Cardiac Sarcoidosis.

Cardiac involvement is a major cause of morbidity and mortality in patients with sarcoidosis. It is important to distinguish between clinical manifest diseases from clinically silent diseases. Advanced cardiac imaging studies are crucial in the diagnostic pathway. In suspected isolated cardiac sarcoidosis, it's key to rule out alternative diagnoses. Therapeutic options can be divided into immunosuppressive agents, guideline-directed medical therapy, antiarrhythmic medications, device/ablation therapy, and heart transplantation.

Lymphocytic Perimyocarditis Masquerading as Steroid-Dependent Recurrent Pericarditis.

Lymphocytic myocarditis is a pattern of myocardial inflammation typically associated with viral, autoimmune, or idiopathic causes. We present a case of lymphocytic perimyocarditis masquerading as steroid-dependent recurrent pericarditis. This case shows the advantages of using multimodal cardiac imaging and endomyocardial biopsy in clarifying diagnosis in treatment-resistant cases. (Level of Difficulty: Advanced.).

Optimal left ventricular ejection fraction in risk stratification of patients with cardiac sarcoidosis.

AIMS: Identifying patients with cardiac sarcoidosis (CS) who are at an increased risk of sudden cardiac death (SCD) poses a clinical challenge. We sought to identify the optimal cutoff for left ventricular ejection fraction (LVEF) in predicting ventricular arrhythmia (VA) and all-cause mortality and to identify clinical and imaging risk factors in patients with known CS. METHODS AND RESULTS: This retrospective cohort included 273 patients with well-established CS. The primary endpoint was a composite of VA and all-cause mortality. A modified receiver operating curve analysis was utilized to identify the optimal cutoff for LVEF in predicting the primary composite endpoint. Cox proportional hazard regression analysis was used to identify independent risk factors of the outcomes. At median follow-up of 7.9 years, the rate of the primary endpoint was 38% (83 VAs and 32 all-cause deaths). The 5-year overall survival rate was 97%. The optimal cutoff LVEF for the primary composite endpoint was 42% in the entire cohort and in subjects without a history of VA. Younger age, history of VA, lower LVEF, and any presence of scar by cardiac magnetic resonance (CMR) imaging and/or positron emission tomography (PET) were found to be independent risk factors for the primary endpoint and for VA, whereas lower LVEF, baseline NT-proBNP, and any presence of scar were independent risk factor of all-cause mortality. CONCLUSION: Among patients with CS, a mild reduction in LVEF of 42% was identified as the optimal cutoff for predicting VA and all-cause mortality. Prior VA and scar by CMR or PET are strong risk factors for future VA and all-cause mortality.

Association of timing of percutaneous left ventricular assist device insertion with outcomes in patients undergoing cardiac surgery.

Objectives: The aim of this study was to explore the associations between percutaneous ventricular assist device (pVAD) insertion timing relative to cardiac surgery and patient outcomes. Methods: The Nationwide Inpatient Sample was queried for patients undergoing cardiac surgery and pVAD insertion in the same admission from 2016 to 2019. Patients were stratified by timing of pVAD insertion. Preoperative characteristics, postoperative complications, and mortality were compared among groups. Results: Overall, 3695 patients underwent cardiac surgery and pVAD insertion during the same hospitalization (pre: 1130, intra: 1690, and post: 875). The distribution of cardiac surgery procedures was similar across groups. Median Elixhauser Comorbidity Index was 13 for pre-, 15 for intra-, and 17 for postoperative pVAD patients (P = .021). Patients who received a postoperative pVAD were associated with increased mortality (pre: 18%, intra: 39%, and post: 54%; P < .01). Increased complication rates were also associated with postoperative pVAD insertion (pre: 61%, intra: 55%, and post: 75%; P < .01). Preoperative pVAD insertion was associated with increase rates of sepsis (pre: 18%, intra: 9.8%, and post: 17%; P = .01) and pneumonia (pre: 38%, intra: 23%, and post: 31%; P < .01). Postoperative pVAD insertion was associated with increased rates of gastrointestinal bleeding (pre: 2.2%, intra: 3.0%, and post: 7.4%; P = .01), renal failure (pre: 10%, intra: 9.2%, and post: 17%; P = .01), and prolonged ventilation (pre: 44%, intra: 41%, and post: 54%; P = .02). Conclusions: Postoperative pVAD insertion following cardiac surgery was associated with increased complications and mortality compared with preoperative or intraoperative insertion. Further studies should explore optimal utilization and timing of pVAD insertion in patients undergoing cardiac surgery.

