Cardiac Manifestations in Inherited Metabolic Diseases.

Inherited metabolic diseases (IMDs) stem from genetic defects affecting enzyme function within specific metabolic pathways, collectively constituting rare conditions with an incidence of less than 1/100,000 births. While IMDs typically manifest with multisystemic symptoms, cardiac manifestations are common, notably hypertrophic cardiomyopathy. Additionally, they can lead to dilated or restrictive cardiomyopathy, as well as noncompacted left ventricular cardiomyopathy. Rhythm disturbances such as atrioventricular conduction abnormalities, Wolff-Parkinson-White syndrome, and ventricular arrhythmias, along with valvular pathologies and ischemic coronary issues, are also prevalent. This study aims to provide a narrative review of IMDs associated with cardiac involvement, delineating the specific cardiac manifestations of each disorder alongside systemic symptoms pivotal for diagnosis.

Utility of Cerebral Embolic Protection Devices in Transcatheter Procedures: A Systematic Review and Meta-Analysis.

With the emergence of the largest randomized control trial to date-the Stroke Protection With Sentinel During Transcatheter Aortic Valve Replacement (PROTECTED TAVR) study-we sought to conduct an updated meta-analyses to evaluate the utility of CEP devices on both clinical outcomes and neuroimaging parameters. Electronic databases were queried through November 2022 for clinical trials comparing the utility of Cerebral Embolic Protection (CEP) devices in Transcatheter Aortic Valve Replacement (TAVR) with non-CEP TAVR procedures. Meta-analyses were performed using the generic inverse variance technique, and a random-effects model, and results are presented as weighted mean differences (WMD) for continuous outcomes, and hazard ratios (HR) for dichotomous outcomes. Outcomes of interest included stroke, disabling stroke, nondisabling stroke, bleeding, mortality, vascular complications, new ischemic lesions, acute kidney injury (AKI), and total lesion volume. Thirteen studies (8 RCTs, 5 observational studies) consisting of 128,471 patients were included in the analysis. Results from our meta-analyses showed a significant reduction in stroke (OR: 0.84 [0.74-0.95]; P < 0.01; I2 = 0%), disabling stroke (OR: 0.37 [0.21-0.67]; P < 0.01; I2 = 0%) and bleeding events (OR: 0.91 [0.83-0.99]; P = 0.04; I2 = 0%) through CEP device use in TAVR. The use of CEP devices had no significant impact on nondisabling stroke (OR: 0.94 [0.65-1.37]; P < 0.01; I2 = 0%), mortality (OR: 0.78 [0.53-1.14]; P < 0.01; I2 = 17%), vascular complications (OR: 0.99 [0.63-1.57]; P < 0.01; I2 = 28%), AKI (OR: 0.78 [0.46-1.32]; P < 0.01; I2 = 0%), new ischemic lesions (MD: -1.72 [-4.01, 0.57]; P < 0.001; I2 = 95%) and total lesion volume (MD: -46.11 [-97.38, 5.16]; P < 0.001; I2 = 81%). The results suggest that CEP device use was associated with a lower risk of disabling stroke and bleeding events in patients undergoing TAVR.

Intensive Cardiac Rehabilitation Attenuates the Gender Gap in Cardiac Rehabilitation Participation.

Traditional cardiac rehabilitation (CR) programs historically have had considerable gender gaps in CR outcomes. Intensive-Cardiac-Rehabilitation (ICR) has nonexercise components such as stress management, peer support and diet modification, in addition to exercise, which may reduce barriers to CR completion. However, there is a paucity of data in ICR outcomes based on gender. We conducted a nationwide retrospective cohort study of 15,613 patients enrolled in 46 Ornish-ICR programs across the United States from January 2016 to December 2020 and compared ICR physical and psychological outcomes in women vs men. Primary endpoints were depression scores (CESD), health status (SF-36 physical and mental composite-scores) and exercise minutes per week. Of 15,613 ICR participants who participated in the program, 6788 (44%) were women. ICR completion rates were 64.7% overall and nearly equal between men and women (63.3% women vs 65.9% men). There were a few small differences in outcomes after ICR completion: women had slightly more improvement in depression scores (women: -6.10 ± 8.79, 49.6% reduction vs men: -4.92 ± 7.80, 46.4% reduction; P < 0.01) and SF-36 physical (women: 5.95 ± 6.86 vs men: 5.28 ± 6.71, P < 0.01) and SF-36 mental (women: 5.76 ± 8.11 vs men: 4.60 ± 7.33, P < 0.01) composite scores than men. There was no significant difference in exercise-minutes per week between the 2 groups. ICR attenuates the gender gap in CR completion. Further, of the completers, women achieved slightly higher depression and quality-of-life improvements compared to men. As such, ICR can be a good option to reduce gender-based differences in CR participation.

