Prognostic value of exercise longitudinal right ventricular free wall strain in patients with sickle cell disease.

Longitudinal right ventricular free wall strain (RVFWS) has been identified as an independent prognostic marker in patients with pulmonary hypertension. Little is known however about the prognostic value of RVFWS in patients with sickle cell (SC) disease, particularly during exercise. We therefore examined the prognostic significance of RVFWS both at rest and with exercise in patients with SC disease and normal resting systolic pulmonary artery pressure (SPAP). Consecutive patients with SC disease referred for bicycle ergometer stress echocardiography (SE) were enrolled ftom July 2019 to January 2021. All patients had measurable tricuspid regurgitation velocity (TRV). Conventional echocardiography parameters, left ventricular global longitudinal strain (LVGLS), RVFWS, and ventriculoarterial coupling indices (TAPSE/SPAP and RVFWS/SPAP) were assessed at rest and peak exercise. Repeat SE was performed at a median follow-up of 2 years. The cohort consisted of 87 patients (mean age was 31 ± 11 years, 66% females). All patients had normal resting TRV < 2.8 m/s, RVFWS and LVGLS at baseline. There were 23 (26%) patients who had peak stress RVFWS < 20%. They had higher resting and peak stress TRV and SPAP, but lower resting and peak stress TAPSE/SPAP, RVFWS/SPAP, and LVGLS as well as lower peak stress cardiac output when compared to patients with peak stress RVFWS ≥ 20% (p < 0.05). Patients with baseline peak stress RVFWS < 20% had a significant decrease in exercise performance at follow-up (7.5 ± 2.7 min at baseline vs. 5.5 ± 2.8 min at follow-up, p < 0.001). In the multivariate analysis, baseline peak stress RVFWS was the only independent predictor of poorer exercise performance at follow-up [odds ratio 8.2 (1.2, 56.0), p = 0.033]. Among patients with SC disease who underwent bicycle ergometer SE, a decreased baseline value of RVFWS at peak stress predicted poorer exercise time at follow-up.

Clinical significance of the hemodynamic gain index in patients undergoing exercise stress testing and coronary computed tomography angiography.

BACKGROUND: Many hemodynamic parameters provide limited information regarding obstructive coronary artery disease (CAD) during exercise stress testing particularly when exercise is suboptimal. Hemodynamic gain index (HGI) is a recent sensitive indicator of ischemia and has been associated with increased mortality. This study evaluated the clinical impact of HGI in patients who underwent concomitant exercise stress testing and coronary computed tomography angiography (CCTA). METHODS: A total of 284 consecutive patients from the executive health program between 2010 and 2018 were identified. Resting and peak heart rate (HR) as well as systolic blood pressure (SBP) measurements were recorded. Framingham risk score (FRS), Duke treadmill score (DTS) and HGI [Formula: see text] were calculated. The latter was divided into quartiles. CCTA was used as a reference test to detect any CAD. Multivariate analysis and artificial neural network were used to determine the independent predictors of obstructive CAD. RESULTS: Mean age was 53 ± 12 years with 83% male. Mean HGI was 1.74 ± 0.67, with cut-off value of severely blunted HGI ≤ 1.25 (Quartile 4). Patients with severely blunted HGI were older, had higher FRS, and worse DTS. Patients with obstructive CAD had lower HGI when compared to those with normal CCTA/non-obstructive CAD (1.36 ± 0.53 vs. 1.77 ± 0.67, P = 0.005), and showed a higher prevalence of severely blunted HGI (44% vs. 22%, P = 0.019). After adjusting for traditional risk factors, HGI remained an independent predictor of obstructive CAD while severely blunted HGI was associated with threefold increased odds of having obstructive CAD (P = 0.05). Using artificial intelligence analysis, severely blunted HGI independently predicted obstructive CAD with an area under the curve of 0.83 and 0.96, and normalized importance of HGI of 100% and 63%, respectively for different models. CONCLUSIONS: Among patients who underwent concomitant exercise stress testing and CCTA, severely blunted HGI independently predicted obstructive CAD after multivariate adjustment for traditional risk factors.

