End-stage heart failure in congenitally corrected transposition of the great arteries: a multicentre study.

BACKGROUND AND AIMS: For patients with congenitally corrected transposition of the great arteries (ccTGA), factors associated with progression to end-stage congestive heart failure (CHF) remain largely unclear. METHODS: This multicentre, retrospective cohort study included adults with ccTGA seen at a congenital heart disease centre. Clinical data from initial and most recent visits were obtained. The composite primary outcome was mechanical circulatory support, heart transplantation, or death. RESULTS: From 558 patients (48% female, age at first visit 36 ± 14.2 years, median follow-up 8.7 years), the event rate of the primary outcome was 15.4 per 1000 person-years (11 mechanical circulatory support implantations, 12 transplantations, and 52 deaths). Patients experiencing the primary outcome were older and more likely to have a history of atrial arrhythmia. The primary outcome was highest in those with both moderate/severe right ventricular (RV) dysfunction and tricuspid regurgitation (n = 110, 31 events) and uncommon in those with mild/less RV dysfunction and tricuspid regurgitation (n = 181, 13 events, P < .001). Outcomes were not different based on anatomic complexity and history of tricuspid valve surgery or of subpulmonic obstruction. New CHF admission or ventricular arrhythmia was associated with the primary outcome. Individuals who underwent childhood surgery had more adverse outcomes than age- and sex-matched controls. Multivariable Cox regression analysis identified older age, prior CHF admission, and severe RV dysfunction as independent predictors for the primary outcome. CONCLUSIONS: Patients with ccTGA have variable deterioration to end-stage heart failure or death over time, commonly between their fifth and sixth decades. Predictors include arrhythmic and CHF events and severe RV dysfunction but not anatomy or need for tricuspid valve surgery.

PCSK9 Inhibitors in Heart Transplant Patients: Safety, Efficacy, and Angiographic Correlates.

BACKGROUND: 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. METHODS AND RESULTS: 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 of <70 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. CONCLUSIONS: 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 novel echocardiographic approach for assessing coronary artery origins.

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.

Prognostic Significance of Hemodynamics in Patients With Transposition of the Great Arteries and Systemic Right Ventricle.

BACKGROUND: Patients with transposition of the great arteries (TGA) and systemic right ventricle often confront significant adverse cardiac events. The prognostic significance of invasive hemodynamic parameters in this context remains uncertain. Our hypothesis is that the aortic pulsatility index and hemodynamic profiling utilizing invasive measures provide prognostic insights for patients with TGA and a systemic right ventricle. METHODS: This retrospective multicenter cohort study encompasses adults with TGA and a systemic right ventricle who underwent cardiac catheterization. Data collection, spanning from 1994 to 2020, encompasses clinical and hemodynamic parameters, including measured and calculated values such as pulmonary capillary wedge pressure, aortic pulsatility index, and cardiac index. Pulmonary capillary wedge pressure and cardiac index values were used to establish 4 distinct hemodynamic profiles. A pulmonary capillary wedge pressure of ≥15 mm Hg indicated congestion, termed wet, while a cardiac index <2.2 L/min per m2 signified inadequate perfusion, labeled cold. The primary outcome comprised a composite of all-cause death, heart transplantation, or the requirement for mechanical circulatory support. RESULTS: Of 1721 patients with TGA, 242 individuals with available invasive hemodynamic data were included. The median follow-up duration after cardiac catheterization was 11.4 (interquartile range, 7.5-15.9) years, with a mean age of 38.5±10.8 years at the time of cardiac catheterization. Among hemodynamic parameters, an aortic pulsatility index <1.5 emerged as a robust predictor of the primary outcome, with adjusted hazard ratios of 5.90 (95% CI, 3.01-11.62; P<0.001). Among the identified 4 hemodynamic profiles, the cold/wet profile was associated with the highest risk for the primary outcome, with an adjusted hazard ratio of 3.83 (95% CI, 1.63-9.02; P<0.001). CONCLUSIONS: A low aortic pulsatility index (<1.5) and the cold/wet hemodynamic profile are linked with an elevated risk of adverse long-term cardiac outcomes in patients with TGA and systemic right ventricle.

Tricuspid Regurgitation in Patients with Tetralogy of Fallot.

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.

Cardiovascular Medications in Pregnancy: A Primer.

Cardiovascular disease and cardiovascular disease-related disorders remain among the most common causes of maternal morbidity and mortality in the United States. Due to increased rates of obesity, delayed childbearing, and improvements in medical technology, greater numbers of women are entering pregnancy with preexisting medical comorbidities. Use of cardiovascular medications in pregnancy continues to increase, and medical management of cardiovascular conditions in pregnancy will become increasingly common. Obstetricians and cardiologists must familiarize themselves with the pharmacokinetics of the most commonly used cardiovascular medications in pregnancy and how these medications respond to the physiologic changes related to pregnancy, embryogenesis, and lactation.

Intracardiac Echocardiography to Diagnose Bioprosthetic Pulmonary Valve Thrombosis.

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.).

Melody Valve Endocarditis Due to Rothia dentocariosa: A Diagnostic Challenge.

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.

Women Hospitalized for Acute on Chronic Decompensated Systolic Heart Failure Receive Less Furosemide Compared to Men.

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.

Radiation-Induced Coronary Artery Disease and Its Treatment: A Quick Review of Current Evidence.

As advances in medical technology arise and the availability of cancer treatment increases, an increased number of patients are receiving cancer treatment. Radiation therapy has evolved to become one of the cornerstones of treatment for various types of cancers. One of the long-term consequences of radiation therapy is radiation-induced coronary artery disease (RICAD). Although the pathophysiology of RICAD may be slightly different and more acute onset than the commonly seen "generic" coronary artery disease, it is common practice to treat RICAD in the same method as nonradiation-induced CAD. This paper summarizes the current research available on the topic and shows there is not enough research to obtain significant data about outcomes and restenosis rates of PCI or outcomes of CABG in RICAD. The aim of this review is to create a concise and easy-to-follow review of the relevant data regarding RICAD and hopefully spark further interest in future studies in this field.

Secret Sarcoma: A Cardiac Mass Disguised as Influenza.

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.

Cerebral amyloid angiopathy mimicking central nervous system metastases: a case report.

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.

Malignant Course of Anomalous Left Coronary Artery Causing Sudden Cardiac Arrest: A Case Report and Review of the Literature.

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.