Trajectory of Decongestion and Mortality in Young Adults with Acute Heart Failure.

Although the prevalence of HF in young adults (age <50 years) is increasing, there are limited data on the trajectory of decongestion and short-term outcomes in young adults with acute heart failure (AHF). We pooled patients from 3 randomized trials of AHF conducted within the Heart Failure Network (the Diuretic Optimization Strategies trial, the Renal Optimization Strategies Trial, and the Cardiorenal Rescue Study in Acute Decompensated Heart Failure). The association between young age (<50 years and >50 years) and in-hospital changes in various measures of decongestion as well as short-term outcomes including risk for rehospitalization, and all-cause mortality was evaluated. Of 762 patients, 72 (10.3%) patients were young. Young adults were more likely to be African American (53.8% vs 19.3%), to have a lower rate of ischemic HF etiology (25.6% vs 60.4%, P <0.001), and a lower burden of hypertension, chronic kidney disease and atrial fibrillation. Young adults had a lower left ventricular ejection fraction (median 20% vs 33%, P < 0.001); they had a higher admission weight (median 242.7 lbs vs 201.5 lbs, P < 0.001), but lower NT-pro BNP levels (median 3622 pg/mL vs 4676 pg/mL, P = 0.003). After covariate adjustment, there was no difference in the change in NT-pro BNP (P = 0.25), net fluid loss (P = 0.42), or renal function (P = 0.56) between young and older adults by 72 or 96 hours of randomization. There was no difference in orthodema congestion score or the composite clinical endpoint during the follow-up (all-cause mortality or any rehospitalization) (adjusted odds ratios (95% confidence intervals): 2.51 (0.78-8.01), P = 0.12). In this pooled analysis of 3 clinical trial cohorts, compared with older adults, younger adults had a unique demographic and clinical profile. Despite these differences, there was no difference by age group in in-hospital decongestion or post-discharge readmission or mortality.

Outcomes of hospitalizations with acute respiratory distress syndrome with and without atrial fibrillation - Analyses from the National Inpatient Sample (2004-2014).

BACKGROUND: Acute respiratory distress syndrome (ARDS) is associated with high mortality. Atrial fibrillation (AF) is a common arrhythmia seen in critically ill patients. The impact of AF on the outcomes in patients with ARDS is less understood. In this analysis we attempt to evaluate the association of concurrent AF and various clinical outcomes in patients with ARDS. METHODS: We conducted a retrospective analysis of adult discharges from the National Inpatient Sample (NIS) between 2004 and 2014. International Classification of Disease codes were used to identify those with ARDS and AF. RESULTS: We found 1,200,737 hospitalizations with ARDS, out of which 238,455 had concomitant diagnosis of AF. Hospitalizations with AF had higher prevalence of comorbidities including chronic pulmonary disease, diabetes mellitus, hypertension, obesity, congestive heart failure and renal failure. On adjusted analysis, AF was associated with increased odds of acute myocardial infarction, cardiogenic shock, pressor use, acute kidney injury, permanent pacemaker implantation, cardiac arrest, mechanical circulatory support use and higher length of stay and inflation-adjusted cost in hospitalizations with ARDS. However, there was no significant difference in adjusted all-cause mortality in ARDS with and without AF (25.42% vs 20.23%, p=0.53). CONCLUSIONS: AF is associated with worse clinical outcomes, higher length of stay and cost in ARDS hospitalizations as compared to those without AF.

Left Atrial Appendage Closure Versus Oral Anticoagulation in Non-Valvular Atrial Fibrillation: A Systematic Review and Meta-Analysis.

