bluuebunny/crossref_metadata_2025_split huggingface dataset:
38 PERIPHERAL ENDOTHELIAL FUNCTION AND CARDIOPULMONARY FUNCTION IN PATIENTS AFFECTED BY PULMONRY HYPERTENSION
Authors: Tricarico Lucia , Casiere Vivian , Alfieri Simona , Croella Francesca , Nunno Nicola Di , Amatruda Marco , D´alessandro Damiano , Granatiero Michele , Correale Michele , Iacoviello Massimo , Di Biase Matteo , Brunetti Natale D 
Published: December 2022

Abstract

Background
Pulmonary arterial hypertension (PAH) is a progressive and severe rare disease. It is a pulmonary vasculature obstructive disease that leads to right heart failure and death. Pulmonary circulation dysfunction endotelial is characterized by vasoconstriction and proliferation.

Aim of study
to search possible correlations between peripheral endothelian function and cardiopulmonary function in patients with suspect PAH.

Materials
Between February 2021 and May 2022, consecutive patients with suspect PAH underwent to assessment of peripheral endotelial function by FMD before of right heart catheterization and cardiopulmonary exercise testing.

Results
46 patients (22 male, 67,9 ± 10,4 years old) with pulmonary hypertension were enrolled in the study. The following correlations were found between stress-resting absolute difference and % difference radial diameter: ramp (r: 0,69; p: 0,003 and r: 0,54; p: 0,028, respectively), Watt_AT (r: 0,63; p: 0,01 and r: 0,54; p: 0,03, respectively) and WATT_peak (r: 0,64, p: 0,009).

Conclusions
peripheral endothelial function may be related to funtional capacity in patients affected by pulmonary hypertension.
117 PROGNOSTIC VALUE OF ELECTROCARDIOGRAPHIC PARAMETERS IN PATIENTS WITH IDIOPATHIC/HERITABLE/DRUG-INDUCED PULMONARY ARTERIAL HYPERTENSION
Authors: Dardi Fabio , Bertozzi Riccardo , Guarino Daniele , Magnani Ilenia , Rotunno Mariangela , Ballerini Alberto , Donato Federico , Martini Giulia , Manes Alessandra , Palazzini Massimiliano , Galiè Nazzareno 
Published: December 2022

Abstract

Background
despite therapeutic improvement, pulmonary arterial hypertension (PAH) is still a chronic, incurable, high mortality disease. Several clinical, laboratory, hemodynamic and instrumental prognostic factors have been already established in PAH patients. However, no electrocardiographic prognostic factors have been identified so far in a systematic multivariate analysis together with clinical, exercise and haemodynamic parameters.

Methods
patients with idiopathic/heritable/drug-induced PAH (I/H/D-PAH) referred to a single centre were included from 1993 to 2021. All patients underwent baseline clinical, 12-lead ECG, hemodynamic with vasoreactivity test, 6 minutes walking distance (6MWD), brain natriuretic peptide (BNP) or N-terminal pro-BNP (NT-proBNP), WHO functional class evaluation and risk stratification (according to a recently validated simplified version of 2015 ESC/ERS guidelines’ risk table). All cause mortality has been considered to identify the predictors of mortality using univariate and multivariate Cox regression analysis.

Results
570 PAH patients were included: 448 idiopathic (79%), 105 heritable (18%) and 17 drug-induced (3.0%). 20 patients were on atrial flutter/fibrillation at baseline. Considering only electrocardiographic parameters the variables independently associated with prognosis are shown in the table below:
Taking into account also the risk stratification the independent prognostic variables are:
Considering also clinical, exercise and haemodynamic variables the independent prognostic variables are:

Conclusions
in patients with I/H/D-PAH heart rate is a prognostic factor independently from other electrocardiographic, clinical, exercise and haemodynamic parameters as reflects the attempt to preserve the cardiac output in patients with an increased right ventricular afterload causing a reduced stroke volume; both PR interval prolongation, marker of right atrial overload, and QTc prolongation, marker of right ventricle's electrical remodeling, are prognostic variables independently from baseline patient's risk level; right bundle branch block, expression of right ventricular electrical remodeling, has proved to be an independent predictor of mortality merely if we consider only the electrocardiographic parameters.
498 PERSISTENT FIFTH AORTIC ARCH IN A PATIENT WITH CHARGE SYNDROME: A CASE REPORT AND SYSTEMATIC REVIEW OF LITERATURE
Authors: Ferraioli Mario , Nocilla Alessandra , Vincenza Polito Maria , Malinconico Marisa , Coppola Guido , Ferrara Francesco , Ciccarelli Michele , Vecchione Carmine , Galasso Gennaro 
Published: December 2022

Abstract

Background
CHARGE syndrome (CS) is a rare genetic disease characterized by a constellation of clinical findings including Coloboma, Heart defects, choanal Atresia, Retardation of growth and/or development, Genitourinary malformation and Ear abnormalities. We present a case of persistent fifth aortic arch (PFAA), an extremely rare congenital anomaly of aortic arch (AA), in a child with genetically confirmed CS and perform a systematic review of published studied, in an effort to examinate the distribution of congenital heart diseases (CHDs) and their impact in CHARGE patients.