A Pragmatic Approach to Weaning Temporary Mechanical Circulatory Support: A State-of-the-Art Review.

Temporary mechanical circulatory support (TMCS) provides short-term support to patients with or at risk of refractory cardiogenic shock. Although indications, contraindications, and complications of TMCS may guide device selection, optimal strategies for device weaning and explant remain poorly defined. Under the revised adult heart allocation policy implemented by the United Nations for Organ Sharing in October 2018, rejustification of heart transplant listing status includes demonstrating TMCS dependency with attempted device wean trials. However, standardized device-specific weaning and explant protocols have not been proposed or evaluated. This review highlights when to use percutaneous TMCS in cardiogenic shock, with a focus on weaning and explant considerations. Terminology for important concepts that guide device escalation, de-escalation, and explantation have been defined. Clinical, hemodynamic, metabolic, and imaging features have been defined, which can guide a tailored approach to TMCS weaning and explant based on the approach used at the Cleveland Clinic. A narrative review of published studies that have reported TMCS weaning protocols and survey results of member centers from CS-MCS working group centers is also provided. Future research is needed to better understand optimal timing and implementation of standardized protocols to achieve successful TMCS weaning and explant.

Outcomes of Repeat Left Ventricular Assist Device Exchange.

Implantable continuous-flow left ventricular assist devices (CF-LVADs) are used for long-term LV support in bridging patients to heart transplantation or as destination therapy. With prolonged support times, some patients will have repeat complications necessitating multiple device exchanges. To elucidate the safety and efficacy of repeat device exchange, we retrospectively reviewed data from 25 patients who underwent two or more CF-LVAD implantations between July 2005 and August 2017. Indications for exchange were thrombus/hemolysis (n = 8, 32%), electromechanical device malfunction (n = 14, 56%), and infection (n = 3, 12%). The implanted devices were the HeartMate II (n = 13, 52%), the HeartWare HVAD (n = 11, 44%), and the Jarvik 2000 (n = 1, 4%). Average hospital length of stay was 44 days (range 4-221 days), and 17 patients (68%) survived to discharge. Average duration of support after the most recent LVAD implantation was 802 days (range 1-3,229 days). Overall survival was 72% at 1 year and 60% at 2 years. Postoperative complications included respiratory failure in five patients (20%), device infection in five (20%), bleeding requiring reoperation in four (16%), neurologic dysfunction in four (16%), and acute renal failure in two (8%). Overall, our data suggest that repeat LVAD exchange is a feasible option for patients with recurrent device-related complications.

Assessment and treatment of the failing right heart: considerations for transplantation referral.

Long considered an insignificant chamber, the right ventricle has been recently under spotlight, especially with emerging data supporting its critical role in disease progression. The right ventricle is a triangular heart chamber with complex physiology and pathophysiology. In this review, we discuss the normal physiology of the right ventricle, right ventricular failure, and recent advances in treatment with emphasis on optimal timing for transplantation referral.

Use of Remote Pulmonary Artery Pressure Monitoring (CardioMEMS System) in Total Artificial Heart to Assess Pulmonary Hemodynamics for Heart Transplantation.