From the clinical spectrum of myocardial calcification to a cardiovascular imaging perspective.

We herein report the case of a 74-year-old man with recurrent transient ischemic attacks and falls who was found to have an unusual large mass of the interventricular septum. After extensive workup, isolated myocardial calcification was confirmed using multimodality cardiovascular imaging. This rare finding might be a reflecting cause of the patient's clinical presentation.

Closing the door in front of a torrential mitral regurgitation.

BACKGROUND: Treatment of symptomatic severe mitral regurgitation (MR) is challenging in patients who are not candidates for surgical intervention. CASE DESCRIPTION: We report the case of a 64-year-old female with multiple comorbidities who was found to have severe MR due to a large mitral valve non coaptation. CONCLUSIONS: In this case report, we show the potential of transcatheter edge-to-edge mitral valve repair using MitraClip for the treatment of torrential MR due to a large mitral valve non-coaptation in a patient with no prior history of coronary artery disease and in whom surgical intervention was not an option.

One year outcome and analysis of peri-device leak of left atrial appendage occlusion devices.

BACKGROUND: The prevalence of peri-device leak (PDL) of left atrial appendage occlusion (LAAO) devices has been previously reported. However, there have been only few data that compared different existing devices. The aim of this study was to assess the incidence of PDL with both devices WATCHMAN®, Boston Scientific and AMPLATZER Amulet®, Abbott Laboratories and to evaluate the clinical outcome at 12 months. METHODS: Consecutive patients who underwent LAAO between January 2018 and 2020 were randomly assigned to either WATCHMAN or AMPLATZER Amulet implantation based on a systematic 2-week alternation between both devices. LAA measurements were assessed using cardiac computed tomography angiography (CCTA) prior to and transesophageal echocardiography (TEE) during the procedure. At 8 weeks post-LAAO, patients underwent TEE and/or CCTA to identify the presence of PDL and/or device-related complications. Patients were then followed for 12 months to identify major adverse cardiovascular/embolic events. RESULTS: The cohort consisted of 51 patients (25 WATCHMAN, 26 AMPLATZER Amulet; mean age 76 ± 7 years; male gender 76%). Both groups were identically matched for demographics, comorbidities, and indication for LAAO. There were 19 patients who had PDL (13 WATCHMAN vs. 6 AMPLATZER Amulet, P-value = 0.033). Of them, 8 (15%) patients had significant PDL (7 WATCHMAN vs. 1 AMPLATZER Amulet, P-value = 0.018). On CCTA, the landing zone maximal diameter of the AMPLATZER Amulet device had the strongest correlation with the final deployed device size (Spearman's rho 0.92, P-value < 0.0001). In the multivariate analysis, male gender and device type were independent predictors of any PDL (P-values 0.016 and 0.031, respectively). On a mean follow-up of 12 months, the total number of events was more prevalent in the WATCHMAN group (P-value 0.008), but the incidence of cardio-embolic events reached borderline significance (16% vs. 0%, P-value = 0.051). CONCLUSIONS: Among patients who underwent LAAO, almost 15% had significant PDL with the majority belonging to the WATCHMAN group. Still, larger studies are warranted to evaluate its effectiveness in stroke prevention.

Do we need cardiovascular computed tomography angiography in the setting of acute ST-segment elevation myocardial infarction?