Case presentations and results
A 12 years-old child was referred to our echocardiography laboratory for atypical chest pain. He had bilateral ocular coloboma, left hypoacusis, scoliosis, mild motor impairments, nocturnal enuresis, micropenis and facial dysmorphisms. Molecular diagnostic testing identified a de novo mutation (variant c.5290_5300+10del) in the CHD7 gene and CS was diagnosed. Echocardiography showed a single posterolateral papillary muscle and a cleft of anterior mitral leaflet. Interestingly, AA had a double-lumen appearance without Doppler signs of coarctation. A PFFA was hypothesized and then confirmed at angioCT. A systematic review of the literature was performed according to PRISMA guidelines. All published articles reporting the association CS and CHDs were chosen. A total of 975 records were identified. After the screening of title and abstract, assessed for eligibility were 219 papers. Finally, inclusion and full-text analysis was made in 60 studies, of which 37 case reports and 23 case series. We found that ventricular septal defects emerged as the most prevalent heart defect (32%), followed by atrial septal defects reported in 23% of cases. Complex CHDs were also described. Interestingly, AA abnormalities were reported in a high percentage (27%) of patients, right AA in 20% in association or not with aberrant subclavian artery and vascular ring, interrupted AA in 5% of cases, as well as aortic coarctation in 10%. Of note, almost half of the cases (49%) required cardiac surgery, mostly performed within 1 year from birth and, although outcome was available in a minority of patients, the death was reported in almost 30%.

Conclusions
Our case is the first that reports PFFA in CS and may be mistaken for AA dissection. In comparison with other syndromic diseases, a high prevalence of AA abnormalities was found in patients with mutations in the CHD7 gene.
740 TREATMENT STRATEGY IN CASE OF REPAIRED TRUNCUS ARTERIOSUS AND AORTIC COARCTATION
Authors: Leonardi Denis , Iseppi Manuela , Ciuffreda Matteo , Cristofaletti Alessandra , Prioli Maria Antonia , Rossetti Lucia , Sandrini Camilla , Luciano Ribichini Flavio 
Published: December 2022

Abstract

Background
Common Truncus arteriosus (TA) is a rare congenital, cyanotic heart disease with a single vessel coming out from both ventricles and related low interventricular defect, right-left shunt, low pulmonary flow and right ventricle (RV) hypertension. TA is associated with aortic coarctation (CoA) in 10-20% of cases.

Case
A 15-year-old male affected by type 2 TA and CoA undergoing first post-natal cardiac surgery with pulmonary valved conduit (PVC) implantation and CoA repair, subsequently multiple percutaneous (PC) interventions due to right pulmonary artery (RPA) stenosis and re-CoA.
He was referred to our Centre for dyspnoea on mild efforts. Echocardiography revealed RV hypertension (75% of systemic pressure) and mild disfunction. Angio-CT showed RPA re-stenosis due to peeling of previous stents, a degenerative PVC and mild residual CoA.

Results
Cardiac catheterization confirmed imaging findings, revealing the following systolic/diastolic blood pressures [mmHg]: 100/15 in RV, 33/10 in left pulmonary artery (LPA), 13/7 in RPA, 132/65 in ascending and 125/60 in descending aorta. First, extensive balloon-interrogation of stented RPA and RV outflow tract (RVOT) with semi-compliant balloon was performed followed by PC intra-stenting transluminal angioplasty of RPA with modified undersized non-compliant balloon. As RPA was very closed to CoA with risk of aortic rupture, and mostly the main pulmonary OT accounted for increased pressure gradient, RPA enlargement with re-stenting was avoided. Then, a long semi-opened-cell stent was arranged on the prosthetic RVOT and Melody valve was implanted as Valve-in-Valve (ViV), both with balloon post-dilatation. Dilated LPA with only moderate focal stenosis and mild residual CoA were excluded from intervention. Post-interventional right heart pressures were reduced: 45/4 in RVOT and 42/13 mmHg in the main PA. At 1 month dyspnoea disappeared with good effort tolerance, echocardiographic ViV mean gradient was 12 mmHg with improved RV systolic function and pressure (50% of systemic).

