAD) support remains an unsolved issue so far. We had a 44-year-old male patient who was diagnosed with arrhythmogenic right ventricular cardiomyopathy and received HeartMate 3 LVAD (Abbott, USA) implantation as a bridge-to-transplant indication. The pump speed was adjusted as low as 4500 rpm to avoid the left ventricular narrowing and interventricular septal leftward shift. Riociguat was administered to decrease the afterload of the right ventricle and increase the preload of the left ventricle, in addition to the combination of neurohormonal blockers. Frequent low-flow alarm events eventually disappeared after amlodipine administration, and he was successfully returned to work. We here present a unique management in a patient with right heart failure due to arrhythmogenic right ventricular cardiomyopathy during HeartMate 3 LVAD support.

A case of destination therapy for post-fulminant myocarditis with myelodysplastic syndrome.

We encountered a 64-year-old woman who experienced fulminant myocarditis and underwent treatment with veno-arterial extracorporeal membrane oxygenation and Impella CP support. Subsequently, she underwent a device upgrade to Impella 5.5 and received continuous hemodiafiltration for 3 months. During mechanical circulatory support, she developed refractory anemia and thrombocytopenia, leading to a diagnosis of myelodysplastic syndrome. Following the removal of the devices, she no longer required blood transfusions. She received HeartMate 3 left ventricular assist device implantation as a destination therapy indication despite the presence of myelodysplastic syndrome. She was successfully managed by aspirin-free antithrombotic therapy without any hemocompatibility-related adverse events for 4 months after index discharge on foot. We present a patient with a unique and rare presentation, wherein HeartMate 3 was implanted and successfully managed without aspirin to prevent bleeding complications associated with myelodysplastic syndrome.

[Thoracic Endovascular Aortic Repair for Mycotic Thoracic Aortic Aneurysm After BCG Intravesical Therapy].

Bacille Calmette-Guérin( BCG) intravesical therapy is an effective and safe treatment for bladder cancer; however, mycotic aneurysms have been reported as a rare complication. Case 1:A 64-year-old man with a history of BCG intravesical therapy underwent emergent thoracic endovascular aortic repair (TEVAR) for a ruptured thoracic aortic aneurysm (TAA). He was diagnosed with BCG infection by hemosputum specimen culture five months later;then, antituberculous therapy was initiated. However, his follow-up computed tomography scan revealed stent-graft infection and new aneurysm formation. Therefore, we performed a repeated TEVAR with abdominal 4-vessel debranching. There was no recurrence of infection for six years while continuing postoperative antituberculous therapy. Case 2:A 72-year-old man who had undergone BCG intravesical therapy underwent TEVAR for a rapidly enlarging mycotic TAA. He received anti-tuberculous therapy for one year with no recurrent infection for one year. TEVAR may be an effective alternative to the open surgical procedure;however, multidisciplinary treatment including anti-tuberculous therapy and careful long-term follow up are required.

Transcatheter Aortic Valve Implantation Improves Cardiac Sympathetic Nerve Activity on 123I-Metaiodobenzylguanidine Myocardial Scintigraphy in Severe Aortic Valve Stenosis.

BACKGROUND: There is a consensus that overactivation of the cardiac sympathetic nervous system (CSN) proportionately increases the severity of heart failure and is accompanied by worse prognosis. Because it is unknown whether patients with aortic valve stenosis (AS) have similar CSN activation, we investigated the effect of transcatheter aortic valve implantation (TAVI).Methods and Results:We enrolled 31 consecutive patients with AS treated by TAVI. 123I-metaiodobenzylguanidine (MIBG) myocardial scintigraphy was performed at baseline and at 2 weeks after TAVI. At baseline, the early heart-mediastinum ratio (H/M) was within normal limits (3.0±0.5), but the delayed H/M was low (2.6±0.6) and the washout rate (WR) was high (34±13%). WR negatively correlated with aortic valve area (r=-0.389, P<0.01) and cardiac output (r=-0.595, P<0.01) and positively correlated with norepinephrine (r=0.519, P<0.01) and log NT-proBNP level (r=0.613, P<0.01). After TAVI, there were significant decreases in the norepinephrine level (366±179 ng/mL vs. 276±125 ng/mL, P<0.01) and WR (34±13 vs. 26±11%, P<0.01). CONCLUSIONS: The WR of MIBG was a useful marker of CSN activity and severity of AS. Immediate improvement of CSN activity after TAVI implied that AS hemodynamics per se enhanced CSN.