The temporary total artificial heart (TAH-t) has been valuable as a bridge to transplantation in patients with biventricular failure. However, the challenges of accurately assessing pulmonary vascular resistance after TAH-t implantation can preclude these patients from heart transplantation, especially those with pre-existing pulmonary hypertension. The CardioMEMS Heart Failure System (St. Jude's Medical, Little Canada, MN) comprises a wireless pressure sensor that is implanted percutaneously in the pulmonary artery and transmits real-time measurements of pulmonary artery pressures. Systolic and diastolic pulmonary artery (PA) pressures measurements have been well correlated between the CardioMEMS PA Sensor and traditional Swan-Ganz catheter and between the CardioMEMS PA Sensor and standard echocardiography. Here, we report the use of the CardioMEMS device in a patient with severe pulmonary hypertension supported with a SynCardia TAH-t (Tucson, AZ) during assessment for candidacy for transplantation.

Erythrocyte aging as a mechanism of anemia and a biomarker of device thrombosis in continuous-flow left ventricular assist devices.

BACKGROUND: Blood trauma caused by continuous-flow left ventricular assist devices (CF-LVADs) has been associated with device thrombosis and anemia. Accurate in vivo quantification of erythrocyte turnover and its contribution to CF-LVAD complications have yet to be elucidated. METHODS: We investigated the age (lifespan) of circulating erythrocytes in subjects with CF-LVAD. Erythrocyte lifespan is a quantitative indicator of in vivo erythrocyte turnover that can be accurately derived from measurement of the exhaled carbon monoxide (CO) level. Sixty non-smoking subjects were prospectively enrolled: 25 had a CF-LVAD without thrombosis; 10 had a CF-LVAD with thrombosis; and 25 were normal controls. End-tidal breath CO levels were measured and used to calculate erythrocyte lifespan. RESULTS: The mean erythrocyte lifespan was significantly shorter in CF-LVAD subjects with (29.7 ± 14.9 days) compared to those without (65.0 ± 17.3 days) device thrombosis (p < 0.0001). The lifespans in these 2 groups were significantly shorter compared with normal controls (96.0 ± 24.9 days, both p < 0.0001). A receiver operator curve demonstrated high sensitivity-specificity for use of erythrocyte lifespan to detect device thrombosis (AUC = 0.94). In addition, all CF-LVAD subjects had low hemoglobin (11.8 ± 2.0 g/dl), and their anemia was normochromic normocytic with elevated mean reticulocyte counts. Erythrocyte lifespan correlated significantly with mean corpuscular hemoglobin concentration (r = 0.56, p = 0.0005) and red cell distribution width (r = -0.65, p < 0.001), but not with reticulocyte count (r = 0.27, p = 0.32). CONCLUSIONS: Erythrocyte lifespan is substantially reduced in subjects with a CF-LVAD, which was more pronounced in the presence of device thrombosis. The etiology of anemia in CF-LVAD was primarily due to accelerated erythrocyte aging. Further studies are needed to determine whether erythrocyte lifespan could provide a practical means of detecting subtle pre-clinical thrombosis.

Lung transplantation for pulmonary sarcoidosis. Twenty-five years of experience in the USA.

OBJECTIVE: Lung transplantation is the ultimate treatment for end-stage pulmonary sarcoidosis. Post-transplant survival outcomes remain unclear. METHODS: Survival models were used to assess survival and graft outcomes in patients with sarcoid among 20,896 lung transplants performed in the USA. RESULTS: 695 lung recipients were transplanted for pulmonary sarcoidosis. Sarcoid lung recipients had similar median survival rate (69.7 months (IQR 60.2-79.3)) compared with the non-sarcoid lung recipients (63.1 months (IQR 61.4-64.8), p=0.88). In multivariate Cox regression, sarcoidosis was not independently associated with worse mortality (HR 0.96 (95% CI 0.85 to 1.08), p=0.51). Among the sarcoid lung recipients, double lung transplantation (HR 0.76 (0.58 to 0.99), p=0.04) and lung allocation score era (HR 0.74 (0.56 to 0.97), p=0.03) were associated with improved survival. CONCLUSIONS: Recipients of lung transplants for pulmonary sarcoidosis had similar outcomes compared with non-sarcoid lung recipients.

Impact of pre-transplant pulmonary hypertension on survival after heart transplantation: a UNOS registry analysis.

