Heart disease remains a major health threat in women. Cardiometabolic risk factors such as obesity and diabetes differentially and adversely impact heart disease risk. Although obstructive coronary artery disease is an important cause of ischemic heart disease in women and is prognostic, women are more likely to have angina and myocardial ischemia without obstructive atherosclerosis, which has been attributed to coronary microvascular dysfunction (CMD). Heart failure with preserved ejection fraction (HFpEF) is another condition that predominates in women. CMD and HFpEF are both associated with cardiometabolic risk factors that are prevalent in women. Women are also more likely to have additional risk-enhancing conditions such as autoimmune dysfunction, chronic inflammation, and sex-specific hormonal factors that adversely influence risk. In this review, we focus on cardiometabolic risk factors of obesity and diabetes in heart disease in women, including ischemic heart disease from CMD, HFpEF, and arrythmias. Team-based care to focus on cardiometabolic risk reduction is needed to alter adverse heart disease outcomes in women. Identification, education, treatment, and active surveillance of these dysmetabolic risk factors are imperative in the primary and secondary prevention of heart disease in women.

Background: Cardiac amyloidosis (CA) is most commonly caused by the infiltration of misfolded amyloid transthyretin (ATTR) or light chain (AL) molecules in the myocardium. The workup for CA requires serum assessment to exclude the rapidly progressive AL subtype before imaging to assess the more common and indolent ATTR form. The diagnostic algorithm has the potential to become complex as in this case about a woman with Sjogren’s syndrome which predisposes to plasma cell dyscrasias. Case: A 68-year-old female with congenital long QT syndrome requiring implantable cardioverter-defibrillator (ICD), bilateral carpal tunnel, and recent diagnosis of Sjogren’s syndrome was found to have new systolic heart failure during workup for interstitial lung disease. Labs revealed pancytopenia, abnormal kappa/lambda ratio (2.25), and polyclonal gammopathy on immunofixation. Echocardiogram showed moderately reduced systolic function with an ejection fraction of 35-40% and moderate concentric left ventricular hypertrophy. Decision-making: Cardiology obtained cardiac magnetic resonance imaging (CMR) that returned indeterminate due to ICD artifact. Hematology performed a bone marrow biopsy that was negative for plasma cell dyscrasia, though stained positive for amyloid on congo red with mass spectrometry revealing the valine 142 isoleucine (Val142Ile) amino acid substitution consistent with hereditary ATTR-CA. To definitively assess for endomyocardial ATTR involvement the patient was sent for cardiac scintigraphy using 99mTc-labeled pyrophosphate which returned negative with a cardiac to contralateral lung uptake ratio of 1.001 and visual grade of 0. With suspicion still high, an endomyocardial biopsy was pursued and stained positive for congo red with mass spectrometry confirming hereditary Val142Ile ATTR-CA, allowing for tafamidis initiation. Conclusion: We present a complex diagnostic case of ATTR amyloid in a patient who is at high risk for plasma cell dyscrasias with an abnormal kappa/lambda ratio, non-diagnostic CMR, and negative nuclear scan. Despite significant advances in imaging techniques allowing for less invasive diagnostics, this case highlights the role of endomyocardial biopsy in complicated cases.

This review summarizes current data supporting a minimalist TAVR approach and identifies the need for additional study to optimize TAVR care. The authors discuss future directions of the TAVR landscape and how this necessitates evolution of minimalist care pathways. Transcatheter aortic valve replacement (TAVR) has become a mainstay in the treatment of aortic stenosis since the initial procedure in 2002. Recently, attention has shifted to TAVR optimization and the minimalist approach with a focus on minimizing procedural sedation, protocolization of perioperative management, and prioritization on early discharge. This approach has been shown to be safe and reduce procedure time, length of stay, and overall cost for hospital systems. The minimalist care pathway avoids general anesthesia, shortens procedure time and length of stay, and reduces cost without changing mortality or readmission rates at 30 days. A variety of protocols have been proposed without a clear consensus on specific components or patient eligibility. There is a continued need for data regarding patient risk stratification, valve selection, and discharge strategy as TAVR becomes increasingly common.

Cardiac rehabilitation(CR) has known proven benefits in reducing mortality related to cardiovascular disease (CVD), improving quality of life (QoL), and preventing CVD-related readmissions. Despite these known benefits, CR remains underutilized among women relative to men. Disparities exist at the level of referral, enrollment, and program completion. Much is left to be understood regarding the utility of CR in female-predominant CVD such as postpartum cardiomyopathy and Spontaneous Coronary Artery Dissection. This review identifies the benefits of CR for specific populations of women and elucidates on the barriers to CR. We also describe current recommendations to overcome barriers to CR in women.

