Initial two-day blood pressure management after endovascular aneurysm repair improves midterm outcomes by reducing the incidence of early type II endoleak.

OBJECTIVE: The aim of this study was to evaluate midterm outcomes of our novel strategy of postoperative initial 2-day blood pressure management (BPM) after endovascular aneurysm repair (EVAR) for the prevention of subsequent type II endoleak (T2EL) in a single-center series. METHODS: Between 2008 and 2014, 137 patients who underwent EVAR for abdominal aortic aneurysm (AAA) were reviewed. Starting from 2013, the mean blood pressure was maintained between 75 and 90 mmHg for the initial 24 hours after EVAR followed by systolic pressure controlled below 120 mmHg during the next 24 hours in the treatment group (n = 76). The incidence of T2EL detected at 7 days, reintervention, and AAA sac diameter up to 5 years after EVAR were compared with those of the control group comprising of 60 consecutive patients who underwent standard EVAR without BPM prior to 2013. RESULTS: Between the treatment group and the control group, significant differences were achieved in the incidence of T2EL at 7 days (19.7% vs 40.0%; P = .009), a mean decrease of AAA sac diameter at 1-year (-5.1 ± 4.9 vs -2.2 ± 6.7 mm; P = .013) and 2-year (-5.4 ± 7.7 vs -1.7 ± 10.8 mm; P = .045). In addition, there was a significant decrease in the incidence of T2EL detected at 7 days with the use of the Gore Excluder with 22.7% in the treatment group vs 80.0% in the control group (P < .001), which resulted in a significant decrease in the aneurysm sac diameter up to 4 years after EVAR. Survival rate without AAA sac enlargement at 5 years after EVAR (83.0% vs 70.0%; P = .021) in the treatment group was significantly higher than that of the control group, whereas no significant differences were observed in the freedom rates of reintervention, T2EL-related reintervention, and all-cause mortality between the groups. CONCLUSIONS: Postoperative initial 2-day BPM had a preventive effect on AAA sac enlargement until midterm periods, by reducing the incidence of T2EL at 7 days after EVAR. The usage of Gore Excluder under BPM was especially associated with sustained positive effects until the midterm follow-up.

Injection of n-Butyl-2-Cyanoacrylate into the Abdominal Aortic Aneurysm Sac during Endovascular Aortic Repair to Prevent Type II Endoleaks Caused by Lumbar Arteries.

PURPOSE: To evaluate midterm results of whether the strategy to occlude target lumbar arteries using n-butyl-2-cyanoacrylate (nBCA) injection during endovascular aneurysm repair (EVAR) reduced the incidence of Type II endoleak (T2EL) after EVAR. MATERIALS AND METHODS: Between 2013 and 2020, 187 patients underwent EVAR; 106 in the treatment group received nBCA injection during EVAR, whereas 81 in the historical control group did not. The incidence of T2EL at 7 days, need for reintervention, and post-EVAR aneurysmal shrinkage were compared between the groups. RESULTS: Between the treatment group and the control group, significant differences were achieved in the incidence of T2EL (2.8% vs 28.4%; P < .0001) and decreased aneurysmal diameter was observed at 1 year after EVAR (-5.2 vs -3.8 mm; P = .034). In multivariate analysis, nBCA injection (odds ratio [OR], 0.04; P = .001) and younger age (OR, 0.92; P = .036) were significantly associated with a reduced incidence of T2EL. As a possible adverse event associated with nBCA injection, 2 cases of transient lower-limb motor dysfunction (1.9%) were observed. Propensity score analysis revealed that the treatment group had a significantly lower incidence of T2EL than that in the control group (P = .0002) even though there was no difference in the incidence of inferior mesenteric artery coil embolization between the groups. The survival rate without aneurysm sac enlargement (100.0% vs 69.8%; P = .014) and the reintervention-free rate (100.0% vs 63.1%; P = .034) in the treatment group were significantly higher than those in the control group. CONCLUSIONS: Concomitant nBCA injection can provide durable EVAR without T2EL, as supported by the avoidance of reintervention associated with aneurysm sac enlargement.

