Case report: Coronary atherosclerosis in a patient with long-standing very low LDL-C without lipid-lowering therapy.

Background: ApoB-containing lipoproteins including low-density lipoprotein cholesterol (LDL-C) are necessary for the development of atherosclerosis, and lifelong exposure to low serum levels of LDL-C have been associated with a substantial reduction of cardiovascular risk. Although plaque regression has been observed in patients with serum LDL-C less than 70-80 mg/dl on lipid-lowering therapy, an LDL-C level under which atherosclerosis cannot develop has not been established. Case presentation: In this case we describe a 60-year-old man with well-controlled diabetes mellitus and hypertension who presented to the hospital after an acute stroke likely due to an atrial myxoma discovered on imaging. A coronary computed tomography angiography scan performed in preparation for the planned surgical myxoma resection revealed an anomalous origin of the right coronary artery as well as evidence of nonobstructive coronary atherosclerosis in the right coronary and non-anomalous left coronary system. Despite not having ever been on any lipid-lowering therapy, this patient was found to have low LDL-C levels (<40 mg/dl) during this admission and on routine laboratory data collected over the prior 16 years. His family history strongly suggested heterozygous familial hypobetalipoproteinemia as a possible diagnosis. Conclusions: This case illustrates that even long-standing, very low levels of LDL-C may be insufficient to completely prevent atherosclerosis and emphasizes the importance of primordial prevention of all cardiovascular risk factors.

Left ventricular myocardial strain and tissue characterization by cardiac magnetic resonance imaging in immune checkpoint inhibitor associated cardiotoxicity.

BACKGROUND: Immune checkpoint inhibitors (ICIs) are highly effective in treating cancer; however, cardiotoxicity can occur, including myocarditis. Cardiac magnetic resonance (CMR) imaging is useful for evaluation of myocarditis, although it has not been well studied in ICI cardiotoxicity. METHODS: We identified patients referred for CMR evaluation of ICI cardiotoxicity from September 2015 through September 2019. We assessed structural and functional parameters, feature tracking (FT) left ventricular and atrial strain, T2- weighted ratios and quantitative late gadolinium enhancement (LGE). We also applied the Updated Lake Louise Criteria for diagnosis of myocarditis. RESULTS: Of the 20 patients referred, the median left ventricular ejection fraction (LVEF) was 52.5% ± 19.1 and 50% had a normal LVEF (≥53%). FT strain analysis revealed an average abnormal global longitudinal strain (GLS) of -9.8%± 4.2%. In patients with a normal LVEF, the average GLS remained depressed at -12.3%± 2.4%. In all patients, GLS demonstrated a significant negative correlation with LVEF (rs = -0.64, p 0.002). Sixteen patients (80%) had presence of LGE (14 non-ischemic pattern and 2 ischemic). Percent LGE did not correlate with any CMR parameters and notably did not correlate with LVEF (rs = -0.29, p = 0.22) or GLS (rs = 0.10, p = 0.67), highlighting the value of tissue characterization beyond functional assessment. Nine patients (45%) met full Updated Lake Louise Criteria and 85% met at least one criterion, suggestive of myocarditis in the correct clinical context. Thirteen patients (65%) were treated for ICI-associated myocarditis and, of these, 54% (n = 7) had recovery of LVEF to normal. There was no correlation between LVEF (p = 0.47), GLS (0.89), or % LGE (0.15) and recovery of LVEF with treatment. CONCLUSION: In patients with suspected ICI cardiotoxicity, CMR is an important diagnostic tool, even in the absence of overt left ventricular dysfunction, as abnormalities in left ventricular strain, T2 signal and LGE can identifying disease.

Simple Renal Cysts as Markers of Thoracic Aortic Disease.

BACKGROUND: Thoracic aortic aneurysm is usually a clinically silent disease; timely detection is largely dependent upon identification of clinical markers of thoracic aortic disease (TAD); (bicuspid aortic valve, intracranial aortic aneurysm, bovine aortic arch, or positive family history). Recently, an association of simple renal cysts (SRC) with abdominal aortic aneurysm and aortic dissection was established. The aim of our study was to evaluate the prevalence of SRC in patients with TAD in order to assess whether the presence of SRC can be used as a predictor of TAD. METHODS AND RESULTS: We evaluated the prevalence of SRC in 842 patients with TAD (64.0% males) treated at our institution from 2004 to 2013 and compared to a control group of patients (n=543; 56.2% males). Patients were divided into 4 groups: ascending aortic aneurysm (456; 54.2%); descending aortic aneurysm (86; 10.2%); type A aortic dissection (118; 14.0%); and type B aortic dissection (182; 21.6%). SRC were identified by abdominal computed tomography or magnetic resonance imaging of these patients. Prevalence of SRC is 37.5%, 57.0%, 44.1%, and 47.3% for patients with ascending aneurysm, descending aneurysm, type A dissection, and type B dissection, respectively. Prevalence of SRC in the control group was 15.3%. Prevalence of SRC was not significantly different between male and female aortic disease patients, despite reported general male predominance (2:1), which was also observed in our control group (1.7:1). CONCLUSIONS: This study establishes an increased prevalence of SRC in patients with TAD. SRC can potentially be used as a marker for timely detection of patients at risk of TAD.