BACKGROUND: Left atrial appendage closure (LAAC) devices are an alternative therapy in non-valvular atrial fibrillation (NVAF) patients with contraindications to oral anticoagulation (OAC). However, there are limited data about the clinical outcomes of LAAC devices compared to medical treatment. METHODS: A comprehensive research for studies comparing LAAC devices and OAC for patients with NVAF was performed from inception to January 1, 2021. A meta-analysis was performed using a random effect model to calculate odds ratios (OR) with 95% confidence intervals (CIs). RESULTS: Five studies were eligible that included a total of 4778 patients with a median-weighted follow-up period was 2.6 years. Compared to OAC, the LAAC device arm was associated with a lower risk of the composite of stroke, systemic embolism, and cardiovascular death (OR 0.71; 95% CI 0.51-1.00; p = 0.05). LAAC device arm was also associated with a lower risk of all-cause mortality (OR of 0.60, 95% CI 0.46-0.77; p < 0.0001), cardiovascular mortality (OR of 0.57, 95% CI 0.46-0.70; p < 0.00001), hemorrhagic stroke (OR of 0.19, 95% CI 0.07-0.50; p= 0.0006), all major bleeding (OR of 0.61, 95% CI 0.43-0.88; p = 0.007) and non-procedural major bleeding (OR of 0.46, 95% CI 0.32-0.65; p < 0.0001). There was no significant difference in all stroke, ischemic stroke, and systemic embolization between the two groups. CONCLUSIONS: Our meta-analysis showed lower all-cause mortality, cardiovascular mortality, hemorrhagic stroke, major bleeding, non-procedural major bleeding and the composite of stroke, systemic embolism, and cardiovascular death in the LAAC device arm when compared to OAC. However, the risk of all stroke, ischemic stroke, and systemic embolism were similar between the two arms.

Correlating Toe-Brachial Indices and Angiographically Confirmed Peripheral Artery Disease: A Retrospective Review.

In advanced peripheral arterial disease (PAD), medial arterial calcification is known to inflate the ankle-brachial index. An alternative method of evaluating symptomatic patients is toe-brachial indexes (TBI), where a ratio less than .7 indicates PAD and less than .4 indicates a severe form. The objective of this retrospective analysis was to investigate the association between TBIs less than .7 and angiographically verified PAD. Patients were required to have either a leg angiogram 13 months prior to or 12 months after a 6-minute walk test. Of the 174 included patients, the mean overall TBI was .450. The mean TBI by location was highest at iliac and infra-geniculate with .544 and lowest at supra-geniculate with .372. Infra-geniculate lesions were also the most frequent (n = 46). A TBI less than .4 was found in 47.7% of patients. TBIs greater than .7 were present in 36 patients; however, only 16 had significant angiographic stenosis. In conclusion, the majority of patients with angiographic PAD had a TBI less than .7, especially less than .4. Contrary to suspicion, infra-geniculate lesions were the most common and had the highest TBI.

Atrial Fibrillation as a Predictor of Mortality in High Risk COVID-19 Patients: A Multicentre Study of 171 Patients.

PURPOSE: Certain patient demographics and biomarkers have been suggested to predict survival in patients infected with COVID-19. However, predictors of outcome in patients who are critically ill are unclear. MATERIALS AND METHODS: We performed a multicentre analysis of 171 consecutive patients with confirmed COVID-19 who were admitted to the intensive care unit (ICU) between 1 March 2020 and 30 April 2020 and were followed until 23 May 2020. Demographic data, past medical history, laboratory values, echocardiographic and telemetry data were analysed. Patient status was classified as either alive or deceased at hospital discharge or the end of follow-up period. RESULTS: Mean patient age was 66±13 and 57% were male. Mortality rate of this ICU cohort at the end of follow-up was 46.2%. A multivariable logistic regression analysis identified the presence or history of atrial fibrillation (Odds Ratio 4.8, p=0.004) as a significant cardiovascular attribute that contributed to increased mortality. CONCLUSION: Mortality of critically ill COVID-19 patients is high. This study suggests a relationship between atrial fibrillation and increased mortality from COVID-19. Early aggressive treatment patients with high risk characteristics, such as atrial fibrillation could improve clinical outcome.

Meta-Analysis of Valve-in-Valve Transcatheter Aortic Valve Implantation Versus Redo-surgical Aortic Valve Replacement in Failed Bioprosthetic Aortic Valve.

