bluuebunny/crossref_metadata_2025_split huggingface dataset:
A CASE OF A HUGE AORTIC PSEUDO–ANEURYSM FOLLOWING AORTIC BIO PROSTHETIC ENDOCARDITIS: KEY ROLE OF 3D ECHOCARDIOGRAPHY
Authors: Sordelli C , Fele N , Weisz S , Verde R , Guarino A , Mazzella F , Claudia S , Severino S 
Published: May 2024

Abstract
Complications of infective endocarditis (IE) are associated with increased mortality and morbidity and therefore guidelines recommend early surgery [. Although two–dimensional echocardiography (2D) is the first line investigation, three–dimensional (3D) imaging provides incremental diagnostic information by allowing visualisation of structures in multiple planes. We present a cases which demonstrate how 3D imaging provides better morphological assessment of valve leaflets and associated structures, allows greater accuracy in identifying complications of IE. A 47 year old male with previous meccanic prosthetic aortic valve replacement was admitted to our department for evaluation of pyrexia. Lab–work was notable for leukocytosis with a white blood cell count of 13.000/mm3 and a hemoglobin level of 10.8 gr/dl. Blood cultures grew Staphylococcus Epidermidis. A transesophageal echocardiogram was performed showing a pulsatile perivalvular echo free–space with colour Doppler flow inside suggestive of an aortic pseudoaneurysm . 3D–TEE imaging provides better morphological assessment of aortic pseudoaneurysm. 3D TEE clearly demarcates an irregularly shaped of perivalvular cavity and through multiplanar reconstruction allowed a correct evaluation of the area and circumference. Conclusione: Decision making in IE is complex. The most common indications for early surgery are development of heart failure, uncontrolled infection or risk of embolism. In particular uncontrolled infection is commonly due to perivalvular abscess formation. 3D–TOE allows rapid, accurate, unique assessment of IE complication through unlimited multiplanar reconstructions, volume–rendered real–time imaging and colour full–volume regurgitation assessment which may not always possible on 2D–TTE or 2D–TOE. This case demonstrate how traditional 2D TEE may provide incomplete anatomical information. 3D imaging improved identification and characterisation of the complications of IE. The complementary data provided aids in decision making with regard to both requirements for timing and planning of surgery.
A RARE CASE OF LOEYS–DIETZ SYNDROME: THE IMPORTANCE OF DIAGNOSIS IN PATIENTS UNDERGOING CARDIOVASCULAR SURGERY
Authors: Mandi O , Agusto S , Contiello C , D‘Ambrosio G , Baldasseroni S , Orso F , Silverii M , Burgisser C , Fattirolli F , Ungar A 
Published: May 2024

Abstract

Background
Loeys–Dietz syndrome (LDS) is a rare autosomal dominant genetic disorder of connective tissue, characterised by a broad spectrum of craniofacial, vascular (arterial tortuosity and aneurysms) and skeletal clinical signs. Four subtypes have been described, related to mutations in genes encoding for components of the transforming growth factor beta signalling pathway (TGFBR1, TGFBR2, SMAD2, SMAD3, TGFB2 and TGFB3). Diagnosis is based on the evaluation of clinical manifestations and family history. Therefore, many radiologists remain unfamiliar with the imaging and clinical findings in LDS.

The Case
75–year–old woman, history of surgery for ruptured gastric a. aneurysm and ectasia of visceral arterial vessels. One year onset of worsening dyspnoea and asthenia, at echocardiography (ECOCG) severe aortic insufficiency has been detected and patient underwent aortic valve replacement with bioprosthesis. Cardiac surgery was complicated by aortic dissection (Stanford A; DeBakey I type) treated with replacement of ascending aorta and anterior hemiarch with tubular prosthesis. On that occasion a family diagnosis of SMAD3 gene mutation was made (both sister and nephew typed), compatible with Loeys–Dietz syndrome type 3. At the control angioTC evidence of proximal and distal anastomosis in order, in the absence of signs of periprosthetic leak. At ECOCG (FE 55%), thickened septum with marked postcardiac dyskinesia. No abnormality detected on ECG. Due to persistence of dyspnoea on mild exertion (NYHA class III) and reduced tolerance to orthostatism, cardiac rehabilitation program was undertaken on an outpatient basis for three months, providing an improvement in exertion tolerance (NYHA II) and a benefit on functional autonomy. LDS is a multisystem connective tissue disorder that is associated with a high burden of complications, a correct diagnosis will allow clinicians to appropriately establish the best therapeutic strategy, especially in cardiac surgery, taking into account the intraoperative and perioperative potential risks.
MULTIDRUG RESISTANT DYSLIPIDEMIA: A CASE REPORT
Authors: Daus F , Maloberti A , Pavanello C , Toscani G , Riccio A , Fabbri S , Ciampi C , Caccia A , Tognola C , Algeri M , Intravaia R , Merlini P , Mosca L , Cavallari U , Giannattasio C 
Published: May 2024

Abstract

Introduction
Recently introduced lipid–lowering agents (PCSK9–i, bempedoic acid) can efficiently treat statin–intolerant patients, who couldn‘t reach LDL target before. Nonetheless, there are still cases where the pharmacological management may have strong limitations.

