bluuebunny/crossref_metadata_2025 huggingface dataset:
C36 NON–CYCLIC INTERMITTENT PROSTHETIC MITRAL VALVE DYSFUNCTION: A CASE REPORT OF A DIFFICULT DIAGNOSIS CONFIRMED WITH MULTIMODALITY IMAGING
Authors: Piroli F , Mantovani F , Faragasso E , Andreoli A , Guiducci V , Fiorani V , Pergola V , Navazio A
Published: May 2023

A 59–year–old man,obese,with history of hypertension, 3 years ago underwent mitral valve (MV) replacement with a mechanical prothesis(Sorin Bicarbon 31) and preservation of the native subvalvular apparatus for severe mitral regurgitation due to anterior leaflet flail. Pre–discharge transthoracic echocardiography (TTE) showed normal prothesis function (mean pressure gradient (PG) 3,2mmHg), no valvular leaks and a normal ejection fraction (EF). Lately, the patient was admitted twice to emergency department for signs of heart failure, solved with increasing dose of oral diuretics. In both cases a TTE showed a normal EF, a trans–prosthetic mean PG of 6,5mmHg and no valvular impairment/leaks. Since the suboptimal TTE acoustic windows, a transesophageal echocardiography (TEE) was recommended. TEE showed a normal motion of both MV discs and no pannus/thrombus or leaks with the use of a 2–beat acquisition protocol. However, with longer observation a pattern of non–cyclic intermittent valve dysfunction was noted: whereas some cardiac cycles showed normal discs’ motion, other cycles demonstrated an emi–disc opening delay or blockage during diastole with significant prolongation of transmitral pressure half–time and higher transvalvular gradient, suggestive of flow impairment. A cine fluoroscopy and a CT assessment were also performed, showing the visible intermittent pattern of valve dysfunction without radiological signs of thrombosis or pannus. At this point, the interference of the MV apparatus with the emidisc was considered the most probable mechanism of dysfunction. A Heart Team decision toward reintervention was taken since the risk of a sudden worsening of the mechanical valve function, with potentially lethal consequences, was considered high. The surgeon at visual inspection confirmed the interference of the subvalvular apparatus with the posterior emidisc motion. The chronic shear stress generated on the mitral remnant likely developed a progressive fibrotic response that determined the thickening of the tissue and the periodical entrapment of the disc. MV prothesis was replaced with resection of the subvalvular apparatus. At dismissal,TTE evaluation showed normal valvular function confirmed at 6 months follow–up. Intermittent prosthetic MV dysfunction is a rare but potentially serious complication of MV replacement with subvalvular apparatus preservation. TEE, cine–fluoroscopy and CT may be useful as complementary tools to detect this rare complication.
C37 PROGNOSTIC IMPLICATION OF PREOPERATIVE ANEMIA IN REDO CARDIAC SURGERY: A SINGLE CENTER PROPENSITY MATCHED ANALYSIS
Authors: Rubino A , Montella A , Golini Petrarcone C , Palmieri L , Galbiati D , De Feo M , De Santo L
Published: May 2023

Preoperative anemia has been associated with increased morbidity and mortality after cardiac surgery, but little is known on its prognostic value in the setting of redo procedure. This study aimed to investigate whether preoperative anemia is an independent determinant of adverse events in this augmented risk patients’ subset. A retrospective, observational, cohort study of prospectively collected data was undertaken on 409 consecutive patients referred for redo cardiac procedures between January 2011 and December 2020 (mean age of 62 ± 12.7 years, octogenarians 5.6%, female 52.8%, diabetes 19.5%, chronic kidney disease 30%, urgent/emergent status 34.8%, third–time sternotomy 9.8%). The EuroSCORE II calculated an average mortality risk of 25.7 ± 15.4%. The prevalence of preoperative anemia (according to World Health Organization definition) and its relationships with postoperative morbidity were obtained. Selection bias was balanced with propensity–matching method. The prevalence of preoperative anemia was 41%. In unmatched analysis, significant differences between the anemic and non–anemic group emerged in the risk of postoperative stroke (0.6% vs 4.4%, p = 0.023), postoperative renal dysfunction (29,7% vs 15.6%, p = 0.001), need for prolonged ventilation (18.1% vs 7.2%, p = 0.002), high dosage inotropes (53.1% vs 32.9%, p < 0.001) along with both length of ICU and hospital stay (8.2±15.9 vs 4.3±5.4 days, p=0.003 and 18.8±17.4 vs 14.9±11.1, p=0.012 ). After propensity matching (143 pairs), preoperative anemia was still significantly associated with postoperative renal dysfunction, stroke and need for high dosage inotropes. Preoperative anemia is significantly associated with acute kidney injury, stroke and need for high dosage inotropes in patients referred for redo procedures. Further studies are warranted to determine whether preoperative low hemoglobin concentration is a marker of severity of illness or a modifiable risk factor.
C39 TOTALLY ENDOSCOPIC VALVE SURGERY: FEASIBILITY AND ADVANTAGES IN OBESE PATIENTS
Authors: Zoni D , Cresce G , Hinna–danesi T , Salvador L
Published: May 2023

Objective

Obesity is a relative contraindication to Minimally Invasive Cardiac Surgery because of its technical challenges. Aim of the study is to evaluate if totally endoscopic approach is feasible in treating heart valve’s pathology in obese patients and if 30–days outcomes and complications are non–inferior to non–obese patients.

Methods

From January 2018 to June 2022, 1544 consecutive unselected patients underwent totally endoscopic heart valve treatment at our institution. Treated valves were aortic, mitral, tricuspid in various combination. Concomitant procedures and different etiology were included. Among them, 177 were obese people and 1367 were non–obese based on Body Mass Index (BMI). Main working port was obtained with limited skin incision: peri–areolar/sub–mammary for mitral and tricuspid treatment, right anterior mini–thoracotomy for aortic valve treatment. We used a 5 mm 30° endoscope and 2 additional 5 mm miniport for vent line and aortic clamp. Neo–chordae implantation plus annuloplasty ring was the preferred technique for mitral repair.

