bluuebunny/crossref_metadata_2025_split huggingface dataset:
C32 CONSTRICTIVE PERICARDITIS WITH PLATYPNEA ORTHODEOXIA
Authors: Gaiero L , Capriolo M , Gallo C , Annone U , Biasco L , Senatore G 
Published: May 2023

Abstract
A 56–year–old woman is admitted to our centre for biventricular heart failure. In anamnesis she reports a submandibular phlegmon complicated by pleuropericarditis at the age of 13 years requiring repeated pericardiocentesis. In the last three years she has been hospitalised twice for ascites and discharged with a diagnosis of idiopathic liver cirrhosis. On admission she presented with peripheral oedema, ascites and hippocratic fingers; oxygen saturation is 80% in room air. On blood tests creatinine, transaminases, albumin, coagulation and NT–proBNP are normal, bilirubin is elevated. An echocardiography is performed showing a calcific spicola impinging the free wall of the right ventricle producing endocavitary obstruction; diffusely thickened pericardium, septal bounce, annulus reversus and expiratory reflux in the suprahepatic veins. Biventricular contractile function is normal and no significant valvulopathy is present. CT scan confirms the presence of diffuse pericardial calcifications, small liver and irregular profile with large ascitic effusion. Decongestive therapy is started with weight loss of 22 kg and regression of oedema but severe hypoxia persists with evidence of platypnea–orthodeoxia syndrome. An echocardiography is repeated showing passage of microbubbles in the left atrium from the sixth beat onwards, compatible with intrapulmonary shunt. A large arteriovenous malformation (AVM) of the right lower pulmonary branch is found at CT angiography, which is also revealed by targeted invasive angiography. Cardiac catheterisation and cardiac MRI confirmed the diagnosis of constrictive pericarditis. Percutaneous closure of the AVM with complete resolution of the hypoxia and subsequent pericardiectomy is performed. The patient is discharged on day XXVII with normal saturation on room air, in good haemodynamic compensation and with normal liver stasis parameters. In conclusion, constrictive pericarditis is a rare cause of heart failure and to our knowledge this is the second case described reporting a pulmonary AVM secondary to constrictive pericarditis.
C33 AN UNEXPECTED CARDIOGENIC SHOCK
Authors: Leiballi E , Lestuzzi C , Viel E , Riccio A , Valente M , Turazza F , Pecoraro R , Pavan D 
Published: May 2023

Abstract

Background
Chemotherapy regime based on 5–fluorouracil (5–FU) are frequently administered in the treatment of gastro–intestinal malignancies, especially colorectal carcinomas. A potential severe side effect of 5–FU is cardiotoxicity with often presents with chest pain related to coronary vasospasm. More serious cardiotoxicity, including dilated cardiomyopathy, ventricular arrhythmia and sudden cardiac death has also reported in the literature. Case report. We present a 40–years–old man with stage IV colon adenocarcinoma with metastases. The patient had no history, or risk factors, of cardiac disease. The patient was started on palliative chemotherapy with FOLFOXIRI (Leucovorin/Irinotecan/Fluorouracil). Approximately 24 h into receiving the first infusional dose of 5–FU the patient developed progressive chest pain. Electrocardiography (ECG) revealed sinus tachycardia without repolarization changes. Cardiac biomarkers indicated a moderate elevated troponin (430–580 ng/L). Echocardiogram revealed severely reduced left ventricular function with an ejection fraction (EF) of 23%. Subsequently, the patient developed a hemodynamic instability treated with noradrenaline and dobutamine, but the patient remained in cardiogenic shock. The patient was transferred to cardiac surgery for ECMO placement. Despite ECMO placement the patient was hemodinamically unstable, it was necessary to position also IMPELLA and IABP. The improvement was very slow and the ventricular function returned to normal after 5 days. A subsequent cardiac MRI showed delayed enhancement with diffuse subepicardial distribution (non–ischaemic pattern).

Conclusion
Our case emphasizes the importance of early recognition of this rare complication and prompt cessation of 5–FU, as cardiac dysfunction in this context is potentially reversible. The precise mechanism remains unclear, but several mechanisms have been proposed including coronary vasospasm, direct toxicity to the myocardium, endothelial dysfunction. It should be noted that these mechanisms were elucidated though animal modeling, case reports, and small clinical studies.
C34 AN UNUSUAL CASE OF CARDIAC ARREST DUE TO TRAUMA–INDUCED QT PROLONGATION
Authors: Astuti M , Somaschini A , Olivotti L , Cornara S , Ghione M , Buscemi M , Buscaglia E , Botta M , Cordone S , Pentimalli F , Bellone P 
Published: May 2023

Abstract

Background
QT prolongation is a well–known cause of sudden cardiac death due to polymorphic ventricular tachycardia triggered by early afterdepolarizations. Acquired long QT is a common finding in several clinical scenarios and invariably represents a high risk feature.

