A systematic review supporting the Society for Vascular Surgery guidelines on the management of heritable aortopathies.

BACKGROUND: To support the development of clinical practice guidelines on the management of patients with genetic aortopathies and arteriopathies, a writing committee from the Society for Vascular Surgery has commissioned this systematic review. METHODS: We conducted a systematic review and searched multiple databases for studies addressing six questions identified by the Society for Vascular Surgery guideline committee about evaluating and managing patients with genetic aortopathies and arteriopathies. Studies were selected and appraised by pairs of independent reviewers. RESULTS: We included 12 studies in this systematic review. We did not identify studies about the long-term outcomes of endovascular repair for aortic aneurysm in patients with heritable aortopathy or about new aortic events in pregnant women with a history of aortic dissection (AD) or aneurysm. A small case series demonstrated a 100% survival rate and 100% aortic intervention-free survival at 15 months (range, 7-28 months) after endograft repair for type B AD. A positive genetic diagnosis was discovered in 36% of patients with aortic aneurysms and dissections who had no risk factors for hereditary aortopathies, and these patients had a mortality rate of 11% at a median follow-up duration of 5 months. Black patients had lower 30-day mortality than White patients (5.6% vs 9.0%, respectively), but they had a higher overall aortic reintervention rate at 30 days after AD repair (47% vs 27%, respectively). Aortic reinterventions owing to aneurysmal expansion and endoleak at 30 days were higher in Black patients than White patients. The certainty of evidence was judged to be very low across all the outcomes evaluated in this systematic review. CONCLUSIONS: The available evidence suggests high survival after thoracic endovascular aortic repair for type B AD in young patients with heritable aortopathies, but with limited long-term follow-up. Genetic testing in patients with acute aortic aneurysms and dissections had a high yield. It was positive for most patients with risk factors for hereditary aortopathies and in more than one-third for all other patients, and was associated with new aortic events within 15 years.

Inpatient Outcomes of Gastric Antral Vascular Ectasia in Those With Aortic Stenosis: A Retrospective Study of 85,000 Hospitalizations.

GOALS: To investigate the outcomes of hospitalized patients with gastric antral vascular ectasia (GAVE) in the setting of aortic stenosis (AS). BACKGROUND: Although AS is associated with gastrointestinal arteriovenous malformations, its association with GAVE, a rare cause of upper gastrointestinal bleeding, remains unknown. STUDY: The National Inpatient Sample database from the years 2016 to 2019 was searched for patients admitted with a diagnosis of GAVE, with and without a history of AS. Univariate and multivariate logistic regression analysis was performed to determine the risk of mortality and in-hospital complications in the GAVE/AS group compared with the GAVE-only group. RESULTS: Patients with AS had a 2-fold increase in the risk of GAVE [odds ratio (OR): 2.08, P < 0.001], with no statistically significant difference in inpatient mortality between the study groups (OR: 1.36, P = 0.268). Patients with GAVE-AS had a higher risk of hypovolemic shock (OR: 2.00, P = 0.001) and acute coronary syndromes (OR: 2.25, P < 0.001) with no difference in risk of cardiogenic shock (P = 0.695), acute kidney injury (P = 0.550), blood transfusion (P = 0.270), sepsis (P = 0.598), respiratory failure (P = 0.200), or in-hospital cardiac arrest (P = 0.638). The cost of care in patients with GAVE-AS was increased by a mean of $4729 (P = 0.022), with no increase in length of stay (P = 0.320) when compared with patients with GAVE-only. CONCLUSIONS: Patients with AS have a 2-fold increase in the risk of development of GAVE. Patients with AS admitted for GAVE-related bleeding are at higher rates of hypovolemic shock, acute coronary syndrome, and higher resource utilization when compared with admitted patients with GAVE without AS.

Secondary Prophylaxis of Gastric Variceal Bleeding: A Systematic Review and Network Meta-Analysis.

