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Statins are recommended in heart transplant patients, but are sometimes poorly tolerated. Alternative agents are often considered including proprotein convertase subtilisin/kexin type-9 inhibitors (PCSK9i). We sought to investigate the use of PCSK9i after heart transplantation.We identified patients who received a heart transplant from 1999 to 2019 and were started on PCSK9i at our institution. Clinical, laboratory, and coronary angiography with intravascular ultrasound results were compared. Among 65 patients initiated on PCSK9i (48 for statin intolerance and 17 for refractory hyperlipidemia), the median time from transplant was 5.5 years (interquartile range [IQR], 2.8-9.9 years) with a median PCSK9 treatment duration of 1.6 years (IQR, 0.8-3.2 years) and 80% still on treatment. Evolocumab was used in 73.8%, alirocumab in 12.3%, and both in 13.8% owing to insurance coverage. All patients required prior authorization; initial denial occurred in 18.5% and 32.3% had denials in subsequent years. The median low-density lipoprotein cholesterol decreased from 130 mg/dL (IQR, 102-148 mg/dL) to 55 mg/dL (IQR, 35-74 mg/dL) after starting PCSK9i (P.001), with 72% of patients achieving a low-density lipoprotein cholesterol of70 mg/dL after treatment. There were also significant reductions of total cholesterol, non-high-density lipoprotein cholesterol, total/high-density lipoprotein cholesterol ratio, and triglycerides, with a modest increase in high-density lipoprotein cholesterol. These changes were durable at latest follow-up. In 33 patients with serial coronary angiography and intravascular ultrasound, PCSK9i were associated with stable coronary plaque thickness and lumen area.Among heart transplant recipients, PCSK9i are effective in lowering cholesterol levels and stabilizing coronary intimal hyperplasia with minimal side effects. Despite favorable effects, access and affordability remain a challenge.

A 48-year-old woman presented with heart failure and bioprosthetic pulmonary valve regurgitation 2 years after pulmonary valve replacement. Intracardiac echocardiography demonstrated uniform thickening of a single prosthetic valve leaflet suggesting leaflet thrombosis rather than bioprosthetic valve degeneration. After 3 months of anticoagulation, valve regurgitation and symptoms improved. (Level of Difficulty: Intermediate.), Central Illustration

Tricuspid regurgitation in patients with repaired tetralogy of Fallot is an important finding with a wide spectrum of primary and secondary etiologies. Moderate or severe tricuspid regurgitation is associated with a greater incidence of atrial tachyarrhythmias in these patients. It remains uncertain which patients with repaired tetralogy of Fallot may benefit from a tricuspid valve intervention at the time of pulmonary valve replacement.

Introduction Anomalous aortic origin of a coronary artery (AAOCA) is a potential etiology of sudden cardiac death (SCD) in physically active individuals. Identification of coronary artery origins is an essential part of comprehensive pre-participation athletic screening. Although echocardiography is an established method for identifying AAOCA, current imaging protocols are time intensive and readers frequently have low confidence in coronary artery identification. Methods Echocardiographic images from a sample of 110 patients from a database of competitive athletes ages 13-22 years from the Kansas City metropolitan area were reviewed by six echocardiographers of varying experience. Coronary artery images were provided to the readers in the conventional single plane for all the patients; then biplane images of the same patients were presented to the readers. While reviewing the images, readers recorded perceived confidence level of identifying the coronary artery from 1 (least confident) to 5 (most confident). Ratings and differences between ratings were summarized descriptively by means and standard deviations across all readings as well as by individual reader. Results The mean confidence level of echocardiogram readers in identifying coronary artery origins increased by 0.4 points (P = .05) on a five-point confidence scale when using biplane imaging rather than single plane imaging. When assessing the variability of confidence of readers on the same patient, the between-reader variability improved from 25.9% to 10.3%. Conclusions Biplane echocardiographic imaging increases the confidence of readers in identifying coronary artery origins.

Background: Occasionally patients with congenitally corrected transposition of the great arteries (ccTGA) exhibit little clinical evidence of cardiovascular limitation even to their 8th decade. We aimed to assess survival prospects in a large cohort of ccTGA adults. Methods & Results: We included 555 ccTGA adults (median age 33.0 years, 48.3% female) under regular follow-up at 28 institutions between 2002 and 2019. The primary outcome was a composite of death, mechanical circulatory support (MCS) and heart transplant. During a median follow-up of 8.1 [IQR 4.4 - 13.3] years, 56 (10.1%) patients died, 10 (1.8%) patients underwent MCS and 14 (2.5%) had a heart transplant. Median age at time of primary outcome was 51.1 [IQR 37.5 - 63.2] years and cumulative incidence at 15 years from baseline was 21.5% [95% CI 16.1 - 26.5]. Leading causes of death were worsening of heart failure (43%) and sudden death (10%). Patients who died were more likely to use heart failure (HF) medications. In multivariable Cox analyses for baseline variables, age, prior atrial arrhythmia and HF admission were each associated with an increased risk of the primary outcome. Figure shows cumulative incidence according to history of atrial arrhythmia. During follow-up, 91 (16.4%) were admitted for HF, pacemaker implantation was performed in 68 (12.3%) patients, ICD in 82 (14.7%), and major cardiac surgery (mostly for systemic AV-valve) in 89 (15.8%) patients. Conclusion: In this large cohort of ccTGA adults, survival seemed to be primarily determined by heart failure-related complications. Prior atrial arrhythmia also seems to be a harbinger for adverse outcome. Few patients underwent advanced HF therapies. Figure: Cumulative incidence of the composite primary outcome (MCS, heart transplant or death) over a period of 14 years from first visit at an adult congenital heart disease clinic since 2002 stratified according to history of atrial arrhythmia. Shading represents upper and lower 95% confidence limits.

