Reply to Kawasaki et al. Comment on "Manole et al. Primary Pericardial Synovial Sarcoma: A Case Report and Literature Review. Diagnostics 2022, 12, 158".

Thank you for your comment; it adds value to the article and highlights the importance of molecular testing [...].

Diagnosis of Left-Sided Mechanical Prosthetic Valve Thrombosis: A Pictorial Review.

Although transcatheter valve therapy is rapidly evolving, surgical valve replacement is still required in many patients with severe left-side valve stenosis or regurgitation, the mechanical bi-leaflet heart valve being the standard prosthesis type in younger patients. Moreover, the prevalence of valvular heart disease is steadily increasing, especially in industrialized countries, and the problem of lifelong efficient anticoagulation of these patients remains fundamental, especially in the context where vitamin K antagonists continue to be the current standard of anticoagulation despite a level of oscillating anticoagulation. In this setting, avoiding prosthetic valve thrombosis after surgery is the number one objective for both the patient and the responsible physicians. Although rare, this complication is life threatening, with the sudden onset of acute cardiac failure such as acute pulmonary edema, cardiogenic shock, or sudden cardiac death and inadequate anticoagulation remaining the leading cause of prosthesis thrombosis, along with other risk factors. The availability of multimodal imaging techniques enables and encompasses to a full extent the diagnosis of mechanical valve thrombosis. The gold-standard diagnostic methods are transthoracic and transesophageal echocardiography. Moreover, 3D ultrasound has undoubted value in giving a more accurate description of the thrombus's extension. When transthoracic and transesophageal echocardiography are uncertain, the multidetector computer tomography examination is an important complementary imaging method. Fluoroscopy is also an excellent tool for evaluating the mobility of prosthetic discs. Each method complements the other to differentiate an acute mechanical valve thrombosis from other prosthetic valve pathologies such as pannus formation or infective endocarditis and aids the physician in accurately establishing the treatment method (surgical or pharmaceutical) and its optimal timing. The aim of this pictorial review was to discuss from an imagistic perspective the mechanical prosthetic aortic and mitral valve thrombosis and to provide an overview of the essential role of non-invasive exploration in the treatment of this severe complication.

The Role of Multimodality Imaging in the Diagnosis and Follow-Up of Malignant Primary Cardiac Tumors: Myxofibrosarcoma-A Case Report and Literature Review.

Cardiac tumors are a very rare but heterogenous group of diseases that may reveal themselves through a variety of nonspecific cardiac symptoms that may pose a challenge to the diagnostic process. Myxofibrosarcoma is a particularly rare type of cardiac tumor that carries a poor prognosis, thus making accurate and timely diagnosis essential. A 61-year-old woman presented with fatigue and shortness of breath during mild exercise, symptoms that have progressively worsened during the previous year. Multimodality imaging consisting of transthoracic and transesophageal echocardiography (TTE and TEE), cardiac magnetic resonance (CMR), cardiac computer tomography (CCT), and fluorodeoxyglucose positron emission computer tomography (18F-FDG PET-CT) was used for the diagnosis and postoperative follow-up of a myxofibrosarcoma.

The Role of Echocardiography in the Diagnosis of Cardiac Involvement in a Rare Systemic Condition: The Carcinoid Heart Disease: A Case Report and Review of Literature.

Carcinoid heart disease is a rare presentation of the carcinoid syndrome, which is caused by excessive tumoral hormone production and the abundant release of vasoactive substances with systemic expressions. A 62-year-old woman presented with flushing, diarrhea, weight loss, and right-sided heart failure symptoms. Specific carcinoid heart disease features were identified using transthoracic and transesophageal echocardiography at the tricuspid and pulmonic valves. Biomarkers, 99mTc-Tektrotyd scintigraphy, SPECT-CT, and a biopsy later confirmed the diagnosis, and the patient began treatment for the underlying condition.

Diagnostic Challenges in Rare Causes of Arrhythmogenic Cardiomyopathy-The Role of Cardiac MRI.

