bluuebunny/crossref_metadata_2025 huggingface dataset:
110 ANEURYSMECTOMY AND SURGICAL ABLATION: WHEN?
Authors: Rizzi Jacopo Giulio , Savonitto Giulio , Franzese Ilaria , Ruggiero Danilo , Rauber Elisabetta , Zecchin Massimo , De Luca Antonio , Mazzaro Enzo , Sinagra Gianfranco
Published: December 2022

A 60-year-old man with a previous myocardial infarction was admitted to our ICU for acute pulmonary edema in the context of an acute antero-lateral non-ST elevation myocardial infarction. Echocardiography showed severe left ventricular (LV) dilatation with extensive wall motion abnormalities, large apical aneurysm and severe systolic dysfunction. Coronary angiography showed a triple-vessel disease with proximal occlusion of the left anterior descending artery.

During the hospitalization, multiple episodes of drug-resistant monomorphic ventricular tachycardias occurred, requiring repeated DC Shock.

After Heart Team discussion, the patient underwent coronary artery bypass grafting, LV restoration according to the procedure described by Guilmet and surgical cryoablation.

The Guilmet septoexclusion is indicated when the interventricular septum is more involved than the free wall. After aneurysm incision, cryolesions were applied at the septum and at the transitional zone of the scar and viable tissue. Thus, the anterior free wall was sewn obliquely to the septum. Finally, the edges of the incision, anterior and septal, were sewn together to assure a definitive hemostasis (overcoat technique).

After surgery, an implantable cardioverter-defibrillator was implanted in secondary prevention.

The postoperative course and subsequent cardiological follow-up were characterized by a gradual clinical improvement with mild increasing in LV function and reduction in ventricular arrhythmias.

Nowadays, combined aneurysmectomy and endocardial ablation are rarely performed, but should be considered in patients with LV aneurysm who manifest drug-resistant ventricular arrhythmias. Encircling cryoablation in a remodelled ventricle is safe and effective in reducing ventricular arrhythmias which are a negative prognostic factor.
78 FOUR YEARS ECHOCARDIOGRAPHIC FOLLOW-UP OF A LEFT VENTRICULAR PSEUDOANEURYSM
Authors: Passaniti Giulia , Deste Wanda , Cannizzaro Maria Teresa , Tosto Giuseppe Giovanni , Tamburino Corrado
Published: December 2022

Introduction

Left ventricular pseudoaneurysm is a rare and often fatal complication of myocardial infarction. It has an incidence of 0,2-0,4% of all the STEMI and a very high mortality, particularly in the first month. Symptoms can vary from shortness of breath on exertion, orthopnea, chest pain, fatigue. At least 10% of patients do not present any symptoms and the pseudoaneurysm is diagnosed incidentally during a follow-up appointment.

Signs include persistent ST elevation on EKG and its diagnosis can easily be made with a transthoracic echocardiogram and more in depth with a CT scan and a cardiac MRI. Its treatment is surgical, but the choice is up to the anesthesiologist and cardiothoracic surgeon, depending on patient's comorbidity and surgery risk. Surgical treatment has a high rate of death, reaching even the 37%.

Background

We present the case of a 74 years old patient with an history of chronic hypertension and cerebral vasculopathy, who sustained a STEMI in 2017. Urgent coronarography study was performed, but unfortunately the angioplasty treatment was unsuccessful and the left anterior descending artery could not be recanalized. Therefore, the patient was transferred to the cardiothoracic department in order to undergo bypass surgery: surgery was performed five days after, using left internal mammary artery to the left anterior descending artery and great saphenous vein to the first diagonal. Surgery went well with no complications; the post-operative period passed by without any complications and, after 15 days, patient was discharged with no complains.

Two months later, he was admitted to intensive care unit of a hospital of our town, because of dyspnea (NYHA 3), peripheral edema, moderate-severe mitral insufficiency, moderate pleural and pericardial effusion. He underwent a CT-scan that showed “9×6,4 cm cavity with blood that seemed to have a connection with the postero-lateral wall of the left ventricle”. He also underwent a cardiac MRI that displayed a pseudoaneurysm with an endoluminal thrombus. After an accurate evaluation of the case, the Heart Team decided to do not perform surgery because of the high risk of the patient. Thence, we have been followed him with close echocardiographic exams for more than four years.

Methods

From March 2018 to September 2022, we have been followed the patient with complete echocardiographic follow-ups, done every three-six month.

Results and conclusions

Patient with left ventricle pseudoaneurysm due to an extensive myocardial infarction has been followed in our echo lab for more than 4 years. He has been stable during these years, NYHA II, in a good clinical and hemodynamical state.
855 PREDICTORS OF LATE GADOLINIUM ENHANCEMENT DEVELOPMENT AND EXTENSION IN MYOCARDIAL INFARCTION WITH NON-OBSTRUCTIVE CORONARY ARTERIES
Authors: Amicone Sara , Bergamaschi Luca , Armillotta Matteo , Sansonetti Angelo , Stefanizzi Andrea , Impellizzeri Andrea , Suma Nicole , Canton Lisa , Fedele Damiano , Bodega Francesca , Tattilo Francesco Pio , Angeli Francesco , Rinaldi Andrea , Paolisso Pasquale , Foa´ Alberto , Casella Gianni , Galie´ Nazzareno , Pizzi Carmine
Published: December 2022

Background

Myocardial infarction with non-obstructive coronary arteries (MINOCA) is a heterogeneous clinical entity and in its complex diagnostic approach cardiovascular magnetic resonance (CMR) plays a pivotal role.