Case Report
An 84 years–old woman presented to our emergency department (ED) after experiencing a blunt chest trauma due to a car accident. She reported mild dyspnea and chest pain exacerbated by breathing and movement while denied palpitations and loss of consciousness. Baseline ECG showed atrial fibrillation with normal ventricular rate and a prolonged QT interval (QTc 640, Fig 1A) not present at previous ECGs. She did not assume any QT–prolonging drug and laboratory tests at admittance revealed troponin I values slightly elevated (29,6 ng/ml; n.v.: &lt;11,6 ng/l) and no other significant abnormalities. A CT scan demonstrated a displaced fracture of the sternal body. Due to the QT prolongation, the patient was admitted for ECG monitoring.. During the stay in our ED the patient suffered a cardiac arrest. A torsade des pointes triggered by a late–coupled premature ventricular complex (PVC) was documented (Fig 1B). The arrhythmia was interrupted with external DC shock and magnesium solfate infusion was started. The coronary angiography demonstrated normal coronary arteries. The patient underwent cardiac magnetic resonance (CMR) four days later which showed transmural necrosis of the middle third of the lateral wall of the left ventricle at the insertion of the anterolateral papillary muscle (Fig 2). No further ventricular arrhythmias were observed and the patient was discharged after QTc normalization. Cardiac arrhythmias are a rare complication of cardiac contusion ranging from isolated PVCs to atrial or ventricular fibrillation. However, the role of the ECG in this peculiar subset is unclear. The most common ECG abnormalities are non specific ST–T wave changes, whereas in our case the marked QTc prolongation resulted in a closer monitoring of the patient allowing a prompt recognition of cardiac arrest. On the other hand CMR proved to be an effective method to identify a cardiac injury showing the typical pattern of myocardial damage associated with cardiac contusion (stress–induced lesions at the insertions of papillary muscles due to sudden increase of left ventricular pressure) and, thus, could be adopted as a useful tool to identify patients at higher risk for arrhythmic complications.
C35 A RARE CASE OF NON–BACTERIAL THROMBOTIC ENDOCARDITIS IN YOUNG WOMAN AFFECTED BY ADVANCED UTERINE CANCER
Authors: Molisana M , Belleggia S , Gizzi G , Ianni U , D’Agostino S , Di Eusanio M , Dello Russo A , Parato V 
Published: May 2023

Abstract
A 37–year–old woman affected by uterine HPV–related adenocarcinoma complicated by paraneoplastic pulmonary thromboembolism, came to the emergency department for recurrent episodes of dysarthria and blurred vision with spontaneous resolution. Home medications: Enoxaparin 5000 UI b.i.d, levofloxacin 500 mg/die, deltacortene 25 mg/die and omeprazole 20 mg/die. Blood analysis: normocytic anemia (Hb 9 gr/dl),elevated D–dimer (22.5 mcg/ml) and elevated C–reactive protein (18.3 mg/dl). Procalcitonin and blood cultures resulted negative. Brain CT scan: ischemic brain lesions of embolic nature in the right precentral gyrus and in the left post central gyrus. EKG: sinus rhythm, T–wave inversion in inferior leads. In the suspect of cardioembolic stroke a transthoracic (TTE) and subsequent transesophageal (TOE) echocardiography was performed: TTE: isoechoic oval–shaped thickening of the aortic valve cusp tips, producing aortic regurgitation with double jet regurgitation. TOE: evidence of multiple isoechoic masses involving all the aortic cusps, without perivalvular aortic extension, producing significant aortic regurgitation due to coaptation– gap (Figure 1). The diagnosis of non–bacterial thrombotic endocarditis (NBTE) was made up. Although the standard medical therapy (anticoagulation and chemotherapy) was promptly started, it wasn’t effective with progression of the disease leading to surgical indications of valve intervention. The patient underwent aortic valve replacement with bioprosthesis avoiding mechanical prosthesis, despite the young age, in anticipation of major surgery for the uterine cancer. Surgical specimen turned negative for microorganism growth confirming the diagnosis of marantic endocarditis. No further episodes of ischemic stroke appeared after the replacement of the valve and the patient had no neurological sequelae. NBTE is a rare condition of non–infective endocarditis characterized by the formation of aggregates of platelets and fibrin that affects usually undamaged heart valves in the absence of bacteremia and mostly occurs in patients with a predisposing state like hypercoagulability. Surgical indications of valve intervention are not well encoded but in most cases they follow the raccomandations for infective endocarditis. Our experience highlights the need of a prompt intervention in order to reduce the damage of systemic embolization and preserve patient’s hemodynamics with a strict checking of disease progression.
C41 CAROTID OCCLUSION AT THE CAROTID SIPHON LEVEL IN A CHILD WITH PROGERIA
Authors: Greco A , Mastroianno S , Bevere E , Massaro R , Coli C , Potenza D , Salvatori M , Brunetti N , Di Stolfo G 
Published: May 2023

