The relative risk of ischemic cerebrovascular accident in patients with Von Willebrand disease.

Cerebrovascular accident (CVA) is one of the leading causes of death in the United States. Von Willebrand factor plays an important role in platelet activation and adhesion. It remains unclear whether Von Willebrand disease (vWD) is associated with a decreased risk of developing CVA. The study aimed to compare the relative risk (RR) of CVA in patients with and without vWD. We queried the National Inpatient Sample from 2009 to 2014 for discharge data and records for vWD and CVA using International Classification of Diseases, Ninth-Revision codes. The unadjusted and adjusted RR of CVA in patients with and without vWD were estimated using log-binomial model. Descriptive measures including means, medians, standard deviations, and range were presented based on normality test of continuous data. The prevalence of CVA was lower in patients with vWD than in those without vWD (1.31% vs 2.04%), with a RR of 0.64 (95% confidence interval (CI): 0.60-0.68). After adjusting for common CVA risk factors, the RR remained lower in vWD patients: 0.81 (95% CI: 0.76-0.86). vWD is associated with a lower RR of developing CVA. This suggests that deficiency of Von Willebrand factor is potentially protective against the development of CVA. To the best of our knowledge, this is the first study in humans to compare the RR of CVA in patients with and without vWD. Future studies are needed to explore causal relationships and therapeutic benefits.

Surgical Management of Aortic Regurgitation in Takayasu's Arteritis: A Systematic Review of Techniques and Outcomes.

IntroductionTakayasu's arteritis (TA) is an inflammatory condition that affects large vessels and frequently involves the aortic valve causing valve regurgitation. Surgical management is recommended for symptomatic severe aortic regurgitation (AR); however, the optimal surgical approach is yet unclear. This study aims to review surgical treatment options for AR in TA and determine which procedure has a lower chance of late postoperative events and/or mortality. MethodsAn electronic database search was performed within PubMed, EMBASE, Web of Science, and SCOPUS to identify articles from 1975 to 2016 focusing on surgical management of the AR in TA. ResultsTwenty seven studies encompassing a total of 194 cases (77% females) were included. Isolated aortic valve replacement (AVR) was performed in 105/194 cases (54%) (Group A), while combined aortic valve and root replacement (CAVRR) was performed in 87/194 (45%) (Group B). Prosthetic valve detachment was reported in 10/105 cases (9.5%) in group A and 1/87 cases (1.2%) in group B (p = 0.02). Dilation of the residual aorta was reported in 10/105 cases (9.5%) in group A and 1/87 cases (1.2%) in group B (p = 0.02). Any late (≥ 30 d) postoperative cardiac event was reported in 26/105 cases (24.8%) in group A, and in 7/87 cases (8.1%) in group B (p = 0.003). ConclusionsAlthough CAVRR is a more complex procedure, it might offer a better outcome in terms of late postoperative cardiac events compared to isolated AVR procedure. Future prospective studies are required to help determine the best surgical approach in such a population.

The Cardioprotective Potential of von Willebrand Disease in Ischemic Heart Disease.

von Willebrand factor (vWF) aids coagulation at sites of vessel injury. Elevated vWF levels have been associated with an increased risk of ischemic heart disease (IHD); however, it is unclear whether vWF deficiency, seen in patients with von Willebrand disease (vWD), protects people against IHD. We determined and compared the prevalence and risk of IHD in patients with versus without vWD by using data from the National Inpatient Sample (2009-2014), excluding patients younger than 18 and older than 75 years. The primary outcome was the odds ratio (OR) of IHD in patients with versus without vWD. Secondary outcomes were major medical comorbidities and demographic characteristics in patients with vWD. Of 224,475,443 weighted hospital-discharge samples, we identified 82,809 patients with a vWD diagnosis. The odds of IHD were lower in patients with vWD than in those without (OR=0.54; 95% CI, 0.52-0.56). After multivariable logistic regression analysis and adjustment for age, sex, and typical IHD risk factors (hypertension, smoking, diabetes, hyperlipidemia, chronic kidney disease, obesity, and family history of IHD), the likelihood of IHD remained lower in patients with vWD than in patients without (OR=0.65; 95% CI, 0.63-0.67). Our study shows that vWF deficiency, as seen in patients with vWD, is associated with a decreased prevalence of IHD. Further investigation may confirm these findings.

Secundum Atrial Septal Defect Closure via the Transhepatic Approach in a Patient With Situs Ambiguus and a Left-Sided Inferior Vena Cava.

Secundum atrial septal defect (ASD) is the most common type of ASD. Symptoms including dyspnea on exertion usually manifest in the third and fourth decade of life. The transcatheter closure is the treatment of choice for secundum ASD. The transfemoral venous approach has been the mainstay. However, this approach can be challenging or impossible in patients with congenital absence or interruption of the inferior vena cava (IVC). The latter has been reported in patients with situs ambiguus and inversus. In this patient population, other forms of venous access such as the transjugular or transhepatic approach are used. We present a unique case of symptomatic secundum ASD in a patient who was incidentally found to have situs ambiguus with a left-sided intact IVC. An initial attempt at the ASD closure via the transfemoral approach was unsuccessful due to acute angulation. A repeat attempt was successful via the transhepatic approach with the guidance of real-time ultrasound, transesophageal echocardiogram, and the involvement of an interventional radiologist. The procedure was well tolerated without any complications. Repeat transthoracic echocardiogram with agitated saline the day after the procedure was negative for interatrial shunting.