Arrhythmogenic right ventricular dysplasia (ARVD) is a rare genetic condition of the myocardium, with a significantly high risk of sudden death. Recent genetic research and improved understanding of the pathophysiology tend to change the ARVD definition towards a larger spectrum of myocardial involvement, which includes, in various proportions, both the right (RV) and left ventricle (LV), currently referred to as ACM (arrhythmogenic cardiomyopathy). Its pathological substrate is defined by the replacement of the ventricular myocardium with fibrous adipose tissue that further leads to inadequate electrical impulses and translates into varies degrees of malignant ventricular arrythmias and dyskinetic myocardium movements. Particularly, the cardio-cutaneous syndromes of Carvajal/Naxos represent rare causes of ACM that might be suspected from early childhood. The diagnostic is sometimes challenging, even with well-established rTFC or Padua criteria, especially for pediatric patients or ACM with LV involvement. Cardiac MRI gain more and more importance in ACM diagnostic especially in non-classical forms. Furthermore, MRI is useful in highlighting myocardial fibrosis, fatty replacement or wall movement with high accuracy, thus guiding not only the depiction, but also the patient's stratification and management.

Primary Pericardial Synovial Sarcoma: A Case Report and Literature Review.

We report a case of a 52-year-old woman who was referred to our institution with a superior vena cava syndrome and was investigated through echocardiography, CT and MRI revealing a well-defined, encapsulated pericardial mass. The pathology, correlated with the immunohistochemical analysis, concluded it was an extremely rare primary pericardial synovial sarcoma. The patient underwent surgery and chemotherapy with a 16-month disease-free survival and passed away after a contralateral aggressive relapse. Moreover, we discuss the role of each imaging modality together with their pericardial synovial sarcoma reported features.

Transthyretin cardiac amyloidosis.

Transthyretin amyloid cardiomyopathy (ATTR-CM) may be an under recognized cause of heart failure (HF). TTR amyloidosis can be inherited, caused by variants in the TTR gene (ATTRv) or by deposition of wild-type TTR protein (ATTRwt), leading to high mortality if untreated. We report the case of a patient with hereditary TTR amyloidosis and mixed phenotype (both cardiac and neurological involvement). We highlight the importance of multimodal imaging in the evaluation of these patients, as early diagnosis and treatment might lead to better outcome.

Major Left Bundle Branch Block and Coronary Heart Disease-Are There Any Differences between the Sexes?

Left bundle branch block is not a benign pathology, and its presence requires the identification of a pathological substrate, such as ischemic heart disease. Left bundle branch block appears to be more commonly associated with normal coronary arteries, especially in women. The objectives of our study were to describe the particularities of left bundle branch block in women compared to men with ischemic heart disease. Result: We included seventy patients with left bundle branch block and ischemic heart disease, with a mean age of 67.01 ± 8.89 years. There were no differences in the profile of risk factors, except for smoking and uric acid. The ventricular depolarization (QRS) duration was longer in men than women (136.86 ± 8.32 vs. 132.57 ± 9.19 msec; p = 0.018) and also men were observed to have larger left ventricular diameters. Left bundle branch block duration was directly associated with ventricular diameters and indirectly associated with left ventricular ejection fraction value, especially in women (R = -0.52, p = 0.0012 vs. R = -0.50, p = 0.002). In angiography, 80% of women had normal epicardial arteries compared with 65.7% of men; all these patients presented with microvascular dysfunction. Conclusion: The differences between the sexes were not so obvious in terms of the presence of risk factors; instead, there were differences in electrocardiographic, echocardiographic, and angiographic aspects. Left bundle branch block appears to be a marker of microvascular angina and systolic dysfunction, especially in women.

The role of multimodal imaging in the diagnosis of an asymptomatic patient with congenital anomaly.

Anomalous left coronary artery from the pulmonary artery (ALCAPA) syndrome is a rare congenital coronary anomaly, which can cause potentially fatal complications, such as heart failure, myocardial infarction and sudden cardiac death. Only a few patients left untreated survive to adulthood. We highlight the importance of multimodal imaging in the diagnosis of ALCAPA syndrome in a young asymptomatic female patient with inducible ischemia on exercise. The patient was successfully treated with surgery.

Diagnostic Dilemma of Recurrent Pulmonary Embolism.

Popliteal venous aneurysms are rare vascular disorders associated with a high risk of pulmonary embolism. We present the case of a 56-year-old woman hospitalized for a third episode of unprovoked pulmonary embolism. Venous ultrasonography identified a popliteal aneurysm, repeatedly missed by two-point compression venous ultrasonography, which was eventually confirmed by a magnetic resonance examination. Because of its highly symptomatic nature despite optimal anticoagulant treatment, the decision was made to undergo surgery, consisting of aneurysmectomy followed by patch angioplasty. The goal of this paper is to report a rare case of popliteal venous aneurysm and its treatment strategies and postoperative evolution.