bluuebunny/crossref_metadata_2025_split huggingface dataset:
UNEXPECTED RIGHT ATRIAL MASS IN A YOUNG WOMAN: AN UNPLEASANT SURPRISE
Authors: Mastroberardino S , Piccirillo F , Fiorentino M , Grigioni F , Ussia G 
Published: May 2024

Abstract

Introduction
Atrial masses represent a diagnostic challenge as the differential diagnosis includes a broad spectrum of formations. Thrombus is the most common form of non–neoplastic mass, whereas cardiac tumors are rare. We reported a case of a young woman with high–grade endometrial stromal sarcoma, complicated by cardiac metastatic dissemination.

Case Report
A previously healthy woman of 36–years old, without medical history, underwent abdominal ultrasound due to frequent menorrhea. As suspicious uterine formation was observed, an abdomen magnetic resonance (MRI) was performed, showing a large formation located in correspondence of uterine corpus and fundus. Indeed, she underwent hysterectomy plus bilateral salpingectomy, and the subsequent histological examination revealed a high–grade endometrial stromal sarcoma (ESS). Consequently, the patient was hospitalized to undergo a total body computed tomography (CT) for tumor staging and to start chemotherapy (potential cardio–toxic). A trans–thoracic echocardiogram (TTE) showed normal function of LV, without valvulopathies. However, in correspondence of right atrium (RA) roof, a large, rounded mass, hypomobile and iso/hyper–echogenic, without extension into Superior Vena Cava, was detected. The total body CT confirmed the presence of a hypodense mass in the RA, and secondary lesions within left large pectoral muscle and right inferior lung lobe were founded. Thus, a Cardiac MRI was performed, showing a solid formation, with lobulated margins and oval morphology, at the level of the posterior wall of the RA, of about 2.5 x 1.8 cm and with longitudinal extension of about 3 cm, hypomobile and with a wide implant base. The lesion appeared heterogeneously hypo–isointense in T1–weighted images and hyperintense in T2–weighted images; in Delayed–Enhancement acquisitions, the formation resulted hyper–intense, more evidently in long–inversion recovery sequences. Indeed, considering clinical condition, right atrial location and morphological features, the diagnosis of secondary lesion of the ESS was entertained. First–line chemotherapy regimen was started. After 6 months follow–up the patients remained asymptomatic; at TTE, the cardiac mass resulted unchanged. Unfortunately, after 13 months, the patient died.

Conclusion
A multimodality imaging approach to the evaluation of atrial masses is crucial for obtaining a non–invasive evaluation to provide the appropriate therapeutic approach.
AN UNUSUAL “BLACKBERRY SHAPED” MASS OF DUBIOUS INTERPRETATION (OUT OR IN THE JUGULAR VEIN?) IN A PATIENT WITH METASTATIC MELANOMA. A SPECIAL IMAGING CLINICAL CASE
Authors: Fioretti A , Leopizzi T , Strippoli S , Garofalo M , Pesola F , Pizzutilo P , La Forgia D , De Luca R , Oreste D , Galgano G , De Giosa A , Saliani P , Scicchitano P , Zito A , Oliva S 
Published: May 2024

Abstract

A 43 y–o woman, without cardiovascular risk factors, was diagnosed with BRAF+ IIIC melanoma of unknown origin with bilateral axillary lymph node metastases. She started a combined treatment with vemurafenib, cobimetinib (braf/mek inhibitors) and atezolizumab (PDL1 inhibitor) as neoadjuvant therapy, followed after 6 weeks by surgical treatment (that showed pathological complete response) and adjuvant immunotherapy with atezolizumab hitherto ongoing. A serial CT–scan revealed a 0.9x1.3 cm mass (Fig.1) close to the right jugular vein (RJV) with increased contrast enhancement susceptible of vessel ectasia by the radiologist who expressed uncertainty about its site (intra or extravascular?) and asked for further imaging assessment. Therefore, a duplex ultrasound (DU) was performed with the detection of a 0.6x1.2 cm endovascular mobile “blackberry shaped” mass in the RJV and the indication for a vascular surgery evaluation. The patient started enoxaparin 1 mg/Kgx2/die s.c. according to “ex iuvantibus therapy”. However, the vascular surgeon diagnosis confirmed the undetermined site of the mass. The patient decided for a second DU opinion and reached our Cancer Angiology Lab, where a novel DU detected a deep vein thrombosis (DVT) of the RJV (Fig.2–Fig.3). Enoxaparin was replaced with edoxaban 60 mg 1 cp/die since the patient preferred a more manageable root of administration for the requested long–term anticoagulant treatment. After 2 months a DU follow up showed no regression of the mass. Accordingly, the patient proceeded with edoxaban 60 mg/die without any side effects, whereas still in treatment with immunotherapy.