Purpose

To characterize the differences of MINOCA patients with and without late gadolinium enhancement (LGE) at CMR and to identify the predictors for ischemic LGE development and extension.

Methods

We assessed 461 MINOCA cases from January 2016 to June 2021. MINOCA were defined according to the current European guidelines criteria. We excluded acute myocarditis, Tako-tsubo syndromes, cardiomyopathies, or non-pathological CMR. According to CMR imaging findings, our cohort was divided into two CMR phenotypes based on regional myocardial necrosis detected throughout LGE (“LGE-positive MINOCA”) or regional ischemic injury without LGE (“LGE-negative MINOCA”). Extended LGE was considered as the presence of >2 segments with transmural LGE. Multivariate logistic regression analysis was used to determine the predictors of LGE and extended LGE.

Results

The final cohort included 175 MINOCA: 121 (69.1%) constituted the LGE-positive group. The mean time delay between acute clinical presentation and CMR was 6 ± 2.9 days. At admission, MINOCA LGE-patients more frequently presented angina and ST segment elevation (24% vs 7.4%, p = 0.01), compared to the LGE negative ones. Furthermore, the LGE positive group had a significantly greater infarct size, measured by peak hs-Troponin I values and left ventricular function. The only predictor of LGE was the peak troponin value (OR 1.64, 95% CI 1.18–2.28, p = 0.003), while predictors of extended LGE were ST-segment elevation at admission (OR 7.44, 95% CI 1.57–35.22, p = 0.01), peak troponin values (OR 1.07, 95% CI 1.02–1.13, p = 0.01) and the presence of non-obstructive coronary artery disease at coronary angiography (OR 5.49, 95% CI 1.20–25.09, p = 0.028).

Conclusion

The presence and extension of LGE at early CMR evaluation is an important feature in the setting of MINOCA. In addition, simple baseline characteristics (such as ST elevation, peak troponin value and LVEF) may aid the identification of a greater ischemic necrosis burden at CMR and therefore these high-risk MINOCA subjects could be benefit from a stricter management effort.
1025 PROGNOSTIC IMPACT OF STATINS AND DUAL ANTIPLATELET THERAPY ON LONG-TERM PROGNOSIS IN MINOCA PATIENTS
Authors: Armillotta Matteo , Sansonetti Angelo , Bergamaschi Luca , Stefanizzi Andrea , Angeli Francesco , Fabrizio Michele , Amicone Sara , Impellizzeri Andrea , Suma Nicole , Canton Lisa , Bodega Francesca , Fedele Damiano , Tattilo Francesco Pio , Rinaldi Andrea , Paolisso Pasquale , Foà Alberto , Casella Gianni , Galiè Nazzareno , Pizzi Carmine
Published: December 2022

Background

Myocardial Infarction with Non-Obstructive Coronary Artery disease (MINOCA) is a heterogeneous entity with relevant long-term major adverse cardiovascular events (MACE). There is solid evidence that secondary prevention strategies improve prognosis of patients with obstructive myocardial infarction. However, evidence-based treatments for MINOCA are lacking as no published randomized clinical trials have ever exclusively enrolled this population. In fact, treatment recommendations in current guidelines are mainly based on expert opinions and MINOCA patients are frequently discharged with statins and dual antiplatelet therapy (DAPT).

Purpose

To evaluate the effects of statins and DAPT as secondary prevention treatments on long-term outcomes in MINOCA patients.

Methods

We enrolled all consecutive MINOCA patients admitted to our Centre from 2016 to 2021. The diagnosis of MINOCA was made according to the current European Society of Cardiology diagnostic criteria (angiographic conventional cut-off of < 50% coronary stenosis without a clinically apparent alternative diagnosis). Second-level diagnostic work-up including cardiac magnetic resonance was performed to exclude non-ischemic troponin elevation cause.

All-cause mortality and MACE (a composite of all-cause mortality, hospitalization for heart failure, myocardial re-infarction and stroke) were collected during follow-up. The prognostic impact of statins and DAPT at discharge was assessed. The relationship between treatments and outcomes was evaluated by using Kaplan-Meier survival analysis.

Results

278 MINOCA patients were enrolled, of whom 203 (73%) were discharged on statins and 123 (44.2%) on DAPT. After a median follow-up of 36 ± 14.8 months, the overall all-cause mortality was 11.8% and the composite endpoint (MACE) was achieved in 28.4% of the entire population. Kaplan-Meier curves showed that patients treated with statins had a significantly lower rate of all-cause mortality (9.3% vs 18.2%, p=0.04) and MACE (24.6% vs 39.2%, p=0.02). On the other hand, rates of death (9.8% vs 13.2%, p=0.4) and MACE (23.6% vs 31.6%, p=0.1) were similar in MINOCA patients treated with DAPT or single antiplatelet therapy.

