bluuebunny/crossref_metadata_2025_split huggingface dataset:
A RARE CAUSE OF PULMONARY EDEMA, INCREASED AORTIC GRADIENTS AND DECOMPENSATED HEART FAILURE: A CASE REPORT
Authors: Desalvo P , Giordana F , Coppini L , Borzillo I , Ferraro I , Ruffino E , Maiellaro F , De Benedetto Fabrizi M , Cinconze S , Baralis G , Annoni G , Rossini R 
Published: May 2024

Abstract
A 58–year–old male consulted our emergency department (ED) in May 2023 for new–onset severe dyspnea and ankle swelling. Patient history included previous aortic valve replacement (STJ 23 mechanical aortic valve) in 2004 for sub–valvular stenosis due to subaortic membrane. ED transthoracic echocardiogram (TTE) showed a dilated left ventricle (LV) with severely depressed ejection fraction (EF: 25%), LV non–compaction (LVNC), aortic mechanic prosthesis with increased gradients (49/32 mmHg), no perivalvular leaks, dilated pulmonary artery. Increased aortic gradients raised suspicion of aortic prosthetic thrombosis as the cause of acute pulmonary edema. However, urgent fluoroscopic investigation proved normal mobility of the hemidisks (Fig.1). Patient was admitted to cardiac intensive care unit. TTE also showed increased LVOT gradients, suggesting a hyperdynamic condition. Lab tests ruled out hyperthyroidism, sepsis and anemia. RV stroke volume was measured: QpQs was 0.7. Careful search for intra/extra–cardiac shunts with TTE ruled out intra–cardiac shunts, but highlighted a patent ductus arteriosus (PDA). PDA represents a peculiar case in which, despite being a left to right shunt, echo–measured QpQs results &lt;1 as pulmonary flow is measured in the RVOT, upstream of the shunt. CT ruled out pulmonary embolism and coronary artery disease and confirmed the presence of PDA and pulmonary artery dilation. Transesophageal echo confirmed the PDA (Fig.2) and highlighted coronary sinus dilation due to persistence of left superior vena cava (PLSVC). Right heart catheterization (RHC) showed a significant shunt (Qp/Qs 1,57) with increased pulmonary pressure (mPAP 33 mmHg) and pulmonary vascular resistance (PVR, 4,03 WU). Cardiac MRI confirmed LVNC with late gadolinium enhancement in the basal septum. LV function improved (EF 36%, Fig.3) after optimal unloading. Patient was discharged with optimal medical therapy for HFrEF (ARNI, SGLT2i, MRA, BB) and was referred to hub congenital heart disease Center (Regina Margherita Hospital, Torino) for percutaneous duct closure after balloon closure test. Percutaneous closure was successfully performed without any complications. RHC three months after closure showed normalization of PVR (0,9 WU) and pulmonary pressure (mPAP 19 mmHg). To our knowledge, this is the first reported case of subaortic membrane, LVNC, PDA and PLSVC. Patient was successfully treated with percutaneous PDA closure and is currently asymptomatic, class NYHA 1.
OVERCOMING EXTREME MEDICAL–SURGICAL EVENTS, IN CARRIERS OF CRTD AND CCM. CLINICAL STABILIZATION
Authors: Incampo G , Rizzo D , Mallardi A , Bux F , Campagna M , Ruggeri R , Cuonzo M , Valecce R , Moramarco M , Montemurro M , Giannoccaro V , Marino F , Bonfantino M , Pirozzi M 
Published: May 2024

Abstract
A 63–year–old male patient suffering from chronic dilated ischemic heart disease, with severe depression of contractility and estimated EF of 16%, carrier of CRT–D and CCM, in follow up at our center. Extreme events of medical/surgical interest have occurred in the last period: the patient underwent strangulated hernia surgery with ileostomy and subsequent stay in intensive care. Transferred spontaneously to cardiology where he was treated for heart failure also with norepinephrine and levosimendan. Once the CCM was reprogrammed with sensing recalibration and optimal delivery of therapy for 7 hours a day, interspersed with regular breaks, the patient showed a gradual improvement in vital parameters and in general clinical conditions, with more adequate blood pressure and diuresis regimes. The presence of the double device (CRT–D and CCM), together with medical therapy, allowed the patient‘s stabilization and clinical remission, with discharge and transfer to a cardiovascular rehabilitation center. The Cardiac Rhythm Management guaranteed by the CRTD, with optimization of medical therapy, together with the positive inotropic action of cardiac contractility modulation (CCM), allowed the patient to overcome high intensity surgical and medical events.
“A BROKEN TRANSPLANTED HEART”: A CASE OF TAKOTSUBO SYNDROME IN A HEART TRANSPLANT RECIPIENT
Authors: Delle Femine F , Liccardo B , Loffredo F , Pezzullo E , D‘Arienzo D , Amarelli C , Golino P 
Published: May 2024