Background: Although several factors have been cited for risk stratification in patients with simple transposition of the great arteries (dTGA), no single predictor emerges consistently. We aimed to assess survival and determine factors associated with survival in a large cohort of dTGA adults with atrial switch. Methods and Results: We included 1,169 dTGA adults (median age 28.1 years, 38.7% female) under regular follow-up at 28 institutions between 2002 and 2019. The primary outcome was a composite of death, mechanical circulatory support (MCS) and heart transplant. During a median follow-up of 9.2 [IQR 5.5-14.2] years, 67 (5.7%) patients died, six (0.5%) patients underwent MCS and 21 (1.8%) had a heart transplant. Cumulative incidence of composite endpoint at 15 years was 12.8% [95% CI 9.8 - 15.7], see Figure). Median age at time of primary outcome was 39.5 [IQR 33.9 - 45.1] years. Leading causes of death were worsening of heart failure (34%), non-cardiac (21%) and sudden unexplained death (12%). In multivariable Cox analyses for baseline variables, age, VSD, ventricular arrhythmia and heart failure admission were each associated with increased risk of the outcome, whereas prior pacemaker (26% of patients) was not. New pacemaker implantation was performed in 107 (9.1%), ICD in 109 (9.3%), and cardiac surgery in 35 (3%) patients. Patients who died were more likely to develop arrhythmias, be admitted for heart failure or require surgery during follow-up. Conclusion: In this large contemporary cohort of dTGA adults after atrial switch, late survival was excellent and seemed to be determined by arrhythmia and heart failure-related complications. Few patients underwent advanced heart failure therapies. Figure. Cumulative incidence of the composite primary outcome (MCS, heart transplant or death) over a period of 15 years from first visit at an adult congenital heart disease clinic since 2002. Shading represents upper and lower 95% confidence limits.

Recently, there have been several advances in the field of adult congenital heart disease, such as the percutaneous pulmonic valve implantation (PPVI) to treat right ventricular outflow obstruction. Complications from this technique are seldom but essential to understand. We present a case of a 37-year-old Caucasian male with complicated congenital heart disease, including prior Melody valve implantation, who presented to our hospital with recurrent episodes of pneumonia of two months duration. He was diagnosed with prosthetic valve endocarditis (PVE) from an unusual organism, Rothia dentocariosa. He eventually underwent surgical replacement of the infected valve. Our report is the first case of Melody valve endocarditis due to Rothia dentocariosa reported from the United States.

Purpose To determine differences in donor transmitted atherosclerosis or rapidly progressive Cardiac Allograft Vasculopathy at 1-year post cardiac transplantation in patients with congenital heart disease. Methods This is a retrospective study of patients who underwent heart transplantation at a single center from 2009-2016 who had subsequent coronary angiography with protocol-driven IVUS assessment of the left anterior descending artery. Non-congenital cardiac transplantation patients (N=237) were compared to a cohort of patients with adult congenital heart disease (N=12). Five IVUS sections were prospectively analyzed for intimal thickness. Donor transmitted atherosclerosis was defined as maximum intimal thickness ≥0.5 mm at baseline and rapidly progressive CAV as an increase in maximum intimal thickness by ≥0.5 mm at 1-year. Results Donor transmitted atherosclerosis was present in 121 (51.1%) patients with non-congenital etiology vs. 7 (58.3%) patients with congenital etiology. Rapidly progressive CAV at 1-year post-transplantation was present in 54 (23.0%) vs. 2 (18.2%) patients, respectively. Over a median follow up of 6.1 years, 39 patients died or required re-transplantation. ACHD as an etiology of heart failure was not associated with age- and sex-adjusted outcomes. In a subsequent analysis, there were 45 patients who reached a composite outcome of coronary revascularization, epicardial CAV, or cardiovascular death. Likewise, there was no association between ACHD and this composite endpoint. Conclusion Pre-transplant adult congenital heart disease was not associated with a significant difference in donor transmitted atherosclerosis or rapidly progressive CAV in patients with heart transplantation. Long term outcomes including all-cause mortality and cardiovascular events were not different in patients with versus without ACHD.

