Pharmacological management of heart failure in adults with congenital heart disease.

Congenital heart disease (CHD) is the most common global congenital defect affecting over 2.4 million individuals in the United States. Ongoing medical and surgical advancements have improved the survival of children with CHD leading to a shift where, as of 2010, adults constitute two-thirds of the CHD patient population. The increasing number and aging of adult congenital heart disease (ACHD) patients present a clinical challenge due to heightened complexity, morbidity, and mortality. Studies indicate that 1 in 13 ACHD patients will develop heart failure (HF) in their lifetime. ACHD-HF patients experience more frequent emergency department visits, higher hospitalization rates, longer hospital stays, and higher mortality compared to non-ACHD patients with heart failure (non-ACHD-HF). Despite HF being the leading cause of death in ACHD patients, there is a notable gap in evidence regarding treatment. While guideline-directed medical therapy (GDMT) has been extensively studied in non-ACHD-HF, research specific to ACHD-HF individuals is limited. This article aims to comprehensively review available literature addressing the pharmacological treatment of ACHD-HF.

Successful Visceral and Parietal Pericardiectomy for Refractory Effusive-Constrictive Pericarditis: A Challenging Case Report.

BACKGROUND Effusive-constrictive pericarditis (ECP) is an uncommon clinical syndrome characterized by the coexistence of pericardial effusion and constriction involving the visceral pericardium. This differs from constrictive pericarditis, which presents with thickening of the pericardium without effusions. Specific diagnostic criteria of ECP include the failure of right atrial pressure to decrease by 50% or reach a new level below 10 mmHg after normalization of intrapericardial pressure. CASE REPORT We present the case of a 32-year-old obese man with multiple comorbidities who initially presented with flu-like symptoms and pleural effusion with development of constrictive-like symptoms. Despite undergoing numerous pericardiocentesis and appropriate medical management, the patient's condition failed to improve, leading to the likely diagnosis of effusive-constrictive pericarditis. Cultures of pericardial fluid revealed E. -faecium, which required multiple antimicrobial therapy. Despite infection, the exact etiology of ECP remained unknown and likely idiopathic. Common causes of ECP include idiopathic, tuberculosis, cardiac surgery complications, radiation, or neoplasia. Ultimately, the patient underwent a pericardiectomy involving the visceral and parietal pericardium, resulting in hemodynamic stability and resolution of symptoms. CONCLUSIONS This case highlights the challenges in diagnosing and managing ECP, emphasizing the importance of considering surgical intervention in refractory cases. ECP initially presents as a pericardial effusion, often addressed through pericardiocentesis; however, in a small subset of patients, sustained symptoms and altered hemodynamics persist following pericardiocentesis, necessitating further evaluation and management. The success of pericardiectomy in our patient highlights the potential efficacy of surgical intervention in improving outcomes for patients with ECP.

A challenging case of eosinophilic myocarditis leading to heart failure and transplantation.

The large spectrum of etiologies, severities, and histologic appearances of eosinophilic myocarditis (EoM) poses challenges to its diagnosis and management. Endomyocardial biopsy is the current gold standard for diagnosis. However, cardiovascular magnetic resonance imaging is becoming more frequently used to diagnose acute myocarditis because of enhanced sensitivity when compared to histopathologic examination, and its less invasive nature. We report a complicated case of EoM in a male in his mid-thirties that led to fulminant cardiogenic shock that required immunosuppressive therapy on day 5 of admission and implantation of a left ventricular assist device (LVAD) on day 30. EoM was diagnosed on histopathologic examination of the resected fragment of the left ventricular myocardium. Nine months after the initial presentation, the patient ultimately required heart transplantation. The explanted heart showed minimal residual interstitial inflammation with evidence of mildly active intimal arteritis and patchy areas of interstitial fibrosis. In this report, we describe our patient's clinical features and correlate them with imaging and histopathologic findings to illustrate the difficulty in diagnosing EoM, particularly in this complicated patient that ultimately required heart transplantation. The diagnosis can be challenging due to the variable histopathologic features, clinical presentation, and utilization of therapeutic medications and devices.