While the role of cardiovascular computed tomography angiography (CCTA) is evolving in low-intermediate risk patients with myocardial injury due to equivocal causes, this report highlights the added value of CCTA in evaluating the mechanism of acute ST-segment elevation myocardial infarction in a patient with aortic dissection and mechanical aortic valve replacement. CCTA provided accurate diagnosis and guided therapy without delay. A 51-year-old man with mechanical aortic valve replacement was admitted for severe chest pain. CCTA showed type A aortic dissection with an entry tear at the ostial left main coronary artery. Given the high surgical risk, he underwent fenestration of the false lumen. Twelve days postoperatively, he had chest pain with anterior STEMI. Urgent CCTA showed two left coronary thrombi with transmural extensive ischemia. Urgent thrombus aspiration and percutaneous coronary intervention were performed. A drug-eluting stent was placed on the proximal LAD. CCTA was crucial prior to PCI in order to define the etiology of STEMI and evaluate the vascular access.

Multimodality imaging approach for the diagnosis of a left atrial mass.

BACKGROUND: Cardiac masses may be incidentally found or in relation to the clinical presentation. Depending on the complexity of the lesion, a multimodality imaging approach may be necessary for a proper diagnosis. CASE REPORT: We report the case of a 64-year-old male patient who was referred for pre-chemotherapy work-up of a metastatic scrotal melanoma. Echocardiography showed incidental heterogeneous left atrial mass. Further investigation using cardiovascular magnetic resonance imaging, computed tomography angiography, and positron emission tomography confirmed the findings that were consistent with a left atrial myxoma vascularized by a left circumflex coronary-cameral fistula. CONCLUSIONS: When the clinical context may be misleading, assessment of a cardiac mass with a multimodality approach is essential in order to define its exact benign or malignant nature.

Late discovery of left atrial appendage occluder device embolization: a case report.

BACKGROUND: Left atrial appendage (LAA) closure has been well evaluated in the prevention of stroke in patients with atrial fibrillation. Device embolization remains one of the most common complications. To the best of our knowledge, there have been no reports of late discovery of LAA occluder device embolization at 1.5 years after implantation. CASE PRESENTATION: We describe the case of a 77-year-old man who underwent uneventful LAA closure. Echocardiography performed the next day showed the device in place. The patient was discharged but was then lost to follow-up. 1.5 years later, he was admitted for ischemic stroke. Transesophageal echocardiography showed the absence of the occluder device in the LAA. Computed tomography scan of the abdomen showed the device in the abdominal aorta. Due to the high cardiovascular risk, the device was kept in place and the patient was treated medically. CONCLUSIONS: Per-procedural and late device embolization are not uncommon. Review of the literature however showed no report of late discovery of device embolization at 1.5 years. Follow-up echocardiography is mandatory for the detection of endothelialization or embolization.

Value of myocardial work for assessment of myocardial adaptation to increased afterload in patients with high blood pressure at peak exercise.

Non-invasive myocardial work (MW) indices are superior to global longitudinal strain (GLS) to assess left ventricular (LV) mechanics in situations where afterload is abnormally high. The rotational motion of the apex provides an accurate index of LV contractility. To date, there are no data about MW values during stress echocardiography (SE), particularly for the apex. Our study aims at assessing MW indices at peak exercise to better understand LV mechanics in patients with high peak systolic blood pressure (SBP). A total of 81 patients without structural or ischemic heart disease referred for SE between January and June 2019 were included. BP measurements, GLS and MW indices were performed at rest and peak exercise. In order to quantify the role of the apex, apical work fraction (AWF) was also calculated and further stratified by peak SBP. A peak SBP > 180 mmHg was considered abnormally high. There were 36 (44%) patients who had peak SBP > 180 mmHg. They showed similar resting GLS and MW values when compared to control. However, peak stress global work index, constructive work and wasted work (GWW) increased significantly (P-value < 0.05), with a relatively constant global work efficiency. In the multivariate analysis, peak SBP > 180 mmHg was associated with threefold increased odds of abnormal peak GWW > 96.5 mmHg% (median value) (odds ratio 3.0, P-value 0.003). Apical work increased significantly when stratified by peak SBP (P-value 0.003) and AWF increased from 33 ± 3% to 37 ± 3% (rest-stress), P-value < 0.0001, but remained constant when stratified by peak SBP. Our analysis provides new data on MW indices at peak stress, particularly for patients with abnormal peak SBP response, and supports the role and the contribution of the apex to MW.

