Case report: Characteristics and nature of primary cardiac synovial sarcoma.

Primary malignant cardiac tumors rarely occur, and cardiac synovial sarcoma (SS) is especially rare among such tumors. Herein, we present the case of a 35-year-old female with primary cardiac SS treated with surgery, chemotherapy, and radiotherapy. She presented with chest symptoms and underwent imaging examinations. A cardiac tumor was suspected, and an open biopsy was performed. The pathological findings suggested cardiac SS. Next, we performed a resection, and the tumors persisted at a macroscopic level. Immunohistochemistry was negative for SS18-SSX and positive for the SSX C-terminus and cytokeratin CAM5.2, a reduction of SMARCB1/INI1 was observed, and fluorescence in situ hybridization showed positive SS18 split staining. Owing to the FNCLCC grade 3 tumor and R2 margins, adjuvant chemotherapy with ifosfamide, doxorubicin, and radiotherapy was initiated, and the patient was diagnosed with cardiac SS. The differences in patients with cardiac SS compared with general SS include male predominance, larger tumor size, and poorer prognosis. Pathological findings of immunohistochemistry and fluorescence in situ hybridization were found to be more reliable than imaging findings for a correct diagnosis. Additionally, because incomplete resection is frequently performed, adjuvant therapy, including chemotherapy and radiation therapy, may be performed. The findings indicate that multiple therapies, including surgery, chemotherapy, and radiotherapy, are essential treatment strategies for improving the prognosis of patients with cardiac SS.

Systolic anterior motion due to morphology changes in constrictive pericarditis.

Systolic anterior motion (SAM) can be caused by multifactorial mechanisms, including structural, morphological and functional factors. We report an unusual case of a 76-year-old woman presenting with SAM associated with constrictive pericarditis. Echocardiography showed no septal hypertrophy but SAM and left ventricular outflow tract obstruction and moderate mitral regurgitation. The restoration of diastolic function after complete pericardiectomy successfully eliminated it.

Total arch replacement with both frozen elephant trunk and transthoracic aorto-axillary extra-anatomical bypass through partial sternotomy for acute aortic dissection in a tracheostomy patient.

Median sternotomy near an existing tracheostoma risks deep sternal wound infection after cardiac surgery. We present herein a case of acute type A aortic dissection in a patient with a permanent tracheostoma after laryngectomy. Total arch replacement with both frozen elephant trunk and extra-anatomical bypass for supra-aortic trunks was performed through T-shaped partial sternotomy, resulting in recovery without deep sternal wound infection.

Aortic prosthetic size predictor in aortic valve replacement.

BACKGROUND: Patient-prosthesis mismatch (PPM) is a major concern in aortic valve replacement (AVR) and leads to perioperative morbidity and rehospitalization. Predicting aortic annulus diameter pre-procedurally is crucial to managing patients with high-risk of PPM. OBJECTIVES: To compare preoperative measurements of aortic annulus from echocardiography and CT scan with surgical sizing and develop an imaging-based algorithm to predict PPM. METHODS: From January 2017 to December 2020, patients underwent AVR at a teaching hospital were examined. The relationship between imaging measurements with operative values was assesed using scatter plots and Pearson's correlation coefficient. Univariable linear regression was then used to build the predictive model. RESULTS: A total of 144 patients underwent AVR during the study period. Suture types and surgical approaches were not significantly associated with prosthesis size. CT scan-based measurements showed strong correlation with prosthesis size: mean diameter (R = 0.79), perimeter-derived diameter (R = 0.76), and area-derived diameter (R = 0.75). Mechanical valve and tissue valve shared similar correlation coefficients. Prosthesis size predictive models based on CT scan were 12.89 + 0.335 × d for mean diameter, 13.275 + 0.315 × d for perimeter-derived diameter and 13.626 + 0.309 × d for area-derived diameter. CONCLUSIONS: Preoperative CT scan measurements are a reliable predictor of aortic prosthesis size. Transthoracic echocardiography is a possible alternative, though it is highly performer-dependent and unable to represent the aortic annulus fully. Together, these two imaging modalities can be used to quantitatively anticipate PPM preoperatively.

