Outcomes for patients with systemic light chain amyloidosis and Mayo stage 3B disease.

Patients with cardiac light chain amyloidosis and Mayo stage 3b disease define a high-risk population with very poor prognosis. Here, we report treatment outcomes of 80 consecutive patients with newly diagnosed AL and Mayo 3b who received novel regimens. Early mortality (<1 month) rate was 12.5%. On intention-to-treat, overall hematologic response rate was 40%, with complete response (CR)/very good partial response (VGPR) in 25% and partial response (PR) in 15%. At 1- and 3- month landmark analysis CR or VGPR/PR rates were 25%/23.5% and 34%/25.5%, respectively. Among patients that were treated with daratumumab-based therapies, 52.6% and 85.7% achieved at least VGPR within one 1 and 3 months, respectively. Three-month cardiac response rate was 11.3% and 6-month was 18.8%. At least hemVGPR at 3 months was associated with cardiac response at 6 months (p = 0.034). Median overall survival (OS) was 6.3 months. At 1-month landmark at least hemPR was associated with better median OS (24.1 vs. 4.9 months, p = 0.017) and at 3-month landmark, at least hemVGPR was associated with a median OS of 40.7 versus 17 months for hemPR and 7.4 months for those without hematologic response (p = 0.028). Cardiac response at 3 months was associated with longer median OS (59.7 vs. 10.9 months, p = 0.044). Factors associated with poorer survival were κ-light chain amyloidosis (median OS 2.9 vs. 7.4 months, p = 0.028), peripheral nerve involvement (3.4 vs. 10.45 months, p = 0.024), systolic blood pressure <90 mmHg (2 vs. 8 months, p = 0.002), baseline LVEF <55% (median OS 3.4 vs. 32 months, p = 0.29) and New York Heart Association (NYHA) class (2.7 months for NYHA 3B-4 vs. 8 months for NYHA 2-3A, p = 0.02). Twenty-one patients (26.3%) received salvage therapy and ORR was 57.1%. Median OS for patients who received second line therapy was 24 months. In conclusion, patients with Mayo 3b disease benefit from early hematologic response but cardiac response rates remain low.

Peripheral blood natural killer cells in sarcoidosis are associated with early cardiac involvement.

AIM: To evaluate the distribution of circulating immune cell subsets in peripheral blood of patients with sarcoidosis and investigate if there is an association with an underlying cardiac involvement. METHODS AND RESULTS: Eighty-five newly diagnosed treatment-naïve patients with sarcoidosis (50 women) were included in the study. All patients underwent a thorough cardiac investigation, including cardiac magnetic resonance imaging (CMR). Of all patients, 19 (23.53%) had myocardial involvement, and the NK subpopulation in these patients in peripheral blood was significantly decreased compared to patients without (n = 63, p = 0.001 and p = 0.003 respectively). The absolute number of NKT cells (CD3+CD16/56+ ) in patients with cardiac involvement was highly correlated with T2 map increased values in MRI (r = -686, p = 0.041) showing that low NKT cell count correlates with the inflammatory process of the heart. No difference in CD19, CD3, CD4, CD8 and CD3- NK cell counts was found between groups. Lung severity was not found to correlate with the number of NK cells. CONCLUSION: We found that low NK cell count in peripheral blood of patients with sarcoidosis is associated with cardiac involvement, and the number of NK-T cells correlates with CMR findings indicative of myocardial inflammation. This finding might have a potential clinical application in detecting clinically silent cardiac involvement in sarcoidosis and may also suggest potential targets for therapeutic interventions.

Delayed disseminated intravascular coagulopathy post thoracic endovascular aortic repair (TEVAR).

Disseminated intravascular coagulopathy (DIC) is a very rare outcome post endovascular repair of aortic aneurysm dissections. We present a case of a 70-year-old male who presented with DIC post a thoracic endovascular aortic repair (TEVAR) procedure due to a type 1A endoleak. Initially, the patient was treated with red blood cells and blood products; however, when failing to improve, he underwent a hybrid arch replacement. In this study, we will analyze the management of DIC post TEVAR and look at its presentation more extensively, as it is currently a topic that is poorly studied.

"Health-Related Quality of Life in Operated Adult Patients with Tetralogy of Fallot and Correlation with Advanced Imaging Indexes and Cardiopulmonary Exercise Test" A Narrative Review.