Results

Median BMI and 1°– 3° quartiles in non–obese group was 24 [22 – 26] and inferior to obese group 32 [30 – 33] (p < 0.01). In both group CPB and Cross Clamp median time was not statistically significant: 147 minutes [121 – 181] vs 150 minutes [126 – 195] (p = 0.69), 95 [77 – 120] vs 97 [79 – 122] (p = 0.39). Conversion to full sternotomy occurred in 14 cases (0.9%) in non–obese patients vs 2 cases (1.12%) in obese patients (p = 0.89). Oro–Tracheal intubation median time was 5 hours [4 – 7] in non obese versus 6 hours [4 – 8] in obese (p < 0.01). ICU median hours stay were 20 [16 – 40] vs 24 [18 – 48] respectively in non–obese and obese group (p < 0.01). Post–operative hospitalization median stay was 5 days [4 – 7] vs 6 days [4 – 8] in obese group (p = 0.06). Stroke occurred in 15 non–obese patients (0.9%) vs 3 obese patients (1.6%) (p = 0.42). Wound complications occurred in 9 non–obese patients (0.65%) vs 5 obese patients (2.8%) (p = 0.52). All cause of mortality was 18 (1.1%) in non–obese vs 6 (3.3%) in obese (p = 0.11).

Conclusions

Surgery was feasible in both groups with comparable CPB and Cross–Clamp time, no difference in surgical revision rate and sternotomic conversion. Obese patients had slightly longer OTI time and ICU stay. However hospitalization days, mortality, stroke and wound complications rate were not different. Totally endoscopic approach was essential to achieve these results.
C42 OR 1896 EXERTS POSITIVE INOTROPIC EFFECTS IN ISOLATED HUMAN RIGHT ATRIAL MUSCLE
Authors: Neumann J , Gergs U , Berbhardt M , Hofmann B , Rayo–Abella L
Published: May 2023

Levosimendan is approved for use as an intravenous agent to treat temporarily patients with severe systolic heart failure. It is thought, but hitherto never tested in isolated human cardiac tissue, that the main metabolite of levosimendan, called OR 1896, might cause the positive inotropic effect of levosimendan in patients. For comparison, we studied mouse atrial preparations. OR alone (up to 10 µM) failed to increase force of contraction in mouse atrial preparations while in the presence of the phosphodiesterase (PDE) IV–inhibitor rolipram (0.1 µM) profound positive inotropic effects and positive chronotropic effects of OR 1895 were recorded in mouse atrial preparations. In contrast to mouse atrium, we noticed that OR 1896 alone (0.01 µM to 10 µM cumulatively applied) exerted time– and concentration–dependent positive inotropic effects 1 µM increase by 72 ± 14.7%, p<0.05, n=7) and shortened the time of relaxation (by 7 .3 %, p<0.05, n=7) in isolated electrically paced human right atrial muscle preparations (obtained during cardiac surgery), that started at 0.1 µM and plateaued at 1 µM OR 1896. The positive inotropic effect of OR 1896 was antagonized by 10 µM propranolol. The positive inotropic effect of OR 1896 in mouse atrial preparations and human right atrial preparations was less effective than that of 1 µM isoprenaline (human atrium by 154 %). The positive inotropic effect of OR 1896 was diminished by pre–incubation of the samples with the PDE III inhibitor cilostamide (10 µM). EMD 57033 (10 µM), a calcium sensitizer, increased force of contraction further in the additional presence of 1 µM OR 1896. Our data suggest that OR 1896 is indeed a positive inotropic agent in the human heart and that OR 1896 acts mainly as a PDE III – inhibitor because the effect of OR 1896 was attenuated by a β–adrenoceptor antagonist and thus OR 1896 is unlikely to act as a calcium sensitizer in the human heart.
C44 ATRIAL SEPTAL ABNORMALITIES: A CASE OF DOUBLE INTERATRIAL SEPTUM
Authors: Molisana M , Cicchitti V , Bagicalupi E , Ianni U , Procopio A , Toro A , Parato V , Gallina S , Ricci F , Zimarino M
Published: May 2023

The Case A 41–year–old asymptomatic male was referred to our cardiology outpatient clinic for diagnostic evaluation of a suspected bicuspid aortic valve at transthoracic echocardiography. Trans–oesophageal imaging ruled–out the bicuspid aortic valve, but detected an accessory membrane lining the interatrial septum with a double–layered appearance encompassing most of the fossa ovalis. Agitated saline infusion demonstrated an inter–atrial communication with right–to–left shunt under Valsalva manoeuvre. Cardiovascular magnetic resonance cine imaging confirmed the presence of a double interatrial septum featuring a midline recess enclosed by the two septa. Under end–expiratory breath–holding conditions, a transient right–to–left shunt was observed confirming the echocardiographic evaluation (Panel 1). No history of cerebrovascular disease and no clinical or imaging findings of right heart overload were registered.