First Clinical Trial of a Newly Developed, Low Menaquinone-7, Fermented Soybean Natto in Warfarin-Dependent Patients.

Bacillus subtilis fermented soybeans (natto) contain high vitamin K2 levels, mostly as menaquinone-7 (MK-7), and must be avoided by warfarin-dependent patients. This is the first report which demonstrates the characteristics and clinical relevance of a low MK-7 natto for such patients. We generated a novel, mutant B. subtilis strain TTCC2051 with short-term fermentation and reduced MK-7 production, yielding 19-24% of the normal MK-7 content. After functional assessments and a preclinical trial, 10 warfarin-dependent patients underwent a clinical trial with a 7-day ingestion test of the low MK-7 natto. Functional assessments were satisfactory, and the preclinical trial showed no increases in plasma MK-7 levels after 7 days of ingestion. In the clinical trial, 20 g/day of the low MK-7 natto significantly increased plasma MK-7 levels while 10 g/day did not. However, neither dose of low MK-7 natto changed international normalized ratio of prothrombin time (PT-INR) values in either group. The low MK-7 natto neither changed PT-INR values nor precipitated adverse events if ingested with a once-daily maximum of 20 g (46 µg of MK-7). Thus, this novel food product has potential for consumption by warfarin-dependent patients.

Adult Obstructive Cor Triatriatum with Severe Mitral Regurgitation: A Case Report.

Adult cor triatriatum sinister associated with severe mitral regurgitation is extremely rare. As these obstructive cor triatriatum feature hemodynamics that mimic mitral stenosis, a pressure load is theoretically generated only on the left atrial proximal chamber, and therefore the left ventricle is less likely to suffer volume loading. Here, we report a surgical case with such rare hemodynamics. A 22-year-old man with obstructive cor triatriatum and severe mitral regurgitation received an anomalous membrane excision and mitral annuloplasty. An abnormal membrane with an orifice 7 mm in size was completely resected while a grossly dilated mitral annulus was repaired via annuloplasty ring. Mitral regurgitation was controlled well, and the postoperative course was uneventful. Even with obstructive cor triatriatum, severe mitral annular dilatation and subsequent left ventricular dilatation may occur, causing the progressive heart failure encountered in this case.

Giant Right Ventricular Outflow Tract Myxoma Mimics Pulmonary Embolism: A Case Report.

Right ventricular myxoma is very rare, especially its originating from the right ventricular outflow tract (RVOT) and extending to the main pulmonary artery. Here, we report a case of a giant RVOT myxoma, indistinguishable from pulmonary embolism (PE). Although the myxoma is a candidate for urgent surgery, this case satisfied diagnostic criteria for PE and had no indication for intervention, according to the guideline. The strategy for this mass can be completely different, depending on the diagnosis. Surgical extraction was selected because of atypical clinical course, findings, and nagging debut for neoplasm. Then it made hemodynamic status stable by releasing RVOT obstruction and allowed to reveal the diagnosis as myxoma histopathologically.

Hybrid surgical and endovascular repair for anastomotic pseudoaneurysms after total arch replacement.

An 83-year-old woman who had undergone total arch replacement for a thoracic aortic aneurysm seven years prior experienced concurrent rupture of second and third branch anastomotic pseudoaneurysms. A stent graft was initially deployed across the pseudoaneurysm from the third branch to the left subclavian artery. Following the left axillary and left carotid arterial bypass, the origin of the second branch was embolized with metallic coils. As a result, both anastomotic pseudoaneurysms were undetectable by both completion angiogram and postoperative CT. By combining surgical and endovascular procedures, ruptured anastomotic pseudoaneurysms after total arch replacement can be repaired without a risky resternotomy.

The effect of roller head pump on platelet deterioration during the simulated extracorporeal circulation.

