Mortality Risk Assessment Using the REVEAL 2.0 Score in Pulmonary Hypertension Secondary to Left Heart Disease.

Background: Pulmonary hypertension (PH) frequently complicates the course of patients with left heart disease (PH-LHD) and is associated with worse clinical outcomes. Mortality calculators for PH-LHD are lacking, and it is unclear whether any risk prediction tools originally derived from other forms of PH can accurately predict outcomes in patients with PH-LHD. Methods: We retrospectively analyzed data from 161 patients diagnosed with PH-LHD referred to our pulmonary hypertension center from 2016 to 2022. We calculated the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL 2.0) risk score and categorized patients as low, intermediate, or high-risk. We assessed survival at 1 and 3 years using Kaplan-Meier and Cox proportional hazards, as well as classification performance using a concordance index. Results: At the first outpatient visit, 15% of patients were stratified as low-risk, 27% as intermediate, and 57% as high-risk. Cumulative 1-year survival rates were 100%, 94%, and 91% for the low, intermediate, and high-risk strata, respectively. Cumulative 3-year survival rates were 96%, 89%, and 70% for the low, intermediate, and high-risk strata, respectively. We found no difference in outcomes at 1 year between risk groups. High-risk patients had an increased risk of death at 3 years using REVEAL 2.0 (HR 5.32, p < 0.001). However, while REVEAL 2.0 accurately discriminated high-risk patients, the hazard ratio was not statistically different between patients classified as intermediate-risk compared to low-risk. Conclusion: REVEAL 2.0 accurately predicted 3-year survival in PH-LHD patients with high-risk features. However, the mortality risk between patients classified as intermediate-risk was not different from the low-risk stratum, suggesting inaccurate classification for this group of patients.

Effects of COVID-19 pandemic on the management of pulmonary hypertension.

The coronavirus of 2019 (COVID-19) disrupted delivery of healthcare. Patients with pulmonary hypertension (PH), especially pulmonary arterial hypertension (PAH), require significant resources for both diagnosis and management and are at high risk for decompensation due to disruption in their care. A survey consisting of 47 questions related to the care of patients with PH was designed by the American College of Chest Physicians 2020-2021 Pulmonary Vascular Disease (PVD) NetWork Steering Committee and sent to all members of the PVD NetWork, as well as the multiple other professional networks for PH. Participation was voluntary and anonymous. Responses were collected from November 2020 through February 2021. Ninety-five providers responded to this survey. The majority (93%) believe that care of PH patients has been affected by the pandemic. Sixty-seven percent observed decreased referrals for PH evaluation. Prior to the pandemic, only 15% used telemedicine for management of PH patients compared to 84% during the pandemic. Telemedicine was used most for follow up of selected low-risk patients (49%). While 22% respondents were completely willing to prescribe new PAH therapy via telemedicine, 11% respondents were completely unwilling. Comfort levels differed based on type of medication being prescribed. Over 90% of providers experienced disruptions in obtaining testing and 31% experienced disruptions in renewal or approval of medications. Overall, providers perceived that the COVID-19 pandemic caused significant disruption of care for PH patients. Telemedicine utilization increased but was used mostly in low-risk patients. Some providers had a decreased level of comfort prescribing PAH therapy via telemedicine encounters.

Utilization of risk assessment tools in management of PAH: A PAH provider survey.

Pulmonary arterial hypertension (PAH) is a chronically progressive fatal disease. A goal-oriented approach to achieve low risk status has been associated with improved survival. A variety of risk stratification tools are available, but use is low. We conducted a survey to assess potential reasons for under-utilization. We conducted a survey-based study of global PAH disease specialists with a goal of assessing risk assessment utilization and identifying modifiable barriers to use. The survey was designed by the American College of Chest Physicians' Pulmonary Vascular Diseases (PVD) NetWork. Respondents were global members of the PVD NetWork and Pulmonary Hypertension Association. Survey invitations were sent electronically to all members. Participation was anonymous and no provider or patient level data was collected. Participants from four countries responded with the majority (84%) being from the United States. Our survey found suboptimal use of any risk stratification tool with 71/112 (63%) reporting use. A total of 85% of the respondents had more than 5 years of experience in managing PAH. REVEAL 2.0 and European Society of Cardiology/European Respiratory Society risk tools were the most commonly used. A total of 44 (65%) surveyed felt that use of risk tools led to change in PAH therapies. Only 6 (9%) felt they prompted additional testing or changed the frequency of follow-up. A total of 5 (7%) reported they prompted goals of care/palliative care discussions and 2 (3%) that they triggered lung transplant referral. The vast majority indicated that incorporation of risk tools into electronic medical records (EMR) would improve utilization. PAH risk assessment tools remain under-utilized. Most respondents were experienced PAH clinicians. More than one-third were not routinely using risk tools. Most felt that risk tools led to PAH therapy changes but few reported impacts on other aspects of care. The most commonly identified barriers to use were time constraints and lack of integration with EMR.

A 57-Year-Old Man With Stridor and Critical Tracheal Narrowing.

CASE PRESENTATION: A 57-year-old man with a history of polysubstance use presented with shortness of breath, wheezing, productive cough, subjective fever, and chills of 3-day duration. Additionally, he reported worsening shortness of breath for the last 3 months. Of note, the patient was reported to have had, in the previous 6 months, two episodes of pneumonia that was treated with antibiotics and steroids. He was also diagnosed several years prior with adult-onset asthma due to intermittent wheezing and was prescribed an albuterol inhaler. The albuterol did not help relieve his wheezing, and he stopped refilling it.

A 58-Year-Old Man With Acute Encephalopathy, Fever, and Multi-Organ Dysfunction.