Discussion

Double interatrial septum is a rare congenital condition, by morphological side, allegedly associated with an increased risk of left–sided embolic events due to blood stasis within the accessory interatrial chamber, that is often found in continuity with both atria through patent foramen ovale or additional septal fenestrations. In this case, multimodality imaging effectively was able to detect the presence of this rare atrial septal abnormality and to distinguish that from others alterations of the interatrial septum shown in the table below (Table 1). Nevertheless, the clinical significance of double interatrial septum remains uncertain, no clear relationship with embolic stroke is known and no recommendation can be made regarding the decision–making for long–term anti–thrombotic management. High quality evidences are needed to evaluate whether double atrial septum or other atrial septal abnormalities might be considered as actionable risk factors of ischemic stroke or not.
C45 MULTI–INSTITUTIONAL ITALIAN EXPERIENCE OF ATRIAL FLOW REGULATOR DEVICE IN CONGENITAL AND ACQUIRED HEART DISEASE
Authors: Cuppini E , Castaldi B , Sirico D , Piccinelli E , Butera G , Di Salvo G
Published: May 2023

The Atrial Flow Regulator is a self–expandable nitinol device implanted into the interatrial septum to establish a communication with a predetermined diameter.The creation of such shunt is advisable in several cardiovascular diseases.In pediatric population,the experience is limited to a few case reports describing patients with failing Fontan,pulmonary hypertension,or severe heart failure.We aim to report the preliminary data of Italian registry of AFR implantation in children with heart failure in congenital and acquired heart disease.The goal is to create a local and national register,to monitoring the safety and efficacy of the AFR in pediatric population.The register is prospective and multi–institutional, with 2 centers involved: Pediatric Cardiology department of Padova Hospital and of Bambino Gesù Hospital in Rome,started on the 27/11/2021 and ongoing.AFR implantation was proposed for all patients with symptomatic heart failure,not responding to maximal medical therapy and a history of complex congenital or acquired cardiac diseases.We enrolled 8 patients (median age 124 months)indications were LV systolic dysfunction in 4 patients,restrictive cardiomyopathy with pulmonary hypertension in one, post–operative RV dysfunction after surgical repair of a native TOF and 2 patients with failing Fontan.AFR implantation was successfully reached in all 8 patients.In one case,it was performed a hybrid procedure during pulmonary artery surgical banding, with peratrial approach through the right atrial free wall.Atrial septostomy was needed in 5 cases, in the other 3 cases pre–existing PFO and ASD were used.Balloon pre–dilation was performed in 4 cases.Mean time of procedure was 50 minutes and median radiation exposure dose was 3 Gy/cm2.No complications were reported during the procedure.No acute or sub–acute shunt occlusion or stroke was observed.One patient died of sepsis not related to the procedure;one patient presented a cardiac arrest due to a cerebral ischemia 24 hours after the procedure.Heparin or aspirin were administered to all patients for at least 6 months.Clinical improvement was observed in all patients with an increase in ejection fraction (p=0.02), reduction in LA dilation(p< 0.05)and in HF symptoms(62% of patients improved their NYHA class).AFR implantation is safe and feasible in children with specific congenital and acquired heart diseases,allowing right/left cavities unloading and improvement of hemodynamics and symptoms.
C50 VERY LONG TERM FOLLOW UP OF PRIMARY TRANSCATHETER PATENT FORAMEN OVALE CLOSURE FOR MIGRAINE CURE
Authors: Rigatelli G , Filippo G , Russo M , Adami A , Aggio S , Roncon L , Zuin M
Published: May 2023

Background

Results of previous trials about PFO closure as a treatment for migraine are still doubtful and inconclusive. We sought to evaluate the long–term (>10 years) effectiveness of migraine treatment by means of patent foramen ovale (PFO) closure.

Methods

From 2006 to 2010, 86 patients (68 female, mean age 40.0 ±3.7 years) with disabling, medication–refractory migraine and PFO were enrolled in a prospective study over a 48–month period. Criteria for transcatheter closure were all the following: presence of a permanent right–to–left shunt (RLS) having a shower/curtain pattern, presence of interatrial septal aneurysm and Eustachian valve, 3 to 4 class Migraine Disability Assessment Score (MIDAS), coagulation abnormalities, and medication–refractory migraine with or without aura.

Results

Forty patients (46.5%) (34 females, mean age 35.0±6.7 years, mean MIDAS 35.8 ± 4.7) underwent transcatheter closure. After a mean follow–up of 118.2 ±19.1 months (range 96 to 144), 37 patients continued the follow–up and experienced symptomatic improvement: aura was abolished in the totality of patients along with a durable improvement of migraine throughout the extended follow up also in patients without aura. The mean MIDAS significantly decreased in the closure group (p<0.0001) (Figure 1). Patients with complete migraine resolution presented a shorter history of migraine, a more severe thrombophilic profile, more severe RLS and larger left atrial diameter and volume.

Conclusions

Primary transcatheter PFO closure in selected high–risk patients having disabling refractory migraine resulted in abolition of aura and in a sustained reduction in migraine in the very long–term period.
C53 EFFECTS OF CARDIAC CONTRACTILITY MODULATION THERAPY ON LEFT VENTRICLE GLOBAL LONGITUDINAL STRAIN AND MYOCARDIAL MECHANO–ENERGETIC EFFICIENCY IN PATIENTS WITH HEART FAILURE WITH REDUCED EJECTION FRACTION
Authors: Martucci M , Kittleson M , De Vivo S , D’Onofrio A , Ammendola E , Nigro G , Contaldi C , Errigo V , Pacileo G , Masarone D
Published: May 2023

Background

Impairment of myocardial mechano–energetic efficiency (MEE) is present in virtually all patients with heart failure with reduced ejection fraction (HFrEF). Cardiac contractility modulation (CCM) improves the quality of life and functional capacity, reduces hospitalizations, and induces biventricular reverse remodeling in patients with HFrEF. However, the effects of this innovative therapy on mechano–energetics efficiency and global longitudinal strain (GLS) are still unknown; therefore, the aim of this study is to evaluate whether CCM therapy can improve the MEE of patients with HFrEF.

Methods

We enrolled 25 patients with HFrEF who received an Optimizer Smart implant (the device that develops CCM therapy) between January 2018 and January 2021. Clinical and echocardiographic data were obtained from all patients 24 hours before and six months after CCM therapy.

Results

At six months, follow–up patients who underwent CCM therapy showed an increase of left ventricular ejection fraction (30.8+7.1 vs. 36.1+6.9%; p = 0.032) as well a rise of GLS 10.3+2.7 vs.–12.9+4.2; p = 0.018), of MME (32.2 + 10.1 vs. 38.6 + 7.6 mL/s; p = 0.013) and of MEE index (18.4 + 6.3 vs 24.3 + 6.7 mL/s/g; p = 0.022).