Roller pumping results in hemolysis and adverse effects on coagulation, but there are few reports on the influence of roller heads on platelets. Here, we evaluate the interaction between roller pumping and platelet function using a simulated extracorporeal circuit incorporating a vinyl chloride tube and roller head pump with 30 min recirculation. Platelet aggregation, platelet count, microparticle, P-selectin, Phosphatidylserine (PS) exposure and Ricinus Communis Agglutinin 1 (RCA-1) were measured before, 5, 10, 20, and 30 min after the recirculation using 100 ml of fresh human blood that had obtained from healthy volunteers (n = 9). Platelet aggregation and platelet count gradually decreased but microparticles significantly increased after the recirculation (P < 0.05). P-selectin, PS exposure and RCA-1 were measured using flow cytometry. There were no significant differences in the P-selectin and PS exposure expression during recirculation. RCA-1, a platelet apoptosis markers, significantly increased 30 min after recirculation (P < 0.05). We thus conclude that roller pumping induced platelet apoptosis and caused decreases in platelet count and aggregation after the recirculation.

TEG improves anticoagulation management during cardiopulmonary bypass complicated by antiphospholipid syndrome.

BACKGROUND AND AIM: Cardiopulmonary bypass (CPB) in antiphospholipid syndrome (APS) patients carries a high risk of thrombosis and hemorrhage. However, optimal anticoagulation surveillance methods have not been established and heparin optimization has not yet been totally validated as reflective of anticoagulation status. METHODS AND RESULT: Here, a 45-year-old female with APS underwent mitral valvuloplasty due to infective endocarditis. We used a perioperative, in vitro, heparin-activated clotting time (ACT) titration line coupled with synergistic, intraoperative thromboelastography (TEG) to monitor coagulation activity. After the ACT target was reached, TEG monitored the suppression of both intrinsic and extrinsic coagulation activity throughout the surgery. CONCLUSION: TEG thus provided valuable temporal information on both intrinsic and extrinsic coagulation suppression validating heparin-ACT titration targets.

Surgical extraction of a giant intracardiac lead vegetation and epicardial pacemaker reimplantation in a pacemaker-dependent hemodialysis patient.

A 57-year old male with a dual-chamber pacemaker and 40-year history of hemodialysis and autoinflammatory disease developed a large, 35 × 35 mm intracardiac vegetation on the right ventricular pacing lead. As this mass was large enough to occlude the tricuspid valve orifice, transvenous lead extraction was deemed unsuitable. Instead, an urgent surgical extraction of the whole pacemaker system, including leads and vegetation, was conducted under cardiopulmonary bypass. In light of a high risk of recurrent blood infection, a new dual-chamber pacing system was then immediately re-established using epicardial pacing leads on the right atrium and ventricle instead of transvenous electrodes. This case of a rare, giant intracardiac lead vegetation lacked most known causal factors, except for renal failure, but a possibly immunosuppressed cardiac microenvironment due to long-term steroid therapy may have been an important influencing factor.

Landiolol Hydrochloride Rapidly Controls Junctional Ectopic Tachycardia After Pediatric Heart Surgery.

OBJECTIVES: Junctional ectopic tachycardia is a supraventricular tachyarrhythmia with atrioventricular dissociation that causes life-threatening postsurgical conditions in pediatric heart patients. This study evaluates the efficacy of landiolol hydrochloride for managing junctional ectopic tachycardia. DESIGN: A single-center retrospective study. SETTING: PICU at the university hospital. PATIENTS: Of 561 pediatric patients who underwent open-heart surgery between 2006 and 2017, 10 patients developed sustained junctional ectopic tachycardia and were selected for landiolol treatment. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Landiolol decreased mean heart rate significantly from 206.1 ± 14.5 to 158.0 ± 8.6 beats/min within 2 hours after administration (p < 0.01). Mean time to achieve 20% heart rate reduction was 2.1 ± 0.5 hours. Systolic blood pressure between pre and post landiolol administration did not change significantly (72.6 ± 5.9 to 79.7 ± 6.2 mm Hg). Once junctional heart rate was sufficiently suppressed, atrioventricular sequential pacing was introduced to stabilize hemodynamics. Nine of 10 cases (90%) had atrioventricular sequential pacing to maintain appropriate heart rate and restore atrioventricular synchronicity under suppressed junctional heart rate. Subsequently, eight of 10 cases (80%) were converted to regular sinus rhythm within 24 hours after starting landiolol administration. The average time to achieve sinus rhythm conversion was 7.9 ± 3.4 hours. CONCLUSIONS: Landiolol rapidly suppresses junctional heart rate in junctional ectopic tachycardia after pediatric heart surgery without significant blood pressure compromises. Subsequent atrioventricular sequential pacing was effective at restoring atrioventricular synchronicity and stabilizing hemodynamics. Combining junctional rate control with landiolol and atrioventricular sequential pacing is therefore suggested as a promising option for prompt management of postoperative junctional ectopic tachycardia.