Conclusions
Thromboembolism is the second leading cause of death in malignancy. Overall, biomarkers and thrombotic risk factors related to patient, tumor and anticancer regimens favor the development of cancer associated thrombosis. Delay in DVT diagnosis and, mostly, in the initiation of a full and long–term anticoagulant treatment, like in this case, enables a chronic evolution of the clot which makes its timely regression more challenging.
A CURIOUS CASE OF CRYPTOGENIC MYOCARDIAL INFARCTION
Authors: Peano V , Gallone G 
Published: May 2024

Abstract

Introduction
Myocardial Infarction with Non–Obstructive Coronary Artery disease (MINOCA) is an increasingly recognized clinical entity requiring a comprehensive and precise work–up to detect the correct diagnosis and to appropriately institute treatment strategies. We report a case explanatory of a rare cause of MINOCA, highlighting the importance of a comprehensive diagnostic algorithm.

Case
A 55 years old man with an intermediate cardiovascular risk profile and no prior medical history presented to the emergency department. He experienced typical chest pain at rest 6 days prior to admission, but refused to attend the hospital and, instead, performed outpatient labs on day 5 demonstrating elevated high sensitivity troponin I (Hs–TnI, 3192 ng/L). At admission, the patient was asymptomatic and well compensated. The ECG was normal, echocardiography revealed a mid–basal inferior hypokinesia, and lab. exams showed a hs–TnI of 159 ng/L. The coronary angiogram was normal: no atherosclerosis was present and, upon independent revision of the images from experienced operators, no coronary dissection, myocardial bridges or emboli were observed. Ventriculography ruled–out ongoing Takotsubo Cardiomyopathy, nor the history was suggestive of any trigger. No clinical, ECG and echocardiographic features of myocarditis or cardiomyopathy were present and a classic troponin curve was observed. No clinical features of endocarditis were present. No ventricular or atrial arrhythmias were detected on continuous monitoring. A working diagnosis of MINOCA was established. Cardiac magnetic resonance revealed a focal area of transmural late gadolium enhancement and T2–based enhancement of the inferior mid–basal wall consistent with acute myocardial damage. In the suspect of an embolic source, transesophageal echocardiogram documented the presence of a patent foramen ovale (PFO) with characteristics at high embolic risk: large aneurysmatic fossa ovale type 4 LR (Olivares Reyes) with left to right shunting at baseline which increased during Valsalva maneuver, prominent Chiari network and Eustachian valve. Given the high probability of association between PFO and systemic embolism phenomena, percutaneous closure of the PFO was proposed.

Conclusion
With this case we want to highlight the importance of the correct and precise diagnostic work up in this emerging pathology in the field of cardiology for a better definition of the therapeutic strategy.
TREATMENT OF CALCIFIC CORONARY STENOSIS WITH SHOCKWAVE: A CASE REPORT
Authors: Faraci A , Adorno D , Messana D , Grimaudo R , Sucato V , Novo G 
Published: May 2024

Abstract

Introduction
Woman 72 years old, suffering from hypertensive ischemic heart disease, type II diabetes mellitus with accessory circulatory complications and pure hypercholesterolemia. He arrives at our cath lab urgently for ST–segment elevation myocardial infarction (STEMI). We proceed with coronary angiography to treat the culprit lesion. On the coronary angiographic picture, thrombotic occlusion is found at the proximal edge of the stent previously implanted in the middle right coronary artery, the left coronary artery shows mild diffuse atheromasia in the absence of angiographically significant stenosis. An attempt is made to reopen the vessel with multiple pre–dilations with semi–compliant and non–compliant balloon catheters without obtaining complete expansion. Given the impossibility of implanting the stent, dilation is performed with a 2.5x20mm medicated balloon for one minute and the use of Shockwave intravascular lithotripsy (IVL) is planned as a second step.

Methods
IVL involves the generation of pulsatile sound waves in the target site aimed at combating the occurrence of microfractures of the calcific tissue, modifying the compliance of the vessel, without however damaging it. In detail, the system is composed of an electrical pulse generator, a connection cable and a balloon catheter. After the standard preparation of the balloon with a mixture of saline solution/contrast medium, it is advanced on a 0.014" guide up to the level of the stenosis to be treated.