The cumulative incidence of systolic heart failure is similar in men and women. However, major prognostic differences exist between genders. We sought to measure gender differences in furosemide prescribing patterns for patients with preexisting heart failure with reduced ejection fraction (HFrEF) admitted with Stage C acute decompensation, regardless of the underlying cause. We conducted a single-center retrospective analysis of patients admitted between 2015 and 2018 for acute on chronic decompensated HFrEF. Primary outcomes were differences in initial furosemide dose, total dose over the first 24 hours of hospitalization, and total dose during the entire hospitalization between women and men. Secondary outcomes included acute kidney injury (AKI), intubation, noninvasive ventilation (NIV), and in-hospital 30-day and 1-year mortality. We studied 434 patients (31% female) with similar baseline characteristics. Females received significantly less furosemide compared to men for the initial dose, over the first 24 hours, and throughout their hospitalization. However, AKI was more prevalent in women versus men (p=0.008). Females admitted for acute on chronic decompensated HFrEF receive significantly less furosemide when compared to men, but developed more AKI prior to discharge.

Administrative data that utilize International Classification of Diseases-Ninth Revision (ICD-9) diagnosis and procedure codes are increasingly used to study patterns of care and outcomes in patients with out-of-hospital cardiac arrest (OHCA) and in-hospital cardiac arrest (IHCA). However, the

This case presentation discusses an extremely rare diagnosis presenting with common symptoms, attributed to influenza, which were not investigated further when clear cardiac symptoms developed. The patient initially presented with symptoms consistent with influenza, but when orthopnea and dyspnea on exertion developed, these cardiac symptoms continued to be attributed to a postviral syndrome and were not further evaluated. Premature closure bias contributed to a delay in diagnosing a rare cardiac condition. The diagnostic momentum, or the continuing of a diagnostic label, occurred across multiple providers and settings. This case demonstrates the risk of premature closure and diagnostic momentum and reminds clinicians to reframe the differential diagnosis as more information on history or physical exam becomes available.

Background This case describes an unusual presentation of an intracranial hemorrhage first thought to be metastatic disease on computed tomography and magnetic resonance imaging. The healthcare team completed an exhaustive search for a primary malignancy that was negative. Final diagnosis on brain biopsy showed intercranial hemorrhage secondary to cerebral amyloid angiopathy. With an increasing number of elderly patients and the rising cost of health care, this case can serve as a reminder to clinicians about their own responsibilities in limiting the cost of health care. Case presentation This is a case report about a 72-year-old white woman with an intracranial hemorrhage secondary to cerebral amyloid angiopathy. The brain lesions on computed tomography/magnetic resonance imaging mimicked a metastatic process until a brain biopsy could give a definitive diagnosis that was completely unexpected. Cerebral amyloid angiopathy is a rare cause of intracerebral hemorrhage and this diagnosis is important to consider in older patients on anticoagulation. Conclusions Cerebral amyloid angiopathy is a rare diagnosis but should be considered in elderly patients on anticoagulation presenting with imaging findings consistent with intracerebral hemorrhage. While metastatic disease is a more common cause of intracerebral hemorrhage, cerebral amyloid angiopathy should remain in the differential diagnosis. This case report serves as a teaching point to clinicians in cases involving an older patient on anticoagulation.

Objectives Previous studies have shown benefit of catheter ablation of atrial fibrillation in patients with heart failure(HF). This study aims to study characteristics of patients with heart failure undergoing catheter ablation of atrial fibrillation (AF). Methods In this retrospective cohort study, we analyzed 269 consecutive patients with mean age of 61 years who underwent catheter ablation for AF. 64 patients (24%) had known history of Stage C heart failure including both Heart failure with reduced ejection fraction and preserved ejection fraction. We compared baseline characteristics at the time of ablation and procedure outcomes between patients who had known heart failure to those who did not. Results At the time of the procedure, patients with HF had significantly higher body mass index (34.4vs32.3 p=0.0432), CHADS2Vasc score (2.6 vs 1.8 p Conclusion Patients with HF undergoing catheter ablation of AF tend to have more risk factors for recurrence but after adjustment for risk factors, the recurrence rates were similar at 3 months and 1 year.

Sudden cardiac arrest has been reported to occur in patients with congenital anomalous coronary artery disease. About 80% of the anomalies are benign and incidental findings at the time of catheterization. We present a case of sudden cardiac arrest caused by anomalous left anterior descending artery. 61-year-old African American female was brought to the emergency department after sudden cardiac arrest. Initial EKG showed sinus rhythm with RBBB and LAFB with nonspecific ST-T wave changes. Coronary angiogram revealed no atherosclerotic disease. The left coronary artery was found to originate from the right coronary cusp. Cardiac CAT scan revealed similar findings with interarterial and intramural course. Patient received one-vessel arterial bypass graft to her anomalous coronary vessel along with a defibrillator for secondary prevention. Sudden cardiac arrest secondary to congenital anomalous coronary artery disease is characterized by insufficient coronary flow by the anomalous left coronary artery to meet elevated left ventricular (LV) myocardial demand. High risk defects include those involved with the proximal coronary artery or coursing of the anomalous artery between the aorta and pulmonary trunk. Per guidelines, our patient received one vessel bypass graft to her anomalous vessel. It is important for clinicians to recognize such presentations of anomalous coronary artery.