A low-dose thrombolytic infusion protocol for safe and successful treatment of left ventricular assist device thrombosis: a case series.

Background: Pump thrombosis is a serious complication of continuous-flow left ventricular assist device (CF-LVAD) therapy. In this study, we aim to report a novel protocol of an intermittent, low-dose, and slow infusion of tissue plasminogen activator (alteplase). Case summary: We treated seven LVAD pump thrombosis events (HeartMate® II and HeartWare) in four patients with a median age of 52 years (31-63), and all were female. The protocol was applied from January 2015 to December 2018, and it consisted of an intermittent, low-dose, and slow infusion of systemic thrombolytic therapy in the intensive care unit. This therapy resulted in successful resolution of pump thrombosis in six out of seven events. Bleeding complication occurred in one patient, which included a ruptured haemorrhagic ovarian cyst and a small cerebellar intra-parenchymal haemorrhage. All patients were discharged home in a stable condition, except one patient who died during hospitalization because of severe sepsis, pump thrombosis with subsequent pump exchange, and multi-organ failure. Discussion: A low-dose, prolonged, and systemic thrombolytic infusion protocol is an effective and relatively safe treatment that can lead to a sustained resolution of pump thrombosis with low bleeding complications and failure rates.

Perioperative outcomes of hypertrophic cardiomyopathy: An insight from the National Readmission Database.

BACKGROUND: Data regarding hypertrophic obstructive cardiomyopathy (HOCM) patients undergoing noncardiac surgery is lacking. We sought to examine the perioperative outcomes of HOCM patients undergoing noncardiac surgery using a national database. METHODS: We used the National readmission database from 2016 to 2019. We identified HOCM, heart undergoing noncardiac surgery using ICD 10 codes. We examined hospital outcomes as well as 90 days readmission outcomes. RESULTS: We identified 16,098 HOCM patients and 21,895,699 non-HOCM patients undergoing noncardiac surgery. The HOCM group had more comorbidities at baseline. After adjustment for major clinical predictors, the HOCM group experienced more in-hospital death, odds ratio (OR) 1.33 (1.216-1.47), P < 0.001, acute myocardial infarction (AMI), OR 1.18 (1.077-1.292), P < 0.001, acute heart failure odds ratio OR 1.3 to (1.220-1.431), P < 0.001, 90 days readmission OR 1.237 (1.069-1.432), P < 0.01, cardiogenic shock OR 2.094 (1.855-2.363), P < 0.001. Cardiac arrhythmia was the most common cause of readmission, out of the arrhythmias atrial fibrillation was the most prevalent. Acute heart failure was the most common complication of readmission. There was no difference in major adverse cardiovascular events (MACE), and AMI between both groups and readmission. CONCLUSION: HOCM patients undergoing noncardiac surgery may be at increased risk of in-hospital and readmission events. Acute heart failure was the most common complication during index admission, while cardiac arrhythmias were the most common complication during readmission. More research is needed to address this patient population further.

Evaluation of Right-Side Filling Pressure in Patients With Obesity With Heart Failure Using Handheld Ultrasound Score.

The goal of this investigation is to evaluate the accuracy of handheld ultrasound score in assessing right atrial (RA) pressure in patients with obesity with heart failure. We prospectively studied 123 patients with heart failure referred for right-sided cardiac catheterization. Handheld ultrasound was performed before catheterization to evaluate volume status by estimating RA pressure using end-expiratory inferior vena cava (IVC) dimension, IVC respiratory collapsibility, and right internal jugular (RIJ) vein respiratory collapsibility. A 3-point simple score was created using multiple logistic regression. The patients were divided into 2 groups based on body mass index. The performance of this score was assessed using the receiver operating characteristics curve in each subgroup and was compared with the performance of the 2-point score (expiratory IVC dimension, IVC respiratory collapsibility). Median age was 58 years (interquartile range 48 to 65), and 37% were women. The 3-point score including RIJ performed better than did the 2-point score in patients with obesity (area under the curve 0.84 [0.74 to 0.95] vs 0.69 [0.58 to 0.81], p = 0.001). The performance of the scores did not differ in patients without obesity (area under the curve 0.85 [0.74 to 0.95] vs 0.82 [0.71 to 0.93], p = 0.49). In patients with obesity, the 3-point score had a specificity of 100% and sensitivity of 21% (11% to 31%) for elevated RA pressure ≥10 mm Hg. In conclusion, a 3-point score including both RIJ and IVC assessment performed better in patients with obesity with heart failure and highlights the importance of comprehensive evaluation in patients with obesity to achieve an accurate, noninvasive assessment of volume status.

