Case report: Navigating treatment pathways for cardiac intimal sarcoma with PDGFRß N666K mutation.

In the realm of rare cardiac tumors, intimal sarcoma presents a formidable challenge, often requiring innovative treatment approaches. This case report presents a unique instance of primary intimal sarcoma in the left atrium, underscoring the critical role of genomic profiling in guiding treatment. Initial genomic testing unveiled a somatic, active mutation in PDGFRß (PDGFRß N666K), accompanied by MDM2 and CDK4 amplifications. This discovery directed the treatment course toward pazopanib, a PDGFRß inhibitor, following irradiation. The patient's response was remarkable, with the therapeutic efficacy of pazopanib lasting for 16.3 months. However, the patient experienced a recurrence in the left atrium, where subsequent genomic analysis revealed the absence of the PDGFRß N666K mutation and a significant reduction in PDGFRß expression. This case report illustrates the complexities and evolving nature of cardiac intimal sarcoma treatment, emphasizing the potential of PDGFRß signaling as a strategic target and highlighting the importance of adapting treatment pathways in response to genetic shifts.

Motor-Evoked Potential Monitoring With Multi-train Electrical Stimulation During Thoracoabdominal Aortic Aneurysm Surgery: A Case Report.

Intraoperative motor-evoked potentials (MEPs) are measured for assessing motor function during surgery. MEP monitoring is often performed in thoracoabdominal aortic aneurysm (TAAA) surgery, but false positives are common and amplification methods are needed to obtain waveforms under severe conditions to assess proper spinal cord function. One method of amplitude amplification in transcranial-stimulated MEP monitoring is multitrain stimulation. There are few reports on multitrain-stimulated MEP monitoring for this surgery. A 57-year-old woman underwent open repair of the thoracoabdominal aorta due to a dissecting aortic aneurysm. After opening the chest, the aneurysm was incised proximally, and anastomosis with an artificial vessel was initiated. The lumbar artery leading to the Adam-Kiewicz artery was reconstructed at a body temperature of 25 °C. However, the single-train stimulation did not produce MEPs. When the measurement was switched to multitrain stimulation, MEPs were elicited in the lower extremity muscle groups and the waveforms were maintained until the end of the measurement. This case illustrates that MEP monitoring using multitrain stimulation during descending thoracic aortic aneurysm surgery can effectively elicit MEPs under challenging conditions, in which conventional single-train stimulation may be insufficient.

[Right Atrial Blood Cyst with Patent Foramen Ovale:Report of a Case].

A woman in 70s was diagnosed with lung cancer, and a right atrial mass was discovered incidentally during preoperative examination by contrast-enhanced computed tomography (CT). Transesophageal echocardiography revealed a 20-mm, stemmed, spherical mass with low internal echogenicity and partially high echogenicity extending from the junction of the inferior vena cava to the posterior wall of the right atrium. Patent foramen ovale( PFO) was also confirmed. To avoid embolization and obtain diagnosis, the patient was referred for right atrial tumor resection. Cardiopulmonary bypass was established; the right atrial tumor was removed while the patient was in cardiac arrest. The tumor membrane was thin and easily ruptured, revealing jelly-like blood content and calcified mass. The patient recovered well after surgery and was discharged on day 15. According to the pathological examination, the tumor was a blood cyst. This is an extremely rare case of a blood cyst with PFO.

Single-cell transcriptomics identifies adipose tissue CD271+ progenitors for enhanced angiogenesis in limb ischemia.

Therapeutic angiogenesis using mesenchymal stem/stromal cell grafts have shown modest and controversial effects in preventing amputation for patients with critical limb ischemia. Through single-cell transcriptomic analysis of human tissues, we identify CD271+ progenitors specifically from subcutaneous adipose tissue (AT) as having the most prominent pro-angiogenic gene profile distinct from other stem cell populations. AT-CD271+ progenitors demonstrate robust in vivo angiogenic capacity over conventional adipose stromal cell grafts, characterized by long-term engraftment, augmented tissue regeneration, and significant recovery of blood flow in a xenograft model of limb ischemia. Mechanistically, the angiogenic capacity of CD271+ progenitors is dependent on functional CD271 and mTOR signaling. Notably, the number and angiogenic capacity of CD271+ progenitors are strikingly reduced in insulin-resistant donors. Our study highlights the identification of AT-CD271+ progenitors with in vivo superior efficacy for limb ischemia. Furthermore, we showcase comprehensive single-cell transcriptomics strategies for identification of suitable grafts for cell therapy.