Results
After preparation and use of the shockwave intravascular lithotripsy (IVL) system with a 2.5x12 mm balloon, 5 cycles of 10 pulses were delivered for a total of 50 pulses. Dilation follows with a non–compliant 2.5x15 mm balloon with optimal expansion of the balloon. We then proceeded with the implantation of the 2.75x16 mm drug–eluting stent in the middle section overlapping with the stent already present. Obtaining a good final angiographic result and adequate coronary flow (TIMI 3).

Conclusions
The use of coronary intravascular lithotripsy made it possible to optimize the result of the previously failed angioplasty, pursuing an excellent angiographic result with limited risks, compared to other debulking systems.
DOUBLE ST–ELEVATION, DOUBLE OCCLUSION?
Authors: Ajmone F , Mancone M , Sardella G , Tocci M , Bruno E , Improta R , Di Pietro G , Giansante A , Vizza C , Colantonio R 
Published: May 2024

Abstract

Case Presentation
A 71–year–old woman was referred to our outpatient clinic for two episodes of chest pain during last two days. Her past medical history was remarkable for arterial hypertension, obesity, diabetes mellitus type 2 treated by oral hypoglycaemic agents complicated by diabetic maculopathy.

Diagnostic Work–Up
At the presentation, the patient was symptomathic for chest pain, hemodynamically stable. The ECG showed synus rhythm, left–axis deviation, Q wave and ST–elevation from V2 to V5 and in DIII and ST–elevation in DII and aVF (FIGURE 1). Transthoracic echocardiography revealed concentric hypertrophy, mildly reduced LV ejection fraction (EF 45%) with akinesia of intreventricular septum and apex, without severe valvulopathies and no pericardial effusion. Given the clinical data the patient was addressed to urgent coronary angiography that showed LAD occlusion at the end of his proximal tract and right coronary artery occlusion at the beggining of his second tract (FIGURE 2); furthermore omo and etero–coronaric collaterals for right coronary artery area were detected. By using a workhorse guidewire flow was restored in both coronary arteries and PTCA was performed with 3 DES on LAD and other 2 DES on right coronary artery (FIGURE 3). The patient was transferred to our coronaric intensive unit care after the procedure; no complications were noticed during the hospitalization. A week after the patient was discharged in good general health.

Conclusion
Multiple simultaneous coronary occlusions are reported in literature, yet the accurate incidence and physiopathology of this occurrence is still uncertain. Probably the symphathetic system activation caused by first coronary occlusion could promote an increased basal platelets activation and then the second coronary occlusion. Our case shows that during coronary angiography identifying a culprit lesion may be delicate and cardiologists should be prepared to manage multiple coronary occlusions.
WAITING FOR A CONCERT IN SUMMER HEAT: A TRANSIENT ST ELEVATION IN A YOUNG MAN
Authors: Benzoni G , Perelli F , Bilo B , Giuliano A , Parati G , Brambilla R 
Published: May 2024

Abstract
A 22–year–old man, without prior cardiological history, was waiting for a concert to start many hours in summer heat. Suddenly he started to feel chest pain, dyspnea, and palpitations. Electrocardiogram (EKG), registered from first aid responders, showed a ST elevation in V1–V3 leads and ventricular repolarization alterations in inferior leads. The patient was brought to Emergency department (ED) where a new EKG resulted normalized, blood test samples showed High Sensitive Troponin T (HsTn) 35 ng/L (at plateau in three seriate measures), serum creatinine 2.0 mg/dL, total Creatine Kinase 646U/L, Ck–MB 10.6 ng/mL, Reactive C Protein (RCP) 1.2 mg/dl. The toxicological evaluation was negative. Transthoracic Echocardiography (TTE) did not show any regional ventricular kinesis alterations nor valvular defects, global systolic function was preserved. A Coronary Computed Tomography (CT) scan showed an intramyocardial bridge on Anterior Descending (DA) Coronary Artery associated to 7 mm pericardial effusion without coronary stenosis. A Cardiac Magnetic Resonance (CMR) with gadolinium was performed, which did not show (in T2 mapping sequences) oedema nor Late Gadolinium Enhancement (LGE). Considering a coexistence of Brugada syndrome pattern at EKG, an Ajmaline testing was performed, which resulted negative. Discussion: Dehydration due to any intake of water after standing many hours in summer heat could have been the trigger for a vasospasm of DA that in this patient has a partially intramyocardial course. This anatomical finding was not known until CT scan was performed. Our diagnostic hypothesis of a vasospasm on DA is supported by EKG alterations and elevation enzymes of myocardionecrosis, in absence of any coronary stenosis. Elevated creatinine values in the absence of other renal concomitant comorbidities were suggestive for acute dehydration too. Differential diagnosis took also into consideration myo–pericarditis, especially for the pericardial effusion finding revealed by CT scan: this diagnosis was not confirmed through CMR but also by the evolution of clinical conditions of our patient. We discharged the patient, asymptomatic with normalized renal function after some intravenous hydration and we prescribed a calcium channel blocker. At a follow up visit, three months after the acute event, our patient was in good clinical condition and denied occurrence of any other similar events.
THE GLA D313Y MUTATION IN FABRY DISEASE: DIAGNOSTIC DILEMMAS AND THERAPEUTIC CONSIDERATIONS
Authors: Dall‘Ospedale V , Valsania T , Di Spigno F , Matrone B , Novara P , Tedeschi A , Turchio P , Bolognesi M , Aschieri D 
Published: May 2024