Conclusions

CCM therapy increased left ventricular performance, improving left ventricular ejection fraction, GLS, as well as MEE and MEEi.
C54 ACUTE AND LONG TERM PERFORMANCE OF MAGMARIS BIORESORBABLE SCAFFOLD IMPLANTATION IN SPONTANEOUS CORONARY ARTERY DISSECTION (SCAD): THE EXPERIENCE OF THE COORDINATOR CENTER OF THE INTERNATIONAL MULTICENTER REGISTRY DISCO
Authors: Giacobbe F , Cerrato E , Pavani M , Rolfo C , Biolé C , Bianco M , Franzé A , Zanda G , Piedimonte G , Lo Savio L , Celentani D , Lococo M , Tizzani E , Tomassini F , Chinaglia A , Varbella F
Published: May 2023

Background

Spontaneous Coronary Artery Dissection (SCAD) is a challenging clinical scenario and represents an increasingly studied cause of acute coronary syndrome (ACS). An interventional strategy in these cases remains controversial since it may require an extensive permanent metallic stenting of vessel to seal the dissection with a non negligible rate of complications. Moreover, SCAD patients are usually young women with non–calcified disease where Bioresorbable Scaffolds (BrS) may be an intriguing option.

Methods

DISCO IT/SPA is a International observational registry which retrospectively enrolled patients admitted with a diagnosis of SCAD across 26 Italian and Spanish centers. Baseline, procedural characteristics, in–hospital management and outcomes of 369 SCAD patients were collected, of these 12 patients were treated with PCI with BrS MAGMARIS at the coordinator center of Rivoli and Orbassano. Major cardiovascular events (MACEs) were defined as the composite of cardiovascular death, non–fatal Myocardial infarction (MIs) and any PCI performed.

Results

In our cohort of SCAD patients treated with BrS there was a slight prevalence of women (n=7, 58,3%) with an average age of 50,9±7,4 years. NSTE–ACS was the most common clinical presentation (83,3%), followed by STEMI (16,7%). In 7 patients the first treatment choice for SCAD was PCI with BrS deployment, in 5 patients BrS were deployed during hospitalization for unplanned PCIs (four of them) and after one month for a MI due to SCAD recurrence in the last patient. Multivessel disease was present only in one case and LAD–DG was the main vessel affected (n=10, 83,3%). SCAD type 2A was the most frequent (41,7%). Intravascular imaging was performed in 10 cases (83,3%) and procedural success was achieved in all cases. Angiographic follow–up was obtained in 9 patients (75%), through coronary angiogram (n=8) or CT scan (n=1), at a mean length of 5,2±5,6 months. In–hospital MACE were 5 (23,8%) but none of them occurred after BrS deployment. The only MACE reported after BrS deployment was an unplanned PCI for SCAD progression.

Conclusions

In SCAD bioresorbable scaffolds represent an excellent option to preserve the natural physiology of the coronary vessels. Notably, in our cohort we reported a very high procedural success rate in PCI assisted in large part by intracoronary imaging. Further studies with larger numerosity are needed to demonstrate the advantages of this type of scaffold in these patients.
C60 MYOCARDIAL T1 MAPPING IN ANDERSON–FABRY DISEASE AND ITS CORRELATION WITH GENETIC ANOMALITIES
Authors: Casavecchia G , Mautone F , Pesolo M , Gravina M , Brunetti N
Published: May 2023

Objective

Cardiac involvement in Anderson–Fabry Disease is possible even in mild forms, and new analytical methods and the quantitative value evident with the T1 Native sequence for Mapping study demonstrated a linear correlation with disease severity compared with traditional MR sequences. GLA gene alterations on the X chromosome are very heterogeneous and correlated with decreased or absent alpha–galactosidase enzyme, resulting in different timing of manifestation and clinical evolution. In addition to the classic form of central nervous system, kidney and heart involvement there is a variant with only cardiac involvement, also found in our study. Materials and

Methods

We retrospectively analyzed 18 consecutive Cardiac MRI examinations performed in patients with established Anderson–Fabry disease at the University Radiology Department of Policlinico Riuniti di Foggia from August 2019 to January 2022. Native T1 sequences for T1 Mapping study are currently the gold standard for evaluation of cardiac involvement in Anderson–Fabry disease.

Results

T1 Mapping values were lower with characteristic normality of ECV after mdc. In PSIR sequences acquired after mdc for the study of "Late Gadolinium Enhancement" usually a mesocardial signal hyperintensity of the infero–lateral wall of the left ventricle characterized by fibrotic deposits is evident but this sign usually occurs when the heart is already hypertrophied. Instead, reduced Native T1 values serve as an early marker of myocardial involvement, even before the development of hypertrophy as also found in our study population. In fact in 10 out of 18 patients the only value indicating myocardial involvement was reduced T1 Mapping Native values below 1000 in at least 3 out of 17 segments. In 6 cases there was concentric left ventricular wall hypertrophy and in 2 cases mesocardial hyperintensity was evident in the PSIR sequences for the "Late Gadolinium Enhancement" study of the basal segments of the infero–lateral left ventricular wall.

Conclusions

Magnetic resonance imaging plays a key role in the study of cardiac involvement in Anderson–Fabry disease with potential to predict the extent of detectable accumulation at the cardiac level based on the genetic abnormality.
C63 PREVALENCE AND CLINICAL SIGNIFICANCE OF RIGHT VENTRICULAR PULMONARY ARTERIAL UNCOUPLING IN CARDIAC AMYLOIDOSIS
Authors: Palmiero G , Monda E , Verrillo F , Dongiglio F , Caiazza M , Rubino M , Lioncino M , Diana G , Vetrano E , Fusco A , Cirillo A , Mauriello A , Ciccarelli G , De Rimini M , D’Alto M , Cerciello G , D‘Andrea A , Golino P , Calabrò P , Bossone E , Limongelli G
Published: May 2023

Background

This study aims to evaluate the prevalence and clinical significance of the right ventricular pulmonary arterial (RV–PA) uncoupling in patients with cardiac amyloidosis (CA).