Case Report
A 71 year–old woman, with multiple cardiovascular risk factors (diabetes, hypertension and familial dylipidemia, LDL–C 276) underwent heart transplantation for a hypertrophic cardiomyopathy with an evolution towards a dilated phenotype in 2005. Before the transplant, she was on low–dose statin treatment (pravastatin 10 mg/die) that was suspended years later owing to hyper–CKemia (600 U/L), despite her being totally asymptomatic. A second attempt with simvastatin/ezetimibe 40/10 mg resulted again in elevation of CK levels, associated with significant muscular pain. Thus, just ezetimibe was continued. In 2020, 15 years after the transplant, the patient underwent a coronary angiography showing a moderate coronary allograft vasculopathy (ISHLT CAV2). Specifically, a sub occlusive ostial left main coronary artery lesion was treated by percutaneous angioplasty and stent implantation. Since a lower LDL target was needed (LDL–C 55 mg/dL), Alirocumab was introduced. However, 6 months later, no significant LDL levels reduction had been observed. The shift to evolocumab led to the same result, although the patient reported full therapeutic compliance. In the suspicion of anti–drug antibodies development, we opted for Inclisiran without achieving a significant decrease in LDL–C levels. In parallel with the search for genetic mutations in the PCSK9 gene and other genes involved in cholesterol metabolism (including LDLR, APOB, and LDLRAP1 and resulting positive only for common variants in LDLR receptor), therapy with bempedoic acid was initiated, again without substantial benefit (LDL–C 118 mg/dL).

Conclusion
Despite cholesterol management has been simplified by the introduction of new drugs, it may still be complex in patients requiring very low LDL–C levels in some specific cases. Drugs with a LDL receptor–independent mechanism of action (e.g., evinacumab) may allow the achievement of therapeutic goals suggested by clinical guidelines.
EARLY DIAGNOSIS OF A RARE PULMONARY VASCULAR CONDITION: THE NEED FOR A HIGH INDEX OF SUSPICION, A GOOD NETWORK, AND THE RIGHT TOOLS
Authors: Gagliardi M , Di Marco F , Bandera F , Perego G , Badano L , Parati G , Baratto C , Caravita S 
Published: May 2024

Abstract
Pulmonary veno–occlusive disease (PVOD) is a rare and deadly form of pulmonary arterial hypertension (PAH), characterized by preferential venular involvement/obliteration. This leads to reduced diffusion capacity of the lung (DLCO), oxygen desaturation, and pathognomonic alterations at chest computed tomography (CT): mediastinal lymph node enlargement, centrilobular ground–glass opacities, smooth thickening of the interlobular septa. PVOD is generally a late diagnosis that may benefit of lung transplantation only: acute pulmonary edema may occur after initiation of PAH–specific treatment. We report the case of a middle–age man in whom PVOD diagnosis was obtained only within 6 months from onset of symptoms and despite the absence of obvious CT findings. Treatment with PAH–specific drugs resulted in clinical and hemodynamic benefit. A 57–year–old male triathlete started complaining exertional dyspnea since December 2022. In February 2023, acute and chronic pulmonary embolism, as well as intracardiac and intrapulmonary shunt were excluded. Chest CT did not reveal relevant significant abnormality. Lung volumes were normal but DLCO was severely reduced (40%). Echocardiography suggested a low probability of PH, and BNP was normal. Right ventricular dimensions and function were within normal limits (RVEDV 90 mL/m2, RVEF 53%). Cardiopulmonary exercise test showed a moderate reduction of exercise capacity, an exercise–induced oxygen desaturation and a marked exercise–induced hyperventilation. He was thus referred to our center to undergo right heart catheterization (RHC) and invasive CPET (table). Genetic testing excluded EIF2AK4 gene mutation. A presumptive diagnosis of sporadic PVOD was made. Treatment with macitentan 10 mg was started, and well–tolerated. Three months later, both hemodynamics and exercise capacity were nearly normalized (table). Further treatment escalation adding tadalafil 40 mg was discussed and started. After that, the patient reported additional symptomatic benefit with improved exercise tolerance. This case raises several considerations: i) we need a high index of suspicion and a network approach to obtain early diagnoses of rare pulmonary vascular diseases; ii) CPET can enhance the diagnostic suspicion; iii) a low threshold for RHC in patients with unexplained dyspnea might be considered; iv) an early diagnosis and treatment of PVOD with PAH–specific drugs in our case was associated with clinical improvement, despite their intrinsic risk.
ONE STEP BEFORE EOSINOPHILIC CARDIOMYOPATHY
Authors: Mastroberardino S , Segreti A , Piccirillo F , Grigioni F , Ussia G 
Published: May 2024

