Coronary perfusion pressure is associated with adverse outcomes in advanced heart failure.

BACKGROUND: Myocardial perfusion is an important determinant of cardiac function. We hypothesized that low coronary perfusion pressure (CPP) would be associated with adverse outcomes in heart failure. Myocardial perfusion impacts the contractile efficiency thus a low CPP would signal low myocardial perfusion in the face of increased cardiac demand as a result of volume overload. METHODS: We analyzed patients with complete hemodynamic data in the Evaluation Study of Congestive Heart Failure and Pulmonary Artery Catheterization Effectiveness trial using Cox Proportional Hazards regression for the primary outcome of the composite risk of death, heart transplantation, or left ventricular assist device [(LVAD). DT × LVAD] and the secondary outcome of the composite risk of DT × LVAD and heart failure hospitalization (DT × LVADHF). CPP was calculated as the difference between diastolic blood pressure and pulmonary artery wedge pressure. Heart failure categories (ischemic vs non-ischemic) were also stratified based on CPP strata. RESULTS: The 158 patients (56.7 ± 13.6 years, 28.5% female) studied had a median CPP of 40 mmHg (IQR 35-52 mmHg). During 6 months of follow-up, 35 (22.2%) had the composite primary outcome and 109 (69.0%) had the composite secondary outcome. When these outcomes were then stratified based on the median, CPP was associated with these outcomes. Increasing CPP was associated with lower risk of both the primary outcome of DT × LVAD (HR 0.96, 95% CI 0.94-0.99 p = .002) and as well as the secondary outcome of DT × LVADHF (p = .0008) There was significant interaction between CPP and ischemic etiology (p = .04). CONCLUSION: A low coronary artery perfusion pressure below (median) 40mmHg in patients with advanced heart failure undergoing invasive hemodynamic monitoring with a pulmonary artery catheter was associated with adverse outcomes. CPP could useful in guiding risk stratification of advanced heart failure patients and timely evaluation of advanced heart failure therapies.

A systemic congestive index (systemic pulse pressure to central venous pressure ratio) predicts adverse outcomes in patients undergoing valvular heart surgery.

BACKGROUND AND AIMS: Invasive hemodynamics may provide a more nuanced assessment of cardiac function and risk phenotyping in patients undergoing cardiac surgery. The systemic pulse pressure (SPP) to central venous pressure (CVP) ratio represents an integrated index of right and left ventricular function and thus may demonstrate an association with valvular heart surgery outcomes. This study hypothesized that a low SPP/CVP ratio would be associated with mortality in valvular surgery patients. METHODS: This retrospective cohort study examined adult valvular surgery patients with preoperative right heart catheterization from 2007 through 2016 at a single tertiary medical center (n = 215). Associations between the SPP/CVP ratio and mortality were investigated with univariate and multivariate analyses. RESULTS: Among 215 patients (age 69.7 ± 12.4 years; 55.8% male), 61 died (28.4%) over a median follow-up of 5.9 years. A SPP/CVP ratio <7.6 was associated with increased mortality (relative risk 1.70, 95% confidence interval [CI] 1.08-2.67, p = .019) and increased length of stay (11.56 ± 13.73 days vs. 7.93 ± 4.92 days, p = .016). It remained an independent predictor of mortality (adjusted odds ratio 3.99, 95% CI 1.47-11.45, p = .008) after adjusting for CVP, mean pulmonary artery pressure, aortic stenosis, tricuspid regurgitation, smoking status, diabetes mellitus, dialysis, and cross-clamp time. CONCLUSIONS: A low SPP/CVP ratio was associated with worse outcomes in patients undergoing valvular heart surgery. This metric has potential utility in preoperative risk stratification to guide patient selection, prognosis, and surgical outcomes.

Echocardiographic derived pulmonary artery wedge pressure is associated with mortality, heart hospitalizations, and functional capacity in chronic systolic heart failure: insights from the HF-ACTION trial.