Systemic Inflammatory Disease and Its Association With Type II Endoleak and Late Interventions After Endovascular Aneurysm Repair.

IMPORTANCE: Abdominal aortic aneurysms are associated with chronic inflammation within the aortic wall, and previous studies have suggested that chronic inflammation may be a consequence of a dysregulated and persistent autoimmune response. Persistent aortic remodeling after aneurysm repair could place the patient at risk for endoleak or sac rupture. OBJECTIVE: To determine whether patients with systemic inflammatory disease and large aneurysms have persistent aortic remodeling after endovascular aneurysm repair (EVAR). DESIGN, SETTING, AND PARTICIPANTS: The records of all patients who underwent EVAR between July 2002 and June 2011 at the Veterans Affairs Connecticut Healthcare System were included in this retrospective review. Patients were considered to have a systemic inflammatory disease when confirmed by a referring specialist. Post-EVAR surveillance was performed by yearly imaging. INTERVENTION: Endovascular aneurysm repair. MAIN OUTCOMES AND MEASURES: Significant endoleak, defined as endoleak and sac diameter increase of 0.5 cm or greater. RESULTS: A total of 51 of 79 patients (65%) had a systemic inflammatory disease. These patients had similar comorbid conditions compared with patients without inflammation but significantly greater numbers of major postoperative complications after EVAR (23.5% vs 3.6%; P = .02) and overall postoperative complications after EVAR (27.5% vs 7.1%; P = .03). Patients with a history of systemic inflammatory disease developed more endoleaks (45.1% vs 17.9%; P = .02) and late sac expansion (51.0% vs 21.4%; P = .01) and required more interventions (21.6% vs 3.6%; P = .03) during long-term follow-up. Systemic inflammatory disease was significantly associated with significant endoleak (odds ratio, 5.18; 95% CI, 1.56-17.16; P = .007). CONCLUSIONS AND RELEVANCE: Patients with systemic inflammatory disease are at high risk for postoperative complications, type II endoleak, sac expansion, and additional interventions after EVAR. Additional strategies for improving the efficacy of EVAR in these patients may be warranted.

The chimney procedure is an emergently available endovascular solution for visceral aortic aneurysm rupture.

A 79-year-old woman presented with a ruptured saccular thoracoabdominal aortic aneurysm involving the celiac and mesenteric artery. The patient was unfit for open surgical repair. A "chimney" procedure was performed, which involved placement of stents in the aortic side branches alongside the endograft. The patient underwent another chimney procedure 2 weeks later for a type I endoleak. Computed tomography angiography (CTA) at 1 and 6 months showed a good result with no endoleaks or graft migration. The chimney procedure provides an alternative for emergency patients unfit for open repair and has the advantage that stents can be used that are already available in most institutions.

Simultaneous sizing and preoperative risk stratification for thoracic endovascular aneurysm repair: role of gated computed tomography.

OBJECTIVES: Risk factors are similar for the development of both thoracic aortic aneurysms (TAA) and other cardiovascular diseases. Coronary artery disease is highly prevalent in patients with TAA, with a reported prevalence of 30% to 70%. Knowledge of the underlying cardiac pathology can minimize perioperative risk and improve patient selection. This study investigated the feasibility of simultaneous assessment of thoracic aortic pathology and cardiac structures and function, including the coronary arteries, using electrocardiogram-gated 64-slice computed tomography (CT) angiography. METHODS: ECG-gated 64-detector row CT examinations of 11 patients (8 men, 3 women; mean age, 67 +/- 16; range, 41-83 years) with thoracic aortic pathology, including aneurysms and dissections, were reviewed. Images were assessed for coronary artery disease, calcifications, cardiac function, and valve characteristics. Simultaneous assessment and measurements of thoracic aortic pathology were performed with the same scan. RESULTS: All images of the patients could be successfully assessed for calcium scores, coronary artery stenoses, coronary artery anomalies, interventricular septal wall thickness, myocardial scar, left ventricular ejection fraction, muscle mass, and aortic and mitral valve calcification, mobility, and valve anatomy. Diagnostic image quality was also achieved in all patients for the underlying thoracic aortic disease. CONCLUSION: This study introduces the feasibility of dynamic imaging of the thoracic aorta and cardiac structures and function, including the coronary arteries, with just one CT scan. The images could be successfully assessed for thoracic aorta pathology, cardiac disease, and extracardiac pathology. With further developments of CT scanners-and more detailed insight in the prognosis of patients based on ECG-gated CTA findings-this new technique may become the initial imaging modality for preoperative cardiac risk stratification in patients with TAAs or dissections.