A unique cardiovascular presentation of Marfan syndrome.

We report a neonate with severe Marfan syndrome (MS), prenatally identified to have persistent atrial tachycardia, biventricular dysfunction, and an unusual structure within the atria. Detailed postnatal echocardiographic evaluation and cross-sectional imaging confirmed congenital pseudoaneurysm of the mitral-aortic intervalvular fibrosa. Emergent testing by next-generation sequencing identified a FBN1 pathological variant, key to establishing goals of care. To our knowledge, this is the first reported case of a congenital pseudoaneurysm of the mitral-aortic intervalvular fibrosa in MS.

Exsanguination from ruptured femoral artery pseudoaneurysm - A fatal complication of groin heroin injection.

Intravenous drug users (IDUs) eventually encounter a common problem- a need to turn to a new vessel to inject drugs. Whether it is because no other spot is available due to scarring or convenience, the groin is the preferred spot for some. Chronic puncture of femoral vessels can lead to a rare but significant complication- femoral artery pseudoaneurysm (FAP). Its fatal consequence- rupture and bleeding is well recognized, but the forensic literature on this subject is limited. We present eight cases of exsanguination due to the ruptured FAP in IDUs who share most or all the following characteristics: long-term heroin use and/or pronounced drug use stigmata, chronic groin injection-related lesions, absence of significant precipitating pseudoaneurysm trauma, and no or minimal concentrations of heroin metabolites in blood. The FAP presentation varied greatly, from palpable fist-sized mass or slight elevation under the skin defect to infundibular arterio-cutaneous fistula that ruptured through the skin induration. In some, surrounding skin or soft tissue showed signs of inflammation but without suppuration. The most prominent FAP characteristic was smooth-surface cavitation on cross-sections. We performed microscopic evaluation in two cases and verified disruption of the artery wall (i.e., pseudoaneurysm) with elements of acute and chronic inflammation and fibrosis; foci of fibrinoid necrosis were noticed on the arterial wall. All subjects were pale, with faint hypostasis and organ anemia, consistent with reported massive hemorrhage. Because such sudden, unwitnessed, and suspicious deaths may raise the question of injury infliction, proper autopsy evaluation is crucial, for which we propose guidelines.

Superficial temporal artery pseudoaneurysm: Report of two cases and review.

Superficial temporal artery pseudoaneurysm is a rare complication of arterial injury developing on the forehead following blunt trauma. There is little written on this entity in the dermatopathology literature. We describe two cases of superficial temporal artery pseudoaneurysm in two men aged 53 and 25 years, one of whom had a recent history of head trauma. This report reviews the classic histopathologic findings of STA pseudoaneurysm and highlights ways to distinguish it from other entities in the differential diagnosis.

Immune checkpoint programmed death-1 mediates abdominal aortic aneurysm and pseudoaneurysm progression.

PURPOSE: The causes and pathogenetic mechanisms underlying abdominal aortic aneurysms (AAAs) and pseudoaneurysms are not fully understood. We hypothesized that inhibiting programmed death-1 (PD-1) can decrease AAA and pseudoaneurysm formation in mouse and rat models. METHODS: Human AAA samples were examined in conjunction with an adventitial calcium chloride (CaCl2) application mouse model and an aortic patch angioplasty rat model. Single-dose PD-1 antibody (4 mg/kg) or BMS-1 (PD-1 inhibitor-1) (1 mg/kg) was administered by intraperitoneal (IP) or intraluminal injection. In the intramural injection group, PD-1 antibody was injected after CaCl2 incubation. The rats were divided into three groups: (1) the control group was only decellularized without other special treatment, (2) the PD-1 antibody-coated patch group, and (3) the BMS-1 coated patch group. Patches implanted in the rat abdominal aorta were harvested on day 14 after implantation and analyzed. RESULTS: Immunohistochemical analysis showed PD-1-positive cells, PD-1 and CD3, PD-1 and CD68, and PD-1 and α-actin co-expressed in the human AAA samples. Intraperitoneal (IP) injection or intraluminal injection of PD-1antibody/BMS-1 significantly inhibited AAA progression. PD-1 antibody and BMS-1 were each successfully conjugated to decellularized rat thoracic artery patches, respectively, by hyaluronic acid. Patches coated with either humanized PD-1 antibody or BMS-1 can also inhibit pseudoaneurysm progression and inflammatory cell infiltration. CONCLUSION: PD-1 pathway inhibition may be a promising therapeutic strategy for inhibiting AAA and pseudoaneurysm progression.

Incidence of and predisposing factors for pseudoaneurysm formation in a high-volume cardiovascular center.