Abstract
75–year–old female patient went to the emergency department for oppressive chest pain radiating to the left arm and dyspnea, developed after an argument with family members. The patient underwent heart transplant in 2010 because of chronic ischemic heart disease. In history: systemic arterial hypertension, former smoking, dyslipidemia, and chronic renal failure. At blood tests: TnIHS 78.4 ng/L (&lt;29 ng/L), CK–MB 1.9 microg/L, PROBNP–NT 43,000 pg/ml, creatinine 2 mg/dL. ECG showed inverted T waves in DI, AVL and V4 to V6 (fig 1). Transthoracic echocardiography was remarkable for a moderate reduction in systolic function (EF 38%), akinesia of apical portion, mid–segment of the IVS and mid–segment of posterior wall. Coronarography showed epicardial coronaries free of angiographically significant lesions. We decided to not perform ventriculography to avoid worsening renal function. At clinical stabilization, the patient underwent cardiac MRI with evidence of hypokinesia of the mid–ventricular segments with preserved global systolic function and hypo/akinesia of right ventricular apex with endocavitary thrombosis. Tissue characterization images were compatible with the presence of diffuse phlogistic involvement of the left ventricular myocardium (most evident at the mid–ventricular segments), in the absence of areas of myocardial necrosis. On pre–dimission echocardiogram, global left ventricular systolic function appeared markedly improved (auto EF 52%). DISCUSSION Our case is a rare case of Takotsubo syndrome (TS) in a transplanted heart. While TS accounts for 1–2% of all ACS in the general population, there is no precise data on its prevalence in heart transplant recipients. Although the pathophysiological mechanism underlying TS is still debated, there is evidence of hyperactivation of the sympathetic system with excessive catecholamine release. What is particular in our clinical case is that transplanted heart is denervated (because of resection of the post–ganglionic neural axons), although in some patients there is progressive reinnervation starting around the second year. Myocardial scintigraphy with iodine–123 meta iodobenzylguanidine can be used to study reinnervation. In our case, the presence of typical angor may be a clinical indicator of reinnervation, as many transplant patients present with CAV in the absence of chest pain.
SURGICAL REPAIR AND PERCUTANEOUS CLOSURE FOR POSTINFARCTION VENTRICULAR RUPTURE
Authors: Gattari B , Betta D , Gasparetto N 
Published: May 2024

Abstract

Case Report
A 44–year–old male was admitted to ICU for inferior STEMI in cardiogenic shock. The echocardiogram documented pericardial effusion in the right ventricle with initial signs of tamponade and the left ventricle with akinesia of the inferior–posterior wall and thinning of a small lateral portion, as from a pseudoaneurysm. Pericardiocentesis was performed with subsequent restoration of haemodynamic stability. The following emergency coronary angiography showed subocclusive stenosis of a thin postero–lateral branch (culprit lesion), critical stenosis at the origin of the anterior descending artery and occlusive stenosis of a developed diagonal branch. A CT scan documented a parietal breach in the middle of the left ventricle lateral wall with a pseudoaneurysm in close contiguity with the pericardium, in a setting compatible with contained cardiac rupture [Fig.1]. Hence, the patient underwent emergency cardiac surgery: a bovine pericardium patch was placed in the infarcted area, without complications. Later, percutaneous revascularisation on the left main–left anterior descending artery was performed. A few months later the patient was asymptomatic at a cardiological check–up, nonetheless, the echocardiogram revealed aneurysmal dilatation in the middle of the left ventricle lateral wall [Fig.2]; heart CT scan and ventriculography confirmed a pseudo–aneurysmal formation at the site of previous patch placement. Cardiac surgery was unfeasible due to high intra–operative risk, therefore pseudoaneurysm percutaneous closure under radioscopic and transthoracic echocardiographic monitoring was performed, with good final echocardiographic result [Fig.3].