Distinct Coagulopathy With Myocardial Injury and Pulmonary Embolism in COVID-19.

In this article, we report a case of a 61-year-old male who was diagnosed with SARS-CoV-2 (severe acute respiratory syndrome coronavirus 2), presenting with acute respiratory distress syndrome requiring intubation and hemodynamic support, marked D-Dimer and troponin I elevation, worsening ST-elevation myocardial infarction on repeat electrocardiograms, and a negative coronary angiogram ruling out a coronary artery thrombosis or occlusion. With worsening diffuse ST-segment elevation on electrocardiograms and reduced ejection fraction on echocardiography in the setting of systemic inflammation, fulminant myocarditis was highly suspected. Despite optimal medical treatment, the patient's condition deteriorated and was complicated by cardiac arrest that failed resuscitation. Although myocarditis was initially suspected, the autopsy revealed no evidence of myocarditis or pericarditis but did demonstrate multiple microscopic sites of myocardial ischemia together with thrombi in the left atrium and pulmonary vasculature. Additionally, scattered microscopic cardiomyocyte necrosis with pathological diagnosis of small vessel micro-thrombotic occlusions. These findings are potentially exacerbated by inflammation-induced coagulopathy, hypoxia, hypotension, and stress, that is, a multifactorial etiology. Further research and an improved understanding are needed to define the precise pathophysiology of the coagulopathic state causing widespread micro-thrombosis with subsequent myocardial and pulmonary injury.

A Unique Compensatory Mechanism for Total Pulmonary Vein Occlusion Post Atrial Fibrillation Catheter Ablation Visualized by Multimodality Imaging.

Pulmonary vein (PV) stenosis is a rare and serious complication of radiofrequency catheter ablation (RFCA) for atrial fibrillation. However, it can be asymptomatic or mildly symptomatic depending on the severity of the stenosis and the development of compensatory mechanisms. This study provides a detailed description and visualization of a unique type of venous collaterals that bypass the PV stenosis and drain directly in the left atrium alleviating PV stenosis sequelae. This study reports a case of a 61-year-old male who presented with mild dyspnea and fatigue 3 years post atrial fibrillation RFCA. After a thorough evaluation of the case, a redo-ablation was planned. As a part of the preablation workup, a transesophageal echocardiography (TEE), a ventilation-perfusion (V/Q) scan of the lungs, and a chest computed tomography angiogram (CTA) were performed. The TEE revealed total obstruction of the left superior PV, with no color Doppler flow detected. It also showed evidence of multiple collateral flows at the os of the left superior PV. The V/Q scan showed a large perfusion defect involving the entire left upper lobe consistent with a compromised left upper PV flow. The CTA with 3D volume rendering revealed the total occlusion of the left superior PV at its ostium. Moreover, the scan confirmed the pulmonary venous drainage via small collateral channels that was suggested by the TEE.

An Unusual Case of Neisseria flavescens/subflava Group Tricuspid Valve Endocarditis in a Patient With Previously Treated Methicillin-Resistant Staphylococcus aureus Endocarditis.

Infective endocarditis (IE) is classified as an infection of any cardiac valve or endocardial surface and is often associated with high morbidity and mortality. Certain bacteria such as gram-positive cocci and viridans group streptococci have high predilection for both naïve and damaged cardiac valvular tissues, accounting for the majority of IE cases. In very infrequent instances, gram-negative bacteria (GNB), more specifically non-meningococcal, non-gonococcal GNB, have been shown to cause IE. The following is a case of a young male diagnosed with Neisseria flavescens/subflava tricuspid valve endocarditis after being previously treated for Methicillin-resistant Staphylococcus aureus (MRSA) endocarditis.

Multimodality Imaging Demonstrating an Apical Variant Hypertrophic Cardiomyopathy in an Uncommon Pentad.

A 79-year-old man was admitted for a transcatheter aortic valve replacement due to severe aortic stenosis. A preoperative chest computed tomography with angiography revealed an apical variant hypertrophic cardiomyopathy with a prominent apical pouch. In addition, there was near-complete obliteration of the left ventricle in the mid to apical aspect during systole suggesting a midventricular gradient. Postoperative transthoracic echocardiography confirmed the apical variant hypertrophic cardiomyopathy with an apical aneurysm and a gradient with a peak velocity of 2 m/s, and mid-cavitary gradient with a peak velocity of 3 m/s. It also revealed a fusiform aneurysmal dilatation of the ascending aorta.

Pericardial Effusion and Tamponade Complicating Acute Pericarditis in a Human Metapneumovirus-Infected Adult: A Unique Case Report.

Acute pericarditis (AP), or pericardial sac inflammation, is a self-limited condition in healthy individuals. Viruses, including adenoviruses, enteroviruses, cytomegalovirus, and influenza virus, have been well documented to cause AP. In contrast, human metapneumovirus (hMPV), a relatively newer virus, has been described in a few cases to cause serious cardiac complications. Here we report a patient who developed an imminent cardiac tamponade associated with hMVP respiratory infection.