BACKGROUND: Heart Failure (HF) is associated with increased morbidity and mortality. Identification of patients at risk for adverse events could lead to improved outcomes. Few studies address the association of echocardiographic-derived PAWP with exercise capacity, readmissions, and mortality in HF. METHODS: HF-ACTION enrolled 2331 outpatients with HF with reduced ejection fraction (HFrEF) who were randomized to aerobic exercise training versus usual care. All patients underwent baseline echocardiography. Echocardiographic-derived PAWP (ePAWP) was assessed using the Nagueh formula. We evaluated the relationship between ePAWP to clinical outcomes. RESULTS: Among the 2331 patients in the HF-ACTION trial, 2125 patients consented and completed follow-up with available data. 807 of these patients had complete echocardiographic data that allowed the calculation of ePAWP. Of this cohort, mean age (SD) was 58 years (12.7), and 255 (31.6%) were female. The median ePAWP was 14.06 mmHg. ePAWP was significantly associated with cardiovascular death or HF hospitalization (Hazard ratio [HR] 1.02, coefficient 0.016, CI 1.002-1.030, p = 0.022) and all-cause death or HF hospitalization (HR 1.01, coefficient 0.010, CI 1.001-1.020, p = 0.04). Increased ePAWP was also associated with decreased exercise capacity leading to lower peak VO2 (p = < 0.001), high Ve/VCO2 slope (p = < 0.001), lower exercise duration (p = < 0.001), oxygen uptake efficiency (p = < 0.001), and shorter 6-MWT distance (p = < 0.001). CONCLUSIONS: Among HFrEF patients, echocardiographic-derived PAWP was associated with increased mortality, reduced functional capacity and heart failure hospitalization. ePAWP may be a viable noninvasive marker to risk stratify HFrEF patients.

A case-report of successful use of thromboelastography to manage anticoagulation therapy in a patient with concomitant catastrophic antiphospholipid syndrome and heparin-induced thrombocytopenia.

: Catastrophic antiphospholipid syndrome (CAPS) is a severe but rare form of antiphospholipid syndrome (APS) that results in multiple thrombosis of multiple organs within a week . Similarly, heparin-induced thrombocytopenia (HIT) has been associated with severe and life-threatening thrombosis, both conditions mediated by an autoimmune disorder resulting in a highly thrombotic state . Both conditions requiring aggressive therapeutic anticoagulation when coinciding CAPS complicated by HIT presents a therapeutic challenge. Current recommendations advocate for the use of anti-Xa activity monitoring in the setting of APS because of the common laboratory interaction with commercially available tests, such as prothrombin and activated partial thromboplastin times . With the recommendations to utilize direct thrombin inhibitors (DTI) in the presence of HIT this precludes the possibility of anti-Xa monitoring making for a monitoring predicament. The current case presents a patient where thromboelastography (TEG) was utilized to direct anticoagulation in the setting of concurrent CAPS and HIT without complication.

Coexistent Ipsilateral Internal Carotid Artery Occlusion and Cerebral Venous Thrombosis in Hepatitis C.

A 58-year-old male, known to have hepatitis C virus (HCV), presented with intermittent headaches and left-sided sensorimotor symptoms. There were no focal neurological deficits on examination. Electrocardiogram was unremarkable. Computed tomography angiography head and neck displayed extracranial right internal carotid artery occlusion. Magnetic resonance imaging showed right cortical vein thrombosis, with hemorrhagic infarction. Echocardiography with bubble study was unremarkable. Hypercoagulable workup was significant for protein S deficiency. He was treated with warfarin for 6 months. Repeat protein S levels remained low 9 months later. The coexistence of arterial and venous thrombotic events gives rise to a limited differential. In this case, it may be related to chronic HCV infection. The underlying pathogenesis is not clear; however, it is possible the patient had chronic high-grade internal carotid artery stenosis, which occluded leading to his presenting symptoms. The cortical vein thrombosis is likely an incidental finding here. The extent by which HCV contributed to the cerebral thrombosis and carotid artery occlusion in our case is not clear; however, the hypercoagulable and atherosclerotic properties of the virus cannot be disregarded. The virus can promote carotid atherosclerosis and cerebral venous thrombosis as well as other venous and arterial thromboembolic events. Furthermore, HCV is associated with impaired venous flow and procoagulant properties, which can fuel a hypercoagulable state. Also of note cirrhosis is associated with protein S deficiency. We recommend considering an underlying hypercoagulable state including both arterial and venous thrombosis in HCV infection.

Immune Thrombocytopenia as a Consequence of Rocky Mountain Spotted Fever.