Cardiac Contractility Modulation in Patients with Advanced Heart Failure: A Comprehensive Review of Literature.

Heart failure is a significant cause of morbidity and mortality worldwide. Despite advancements in guideline-directed medical therapy and improvements in device-based therapies, patients with advanced heart failure have high rates of mortality regardless of ejection fraction. For patients with reduced ejection fraction who meet criteria, cardiac resynchronization therapy or implantable cardiac defibrillators are options available to improve outcomes. However, not all heart failure patients meet those criteria. Cardiac contractility modulation is an innovative therapy that serves to improve functional outcomes and quality of life, while also modifying pathologic gene expression and preventing further remodeling. In this article, we aim to discuss the major clinical trials investigating cardiac contractility modulation as a suitable therapy for patients with advanced heart failure.

Healthcare and economic burden of heart failure with amyloidosis: An insight from National Readmission Database.

INTRODUCTION: We analyzed trends, causes and predictors of 30-days readmission in cardiac amyloidosis and inspected the impact of these readmissions on mortality, morbidity, and utilization of healthcare resources. METHODS: Heart Failure with cardiac amyloidosis patients were selected from National readmission Database (NRD) using ICD-10 CM codes. Patients younger than 18 years, elective readmissions, readmissions due to trauma, patients with missing data and December 2018 admissions were excluded. Primary outcome was all-cause 30-day readmissions rate, secondary outcomes were factors associated with 30-days readmissions and their effect on morbidity, mortality, and healthcare resource utilization. RESULTS: Out of 4123 total heart failure with cardiac amyloidosis index admissions in 2018, 3374 patients were included in final analysis. 19.6% were readmitted within 30 days. Readmitted patients were younger, sicker, admitted to small or large hospital. Hypertensive heart and Chronic Kidney Disease (CKD Stage I-IV) with Congestive Heart Failure (CHF), hypertensive heart and CKD (Stage V) or End Stage Renal Disease (ESRD) with CHF, hypertensive heart disease with CHF, acute kidney failure, and sepsis were the most common causes of readmissions. Young age, admission to small and large size hospitals were independent predictors of 30-day readmissions. Readmissions had higher mortality, costed 6.6 extra in hospital days to patients and $16380 per admission to healthcare system. CONCLUSIONS: Cardiac amyloidosis readmissions were associated with increased morbidity and mortality of patients and extra burden on the healthcare system. There is a need to identify patients at risk for readmissions to improve patient outcomes and decrease healthcare cost.

Atrial Speckle Tracking Echocardiography for Detecting Atrial Fibrillation in Cryptogenic Stroke: A Meta-Analysis.

Cryptogenic stroke (CS) accounts for approximately 25% of ischemic stroke cases, with atrial fibrillation (AF) accounting for 30% of CS cases. We investigated the utility of left atrial (LA) speckle-tracking echocardiography in identifying patients at high risk of AF after CS and potentially guiding patients who will benefit from long-term rhythm monitoring devices. Cochrane Library, MEDLINE, and EMBASE were searched for relevant studies. We included studies that examined patients with new CS without a history of AF and further examined LA strain parameters (peak and/or reservoir strain). Continuous data were pooled as a mean difference (MD) comparing patients who developed AF vs no AF.  We used the inverse variance method with the DerSimonian-Laird estimator for tau2 and Hartung-Knapp adjustment for random effect analysis. I2 was used to assess heterogeneity. Thirteen observational studies met our criteria and included 3031 patients with new CS. Of those, 420 patients developed AF on follow-up, and 2611 patients did not develop AF. The AF group vs. no AF had significantly reduced LA reservoir strain (LARS) [MD: -8.61; 95% CI: -10.76, -6.47, I2 = 85%, p < 0.01] at presentation. LARS is significantly lower in patients who developed AF after CS. More studies are needed to validate this data.