Abstract

Introduction
Hutchinson–Gilford Progeria syndrome (HGPS) is a rare disease affecting 1 in 4–8 million children, caused by a sporadic mutation of the LMNA gene and expression of a mutant product called progerin. Patients develop a progressive and severe premature aging disease characterized by failure to thrive, alopecia, bone and joint abnormalities, loss of subcutaneous fat, and severe atherosclerosis. Patients live an average of 14.6 years, and most die in their early teens from myocardial infarction or stroke due to rapidly progressive atherosclerosis.

Clinical case
The 8–year–old patient, affected by progeria, arrived for relapsing strokes. The clinical history began with a picture of severe acro–mandibular hypoplasia with obstruction of the upper airways, treated with early tracheostomy and subsequent percutaneous gastrostomy. Further progeroid phenotypic characters developed were: poor body growth, sui generis facies with prominent eyes and beaked nose, alopecia, micrognathia, discolored, thin, hypoelastic skin; poorly represented panniculus adiposus; scoliosis. Genetic tests have highlighted two pathogenic heterozygous mutations, of the LMNA gene (C.168 C&gt;A) probably deriving from the mother (mosaicism), and the other of the ZMPSTF24 gene, (c.846 C&gt;T) paternally inherited. The major complications are secondary to severe atherosclerosis of the cerebral district, resulting in repeated strokes. Brain MRA (Fig.1 and 2) documented the absence of signal at the level of the right carotid siphon and of the whole intracranial tract of the right carotid artery and poor signal in correspondence with the intracranial tract of the left carotid artery distal to the carotid siphon in a picture of diffuse atherosclerosis. The marked predisposition and the recurrence of ischemic episodes required the patient to have thrombophilic screening, positive for a heterozygous mutation for the prothrombin gene (G20210A), known for thrombophilic predisposition.

Conclusions
Progeria is a rare syndrome that requires an adequate multidisciplinary approach and clinical care management aimed at improving the quality of life of our patients.
C43 RELATIONSHIP BETWEEN ECHOCARDIOGRAPHIC DATA AND CARDIOPULMONARY EXERCISE TEST PARAMETERS IN ADULT CONGENITAL HEART DISEASE PATIENTS: A RETROSPECTIVE CLINICAL STUDY
Authors: Olimpieri A , Ferraro F , Gambardella R , Di Molfetta A , Grandinetti M , Lillo R , Graziani F , Delogu A , Bruno P , Massetti M 
Published: May 2023

Abstract

Introduction
The interpretation of cardiopulmonary exercise test (CPET) in adult congenital heart disease (ACHD) is challenging because of the high variability in cardiac lesions and because the poor exercise tolerance is often associated with abnormal heart function. The aim of this work is to study the correlation between echocardiographic evaluation and CPET parameters in ACHD population.

Methods
This is a retrospective clinical study enrolling all ACHD patients afferent to our institution from March 2016 to February 2020 collecting clinical, CPET and echocardiographic data. Population was divided into three groups and compared: patients with prevalent left heart lesions (L group), patients with prevalent right heart lesions (R group) and patients with single ventricle physiology (SV group). Finally, correlation analysis were performed to assess any relationship between echocardiographic and CPET data.