Conclusions

Among MINOCA patients, DAPT at discharge neither reduced long-term all-cause mortality nor MACE. In contrast, statin treatment provided beneficial effects on long-term outcomes. These results should be considered preliminary and require confirmation from randomized clinical trials.
990 IMMUNE-MEDIATED NECROTIZING MYOPATHY IN A PATIENT WITH MULTI-VESSEL CORONARY ARTERY DISEASE
Authors: Benvenuto Ciro Pollio , Liuzzo Giovanna
Published: December 2022

Inflammatory muscle diseases (often referred to as “inflammatory myopathies” or “myositis”) are a group of muscle diseases characterized by chronic muscle inflammation, inflammation of tissues associated with the muscles, muscle weakness and occasionally muscle pain (depending on the specific case).

Ischemic heart disease is the leading cause of cardiovascular mortality in Western countries; the underlying cause is coronary atherosclerosis, a multifactorial disease determined by the concomitant presence of classic cardiovascular risk factors and inflammation of the vessel wall.

We present the case of a 60 y/o man who previously underwent CABG (LIMA for ADA e GSV for OM1) for multi-vessel CAD and then developed immune-mediated necrotizing myopathy (probably due to statin treatment) with muscle weakness and pain and with elevation of myolysis markers: in this context, he also presented a recurrence of chest pain with troponin elevation, so that it was needed the integration of functional imaging from myocardial scintigraphy and from cardiac magnetic resonance to understand that troponin elevation (and the chest pain) were due to ischemia and not to muscle damage. This case report underlines how a critical approach to imaging and a deep knowledge of pathophysiology is mandatory in cardiovascular medicine.
403 ECG EXERCISE STRESS TEST PREDICTORS OF LEFT MAIN CORONARY ARTERY DISEASE
Authors: Tremamunno Saverio , Yuksek Ayda Naz , Telesca Alessandro , Cambise Nello , Tinti Lorenzo , Di Renzo Antonio , Belmusto Antonietta , De Vita Antonio , Crea Filippo , Lanza Gaetano Antonio
Published: December 2022

Background

Left main coronary artery disease (LMCAD) heavily affects prognosis of patients with suspected CAD. Thus, its identification/exclusion is an important step in the assessment of these patients. Although being the gold standard to identify LMCAD, invasive coronary angiography (ICA) is burdened by some risks, while coronary computed tomography angiography (CCTA) has still limited availability, quite high costs and associated radiologic risks.

In the past decades, several studies demonstrated the utility of ECG exercise stress test (EST) for the identification of LM disease in patients with suspected CAD. However, the pre-test probability of CAD of subjects undergoing EST has significantly changed in the last decades.

Accordingly, in this study we aimed to assess the predictive value of EST for the presence/absence of LMCAD in a contemporary population of patients with suspected CAD.

Methods

We retrospectively enrolled 495 consecutive patients, referred to our Center between years 2018 and 2021 because of suspected CAD, who underwent both an EST (standard treadmill Bruce protocol) and ICA (within 12 months of the EST). Patients with a history of coronary artery bypass surgery were excluded.

Results

Overall, 24 patients (4.8%) were found to have LMCAD at ICA. Among clinical variables, only male gender (p=0.025) and smoking (p=0.003) were associated with LMCAD. A number of ECG leads with EST-induced ST-segment depression (STD) ≥5 and a maximal STD ≥2 mm were more frequently found in patients with, compared to those without LMCAD (29.2 vs 9.8%, p=0.003; and 58.3 vs 24.8%, p<0.001, respectively). No other EST variable was associated with LMCAD, including ST-segment elevation in lead aVR (4.2 vs. 2.6% in the 2 groups, respectively, p=0.63). On the other hand, among the 206 patients (41.6% of the population) who achieved 85% of maximal heart rate predicted for age and had ST-segment depression < 2mm, LMCAD was present in 1.9% only.

Conclusions

Our data indicate that EST continues to be a valuable tool for predicting the presence/absence of LMCAD in contemporary populations of patients with suspected coronary artery disease.
758 CASE REPORT OF A CATHETER-RELATED THROMBOSIS IN THE RIGHT HEART
Authors: Pezzini Sara , Daus Francesca , Lobiati Elisabetta , Giannattasio Cristina , Savonitto Stefano
Published: December 2022

Introduction

Central venous catheters (CVCs) are commonly used in clinical practice. Their use may be associated to complications like catheter-related thrombosis, that may not only result in vascular and catheter occlusion but also infection, pulmonary embolism, and formation of right heart thromboemboli. Patients who develop catheter-related thrombosis have an increased risk of mortality due to the potential risk of embolization to the pulmonary vasculature.

Case presentation

We describe the case of a 76-year-old man with multiple cardiovascular risk factors, admitted to the Nephrology Department for a suspected UTI. The patient develops a central venous catheter-related thrombosis on the CVC placed in the right jugular vein. The finding of the thrombus was accidental and happened during a transthoracic echocardiography performed to exclude endocarditis. The thrombus was successfully treated with LMWH infusion, preventing complications like pulmonary embolism.