Characteristics and Outcomes of Early vs Late Initiation of Mechanical Circulatory Support in Non-Acute Myocardial Infarction related Cardiogenic Shock: An Analysis of the National Inpatient Sample Database.

Cardiogenic shock (CS) is significant cause of mortality. The use of mechanical circulatory support (MCS) in patients with non-acute myocardial infarction (Non-AMI) CS is lacking. We inquired data regarding the trends and outcomes early vs late initiation of MCS in non-AMI CS. We investigated National Inpatient Sample database between October 2015-December 2018, identifying hospitalizations with CS, either complicated by AMI or Non-AMI. Patients were divided into 2 cohorts, early initiation of MCS (<48 hours) and late initiation of MCS (>48 hours). The primary analysis included death within first 24 hours. A secondary analysis was adjusted after excluding patients who died in first 24 hours. A total of 85,318 patients with non-AMI-related CS with MCS placement were identified. Among this cohort, 54.6% (n=46,579) underwent early initiation of MCS within 48 hours, and 45.4% (n=38,739) underwent late initiation of MCS after 48 hours. In primary analysis, early MCS initiation was associated with more in-hospital mortality in primary outcome of all-cause hospital mortality (35.72% vs 27.63%, P<0.0001, OR 1.44, 95% CI: 1.40-1.49, P<0.0001), however, adjusted secondary analysis showed a statistically significant decrease in all-cause hospital mortality (23.63% vs 27.63%, P<0.0001, OR 0.80, 95% CI: 0.78-0.83, P<0.0001). In non-AMI-related CS and based on survival to 24 hours after admission, early initiation of MCS had statistically significant decrease in all-cause hospital mortality, with less incidence of vascular and renal complications, and shorter hospital stay. Late initiation of MCS was associated with a higher incidence of advanced therapies, including LVAD and transplant.

Insight on the Etiologies of Chronically Elevated Troponin.

Elevated troponins signify myocardial damage and raise concern for acute coronary syndrome (ACS). However, there are medical conditions that may cause a patient to have chronically elevated troponin levels in the absence of ACS. In our extensive review, we look at the conditions and their mechanisms that cause chronically elevated troponin levels and discuss them comprehensively. We also aim for our review to serve as a guide for physicians evaluating this complex group of patients.

In-Hospital Outcomes of Chronic Total Occlusion Percutaneous Coronary Interventions in Heart failure patients.

In-hospital outcomes of chronic total occlusion Percutaneous Coronary Interventions (CTO PCI) in heart failure patients has not been evaluated on a national base and was the focus of this investigation. We used the Nationwide Inpatient Sample database from 2008 to 2014 to identify adults with single vessel CTO PCI for stable ischemic heart disease (SIHD). Patients were divided into 3 groups: patients without heart failure, heart failure with reduced ejection fraction (HFrEF) and heart failure with preserved ejection fraction (HFpEF). Clinical characteristics and in-hospital outcomes were studied using relevant statistics. Multiple logistic regression models were performed to assess in-hospital mortality, acute renal failure, and the use of mechanical support devices. Of 112,061 inpatients with SIHD from 2008 to 2014 undergoing CTO PCI, 21,185 (19%) had HFrEF and 3309 (3%) had HFpEF. Compared to patients without heart failure, HFrEF and HFpEF patients were older (mean age 69.2 vs 66.3, 70.3 vs 66.3 respectively, P < 0.001), had more comorbidities and higher acute in-hospital complications. HFrEF patients had higher adjusted in-hospital mortality [AOR 1.73, 95% CI (1.21-2.48)], acute renal failure [AOR 2.68, 95% CI (2.34-3.06)], and need for mechanical support [AOR 2.76, 95% CI (2.17-3.51)]. Compared to patients without heart failure, HFpEF patients had similar mortality and need for mechanical support, but higher incidence of acute renal failure. Older age was significantly associated with increased in-hospital mortality. chronic total occlusion PCI in patients with heart failure is associated with higher in-hospital morbidity and mortality and warrants further investigation to optimize health care delivery.