[Left Metastatic Lung Tumor for a Patient with Scimitar Syndrome:Report of a Case].

Scimitar syndrome is a subtype of partial anomalous pulmonary venous connection, a rare congenital disorder associated with hypoplasia of the right lung. In addition to the difficulty of isolated lung ventilation, resection of the left lung is associated with the risk of developing right heart failure due to increased right-to-left shunts. We report a case of a left lung metastasis of a patient with scimitar syndrome. The patient, a 58-year-old male, was diagnosed with scimitar syndrome at the age of 26 but had never experienced any symptoms. He underwent chemoradiotherapy for mid-pharynx carcinoma and achieved complete response. During follow-up, a nodule appeared in the lower lobe of the left lung. Since right heart catheterization revealed a pulmonary blood flow/systemic blood flow ratio (Qp/Qs) ratio of 2.6, intra-cardiac blood flow was diverted prior to pulmonary resection. Stanford type A acute aortic dissection occurred intra-operatively, and total aortic arch replacement was performed. Three months later, partial pulmonary resection was performed with extracorporeal membrane oxygenation (ECMO) on standby. As oxygenation was maintained by placing a blocker in the left lower lobe bronchus and ventilating the left upper lobe with high frequency jet ventilation, the operation was completed without using ECMO. The nodule was pathologically diagnosed as metastasis of mid-pharynx carcinoma. He did not develop heart failure and was discharged on post operated day 15.

Single cell transcriptomics identifies adipose tissue CD271+ progenitors for enhanced angiogenesis in limb ischemia.

Therapeutic angiogenesis using mesenchymal stem/stromal cell grafts have shown modest and controversial effects in preventing amputation for patients with critical limb ischemia. Through single-cell transcriptomic analysis of human tissues, we identified CD271 + progenitors specifically from subcutaneous adipose tissue (AT) as having the most prominent pro-angiogenic gene profile distinct from other stem cell populations. AT-CD271 + progenitors demonstrated robust in vivo angiogenic capacity, over conventional adipose stromal cell grafts, characterized by long-term engraftment, augmented tissue regeneration, and significant recovery of blood flow in a xenograft model of limb ischemia. Mechanistically, the angiogenic capacity of CD271 + progenitors is dependent on functional CD271 and mTOR signaling. Notably, the number and angiogenic capacity of CD271 + progenitors was strikingly reduced in insulin resistant donors. Our study highlights the identification of AT-CD271 + progenitors with in vivo superior efficacy for limb ischemia. Furthermore, we showcase comprehensive single-cell transcriptomics strategies for identification of suitable grafts for cell therapy. HIGHLIGHTS: Adipose tissue stromal cells have a distinct angiogenic gene profile among human cell sources. CD271 + progenitors in adipose tissue have a prominent angiogenic gene profile. CD271 + progenitors show superior therapeutic capacities for limb ischemia. CD271 + progenitors are reduced and functionally impaired in insulin resistant donors.

15-year outcomes of the JOCRI study (JOCRIED study): a randomised comparison of off-pump and on-pump multiple arterial coronary revascularisation.

OBJECTIVE: The Japanese Off-Pump Coronary Revascularization Investigation (JOCRI) study reported a non-significant difference in early outcomes and graft patency between off-pump coronary artery bypass grafting and on-pump coronary artery bypass grafting in 2005. The JOCRIED study aimed to review the long-term outcomes of the JOCRI study participants. METHOD AND RESULTS: The JOCRIED study enrolled 123 of the JOCRI study participants completing the clinical follow-up between August 2018 and August 2020; 61 patients in the off-pump group and 62 patients in the on-pump group. The follow-up period was 13.8 ± 2.8 years. The groups were compared regarding mortality, the incidence of major adverse cardiac and cerebrovascular events and repeat revascularisation. The 15-year cumulative survival rate (off-pump vs on-pump, respectively; 77.7% vs 75.3%; p = 0.85), major adverse events-free survival rate (62.5% vs 55.6%; p = 0.27) and repeat revascularisation-free rate (84.8% vs 78.0%; p = 0.16) were not significantly different between the two groups. Revascularisation was the most common major adverse events in the JOCRIED participants. Although percutaneous coronary intervention was performed in 8 patients (13%) in the off-pump group and in 14 patients (23%) in the on-pump group (p = 0.23), no patients underwent redo coronary artery bypass grafting. CONCLUSIONS: Off-pump coronary artery bypass grafting provides comparable 15-year outcomes to on-pump coronary artery bypass grafting.