Conclusions
Repaired complex TA of type described may develop multiple re-stenosis of RPA. RPA optimization was not the goal of the procedure because at high risk of vessels’ injury due to the very closed anatomy with otherwise prosthetic CoA. RV pressures and dysfunction could depend on degenerative obstructive PVC. Thus, interventional approach mostly focused on the main PA aimed at working PVC could be the best effective treatment.
1033 A STRANGE CASE OF PULMONARY EMBOLISM IN A YOUNG PATIENT WITH CARDIAC RESYNCHRONIZATION THERAPY
Authors: Restelli Davide , Poleggi Cristina , Carlo Alessandro Di , Pelaggi Giuseppe , Di Bella Gianluca , Micari Antonio , Bonanno Marco , Trifirò Maria Paola , Paleologo Giuseppe , Carerj Scipione 
Published: December 2022

Abstract

Introduction
Clinically relevant pulmonary embolism (PE) related to pacemaker leads is reported in up to 3.5% of cases of implantations. We reported a particular case of acute massive PE in a patient bearer of a Cardiac Resynchronization Therapy-Defibrillator (CRT-D).

Case presentation
A 49-year-old man presented to our ER reporting dyspnea and localized pain to the left hemithorax. Past medical history included HFrEF due to idiopathic dilated cardiomyopathy complicated by apical thrombosis, CRT-D, Charcot-Marie-Tooth syndrome, dyslipidemia, and a hepatic nodule undergoing diagnosis. Recently, he reported COVID-19 and successive pleuropneumonia requiring hospitalization. At home, he was taking Acenocoumarin, Sacubitril/Valsartan 97/103mg bid, Carvedilol 6.25mg bid, Digoxin 0.0625mg, and Canrenon 50mg with questionable adherence. On physical examination, he was pyretic, mildly hypotensive and hypoxemic. An ECG showed atrio-guided biventricular stimulation at 110 bpm. A transthoracic echocardiogram (TTE) revealed biventricular disfunction and dilation, mitral and tricuspid regurgitation, PAPs 60 mmHg, and a thrombotic formation in the left ventricle apex. Blood tests found elevated D-Dimer and infection markers, INR 1.1. A CT angiography confirmed a bilateral acute massive PE associated with lung consolidations and left pleural effusion. We excluded deep vein thrombosis in the lower limbs. Patient was admitted to CCU and treated with intravenous heparin, antibiotics, and support therapy. After one week, we performed a transesophageal echocardiogram that confirmed the previous TTE except for a minor PAPs and unknown thrombotic formations on the atrial side of the tricuspid valve, adhering to CRT-D leads, pedunculated, highly mobile, the largest with an area of 1.9cm2. Cardiac surgery wasn't indicated; we excluded thrombophilia and thoracic cancer. Patient is still hospitalized, asymptomatic and with a precarious cardiovascular status.

Discussion
The incidence of asymptomatic clot adhering to cardiac device's leads found by echocardiography is 1.4%, and thromboembolic complications are even rarer, especially after years from the implant. In our patient, the right atrium thrombosis is probably the cause of PE, and possible precipitating factors were pneumonia or unknown hepatic neoplasm. In the diagnostic workup, TTE probably wasn't sensitive enough to detect those thrombi.

Conclusions
We presented a particular case of PE in a relatively young patient affected with HFrEF. Even if thrombosis related to pacemaker leads is rare, it should be considered as a possible cause of PE in a patient bearer of a cardiac stimulating device.
723 PREDICTORS OF PERCUTANEOUS CORONARY INTERVENTION DERIVED FROM CORONARY CT ANGIOGRAPHY IN PATIENTS WITH CHRONIC CORONARY SYNDROME
Authors: Belmonte Marta , Paolisso Pasquale , Bertolone Dario Tino , Gallinoro Emanuele , Caglioni Serena , Leone Attilio , Colle Cristina De , Bermpeis Konstantinos , Storozhenko Tatyana , Sonck Jeroen , Wyffels Eric , Penicka Martin , Collet Carlos , De Bruyne Bernard , Barbato Emanuele , Andreini Daniele 
Published: December 2022

Abstract

Aims
To assess whether quantitative and qualitative plaque features derived from coronary CT angiography (CCTA) could predict subsequent coronary revascularization by percutaneous coronary intervention (PCI) in patients with suspected chronic coronary syndrome (CCS).