A comparison of Atrial Fibrillation Detection Strategies After Ischemic Stroke-A Retrospective Study.

Objective of this retrospective study was to determine if long-term continuous cardiac monitoring with Implantable loop recorder (ILR) in patients with Cryptogenic strokes or TIA is superior at detecting Atrial Fibrillation (AF) than 30-day Event Monitor (EM) and 48-hour Holter Monitor (HM). Furthermore, we aimed to deduce if uncovering AF leads to lower risk of future ischemic strokes, or reduction in mortality. In 20%-30% cases, the cause of stroke remained unexplained after diagnostic workup which has led to coining of the term, Cryptogenic Stroke (CS). Undiagnosed AF is a prime suspect in CS, but guidelines do not recommend initiation of anticoagulation unless AF has formally been detected. IRB approved retrospective study included patients with at least 1 episode of ischemic stroke or TIA without identifiable cause and was monitored with either HM, EM or ILR to diagnose any undiscovered AF. All patients (n = 531) had at least 1 year, and up to 3 years, of follow-up after device placement. Chi-Squared analysis and Multivariable logistic regression demonstrated no statistically significant difference among 3 devices for detection of AF within 1 month of index stroke but a significant difference in AF detection was observed at 6, 12 and 24 months. Cox proportional hazard model showed device type had no significant impact on secondary outcomes: Subsequent ischemic stroke or TIA, Initiation of anticoagulation, Mortality and Incidence of major bleeding. Despite the superiority of AF detection by ILR, it is not superior to HM or EM in lowering the risk of subsequent stroke or TIA, or in reducing mortality.

Coronary Artery Disease and Aspirin Intolerance: Background and Insights on Current Management.

Aspirin is one of the most widely used medications across the global healthcare system and is the foundation in treating ischemic heart disease, as well as secondary prevention for ischemic and valvular heart disease. Challenges arise in treating patients with cardiovascular disease who have concomitant aspirin intolerance. Through an extensive review of the literature, we provide a comprehensive background on the pharmacology of aspirin, the mechanisms behind aspirin intolerance, the importance of aspirin in cardiovascular disease, and the management of aspirin intolerance in both acute coronary syndrome and stable coronary artery disease. Our review includes a multidisciplinary approach from the internist, allergist/immunologist, and cardiologist when evaluating this important patient population.

Safety of Transcatheter Aortic Valve Replacement in Patients with Aortic Aneurysm: A Propensity-Matched Analysis.

INTRODUCTION: There is a paucity of data regarding the outcomes of transcatheter aortic valve replacement (TAVR) among patients with thoracic or abdominal aortic aneurysms (AA). Using the Nationwide Inpatient Sample (NIS) database, we explored the safety of TAVR among patients with a diagnosis of AA. METHODS: We queried the National Inpatient Sample database (2012-2017) for hospitalized patients undergoing TAVR, using ICD-9 and ICD-10 codes for endovascular TAVR. Reports show that > 95% of endovascular TAVR in the US is via transfemoral access, so our population are mostly patients undergoing transfemoral TAVR. Using propensity score matching, we compared the trends and outcomes of TAVR procedures among patients with versus without AA. RESULTS: From a total sample of 29,517 individuals who had TAVR procedures between January 2012 and December 2017, 910 had a diagnosis of AA. In 774 matched-pair analysis, all-cause in-hospital mortality was similar in patients with and without AA OR 0.63 [(95% CI 0.28-1.43), p = 0.20]. The median length of stay was higher in patients with AA: 4 days (IQR 2.0-7.0) versus 3 days (IQR 2.0-6.0) p = 0.01. Risk of AKI [OR 1.01 (0.73-1.39), p = 0.87], heart block requiring pacemaker placement [OR 1.17 (0.81-1.69), p = 0.40], aortic dissection [OR 2.38 (0.41-13.75), p = 0.25], acute limb ischemia [OR 0.46 (0.18-1.16), p = 0.09], vascular complications [OR 0.80 (0.34-1.89), p = 0.53], post-op bleeding [OR 1.12 (0.81-1.57), p = 0.42], blood transfusion [OR 1.20 (0.84-1.70), p = 0.26], and stroke [OR 0.58 (0.24-1.39), p = 0.25] were similar in those with and without AA. CONCLUSIONS: Data from a large nationwide database demonstrated that patients with AA undergoing TAVR are associated with similar in-hospital outcomes compared with patients without AA.