PURPOSE: To evaluate factors associated with pseudoaneurysm (PSA) development. METHODS: Between January 2016 and May 2020, 30,196 patients had invasive vascular radiological or cardiac endovascular procedures that required arterial puncture. All patients with PSA were identified. A matched (age, gender, and type of the procedure) control group of 134 patients was created to reveal predictors of PSA formation. RESULTS: Single PSAs were found in 134 patients. Fifty-three PSAs developed after radiological procedures (53/6555 [0.8%]), 31 after coronary artery procedures (31/18038 [0.2%]), 25 after non-coronary artery cardiac procedures (25/5603 [0.4%]), and 25 due to procedures in which the arterial puncture was unintended. Thirty-four PSAs (25.4%) were localized to the upper extremity arteries (vascular closure device [VCD], N = 0), while 100 (74.6%) arose from the lower extremity arteries (VCD, N = 37). The PSA prevalence was 0.05% (10/20478) in the radial artery, 0.1% (2/1818) in the ulnar artery, 1.2% (22/1897) in the brachial artery, and 0.4% (99/22202) in the femoral artery. Treatments for upper and lower limb PSAs were as follows: bandage replacement (32.4% and 14%, respectively), ultrasound-guided compression (11.8% and 1%, respectively), ultrasound-guided thrombin injection (38.2% and 78%, respectively), and open surgery (17.6% and 12%, respectively). Reintervention was necessary in 19 patients (14.2%). The prevalence of PSA for the punctured artery with and without VCD use was 37/3555 (1%) and 97/27204 (0.4%), respectively (OR, 2.94; 95% CI, 1.95-4.34; P<0.001). The effect of red blood cell (RBC) count (P<0.001), hematocrit value (P<0.001), hemoglobin value (P<0.001), international normalized ratio (INR; P<0.001), RBC count-INR interaction (P = 0.003), and RBC count-VCD use interaction (P = 0.036) on PSA formation was significant. CONCLUSION: Patients in whom the puncture site is closed with a VCD require increased observation. Preprocedural laboratory findings are useful for the identification of patients at high risk of PSA formation.

A novel intramural TGF ß 1 hydrogel delivery method to decrease murine abdominal aortic aneurysm and rat aortic pseudoaneurysm formation and progression.

OBJECTIVES: Aneurysms are generally the result of dilation of all 3 layers of the vessel wall, and pseudoaneurysms are the result of localized extravasation of blood that is contained by surrounding tissue. Since there is still no recommended protocol to decrease aneurysm formation and progression, we hypothesised that intramural delivery of TGF ß1 hydrogel can decrease aneurysm and pseudoaneurysm formation and progression. MATERIALS: Male C57BL/6 J mice (12-14 wk), SD rats (200 g) and pig abdominal aortas were used, and hydrogels were fabricated by the interaction of sodium alginate (SA), hyaluronic acid (HA) and CaCO3. METHODS: A CaCl2 adventitial incubation model in mice and a decellularized human great saphenous vein patch angioplasty model in rats were used. TGF ß1 hydrogel was intramurally delivered after CaCl2 incubation in mice; at day 7, the abdomen in some mice was reopened, and TGF ß1 hydrogel was injected intramurally into the aorta. In rats, TGF ß1 hydrogel was delivered intramurally after patch angioplasty completion. Tissues were harvested at day 14 and analysed by histology and immunohistochemistry staining. The pig aorta was also intramurally injected with hydrogel. RESULTS: In mice, rhodamine hydrogel was still found between the medium and adventitia at day 14. In the mouse aneurysm model, there was a thicker wall and smaller amount of elastin breaks in the TGF ß1 hydrogel-delivered groups both at day 0 and day 7 after CaCl2 incubation, and there were larger numbers of p-smad2- and TAK1-positive cells in the TGF ß1 hydrogel-injected groups. In the rat decellularized human saphenous vein patch pseudoaneurysm model, there was a higher incidence of pseudoaneurysm formation when the patch was decellularized using 3% SDS, and delivery of TGF ß1 hydrogel could effectively decrease the formation of pseudoaneurysm formation and increase p-smad2 and TAK1 expression. In pig aortas, hydrogels can be delivered between the medium and adventitia easily and successfully. CONCLUSIONS: Intramural delivery of TGF ß1 hydrogel can effectively decease aneurysm and pseudoaneurysm formation and progression in both mice and rats, and pig aortas can also be successfully intramurally injected with hydrogel. This technique may be a promising drug delivery method and therapeutic choice to decrease aneurysm and pseudoaneurysm formation and progression in the clinic.

A case of idiopathic extracranial carotid artery pseudoaneurysm with a rare clinical course and pathological features.