Thoracic Endovascular Aortic Repair on Zone 2 Landing for Uncomplicated Type B Aortic Dissection with Measurement of Intra-False Lumen Pressure.

BACKGROUND: This study aimed to evaluate the midterm results of zone 2 thoracic endovascular aortic repair (TEVAR) for uncomplicated type B aortic dissection (TBAD) by measuring the intra-false lumen pressure (IFLP) during TEVAR. METHODS: Fifteen patients (9 men; mean age, 57 years) who underwent zone 2 TEVAR for uncomplicated TBAD were reviewed. Delta systolic pressure (defined as the difference between systemic pressure and IFLP) was measured before and after primary entry closure, and aortic remodeling and thrombo-occlusion of the false lumen (FL) were evaluated 12 months after TEVAR at 5 different levels of the aorta. RESULTS: Median duration from onset to TEVAR was 34 days. The left subclavian artery was preserved in 13 patients (87%) by using stent graft fenestration. Although 1 patient (6%) had a transient cerebral infarction, there were no severe TEVAR-related complications. Entry closure significantly reduced delta systolic pressure (mm Hg) compared to preoperative pressure at all levels (distal arch: -22.2 ± 10.8 vs. -5.2 ± 9.6; Th8: -20.1 ± 12.4 vs. -6.9 ± 7.2; Th10: -14.3 ± 14.6 vs. -4.7 ± 7.5; Th12: -14.4 ± 14.5 vs. -4.9 ± 7.8; L2: -14.5 ± 14.2 vs. -3.4 ± 6.9). The percentages of aortic remodeling with expansion of the true lumen (distal arch: 82%; Th8: 80%; Th10: 54%; Th12: 45%; L2: 50%) and complete false lumen thrombosis (distal arch: 100%; Th8: 100%; Th10: 67%; Th12: 11%; L2: 0%) were approximately consistent with the change in delta systolic pressure. During a follow-up of 41 months, distal stent-induced new entry occurred in 2 patients (13%) requiring secondary intervention; however, there were no cases of FL enlargement or aorta-related mortality. CONCLUSIONS: Zone 2 TEVAR for uncomplicated TBAD may prevent TEVAR-related complications. Measuring IFLP could be a new predictive marker for assessing the extent of aortic remodeling.

Ultrastructural Features of the Aortic Wall in a Patient with Kommerell Diverticulum.

We report on the ultrastructural features of the aortic wall in a patient with Kommerell diverticulum. A 70-year-old woman with a right aortic arch, aberrant left subclavian artery, and Kommerell diverticulum underwent a successful total arch replacement plus the frozen elephant trunk procedure with anatomical left subclavian artery reconstruction. Small pieces of the ascending aorta, distal arch, right common carotid artery, and left subclavian artery were investigated ultrastructurally. In the ascending aortic wall, multiple cystic cavities were observed in the subintimal region of the media by scanning electron microscopy. Changes in organelles, including mild dilation of rough-surfaced endoplasmic reticulum and mitochondrial swelling and degrading, were also observed in all specimens by transmission electron microscopy. These ultrastructural features may indicate the fragility or stress of the aortic wall and are useful when considering the early surgical intervention of a patient with Kommerell diverticulum.

[Surgical Strategy for Aortic Arch Aneurysm Depending on the Patient's Condition:Proper Selection of Open Surgery or Thoracic Endovascular Repair].