Congenital heart diseases (CHD) are considered to be the second leading cause of death during infancy and childhood. However, due to advanced imaging techniques, more than 90% of children with complex CHD survive into adulthood, increasing the prevalence of CHD in the population. Tetralogy of Fallot (ToF) is considered to be the most common, complex, cyanotic CHD. Health is an important determinant of a patient's quality of life and this drove physicians to examine ToF patient's health related quality of life (HRQOL), in order to improve medical and surgical interventions. The purpose of this narrative review was to identify parameters derived through modalities, such as cardiovascular magnetic resonance, echocardiography and cardiopulmonary exercise testing and correlate them with repaired ToF (rToF) patient's perceived HRQOL. It is widely proven that rToF patients have worse physical capabilities than healthy population and this can be safely attributed to their cardiac condition. Unfortunately, rToF population is relatively small, making most of the included trials reliable and unable to uniformly agree to the same results. Further investigation is mandatory in order to discover those parameters that will allow physicians to better understand HRQOL in this population, leading to better medical management and rehabilitation.

Diagnostic and Prognostic Value of Non-late Gadolinium Enhancement Cardiac Magnetic Resonance Parameters in Cardiac Amyloidosis.

Early diagnosis is crucial for the improvement of outcomes of patients with cardiac amyloidosis (CA). Emerging non-late gadolinium enhancement (LGE) based cardiac magnetic resonance (CMR) parameters may facilitate early identification of CA. We sought to investigate the diagnostic and prognostic value of T1, T2 mapping and extracellular volume (ECV) in CA. This single-center prospective analysis included 88 patients with CA, 33 patients with aortic stenosis (AS) and left ventricular hypertrophy (LVH), and 15 healthy controls who completed 3T cardiac MRI at the time of their diagnosis and were assessed with T1, T2 (modified Look-Locker inversion recovery), and ECV mapping of the heart and spleen. Echocardiographic, and biochemical parameters and clinical characteristics and outcomes were collected and analyzed. Of the patients with CA, 71 had light-chain (AL) and 17 had transthyretin (ATTR) amyloidosis. Native T1, native T2 and ECV were significantly higher in patients with CA compared to both patients with LVH-AS (P<0.001) and healthy controls (P<0.001). Good diagnostic accuracy was also demonstrated by measuring the area under the curve (AUC) of the receiver operating characteristic (ROC) curves for native T1 in the region of interest (ROI) (AUC=0.90), native T2 ROI (AUC=0.88), and ECV (AUC=0.90). Furthermore, native T1 ROI, native T2 ROI and ECV, correlated with both NT-proBNP levels and Mayo stage of patients (with AL). Spleen ECV was significantly increased in patients with AL versus ATTR amyloidosis (38.5 vs 30.5; P=0.004) and demonstrated good diagnostic accuracy in differentiating between the two types (AUC=0.79). Native T2 ROI was prognostic of mortality in AL CAwith a HR of 1.97 per 5 ms increase (P=0.001) and remained prognostic after adjustment for age, and Mayo stage. Non-LGE based CMR techniques correlated with established markers of disease and demonstrated good diagnostic accuracy, while native T2 ROI was also prognostic of mortality, thus reinforcing their use in the diagnosis and prognosis of CA.

Cardiovascular magnetic resonance for the evaluation of patients with cardiovascular disease: An overview of current indications, limitations, and procedures.

Cardiovascular disease (CVD) is the most common cause of morbidity/mortality worldwide. Early diagnosis is the key to improve CVD prognosis, and cardiovascular imaging plays a crucial role in this direction. Echocardiography is the most commonly used imaging modality. However, the need for early diagnosis/treatment favors the development of modalities providing information about tissue characterization beyond echocardiography. In this context, the rapid evolution of cardiovascular magnetic resonance (CMR) led to the coexistence of cardiologists and radiologists in the CMR field. Our aim was to provide an overview of indications, sequences, and reporting of CMR findings in various CVDs. The indications/limitations of CMR as well as the pathophysiological significance of various sequences in adult/pediatric CVDs are presented and discussed in detail. The role of CMR indices in the evaluation of the most common clinical scenarios in cardiology and their impact on CVD diagnosis/prognosis were analyzed in detail. Additionally, the comparison of CMR versus other imaging modalities is also discussed. Finally, future research directions are presented. CMR can provide cardiac tissue characterization and biventricular/biatrial functional assessment in the same examination, allowing for early and accurate identification of important subclinical abnormalities, before clinically overt CVD takes place.

Is Left Innominate Vein Ligation during Aortic Arch Surgery Always Safe? A Case Report.

Ligation of the left innominate vein (LIV) expands the surgeon's surgical field for ascending aorta and aortic arch procedures. Although it is considered a safe technique by most surgeons in that it is associated with only minor drawbacks, conflicting views exist regarding this method. We herein describe a 70-year-old woman who underwent ascending aorta replacement due to an aneurysm with subsequent cerebral dysfunction caused by extended brain edema, possibly related to LIV ligation, leading to her death.

The utility of splenic imaging parameters in cardiac magnetic resonance for the diagnosis of immunoglobulin light-chain amyloidosis.