There is no clear consensus regarding the optimal approach for secondary prophylaxis of gastric variceal bleeding (GVB) in patients with cirrhosis. We conducted a systematic review and network meta-analysis (NMA) to compare the efficacy of available treatments. A comprehensive search of several databases from each database's inception to March 23, 2021, was conducted to identify relevant randomized controlled trials (RCTs). Outcomes of interest were rebleeding and mortality. Results were expressed as relative risk (RR) and 95% confidence interval (CI). We followed the Grading of Recommendations Assessment, Development, and Evaluation approach to rate the certainty of evidence. We included 9 RCTs with 647 patients who had histories of GVB and follow-ups >6 weeks. A total of 9 interventions were included in the NMA. Balloon-occluded retrograde transvenous obliteration (BRTO) was associated with a lower risk of rebleeding when compared with ß-blockers (RR, 0.04; 95% CI, 0.01-0.26; low certainty), and endoscopic injection sclerotherapy (EIS)-cyanoacrylate (CYA) (RR, 0.18; 95% CI, 0.04-0.77; low certainty). ß-blockers were associated with a higher risk of rebleeding compared with most interventions and with increased mortality compared with EIS-CYA (RR, 4.12, 95% CI, 1.50-11.36; low certainty), and EIS-CYA + nonselective ß-blockers (RR, 5.61; 95% CI, 1.91-16.43; low certainty). Analysis based on indirect comparisons suggests that BRTO may be the best intervention in preventing rebleeding, whereas ß-blocker monotherapy is likely the worst in preventing rebleeding and mortality. Head-to-head RCTs are needed to validate these results.

Bilateral Nephrectomy, the Forgotten Measure in the Treatment of Refractory Hypertension in Patients With End-Stage Renal Disease: A Case Report and Literature Review.

It's not uncommon for patients with end-stage renal disease (ESRD) to develop hypertension that is resistant to antihypertensive medications and volume control, making it a challenge to control blood pressure in those patients. In this article, we present a 71-year-old female with a history of ESRD on intermittent hemodialysis (IHD), who developed refractory hypertension despite the use of seven antihypertensive agents in addition to IHD. The patient underwent bilateral nephrectomy as a last resort therapy for managing resistant hypertension, which led to a significant improvement in blood pressure (BP) and decreasing the number and doses of antihypertensive agents. This article aims to raise the awareness and alertness of clinicians to the efficacy of bilateral nephrectomy as rescue therapy for refractory hypertension in hemodialysis patients.

Moyamoya Disease in a Middle-Aged Hispanic Woman: A Case Illustration.

Moyamoya disease is a rare cerebrovascular disease of unknown etiology, once known to be rare in the United States as compared to East-Asian countries, it is now an increasingly recognized cause of strokes in the United States, as the prevalence of the disease appears to be increasing. We describe a case of a 41-year-old Hispanic female patient presenting to our hospital with a stroke. She had two episodes of right arm weakness and clumsiness prior to presentation to the hospital that had resolved upon arrival. Despite a CT head negative for stroke, further imaging work-up was performed including MRI of the brain with magnetic resonance angiography (MRA) and conventional angiogram, which showed characteristic imaging findings leading to the diagnosis of Moyamoya disease. The patient subsequently underwent elective surgical intervention with Encephaloduroarteriosynangiosis (EDAS) procedure to prevent further complications.

Critical Finding of Wellens' Syndrome in a Patient who Presented With a Fibular Fracture.

Diagnosing myocardial infarction is not always straightforward; there are many insidious presentations that can be overlooked resulting in deadly consequences. We present a 76-year-old male who came to the ED complaining of right ankle pain. A routine electrocardiogram (ECG) done was noted to have biphasic T waves in leads V2 and V3 which was characteristic of Wellens' syndrome. Subsequent workup showed an increase in troponin T levels in a chest pain-free setting. The patient underwent urgent cardiac catheterization which showed significant triple vessel coronary artery disease, with 90% proximal occlusion of the left anterior descending artery, eventually requiring coronary artery bypass grafting (CABG). Timely diagnosis and management prevented serious consequences of his underlying severe coronary artery disease.