Current topics in surgery for multiple ventricular septal defects.

In this review article, we describe several topics, including the sandwich technique, the transatrial re-endocardialization technique, the limited apical left ventriculotomy approach and device closure. The sandwich technique was introduced for the closure of muscular ventricular septal defects (VSD) by sandwiching the septum between two felt patches placed in the left and right ventricle. This technique requires neither the transection of muscular trabeculae nor ventriculotomy. Although the sandwich technique has resulted in the improvement of surgical outcomes, cases of postoperative cardiac dysfunction have been reported. Multiple smaller VSDs have been closed with transatrial re-endocardialization. Septal dysfunction may be avoided through this technique, in which the septal trabeculae are approximated in two layers of superficial, endocardial running sutures. Recently, a number of reports have recommended a limited apical left ventriculotomy approach. With this technique, a much shorter incision of around 1 cm at the apex of the left ventricle may be sufficient for achieving the complete closure of apical muscular VSDs. The transcatheter or perventricular device closure of muscular VSDs has increasingly been performed with good results. Although favorable early and mid-term results of device closure have been reported, this method is not always safer or less invasive than surgical closure. Long-term evaluations should be performed to determine whether the right and left ventricular functions are affected by treatment with relatively large devices in the heart.

[Isolated Pulmonary Valve Endocarditis in a Patient with Aortic Regurgitation and Patent Foramen Ovale;Report of a Case].

Isolated pulmonary valve endocarditis is an extremely rare clinical condition. Here, we report a case of pulmonary valve endocarditis caused by methicillin-resistant Staphylococcus aureus (MRSA). An 84-year-old man with a history of aortic regurgitation and patent foramen ovale was admitted to our hospital due to fever of unknown origin for 4 weeks' duration. MRSA was detected in his blood cultures. Transthoracic echocardiography demonstrated a mobile vegetation attached to the pulmonary valve, moderate to severe aortic regurgitation, and patent foramen ovale with left-to-right shunt. After 30-days' treatment with vancomycin, gentamicin and rifampicin, he defervesced and blood cultures became negative. At surgery, a large vegetation was still attached to the pulmonary valve, but the leaflets remained with minimum damage. Aortic valve replacement, direct closure of the patent foramen ovale, and simple resection of the vegetation were performed. The postoperative course was uneventful.

Current topics in surgery for isolated total anomalous pulmonary venous connection.

Surgical correction of total anomalous pulmonary venous connection (TAPVC) remains a challenge, with reported early mortality rates of up to 20%. In this review article, we describe several topics, including surgery for neonates, diagnoses with multidetector computed tomography (MDCT), and primary sutureless repair. Several studies have reported mortality rates of around 10%, and demonstrated unchanged hospital mortality in neonates, despite improvement of the overall mortality of cohorts including older patients. Previous reports identified a low body weight at the time of the operation, preoperative pulmonary venous obstruction (PVO), and a prolonged cardiopulmonary bypass time as risk factors for hospital mortality. With the development of new technologies, MDCT has become a good diagnostic modality for use in the pre- and post-operative evaluation. MDCT delineates the drainage site of the vertical vein and the atypical vessel into the systemic vein, and it can also evaluate the existence of obstruction in the vertical vein. Following favorable experiences with post-repair PVO, the indications for sutureless repair as a primary operation have been expanded for infants, including those at risk of developing PVO after the repair of TAPVC. Primary sutureless repair has proven especially useful for difficult patient groups, such as those with congenital PVO, infracardiac TAPVC with small pulmonary veins, or mixed-type TAPVC.

[Construction of a heart team using network attached storage].

There has been recent emphasis on the importance of an interdisciplinary heart team to allow active communication between physicians and surgeons regarding the management of cardiovascular diseases. Construction of a heart team requires a system that enables doctors to rapidly review patients' data when exchanging opinions. We tried to build up a heart team by sharing patients' data via network attached storage( NAS). We installed a server at the Information Technology Center of the University of Toyama with a high-security firewall and online storage software( Proself) for receipt and transfer of data. Accounts and passwords allowing connection to the file server were allocated to cardiovascular physicians at 18 core hospitals without cardiovascular surgery departments, and they were grouped in Proself for security. All communications are encrypted and file transfer is automatically notified to an internet protocol (IP) address via e-mail when data are uploaded or downloaded. Thus, the hospital sending the data and the type of data can be identified immediately, making it possible to refer to the corresponding image files. Sharing of data via NAS allows cardiovascular physicians and surgeons to communicate safely and effectively, leading to successful building of a heart team.