Total arch replacement and frozen elephant trunk for acute type A aortic dissection.

OBJECTIVE: The present study aimed to evaluate the outcomes of total aortic arch replacement with proximalization of distal anastomosis using the frozen elephant trunk technique with the J Graft FROZENIX (Japan Lifeline, Tokyo, Japan) and Gelweave Lupiae (Vascutek Terumo Inc, Scotland, United Kingdom) graft (distal anastomosis performed in zones 1 and 2) in patients with acute Stanford type A acute aortic dissection. METHODS: A total of 50 patients underwent total aortic arch replacement using the frozen elephant trunk technique, deploying the J Graft FROZENIX into zone 1 or 2 (zone 1: n = 17, zone 2: n = 33) in combination with the Gelweave Lupiae graft for acute Stanford type A acute aortic dissection. Patient characteristics, intraoperative data, and early and midterm outcomes were analyzed. RESULTS: The overall in-hospital mortality rate was 4% (2 patients). The in-hospital mortality rate in patients with visceral malperfusion was 11% (1/9). There were no patients with paraplegia and stent graft-induced new entry. Resection or closure of the most proximal entry tear was achieved in 100% of 42 patients who had postoperative computed tomography. The overall survival was 87.9%, 84.1%, and 84.1% at 1, 2, and 3 years, respectively. However, 1 patient required endovascular extension for the dilatation of the descending thoracic aorta 4 months after the initial surgery. CONCLUSIONS: Total aortic arch replacement with the frozen elephant trunk technique (zone 1-2) and Gelweave Lupiae graft was safe and effective in simplifying surgery for acute Stanford type A acute aortic dissection.

Clinical implications of antegrade diastolic pulmonary artery flow in adults.

BACKGROUND: End-diastolic opening of the pulmonary valve and subsequent antegrade diastolic pulmonary artery flow (ADPAF) reflect restrictive right ventricular (RV) physiology in children. However, this has attracted little attention in adults. PURPOSE: To clarify the clinical implications of ADPAF in adults. METHODS AND RESULTS: The study population consisted of 23,049 consecutive adult patients who underwent echocardiography in our hospital between 2008 and 2015. ADPAF was found in 17 patients (0.07%). The simultaneous recording of RV and pulmonary artery pressures revealed marked elevation of RV diastolic pressure, which exceeded pulmonary artery pressure at the time of atrial contraction. These results suggested that ADPAF implies RV restriction. Based on the level of tricuspid annular plane systolic excursion (TAPSE), we classified these patients into two groups: reduced RV function (R-RVF) group (12 patients with TAPSE <17 mm) and preserved RV function (P-RVF) group (5 patients with TAPSE ≥17 mm). In the R-RVF group, four patients died, one patient underwent left ventricular assist device implantation, and two patients underwent unplanned hospitalization for heart failure during follow-up. The R-RVF group had poorer prognosis and higher mortality rate compared with the P-RVF group. CONCLUSIONS: ADPAF reflects RV restriction in adults. ADPAF suggests a less favorable prognosis in patients with R-RVF.

Partial aortic root remodeling for chronic aortic dissection with coronary intimal tear.

While there are many reports on partial aortic root remodeling, it is rarely performed for chronic aortic dissection of the coronary artery. This report presents a case of a 69-year-old man incidentally diagnosed with aortic dissection during routine checkup. He had a history of percutaneous coronary intervention from the left main trunk to the left anterior descending artery and left circumflex artery. Computed tomography revealed a chronic type A aortic dissection with an aneurysmal aortic root. The false lumen of the Valsalva sinus originated from the left anterior descending artery and expanded largely to the non-coronary Valsalva sinus. We performed partial aortic root remodeling, resecting the dissected non-coronary Valsalva sinus. The postoperative course was uneventful. Partial aortic root remodeling was effective, but its use might be controversial for chronic aortic dissection without resection of the primary entry of the left anterior descending artery. Moreover, close follow-up is required.

Cardiac haemangioma treated with surgical resection involving reconstruction of the right ventricle.

A right ventricular cardiac tumour was incidentally detected in a 50-year-old woman during medical check-up. Transthoracic echocardiography, computed tomography and magnetic resonance imaging revealed that the right ventricular tumour was widely attached to the free wall of the right ventricle and extended to the right ventricular outflow tract. The tumour was excised surgically, and the postoperative course was uneventful. Histological examination revealed that the tumour was a cardiac haemangioma.

