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.

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.

Background: Pulmonary hypertension (PH) is a global health issue that has profound medical and research implications. Methods: This retrospective study examined changes in renal and liver function, as well as lipid metabolism, over a 12-month period in 49 adult patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). All cases were admitted, managed, and followed up with in the PH Center, County Emergency Clinical Hospital of Targu Mures, Romania. Results: Kidney dysfunction was observed in 12.24% of cases at baseline, decreasing to 8.16% at 12 months, and CTEPH patients were more affected. In particular, CTEPH patients exhibited an improvement in renal function, confirmed by an increase in their glomerular filtration rates. Hepatic impairment was present in 57.14% of subjects at baseline, declining to 42.86% at 12 months, with significant improvements noted in the PAH group. Lipid metabolic dysregulations were experienced by 22.45% of all patients at baseline, decreasing to 16.33% at 6 months, with a slow elevation to 24.49% at 12 months, but with no statistically significant differences. Pharmacological regimens were adjusted in accordance with the PH groups, a patient’s functional and clinical response, and laboratory tests. Conclusions: Our results demonstrate the multi-organ damage in PH and the importance of individualized treatment approaches.

Background/Objectives: Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are complex diseases that require precise diagnosis and management. The ESC risk score has been used in both conditions. We assessed the relationship between the EmPHasis-10 questionnaire (patient subjective evaluation) and objective assessment using endorsed tools (simplified four-strata risk assessment and right ventricular imaging by transthoracic echocardiography). Methods: The present study retrospectively extracted data from 40 adult patients (27 PAH and 13 CTEPH cases) diagnosed in a single center in Romania. The EmPHasis-10 questionnaire and the four-strata risk assessment (FSRA) tool were applied to each patient. Mean pulmonary artery pressure (mPAP), tricuspid annular plane systolic excursion (TAPSE), TAPSE/systolic pulmonary artery pressure (TAPSE/sPAP) ratio, and right ventricular outflow tract acceleration time (RVOT-AT) were assessed. Results: A significant correlation was observed between the EmPHasis-10 scores and the FSRA tool, the WHO functional class, and the 6 min walking distance. Emphasis-10 score did not correlate with any of the echocardiographic parameters. The FSRA tool showed a moderate positive correlation with mPAP (r = 0.42, p = 0.01) and a negative correlation with TAPSE (r = −0.46, p = 0.003); additionally, across the entire cohort, it was moderately negatively correlated with both RVOT-AT (r = −0.42, p = 0.01) and TAPSE/sPAP ratio (r = −0.43, p = 0.005). Conclusions: Our study evidenced the alignment between EmPHasis-10 scores and prognostic risk score, with poorer health-related quality of life corresponding to higher FSRA. The EmPHasis-10 questionnaire proves to be a valuable, easy-to-use instrument, offering meaningful insights into patients’ health-related quality of life, underscoring its utility in enhancing comprehensive patient assessment and management.

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. 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. 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.