OBJECTIVES: We investigated the validity of the surgical strategy for aortic arch aneurysm depending on the patient's condition. METHODS: Between 2014 and 2019, 70 patients underwent total arch replacement (TAR) and 14 patients underwent fenestrated thoracic endovascular aortic repair( fTEVAR) for aortic arch aneurysm. We selected the surgical strategy on the basis of the patient's condition with or without frailty and if surgical risks including cancer or a respiratory condition precluded open surgery. RESULTS: The preoperative average ages were 73.3±7.8 years in the TAR group and 73.9±6.1 years in the fTEVAR group (p=0.93). EuroSCORE Ⅱ was 4.3±3.6 in the TAR group and 6.0±3.3 in the fTEVAR group (p=0.03). Frailty was observed in 11 patients (15.7%) in the TAR group and fivepatients (35.7%) in the fTEVAR group (p=0.08). In the fTEVAR group, there were three patients (21.4%) with cancer and three patients (21.4%) with a respiratory condition that precluded open surgery. The overall 30-day mortality rate was 0% for both groups, and the in-hospital mortality rate was 2.9% in the TAR group and 0% in the fTEVAR group (p=0.52). The incidence of stroke was 2.9% (two patients) in the TAR group and 7.1%( one patient) in the fTEVAR group( p=0.43). However, all stroke patients were able to walk at discharge. The cumulative survival rate was 88.9% and 83.5% in the TAR group and 85.1% and 68.1% in the fTEVAR group at two and five years, respectively (p=0.173). There were both 98.1% of patients in the TAR group and 85.7% and 75.0% of patients in the fTEVAR group who were free from reoperations at two and five years, respectively( p<0.01). CONCLUSIONS: Our surgical strategy and outcomes for aortic arch aneurysm were generally appropriate. It is important to select open surgery or TEVAR depending on the patient's condition.

Hemobilia associated with a giant thrombosed aneurysm of the hepatic artery requiring hepatectomy.

Hemobilia is an unusual and potentially catastrophic cause of gastrointestinal bleeding. Although hepatic artery aneurysm is a cause of hemobilia, nontraumatic cases are infrequently reported. Herein, we describe the case of a giant hepatic artery aneurysm requiring hepatectomy because of repeated hemobilia in a patient with Marfan syndrome. A 53-year-old man presented to our hospital with sudden epigastric pain and jaundice. Abdominal computed tomography showed a giant hepatic arterial aneurysm in the porta hepatis, and emergency endoscopic retrograde cholangiography revealed hemobilia. Assuming that the aneurysm caused the hemobilia, we performed an abdominal angiogram for treatment. The study revealed a thrombosed aneurysm along with tortuous abnormal vessels in the periphery of the left hepatic artery, which appeared to surround the aneurysm. Therefore, we embolized the left hepatic artery, and immediate hemostasis was achieved. Rebleeding occurred 3 times thereafter, and each time, transarterial embolization was performed, resulting in prompt but only temporary hemostasis. Then, emergency left hemihepatectomy and resection of the aneurysm were performed. Pathologic examination of the resected specimen revealed that the aneurysm was completely thrombosed and organized; however, abnormal arterioles proliferated between the aneurysmal wall and the bile duct. The unique feature of this case was that the abnormal arterioles induced by the organized hepatic artery aneurysm, not the aneurysm itself, caused the hemobilia.

A case of surgical treatment for recurrence of right ventricular metastasis due to renal cell carcinoma after molecular targeted therapy.

BACKGROUND: Cardiac metastasis including the right ventricle from renal cell carcinoma is rare. No standard treatment for cardiac metastasis and recurrence in renal cell carcinoma has been established. CASE PRESENTATION: We present the case of a 61-year-old man who underwent the resection of recurrent right ventricular metastasis caused by renal cell carcinoma following molecular targeted therapy. The first cardiac operation was performed for right ventricular metastasis due to renal cell carcinoma. The patient had a good postoperative course. Two years after the first operation, however, follow-up computed tomography revealed the recurrence of the right ventricular tumor and metastases in both lungs. Molecular targeted therapy was carried out and effectively controlled the lung metastasis but the right ventricular lesion remained unchanged, leading to reoperation. The recurrent right ventricular tumor was completely resected through a redo median sternotomy assisted by cardiopulmonary bypass. The patient had an uneventful postoperative course and was discharged on the 13th postoperative day. Follow-ups at 2 years showed no cardiac recurrence. CONCLUSION: Surgical intervention was considered useful in managing the recurrence of right ventricular metastasis from renal cell carcinoma after molecular targeted therapy.

