Ryan C. Schenning

Genetics, Pregnancy, and Aortic Degeneration

We present a case of familial thoracic aortic aneurysm and dissection (FTAAD) in a pregnant female. FTAAD is an inherited, nonsyndromic aortopathy resulting from several genetic mutations critical to aortic wall integrity have been identified. One such mutation is the myosin heavy chain gene (MYH11) which is responsible for 1-2% of all FTAAD cases. This mutation results in aortic medial degeneration, loss of elastin, and reticulin fiber fragmentation predisposing to TAAD. Aortic disease is more aggressive during pregnancy as a result of increased wall stress from hyperdynamic cardiovascular changes and estrogen-induced aortic media degeneration. Our patient was a 29-year-old G2P1 woman at 26 weeks gestation presenting with abdominal and back pain. Work-up revealed a 6.4-cm ascending aortic aneurysm with a type A dissection extending into all arch vessels, aortic coarctation at the isthmus, and a separate focal type B aortic dissection with visceral involvement. Surgical management included concomitant cesarean section with delivery of a live premature infant, tubal ligation, ascending aortic replacement with reconstruction of the arch vessels, and aortic valve resuspension. The type B dissection was managed medically without complication. This is the first reported case of aortic dissection in a patient with FTAAD/MYH11 mutation and pregnancy. This case highlights that FTAAD and pregnancy cause aortic degeneration via distinct mechanisms and that hyperdynamics of pregnancy increase aortic wall stress. Management of pregnancy associated with aortopathy requires early transfer to a tertiary center, careful investigation to identify familial aortopathy, fetal monitoring, and a multidisciplinary team approach.

Alveolar Soft Part Sarcoma: Unusual Etiology of Mediastinal Mass in an Adolescent

Alveolar soft part sarcoma (ASPS) is a rare malignancy that usually arises in an extremity. Mediastinal involvement is uncommon, with only two reports of primary mediastinal disease and two reports of metastatic mediastinal disease in the literature, all referencing adult patients. To our knowledge, ours is the first report of ASPS presenting with a mediastinal mass in adolescence. Although ASPS is not generally included in the differential for adolescent mediastinal masses, it should be considered when clinical presentation and imaging appearance are characteristic.

Risk factors for stent graft thrombosis after transjugular intrahepatic portosystemic shunt creation

Background To identify risk factors of stent graft thrombosis after transjugular intrahepatic portosystemic shunt (TIPS) creation. Methods Patients who underwent TIPS creation between June 2003 and January 2016 and with follow-up assessing stent graft patency were included (n=174). Baseline comorbidities, liver function, procedural details and follow-up liver function tests were analyzed in association with hazards of thrombosis on follow-up. Competing risk cox regression models were used considering liver transplant after TIPS creation as the competing risk variable. Results One-, 2- and 5-year primary patency rates were 94.1%, 91.7% and 78.2%, respectively. Patient age [sub-hazard ratio (sHR): 1.13; P=0.001], body mass index (BMI) <30 (sHR: 33.08; P=0.008) and a higher post-TIPS portosystemic pressure gradient (sHR: 1.14; P=0.023) were significantly associated with TIPS thrombosis in multivariate analysis. A higher rate of TIPS thrombosis was observed in those for whom the procedure was clinically unsuccessful (P=0.014). A significant increase in incidence of thrombosis was noted with increasing tertiles of post-TIPS portosystemic gradients (P value for trend=0.017). Conclusions Older age, lower BMI and higher post-TIPS portosystemic gradients were associated with higher hazards of shunt thrombosis after TIPS creation using stent grafts. Higher rates of shunt thrombosis were seen in patients for whom TIPS creation was clinically unsuccessful. The association between TIPS thrombosis and higher post-TIPS portosystemic gradients may indicate impaired flow through the shunt, a finding which may be technical or anatomic in nature and should be assessed before procedure completion.

Expectoration of an inferior vena cava filter strut.

Inferior vena cava (IVC) filter use is widespread in patients with venous thromboembolism (VTE) and temporary contraindication to anticoagulation, though timely removal is often not performed. We report the case of an expectoration of an IVC filter strut. Review of the patients prior imaging confirmed an infrarenal Bard G2 filter with an absent strut, which was visualised in the left lung base. The strut was presumed to have embolised to a pulmonary artery branch and eroded into an adjacent bronchus. Subsequent fluoroscopically guided filter retrieval was successful. The incidence of IVC filter fractures increases with longer dwell times. Filter fragment embolisation has resulted in major adverse events, including sudden death and cardiac tamponade. Recent evidence has suggested that retrieval of IVC filters with prolonged dwell times is feasible and safe. This report brings awareness to the range of complications with indwelling IVC filters, and highlights the importance of timely removal.

Advanced Techniques in Thoracic Endovascular Aortic Repair (TEVAR): Chimneys/Periscopes, Fenestrated Endografts, and Branched Devices ,,✰✰✰

Thoracic endovascular aortic repair (TEVAR) is a rapidly growing and improving technique for the management of thoracic aortic aneurysms, dissections, and traumatic aortic injury. These disease processes were previously treated exclusively by surgery, but TEVAR has substantially improved patient outcomes including reduced mortality, reduced paraplegia rate, and shorter hospital and/or intensive care unit stay compared to open surgery. TEVAR was initially isolated to the descending thoracic aorta, but the advent of advanced techniques has extended the scope to well beyond the left subclavian artery and into Zone 0. Recent techniques include chimney grafts, periscope grafts, in situ fenestrations, and physician modified grafts. In addition, commercial branched aortic devices are currently on trial and will likely vastly extend the off-the-shelf capabilities of TEVAR for aortic arch disease. This paper reviews the data, concepts, and technical aspects of current advanced TEVAR techniques, as well as ongoing clinical trials for thoracic branched aortic devices.