Conclusion

Although generally considered safe, central venous catheters can be associated with complications such as catheter-related venous thrombosis. Intensivists who use these devices should be aware of this possible complication and may use strategies to prevent them in order to improve patient outcome. The present case highlights the importance of maintaining a high index of suspicion for thromboembolic complications in patients with CVCs.
20 LEAD-RELATED SUPERIOR VENA CAVA SYNDROME IN A PACEMAKER-DEPENDENT PATIENT: A HYBRID PROCEDURE
Authors: Pittorru Raimondo , Migliore Federico , Arturi Federico , Tona Francesco , Tarzia Vincenzo , Iliceto Sabino , Gerosa Gino
Published: December 2022

We report a case of a 77-years-old man admitted to our department because of facial plethora, prominent venous collateral over the anterior chest wall, marked swelling of the head and upper extremities. His medical history was notable diabetes, non-Hodgkin lymphoma (NHL), eradicated HCV, AV block, for which he underwent placement of a dual-chamber pacemaker 4 years earlier. A superior vena cava (SVC) syndrome lead-related was documented on angio-CT scan and phlebography. After a multidisciplinary team discussion, a treatment strategy was designed to consist of transvenous lead extraction, SVC reconstruction and epicardial pacing placement. At 5 months follow-up, he was completely relieved of symptoms.

Conclusion

Lead-associated SVC syndrome is a rare condition and there is no standard management protocol. Data are lacking and we need at least a registry to make the treatment homogeneous. In this specific patient, a hybrid procedure based on lead extraction and reconstruction of superior vena cava was safe and effective for the relief of symptoms and maintenance of SVC patency. Heparin alone will not lead to the resolution of the disease if the disease is at an advanced stage.

Learning outcomes

Patients with lead-induced superior vena cava syndrome often required a multidisciplinary approach. A hybrid procedure based on transvenous lead extraction and reconstruction of superior vena cava is safe and effective to achieve relief of symptoms, maintenance of SVC patency, and adequate pacing.
557 RAREFACTION OF MYOCARDIAL LYMPHATIC VESSELS IN PATIENTS WITH CARDIAC AMYLOIDOSIS
Authors: Aimo Alberto , Martinelli Giulia , Bonino Lucrezia , Musetti Veronica , Emdin Michele , Pucci Angela
Published: December 2022

Introduction

In cardiac amyloidosis (CA), amyloid fibers accumulate in the extracellular spaces of the heart. It is reasonable to speculate that this accumulation could lead to a compression and obliteration of lymphatic vessels, and possibly to their eventual disappearance. As lympathic drainage removes proteins from the interstitial spaces, including amyloid precursors, an impairment of lympathic vessels could exacerbate amyloid accumulation, thus triggering a vicious circle.

Methods

We evaluated consecutive endomyocardial biopsies (EMB) performed as part of the standard diagnostic workup for CA or for unexplained left ventricular (LV) hypertrophy, with no specific suspicion of CA. All EMBs were performed in the septal region of the LV. Three samples were analyzed through Congo red staining and immunohistochemistry, with final confirmation of the amyloid subtype through a proteomic approach. Lymphatic vessels were visualized through antibodies targeting the specific marker podoplanin. Echocardiographic variables measured at the time of the EMB were collected.

Results

We examined 15 patients with CA (ATTR-CA, n=10; AL-CA, n=5; 50% men, median age 75 years [interquartile range 71-78]), and 13 patients with CA excluded (55% men, median age 68 years [59-71]). The median density of lymphatic vessels in patients with CA was 0.52 vessels/mm2 (0-3.32), and 10.90 vessels/mm2 (3.10-26.25) in patients without CA (p=0.001). No significant differences were found in LV mass index (p=0.170), interventricular septal thickness (p=0.262), relative wall thickness (p=0.078), or LV ejection fraction (p=0.262), while posterior wall thickness was higher in patients with CA (p=0.042). Three patients with ATTR-CA and 2 patients with AL-CA had no lymphatic vessels on their tissue samples. Patients with ATTR- or AL-CA did not display significant differences in the density of lymphatic vessels, or any metric of increased LV wall thickness or systolic function.

Conclusions

We report for the first time that patients with CA have significantly less myocardial lymphatic vessels than patients without CA, despite no significant differences in the degree of LV wall thickening or LV ejection fraction. These results support the hypothesis that the lymphatic drainage is deranged in patients with CA, possibly promoting further amyloid deposition.
957 EMERGING FROM THE DARKNESS. SUDDEN CARDIAC DEATH IN CARDIAC AMYLOIDOSIS
Authors: Antonelli Giorgio , Cammalleri Valeria , De Luca Valeria Maria , Annibali Ombretta , Nusca Annunziata , Mega Simona , Carpenito Myriam , Ricciardi Danilo , Guerrieri Fiorella , Avvisati Giuseppe , Ussia Gian Paolo , Grigioni Francesco
Published: December 2022

Cardiac amyloidosis (CA) manifests as infiltrative cardiomyopathy with a hypertrophic pattern, usually presenting with heart failure with preserved ejection fraction (HFpEF). In addition, degenerative valvular heart disease, particularly severe aortic stenosis, is commonly seen in patients with CA. However, amyloid fibril deposition may also infiltrate the conduction system and promote the development of electrical disorders, including ventricular tachyarrhythmias (VT), atrio-ventricular block, or acute electromechanical dissociation (EMD). These manifestations can increase the risk of sudden cardiac death (SCD). This review summarizes the pathophysiological mechanisms and risk factors for sudden cardiac death in cardiac amyloidosis and focuses on the major current concerns regarding medical and device management in this challenging scenario.