Discussion
Cardiac rupture is a relevant high–mortality complication of acute myocardial infarction which needs to be treated promptly. Early diagnosis plays a crucial role in changing the patient‘s prognosis and is made possible by clinical, echocardiographic, haemodynamic and radiological criteria. The effectiveness of different treatment modalities is still controversial. In the presented case a surgical approach was preferred under emergency settings, while a less invasive percutaneous approach was chosen in the following and more stable situation.
HALF DOSE THROMBOLYSIS IN INTERMEDIATE – HIGH RISK PULMONARY EMBOLISM WITH TRICUSPID VALVE MOBILE MASS: A BAIL OUT STRATEGY
Authors: Roccabruna A , Giatti S , Pastore G , D’Elia K , Carraro M , Conte L , Marcantoni L , Aggio S , Ribichini F , Picariello C , Zanon F 
Published: May 2024

Abstract
A 82–year–old previously healthy male was referred to the emergency department complaining sudden onset dyspnea and witnessed syncope complicated by head trauma. Recurrent dizziness occurred previously. No history or drug consumption noted. On admission: heart rate 90 beats/min, blood pressure 110/60 mmHg, respiratory rate 25 breaths/min, oxygen saturation 87% in room air, normal body temperature. No lower extremities tenderness or swelling reported. Consistent with peripheral blood oxygen saturation levels, arterial blood gas analysis showed hypoxemia and respiratory alkalosis. Laboratory exams were significant for thrombocytopenia (89.000 cells/L), elevated cardiac troponin T levels (222 ng/L) and natriuretic peptide (NT pro–BNP 3944 pg/mL). Head CT excluded acute cerebral hemorrhage. At the electrocardiogram, signs of RV strain were present (biphasic T–waves in V1–V3 leads, S1–Q3–T3). Transthoracic echocardiography (TTE) showed dilated RV with hypokinetic free wall, “D–shaped” LV, “60/60” sign and a mobile filiform mass crossing the tricuspid valve plan in the right atrium (fig. 1). The patient did not present structural valvular abnormalities or endocarditis–related risk factors. CT pulmonary angiography detected a “saddle” PE and extensive thrombosis in inferior vena cava. After unfractioned heparin (UFH) bolus, the patient was transferred to our intensive care unit and balancing risks and benefits the chosen treatment option consisted in half dose systemic thrombolysis (50 mg rtPA in 60’’; 10 mg as loading dose in 2’). Because of potential harmful effects in thrombocytopenia and recent head trauma, UFH was temporarily withheld and started again after 1 hour. The day after, a beside TTE showed complete tricuspid thrombus resolution, a significant decrease in RV/LV ratio and systolic pulmonary artery pressure (fig. 2). Apart from small hematomas, no bleedings were reported. The patient was shifted to the ward on the next day and following course was uneventful. On day 4 a direct oral anticoagulant (Dabigatran 110 mg) was started. Abdominal and inferior limb–deep–venous ultrasound were both negative for pathological reports. A total body CT was scheduled to exclude silent malignancies at discharge. Take home message: in intermediate–high risk PE, half dose systemic fibrinolysis strategy is controversial since its potential benefit is counterbalanced by concerns about major bleedings, but increasing data suggest its efficacy with low risk profile.
FEAR OF SUDDEN DEATH ‘INTERRUPTED‘ BY WEARABLE DEFIBRILLATOR: CASE REPORT
Authors: Valecce R , Carretta D , D‘Agostino C , Bonfantino M 
Published: May 2024