Methods

The study population consisted in 92 consecutive patients with CA (age 71.1±12.2 years, 71% males; 47% with immunoglobulin light chain (AL), 53% with transthyretin [ATTR]). All patients underwent a comprehensive evaluation. A pre–specified tricuspid annulus plane systolic excursion on pulmonary arterial systolic pressure (TAPSE/PASP) value <0.31 mm/mmHg was used to define RV–PA uncoupling and to dichotomize the study population.

Results

Thirty–two patients (35%) showed RV–PA uncoupling at baseline evaluation (15/44 [34%] AL and 17/48 [35%] ATTR). Patients with RV–PA uncoupling in both AL and ATTR showed worse NYHA functional class, lower systemic blood pressure, and more pronounced left and right ventricular systolic dysfunction than those with RV–PA coupling. During a median follow–up of 8 months (IQR 4–13), 26 patients (28%) experienced cardiovascular death. Patients with RV–PA uncoupling showed lower survival at 12 months follow–up than those with RV–PA coupling (42.7% [95%CI 21.7–63.7%] vs. 87.3% [95%CI 78.3–96.3%], p–value<0.001). Multivariate analysis identified high–sensitivity troponin I values (HR 1.01 [95%CI 1.00–1.02] per 1 pg/mL increase; p–value 0.013) and TAPSE/PASP (HR 1.07 [95%CI 1.03–1.11] per 0.01 mm/mmHg decrease; p–value 0.002) as independent predictors of cardiovascular death.

Conclusions

RV–PA uncoupling is common among patients with CA, and it is a marker of advanced disease and worse outcomes. This study suggest that TAPSE/PASP ratio has the potential to improve risk stratification and guide management strategies in patients with CA.
C64 REDUCTION IN EJECTION AND PRE–EJECTION PERIODS ARE PREDICTIVE OF MORTALITY IN AL AND ATTR HEART AMYLOIDOSIS
Authors: Cicco S , Melaccio A , De Fazio G , Marozzi M , Solimando A , Ria R , Vacca A
Published: May 2023

Background

Amyloidosis is a systemic disease caused by fibrillary misfolded protein deposition in several organs including the heart. Cardiac deposition is the leading cause of death in these patients. Echocardiography is the imaging technique mostly used to study cardiac amyloidosis. Many studies tried to identify earlier markers to stratify the mortality risk. Heart stiffness is the main issue leading the heart dysfunction. However, few data are available on the possible role of connection between heart and possible vessels alterations. Purpose: Aim of this study was to evaluate heart stiffness using Ventricular–arterial coupling (VAC) for relationship between heart and vessel tree.

Methods

We studied 58 patients (22 F and 36 M, aged 67.14± 12.49) with AL or ATTR amyloidosis and heart involvement. They were 42 AL and 16 ATTR patients. All patients were evaluated before treatment with a complete history, physical exam, serum markers of disease, and echocardiogram. The abdominal fat biopsy to confirm amyloid deposition was performed before treatment. Among all patients, 18 died with a mean OS of 12 months [IQR 1–24 months].

Results

At baseline, VAC was increased in died patients (alive 1.53 ± 0.43 vs died 2.06 ± 1.73, p=0.09) but was ineffective to predict the risk of mortality (ROC analysis AUC 0.54, p=0.57). By analysing ejection and pre–ejection periods we found that they were both increased in alive patients (p=0.0016 and p=0.0072, respectively). Moreover, these periods were effective in prediction of the death risk (ejection time AUC 0.81, cut–off 239 msec, sensitivity 61.54% and specificity 88.24%; pre–ejection time AUC 0.75, cut–off 82 msec, sensitivity 84.62% and specificity 58.82%). No difference in VAC, ejection time or pre–ejection time were found comparing AL to ATTR, nor both parameters had a predictive role.

Conclusion

Amyloidosis induces an increase in heart stiffness and reduces heart contractility. This causes a reduction of peripheral blood flow and a worsening in heart failure. We demonstrated that this mechanism affects the capacity to pump blood in vessels due the reduction in time for loading during the systole. These results may predict the mortality with a significant sensitivity and specificity, regardless of the type of amyloidosis.
C68 PREVALENCE OF CARDIOINHIBITORY SYNCOPE IN A SERIES OF PATIENTS WITH THE CORRECT TILT TABLE TEST INDICATION. PRE–TEST PREDISPOSING FACTORS IDENTIFIED
Authors: Cavarra M , Salerno E , Chiarandà M , Chiarandà G , Tamburino C
Published: May 2023

Purpose of study : Data from the last ESC LLGGs on syncope show that the prevalence of cardioinhibitory syncope ( IIB according to VASIS classification ) at the Tilt table test (TTT) is 8% in the general population and 13% in patients with positive TTT ( TTT + ).The purpose of our work was to evaluate a) the prevalence of TTT+ with cardioinhibitory response (IIB VASIS) in a population correctly subjected to TTT for suspected neuromediated reflex syncope; b) if there are predisposing pre–test indicators; c) how many patients (pts) with TTT + with IIB response suitable for permanent pacemaker implant (PPM ) Material and methods : In our laboratory, in the last 6 years, there have been considered 989 patients for suspected neurally reflex syncope (NMRS) and subjected with correct indication to TTT using the Italian protocol. From this population was extrapolated a case study of pt with TTT+ for vasovagal syncope. In this case the prevailing TTT+ with IIB VASIS response was evaluated. Were sought multivariate analysis indicators pre test predisposing. In addiction, it was evaluated how many pts there was a correct indication for a PPM.