Abstract
Fabry disease is a genetic disorder caused by mutations in the GLA gene, which encodes alpha–galactosidase A, leading to enzyme dysfunction and subsequent accumulation of glycosphingolipids. In the classical form, it occurs in childhood or early adolescence with renal, cardiac, and neurological involvement, resulting in a poor prognosis. The GLA D313Y is a missense mutation resulting in a significant reduction of enzyme activity in plasma, while leukocyte enzyme activity remains within normal limits, leading to a "pseudo–deficiency" of enzyme activity with uncertain pathogenicity. In literature, it has been observed that individuals with this mutation may present predominantly with central neurologic manifestations, characterized by multifocal white matter lesions. Historically they do not receive indications for initiating enzyme replacement or molecular chaperone therapies. We present the case of a man affected by arterial hypertension and history of chronic lower limb pain who experienced a lacunar stroke at the age of 35. The GLA D313Y mutation was identified and enzyme activity assay in plasma was within normal limits, precluding the initiation of enzyme replacement therapy. Over the following years, he suffered two additional ischemic strokes, a progressive decline in renal function to stage 3, and acute coronary syndrome. Transthoracic echocardiography revealed hypertrophic non–obstructive cardiomyopathy (SIVd 20 mm) with normal biventricular ejection fraction. Cardiac magnetic resonance showed late gadolinium enhancement of inferior junctional and inferior wall in middle segment (Fig.1). Considering the patient‘s clinical conditions involving multi–organ impairment, an enzymatic activity test was performed on leukocytes, revealing a reduced activity. This prompted the decision to initiate enzyme replacement therapy. This case report supports the hypothesis that the D313Y mutation may be associated with a later presentation and not only neurological involvement. In our experience, it’s mandatory to proceed with the determination of leukocyte enzyme activity in presence of normal enzyme activity in plasma. As an alternative, the detection of glycosphingolipids’ accumulation could be useful, but myocardial and renal biopsy poses a high bleeding risk, especially in anticoagulated or antiaggregated patients. The authors propose considering the initiation of therapy in patients with the D313Y mutation who exhibit significant organ involvement.
STRESS–INDUCIBLE MYOCARDIAL ISCHEMIA DUE TO A VENTRICULO–CORONARY FISTULA: WHAT WE KNOW
Authors: Testa S , Gallina S , Renda G , Ricci F 
Published: May 2024

Abstract
Coronary fistulas represent communications that form between the coronary arteries and a heart chamber or a large vessel. The real incidence is not known as well as their real clinical impact. Typically congenital, they can also develop following surgery. The treatment of fistulas remains complex: in the past surgical treatment was opted for, currently percutaneous closure is preferred although many hemodynamists prefer a conservative approach. We describe the case of a man with stress–inducible ischaemia, probably due to the presence of a ventriculo–coronary fistula. 58–year–old Caucasian man arrives for coronary angiography following a positive ergometric test for signs and symptoms of reduced coronary reserve. The patient had recently undergone a cardiac MRI that showed an hypertrophic cardiomyopathy with partial end systolic obliteration of the apex with areas of intramyocardial fibrosis in middlewall distribution. Coronary angiography showed an epicardial coronary circulation free from angiographically significant lesions with evidence, however, of a ventriculo–coronary fistula (departing from the anterior interventricular artery). Since other causes were excluded, the hypothesis was that the fistula could be the cause of stress–inducible myocardial ischemia, through a coronary steal mechanism.
A "PARADOXICAL" MINOCA
Authors: Sarti A , Cocco M , Campo G , Tonet E 
Published: May 2024

Abstract

Background
The acronym MINOCA refers to myocardial infarction with normal or near–normal coronary arteries on invasive angiography. We report a case of MINOCA caused by paradoxical coronary embolism due to a right–to–left shunting through a patent fossa ovalis. Integrated multimodality imaging diagnostic work–up has been crucial for identifying the mechanism underlying MINOCA.