OBJECTIVES: Cardiac magnetic resonance (CMR) imaging is a key test in the diagnosis of cardiac amyloidosis (CA). Extracardiac involvement is common in light chain (AL) amyloidosis and MRI findings may assist in its diagnosis. We sought to investigate the utility of splenic CMR parameters for the diagnosis of CA. METHODS: Thirty-four patients with AL amyloidosis and 32 patients with severe left ventricular hypertrophy in the setting of aortic stenosis (LVH-AS) who completed 3T cardiac MRI at the time of their diagnosis of AL or LVH-AS were assessed with T1, T2 (modified Look-Locker inversion recovery), extracellular volume (ECV) mapping, and late gadolinium enhancement (LGE) imaging of the heart and spleen. RESULTS: Age, left ventricular mass index, wall thickness, ejection fraction, and splenic dimensions did not differ significantly between groups. All AL patients had cardiac involvement. T1 and T2 spleen mapping did not differ significantly between groups but AL patients had higher median ECV in the spleen than in LVH-AS (AL 46.9%, LVH-AS: 31%, p < 0.001), and significantly lower short tau inversion recovery ratio (AL: 1.7, LVH-AS: 2.7, p < 0.001) both with very good diagnostic performance to diagnose AL. We identified 16 AL patients with spleen involvement and 16 without. Spleen ECV and "normalized" spleen ratio, defined as the ratio of spleen LGE to muscle values exhibited strong correlation and had excellent diagnostic performance to discriminate those with spleen involvement. CONCLUSION: Our findings show that spleen CMR parameters can identify spleen involvement in AL patients and differentiate them from those without AL amyloidosis.

Radiopaque preventive landmarks' placement during stentless bioprosthesis implantation.

BACKGROUND: In patients with aortic stenosis, bioprosthetic valves are increasingly used. Although their benefits, they are also presenting limitations, as their time-related degeneration. Reoperation which was, until a few years ago, the only treatment for this condition, carries a significant surgical risk, especially in patients with multiple comorbidities, so the benefit of less invasive technique enabling the implantation of aortic valve prosthesis [transcatheter aortic valve-in-surgical aortic valve (TAV-in-SAV)] by a percutaneous access is remarkably important. Eligible patients are judged by a heart team, and imaging plays a key role in this selection, focusing on correct identification of bioprosthetic aortic valves type and size, evaluation of patients at increased anatomical risk for coronary artery occlusion. Radiolucency of stentless bioprosthetic valves, represent a significant challenge. METHODS: Surgical aortic valve replacements (SAVRs) with a bioprosthesis were performed using a stentless valve with no radiopaque components (Solo Smart, Sorin). The chosen method, in order to evaluate the results of the operation, was computed tomography (CT) scanning (64-slice MDCT, Brilliance, Philips). The study consisted of a thin sliced contrast electrocardiograph (ECG) gated chest CT (1 systolic cardiac phase), trying to simulate the required assessment of aortic root and the radiopaque placed markers. RESULTS: As surgical implant technique varies and may impact the relationship of the prosthetic annulus to the coronary ostia, marking the aortic annulus during the operation in order to have some useful radiopaque landmarks, is a great assistance promoting better orientation and correct identification of the position of the bioprosthetic valve. Although the implantation of metallic vascular clips at the level of aortic annulus (in any commissure or in the middle of any cups) was considered, the decision was to position three metallic clips bellow the aortic annulus in the three stiches ligated during the solo valve implantation. CONCLUSIONS: We are suggesting the preventive implantation of radiopaque landmarks, during SAVRs using tissue valves which are lacking fixed anatomic markers, as a guide for a presumptive TAV-in-SAV procedure, keeping in mind that appropriate guidance is crucial and can prevent valve misplacement, coronary obstruction and other potentially lethal complications.

Late Development of a Bronchocutaneous Fistula due to an Epicardial Cardioverter-Defibrillator Lead.

Cutaneous complications caused by a pacemaker or defibrillator are widely documented, but the development of a bronchocutaneous fistula has never been described before. We report the case of a 79-year-old man who was admitted to our hospital because of a seemingly superficial cutaneous infection, externalized defibrillator leads, and hemoptysis. Bronchoscopical investigation proved the existence of the fistula, which connected the epicardium, the left main bronchus, and the aforementioned site of skin infection. The patient refused an operation for the complete removal of the epicardial defibrillator and was treated conservatively. This case demonstrated that the long-term presence of foreign bodies in the epicardium may cause serious complications.

Post-trauma "abrasive" right ventricular rupture without mediastinitis early post-CABG. Is the Robicsek closure technique necessary for all elderly patients?