INTRODUCTION: Severe pre-transplant pulmonary hypertension (PH) has been associated with adverse short-term clinical outcomes after heart transplantation in relatively small single-center studies. The impact of pre-transplant PH on long-term survival after heart transplantation has not been examined in a large, multi-center cohort. METHODS: Adults (≥18 years) who underwent first time heart transplantation in the United States between 1987 and 2012 were retrospectively identified from the United Network for Organ Sharing registry. Pre-transplant PH was classified as mild, moderate, or severe based on pulmonary vascular resistance (PVR), trans-pulmonary gradient (TPG), and pulmonary artery (PA) mean pressure. Primary outcome was all-cause mortality. RESULTS: Data from 26,649 heart transplant recipients (mean age 52±12 years; 76% male; 76% Caucasian) were analyzed. During a mean follow-up of 5.7±4.8 years, there were 10,334 (39%) deaths. Pre-transplant PH (PVR≥2.5 WU) was a significant predictor of mortality (hazard ratio 1.10, 95% confidence interval 1.05-1.14, p<0.0001) in multivariable analysis. However, the severity of pre-transplant PH (mild/moderate vs. severe) did not affect short or long-term survival. Similarly, even in patients who were supported with either a left ventricular assist device or a total artificial heart prior to transplant, severe pre-transplant PH was not associated with worse survival when compared to patients with mild/moderate pre-transplant PH. CONCLUSION: Pre-transplant PH (PVR≥2.5 WU) is associated with a modest increase in mortality when compared to patients without pre-transplant PH. However, the severity of pre-transplant PH, assessed by PVR, TPG, or mean PA pressure, is not a discriminating factor for poor survival in patients listed for heart transplantation.

Incidence, predictors, and temporal trends of sudden cardiac death after heart transplantation.

BACKGROUND: Sudden cardiac death (SCD) has been reported to be a significant mode of death after heart transplantation (HT) in small case series. However, the incidence, timing, and predictors of SCD have not been examined in a large multicenter HT cohort. OBJECTIVE: The purpose of this study was to examine the incidence, timing, predictors, and temporal trends of SCD after HT. METHODS: Adults (≥18 years) who underwent first-time HT in the United States between 1987 and 2012 were retrospectively identified from the United Network for Organ Sharing (UNOS) registry. Patients with sudden cardiac arrest as the primary cause of death constituted the SCD group. RESULTS: Data on 37,492 HT recipients (mean age 51.9 ± 11.7 years, 77% male, 78% Caucasian) were analyzed. During mean follow-up of 6.5 ± 5.7 years, there were 17,324 (46%) deaths, of which 1659 (9.6%) were SCD. On multivariate Cox regression analysis, left ventricular ejection fraction (LVEF) ≤40% (hazard ratio [HR] 3.67, 95% confidence interval [CI] 3.23-4.17, P < .0001), allograft rejection (HR 1.51, 95% CI 1.35-1.70, P < .0001), and donor age (HR 1.17, 95% CI 1.13-1.23, P < .0001) were associated with increased risk of SCD, whereas recipient age (HR 0.90, 95% CI 0.86-0.95, P < .0001) and Caucasian race (HR 0.61, 95% CI 0.54-0.69, P < .0001) were associated with reduced risk. The incidence of SCD has shown no significant temporal improvement since 1987 (log-rank P = .84). CONCLUSION: Approximately 10% of deaths after HT are due to SCD. Allograft rejection and LVEF ≤40% are strong predictors of SCD in adult HT patients. Whether implantable cardioverter-defibrillators would reduce mortality in these patients with a relative higher risk of non-SCD remains to be determined.

Vascular endothelial growth factor in heart failure.