Methods
From April 2019 to October 2020, all consecutive patients with suspected CCS undergoing CCTA and computation of Fractional Flow Reserve derived from CCTA (FFRCT), with consequent referral to coronary angiography and invasive physiological assessment, were included. The FFRCT 3D-model was used to calculate i) FFRCT value, in distal coronary segments where invasive FFR was measured, ii) trans-lesion gradient, defined as the difference in FFRCT units across the lesion derived from CTA, iii) vessel gradient, i.e., the difference in FFRCT units along the vessel. Quantitative and qualitative plaque analysis at CCTA was performed by experienced operators as recommended by current guidelines.

Results
Overall, 108 patients (143 vessels) were included. The mean age was 65±8.6 years, and 11% were diabetics. At baseline, 87 (80%) patients had chest pain. Left ventricular ejection fraction was 59±4.6%. Sixty (55.5%) patients underwent PCI, 8 (7.5%) were treated with coronary artery bypass graft (CABG), and 40 (37%) were referred to medical therapy (MT). At CCTA, the lesions undergoing PCI were significantly longer (38.85±17mm, p&lt;0.001), with more severe diameter stenosis (45.43±16.69, p=0.002), lower minimal lumen area (MLA) (2.30± 1.33mm, p=0.013), higher plaque volume (292.31±158.32mm3, p&lt;0.001), greater fibrous-fatty and necrotic core volume (18.87±20.7 and 14.78±27.42, p&lt;0.001), greater maximum calcium arc in the lesion (108.63±65.9, p&lt;0.001) and lower calcium arc the MLA (88.5±10 p=0.037) than the others referred to CABG/MT. In the PCI group, FFRCT was significantly lower (0.69± 0.11, p&lt;0.001), while trans-lesion and vessel gradients were higher (0.09±0.04 and 0.27±0.10 respectively, p&lt;0.001) than the other groups. On multivariate regression model, corrected for all confounding factors, longer lesions (OR 1.11, CI 1,01-1,21, p=0.022), higher maximum calcium arc (OR 1.02, CI 1.01-1.04, p= 0.007) and lower calcium arc at the MLA (OR 0.99, CI 0.98-0.99, p=0.031) were shown to be independent predictors of referral for PCI.

Conclusion
Lesion length and calcium arc derived from CCTA predict subsequent revascularization by PCI in patients suspected of CAD.
459 PREOPERATIVE ANGIOGRAPHY-DERIVED FRACTIONAL FLOW RESERVE MAY PREDICT BYPASS GRAFT OCCLUSION AND DISEASE PROGRESSION AT MID-TERM FOLLOW-UP
Authors: Mushtaq Saima , Gigante Carlo , Conte Edoardo , Capovilla Teresa Maria , Sonck Jeroen , Tanzilli Alessandra , Barbato Emanuele , Pontone Gianluca , Bartorelli Antonio Luca , Collet Carlos , Andreini Daniele 
Published: December 2022

Abstract

Background
Coronary artery bypass grafting (CABG) is the most common revascularization mode for patients with multivessel coronary artery disease (CAD). Despite substantial improvements in the outcome of patients undergoing CABG surgery, graft occlusion remains a weakness of this procedure and has been associated with competitive flow of native coronary arteries. Our study aims to assess graft occlusion and the progression of CAD in native vessels after CABG surgery at follow-up with coronary CT angiography (CCTA), and their relationship with angiography-derived vessel-fractional flow reserve (vFFR) performed before surgery.

Methods
We retrospectively selected patients from two European institutions, treated with CABG between January 2006 and December 2018, who underwent follow-up with CCTA. All patients had a preoperative angiogram performed before CABG.

Results
In 171 consecutive patients, serial preoperative angiograms were suitable for vFFR analysis of 298 grafted and 59 non-grafted vessels. Table 1 shows clinical and main procedural characteristics. Preoperative vFFR was assessed in 131 left anterior descending arteries (LAD), 132 left circumflex arteries (LCX) and 94 right coronary arteries (RCA) and was &lt;0.80 in 255 of 298 bypassed vessels. Graft occlusion was observed at CCTA in 28 of 298 grafts. The median preoperative vFFR value of native coronaries was higher in occluded compared to patent grafts (0.75 vs. 0.60, p&lt;0.001) (Figure 1) and was associated with graft type. The best vFFR cut-off to predict graft occlusion was 0.67. The functional CAD progression was higher in grafted compared to non-grafted vessels (89.6% vs 47.5% p &lt; 0.001) and pre-CABG vFFR predicted disease progression of grafted native vessels (AUC=0.83) (Figure 2).