Results
60 patients were included in the study, 20 were male, median age was 36 [30–46.75] years old, 23 had a prevalent left side pathology, 31 a prevalent right side pathology and 6 had a single ventricle physiology. L group had a significantly lower predicted peak VO2 in comparison to SV group (p=0.03). Statistical analysis showed a correlation between the right ventricular function and the ventilatory reserve in the R group (R2 0.517). In SV group we found correlation between ventricular–arterial coupling and Cardiac mechanical efficiency with the ventilatory reserve (R2 0.6 and 0.65, respectively) and the oxygen pulse (R2 0.97 and 0.98, respectively), and a correlation between the ejection fraction and the Oxygen pulse (R2 0.52). Finally, a correlation between the TAPSE/PAPs index and the ventilation (R2=0.79), the ratio between the oxygen uptake and the workload (R2=0.61) and the oxygen consumption (R2=0.72) was found. Coclusion:In our preliminary experience, echocardiographic parameters of cardiocirculatory efficiency correlate with the oxygen consumption per heart beat evaluated at the CPET in single ventricle patients, and the breath rate is influenced not only by ventricular function but also by the coupling between the ventricle and the arterial system. In patients affected by right–side disease, the breath rate correlate with the right ventricular function. Finally, in the left heart lesion population, a correlation between the TAPSE/PAPs index with the ventilation, the oxygen consumption per beat and the oxygen consumption was found.
C46 ELECTROPHYSIOLOGICAL ASSESSMENT OF ATRIAL TACHYCARDIA SUBSTRATES IN PATIENTS WITH REPAIRED TETRALOGY OF FALLOT
Authors: Mirizzi G , Garibaldi S , Startari U , Rossi A , Panchetti L , Festa P , Ait–ali L , Santoro G , Assanta N , Piacenti M 
Published: May 2023

Abstract

Introduction
Adults with repaired tetralogy of Fallot (rToF) experience episodes of atrial tachycardia (AT) and radiofrequency catheter ablation (RFCA) is often required but systematic evaluation of the mechanisms and recurrences is lacking.

Methods
Between January 2013 and October 2021, 20 rToF patients with AT referred for catheter ablation were enrolled. Electrophysiologic study with 3D electroanatomic mapping with multi–electrode mapping catheters was done, with right atrial bipolar voltage and activation mapping of the AT. Three mechanisms were searched: intra–atrial re–entrant tachycardias (IART), focal (FAT), other. Critical isthmus (CI) for IART was identified with activation mapping. FATs were localized according to the earliest uni/bipolar signal. All induced AT were treated. RFCA was aimed at the earliest activation point for FAT and at the critical isthmus for IART, anchoring lesion to fixed obstacles (valve annuli or scar). Written informed consent was provided.

Results
Among 20 adult (age 46±14 years) and mainly females (n=11, 55%) enrolled pts, 36 AT were documented: 25 (70%) IART, 10 (27%) FAT and only 1 (3%) had a typical atrioventricular nodal reentrant tachycardia. Mean tachycardia cycle length was 307±95 ms. Two ATs were induced in 11 pts, 3 in 4 pts and 4 in 2 during index EPS. Among IART, cavo–tricuspid isthmus (CTI) was the prevalent CI (n=14, 60%), while incisional IART (IARTinc, atriotomy and superior and inferior vena cava orifices, SVC and IVC, respectively) was the second mechanism (n=9, 39%). In two pts, due to non–inducibility, a pre–emptive lesion set comprising in one case CTI and in the second case CTI+SVC–atriotomy–IVC line was performed. Among FAT, in 3 cases the AT was mapped in the coronary sinus, in two at the tricuspid annulus, other at the crista terminalis, right appendage base, posterolateral scar or between atriotomy and SVC. During a median follow–up of 23 months (interquartile range, 6–37) recurrence occurred in 8 pts (25% of pts, 22% of tachycardias). Pts with recurrences were younger (43±7 vs. 48±17 y, p=0.04); no differences were found according to critical isthmus location (4 CTI and 4 IARTinc, 50%, p=ns).

Conclusions
Among rToF pts with AT, IART is the prevalent mechanism and CTI is the prevalent CI; atriotomy scar, however, is involved in a substantial portion of the critical isthmuses of the IART; FAT location is much more variable. Long–term freedom from AT in this clinical setting is encouraging.
C51 INTRAVASCULAR LITHOTRIPSY FOR THE TREATMENT OF SEVERELY CALCIFIED CORONARY STENOSIS IN ACUTE MYOCARDIAL INFARCTION
Authors: Pavani M , Franzè A , Cinconze S , Barbero U , Meliga E , Rognoni A , Nicolino A , Biasco L , Conrotto F , Ugo F , Scacciatella P , Tomassini F , Leoncini M , Menozzi A , Patti G , Rolandi A , Porto I , Musumeci G , Cerrato E , Varbella F 
Published: May 2023

Abstract

Background
Little is known about intraprocedural and mid–term results of intravascular lithotripsy (IVL) use in patients with acute MI.