Heart involvement is the major determinant of survival in patient with amyloidosis and a “sudden death” occurs in approximately two-thirds of patients with cardiac amyloidosis. Moreover, the AL amyloid fibrils are shown to be highly cytotoxic to the ventricular myocardial, explaining why ventricular arrhythmias appear more frequently in AL over ATTR. Proposed mechanisms driving electrophysiological manifestations of CA involve intramural coronaries, microvascular ischemia, or patchy myocardium infiltration of amyloid fibrils, causing the development of anatomical re-entrant circuits responsible for ventricular arrhythmia. The prevalence of non-sustained ventricular tachycardia (NSVT) in AL amyloidosis ranges from 5 to 27% with routine monitoring and 100% during the stem cell transplant period. The guidelines don't provide specific indications regarding insertion of implantabile cardioverter defibrillator (ICD) and they don't clarify whether some patients subtypes can benefit from them.

Amyloid infiltration into the conduction system enhances the genesis of rhythm disturbances, including fatal ventricular arrhythmias and SCD. Current pharmacological anti-arrhythmic therapies are poorly tolerated in CA, and there are no robust recommendations on the management of ventricular arrhythmias in this subset of patients. Furthermore, the benefit of ICD implantation is highly variable according to the different clinical stages of the disease. Therefore, further studies are needed to create a standardized diagnostic algorithm and appropriate treatment strategy for this special population.
620 A RARE CASE OF HYPERTROPHIC CARDIOMYOPATHY
Authors: Bandini Marta , Carrassa Gianmarco , Di Mario Carlo
Published: December 2022

Background

In patients presenting with hypertrophic cardiomyopathy (HCM), the differential diagnosis of “non-sarcomeric” versus “sarcomeric” HCM forms is crucial because of their differences in prognosis and treatment strategies.

Case presentation

We present the case of a 31 years-old male with unexplained syncope, occurring abruptly. His father died of sudden cardiac death (SCD) and his paternal aunt was known for HCM. The electrocardiogram showed left ventricular (LV) hypertrophy and signs of ventricular pre-excitation; the echocardiogram showed marked and asymmetric hypertrophy with normal systolic function. Cardiac magnetic resonance finally confirmed the suspicion of HCM with marked septal fibrosis. In the setting of autosomal dominant HCM coexisting with Wolf-Parkinson-White syndrome, one of the most significant genetic syndrome is PRKAG2-syndrome (PS).

Conclusion

PS represents a rare phenocopy of the classic form of HCM distinguished by ventricular pre-excitation and often accompanied by advanced heart blocks, due to the marked septal fibrosis involving the conduction system. In the presented case, the diagnosis has been then confirmed by genetic testing. What would be the best therapeutic approach in the prevention of SCD? Can subcutaneous defibrillator implantation (S-ICD) be sufficient?
1064 LONG QTC IN HCM: A CONSEQUENCE OF MYOCARDIAL HYPERTROPHY OR A DISTINCT GENETIC DISEASE?
Authors: Cava Francesco , Micolonghi Caterina , Savio Camilla , Musumeci Maria Beatrice , Melato Giacomo Tini , Petrucci Simona , Alesi L , Germani Aldo , Piane Maria , Autore Camillo , Rubattu Speranza Donatella
Published: December 2022

Hypertrophic cardiomyopathy (HCM) is an autosomal dominant condition, characterized by the presence of unexplained left ventricular hypertrophy. This condition is usually associated with electrocardiographic abnormalities including QTc prolongation reported in the 13% of HCM patients. The main explanation for QTc prolongation in HCM is myocardial hypertrophy and the related structural damage. However, mechanisms other than myocardial hypertrophy, including long QT syndrome (LQTS) gene mutations, may also be involved. The addictive effect of 2 disease-causing mutations has been already reported in other pathological conditions. In the present study we investigated the possible co-inheritance of pathogenic variants associated with both LQTS and HCM, exploring the hypothesis of a distinct genetic basis underlying QTc prolongation in HCM. For this purpose, we considered a cohort of 150 HCM patients carrying pathogenic variants in sarcomeric genes. Out of them we selected 25 patients carrying a QTc prolongation not explained by any other cause (drugs, etc). The QTc was considered prolonged if greater than 450 ms in males and greater than 470 ms in females. NGS analysis was performed with Illumina TrueSight Cardio panel genes on Illumina MiniSeq platform to evaluate the presence of pathogenetic variants in LQTS related genes. We identified pathogenic/likely pathogenic variants in LQTS related gene KCNQ1 (c.1781G>A, p. Arg594Gln; c.532G>A, p. Ala178Thr) in two patients (8%) and uncertain significant variants in SCN5A, KCNJ5, AKAP9 and ANK2 in four patients (16%). Although the results of our study are limited by the small number of patients included in the analysis, they highlight a minor contribution of LQTS genes in HCM patients with a QTc prolongation. The screening for ion channel gene mutations should be considered in HCM patients with QTc prolongation, not explained by any other cause. This strategy may be important for risk stratification and treatment planning.
128 AN UNEXPECTED ENDING OF PREGNANCY
Authors: Coccalotto Marco , Cumitini Luca , Giubertoni Ailia , Rossi Lidia , Rosso Roberta , Patti Giuseppe
Published: December 2022

Introduction

Dilated cardiomyopathy (DCM) is a condition whose etiologies may be various. DCM with ventricular disfunction is a frequent complication of chemotherapy. Peripartum cardiomyopathy is characterized by a reduced ejection fraction (EF), presenting toward the end of pregnancy or in the first months after delivery. Lipodystrophy may be associated to dilated and hypertrophic cardiomyopathy.