INTRODUCTION: Right ventricular (RV) rupture with mediastinitis, is a very rare but extremely dangerous (even fatal) complication, following CABG surgery. PRESENTATION OF CASE: In this paper, we present the case of a post-trauma (after fall) RV rupture (without mediastinitis) in a patient who had undergone cardiac surgery several days ago. The cause of the rupture proved to be a broken bone piece from the lower sternal edge. DISCUSSION: RV rupture post-operatively caused by broken bone pieces or bone dislocation may occur through two mechanisms: either penetration of the RV, or through the "sandpaper effect". In order to prevent the rupture, we should be able to recognize patients with aggravating factors (age, weight) and choose intra-operatively a suitable closure technique. CONCLUSION: We propose that the technique that could prevent such ruptures is the Robicsek technique.

Corynebacterium diphtheriae endocarditis with multifocal septic emboli: can prompt diagnosis help avoid surgery?

PATIENT: Male, 23. FINAL DIAGNOSIS: Corynebacterium diphtheriae endocarditis. SYMPTOMS: Abdominal pain • cachexia • diarrhea • fever • vomiting. MEDICATION: -. CLINICAL PROCEDURE: Mitral valve replacement. SPECIALTY: Surgery. OBJECTIVE: Rare disease. BACKGROUND: Although Corynebacterium diphtheriae is well known for causing diphtheria and other respiratory tract infections, in very rare cases it can lead to severe systemic disease. CASE REPORT: This is a case of a previously healthy young man (no prosthetic valve in situ or other known congenital defect), presenting with a Corynebacterium diphtheriae infection leading to endocarditis. The patient reported no I.V. drug use, so it can be assumed that no risk factors for infective endocarditis were present. CONCLUSIONS: This report aims to raise suspicion for this specific infection in order to proceed with the right treatment as soon as possible.

Pyrexia in patients with uncontrolled systemic hypertension: could they have an aortic dissection?

Aortic dissection can present in a variety of ways and one of the most documented risk factors includes systemic hypertension. Occasionally aortic dissection can be diagnosed late due to an insidious presentation. Fever has been described in people with aortic dissection but rarely as the main presenting feature. We present the cases of two patients with type B aortic dissections who shared three pertinent features which could have alerted the clinicians of the potential diagnosis; systemic hypertension, small left sided pleural effusion and a fever of unknown origin.

Off pump repair of left ventricular rupture following mitral valve replacement: The crucial assistance of the IntraAortic Balloon Pump.

INTRODUCTION: Left ventricular (LV) rupture is a not as frequent, but potentially lethal complication of mitral valve replacement or repair. PRESENTATION OF CASE: We report a case of a 67-year-old man who underwent mitral valve replacement and Cox Maze IV procedure. A massive bleed from the LV rupture was noted postoperatively while the patient was extubated. The control of bleeding was impossible until an IntraAortic Balloon Pump (IABP) was inserted. A bovine pericardial patch was applied, overlapping an extensive epicardial area, perimetrically of the hematoma. Between the epicardium and the pericardial patch we applied an autologous fibrin sealant. DISCUSSION: The off-pump technique used to repair the LV rupture after a MVR, is more feasible when the patient is supported by an IABP that subsequently decreases the tension of the myocardial suture site. CONCLUSION: The IABP, is a necessary device, that decreases the tension along the suture site post a left ventricular rupture following a MVR.

Trans-aortic excision of intraventricular lipoma with the assistance of arthroscopic camera.

Cardiac lipomas are extremely rare benign neoplasms of the heart. We report the case of a 64-year-old female complaining of rapidly worsening dyspnea and lower limb edema. Echocardiograms showed a large hyperechoic mass which occupied the left ventricle. The cardiac nuclear magnetic resonance allowed the diagnosis of the left ventricular tumor of lipoma or liposarcoma. The tumor was resected through the ascending aorta, without injuring neither the aortic valve nor the left ventricle. Histological diagnosis showed that the tumor was a lipoma. The patient had an uneventful recovery, with no recurrence two years later.

Left ventricular rupture after double valve replacement in a patient with myocarditis due to myasthenia gravis.

Myasthenia gravis is an autoimmune disease characterised by weakness of the skeletal muscles, with remissions and exacerbations due to antibodies acting on the acetylcholine receptors. This leads to the characteristic defect transmission in the neuromuscular junction. Treatment includes anticholinesterase agents, thymectomy, and immunosuppression. Surgical thymectomy can induce remission or improvement, allowing for reduction in the immunosuppressive treatment. The case of an 84-year-old female patient with myasthenia gravis, aortic valve stenosis, mitral valve regurgitation and myocarditis is described. The development of myocarditis was related to inflammatory cell infiltration, and progressive and additive focal cellular necrosis associated with reactive myocardial fibrosis. After replacement of the mitral valve, complications arose when a rupture of the left ventricular posterior wall occurred, which caused massive bleeding and sudden death on the operating table.