FIP1L1-PDGFRα-Positive Loeffler Endocarditis-A Distinct Cause of Heart Failure in a Young Male: The Role of Multimodal Diagnostic Tools.

The presence of the Fip1-Like1-platelet-derived growth factor receptor alpha (FIP1L1-PDGFRα) fusion gene represents a rare cause of hypereosinophilic syndrome (HES), which is associated with organ damage. The aim of this paper is to emphasize the pivotal role of multimodal diagnostic tools in the accurate diagnosis and management of heart failure (HF) associated with HES. We present the case of a young male patient who was admitted with clinical features of congestive HF and laboratory findings of hypereosinophilia (HE). After hematological evaluation, genetic tests, and ruling out reactive causes of HE, a diagnosis of positive FIP1L1-PDGFRα myeloid leukemia was established. Multimodal cardiac imaging identified biventricular thrombi and cardiac impairment, thereby raising suspicion of Loeffler endocarditis (LE) as the cause of HF; this was later confirmed by a pathological examination. Despite hematological improvement under corticosteroid and imatinib therapy, anticoagulant, and patient-oriented HF treatment, there was further clinical progression and subsequent multiple complications (including embolization), which led to patient death. HF is a severe complication that diminishes the demonstrated effectiveness of imatinib in the advanced phases of Loeffler endocarditis. Therefore, the need for an accurate identification of heart failure etiology in the absence of endomyocardial biopsy is particularly important for ensuring effective treatment.

Right Heart Failure as an Atypical Presentation of Chronic Type a Aortic Dissection - Multimodality Imaging for Accurate Diagnosis and Treatment. A Case Report and Mini-review of Literature.

Background: An intrapericardial organized haematoma secondary to chronic type A aortic dissection is an extremely rare cause of right heart failure. Imaging studies are essential in recognising and diagnosis of this distinctive medical condition and guiding the anticipated treatment. Case presentation: A 70-year-old male patient was admitted for progressive symptoms of right heart failure. His cardiovascular history exposed an aortic valve replacement 22 years before with a Medtronic Hall 23 tilting valve with no regular follow-up. Classical signs of congestion were recognized at physical examination. Transthoracic two-dimensional echocardiography and thoraco-abdominal computed tomography angiography, as essential parts of multimodality imaging algorithm, established the underlying cause of right heart failure. Under total cardiopulmonary bypass and cardiac arrest, surgical removal of the haematoma and proximal repair of the ascending aorta with a patient-matched vascular graft were successfully performed. The patient was discharged in good condition with appropriate pharmacological treatment, guideline-directed; no imagistic signs of acute post-surgery complications were ascertained. Conclusion: This paper highlights the importance of recognizing and providing a timely clinical and imagistic diagnosis of this very rare, potentially avoidable cause of right heart failure in patients with previous cardiac surgery.

Native Aortic Valve Endocarditis Complicated by Splenic Infarction and Giant Mitral-Aortic Intervalvular Fibrosa Pseudoaneurysm-A Case Report and Brief Review of the Literature.

BACKGROUND: Pseudoaneurysm of the mitral-aortic intervalvular fibrosa (P-MAIVF) is an unusual complication related to various injuries or conditions which involve the mitro-aortic region; it communicates with the left ventricular outflow tract and is associated with a high-risk of redoubtable complications or sudden death. The cerebral and splenic localizations are frequently seen as manifestations of systemic embolism in infective endocarditis. Currently, there are no specific recommendations related to the diagnosis, management, treatment, or further evolution of patients with P-MAIVF and concomitant splenic infarction. This paper presents the case of a 43-year-old Caucasian woman with a late diagnosis of mixed bicuspid aortic valve disease, affected by an under-detected and undertreated episode of infective endocarditis leading to asymptomatic P-MAIVF. Prime clinical and imagistic diagnosis of splenic infarction indicated further extended investigations were required to clarify the source of embolism. METHODS: Integrated multimodality imaging techniques confirmed the unexpected diagnosis of P-MAIVF. RESULTS: The case had a fatal outcome following an uncomplicated yet laborious cardiac surgery. Patient death was attributed to a malignant ventricular arrhythmia. CONCLUSION: The present case raises awareness by highlighting an unexplained and unexpected splenic infarction association with P-MAIVF as a result of infective endocarditis related to mixed bicuspid aortic valve disease.