Mid-Term Outcomes of Aortic Valve Repair Without Aortic Root Replacement: A Single-Center Experience.

Background Aortic valve (AV) repair is a challenging procedure due to its complexity, lower reproducibility, and steep learning curve. To examine its durability and validity, we investigated mid-term outcomes following AV repair without aortic root replacement. Methods Between March 2007 and May 2018, we retrospectively identified 14 patients who underwent AV repair without aortic root replacement at our institution. We investigated their baseline characteristics and postoperative outcomes, including the reoperation rate due to aortic regurgitation (AR) recurrence. Furthermore, we divided them into two groups: those who required reoperation due to AR recurrence (Group R) and those who did not require reoperation (Group F), and statistically compared them. Results The median age was 52.5 years (IQR: 42.0-60.8), with 11 male patients (78.6%). Eight patients (57.1%) had a bicuspid AV. Five cases (35.7%) underwent reoperation due to AR recurrence during a median follow-up period of 5.5 years. There were no significant differences in baseline characteristics between Group R (n=5, 35.7%) and Group F (n=9, 64.3%), including AR etiology, AV repair procedure, and intraoperative AR grade after the final declamp. All cases in Group R had at least mild to moderate AR on the echocardiogram before discharge. Regarding the AR grade before discharge, Group R had a significantly higher grade than Group F (p = 0.013). Conclusions The indication for AV repair for AR might need to be reassessed due to the considerable mid-term reoperation rate. Cases of AV repair with more than mild AR at discharge should be carefully monitored, as they are likely to require future reoperation for AR.

Influence of epicardial adipose tissue inflammation and adipocyte size on postoperative atrial fibrillation in patients after cardiovascular surgery.

Epicardial adipose tissue (EAT) is an active endocrine organ that is closely associated with occurrence of atrial fibrillation (AF). However, the role of EAT in the development of postoperative AF (POAF) remains unclear. We aimed to investigate the association between EAT profile and POAF occurrence in patients who underwent cardiovascular surgery. We obtained EAT samples from 53 patients to evaluate gene expression, histological changes, mitochondrial oxidative phosphorylation (OXPHOS) capacity in the EAT, and protein secretion in EAT-conditioned medium. EAT volume was measured using computed tomography scan. Eighteen patients (34%) experienced POAF within 7 days after surgery. Although no significant difference was observed in EAT profile between patients with and without POAF, logistic regression analysis identified that the mRNA expression levels of tumor necrosis factor-alpha (TNF-α) were positively correlated and adipocyte size in the EAT was inversely correlated with onset of POAF, respectively. Mitochondrial OXPHOS capacity in the EAT was not associated with POAF occurrence; however, it showed an inverse correlation with adipocyte size and a positive correlation with adiponectin secretion. In conclusion, changes in the secretory profile and adipocyte morphology of the EAT, which represent qualitative aspects of the adipose tissue, were present before the onset of AF.

Thoracic stent-graft herniation through the aortic wall in a case of lung cancer.

A 67-year-old man had left upper lung cancer with invasion into the descending aorta. He underwent pre-emptive thoracic endovascular aortic repair using a Valiant Navion followed by left lung upper lobectomy with resection of the aortic wall. Because of continuous bleeding, he underwent re-thoracotomy. Since the surgically resected aortic wall was largely cleaved, bleeding around the stent-graft that herniated into the left pleural cavity was observed. Re-thoracic endovascular aortic repair using a GORE TAG was immediately performed to prevent further stent-graft herniation and impending lethal haemorrhage. It may be necessary to consider reinforcement of the resected aortic wall to prevent thoracic endovascular aortic repair-related complications.