Heart failure is a devastating condition, the progression of which culminates in a mismatch of oxygen supply and demand, with limited options for treatment. Heart failure has several underlying causes including, but not limited to, ischaemic heart disease, valvular dysfunction, and hypertensive heart disease. Dysfunctional blood vessel formation is a major problem in advanced heart failure, regardless of the aetiology. Vascular endothelial growth factor (VEGF) is the cornerstone cytokine involved in the formation of new vessels. A multitude of investigations, at both the preclinical and clinical levels, have garnered valuable information on the potential utility of targeting VEGF as a treatment option for heart failure. However, clinical trials of VEGF gene therapy in patients with coronary artery disease or peripheral artery disease have not, to date, demonstrated clinical benefit. In this Review, we outline the biological characterization of VEGF, and examine the evidence for its potential therapeutic application, including the novel concept of VEGF as adjuvant therapy to stem cell transplantation, in patients with heart failure.

Serpentine left circumflex coronary artery aneurysm as a rare cause of angina with contrast-enhanced multislice computed tomographic evaluation.

Left circumflex coronary artery anomalies are rare causes of cardiac symptoms, especially in the adult population. Herein we describe a case of a 40-year-old man presenting with stable angina who was found to have aneurysmal formation and fistulization of the left circumflex coronary artery to the coronary sinus. Contrast-enhanced multislice computed tomography was very useful in our case for the diagnosis of such anomalies.

Endovascular intervention for acute stroke due to infective endocarditis: case report.

The overall incidence of neurological complications due to infective endocarditis is as high as 40%, with embolic infarcts more common than hemorrhagic strokes. The standard of care for typical strokes does not apply to infective endocarditis because there is a substantial risk of hemorrhage with thrombolysis. In the last decade there have been multiple case reports of intravenous and intraarterial thrombolysis with successful outcomes for acute strokes with related infective endocarditis, but successful endovascular interventions for acute strokes associated with infective endocarditis are rarely reported. To the authors' knowledge, this report is the first case in the literature to use a mechanical retrieval device in successful vegetation retrieval in an infective endocarditis acute stroke. Although an interventional approach for treatment of acute stroke related to infective endocarditis is a promising option, it is controversial and a cautious clinical decision should be made on a case-by-case basis. The authors conclude that this approach can be tested in a case series with matched controls, because this condition is rare and a randomized clinical trial is not a realistic option.

Review: recent advances in argatroban-warfarin transition in patients with heparin-induced thrombocytopenia.

Heparin-induced thrombocytopenia is a devastating, life-threatening, immune-mediated complication of therapy with unfractionated heparin, and less frequently, with low molecular weight heparin. Direct thrombin inhibitors are now standard therapy for the prevention of thrombosis in heparin-induced thrombocytopenia. Argatroban, a small synthetic molecule that inhibits thrombin at its active site, is increasingly used as the direct thrombin inhibitors of choice. Transition to longer term oral anticoagulation needs to be instituted after the platelet count has risen, because of the persistent risk of thrombosis. Although guidelines available in the literature outline the management of heparin-induced thrombocytopenia, they are not presented in a concise and comprehensive manner easily followed by physicians. This article reviews current recommendations, relevant studies, and clinical management trials carried out on patients with heparin-induced thrombocytopenia and provides updated, detailed guidelines for treatment of heparin-induced thrombocytopenia with emphasis on a key part of the management, the argatroban-warfarin transition.

Primary myelodysplastic syndrome in Jordan: a single-centre experience.

OBJECTIVE: Study of the disease patterns and clinical evaluation of myelodysplastic syndrome (MDS). SUBJECTS AND METHODS: A retrospective analysis was carried out on 85 patients, with MDS who were followed up over a period of 23 years at Jordan University Hospital, Amman, Jordan. Cases were analyzed according to the French, American and British Classification. RESULTS: Of the 85 patients, 42 (49.4%) were females and 43 (50%) males; mean age was 59 +/- 19 years (range 18-88). Most subtypes found in patients were refractory anemia (RA) in 27 (31.8%) and RA with excess blasts (RAEB) in 28 (32.9%). Adverse prognostic indicators were RAEB subtype and requirement for blood transfusion. CONCLUSION: Our findings showed that MDSs appeared at a younger age and tended to be of the aggressive subtype. Chronic myelomonocytic leukemia subtype seemed to appear dominantly in men.