Intraoperative Heparin Resistance after Administration of Andexanet Alfa to Manage an Internal Iliac Artery Aneurysm Rupture: A Case Report.

Antithrombotic agents are increasingly prescribed to older adults; however, they are associated with bleeding-related complications. We describe a case of intraoperative heparin resistance after administration of andexanet alfa (AA). An 81-year-old man was diagnosed with a ruptured internal iliac artery aneurysm. The patient required emergency endovascular aneurysm repair and was treated with AA because he was receiving apixaban. Despite high-dose intraoperative heparin administration, his activated coagulation time was not prolonged. Our findings suggest that AA should be administered with caution in patients experiencing potentially fatal bleeding (requiring surgical intervention) who are also receiving direct oral anticoagulants.

Aortic valve replacement in a patient with self-reported systemic multiple metal allergy.

We report our experience with aortic valve replacement in a patient with severe aortic stenosis who had self-reported allergies to multiple metals. The patient was a 74-year-old man. He developed palmoplantar pustulosis after using a dental prosthesis, and a patch test revealed reactions to several metals; therefore, he was diagnosed with systemic metal allergy. His condition progressed to severe aortic stenosis, and bioprosthetic aortic valve replacement was planned. The Avalus valve (Medtronic, Minneapolis, MN, USA) was selected for aortic valve replacement, since the patient reported having allergies to several metals. While most devices used in cardiac surgery contain some amount of metal, the Avalus bioprosthetic valve does not contain metal in the stent and has been extremely useful for aortic valve replacement in patients with suspected metal allergies.

Application of the Konno procedure for infective endocarditis in native bicuspid aortic valve with annular abscess extending into the interventricular septum.

Annular abscess is a serious complication of infective endocarditis, which often requires complex surgery and has a very high post-operative mortality rate. The Konno procedure involves valve annuloplasty for a narrow aortic annulus or left ventricular outflow tract stenosis in children; it is also performed for various cardiac conditions in adults. Here, we report a case of the Konno procedure performed in a patient with aortic valve infective endocarditis, with an annular abscess extending into the interventricular septum (IVS). A 58-year-old man who presented to our hospital with fever was diagnosed with aortic valve infective endocarditis caused by Streptococcus saccharolyticus. On echocardiography, an annular abscess in the direction of the IVS was detected, and surgery was planned. The Konno procedure was performed to secure an adequate surgical field and to debride and reconstruct the cavity created by the interventricular septal abscess. The patient was discharged uneventfully 29 days after surgery.

Experimental mitral valve plasty under the beating heart guided by real-time 3-dimensional echocardiography.

BACKGROUND: This study examines whether real-time 3-dimensional echocardiography can provide an image resolution to serve as a substitute for optical visualization in performing mitral valve plasty. METHODS: Three pigs were used in this study. A 3-dimensional echocardiographic system was evaluated on an epicardial surface. Beating heart mitral valve plasty was performed with a surgical stapler inserted from the apex of the left ventricle using only 3-dimensional echocardiographic visualization. RESULTS: The high-quality images of the mitral valve were obtained with the probe at the epicardial position. However, by inserting the surgical instrument into the left ventricle, an acoustic shadow developed on the images. The images became indistinct because of the acoustic shadow, and operation became difficult. For the mitral valve plasty, an edge-to-edge mitral valve repair was carried out using a stapler (10 mm) under the beating heart. The stapler was confirmed to seize both leaflets evenly in only 1 of the 3 pigs. CONCLUSIONS: Real-time 3-dimensional echocardiography provided clear 3-dimensional images of the mitral valve; however, when a surgical instrument was inserted into the left ventricle, an acoustic shadow appeared on the image and made detailed confirmation difficult. Lessening or eliminating the acoustic shadow would be a key point to improve this procedure.

On-Pump Beating Heart Extraanatomical Ascending-Descending Aortic Bypass Using a Beating Heart Positioner in an Adult with Aortic Coarctation.