Coronary Microvascular Disease.

Coronary microvascular disease or dysfunction (CMVD) has been associated with adverse cardiovascular outcomes. Despite a growing prevalence, guidelines on definitive treatment are lacking. Proposed mechanisms of endothelial dysfunction and resultant inflammation have been demonstrated as the underlying cause. Imaging modalities such as echocardiography, cardiac MRI, PET, and in some instances CT, have been shown to be useful in diagnosing CMVD mainly through assessment of coronary blood flow. Invasive measurements through thermodilution and pressure sensor-guided Doppler microcatheters have also been utilized. Treatment options are directed at targeting inflammatory pathways and angina. In our review, we highlight the current literature on the background of CMVD, diagnostic modalities, and management of this disease.

Endomyocardial biopsy facilitates diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA): a case report.

BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare small vessel leukocytoclastic vasculitis that affects multiple organs and is often associated with anti-neutrophil cytoplasmic antibody (ANCA). EGPA presenting with cardiac involvement is often ANCA-negative, difficult to diagnose, and often fatal. The treatment and prognosis and can be quite different for other conditions included in the differential diagnosis. Imaging modalities have proven unreliable, and the skin is the most commonly biopsied site for histological diagnosis. CASE SUMMARY: We report a case of a 55-year-old Hispanic man who presented with a non-ST-elevated myocardial infarction, reduced ejection fraction heart failure, and hypereosinophilia. The patient's clinical history also included poorly controlled asthma and sinonasal polyps. Despite ANCA titers within the normal range and a skin biopsy lacking evidence of EPGA, high clinical suspicion prompted an endomyocardial biopsy on day nine from hospital admission which facilitated the diagnosis of ANCA-negative EGPA-induced cardiomyopathy. Six months of follow-up revealed that therapeutic response, as measured by the cardiac ejection fraction, directly correlated with medication compliance. CONCLUSION: Endomyocardial biopsy aids in the diagnosis of EGPA and initiates use of appropriate therapy.

Outcomes in Hospitalization in Patients with Heart Failure Undergoing Remote Pulmonary Artery Pressure Monitoring: A Systematic Review and Meta-Analysis of Major Trials.

Heart failure is a leading global pandemic and a cause of economic burden. Although, treatments exist to help symptomatic alleviation, patient compliance and monitoring is the basis of ensuring efficacy. With devices that allow for remote wireless PA pressure monitoring such as CardioMEMS, the inconsistency in patient reporting and factors such as symptoms and hospitalizations can be reduced. A systematic review and meta-analysis utilizing the MEDLINE, Cochrane, and Scopus database was performed to identify randomized and non-randomized clinical trials evaluating baseline characteristics and hospitalizations. Five trials for the systematic review and 2 trials for the meta-analysis meeting the inclusion and exclusion criteria were included. Baseline characteristics included an average age of 64.6 years, male predominance, mean BMI of 29.6, predominance of HFrEF, hypertension the most prevalent comorbidity, and a mean PA pressure of 27.2 mm Hg. The follow-up periods ranged from 90 days to 12 months. There was a total of 64 adverse events, mostly non-serious. Patients who underwent remote PA monitoring were less likely to be hospitalized compared with patients who did not (Odds Ratio: 0.52; 95% Confidence Interval 0.39, 0.69). Remote PA pressure monitoring allows for reduced hospitalizations. With the recent and now resurging SARS-CoV-2 pandemic, devices such as CardioMEMS can allow for heart failure patients to be managed from home to not only reduce hospitalizations but for symptom prevention and management.