Primary immune thrombocytopenia (ITP) - also called idiopathic thrombocytopenic purpura or immune thrombocytopenic purpura - is an acquired thrombocytopenia caused by autoantibodies against platelet antigens. It is one of the more common causes of thrombocytopenia in otherwise asymptomatic adults. Rocky Mountain spotted fever (RMSF) is a potentially lethal, but curable, tick-borne disease. We present a case of ITP that was triggered by RMSF.

Bilateral Brachial Artery Disease Presenting with Features of Raynaud's Phenomenon: A Case Report and Review of the Literature.

OBJECTIVE: To present a case of bilateral brachial artery disease presenting with features of Raynaud's phenomenon which was successfully treated with angioplasty and stenting, together with a review of the relevant literature. CASE: A 71-year-old female presented with a one-year history of intermittent pallor of both hands precipitated with cold objects. On examination, bilateral radial pulses were reduced. Prior photos showed pallor of the distal aspect of both palms. Angiogram showed high grade stenosis of the right brachial artery and focal occlusion with likely dissection of the left brachial artery. She underwent angioplasty and stenting for both lesions. She was asymptomatic without further episodes of Raynaud's phenomenon after five months on dual antiplatelet therapy. Upper-extremity vascular stenosis is uncommon. Structural changes in the vessel wall can cause vasospastic attacks, a mechanism described in secondary Raynaud's phenomenon. We hypothesize that these attacks may have been precipitated by the bilateral brachial artery disease. Furthermore, resolution of the symptoms after stent further supports our theory. CONCLUSION: Bilateral brachial artery disease is uncommon. Physicians should consider this in patients presenting with Raynaud's phenomenon. Brachial artery stenosis and occlusion is a treatable disease with good symptomatic outcomes after angioplasty and stenting.

Rapid Onset of B12 Deficiency in the Setting of Worsening Multiple Myeloma: Correlations between B12 Deficiency and Multiple Myeloma.

A 67-year-old female with a relapse of multiple myeloma after being in remission for approximately 2 years following autologous stem cell transplant presented with worsening pancytopenia, over a three-month period. There were an increase in her monoclonal spike at 3.13 g/dL on serum protein electrophoresis, low serum B12 levels, and positive intrinsic factor antibodies. Three months before, she had normal B12 levels and a significantly lower monoclonal spike of 1.07 g/dL. She was diagnosed with B12 deficiency with pernicious anaemia in the setting of her worsening myeloma. Multiple myeloma (MM) has been linked with B12 deficiency and pernicious anaemia. Several mechanisms have been described regarding the pathogenesis of B12 deficiency in such patients. Increased tumour activity can further perpetuate the development of B12 deficiency in such patients. With regard to our case, the increase in tumour activity and onset of pernicious anaemia could have contributed to the rapid development of B12 deficiency. In contrast to this, rapid development of B12 deficiency could also signify relapse or worsening of the myeloma as seen in our case. Physicians ought to consider B12 deficiency in patients with worsening pancytopenia and myeloma.

Mycobacteria Bovis osteomyelitis following intravesical BCG for bladder cancer.

Mycobacteria Bovis osteomyelitis is a rare adverse effect after Bacillus Calmette-Guerin (BCG) intravesical therapy. A 62-year-old male presented with acute spinal cord compression three months after completing his second course of therapy for bladder cancer. The first course with intravesical BCG was complicated with an episode of hematuria. He reported intermittent subjective fever for 3 weeks thereafter which resolved with Tylenol. Interferon-α2 B was added to the second cycle of intravesical BCG with the indication here being residual tumor, and was tolerated well. His complete blood count and liver function tests were unremarkable on admission. MRI showed features of osteomyelitis with cord compression at T4/T5. Biopsy of the affected bone showed caseating granuloma which was positive for acid fact bacilli, later confirmed to be Mycobacterium Bovis by PCR and pyrazinamide resistance. He was started on intravenous steroids and underwent spinal cord decompression. Rifampin, Isoniazid, and Ethambutol were then commenced. His weakness improved and after two months of therapy he was asymptomatic and back to his baseline function. Osteomyelitis is a rare but serious complication. Early diagnosis and treatment is important as the outcomes are good.

An Aggressive Form of Langerhan Cell Histiocytosis in an Adult: Therapeutic Challenges.