This meta-analysis was conducted to compare clinical outcomes of valve-in-valve transcatheter aortic valve implantation (ViV-TAVI) versus redo-surgical aortic valve replacement (Redo-SAVR) in failed bioprosthetic aortic valves. We conducted a comprehensive review of previous publications of all relevant studies through August 2020. Twelve observational studies were included with a total of 8,430 patients, and a median-weighted follow-up period of 1.74 years. A pooled analysis of the data showed no significant difference in all-cause mortality (OR 1.15; 95% CI 0.93 to 1.43; p = 0.21), cardiovascular mortality, myocardial infarction, permanent pacemaker implantation, and the rate of moderate to severe paravalvular leakage between ViV-TAVI and Redo-SAVR groups. The rate of major bleeding (OR 0.36; 95% CI 0.16 to 0.83, p = 0.02), procedural mortality (OR 0.41; 95% CI 0.18 to 0.96, p = 0.04), 30-day mortality (OR 0.58; 95% CI 0.45 to 0.74, p <0.0001), and the rate of stroke (OR 0.65; 95% CI 0.52 to 0.81, p = 0.0001) were significantly lower in the ViV- TAVI arm when compared with Redo-SAVR arm. The mean transvalvular pressure gradient was significantly higher post-implantation in the ViV-TAVI group when compared with the Redo-SAVR arm (Mean difference 3.92; 95% CI 1.97 to 5.88, p < 0.0001). In conclusion, compared with Redo-SAVR, ViV-TAVI is associated with a similar risk of all-cause mortality, cardiovascular mortality, myocardial infarction, permanent pacemaker implantation, and the rate of moderate to severe paravalvular leakage. However, the rate of major bleeding, stroke, procedural mortality and 30-day mortality were significantly lower in the ViV-TAVI group when compared with Redo-SAVR.

Case Report of High-Grade Atrioventricular Block and Takotsubo Cardiomyopathy.

INTRODUCTION: Takotsubo cardiomyopathy (TTC) is a condition with a good long-term prognosis. However, when the TTC is due to a life-threatening arrhythmia, such as atrioventricular block (AVB), several considerations must be made regarding treatment. CASE PRESENTATION: A 71-year-old woman with a history of ischemic stroke presented after a syncopal episode. Before passing out, the patient was walking, nauseous, lightheaded, dizzy, and short of breath. In the emergency department, the blood pressure was 230/120 mmHg, and the heart rate was 38 beats per minute, but the patient was asymptomatic. An electrocardiogram showed a new-onset 2:1 AVB, bifascicular block, and prolonged PR and corrected QT intervals. An echocardiogram revealed a new-onset ejection fraction of 30% to 35%; hypokinesis of the apex, mid-inferoseptum, mid-anterolateral, apical to mid-inferior, and apical to mid-anterior walls; and hyperkinesis of the basal segments. The cardiac catheterization illustrated normal coronary arteries without significant stenosis. Therefore, the patient was diagnosed with TTC and 2:1 AVB. She was treated with lisinopril and metoprolol succinate and received a dual-chamber pacemaker. At the follow-up visit, the patient's ejection fraction and hypokinetic segments improved. She denied any recurrence of syncope, and her pacemaker was functioning appropriately. CONCLUSION: When AVB or other arrhythmias initiate a TTC, the patient can experience sudden cardiac death and decompensate quickly. Therefore, clinicians should understand this rare but fatal complication because these patients require pacemakers and beta blockers.

Clinical Outcomes of the Self-Expandable Evolut R Valve Versus the Balloon-Expandable SAPIEN 3 Valve in Transcatheter Aortic Valve Implantation: A Meta-Analysis and Systematic Review.

BACKGROUND: Transcatheter aortic valve replacement (TAVR) is now indicated in patients with symptomatic aortic stenosis and low, moderate, and high surgical risk. There are multiple types of valves available in TAVR. SAPIEN 3, and Evolut R are two of the most commonly used valves. METHODS: We conducted a systematic review and meta-analysis of all studies that compared SAPIEN 3 vs Evolut R in patients undergoing TAVR. The primary endpoint of this meta-analysis was 30-day mortality. Secondary outcomes included major of life-threatening bleeding, risk of stroke, need of permanent pacemaker implantation, and risk of moderate to severe paravalvular regurgitation (PVR). RESULTS: We included a total of 9 studies. One study was a randomized clinical trial, five were prospective observational studies and three were retrospective. 30-day mortality rate was similar between SAPIEN 3 and Evolut R (odds ratio (OR) 1.19; 95% confidence interval (CI) 0.72 to 1.93; p = 0.47). The risk of major or life-threatening bleeding (OR of 0.83, 95% CI 0.50 to 1.39; p = 0.48), and the risk of stroke (OR of 0.82, 95% CI 0.38 to 1.78; p = 0.62) were also similar between the two types of valves. Compared to SAPIEN 3, Evolut R was associated with statistically significant risk of permanent pacemaker implantation (OR of 1.40, 95% CI 1.15 to 1.70; p = 0.0007), and moderate to severe PVR (OR of 2.56, 95% CI 1.14 to 5.74; p = 0.02). CONCLUSIONS: At 30 day follow up, both Evolut R and SAPIEN 3 shared similar risks of 30-day mortality, major or life-threatening bleeding, and stroke; however greater odds of pacemaker placement implantation and moderate to severe PVR were associated with Evolut R.