Material and Methods

A 28-years-old pregnant woman in her late 33rd week was admitted with acute heart failure complicated by pulmonary edema. The patient had history of acute lymphoblastic leukemia during childhood treated with chemo and radiotherapy, followed by hematopoietic stem cell transplantation (HSCT). Afterwards she developed partial acquired lipodystrophy, causing diabetes mellitus, insulin resistance, dyslipidemia, leptin deficiency and steatohepatitis. A mild left ventricular disfunction had been reported before HSCT, with complete recovery at follow up.

After admission, the scenary rapidly deteriorated towards cardiogenic shock, so a caesarean section was performed. Transthoracic echocardiography revealed diffuse hypokinesia conditioning severe left ventricular (LV) dysfunction (EF 24%), moderate mitral and tricuspidal valve regurgitation and left to right shunt in possible atrial septal defect. A cycle of levosimendan was administrated, with subsequent improvement in LV function (EF 32%). Cardiac magnetic resonance (CMRI) showed hypokinetic DCM with no pathological findings in T1, T2, STIR sequences nor LGE. In parallel, empty sella in possible Sheehan Syndrome was hypothesized. A diagnosis of peripartum cardiomyopathy on the basis of a chemotherapy-induced cardiomyopathy in a good compensation status was made, with no evidence of lipodystrophy-related cardiac involvement. After recovery the patient was discharged with optimal cardiological therapy and a wearable cardioverter defibrillator. During follow up, the patient improved ventricular function (EF 40%) without any registered arrhythmia. She will be evaluated for heart transplantation.

Conclusions

Pregnancy in patients with cardiac disease can be life-threatening for mother and fetus, a multidisciplinary approach and a correct timing are crucial to achieve the best possible result. Left ventricular disfunction can be related to many etiologies, and instrumental tests such as echocardiogram and CMRI are crucial to evaluate the cause of the disfunction. Close follow up in patients who underwent chemo/radiotherapy or are affected by metabolic diseases may avoid long-term cardiac complications.
1072 A RARE CASE OF CONSTRICTIVE PERICARDITIS AFTER A STAB WOUND TO THE CHEST
Authors: Del Giudice Carmen , Cosimo Renato , Pezzullo Enrica , D´aquino Malvezzi Marco Caracciolo , Bussani Rossana , Sinagra Gianfranco , Golino Paolo , Loffredo Francesco
Published: December 2022

A 39 year old man presented to the emergency department with dyspnoea on exertion, chest pain and fatigue. Few years before, the patient was stabbed in the left chest thus developing Pneumothorax and mild pericardial effusion successfully treated with chest tube drainage and medical therapy. Physical examination revelead giugular vein distension and lower extremity edema. The echocardiographic evaluation showed typical signs of constrictive pericarditis which was confirmed by cardiac catheterization. Furthermore, because of clinical worsening and the absence of ongoing inflammation on PET-TC, the patient was referred to surgery. Pericardiectomy was successfully performed with rapid symptoms relief and clinical benefits. Histologic examination of pericardium showed the presence of thick fibrotic tissue with clusters of squamous epithelial cells with no cellular atypias or malignancy. Therefore, we hypothesize that following the chest stabbing wound, squamous and epitheloid cells have invaded the pericardial space producing a fibrotic reaction responsable of the development of the constriction. Furthermore multimodality imaging has been essential for diagnosis and management.
28 CONSTRICTIVE PERICARDITIS: A ROOM FOR MULTI-MODALITY IMAGING
Authors: Del Monaco Guido , Tumminello Fabio , Figliozzi Stefano , Pagliaro Beniamino , Fumero Andrea , Barbone Alessandro , Cuko Enea , Mantovani Riccardo , Fazzari Fabio , Cannata Francesco , Curzi Mirko , Catapano Federica , Monti Lorenzo , Francone Marco , Pini Daniela , Stefanini Giulio Giuseppe , Torracca Lucia , Condorelli Gianluigi , Bragato Renato Maria
Published: December 2022

Introduction

The diagnosis of constrictive pericarditis can be challenging through conventional imaging. Novel imaging techniques have been proposed to improve it.