Predictors of Pericardial Effusion in Patients Undergoing Pulmonary Artery Banding.

BACKGROUND: Although pulmonary artery banding (PAB) is a common palliative procedure for pediatric heart malformation, there are concerns of pressure overload and concomitant immune reactions in the right ventricle causing postsurgical complications such as pericardial effusion. At this time, no clear guidelines as to potential risk factors or procedural contraindications have been widely disseminated. Therefore, a study was undertaken to examine wide-ranging factors to find potential biomarkers for postsurgical pericardial effusion formation risk. METHODS: A retrospective study was conducted on all cardiac surgeries performed over an eight-year period, and the main inclusion criterion was pericardial effusion development after PAB that required surgical drainage. Nine cases were then analyzed against a control group of 45 cases with respect to body measurements, concomitant surgeries, genetic screens, laboratory tests results, and cardiac function parameters. RESULTS: Trisomy 21 was strongly associated with the development of severe pericardial effusion after PAB, and postoperative serum albumin levels in patients with trisomy 21 were associated with pericardial effusion development. Other parameters showed no significant correlation with pericardial effusion development. CONCLUSIONS: Our data indicate a strong association between trisomy 21 and pericardial effusion requiring drainage after PAB, which is in line with translational research findings. Pressure overload from PAB may play a role in the formation of severe pericardial effusion that is exacerbated by cardiac structural defects commonly associated with trisomy 21. Surgical teams should therefore use caution and plan to implement drainage in PAB cases, and postoperative serum albumin may serve as a useful biomarker for pericardial effusion formation.

Localized Aortic Root Dissection with a Superior Mesenteric Artery Aneurysm.

In this study, the case of a 46-year-old female patient with localized aortic root dissection and a superior mesenteric artery (SMA) aneurysm is described. Computed tomographic angiography could not clearly delineate an intimal flap in the aortic root, but it detected SMA aneurysm, which implied the presence of a vulnerability of the aortic wall. Finally, transesophageal echocardiography (TEE) evidently showed the intimal flap localized in the aortic root. The present case suggests that TEE is of paramount importance for detecting localized aortic root dissection. In addition, a coexisting vascular lesion may be a clue to diagnose another vascular lesion.

Perioperative anticoagulation management during aortic valve replacement complicated by antiphospholipid syndrome.

Patients with antiphospholipid syndrome (APS) are at high-risk for thrombotic and bleeding complications during cardiopulmonary bypass. We report an APS patient who successfully underwent aortic valve replacement while heparin concentrations were monitored using a patient-specific titration curve using viscoelastic coagulation testing.

[Simple Evaluation of Left Hand Function after Thoracic Endovascular Aortic Repair with Obstruction of Left Subclavian Artery].

We evaluated left hand function with or without obstruction of left subclavian artery for 29 cases of thoracic endovascular aortic repair (TEVAR). 18 cases of distal true aortic aneurysm were undergone surgery with simple direct closure of left subclavian artery (LSCA). The other 11 cases of true arch aneurysm were revascularized by axilla-carotid-axilla bypass grafting with TEVAR. Later 26 cases were evaluated by blood pressure, maximal grasping power, persisting duration of 60% maximal grasping power, circumferential length of upper and forearms, and simple test for evaluating hand function (STEF). Left hand blood pressure was significantly reduced than right side in obstruction group. But left hand function was not reduced in any other test. We concluded that no relationship between the obstruction of LSCA and the left hand function, and the influence of late survival and mobidity.