Thoracic endovascular aortic repair for an ascending aortic pseudoaneurysm.

An 80-year-old woman underwent aortic valve replacement and ascending aortic replacement. Two years later, computed tomography revealed a pseudoaneurysm of the ascending aorta replaced with a prosthesis. The pseudoaneurysm arose from the stump of a side branch of the prosthesis. Endovascular treatment for the pseudoaneurysm was carried out using the aortic extension cuff of an infrarenal endovascular system. The postoperative course was uneventful.

Asymptomatic carotid intraplaque hemorrhage is associated with a high risk of cerebral infarction and death after cardiovascular surgery.

BACKGROUND: There is a lack of information on the natural history of asymptomatic carotid artery stenosis (AsymCS) associated with cardiovascular diseases that require surgery. The aim of this study was to investigate risk factors for postoperative ipsilateral ischemic stroke and all-cause mortality after cardiovascular surgery in patients with AsymCS. METHODS: Among 2158 patients who underwent cardiovascular surgery, 150 patients with AsymCS who didn't undergo carotid revascularization were included. The relationships between preoperative factors, including carotid intraplaque hemorrhage (IPH), and postoperative ipsilateral ischemic stroke and all-cause mortality were analyzed retrospectively. RESULTS: During the median follow-up of 1087 days of 150 patients with 19 IPH, 12 (8.0%) and 21 (14.0%) encountered ipsilateral infarction and all-cause mortality, respectively. Multivariable Cox regression analyses indicated that IPH was significantly predictive of both ipsilateral infarction (hazard ratio [HR] 21.31, 95% confidence interval [CI], 4.98-91.17; P ≤.001) and all-cause mortality (HR 4.64, 95% CI, 1.61-13.34; P = .004). Another significant factor was peak systolic velocity for ipsilateral infarction with the cutoff velocity of 227 cm/s by the receiver-operating characteristic curve. CONCLUSIONS: In this cohort of patients with AsymCS undergoing cardiovascular surgery, IPH had a close connection with a high risk of both postoperative ischemic stroke and mortality after cardiovascular surgery.

[Coil Embolization for an Aberrant Left Subclavian Artery Aneurysm with Kommerell's Diverticulum;Report of a Case].

A 67-year-old woman encountered a traffic accident and had chest computed tomography(CT) examination. It revealed a 24 mm Kommerell's diverticulum associated with a right-sided aortic arch and a 15 mm saccular aneurysm of an aberrant left subclavian artery. We performed intra-aneurysmal coil embolization for the left subclavian artery aneurysm after a balloon occlusion test of the left subclavian artery. The postoperative course was uneventful.

Manifestation of coronary subclavian steal phenomenon using reactive hyperemia in the ipsilateral forearm.

Coronary subclavian steal syndrome (CSSS) is a coronary steal phenomenon secondary to subclavian artery stenosis in patients who have undergone coronary bypass surgery with the internal thoracic artery. Most commonly, CSSS is diagnosed angiographically. Our case emphasizes that stress ultrasound assessment using reactive hyperemia in the ipsilateral arm elicits a functional diagnosis of CSSS.

Aneurysm resection interposed with a spiral saphenous vein graft in a patient with a popliteal venous aneurysm with thrombosis.

We describe the case of a 46-year-old man with pulmonary thromboembolism caused by a popliteal venous aneurysm with thrombosis. The aneurysm was fusiform and partially saccular with a thrombus, and the caliber of the native popliteal vein was large. Tangential aneurysmectomy with lateral venorrhaphy was difficult because of the aneurysm type, and graft interposition was required because of the large venous diameter of the anastomosis site. The patient underwent aneurysm resection interposed with a spiral saphenous vein graft. The postoperative course was uneventful, and the graft was patent at 1 year after surgery.

Unusual Aorto-mitral Discontinuity Caused by Infective Endocarditis.

Aorto-mitral discontinuity is considered a relatively rare complication of infective endocarditis, usually requiring invasive surgical procedures, including double valve replacement in addition to aortic root replacement/repair. In the present case report, images were produced using transthoracic echocardiograms and transesophageal echocardiograms, which may assist in the planning of surgical procedures. The images captured using real-time three-dimensional transesophageal echocardiograms revealed aorto-mitral discontinuity and a perforated mitral valve aneurysm of the anterior leaflet.