Langerhans cell histiocytosis (LCH) is rare in adults. Regular follow-up is mandatory due to reoccurrence. A 35-year-old male with an incidental left iliac bone lesion was diagnosed with LCH. He later became symptomatic with hip pain and spread of the disease. Despite excision of the symptomatic iliac lesion, he had progression while on cytarabine and nivolumab, evidenced by increased bone pain and involvement of other bones on imaging. He underwent excision of the jaw lesion followed by vinblastine; he was pain free and had stable disease on PET imaging after 3 months. LCH is an uncommon neoplasia. Treatment is reserved for symptomatic patients while asymptomatic patients are observed. Follow-up is imperative due to the risk of reoccurrence. Despite surgical treatment together with one of the front-line agents for refractory disease, in this case cytarabine, he still had progression of the disease. Furthermore, the trial of nivolumab was of no benefit. This case highlights good response to vinblastine which is previously reported to have good success. No trials are published, and the optimal strategy has yet to be defined. LCH with multiple bony involvement can be aggressive and therapeutically challenging.

Legionnaires' Disease Complicated with Rhabdomyolysis and Acute Kidney Injury in an AIDS Patient.

OBJECTIVE: To present a case of an uncommon triad of Legionella pneumonia, rhabdomyolysis, and renal failure, with review of the relevant literature. CASE: A 51-year-old with a history of human immunodeficiency virus (HIV), chronic obstructive pulmonary disease (COPD), and hypertension presented with fever, cough, and shortness of breath over four days. Chest X-ray showed consolidation in left lower lung field; urine was positive for Legionella antigen and myoglobin; creatine kinase was 51092U/L; creatine was 6.9 mg/dL, and his CD4 count was 41 cells/ul. He was managed with azithromycin and levofloxacin and further required dialysis and ventilatory support in the intensive care unit due to renal failure and respiratory failure. He responded well to the treatment and made a complete recovery. Legionella pneumophila infection is a recognized but rare cause of rhabdomyolysis with high morbidity and mortality when there is extrapulmonary involvement. Early diagnosis and appropriate treatment is essential to improve outcomes. CONCLUSION: Physicians should consider Legionella pneumonia in patients with rhabdomyolysis, renal failure, and respiratory symptoms. Early diagnosis and treatment have been shown to have good clinical response. Timely intensive care management, together with early and judicious use of dialysis in patients complicated with rhabdomyolysis and renal failure, may lead to good outcomes.

A Rare Report of Infectious Emphysematous Aortitis Secondary to Clostridium septicum without Prior Vascular Intervention.

The term "mycotic aneurysm" was first used by Osler in 1882 to describe a mushroom-shaped aneurysm in subacute bacterial endocarditis. Mycotic aneurysms account for only 2.6% of all aneurysms of the aorta. Rarer still are anaerobic infections secondary to organisms such as Clostridium septicum, which results in emphysematous aortitis. The vast majority of emphysematous aortic infections occur as a result of instrumentation; however, in this case we present an infection de novo. A 75-year-old male presented with a 2-week history of progressive fatigue and chest pain that then developed into constitutional symptoms. Chest radiograph demonstrated an obvious widened mediastinum. CT angiogram of his chest then confirmed this finding as well as significant periaortic gas and focal outpouching. Numerous diverticuli with inflammatory changes consistent with diverticulitis was observed on CT abdomen. Blood cultures returned positive for Clostridium septicum. Definitive treatment was discussed including debridement and graft insertion; however, patient decided on conservative management and was discharged on intravenous antibiotics. Unfortunately, as in most cases of emphysematous aortitis that do not undergo surgical management, the patient succumbed to his illness. The lesson provided will be the epidemiology of emphysematous aortitis, presentation, diagnosis, management, and prognosis through a case report.

A case of disseminated intravascular coagulation secondary to Acinetobacter lwoffii and Acinetobacter baumannii bacteremia.

Bacteremia is currently one of the infections with the highest mortality in hospitals [1]. Acinetobacter lwoffii and Acinetobacter baumannii are gram-negative bacteria and both represent opportunistic pathogens. In certain cases, the management can be challenging since these organisms can be highly resistant to antimicrobial agents. Clinical illnesses associated with Acinetobacter include pneumonia, meningitis, peritonitis, endocarditis and infections of the urinary tract and skin [1]. Acinetobacter bacteremia represents a serious and ever increasing problem because of the high associated morbidity and mortality.