The rare presentation of the de Winter's pattern: Case report and literature review.

Although not classified as a ST elevated myocardial infarction (STEMI), the patterns known as equivalents also require prompt recognition and treatment. A 50-year-old male with no pertinent history presented to the emergency department for chest pain that radiated to his left shoulder. An electrocardiogram (EKG) revealed findings consistent with the de Winter's pattern, which were greater than 1 mm upsloping ST depressions at the J point in leads V3-V6 (maximally in leads V3-V5), tall, peaked T waves in leads II, III, and V3-V5, ST elevations in lead aVR, and 1 mm ST elevation in V1 and V2. The physical exam, troponins, and other laboratory investigations were unrevealing. Urgent, diagnostic coronary angiography revealed complete occlusion of the proximal left anterior descending (LAD) artery, which was successfully treated with percutaneous coronary intervention (PCI) and two drug-eluting stents. After the stent placement, arterial blood flow was re-established and the ECG normalized. The patient was started on guideline based treatment and discharged home once medically stable. The de Winter's pattern on electrocardiogram indicates a significant coronary artery disease. This pattern requires urgent intervention, typically percutaneous stent placement.

Reining in Sternal Wound Infections: The Achilles' Heel of Bilateral Internal Thoracic Artery Grafting.

Background: Although the survival advantage of bilateral internal thoracic artery grafting (BITA) is well known in patients undergoing coronary artery bypass grafting (CABG), this technique has not been widely adopted. This is mainly because of the increased risk of deep sternal wound infections (DSWI) associated with its use. However, in recent years the overall risk of DSWI has decreased. This is mainly because of strategies that have been adopted to decrease the risk of these infections in patients undergoing CABG. Conclusion: In this review we identified DSWI preventive strategies and described them in detail so that their use by surgeons can be increased. This would minimize the risk of DSWI after BITA grafting and maximize the use of this highly effective surgical technique.

May-Thurner Syndrome Presenting as Acute Unexplained Deep Venous Thrombosis.

May-Thurner syndrome (MTS) is a rare cause of deep venous thrombosis and occurs due to an anatomic anomaly which produces chronic compression of the left common iliac vein by the overlying right common iliac artery when it passes between the right common iliac artery and the spine. Prolonged compression on the vein potentiates thrombus formation by impairing the intima and by leading to the development of membranes within the lumen that may decrease and/or block venous flow. In this case presentation, we elaborate on a case of a 43-year-old woman who presented with worsening left leg swelling and pain. The patient was diagnosed with MTS and underwent successful stent placement to relieve the compressed vein.

Cardiopulmonary Exercise Testing Limitation in Peripheral Arterial Disease.

BACKGROUND: Peripheral artery disease (PAD) is associated with a markedly increased risk of cardiovascular mortality and remains underrecognized and undertreated. New screening approaches are needed to prevent disability in patients with PAD. METHODS: We performed a retrospective analysis of clinically stable cardiovascular patients who were referred for cardiopulmonary exercise testing (CPET) due to a variety of cardiovascular complaints. Angiograms were assessed for patients who prematurely terminated CPET. Estimated functional capacity in metabolic equivalents was also obtained from monitored bicycle exercise at the visit. RESULTS: According to our analysis, 351 patients were unable to complete the CPET test due to some limiting factor. Of these patients, a total of 216 had available angiographic assessment conducted, of whom 42.6% were males, 57.4% were females, and the mean age was 67 years. A total of 135 patients with a similar gender distribution and a mean age of 53 years did not undergo angiography for various reasons including feasibility. Most patients who underwent assessment were found to have lesions. Across all patients, 284 arterial lesions were identified: 55 were iliac, 67 were femoral, and 162 were below the knee. PAD was diagnosed in 165 of these lesions. There was a significant difference observed in the VO2 max in patients with lesions depending on location of lesion. In all vessel groups, the existence of PAD was associated with a significantly reduced VO2 max during CPET testing. CONCLUSIONS: The data obtained in this study provides a preliminary understanding of the underlying effects of PAD and provides a basis for further utilization of CPET termination as a potential screening signal for further PAD investigation in appropriate patients.

Dasatinib-induced chylothorax in chronic myeloid leukemia.

Pulmonary adverse events are common abnormalities associated with the use of dasatinib in chronic myeloid leukemia. We present a case of a 69-year-old man who suddenly developed a rare chylothorax pulmonary adverse event following 10 months of dasatinib treatment.