CASE PRESENTATION: A 58-year-old man with a medical history of type 2 diabetes mellitus and COPD presented with fever, chills, nausea, vomiting, left upper quadrant abdominal pain, and altered mental status for the past week. His mental status rapidly deteriorated and work of breathing increased, which required intubation and mechanical ventilation. The patient's wife reported recent exposure to tick bites after finding several ticks while changing the sheets in their bedroom.

Case report: Re-emerging significance of surgical embolectomy in pulmonary embolism.

INTRODUCTION: Massive pulmonary embolus (PE) is associated with a high mortality if not treated aggressively. Treatment classically includes thrombolysis, catheter embolectomy, or open surgical embolectomy. This is the case report of a 38-year-old female presenting with massive PE three weeks status post gastric sleeve resection. PRESENTATION OF CASE: 38-year-old female status post gastric sleeve resection presented to the emergency department with acute onset shortness of breath and dizziness. Computed Tomography (CT) chest angiography showed extensive PE with pulmonary artery saddle embolus, and an enlarged right ventricle suggesting strain. Her treatment consisted of anticoagulation, AngioVac suction embolectomy, EKOS catheter thrombolysis, fragmentation with catheter, extracorporeal membrane oxygenation (ECMO), and lastly surgical embolectomy due to refractory clinical course. DISCUSSION: This case report details the natural history of a complex massive pulmonary embolism presentation requiring multiple catheter-based measures, ECMO initiation, and subsequent surgical embolectomy. CONCLUSION: This case report should serve as encouragement for early adoption of surgical therapy in pulmonary embolism cases where patients present with a complex presentation.

Dysphonia and dyspnea in idiopathic hypereosinophilic syndrome treated with Mepolizumab.

Hypereosinophilic syndrome (HES) is characterized by a persistently elevated eosinophil count associated with eosinophil-related end-organ damage and thromboembolic events, in the absence of an identifiable cause. We present a case of idiopathic HES with evidence of peripheral and tissue eosinophilia while on high dose prednisone, associated with muscle tension dysphonia, colitis, and jackhammer esophagus. The patient was treated with the interleukin-5 inhibitor, Mepolizumab, with resolution of symptoms including dyspnea, diarrhea and dysphonia.

Accuracy and the Proper Use of the Point-of-Care Analysis in Hemoglobin A1C Measurement.

BACKGROUND: Diabetes Mellitus is one of the most common chronic diseases in the United States and worldwide. In 2009 the American Diabetes Association (ADA) recommended using A1C greater than 6.5% to diagnose diabetes. Hemoglobin A1C can be measured by laboratory and Point-of-Care (POC) methods. It is important to compare results obtained by the two modalities to determine the proper use of these tests. METHODS: One hundred fifteen patients were identified to have both POC and laboratory A1C values obtained on the same day, between August 2013 and August 2014. The differences were analyzed and the average difference and coefficient of determination (R2) was calculated. In addition, a Bland-Altman plot was generated; Kappa statistic calculated. We also computed the sensitivity and specificity of the POC method in diagnosing diabetes while using laboratory analysis as the gold standard. RESULTS: The differences between the two measurements ranged from 0 to 3.2 with an average difference of 0.2% of A1C. The Kappa statistic is 0.761 and R2 was 0.938. Laboratory results were greater than POC in 84% of cases. Interestingly, the Bland-Altman graph also indicates that the variability between the two sets of data increases with higher values of A1C especially for A1C greater than 10%. The POC method has a sensitivity of 88.6% and specificity of 96.3% in diagnosing diabetes. CONCLUSIONS: Per ADA guidelines, the POC method is not recommended to be used to diagnose diabetes. Based on our findings 10.3% of individuals would be missed if one used the POC method to diagnose diabetes. However, given its accuracy supported by our findings it is adequate to be used during routine diabetes follow-up visits. The finding from our study can help improve healthcare quality by reducing unnecessary blood draws and clinic visits.

Levetiracetam-induced pancytopenia.

Pancytopenia is a rare side effect of levetiracetam (LEV) that is associated with severe morbidity that requires hospitalization. Here, we report a patient with a right temporoparietal tumor who underwent a temporal craniotomy with resection of the mass and was started on LEV for seizure prophylaxis per the neurosurgery local protocol. The patient developed LEV-induced pancytopenia, which was successfully managed by discontinuation of this medication. Our report aims to increase awareness of this rare cause of pancytopenia among clinicians.

Heart failure: improving the continuum of care.

In the United States (US), heart failure (HF) is the leading medical condition resulting in hospital admission. Despite advances in treatment, the number of HF deaths has continued to increase. At Carolinas Medical Center (CMC), more than 950 annual HF admissions provided an opportunity to examine morbidity, mortality, and readmission rates. Within the facility there exist two HF disease management programs treating more than 1500 patients annually. Through a systematic approach to identify the root causes of morbid and less severe complications, the facility addressed process improvement steps to positively impact HF treatment. Included in these strategies was a link to the outpatient continuum of care created for the HF patient. An examination of the HF program revealed the care to be fragmented, both organizationally and physically. A majority of readmissions could be prevented through closer patient follow-up and more aggressive therapy. Intensive education for staff about the disease management process, medication interventions, smoking cessation, and nutrition counseling was lacking. An interdisciplinary committee, with strong administrative support, was established to evaluate the current program and recommend changes. Delivery of patient care was changed to an integrated care management system model identifying the root causes of the most prevalent operational and clinical deficits. Process improvement steps were immediately implemented. The 30-day readmission rate (all causes) decreased from 18% to 6.1%, the readmission rate for HF decreasedfrom 7.3% to 1.7%, mortality declined by 25%, and morbid complications decreased by 35%. Evaluation of processes and clinical outcomes are ongoing in order to develop strategies for even greater improvement within the HF program.