Extracranial carotid artery aneurysms (ECAAs) are rare, with the etiology mainly classified as degeneration or dissection. Pseudoaneurysms in the region are even rarer and are seen following trauma, iatrogenic injury, or infection. We report a case of extracranial carotid artery pseudoaneurysm (pseudo-ECAA) with a rare clinical course and pathological features. A 58-year-old man presented with swelling and purpura on the left side of his neck after sneezing. Radiological examinations suggested a ruptured left common carotid artery aneurysm. The operative findings were consistent with a pseudoaneurysm. Pathological examination revealed disarrangement and degeneration of smooth muscle fibers in the media, in addition to scattered foci of mucoid accumulation and irregular-shaped cavitation in the medial extracellular matrix, raising the possibility of an intrinsic dysfunction of the vascular wall in the pathological process of pseudoaneurysm formation.

Factors associated with local invasion in infective endocarditis: a nested case-control study.

OBJECTIVE: A substantial proportion of infective endocarditis (IE) cases are complicated by local invasion. The purpose of this study was to identify patient and disease characteristics associated with local invasion in surgically treated IE patients. METHODS: This was a nested case-control study. All episodes of IE for patients admitted to Cleveland Clinic from 1 January 2013 to 30 June 2016 were identified from the Cleveland Clinic IE Registry. Patients ≥18 years of age who underwent surgery for IE were included. Among these, cases were those with local invasion, controls were those without. Local invasion, defined as periannular extension, paravalvular abscess, intracardiac fistula or pseudoaneurysm, was ascertained from the surgical operative note. Associations of selected factors with local invasion were examined in a multivariable logistic regression model. RESULTS: Among 511 patients who met inclusion criteria, 215 had local invasion. Mean age was 56 years; 369 were male. Overall 345 (68%) had aortic valve, 228 (45%) mitral valve, and 66 (13%) tricuspid or pulmonic valve involvement. Aortic valve involvement (OR 6.23, 95% CI 3.55-11.44), bioprosthetic valve (OR 3.88, 95% CI 2.36-6.44), significant paravalvular leak (OR 3.80, 95% CI 1.60-9.89), new atrioventricular nodal block (OR 3.77, 95% CI 1.87-7.90), infection with streptococci other than viridans group streptococci (OR 7.54, 95% CI 2.42-24.87) and presence of central nervous system emboli (OR 1.85, 95% CI 1.13-3.04) were associated with local invasion. DISCUSSION: Intracardiac and microorganism factors, but not comorbid conditions, are associated with local invasion in IE.

Fatal rupture of pseudoaneurysm following angioplasty.

We report the sudden unexpected death from exsanguination of a 60-year-old white female who underwent balloon angioplasty of her right popliteal artery via a right common femoral arteriotomy 3 weeks before death. This resulted in a mycotic pseudoaneurysm of the right femoral artery that ruptured, causing fatal exsanguination. A pseudoaneurysm is an arterial wall defect in which part of the wall consists of fibrin and fibrous tissue, lacking components of the normal arterial wall, that is intima, media, and adventitia. Pseudoaneurysms result from traumatic arterial injury, infection, rupture of a true aneurysm, or surgery. A literature search revealed no reports with the patient dying unexpectedly outside the hospital. Immediate surgical intervention and antibiotic therapy are preferred treatments.

Spontaneous pseudoaneurysm of the posterior tibial artery successfully treated by open surgery: Case report and review of the literature.

RATIONALE: An extremely rare spontaneous pseudoaneurysm (PSA) of the posterior tibial artery (PTA) in a middle-aged male patient was cured by open surgery effectively. PATIENT CONCERNS: A 53-year-old man presented with the increasing swollen left shank for 1 day, with intermittent pain, pulselessness and pallor. He denied the history of trauma, infection, and other diseases. DIAGNOSES: Physical examination, past medical history, ankle brachial index, ultrasonography, computed tomographic angiography (CTA), 3-dimensional reconstruction image of the popliteal artery and its branches and histological examination of intraluminal thrombus and clots helped us diagnose the patient as spontaneous PSA of PTA. INTERVENTIONS: Our patient underwent excision of PSA and repair operation of PSA. OUTCOMES: The patient recovered well at 2-year follow-up. LESSONS: This rare case provides valuable insights for tissue repair and vascular surgery. Therapeutic methods should be in accordance with the best interest of patient. Open surgery is the effective treatment for spontaneous PSA of PTA.

Post-traumatic preauricular pulsatile swelling in a patient on oral anticoagulation therapy.