Association between left ventricular diastolic dysfunction and subclinical coronary artery calcification.

BACKGROUND: Assessment of diastolic dysfunction (DD) by echocardiography is an integral part of the evaluation of patients with normal ejection fraction and symptoms suggestive of heart failure. However, many patients with DD are asymptomatic. Computed tomography calcium scoring (CTCS) is often used to assess patients at low-intermediate risk for coronary artery disease (CAD). The purpose of this study was to evaluate the association of DD with subclinical coronary artery calcification. METHODS: Consecutive patients presenting for executive checkup who underwent resting transthoracic echocardiography followed by CTCS were retrospectively identified between January 2010 and December 2014. Two-dimensional and tissue Doppler imaging parameters were analyzed for assessing and grading of DD. Coronary artery calcium (CAC) score was quantified. RESULTS: A total of 191 patients (mean age 52 ± 12 years, 17% age ≥65, 20% with diabetes) were included. Of them, 69 (36%) patients had DD. Patients with higher CAC score were older, had more comorbidities, lower e', and were more likely to have DD. In the multivariate analysis, DD alone, age >65 years, or both were associated with almost threefold increase of subclinical atherosclerosis. After propensity analysis, DD was still associated with increased odds ratio (OR) for subclinical CAC (OR 3.66 [1.54-8.72], P-value .03) and similarly for e' < 10 cm/s. Compared to patients age <65 years and normal diastolic function, those age >65 years or DD had OR 3.49 (1.45-8.35) (P-value .005) for subclinical coronary atherosclerosis (CAC > 0), whereas those age >65 and DD had OR 9.30 (2.00-42) (P-value .004). CONCLUSIONS: Our analysis suggests that DD was strongly associated with CAC > 0, particularly among those age <65 years. Assessment of CAC as part of the routine clinical evaluation of patients with normal EF and atypical symptoms without a history of coronary atherosclerotic disease is warranted for further risk stratification.

Patients with Isolated Focal Right Ventricular Dyskinetic Segments: Toward a Better Understanding of This Cohort.

BACKGROUND: The 2010 revised Task Force criteria for the diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) provided guidance for the classification of patients as definitive, borderline or possible ARVC. However, many patients with clinical suspicion for ARVC have isolated RV dyskinetic segments only and partly meet cardiac magnetic resonance (CMR) imaging criteria. This subgroup of patients and the implication of this imaging finding remain not well defined. METHODS: There were 65 consecutive patients with clinical suspicion for ARVC who were referred for CMR between 2015 and 2017. The presence of fatty infiltration and fibrosis were assessed using T2 imaging and myocardial delayed enhancement sequences, respectively. RV wall motions, volumes and ejection fraction (EF) of all patients were re-analysed and quantified. Available data on family history, Holter findings, and electrocardiograms were also reviewed. RESULTS: There were 5 patients (7.7%) that fulfilled major CMR criteria for ARVC: 4 were classified as having definitive ARVC; and 1/5 as borderline. There were 33 patients with no RV dyskinetic segments: none were classified as having definitive or borderline ARVC; 4/33 were classified as possible ARVC, leaving 29/33 as normal or no ARVC. Finally, there were 27 remaining patients (41.5%) with isolated RV dyskinetic segments: 1/27 was classified as definitive ARVC; 4/27 as borderline; 8/27 as possible; leaving 15/27 as indeterminate. Compared to control, those with isolated RV dyskinesia (including the subgroup labelled as indeterminate 15/27) had more abnormal RVEF, larger RV end-diastolic volume index (82 ± 12 mL/m² vs. 72 ± 12 mL/m², p-value 0.0127), and a trend for higher odds of dilated RV (odds ratio 3.0 [0.81-11], p-value 0.09). CONCLUSIONS: Among patients with a clinical suspicion for ARVC, almost 40% had isolated focal RV dyskinetic segments with the majority remaining unclassified. This cohort had more RV dilation and abnormal EF compared to control.