CASE REPORT A 46-year-old woman with a medical history of sarcoidosis and recently diagnosed biopsy-proven idiopathic retroperitoneal fibrosis (RPF) unresponsive to corticosteroids and tamoxifen presented to the hospital with dysphagia, abdominal pain, weight loss, and back pain. She had cachexia with facial swelling and worsening anemia. Abdominal and pelvic computed tomography (CT) revealed diffuse infiltration of the retroperitoneal fat in the para-aortic and pericaval regions consistent with previous diagnosis of RPF. In addition, there was an expansion of the left iliopsoas and lumbar paraspinal musculature with dystrophic calcification concerning for muscle involvement (Figure 1).Figure 1.: (A) CT with intravenous contrast demonstrating marked expansion of the left iliopsoas musculature (arrow) and (B) positron emission tomography (maximum intensity projection) demonstrating metabolically active mass at the level of the pylorus, bilateral thigh masses, hypermetabolic mediastinal adenopathy, and bilateral pulmonary nodules. CT, computed tomography.Upper endoscopy demonstrated 2-cm ulceration at the distal lesser curvature (Figure 2). Biopsy was consistent with poorly differentiated adenocarcinoma with a signet ring component (Figure 2). Biopsies of the left iliopsoas muscle revealed the same. Positron emission tomography (PET) demonstrated increased metabolic activity in the stomach, thighs, mediastinal lymph nodes, and lungs concerning for Stage IV gastric cancer (Figure 1). The patient was started on folinic acid, flourouracil and oxaliplatin for palliative chemotherapy after hospital discharge but subsequently developed gastric perforation requiring emergent surgery and Graham patch repair after several cycles of chemotherapy. Unfortunately, the patient died not long after.Figure 2.: (A) Endoscopic view of the 2-cm ulceration in the distal lesser curvature and (B) gastric biopsy demonstrating poorly differentiated adenocarcinoma with a signet ring component.RPF is a rare condition characterized by chronic inflammatory and fibrotic process in the retroperitoneum that can lead to anatomical compression and obstruction. RPF disproportionately affects men between the age of 40 years and 60 years. The true incidence of RPF is unknown but is estimated to be 0.1 to 1.3 cases per 100,000 persons per year.1,2 RPF can be idiopathic or secondary to malignancy, infection, inflammatory disorder, drugs, radiation therapy, or rare histiocytic disorder. Although most of RPF cases are idiopathic, occurring either in isolation or in association with other autoimmune diseases, a small proportion (8%–10%) of cases occur as a response to malignancy. Several mechanisms have been reported for the pathogenesis of secondary RPF related to malignancy. One proposed mechanism is the presence of malignant cells in the retroperitoneal cavity, causing macrophages to release cytokines and ultimately leading to fibroblast proliferation. Another reported mechanism is the activation of serotonin-mediated and profibrogenic growth factors, such as platelet-derived growth factor, insulin-like growth factors, and epidermal growth factor, identified in carcinoid tumor.2 Diagnosis of RPF is typically performed by the appropriate abdominal imaging, but it is crucial that patients with newly diagnosed RPF to complete age-appropriate cancer screening along with additional studies based on clinical and radiological suspicions. Careful evaluation of abdominal imaging should be performed to identify high-risk characteristics, such as muscle or bone involvement that would raise the suspicion for a secondary process, such as infection or malignancy.3 Additional imaging using PET with 18F-fluorodeoxyglucose (18F-FDG) has been suggested as an adjunctive tool to assess the metabolic or inflammatory activities in RPF along with the identification of primary malignancy. The use of PET/CT has gained traction recently for the evaluation of RPF, but there has been little consensus on its utility in distinguishing idiopathic from secondary causes of RPF. Recent studies have shown that there may be some utility of PET/CT in distinguishing idiopathic RPF from retroperitoneal malignancy-induced RPF. One study demonstrated that idiopathic RPF had a lower frequency of FDG uptake and a lower mean maximum standardized uptake value when compared with malignancy-induced RPF.4 The absence of FDG uptake in the retroperitoneum does not rule out malignant involvement of retroperitoneal cavity, as reflected by this case. If the abdominal imaging does not show typical findings or suspicion or secondary RPF is high, retroperitoneal biopsy may be helpful considering that delayed diagnosis is associated with poor prognosis. Multiple biopsy techniques have been reported, including open, laparoscopic or transcaval and fine-needle aspiration, but multiple deep surgical biopsies may be needed in malignant RPF because of diffuse dispersion of metastatic cells within the fibrotic tissues. The absence of malignant cells on retroperitoneal biopsy does not rule out the possibility of malignancy-related RPF as denoted by this case and other reported cases of malignant RPF.4 Although it has been well-established that patients with a new diagnosis of RPF should undergo age-appropriate cancer screening, the role of endoscopy in the evaluation of secondary RPF is unclear.5 It should be considered because gastrointestinal malignancy presenting as isolated secondary RPF has been reported.6,7 This case highlights the importance of identifying high-risk features of secondary RPF on abdominal imaging and the utility of endoscopy in the diagnosis of secondary RPF. DISCLOSURES Author contributions: N. Shetty, A. Calhoun, D. Sunjaya, and A. Greer wrote the article. F. Willingham revised the article for intellectual content. D. Sunjaya is the guarantor of the article. Financial disclosures: F. Willingham reports grant/research support paid to the institution from Cancer Prevention Pharmaceuticals, Cook Medical, Oncolys Biopharma, RedPath, Boston Scientific, Xlumena, and Steris Medical. Informed consent was obtained for this case report.

BACKGROUND:Aortitis refers to pathologic inflammation of the aortic wall and is broadly categorized into inflammatory (or non-infectious) and infectious aortitis. While rare, isolated non-infectious ascending aortitis (I-NIAA) is a clinical entity that is becoming increasingly recognized but remains poorly understood.CASE SUMMARY:A 72-year-old man presented with an asymptomatic murmur and was found to have severe aortic insufficiency second to a large ascending aortic aneurysm. He underwent surgical repair and pathology revealed isolated non-infectious ascending aortitis. Following successful surgical repair, he developed joint pains which were successfully treated with glucocorticoids.DISCUSSION:Isolated non-infectious aortitis is a rare entity that warrants further investigation. This case highlights the importance of sending surgical specimens for histopathologic evaluation even when a systemic process is not evident at the time of surgical repair. The development of systemic symptoms following surgical repair in this patient emphasizes the importance of thorough rheumatologic evaluation in patients found to have I-NIAA. Isolated non-infectious ascending aortitis remains poorly understood, and further study is needed to evaluate both its existence as a distinct clinical entity and the role of immunosuppressive therapy.