Results

TTT+ occurred in 641 pts ( 64.8%). In this case the TTT+ was found in 79 % of the pts with suspected typical NMRS and 55 % of the pts with suspected atypical NMRS. In 641 pts with TTT+ the prevalence of IIB response was observed in 41.1 %. In particular in 59 % of pts with typical NMRS and 29 % of pts with atypical NMRS ( p 0.0001 ). Multivariate analysis was identified as indicators independently associated with the IIB response male ( HR 0.5981 p .0134 ), syncope with trauma ( HR 3.1123 p .0015 ), hypertensive heart disease ( HR 2.9834 p.0058 ). The TTT+ IIB pts that had a correct indication to the PPM implant according to ESC LLGGs were 21 % ( 134 pcs )

Conclusions

the prevalence of cardioinhibitory syncope in our case studies is reused higher (41 %) than the literature data in previous cohorts ( 13–16 %). The percentage of pts with correct indication at PPM was almost 1 in 4 ( 21%). Trauma and hypertensive heart disease appear to be independent factors of cardioinhibitory TTT response useful to guide therapeutic diagnostic conduct.
C69 SHORT–TERM OUTCOMES OF ABLATION INDEX–GUIDED CAVOTRICUSPID ISTHMUS ABLATION VERSUS UNIPOLAR RECORDED POTENTIAL INVERSION: A RANDOMIZED, SINGLE–CENTRE STUDY
Authors: Matteucci A , Galeazzi M , Russo M , Schiaffini G , Cappuccio C , Di Fusco S , Lombardi E , Pandozi C , Colivicchi F
Published: May 2023

Background

Ablation of the cavotricuspid isthmus (CTI) using contiguous lesions guided by electroanatomical mapping on fluoroscopy assisted by the ablation index (AI) in typical atrial flutter (AFL) is a widely used procedure. Unipolar signal inversion during radiofrequency catheter ablation (RFCA) was found to be a predictive index of transmural lesion, as well as AI–guided strategy showed to be effective in CTI ablation while maintaining an inter–lesion–distance (ILD) ≤ 6mm. Both are related to the block of the CTI, however, it remains unclear how AI is related to the inversion of the negative component of the unipolar signal. The aim of our study is to evaluate the effectiveness of ablation based on unipolar recorded potential inversion and the corresponding AI values.

Methods

Thirty consecutive patients with AFL diagnosis were enrolled in our Centre to undergo CTI ablation. Patients were randomized 2:1 into the control arm and the experimental arm respectively. Contiguous lesions were performed using current AI standards until values of 500 were reached in the control group. In the experimental group, contiguous lesions were dispensed until inversion of the negative component of the unipolar signal was achieved, while simultaneously recording AI values. After CTI ablation, a 30–minute monitoring was performed to evaluate the efficacy and possible inducibility of AFL.

Results

A successful ablation procedure was performed in 30 patients. High–density mapping acquired on average 2766±1767 points vs. 2930±2177 (p=0.82), with a mean mapping time of 10.3±4.9 min vs 13.4±5.8 min (p=o.13). The procedural time between the two groups was comparable ( 77.9±27.6 vs 78.3 78.3±22.4 p 0.96). In both arms, the treatment effectively reduced the mean local potential in CTI (2.42±1.09 mV vs 2.71±1.26 mV p=0.51). Compared to the control group, unipolar signal inversion showed significantly lower ablation index values (500 vs 405±41.1 p < 0.0001).

Conclusions

The inversion of the negative component of the unipolar signal confirms to be an effective strategy in cavotricuspid isthmus ablation. Procedural success can be achieved at significantly lower corresponding ablation index values.
C70 WEARABLE CARDIOVERTER DEFIBRILLATOR IN LONG QT SYNDROME DURING PREGNANCY: TIME TO USE THE SHIELD
Authors: Forleo C , Carella M , Basile P , Memeo R , Dadamo M , Resta N , Vimercati A , Guaricci A , Ciccone M
Published: May 2023

Introduction

Long QT Syndrome (LQTS) is a rare cardiac channelopathy characterized by a high risk of lethal arrhythmias. Pregnancy and post–partum period may induce an increased vulnerability, so an appropriate prevention is needed. Case presentation A 31–year–old primigravid woman at 36 weeks of pregnancy was referred to our Cardiomyopathy Unit located in a tertiary centre for LQTS screening. The patient had a family history of LQTS (mother and brother) and Brugada syndrome (cousin). The 12–lead ECG (Figure 1) showed a long QT interval (QT corrected: 508 msec), while the transthoracic echocardiogram was unremarkable. The genetic test performed is still ongoing. The patient reported sporadic episodes of palpitations at rest and the following 24–hour ambulatory ECG revealed a very high burden of polymorphic ventricular arrhythmias, with about sixteen thousand premature ventricular complexes and a non–sustained ventricular tachycardia. Therefore, due to the patient’s high–risk for life–threatening arrhythmic events, metoprolol 25 mg o.d. was initiated and a wearable cardioverter defibrillator (WCD) with remote monitoring was provided during pregnancy and labour. After 3 weeks a caesarean delivery in epidural analgesia was performed and managed by a pregnancy heart team including a cardiologist, gynaecologist, obstetrician, and anaesthetist in close cardiac monitoring. No maternal or infant complications occurred, without arrhythmic episodes detected by the device. The WCD will be maintained also in the post–partum period with a narrow follow–up of the patient.