Conclusions
Preoperative vFFR derived from invasive coronary angiography was able to predict graft occlusion and CAD progression of grafted coronary arteries.
784 IMPACT OF ELECTIVE, UNCOMPLICATED TARGET LESION REVASCULARIZATION ON CARDIAC MORTALITY AFTER ELECTIVE PERCUTANEOUS CORONARY INTERVENTION OF UNPROTECTED LEFT MAIN CORONARY ARTERY DISEASE
Authors: Celeski Mihail , Compagnone Miriam , Taglieri Nevio , Ghetti Gabriele , Marrozzini Cinzia , Orzalkiewicz Mateusz , Bruno Antonio Giulio , Galiè Nazzareno , Saia Francesco , Palmerini Tullio 
Published: December 2022

Abstract

Background
Percutaneous coronary intervention (PCI) appears a reasonable alternative to coronary artery bypass graft in selected groups of patients with unprotected left main coronary artery (ULMCA) disease. Despite technological progression, improved operator skills and introduction of drug-eluting stents (DES), in-stent restenosis still remains a major limitation after PCI of ULMCA disease. The prognostic relevance of target lesion revascularization (TLR) after PCI of ULMCA disease is unknown.

Objectives
This study sought to evaluate the impact of elective, uncomplicated target lesion revascularization on long-term cardiac mortality after PCI of ULMCA disease.

Methods
Consecutive patients undergoing PCI for ULMCA disease between January 2003 and December 2015 in one interventional center in Italy were included. Patients presenting with cardiogenic shock, ST-segment elevation myocardial infarction (MI), as well as those undergoing urgent or complicated TLR were excluded. The primary endpoint of the study was cardiac mortality. The secondary endpoints of the study were all-cause death, MI, stroke as individual endpoints, or the composite of cardiac death, MI or stroke defined as major adverse cardiac events (MACE).

Results
Among the 418 patients fulfilling the study criteria, 79 (18.46%) underwent elective, uncomplicated TLR. After a median follow-up of 5.5 years, there were 23 cardiac deaths among patients undergoing elective, uncomplicated TLR versus 50 in patients not undergoing TLR. After adjusting for possible confounders, TLR was an independent predictor of cardiac mortality (Hazard ratio [HR] = 1.92, 95% confidence interval [CI]: 1.05 to 3.49; p = 0.03). Patients undergoing TLR had also significantly higher rates of the composite of cardiac death, MI and stroke compared with the no TLR group (adjusted HR = 1.76, 95% CI 1.14 to 2.72).

Conclusion
Elective, uncomplicated TLR after PCI of ULMCA disease is associated with increased risk of long-term cardiac mortality. Reducing the risk of TLR after PCI of ULMCA disease may potentially improve the survival of these patients.
604 PERCUTANEOUS TRANSCATHETER CLOSURE OF SYMPTOMATIC PATENT FORAMEN OVALE IN PATIENT WITH INFERIOR VENA CAVA INTERRUPTION
Authors: De Filippis Aurelio , Ricottini Elisabetta , Gallo Paolo , Cammalleri Valeria , Nobile Edoardo , Nusca Annunziata , Circhetta Simone , Mangiacapra Fabio , Rinaldi Raffaele , Cocco Nino , Melfi Rosetta , Grigioni Francesco , Paolo Ussia Gian 
Published: December 2022

Abstract
Percutaneous closure of a patent forame ovale (PFO) is indicated in selected patients with a confirmed cryptogenic stroke, transient ischemic attack (TIA), or systemic embolism and an estimated high probability of a casual role of the PFO as assessed by clinical, anatomical and imaging features. This procedure is usually undertaken from the femoral vein. In this case, the patient was affected by vena cava agenesis with a compensatory enlargement of azygos vein.
We performed the percutaneous closure of the PFO using a 9F jugular vein access. We advanced a straight guide trough the foramen with a Multipurpose 6F catheter supported by a Destino Oscor 8F. Then we changed the straight guide with an Amplatz Super-Stiff 0.035" guide in the left ventricle, on which a Mullins 9F trans-septal catheter was advanced. Finally we delivered an Amplatzer Multifenestrated Septal Occluder "Cribriform" 25/25 mm.
In our case, it was impossible to perform the PFO closure procedure using the classic femoral vein access due to the inferior vena cava agenesis. Performing PFO closure using the right internal jugular venous approach was technically challenging but safe and it is possible using standard equipment normally present in the Catheterization Laboratory.
In rare cases of an indication for PFO closure and lack of access through the femoral veins, alternative techniques using on the shelf materials help in overcoming the challenging anatomies. The use of steerable catheter is critical for engaging the foramen ovale with unfavourable angulation. The procedure with nitinol double disk devices is safe and effective.