Abstract
A 57–year–old woman was admitted to our Emergency Department (ED) for chest pain, cough, and fever that appeared a few days before. She was affected by bronchial asthma since childhood. The EKG showed a new–onset right bundle block, and the blood samples increased myocardionecrosis markers. A coronary angiography was performed showing coronary arteries free from lesions. Therefore, a transthoracic echocardiogram demonstrated a significant diastolic dysfunction with reduced global longitudinal strain (GLS). A cardiac magnetic resonance (CMR) showed mildly reduced left ventricle ejection fraction (LVEF) with hyper–intensity of the anterolateral wall. The high–resolution chest tomography (HRCT) showed: diffuse parenchymal thickenings, partly with a ribbon–like morphology and a ground–glass appearance. A high eosinophil count was observed in peripheral blood and bronchoalveolar lavage (BAL) during hospitalization. To conclude, even a healthy person with an atopic pre–disposition may experience a hypersensitivity reaction to an inhaled antigen and develop a lung or cardiac affection. Our patient’s symptoms improved with high–dose steroids, as well as the chest computed tomography and the transthoracic echocardiogram showed a general improvement. This may be justified with a possible immune–related origin of the pathology so that eosinophilic pneumonia may be thought to represent the stage before Loeffler endocarditis.
MULTIDISCIPLINARY APPROACH TO A DIFFICULT CASE OF ACUTE HEART FAILURE DUE TO POSSIBILE ISOLATED CARDIAC SARCOIDOSIS
Authors: Mapelli M , Grilli G , Campodonico J , Vignati C , Baggiano A , Majocchi B , Bianchini L , Ianniruberto M , Doni F , Fazzari F , Tassetti L , Pontone G , Agostoni P 
Published: May 2024

Abstract

Background
Cardiac sarcoidosis (CS) is a rare, infiltrative cardiomyopathy that results from granulomatous inflammation affecting the heart with high morbidity and mortality. It is usually associated with a systemic (i.e. pulmonary) involvement, but lone heart involvement has been described. Even with extensive multidisciplinary evaluation making a firm diagnosis is difficult, especially in case of isolated CS where the endomyocardial biopsy (EBM) diagnostic yield is low (20–30%).

Case Description
A 71–year–old patient, heavy smoker with no previously known diseases, was admitted with a 3–month history of dyspnea. On admission he showed typical features of heart failure with reduced ejection fraction (HFrEF). A coronary angiography excluded coronary artery disease, while echocardiography (TTE), cardiac magnetic resonance (CMR), and positron emission tomography (FDG–PET) showed a pattern consistent with a non–ischemic dilated cardiomyopathy with reduction in systolic function, and signs of active inflammation (highly suspected CS). Two electroanatomic mapping driven EBM were made but no clear histological signs of CS (i.e., granulomatous inflammation) were detected. The patient was treated with corticosteroids and HFrEF optimal medical therapy (sacubitril/valsartan, mineralocorticoid receptor antagonists, beta–blockers, dapagliflozin) and a temporary wearable defibrillator (LifeVest) was applied. At 2–month follow–up, despite a moderate improvement in the systolic function and HF symptoms, after a multidisciplinary discussion, considering the extensive left ventricle scar, inflammatory involvement, and the presence of multiple non–sustained ventricular tachycardia, a permanent cardioverter–defibrillator (ICD) was implanted.