Hybrid Repair of Kommerell Diverticulum and Aberrant Subclavian Artery with Compressive Symptoms and a New Strategy: Case Report.

Surgical indication and treatment for patients with Kommerell diverticulum and aberrant subclavian artery are still not well established. A patient with esophageal and tracheal compression resulting from these anatomical abnormalities was successfully treated with a hybrid approach of total arch replacement, frozen elephant trunk technique, aberrant left subclavian artery transection, and left subclavian artery reconstruction through median sternotomy. Compressive symptoms were relieved without resecting the enlarged diverticulum. In this case, the importance of preoperative investigation for the main cause of compressive symptoms is illustrated and a novel treatment strategy is outlined.

Thiamine treatment preserves cardiac function against ischemia injury via maintaining mitochondrial size and ATP levels.

Thiamine (vitamin B1) is necessary for energy production, especially in the heart. Recent studies have demonstrated that thiamine supplementation for cardiac diseases is beneficial. However, the detailed mechanisms underlying thiamine-preserved cardiac function have not been elucidated. To this end, we conducted a functional analysis, metabolome analysis, and electron microscopic analysis to unveil the mechanisms of preserved cardiac function through supplementation with thiamine for ischemic cardiac disease. Male Sprague-Dawley rats (around 10 wk old) were used. Following pretreatment with or without thiamine pyrophosphate (TPP; 300 µM), hearts were exposed to ischemia (40 min of global ischemia followed by 60 min of reperfusion). We measured the left ventricle developed pressure (LVDP) throughout the protocol. The LVDP during reperfusion in the TPP-treated heart was significantly higher than that in the untreated heart. Metabolome analysis was performed using capillary electrophoresis-time-of-flight mass spectrometry, and it revealed that the TPP-treated heart retained higher adenosine triphosphate (ATP) levels compared with the untreated heart after ischemia. The metabolic pathway showed that there was a significant increase in fumaric acid and malic acid from the tricarboxylic acid cycle following ischemia. Electron microscope analysis revealed that the mitochondria size in the TPP-treated heart was larger than that in the untreated heart. Mitochondrial fission in the TPP-treated heart was also inhibited, which was confirmed by a decrease in the phosphorylation level of DRP1 (fission related protein). TPP treatment for cardiac ischemia preserved ATP levels probably as a result of maintaining larger mitochondria by inhibiting fission, thereby allowing the TPP-treated heart to preserve contractility performance during reperfusion.NEW & NOTEWORTHY We found that treatment with thiamine can have a protective effect on myocardial ischemia. Thiamine likely mediates mitochondrial fission through the inhibition of DRP1 phosphorylation and the preservation of larger-sized mitochondria and ATP concentration, leading to higher cardiac contractility performance during the subsequent reperfusion state.

Body mass index as a tool for optimizing surgical care in coronary artery bypass grafting through understanding risks of specific complications.

OBJECTIVES: To investigate the relationship between body mass index (BMI) and early outcomes, and specific types of morbidities associated with low and high BMI, in patients undergoing coronary artery bypass grafting. METHODS: This was a retrospective study on isolated coronary artery bypass grafting patients (aged ≥60 years) between 2008 and 2017 in the Japan Cardiovascular Surgery Database. The primary end point was defined as operative mortality. The secondary end point was combined morbidity (ie, operative mortality, reoperation for bleeding, stroke, new onset of hemodialysis, mediastinitis, and prolonged ventilation). Patient characteristics and outcomes were compared among BMI groups. Spline curves were fit between BMI and outcomes. Multivariable logistic regression models with categorized BMI and generalized additive models with spline-transformed BMI were used to estimate and visualize the effect of BMI adjusted for other covariates. RESULTS: A total of 96,058 patients were included in the analysis. Low (<18.5) and high (≥30) BMI were both associated with a higher risk of mortality (low: adjusted odds ratio, 1.34; 95% confidence interval, 1.16-1.54; P < .0001, and high: adjusted odds ratio, 2.10; 95% confidence interval, 1.70-2.59; P < .0001) and combined morbidity (low: adjusted odds ratio, 1.18; 95% confidence interval, 1.08-1.29; P = .0002 and high: adjusted odds ratio, 1.82; 95% confidence interval, 1.63-2.03; P < .0001). Low and high BMI were associated with different types of morbidities. In models using spline transformation, the deviation of BMI from a proximately 21 to 23 was proportionally associated with increased risk. CONCLUSIONS: In patients undergoing coronary artery bypass grafting, low and high BMI were risk factors of mortality associated with different types of morbidities, which may warrant tailored preventive approaches.