Clinical Case

A 74-year-old man with a previous diagnosis of heart failure with preserved ejection fraction and permanent atrial fibrillation (AF) was admitted because of worsening dyspnea, peripheral edema, and ascites refractory to diuretics therapy. ECG revealed low QRS voltages and AF with normal heart rate and laboratory exams detected increased values of BNP and hepatic stasis indices; blood count and CRP were normal. Echocardiography revealed mildly reduced bi-ventricular pump function (left ventricular ejection fraction = 52%, right ventricular fractional area change = 25%) and slightly increased wall thickness. Atria were moderately dilated (left and right atrium 46 and 44 ml/mq, respectively) and mitral and tricuspid annuli were dilated too. There was inferior vena cava plethora, moderate atriogenic tricuspid regurgitation and mild-to-moderate atriogenic mitral regurgitation. A paradoxical interventricular septal motion and significant respiratory changes on trans-mitral (>25%) and trans-tricuspid (>30%) pulsed Doppler were noted; however, there was no "annulus reversus" (lateral and septal e’ 13 and 11 cm/sec, respectively) at TDI analysis. In contrast, 2D-speckle-tracking global longitudinal strain showed impaired deformation ​​of the lateral segments with relative sparing of the septal segments. The latter pattern has been recently described as "strain reversus" or "hot septum sign". The findings were suspected of constrictive pericarditis, but a chest Computed Tomography excluded pericardial calcifications. Cardiac magnetic resonance (CMR) was then performed revealing a "septal shift" at cine-real time sequences analysis. The pericardium was slightly thickened (4-5 mm) with widespread late gadolinium enhancement but without signs of acute inflammation at T2-weighted imaging. Tricuspid regurgitation was confirmed to be "moderate" after phase-contrast imaging. Cardiac catheterization finally revealed normal coronary arteries and confirmed the diagnosis of constrictive pericarditis (square root sign, equalization of bi-ventricular end-diastolic pressures). The patient underwent pericardiectomy and tricuspid and mitral valve repair and was asymptomatic at 6-months follow-up.

Conclusions

Constrictive pericarditis represents a potentially reversible cause of heart failure but can be easily missed through conventional imaging. In the present case, advanced echocardiography and CMR were essential to come up with an accurate diagnosis, guiding the patient's clinical management with excellent outcome.
938 ARRHYTHMOGENIC CARDIOMYOPATHY IN A PATIENT CARRYING A PATHOGENIC LMNA VARIANT
Authors: Bariani Riccardo , Deola Petra , Rigato Ilaria , Cipriani Alberto , Cason Marco , Pilichou Kalliopi , Bauce Barbara
Published: December 2022

Background

Lamin A (LMNA) protein is one of the main constituents of the nuclear lamina. Pathogenic variants of LMNA gene have been demonstrated to be linked to a wide spectrum of disorders, including cardiac diseases. The first reported heart disease linked to LMNA was dilated cardiomyopathy (DCM), in a form typically characterized by the presence of conduction disorders and high incidence of sudden cardiac death (SCD) disrespectfully of left ventricular systolic function. Genetic variants of the same gene have been associated with Arrhythmogenic Cardiomyopathy (ACM), but its putative role as disease causing gene is still debated.

Case summary

a man with positive familial history for DCM presented to the Emergency Department because of palpitations. The electrocardiogram showed first degree AV-block, complete right bundle branch block and low QRS voltages on limb leads. Cardiac magnetic resonance revealed severe dilatation of the right ventricle with wall motion abnormalities in keeping with ACM, associated with mild dilatation and moderate dysfunction of left ventricle. The genetic test identified a pathogenic variant in LMNA. Moreover, non-sustained ventricular tachycardias were present on the ECG Holter. According to international guidelines on SCD prevention, the patient underwent ICD implantation, with one appropriate ICD discharge on a sustained ventricular tachycardia episode after few months.

Discussion

LMNA pathogenic variants can lead to a broad spectrum of cardiac manifestations, including Arrhythmogenic Cardiomyopathy. A genetic test should be considered if there is a familial cardiomyopathy associated with conduction disorders, in order to achieve a proper risk stratification for sudden death.
242 PRIMARY CARDIAC LYMPHOMA: A CASE REPORT
Authors: Di Cesare Annamaria , Gaudenzi Eleonora , Gardi Andrea , Ruggero Rossella , Moriconi Veronica , Spighi Lorenzo , Testa Paola , Piovaccari Giancarlo , Campo Gianluca , Carigi Samuela
Published: December 2022

Introduction

Primary cardiac lymphomas in immunocompetent patients are an uncommon malignancy, accounting for 1.3% of primary cardiac tumors, while metastases secondaries to extracardiac lymphomas are more common. Mean age at diagnosis is 63 years old. They mainly involve the right side of the heart, particularly right atrium. Symptoms of primary cardiac lymphoma are non-specific. Cardiac lymphoma typically follows an aggressive clinical course and the prognosis is poor, with more than 60% of patients dying within 2 months of diagnosis. Predictors of worse outcome are immune status, left ventricular involvement, arrhythmias and the presence of an extracardiac disease.

Case report

48-year-old obese and hypertensive man, in therapy for OSAS. In February 2022 admission to the emergency department due to jugular pressure and mild exertion dyspnoea. Neck CT excluded inflammatory collections; echocardiography showed normal cavitary dimensions and biventricular systolic function, absence of major valvulopathies and circumferential pericardial effusion of n.d.d. with TD max 2.5 cm and partial collapse of the right atrium, for which started corticosteroid therapy. Chest CT showed mediastinal lymphadenopathy and persistence of pericardial effusion. In April, due to worsening dyspnoea, cardiac CT demonstated a solid mass at the passage from the superior vena cava to the right atrium (50×37×67 mm), leading to compression-infiltration phenomena on the superior vena cava, and thrombosis of the right mammary vein. Cardiac MR confirmed a voluminous expansive-infiltrative lesion, with an irregular lobular profile, projecting into the superior vena cava and extended to the right atrium. This mass appears very iperintensive in T2 stir sequences with an early contrast impregnation and uneven intralesional late enhancement (picture 1). With suspicion of lymphoma, after collegial discussion between thoracic surgeons, cardiologists, interventional radiologists and cardiac surgeons, a right transjugular radio- and echo-guided biopsy was performed to complete diagnostic iter; biopsy was positive for peripheral B lymphocyte-derived large-cell diffuse lymphoma with large necrotic and subnecrotic areas. Chemotherapy showed initial benefit,with a clear reduction in size of the cardiac mass and mediastinal lymphnodes at 2 months follow up, with regression of dyspnoea.