Incremental value of stress echocardiography and computed tomography coronary calcium scoring for the diagnosis of coronary artery disease.

Computed tomography coronary angiography (CTCA) has a higher negative predictive value (NPV) for coronary artery disease (CAD) than stress echocardiography (SE). CT calcium scoring (CTCS) allows detection and quantification of coronary artery calcification (CAC). The NPV of combined SE and CTCS for CAD is not well defined. Consecutive patients from the executive screening program who underwent exercise SE and concomitant CTCA were retrospectively identified between January 2010 and December 2014. Patients with normal SE and CAC score of zero were determined, and the presence or absence of any CAD (obstructive or non-obstructive plaques) on CTCA was confirmed. The NPV of combined SE and CTCS was then re-tested using a validation cohort of subsequent consecutive patients enrolled between January 2015 and July 2018. The initial cohort consisted of 173 patients (19% age > 65 years, 19% diabetic); 40% had normal CTCA, 48% with non-obstructive CTCA (77 with CAC score > 0), and 12% with obstructive CTCA (all with CAC score > 0). There were 16 (9.2%) patients with inducible ischemia on SE. A normal SE had a 93% NPV to exclude obstructive CAD but only 42% NPV to exclude any CAD. A combined normal SE and CTCS had a 100% NPV for obstructive CAD, and 92% for any CAD. In a validation cohort of 111 patients, a normal SE and CAC score of zero had NPV of 100% for obstructive CAD and 92% for any CAD. The combined cohort consisted of predominately low Framingham risk patients; more than 40% (70/181) had CAC score > 0 and 5/70 had obstructive CAD, with the remaining non-obstructive. A concomitant normal SE and CAC score of zero excluded obstructive CAD (NPV 100%) and any CAD in 92% of the testing and validation cohorts. CTCS seems to add incremental risk stratification, particularly for patients with low Framingham score.

Free-Floating Right Heart Thrombus with Acute Massive Pulmonary Embolism: A Case Report and Review of the Literature.

Free-floating right heart thrombus (RHT) is an extreme medical emergency in the context of acute massive pulmonary embolism (PE). Despite the advances in early diagnosis, the management is still very debatable due to lack of consensus. We reported the case of a 66-year-old male, with a history of moderate renal dysfunction and dilated cardiomyopathy, who presented to the emergency department for acute dyspnea. His angiographic magnetic resonance imaging revealed bilateral extensive PE. Transthoracic echocardiography showed RHT with moderate right ventricular dysfunction and pulmonary hypertension. Venous Doppler of the lower extremities noted the presence of a floating clot in the right common femoral vein. The patient was managed successfully by thrombolytic therapy with tenecteplase. To the best of our knowledge, this is the first case report of RHT and PE from Lebanon. Published cases from Middle Eastern countries are scarse.

Cardiac involvement in hypereosinophilic syndrome.

A 9-year-old boy with hypereosinophilic syndrome (HES) was referred for cardiac magnetic resonance (CMR) imaging following an abnormal echocardiogram that showed a large mass layered on the inferolateral wall of the left ventricle, causing secondary severe mitral regurgitation. Cardiac involvement in HES usually affects the ventricular apex. In our case, CMR confirmed the presence of a large mural thrombus of 0.9 cm × 4.2 cm. This unusual cardiac involvement in HES was diagnosed in its intermediate thrombotic stage. CMR is very sensitive and specific in staging the disease. It explained the etiology of mitral regurgitation and guided therapy, especially when echocardiography was nonconclusive.

Multimodality imaging for evaluation of chest pain using strain analysis at rest and peak exercise.