Methods
In Our retrospective analysis, data from 21 Centers in Italy and Spain between January 2020 and November 2021 were collected. Patients were allocated to the AMI–group when IVL was performed during an acute MI. Primary safety endpoint was defined as freedom from major adverse cardiac events (MACE: cardiac death, myocardial infarction (MI), target vessel revascularization (TLR) or stent thrombosis (ST)) within 30 days of the index procedure, and primary effectiveness endpoint was procedural success (defined as stent delivery with a residual stenosis &lt;20% and without intraprocedural MACE). The mid–term endpoint was 6–months MACE.

Results
A total of 468 patients were retrospectively enrolled. The primary safety endpoint was achieved in 98.8% of patients in the AMI–group and in 98.5% in the non–AMI group (p=NS). We showed a lower incidence of the primary effectiveness endpoint in the AMI–group (89.8%) vs Non AMI–group (94.8%) (p=0.048). The secondary endpoint of 6–months MACE was similar between groups (6.7% vs 4.9% respectively for AMI group and Non AMI–group), but death for cardiac causes occurred more frequently in the AMI–group (4.3% vs 0%, p=0.01).

Conclusions
Intravascular lithotripsy demonstrated to be a safe tool for the treatment of calcified coronary lesions during Acute Myocardial infarction, even if we showed some concern for lower procedural success.
C62 CHALLENGING DIAGNOSIS OF A TRIPLE CARDIAC PATHOLOGY: TRANSTHYRETIN AMYLOIDOSIS WITH ASPECTS OF HYPERTROPHIC OBSTRUCTIVE CARDIOMYOPATHY AND ANOMALOUS CORONARY ARTERY
Authors: Patani F , Tofoni P , Lofiego C , Vagnarelli F , Capodaglio I , Lombardi G , Pietrucci F , Schicchi N , Esposto Pirani P , Perna G 
Published: May 2023

Abstract
A 70 years–old man with a history of systemic hypertension and surgically treated bilateral carpal tunnel was referred for dyspnea on exertion for few months. At physical examination a systolic heart murmur exacerbated by Valsalva maneuver was appreciated; no signs of heart failure. EKG with low–voltage QRS is shown in Fig.1A. Blood tests showed elevated NT–proBNP (1434 pg/ml). Transthoracic echo (ETT) revealed hyperdynamic left ventricle (LV) with severe asymmetric hypertrophy (interventricular septum 23 mm, posterior wall 17 mm), ‘granular sparkling’ appearance of myocardium, grade 2 diastolic dysfunction and ‘apical sparing’ pattern at global longitudinal strain (GLS, Fig.1B). Moreover, elongated mitral leaflets, anomalies of mitral sub–valvular apparatus with apically displaced papillary muscles and systolic anterior motion of the mitral valve (SAM) with dynamic outflow tract obstruction (20 mmHg at rest and 90–100 mmHg with Valsalva maneuver) were seen (Fig. 2A–B–C). For ETT suspicion of a coronary artery anomaly (Fig.3A), the patient underwent coronary CT angiography that confirmed a separate origin of left anterior descending artery (LAD) from right coronary sinus with a long mid–proximal intramyocardial bridge (Fig.3B–C). As a part of evaluation of the hypertrophic phenotype, a 99mTC–HDP bone scintigraphy was performed with high–grade of cardiac uptake (Perugini Score 2, Fig.1C). Screening for monoclonal gammopathy and genetic testing for hereditary amyloidosis resulted negative. Cardiac MRI revealed late gadolinium enhancement (LGE) in midwall septum, diffuse endocardial LGE in the LV basal inferior and posterolateral walls (Fig.1D), elevated T1 mapping and ECV (40–42%) and confirmed the asymmetric hypertrophy with apically displaced papillary muscles (Fig.2D) and SAM. These clinical, EKG and imaging features led to a non–invasive diagnosis of wild–type transthyretin cardiac amyloidosis (ATTRwt) with aspects of hypertrophic obstructive cardiomyopathy (HOCM). The patient started a disease–modifying therapy with Tafamidis and, cautiously, Metoprolol tartrate, with good tolerance and relevant reduction of LVOT obstruction at follow–up. Genetic testing for HCM was also performed. In conclusion, in rare cases hypertrophic phenotype may be challenging, showing features overlap between ATTR and HOCM. Multimodality evaluation is crucial for a correct diagnosis, thus identifying the most effective target–therapy for the underlying hypertrophic phenotype.