Urinary Titin Is Increased in Patients After Cardiac Surgery.

Background: Few non-invasive biomarkers have been used to detect myocardial injury in patients with heart diseases. Recently, the N-terminal fragment (N-titin) of titin, a giant sarcomeric protein, which is involved in muscular passive tension and viscoelasticity, has been reported to detect muscle damage in patients with cardiomyopathy as well as in patients with skeletal muscle dystrophy and in healthy volunteers with endurance exercise. In the present study, we evaluated whether urinary N-titin is changed during a perioperative period and whether its increase reflects myocardial damage. Materials and Methods: In 18 patients who underwent cardiac surgery, blood and urine samples were obtained before and after surgery. We measured the urinary levels of N-titin with a highly sensitive ELISA system. Results: Urinary N-titin to creatinine (N-titin/Cr) was significantly increased in all patients postoperatively (43.3 ± 39.5 pmol/mg/dL on the day of operation) and remained significantly high for at least 4 days postoperatively. Urinary N-titin/Cr was positively correlated with serum cardiac troponin T (r = 0.36, p = 0.0006, n = 90) but not creatine kinase-MB (CK-MB). We also found that urinary N-titin/Cr in patients after a coronary artery bypass grafting operation was higher by day 2 postoperatively than in patients following open cardiac surgeries. Conclusion: The cleaved N-titin was significantly increased in urine after cardiac surgery. Urinary N-titin may be useful for detecting the risk of latent postoperative cardiac damage.

Left Atrial Stenosis Induced Pulmonary Venous Arterialization and Group 2 Pulmonary Hypertension in Rat.

The mechanism of mitral stenosis-induced pulmonary venous arterialization and group 2 pulmonary hypertension (PH) is unclear. There is no rodent model of group 2 PH, due to mitral stenosis (MS), to facilitate the investigation of disease mechanisms and potential therapeutic strategies. We present a novel rat model of pulmonary venous congestion-induced pulmonary venous arterialization and group 2 PH caused by left atrial stenosis (LAS). LAS is achieved by constricting the left atrium using a half-closed titanium clip. After the LAS surgery, a rat model with a transmitral inflow velocity greater than or equal to 2.0 m/s on echocardiography gradually develops pulmonary venous arterialization and group 2 PH over an 8- to 10-week period. In this protocol, we provide the step-by-step procedure of how to perform the LAS surgery. The presented LAS rat model mimics MS in humans and is useful for studying the underlying molecular mechanism of pulmonary venous arterialization and for the preclinical evaluation of therapies for group 2 PH.

Rapid expansion of mycotic aneurysm of left coronary sinus of Valsalva causing myocardial ischemia: report of a case.

We described a 71-year-old female of aneurysm of the left sinus of Valsalva from mycotic origin. She underwent aortic valve replacement 11 years ago. Repeated CT scans showed rapidly growing aneurysm below the left coronary ostium. On sixth day after the admission, she suddenly developed myocardial ischemia complicated with ventricular fibrillation. The patient was treated with emergent aortic root replacement and she recovered. We recommend emergent surgical repair of mycotic saccular aneurysm of the left sinus of Valsalva because a delay of surgery could be fatal.