Discussion

In pregnant high–risk LQTS patients, beta–blockers are the first line therapeutic option, but they do not able to interrupt lethal arrythmias in out–of–hospital setting. The implantable cardiac defibrillator is not the best choice, due to the concerns about radiation exposure and anaesthesia during pregnancy. Moreover, lead–associated thrombosis and infections during pregnancy were reported in literature. The WCD is the ideal option in these cases. It is an external device suited for transient higher arrhythmic risk periods. Moreover, it allows a remote monitoring of arrhythmic burden (figure 2) which may help clinicians in guiding the follow–up and the therapeutic management of the patient. One drawback is the need of continuous patient’s compliance. Finally, a pregnancy heart team is mandatory in presence of these rare cardiac syndromes.
C76 FINDING OF MYOCARDIAL CRYPTS IN SUDDEN CARDIAC ARREST PATIENTS: TWO CASE REPORT
Authors: Caraffini A , Bricoli S , Canu E , De Santis N , Pelizzoni V , Binno S , Vermi A , Di Spigno F , Perrino F , Ferraro S , Aschieri D
Published: May 2023

Left ventricular myocardial crypts are definined as deep blood–filled invaginations trasversing > 50% of the myocardial wall and are located predominantly at the mid–basal segment of inferoseptal wall or at the apical segment. To date the incidence of crypts is 9.1% of patients undergoing cardiac CT and are not associated with intermediate–term major adverse cardiovascular events. However several studies have suggested that the presence of crypts is a marker of myocardial disease that should warrant clinical follow–up prior to the potential development of left ventricular hypertrophy and occurrence of serious cardiovascular events, such as heart failure or death. A lot of publications demonstrate that ventricular crypts could be a pre–phenotypic marker of hypertrophic cardiomyopathy (HCM). HCM is a genetic ereditary cardiomyopathy which is associated with fatal ventricular arrhytmias. We present the case of two patients who had a sudden cardiac arrest resuscitated with cardiac defibrillation. These are two males, 59 and 53 years old respectively, without cardiovascular risk factor and with familiarity for HCM. The first patient had a sudden cardiac arrest during exercise and was resuscitated with early cardiac defibrillation. The second patient had ventricular fibrillation after otolaryngology surgery treated with single defibrillator shock. Coronary arteries were free from atherosclerotic lesions at the angiografy study and no other reversible causes of cardiac arrest were identified. CMR showed normal biventricular funtion and ruled out signs of myocardial oedema or fibrosis. The only morphological finding was the detection of single left ventricular myocardial crypt at the CMR study in both patients: in one patient the crypt was located at the inferior wall while in the other at the level of the septum in the mid–apical segment. Ultimately both patients underwent genetic testing for cardiomyopathy. These findings may be a pre–phenotypic markers of HCM in patients who don’t present a clear expression of the pathology but may be a correlation with sudden cardiac arrest. The rarity of their finding in patient who have had sudden cardiac arrest can be explained by the small survival rate in patients with sudden cardiac arrest. Future studies and the increasingly simple access to advanced imaging methods like CMR and CT will allow us to clarify the role of these myocardial anomalies in the risk of developing fatal arrhythmic events.
C80 GENETIC AND NON–GENETIC DETERMINANTS OF CLINICAL VARIABILITY IN A FAMILIAL CASE OF HYPERTROPHIC CARDIOMYOPATHY WITH HETEROZYGOUS PATHOGENIC MYPBC3 MUTATION
Authors: Moscardelli S , Santangelo G , Rusconi D , Bursi F , Micaglio E , Guazzi M
Published: May 2023

Mutations in the MYBPC3 gene, which encodes myosin C–binding protein C (cMyBP–C) are among the main causes of hypertrophic cardiomyopathy (HCM). Truncating mutations are recognized as causative in many HCM patients and recently, even missense mutations (e.g E258K mutation) appear to be clinically related to the disease. E258K is one of the most common MYBPC3 mutations, with a prevalence of 15% in Italians. We report a family case, with father and son affected by HCM in which where this mutation was identified thanks to the NGS. HCM in the father was diagnosed at the age of 70 after several episodes of atrial fibrillation and after a myocardial infarction. The son, a sportsman, had a very early and severe presentation of HCM in the absence of manifest clinical signs. The same E258K mutation was also found in the proband son, in the absence of clinical signs of HCM. Given the diversity of the carrier phenotypes, the molecular analysis of the proband was extended beyond rare variants and we found a common substitution in the DES gene, (encoding desmin) which, acting as an unfavourable modifier, could partly explain the different clinical presentation of HCM. Probably also the intense physical activity carried out by the proband contributed to the phenotype severity. The father, asymptomatic for a much longer period and with a lower hypertrophy grade compared to the proband, may have benefited from a less intense physical activity and from the absence of the DES polymorphism. To date, the role of physical exercise is widely debated: on the one hand, a regular intense physical training could worsen hypertrophy, fibrosis and trigger arrhythmias, on the other hand however, a sedentary lifestyle predisposes to obesity and other cardiovascular risk factors which induce diseases with a worse prognosis than HCM itself. Nowadays 70% of patients with HCM are overweight, and more than 50% are below the minimum recommended exercise threshold. In addition, there is growing evidence that competitive sports are safe and could have beneficial effects in asymptomatic low–risk HCM patients. This case study suggests that the decision–making process must include both genotypic and phenotypic characterization of patients, as well as the evaluation of the causative mutation pathogenicity and of co–existing genetic modifiers.
C82 CARDIOMYOPATHIES AND GENETICS: ACTIVATION OF A SHARED PATH. INSIGHTS FROM CUNEO HOSPITAL
Authors: Coppini L , Coletta G , Rossini R
Published: May 2023

Introduction

In the last few years, genetic investigation has become an active part of the diagnostic and therapeutic pathway of cardiological patients with cardiomyopathies. Genetic investigation assumes a key role in the clinical–decision–making process of the proband acquiring a potential role in changing clinical action. Patients referred for genetic screening are affected by hypertrophic, arrhythmogenic and dilated cardiomyopathy. In the dilated form it is now a well–established notion that some mutations are associated with increased arrhythmic risk, regardless of the ejection fraction. This link was implemented by the 2022 ESC guidelines on ventricular arrhythmias, these patients can in fact benefit from a more aggressive therapeutic approach for the purpose of preventing sudden death.