Conclusion
This case highlights how, even with (repeated) extensive multidisciplinary evaluation (TTE, CRM, FGD–PET, EBM), a definitive diagnosis of isolated CS often remains presumptive. The presence of an experienced cardiomyopathy team (HF specialists, cardiac imager, electrophysiologists, internal medicine doctor) can help in the diagnostic–therapeutic management of these patients with inflammatory heart disease.
A RARE CASE OF IPILIMUMAB/NIVOLUMAB–INDUCED MYOCARDITIS COMPLICATED BY HIGH GRADE ATRIOVENTRICULAR BLOCK IN A PATIENT WITH METASTATIC RENAL CELL CANCER
Authors: Logiacco A , Basile P , Mandunzio D , Gentile M , Carella M , Memeo R , Santobuono V , Forleo C , Ciccone M , Guaricci A 
Published: May 2024

Abstract

Background
Immune checkpoint inhibitors (ICIs) such as Ipilimumab and Nivolumab have revolutionized the field of cancer therapy. These monoclonal antibodies help to reinforce antitumor immunity, resulting in robust activation of the immune system which promotes tumor cell death. T–cell–targeted immunomodulators are now widely used in the treatment of about 50 cancer types, including metastatic Renal Cell Carcinoma (RCC). However, immune responses by ICIs, especially when used in combination, may lead to systemic immune–related adverse events (irAEs), such as inflammatory and non–inflammatory cardiotoxicity.

Case Presentation
A 70–years old woman without previous cardiovascular events, affected by RCC metastasized to lungs, was admitted to our intensive care unit for the onset of worsening fatigue and dyspnea after a second cycle of ICI–based combination therapy (Ipilimumab plus Nivolumab). The 12–lead ECG showed a third–degree atrioventricular block (AVB) with junctional escape rhythm and negative T–waves in inferior and lateral leads. The laboratory examinations revealed increased values of troponine I, myoglobin, C–reactive–protein and NT–pro–BNP. The transthoracic echocardiography was within the normal range. A temporary cardiac pacing was provided and an acute coronary syndrome was rule out by coronary angiography. High doses of intravenous methylprednisolone were initiated for the suspicion of ICIs–related myocarditis. Moreover, due to the persistence of the conduction disorders in the following days, a leadless atrio–ventricular pacemaker (PM) was implanted. During the in–hospital follow up no complications occurred, and the patients was discharged at home in good clinical condition. The therapy with ICIs was not resumed during the follow up.

Discussion
ICIs have improved the clinical outcome for several malignancies, although they may cause a wide spectrum of severe irAEs. ICI–related myocarditis is a rare but potentially life–threatening event with a high mortality without an early recognition. A strict monitoring involving cardiologists and oncologists should be advisable in patients treated with these innovative drugs, for a prompt identification and treatment of irAEs. In presence of these serious adverse events, the discontinuation of the immunotherapy with or without the administration of glucocorticoids should be considered. The development of persistent conduction alterations is infrequent and may require the implantation of a permanent PM.
A CASE OF STEMI COMPLICATED BY CARDIOGENIC SHOCK TREATED USING A MINIMAL APPROACH
Authors: Cangemi S , Inglese F , Vinci D , Barone G , Ferrarello S , Geraci G 
Published: May 2024

Abstract

Introduction
An 88–year–old woman was admitted to our hospital for a posterior STEMI. The medical history was significant only for arterial hypertension. The echocardiography showed a mild left ventricular global dysfunction (EF 48%) with hyperkinesia of the posterior wall. After administration of loading doses of acetylsalicylic acid, clopidogrel and heparin, the patient underwent coronary angiography.

Investigation
The coronary angiography showed an occlusion of a dominant left circumflex (LCx) in the proximal tract and an irregular plaque, determining a critical stenosis of the ostial left anterior descending (LAD) artery. After the diagnostic examination, the patient becomes very hypotensive (65/45 mmHg arterial blood pressure) and sleepy. Management Considering the impossibility of using the impella and the IABP because they were already used for other patients, we decided for a minimal approach guided by intracoronary imaging. We used an XB 3.0 6F guide catheter, crossed the LCx with a BMW wire, and performed an inflation with a Semi–compliant 2.0x20 mm balloon, obtaining TIMI 3 flow. The “culprit lesion” was in the proximal tract of a trifurcation with three vessels of equal size. We decide to implant a 2.75x8 mm stent covering the lesion without protruding inside the left main. Because the patient was still hypotensive and started to have frequent ectopic ventricular beats often organized in run we decided to treat also LAD ostium stenosis avoiding to cover left main in the acute phase. So we implanted a 3.0x15 mm on LAD “nailing” ostium using only angiographic projection. Without the need to administer X, the patient achieved normal blood pressure and became alert. After stabilization of the patient we checked the stent on the LAD and the left main with IVUS. The stent had no significant underexpansion, and the trunk was free of significant plaque or dissection. After primary PTCA in the UTIC, the patient had no complications at 12 months follow up.