Case Report
A 48–year–old male was admitted to the Coronary Care Unit for sudden–onset oppressive chest pain lasting 1 hour, radiating to the left arm; no specific changes in ST–segment on a 12–lead ECG and elevated high–sensitive troponin I (peak value 1562 ng). Transthoracic echocardiography showed normal dimensions and function of the cardiac chambers and excluded valvular and pericardial diseases. Given the characteristics of the chest pain, we performed an urgent coronary angiography that showed normal epicardial coronary, including testing for epicardial coronary spasm and microvascular dysfunction. We also used Optical Coherence Tomography on LAD e RCA to rule out critical stenosis and sign of plaque instability. To investigate the etiology of myocardial damage the patient underwent a cardiac magnetic resonance (CMR) imaging that yielded the following findings: hypokinesis of the inferior–middle segment of the left ventricle associated myocardial edema and near transmural LGE. These features were consistent with a recent ischemic injury. Additionally, CMR showed an atrial septal aneurysm and a channel–like pattern of the interatrial septum, raising the suspicion of patent foramen ovale (PFO). The transesophageal echocardiography and transcranial Doppler with bubble test confirmed the presence of a PFO responsible for right–to–left shunting. Atrial fibrillation was excluded with ECG telemetry throughout the entire 7day hospitalization.

Conclusions
Based on the result of the overall clinical–instrumental evaluation, MINOCA caused by paradoxical coronary embolism due to a right–to–left shunting through a PFO was considered the most plausible diagnostic hypothesis. The patient underwent to interventional percutaneous closure of PFO and was discharged with no symptoms or complication at the follow–up.
CARDIOGENIC SHOCK DUE TO SPONTANEOUS CORONARY ARTERY DISSECTION
Authors: Catellani D , Paoli G , Tadonio I , Grossi S , Niccoli G 
Published: May 2024

Abstract

Spontaneous coronary artery dissection (SCAD) is an important but rare cause of myocardial infarction (MI) in young adults, particularly women. Current guidelines recommend conservative treatment, given the low angiographic success and the high complication rate of percutaneous coronary intervention. However, in SCAD patients presenting with STEMI or cardiogenic shock the needs of urgent revascularization is critical and PCI may be life threatening. We present a case report occurs in our Hospital of a 42–year–old young woman without cardiovascular risk factors or notable comorbidities which was admitted to our emergency department for typical chest pain. At admission the patient had a cardiovascular arrest from refractory ventricular fibrillation who was rescued according to ACLS protocol. After resumption of ROSC, EKG showed anterolateral STEMI (FIGURE 1). Bedside echocardiography showed a severe reduction in systolic function (EF 30%) due to akinesia of the apex, septum and antero–lateral wall, with absence of noticeable valvulopathy, pericardial effusion or pathological findings in the right heart sections. Therefore the patient underwent to Cath–lab for emergent coronary angiography that showed ostial DA subocclusion with SCAD type 2a on middle tract (Figure 2) IVUS (intravascular ultrasound) imaging confirmed the diagnosis of SCAD, served to guided angioplasty on the LM–DA axis and to confirme optimal stent placement (FIGURE 3). Subsequently, the course was complicated by a double shock component: first SCAI C cardiogenic shock that requested or IMPELLA CP implantation and second haemorrhagic shock due to profound hepatic bleeding because of traumatic injury caused by prolonged resuscitation maneuvers. Mechanical ventricular support ensured haemodynamic stability to allow percutaneous embolisation of the hepatic arteries and patient stabilization. Once the haemorrhagic focus was treated and cardiac function recovered, the patient‘s condition gradually improved leading to a rapid weaning of haemodynamic and respiratory support. No neurological sequelae were observed after the acute phase.

Conclusions
1) Urgent revascularization in patients with SCAD and high clinical instability is life threatening. IVUS imaging is crucial to confirm the diagnosis and guide the procedure. 2) The IMPELLA support in this case provided short–term haemodynamic support in a double component of shock.