Abstract
A male patient (pt) 49y,grade II familiarity for sudden cardiac death (SCD).He arrived in the emergency department for worsening dyspnea and he underwent ECG:sinus rhythm and left bundle branch block(QRS duration 170 msec);color Doppler echocardiogram (CDE)showing:Dilated left ventricle, severely and globally hypocontractile(EF30%).Negative cardiac enzymes on laboratory tests.The coronary arteries were normal at angiographic control.The pt did not undergo to cardiac MRI because of claustrophobia.Blood samples were sent for genetic screening for cardiomyopathy.The pt started drug therapy following ESC guidelines.He was transferred to cardiopulmonary rehabilitation department.At the end of the 2 weeks of the rehabilitation cycle, left ventricular systolic dysfunction persisted.The pt was discharged with a wearable defibrillator (WCD) to continue therapy optimization following ESC heart failure guidelines.At the end of 3 months,the pt underwent an echocardiographic recheck which confirmed dilated,severely,and globally hypocontractile left ventricle(EF35%).ICD implantation was indicated, but the pt refused it.Therefore,was prescribed the continuation of SCD protection by WCD.The pt underwent monthly followups,for the next 6 months,reporting no improvement in left ventricular contractile capacity measured by CDE and continuing to refuse biventricular ICD implantation.We noted a correct and consistent wearing of the WCD.After 177 days of wearing,the pt had an episode of loss of consciousness and WCD shock.A remote device check documented ventricular fibrillation at 200 bpm,which was correctly recognized and discontinued.The pt accepted biventricular ICD implantation.At follow–up at 1 month CDE documented improvement in left ventricular contractile capacity(EF 40%),and no arrhythmic events in the absence of symptoms.WCDs have been used extensively in Italy since 2015.This technology provides temporary protection from SCD for pts with an evolving risk profile who are not yet eligible for an ICD.WCD is an effective therapy for the treatment of SCD with a very low complication rate and high pt compliance over time.Our case demonstrates how WCD enabled optimal titration of drug therapy following guidelines and prevented sudden death in young high–risk pts.In addition, our case shows that reduced compliance for definitive ICD implantation due to non–acceptance of the disease,fear of surgery,or subsequent lifestyle changes are not barriers to saving patients from SCD.
PERCUTANEOUS CORRECTION OF "BADLY ACCOMPANIED" MASSIVE TRICUSPID REGURGITATION
Authors: Mancini G , Lucarelli K , Bellomo V , Troisi F , Casamassima V , Lombardi F , Inchingolo R , Grimaldi M 
Published: May 2024

Abstract

Background
Tricuspid regurgitation (TR) is a common valvular pathology that impairs quality of life and survival. Tricuspid transcatheter edge–to–edge repair (t–TEER) has shown promising potential in the TR treatment. There remains several uncertainties such as timing of intervention in relation to clinical status, RV function, pulmonary artery pressure and many comorbidities that are often overlooked.

Case Summary
A 80–year–old man with dilated cardiomyopathy, persistent atrial fibrillation and biventricular defibrillator (ICD), was admitted to our centre for congestive heart failure (CHF). Echocardiography showed massive TR, severe right heart dilatation, increased systolic pulmonary artery pressure, severely dilated inferior vena cava (IVC) and hepatic veins with systolic flow reversal. We also found an uncommonly high blood flow coming to the right atrium from the IVC, due to an anomalous vascular communication between an intrahepatic portal branch and a hepatic vein, a so–called intrahepatic portosystemic venous shunt (IPSVS). Although these relatively rare shunts are usually not treated unless symptomatic, after a collective careful evaluation, we decided to proceed to the embolization of the IPSVS with a vascular plug, to make the t–TEER less challenging, safer and more durable. In better general conditions, after 2 months, the patient underwent the transcatheter tricuspid procedure with a single XTW TriClip™ (Abbott). At 3 months follow–up, the patient showed noticeable improvements in QoL, CHF signs and symptoms, echocardiographic parameters and exercise capacity.

Discussion
Appropriate timing of t–TEER is crucial not only to avoid irreversible RV damage and organ failure, but also to increase the success rate of the procedures. In this case we observed that the IPSVS treatment contributed significatively to improve the patient’s clinical status and to reduce the right heart volume overload before attempting the t–TEER. This combined approach facilitated the technical success of tricuspid procedure, that was itself challenging due to the ICD leads in the right chambers.

