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.

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.

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.

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.

Introduction: Von Willebrand factor (vWF) plays an important role in the coagulation process at the site of vascular injury. It is unclear whether vWF deficiency is protective against cardiovascular disease (CVD) since investigations are limited in this entity. Hypothesis: Our study assessed the hypothesis that the prevalence of CVD is less in patients with than without Von Willebrand disease (vWD). Methods: We queried the National Inpatient Sample (NIS) from 2009-2014. Entire data was used with exclusion of patients below 18 and over 75-year-old. We defined cardiovascular disease as any event of myocardial infarction or a diagnosis of ischemic heart disease using the International Classification of Disease, 9th edition (ICD-9). The primary outcome of interest was to compare the odds ratio of CVD in patients with and without vWD. Results: A total of 224,475,443 weighted hospital discharge samples were identified. Of these, 82,809 carried a diagnosis of vWD. The odds of CVD were less common in vWD patients (OR 0.54; 95% CI 0.52 - 0.56) (table 1). After multivariate logistic regression analysis, with adjustment for age, gender and the most common CVD risk factors including hypertension, smoking, diabetes, hyperlipidemia, family history of CVD, chronic kidney disease and obesity the likelihood of CVD remained lower in vWD patients (OR 0.65; 95% CI 0.63-0.67) (table 2). Conclusions: Our study, the first to involve a large cohort of patients, demonstrated that vWF deficiency is associated with decreased prevalence of CVD. Hence, vWF could potentially be a therapeutic target in CVD management. More investigations are needed for confirmation of these findings and to determine causation.