The prognosis of uncorrected aortic coarctation is poor due to development of heart failure. We performed an on-pump beating heart extraanatomical ascending-descending aortic bypass using a beating heart positioner in an adult with coarctation complicated by severe left ventricular hypertrophy. A 51-year-old woman was referred with severe hypertension. Computed tomography demonstrated severe distal aortic arch narrowing. Coarctation of the aorta was diagnosed. A posterior pericardial beating heart extraanatomical bypass via median sternotomy was performed from the ascending to descending aorta using a heart positioner. Her postoperative course was uneventful and blood pressure was normal on a low-dose beta-blocker.

Two-Stage Hybrid Repair in a Patient with Acute Type A Aortic Dissection Associated with Right Aortic Arch with Aberrant Left Subclavian Artery Originating from a Kommerell Diverticulum.

Right aortic arch with aberrant left subclavian artery and Kommerell diverticulum are rare anomalies. A 42-year-old man was referred with sudden-onset chest pain. Enhanced computed tomography (CT) showed a right aortic arch with early thrombosed acute type A aortic dissection and an aberrant left subclavian artery arising from a Kommerell diverticulum. Medical therapy was instituted; however, follow-up CT revealed an ulcer-like projection. The patient was managed with a two-stage hybrid procedure comprising total arch replacement and endovascular repair and experienced no postoperative complications. Two-stage hybrid repair is a safe and effective surgical option for rare complex aortic anomalies.

Primary cardiac malignant lymphoma: survival for 13 years after surgical resection and adjuvant chemotherapy.

We report a patient who has survived for 13 years after surgical resection of a cardiac malignant lymphoma. A 73-year-old woman underwent partial resection of a tumor occluding the tricuspid valve, which arose from the right atrium. The pathologic diagnosis was diffuse large B cell lymphoma. She was treated with adjuvant chemotherapy for 6 months postoperatively. Since then, she has shown no evidence of recurrence, without any further treatment. To our knowledge, this is the longest surviving case of cardiac malignant lymphoma. The therapeutic strategy for this malignant cardiac tumor is discussed briefly.

Treatment of mediastinitis due to methicillin-resistant Staphylococcus aureus in a renal dysfunction patient undergoing adjustments to the linezolid dose.

This study is the first case report of the treatment of methicillin-resistant Staphylococcus aureus (MRSA) mediastinitis using therapeutic drug monitoring of the serum and wound exudate concentrations of linezolid in a renal dysfunction patient. In the present study, the serum trough concentration of linezolid was maintained between 2 and 7 µg/mL. Therapeutic drug monitoring dosage adjustments may be especially useful in patients with renal dysfunction and severe MRSA infection.

Surgical treatment of complete anterolateral papillary muscle rupture following acute myocardial infarction.

Papillary muscle rupture is a lethal complication of after acute myocardial infarction. We report the successful surgical management of complete anterolateral papillary muscle rupture associated with cardiogenic shock in an 80-year-old woman. The patient was admitted with the sudden onset of chest pain and dyspnea. After hemodynamics deteriorated rapidly, she was intubated and intra-aortic balloon pump was started immediately. Transthoracic echocardiography revealed severe mitral regurgitation due to complete anterolateral papillary muscle rupture associated with lateral myocardial infarction. Coronary angiography demonstrated complete occlusion of the first obtuse marginal artery. She underwent emergency mitral valve replacement and coronary artery bypass grafting. There were no complications of surgery and she was discharged on postoperative day 35. Rapid preoperative assessment and aggressive surgical management are important to improve the prognosis in this setting.

Selective isolation of nanog-positive human amniotic mesenchymal cells and differentiation into cardiomyocytes.