Conclusion
Patients’ selection and timing of percutaneous TR treatment are not clearly defined. This case shows that a winning strategy to approach challenging TR is to solve, before the tricuspid treatment, all the potentially treatable comorbidities that could negatively interfere with the technical success of the procedure itself and with the durability of TR reduction.
DESMOPLAKIN ASSOCIATED NON DILATED LEFT VENTRICULAR CARDIOMYOPATHY TREATED WITH ANAKINRA
Authors: Pezzullo E , Caputo A , Catapano D , Alfieri R , Covino S , D‘Elia S , Golino P , Loffredo F 
Published: May 2024

Abstract
A 24 year old female patient experienced,from 2010 to November 2023,12 myocarditis–like episodes, often related to emotional stress, characterized by chest pain,increased markers of myocardial necrosis, normal C–reactive Protein values,normal left ventricular systolic function on echocardiogram.She was admitted to our division for the first time in July 2019 because of recurrent acute myocarditis, extensive non–ischemic fibrosis and myocardial edema on magnetic resonance imaging (MRI) and interstitial edema with monocytic lymphocytic infiltrates (CD3 +&gt;7/mm2) and cardiomyocyte necrosis at endomyocardial biopsy.A genetic test was performed leading to the identification of a novel frameshift mutation in the tail C domain of desmoplakin (DSP) producing a truncated protein.Destabilization of desmosome can be associated,to massive troponin release,innate immune activation resulting in autoantibody formation to intracellular cardiomyocyte protein.Mutations in DSP can link to arrhythmogenic cardiomyopathy,characterized by intermittent myocardial inflammatory episodes that appear clinically similar to myocarditis,leading to left ventricular fibrosis and to high incidence of ventricular arrhythmias.Our patient was initially treated with intravenous high dose corticosteroid therapy with oral tapering at discharge but two relapses(December 2020 and April 2021)occurred at corticosteroid withdrawal.In April 2021 immunosuppressive therapy with azathioprine was initiated in association with colchicine and oral corticosteroids,however after three months a new recurrence occurred.In July 2021 off–label Anakinra 100 mg subcutaneously daily was started.Anakinra is an interleukin (IL)–1 beta recombinant receptor antagonist used to treat idiopathic recurrent pericarditis.A number of studies have stressed the possible role IL–1 in the pathogenesis of myocardial inflammation involving inflammasome activation,cardiomyocytes loss ultimately leading to heart failure and arrhythmias.Anakinra showed a good response and tolerability and allowed corticosteroid withdrawal.The patient did not experience any further recurrence until November 2023.This episode was however characterized by milder and shorter chest pain,lower release of troponin in absence of edema on magnetic resonance imaging and inflammation on cardiac (18)F–fluoro–2–deoxyglucose positron emission tomography suggesting that modulation of cardiac inflammation may be crucial also when associated with pathogenic mutations.
PREGNANCY IN DANON’S DISEASE: WHAT WE KNOW ABOUT THERAPY IN THIS PARTICULAR SUBGROUP
Authors: Lampus M , Sanna S 
Published: May 2024

Abstract
A twenty–seven year old pregnant woman in her twenty–eighth week, suffering from Danon‘s disease, goes to the emergency room due to inappropriate shock of the ICD on high frequency atrial tachyarrhythmia. Three days before admission, Nadolol was replaced with Metoprolol due to difficulties related to obtaining the drug; since then the patient develops episodes of palpitations and chest pain.The medical history includes Hashimoto‘s thyroiditis, obesity and a family history of hypertrophic cardiomyopathy and CAD. The 12–lead ECG shows arrhythmia due to atrial fibrillation at a rate of 100 bpm, LBBB with secondary repolarization changes. Device interrogation revealed episodes of high frequency atrial fibrillation and atrial flutter. The ECOCG shows a non–dilated left ventricle with slightly increased thicknesses at the level of the interventricular septum and apex, with slight apical hypokinesia and preserved systolic function indices. During hospitalization, the patient‘s condition is stabilized and she always presents with good hemodynamic compensation. Nadolol is reintroduced together with anti–coagulant therapy with low molecular weight heparins. Danon‘s disease represents a rare phenocopy of hypertrophic cardiomyopathy and atrial fibrillation is of particular interest in this population subgroup. Pregnancy is a hypercoagulable state and AF is an arrhythmia associated with significant thromboembolic complications. Patients with hypertrophic cardiomyopathy that develop atrial fibrillation are especially at a higher risk of thrombosis. Furthermore, exposure to beta–blockers during pregnancy has been associated with intrauterine fetal growth restriction, preterm birth and perinatal mortality. There is a non–negligible risk connected to poor fetal growth, but the risk/benefit ratio leans in favor of maternal electrical stability, which, if lacking, would still cause damage to the fetus due to hypoperfusion phenomena.