BACKGROUND: Exercise stress echocardiography (SE) is commonly used for diagnosing coronary artery disease (CAD). The use of quantitative echocardiographic measures such as strain imaging is expected to improve the sensitivity of conventional SE which relies on the visual diagnosis of wall-motion abnormalities (WMAs). This study is set to demonstrate the added value of resting and exercise strain analysis to detect underlying microangiopathy and inducible myocardial ischemia during a resting and SE in comparison with the results of coronary computed tomographic angiogram (CCTA). METHODS: A total of 103 consecutive outpatients from the executive screening program were identified and underwent resting and SE. Global longitudinal strain (GLS) and wall-motion changes at rest and peak exercise were analyzed and compared. Baseline demographics, heart rate, and blood pressure measurements were retrospectively extracted for analysis. CCTA was chosen as an alternative test to match with wall motion and strain imaging. RESULTS: Patients who had abnormal CCTA had lower resting GLS (14.85% ± 3.05 vs 17.99% ± 2.88, P-value = .001) when compared to patients with normal CCTA. All patients who had abnormal CCTA had higher wall-motion score index (1.35 ± 0.2 vs 1.00, P-value < .0001) and lower and abnormal peak stress GLS (pGLS) as compared to patients with normal CCTA (14.89% ± 3.35 vs 18.44% ± 4.27, P-value = .007). However, all patients with pGLS ≥ 20% had normal/nonobstructive CCTA. Patients with multiple comorbidities showed reduced and lower values of resting and pGLS (P-value < .0001), suggesting associated subclinical left ventricular dysfunction. CONCLUSIONS: Global longitudinal strain offers quantitative interpretation of the resting and SE study, detects underlying subclinical left ventricular dysfunction and a peak stress value more than 20% excludes obstructive CAD on CCTA.

Adult Langerhans cell histiocytosis with pulmonary and colorectoanal involvement: a case report.

BACKGROUND: Langerhans cell histiocytosis is a rare systemic disease characterized by the abnormal overproduction of histiocytes that tend to infiltrate single or multiple organ systems leading to significant tissue damage. It mainly affects - by order of decreasing frequency - the bone, the skin, the lymph nodes, the liver, and lungs. Gastrointestinal tract involvement is extremely rare in adults. CASE PRESENTATION: We describe the case of a 32-year-old Middle Eastern man with Langerhans cell histiocytosis involving his lungs and the colorectoanal part of his gastrointestinal tract, with complete resolution of gastrointestinal tract lesions following a non-standardized chemotherapy regimen. CONCLUSIONS: Gastrointestinal tract lesions are a rare manifestation of Langerhans cell histiocytosis, especially when associated with extraintestinal involvement, such as the lungs. Chemotherapy protocols have not been well established for the treatment of the disease. The clinical impact of the effective chemotherapy regimen used to treat this uncommon presentation of Langerhans cell histiocytosis will be viewed in this case report.

Unusual digoxin toxicity with myocardial injury.

A 38-year-old healthy male presented with vomiting and profuse diarrhea, associated with blurry and yellow coloration of the vision (xanthopsia). Laboratory workup was unremarkable, except for hyperkalemia (K 5.2 mEq/L) and mildly elevated troponin level 0.11 ng/mL (cut-off value 0.08). An electrocardiogram showed sinus bradycardia with deep scooping of the T waves. Although the patient denied intake of any drugs, herbs, consumption of plants, a digoxin level was drawn and was significantly elevated >5ng/mL (therapeutic range 0.8-2.0). Further questioning revealed that the patient was a pharmacist mixing raw material to fabricate medication, and that he could have incidentally ingested contaminated water. His symptoms improved with parallel improvement in the electrocardiogram T wave abnormalities. An echocardiogram was normal. The positive troponin was felt to be secondary to severe digoxin toxicity. Review of the literature however showed no report of elevated troponin in the setting of digoxin toxicity.