Conclusions
Our clinical case is an example of how in primary angioplasty, often based on the coronary anatomy, a minimal approach with the aim of guaranteeing a stable TIMI 3 coronary flow and avoiding prolonged dilation on the common trunk can guarantee the patient‘s survival. The stenting technique must be personalized based on the patient‘s clinical conditions and the resources available.
BIFURCATION: A CLINICAL CASE IN ACUTE CORONARY SYNDROME
Authors: Ferrarello S , Vinci D , Priolo L , Cangemi S 
Published: May 2024

Abstract

Bifurcation
A clinical case in acute coronary syndrome. Coronary bifurcation lesions are considered one of challenging entities in the field of coronary intervention due to the risk of side branch loss and higher risk of stent thrombosis. There are limited data on the preferred treatment strategy in ST–segment elevation myocardial infarction (STEMI) patients with bifurcation lesions as most dedicated study excluded patients with acute coronary syndrome. In all comers population a 10–20% of STEMI patients had bifurcation culprit lesion. In 2018 guideline suggested a provisional approach by stenting the main vessel and implanting a second stent only if necessary as a bailout strategy. This reccomandation was supported by a large series of study that uniquely documented the superiority of one versus two stent technique. The same message could also be exported in the context of acute patients, as our clinical case, thanks to registry like COBIS 2 including ST–segment elevation patient. But this precious advice cannot be applied in all clinical subsets. Especially when we manage with a relevant side–branch and when the risk of loosing the side branch is very high. In this subgroup of patient, bifurcation lesion may benefit from a 2 stent approach. Different technical option are available. Among these, DK–crush and inverted Main Branch stenting across Side Branch (Invertend Culotte or inverted T technique) could be the best choice. Our patient was a young lady presenting with a lateral STEMI (D1 and aVL). The culprit lesion was a Medina 1.1.1 bifurcation lesion involving left anterior descending and first–diagonal. Since our side branch had thrombus inside, the vessel was relevant by definition. Other anatomical characteristique to take into account: relevant size and covering a large myocardial territory. Planning the strategy to treat our LAD–D1 bifurcation we consider the angle and the difference in size between main and side branch. We decided to proceed with DK–crush and its recent evolution DK–nano crush. In conclusion “do it provisional” guideline message is not always possible, 2 stent technique seems to be the best choice in presence of a relevant side–branch / high risk to loose it. Our take home message is, if the clinical scenario allow it, don’t hesitate to perform a multistep two vessel strategy technique and to try to reconstruct perfect anatomical carina even in STEMI context.
PROSTHETIC GRAFT INFECTION: WHEN ECHOCARDIOGRAPHY IS NOT ENOUGH!
Authors: Pezzi L , Scollo C , Rossi D , D‘Andria R , Benedetti C , Magnano R , D‘Alleva A , Forlani D , Vitulli P , Genovesi E , Gallina S , Di Marco M , Paloscia L 
Published: May 2024

Abstract
A 68–year–old Caucasian male, post–Bentall procedure with a bioprosthetic valve following extensive aortic dissection involving the ascending aorta and aortic arch, presented to the Emergency Department with persistent fever for over a month. During his hospitalization, he experienced ventricular fibrillation in the setting of hypokalemia, successfully managed with Advanced Cardiovascular Life Support (ACLS) and DC shock, leading to the restoration of sinus rhythm. Subsequently, he was admitted to the Cardiovascular Intensive Care Unite. Blood tests revealed neutrophilic leukocytosis with elevated inflammatory markers, anemia, and a troponin curve consistent with myocardial injury in the context of sepsis and anemia. Echocardiography demonstrated a left ventricle of normal dimensions with globally depressed function (EF 45%), attributed to apical akinesia. The prosthetic valve was functioning normally, and there were no signs of endocarditic vegetations or pericardial involvement. A transesophageal echocardiogram confirmed the absence of vegetations. Given echocardiographic signs of myocardial ischemia, coronary angiography was performed, revealing a coronary circulation free from significant stenosis. Cultural exams showed positivity for Enterococcus faecalis. A PET–CT revealed the presence of radioisotope uptake around the vascular prosthesis situated in the aortic arch. An Infectious Disease specialist recommended antibiotic therapy with Piperacillin/Tazobactam and Teicoplanin. At the one–month follow–up, the patient remained asymptomatic, free from new febrile episodes. The follow–up echocardiogram indicated improved global systolic function, and inflammatory markers remained consistently negative. Repeat blood cultures were negative. This case illustrates a suspected endocarditis on a prosthetic valve where both transthoraci and transesophageal echocardiography yielded negative results. The use of PET–CT, on the other hand, revealed the presence of vascular graft infection, proving to be a valuable method in this clinical setting.