Bladder and rectal incontinence without paraplegia or paraparesis after endovascular aneurysm repair.

Spinal cord ischemia is a well-known potential complication of endovascular aneurysm repair (EVAR), and it is usually manifested by paraplegia or paraparesis. We describe a case in which spinal cord ischemia after EVAR presented by isolated bladder and rectal incontinence without other neurological deficits. A 63-year-old woman presented with intermittent claudication secondary to an infrarenal abdominal aortic aneurysm (AAA), and a left common iliac artery obstruction, for which she underwent EVAR using an aorto-uniiliac (AUI) device and ilio-femoral artery bypass. On postoperative day 3, she developed urinary and fecal incontinence without signs of paraplegia or paraparesis. Magnetic resonance imaging (MRI) showed a hyper-intense signal in the spinal cord. She received hyperbaric oxygen (HBO) therapy and was discharged after 18 days when her urinary and fecal incontinence were almost resolved. This report suggests that spinal cord ischemia after EVAR for aortoiliac occlusive disease might present as bladder and rectal incontinence without other neurological manifestations.

Outcomes of surgical aortic valve replacement using Carpentier-Edwards PERIMOUNT bioprosthesis series in elderly patients with severe aortic valve stenosis: a retrospective cohort study.

OBJECTIVE: We sought to compare the outcomes of Carpentier-Edwards PERIMOUNT (CEP), Magna and Magna Ease valves in Japanese elderly patients with severe aortic valve stenosis (AS). METHODS: We retrospectively identified 136 patients (mean age 76.61 ± 5.5 years old) who had undergone isolated surgical aortic valve replacement (SAVR) using CEP, Magna, and Magna Ease valves at a single institution, from January 2001 to December 2013. We compared the valves according to their survival rates, freedom from major adverse cardiovascular and cerebrovascular events (MACCE), and durability and hemodynamic performance by echocardiographic data. RESULTS: The thirty-day mortality after isolated SAVR in all the valve groups was "zero". The differences among the three groups in terms of survival rates, freedom from MACCE at 2 years, durability and hemodynamic performance of the valves by echocardiographic data was not statistically significant. CONCLUSION: All CEP, Magna and Magna Ease valves seemed to be similarly useful in Japanese elderly patients with severe AS. Our data did not clearly support the superiority of one valve over another. A longer follow-up period might be necessary to compare the durability and hemodynamic performance of these valves with more certainty.

A case report of primary cardiac myxofibrosarcoma presenting with severe congestive heart failure.

BACKGROUND: Primary cardiac sarcomas are extremely rare. Furthermore, the myxofibrosarcomas are one of the rarest forms of cardiac sarcomas, and its prognosis is known to be quite poor. CASE PRESENTATION: This is a case of a 23-year-old man who presented with acute severe congestive heart failure caused by almost complete obstruction of the mitral valve due to a large left atrial tumor. The patient required endotracheal intubation before his arrival to the hospital, and underwent an emergent surgical excision of the tumor. The tumor had a complex shape and originated from the orifice of the right upper pulmonary vein. Because the tumor seemed to extend over most of the surface of the left atrium, it seemed impossible to reconstruct the left atrium had we done a complete transmural resection. Instead, we carefully peeled the tumor leaving the outer layer of the left atrial wall. We applied cryoablation to the attached site, in order to prevent a recurrence of the tumor. The pathology report revealed that the tumor was a myxofibrosarcoma, and it seemed to originate from the heart. The patient received radiation therapy after the surgery and continues to be alive and well after 1-year, without apparent recurrence. CONCLUSIONS: Cardiac myxofibrosarcoma can cause acute, severe left-sided heart failure. Non-transmural atrial wall resection with cryoablation might be effective for patients with cardiac myxofibrosarcomas with extensive atrial attachment.