Coil Embolization-Assisted Thoracic Endovascular Aortic Repair for Multiple Saccular Descending Aortic Aneurysms with Insufficient Landing Zone.

We report a coil embolization-assisted thoracic endovascular aortic repair technique successfully applied to multiple saccular descending aortic aneurysms in a 74-year-old man. Because of the most distal aneurysm being located at the celiac trunk level and the distance between the superior mesenteric artery and the aneurysm being only 10 mm, a coil embolization of the distal saccular aneurysm was performed before stent delivery to secure a sufficient landing zone. Postoperative computed tomography showed an appropriate positioning of the endovascular devices without endoleak. This coil embolization-assisted technique may extend the indication of the endovascular aortic repair when a saccular aneurysm has an insufficient landing zone.

A Case of Intestinal Necrosis after Bilateral Internal Iliac Artery-Preserving Endovascular Repair for Abdominal Aortic Aneurysm.

A 79-year-old man underwent endovascular repair for abdominal aortic aneurysm (AAA), and both internal iliac arteries (IIAs) were preserved. Postoperatively, loss of appetite developed. On the fifth day, computerized tomography (CT) showed inferior mesenteric artery thrombus formation, necrosis of the descending colon and rectum, and generalized peritonitis. The endovascular devices had not migrated. A colonic resection was performed. Histological analysis confirmed intestinal necrosis associated with mesenteric thrombus. The colon can become necrotic even if both IIAs are patent. Ischemic changes in the colon should be detected if it occurs and subsequent laparotomy should be done if it is necessary.

Thick lung wedge resection for acute life-threatening massive hemoptysis due to aortobronchial fistula.

Massive hemoptysis from an aortobronchial fistula due to thoracic aortic dissection is an extremely rare symptom, but is a potentially life-threatening condition. We report a case of acute massive hemoptysis due to aortobronchial fistula that was successfully controlled by a simple and rapid thick wedge resection of the lung with hematoma by using the black cartilage stapler. A 65-year-old man was admitted to our hospital with acute massive hemoptysis. After tracheal intubation, chest computed tomography revealed hematoma in the left lung and ruptured aortic dissection from the distal arch to the descending aorta. He was diagnosed with aortobronchial fistula and underwent an emergency surgery on the same day. We performed posterolateral thoracotomy. A dissecting aortic aneurysm (diameter, ~80 mm) with adhesion of the left upper lobe and the superior segment of the lower lobe was found. The lung parenchyma expanded with the hematoma. We stapled the upper and lower lobes by using the black cartridge stapler along the aortopulmonary window. Massive hemoptysis disappeared, and the complete aortic dissection appeared. Aortic dissection with adherent lung was excised, and graft replacement of the distal arch and descending thoracic aorta was performed. Proximal lung wedge resection using black cartridge stapler is a simple and quick method to control massive hemoptysis from aortic dissection; hence, this procedure is an effective option to control massive hemoptysis due to aortobronchial fistula. This technique could rapidly stop massive hemoptysis and prevent dissection of the adherent lung tissue and intra-thoracic bleeding.

Intravenous Lobular Capillary Hemangioma in the Subclavian Vein.

We report the case of a 73-year-old man with severe edema of the right upper extremity. Computed tomography, magnetic resonance imaging, and venography revealed an intravenous tumor in the middle portion of the right subclavian vein. The tumor was completely resected, and an accompanying thrombus was also removed using the transmanubrial osteomuscular sparing approach. The patient recovered well without any complications or recurrence. The histopathologic diagnosis was intravenous lobular capillary hemangioma, which is also called pyogenic granuloma. To our knowledge, this is the first reported surgical case involving this type of benign tumor originating in a thoracic vein.