Methods and results

In 2022 a diagnostic process was activated in collaboration with the genetics laboratory. Twenty–two patients underwent genetic examination and genetic testing. Of these, 61% were patients with hypertrophic cardiomyopathy, 22% with dilated cardiomyopathy, 11% with arrhythmogenic cardiomyopathy and 6% with Fabry disease. Two HCM patients have mutations in the MYBPC3 gene. A patient with Fabry has an exonic mutation in the GLA gene associated with the late onset phenotype. Two patients have a negative NGS panel and one patient has a mutation of uncertain significance (VUS) in TTN. In the remaining genetic analysis is ongoing.

Conclusions

We believe, as clinical cardiologist who deal with the field of cardiomyopathies, in the importance to acquire an integrated vision where genetic diagnosis is added alongside the traditional tools of cardiological expertise, as a risk stratification tool and as an additional element of the decision–making process.
C87 IMPACT OF THE COVID 19 PANDEMIC ON ADHERENCE TO PATIENT CARE PATHWAYS AFTER ACUTE CORONARY SYNDROME WITH ST ELEVATION (STEMI) IN FRIULI–VENEZIA–GIULIA (FVG). REGIONAL DIAGNOSTIC THERAPEUTIC PLANS INDICATORS
Authors: Leiballi E , Di Chiara A , Werren M , Riccio A , Valente M , Pavan D
Published: May 2023

Background

In recent years, the treatment of STEMI has made significant progress which has led to an increase in survival and age of patients who have become chronic heart disease. Management is a major problem especially in the post–acute and chronic phases, previously considered stable. Post–hospital morbidity and mortality are increased particularly in patients with high residual risk.

Methods

The purpose of this work is to evaluate adherence to patient care pathways after STEMI in FVG from 2018 to 2021. This evaluation must take into account the restrictive measures adopted since March 2020 to contain the COVID 19 pandemic. The data was collected by the Regional Health Agency (ARCS) thanks to the launch of the Heart Network and the regional Diagnostic Therapeutic Plans (PDTA). The indicators of care pathways were: cholesterol–lowering prescription at 30 days; antiplatelet prescription after 30 days; beta–blocker prescription after 30 days; cardiological visit 2 months after discharge; persistence of statin therapy 1 year after discharge; LDL dosage 1 year after discharge; target LDL 1 year after discharge; nursing counseling 1 year after discharge.

Results

Patients with STEMI in FVG in 2018 were 830, in 2019 were 834, in 2020 were 772 and in 2021 were 782. The populations do not differ significantly in terms of age and gender (with a predominance of males). Observing the data on the management of patients in the post–acute phase there are no significant differences between the populations. However, if we analyze the data indicative of the management of patients in the chronic phase, the results differ in the populations. The persistence of statin therapy at 1 year is 82.8% in 2018 and it is 80.4% in 2019 while it is 69.7% in 2020 and 62.2% in 2021. Nursing counseling at 1 year is 60.8% in 2018, 62.5% in2019 while it is 37% in 2020 and 51.9% in 2021.

Conclusions

The COVID 19 pandemic has impacted the post–STEMI patient care process in FVG not so much in the post–acute phase but in the chronic phase. According to international guidelines, Cardiology Rehabilitation (CR) is strongly recommended in STEMI. No treatment in heart disease has stronger scientific evidence or a significantly greater impact on survival. Despite this, the CR facilities in FVG have not been strengthened in recent years, on the contrary they have suffered more significantly from the COVID–related restrictions and the depletion of resources.
C89 CARDIOLOGICAL HYPERTENSIVE EMERGENCIES: REAL WORD DATA COMPARED TO GUIDELINES INDICATIONS
Authors: Maloberti A , Andrian E , Leidi F , Massimiliano M , Galasso M , Colombo V , Giannattasio C
Published: May 2023

Background

The 2018 ESH guidelines have revised the therapeutic goals of cardiological Hypertensive Emergencies (HE) with an indication for a more intensive (target < 140/90 mmHg) and rapid (immediate) Blood Pressure (BP) reduction. Cardiac acute organ damage during HE includes acute myocardial infarction, pulmonary edema, unstable angina pectoris and aortic dissection. However, how much these indications have been applied in clinical practice to date it’s unknown.

Aims

The first purpose of our study is to analyze the prevalence and clinical characteristics of cardiological HE in our institution. The second purpose is to compare the year before the release of the 2018 guidelines (2017) with the subsequent years (2019) trying to verify adherence to guidelines.

Methods

This is a single–center retrospective study conducted at the Niguarda Hospital. All patients aged ≥ 18 years with Systolic BP≥ 180 mmHg and/or a Diastolic BP ≥ 120 mmHg with Cardiological Emergency were enrolled. From the Emergency Department (ED) data clinical, anamnestic, blood pressure, symptoms, drug treatment and target achievement were registered.

Results

Patients with BP > 180/120 mmHg in 2017 were 706 out of a total of 73795 accesses (0.96%) and 601 over 67273 (0.89%) in 2019. 246 (34.84%) in 2017 were HE of which 144 (58.53%) were cardiological: aortic dissection 1 (0.69%), acute coronary syndrome 52 (36.11%), acute pulmonary edema 35 (24.30%), cardiac decompensation 91 (63.19%). During 2019 similar figures were founded with 286 (47.58%) HE of which 286 (47.58%) were cardiological: aortic dissection 2 (1.43%), acute coronary syndrome 43 (30.93%), acute pulmonary edema 20 (14.39%), cardiac decompensation 76 (54.68%). The reduction in BP obtained in ED was significantly greater in 2017 than in 2019 (44.7±31.4 vs 35.4±24.5 mmHg, p = 0.011) with a lower target reaching in 2019 (28.9 vs 51.4%, p<0.001). Pulmonary edema is the cardiological HE on which a greater pressure reduction is obtained and therefore in which the target set by the guidelines is more frequently reached.

Conclusions

The recommendation for a more intense and rapid BP reduction in cardiological HE seems to be not accepted from ED clinicians that persist to reduce BP accordingly to previous guidelines.