Adult cardiomyocytes have little ability to regenerate, thus cardiac regeneration therapy represents a potential method for treating severe heart failure. Human amniotic mesenchymal cells (hAMCs) have the potential to be a useful cell source for cardiac regeneration therapy. We attempted to isolate stem cells from hAMCs and differentiate them into cardiomyocytes. Nanog promoter-Cre plasmid and cytomegalovirus (CMV) promoter-loxP-STOP-loxP-Red-puro(r) plasmid were co-transfected into immortalized hAMCs (iHAMs). Nanog-positive iHAMs were treated with 5-azacytidine (5-aza), trichostatin A (TA), activin A (AA), and bone morphogenetic protein-4 (BMP-4), or co-cultured with murine fetal cardiomyocytes for cardiomyocytes differentiation. Isolated Nanog-positive iHAMs were analyzed by quantitative RT-PCR and immunofluorescent staining before and after differentiation. Expression of Nanog, Oct3/4, Sox2, and Klf4 was significantly higher in Nanog-positive than in Nanog-negative iHAMs. Nanog-positive iHAMs were stained for Nanog and Oct3/4 in the nucleus. Nanog-positive iHAMs treated with 5-aza expressed Nkx2.5, GATA-4, human atrial natriuretic peptide (hANP), cardiac troponin T (cTnT), myocin light chain (Mlc)-2a, Mlc-2v, ß-myosin heavy chain (ß-MHC), hyperpolarization-activated cyclic nucleotide gated channels (HCN)-4, and inwardly rectifying potassium channels (Kir)-2.1. Although Nanog-positive iHAMs treated with TA, AA, or BMP-4 expressed several cardiac markers, no contraction was observed. Co-cultured Nanog-positive iHAMs with murine fetal cardiomyocytes spontaneously contracted in a synchronized manner and expressed the cardiac markers. In conclusion, Nanog-positive hAMCs with characteristics of stem cells were isolated and differentiated into cardiomyocyte-like cells, suggesting that these isolated hAMCs could be a useful cell source for cardiac regeneration therapy.

Effect of exogenous Oct4 overexpression on cardiomyocyte differentiation of human amniotic mesenchymal cells.

Regenerative therapy is a new strategy for the end-stage heart failure; however, the ideal cell source has not yet been established for this therapy. We expected that the amnion might be an ideal cell source for cardiac regenerative therapy and that the differentiation potency of the human amnion mesenchymal cells (hAMCs) could be improved by overexpression of Oct4, a key factor that maintains the undifferentiated state. A plasmid vector was made by insertion of the Oct4 open reading frame (ORF) under control of a cytomegalovirus (CMV) promoter (pCMV-hOct4) and transfected into hAMCs by electroporation. The optimum induction time was investigated by comparing the quantity of stem cell-specific mRNAs, cardiac-specific mRNAs, and cardiac-specific proteins with time. hAMCs already expressed cardiac-specific proteins such as Nkx2.5 and Connexin43. After pCMV-hOct4 transfection, endogenous Oct4 mRNA and other stem cell markers showed a transient increase. With 5-azacytidine treatment, quantities of the cardiac-specific mRNAs, such as GATA4 and myosin light-chain-2v (Mlc-2v), were increased significantly. After Oct4 overexpression, the highest expression of cardiac-specific mRNAs and stem cell makers was seen at almost the same time. Furthermore, more mature myocardial contraction proteins were observed when hAMCs were induced at specific optimal times after gene transfection. In conclusion, hAMCs were activated to an undifferentiated state by overexpression of Oct4, and their cardiac differentiation potency was improved. Thus, the single-time transfection of the Oct4 expression vector may be a useful strategy for effective cell therapy. The use of cryopreserved hAMCs in cell therapy still requires more investigation.

Management of pulmonary venous obstruction.

Although improved surgical techniques have led to significantly better outcomes of surgery for total anomalous pulmonary venous connection, the risk of progressive pulmonary venous obstruction continues to be a clinical problem. Both obstructed total anomalous pulmonary venous connection and post-repair pulmonary venous obstruction are associated with a significant risk of recurrent obstruction or death, requiring reoperation for stenosis. In general, side to side anastomosis of the pulmonary venous confluence to the functional left atrium has been performed for supracardiac and infracardiac total anomalous pulmonary venous connection. Repair of total anomalous pulmonary venous connection to the coronary sinus invariably involved unroofing the coronary sinus, followed by pericardial patch closure of the atrial septal defect. Recently, sutureless technique has been adopted as the primary operation for the subgroups of patients that are thought to be at high risk for post-repair pulmonary venous obstruction, such as those with total anomalous pulmonary venous connection associated with right isomerism, infracardiac total anomalous pulmonary venous connection with small individual pulmonary veins, or mixed-type total anomalous pulmonary venous connection. Because the sutureless technique does not require direct anastomosis to the confluence, aggressive resection of the obstructed pulmonary venous tissue can be achieved, and surgically induced distortion of the suture line can be avoided, which may help to prevent subsequent pulmonary venous obstruction. Conventional management strategies for recurrent pulmonary venous obstruction have typically been associated with poor outcomes. Recent reports have supported the use of the sutureless technique to treat post-repair pulmonary venous obstruction.