The differential diagnoses for preauricular swellings include dermoid cyst, lymph nodes, lipoma, nerve sheath tumours, parotid swelling, mastoiditis, vascular malformations and arterio-venous fistulas aneurysms/pseudoaneurysms. Superficial temporal artery pseudoaneurysm(s) (STAPA) are rare (1% of all aneurysms) vascular complications, which occur following a blunt injury of the head or iatrogenic causes. The use of anticoagulation therapy increases the risk of pseudoaneurysm formation. We present a case of traumatic STAPA while on oral anticoagulation. He was treated with surgical exploration, STAPA excision with ligation of the vessel. He had an uneventful recovery with a good functional and cosmetic outcome at 1 year.

Erodes and bleeds, fistulises and obstructs: Bouveret's syndrome.

One of the rare complications of cholelithiasis is gallstone ileus which occurs in <5% of patients. Among them, <3% develop Bouveret's syndrome: gastric outlet obstruction by a large gallstone in the pylorus or duodenum. One large review of 128 patients showed it to present at a mean age of 74.1 ± 11.1 years with a mean gallstone size of 4.6 ± 1.5 cm, with nausea or vomiting in 86%, abdominal pain in 71% and haematemesis in 15%. The diagnosis is usually clear on oesophagogastroduodenoscopy or abdominal contrast-enhanced computed tomography. We present a case with massive upper gastrointestinal bleeding, due to erosion of a cystic artery pseudoaneurysm.

Dilatation of Aortic Neck and Common Iliac Arteries after Open Repair of Abdominal Aortic Aneurysms: Long-Term Follow-Up According to Aortic Reconstruction Configuration.

BACKGROUND: Several studies in the literature report continued proximal aorta and distal iliac artery dilatation after surgical correction of an abdominal aortic aneurysm (AAA). The purpose of this study is to evaluate these findings, in a South American population, and relate them to the type of configuration of the open procedure aortic reconstruction. METHODS: This is a retrospective review of ultrasonographic follow-up of patients submitted to open repair of AAA from 1989 to 2013, reporting proximal aorta dilatation (≥3 cm) and distal iliac artery dilatation (≥1.5 cm). RESULTS: A total of 155 patients were included. Life-table freedom at the intervals 11 < 15 years and ≥15 years were 47% and 23% for proximal dilatation and 63% and 38% for distal iliac arteries dilatation, respectively. There were more proximal and distal dilatations in patients submitted to more extensive aortic reconstructions (aorto-aortic 13% and 22% vs aorto-bilateral common iliacs 27% and 8% vs aorto-unilateral or bilateral external iliacs 27% and 32% and aorto-femoral 67% and 0%) P < 0.0001. Juxtarenal anastomosis was also correlated with more proximal dilatations (42% vs 21%, P = 0,046). There were two proximal and three distal anastomosis pseudoaneurysms. CONCLUSIONS: The presence of more extensive degenerative disease at the time of operation, requiring juxtarenal or more distal iliac reconstructions, may pose an increased risk of proximal aorta and iliac artery dilatation during follow-up. This study corroborates that significant changes are found after 7 to 10 years of the operation, reinforcing the need for long-term monitoring.

Sudden death due to left ventricular thrombosis: A report of two autopsy cases.

PURPOSE: In this paper, we report two rare cases of sudden death due to giant left ventricular thrombus revealed at autopsy and we discuss the manner and the cause of death. RESULTS: Cases presentation: The two cases reported are about two men aged 55 and 53 years respectively. In the two cases, no past cardiac history was found. Prior to the onset of complications and subsequent death, both patients presented to the emergency department with progressing asthenia, faintness and shortness of breath, were treated symptomatically. At autopsy, atherosclerosis of coronary arteries was found. In the first case, death was attributed to thrombosis of the pseudoaneurysm. In the second case, it was due to thrombosis complicating a myocardial infarction. The mechanism of death in the first case was explained by the hemodynamic shock caused by a total left ventricular pseudoaneurysm thrombosis secondary to old myocardial infraction. In the second case, death was the consequence of a cardiogenic shock secondary to thrombosis of the cardiac pseudoaneurysm complicating a myocardial infraction. CONCLUSION: Complications of myocardial infarction represent frequent causes of adult sudden death. Left ventricular thrombosis is a complication that is often fatal and its discovery during an autopsy remains rare. The first-line doctor must take these types of complications into consideration in order to detect them and thus ensure timely management.

Incidental detection of calcified left coronary artery pseudoaneurysm years after surgery for TOF.

We present images from computed tomography angiography in a 40-year-old female with a calcified pseudo-aneurysm in her left coronary artery, who had previously undergone repair of tetralogy of Fallot.