Conclusions

In our patient, despite an early lymphoma onset and a typical poor prognosis compared to literature, an early diagnostic-therapeutic iter, an immunocompetent status, the absence of extracardiac disease and a preserved left ventricular ejection fraction, ensured an immediate clinical benefit with an improvement of his quality of life.
1080 EFFECTS OF ENZYME REPLACEMENT THERAPY ON CMR PARAMETERS IN PATIENTS WITH FABRY DISEASE: A SYSTEMATIC REVIEW AND META-ANALYSIS
Authors: Figliozzi Stefano , Georgiopoulos Georgios , Stankowski Kamil M , Camporeale Antonia , Pieroni Maurizio
Published: December 2022

Background

Cardiovascular death is the leading cause of mortality in patients affected by Fabry Disease (FD). Cardiac magnetic resonance (CMR) is the gold-standard imaging modality to assess FD heart involvement and enzyme replacement therapy (ERT) has the potential to slow or revert FD organ involvement and dysfunction. However, the effect of ERT on cardiac disease progression remains undefined. Thus, we performed a systematic review and meta-analysis to assess the impact of ERT on CMR parameters in FD patients.

Methods

Data search was conducted from inception through June 1, 2022, using the following Medical Subject Heading terms: “Fabry Disease” AND (“Cardiac MRI” OR “Cardiac Magnetic Resonance” OR “Cardiovascular Magnetic Resonance” OR “CMR”) AND (“ERT” OR “Enzyme Replacement Therapy”. Studies were included if contemplated FD patients undergoing baseline and follow-up CMR after ERT. Pre-defined study outcomes were the change of i) left ventricular wall mass (LVM; grams); ii) LVM indexed (grams/mq); iii) maximum left ventricular wall thickness (MLVWT, mm), iv) Late gadolinium enhancement (LGE; % of LVM) and v) native-T1 mapping (msec) between baseline and follow-up CMR. The study protocol was registered in the PROSPERO database (CRD42022336223).

Results

Ten studies including 517 FD patients were included. Between baseline and follow-up CMR, there was a significant decrease in LVM (pooled hazard ratio: -19.5 grams [95% CIs, -34.3; -4.63]), LVM indexed (-2.84 grams/mq [95% CIs, -5.24; -0.44]), MLVWT (-0.96 mm [95% CIs -1.9; -0.02]), and an increase in LGE (0.8% [95% CIs, 0.47; 1.14). T1-mapping did not significantly change (pooled hazard ratio: 6.12 msec [95% CIs -2.39; 14.62]). There was publication bias in LVM (Egger test: p-value = 0.047) but not in LVM index, MLVWT, T1-mapping and LGE.

Conclusion

In a pooled-analysis including 427 FD patients treated with ERT undergoing baseline and follow-up CMR, there was a significant decrease in LVM, LVM indexed, and LVMWT. In contrast, there was an increase in LGE extent whereas native-T1 mapping values did not significantly change.
584 AN UNUSUAL CASE OF A STRESSED HEART
Authors: Gigante Carlo
Published: December 2022

A 53-year-old woman was admitted to our emergency department complaining severe stabbing and oppressive retrosternal pain radiating posteriorly to left scapulae and left arm The onset was followed by general malaise, nausea, vomiting which she attributed to a period of intense emotional stress. She denied common cardiovascular risk factors, but in 2019 had a suspected acute coronary syndrome with subsequent finding of normal coronary artery at angiography in another institute. Her past medical history included severe asthma on multiple drug therapy with regular follow-up. Physical exam was normal. Electrocardiogram (ECG) showed subtle diphasic-negative T wave in anterior leads with mild ST depression in V4-V5, with patient still symptomatic. Lab examinations showed significantly increased troponins levels (hsTnI 664 ng/L, reference < 34 ng/L) with other lab values within normal range. A transthoracic echocardiogram at the bedside revealed mildly reduced systolic function with hypo-akinesis of the mid-apical anteroseptal and anterior left ventricular myocardial wall. Therefore, acute coronary syndrome was suspected and heparin and aspirin at a loading dose were administered. Still symptomatic, the patient was admitted in the ICU. In the same day a left heart catheterization revealed normal aspect of epicardial coronary arteries and ventriculography showed preserved basal and apical myocardial wall motion but hypo-akinesis of mid-cavitary walls, suggesting a possible mid-ventricular variant of Takotsubo syndrome (Figure 1). The patient was still symptomatic for stabbing chest pain after coronarography. To rule out the suspicion of acute aortic syndrome and pulmonary embolism, she also underwent a contrast-enhanced chest CT scan, that was fortunately normal. During her hospital stay, the patient had no arrhythmic and hemodynamic complications. Peak troponin level was 5027 ng/L. At serial ECGs, she developed diffuse deep T-wave inversion and a mild prolonged QT interval. Echocardiogram was repeated after 4 days showing complete normalization of left ventricular wall motion and restored ejection fraction also.

From the traditional “apical ballooning” Takotsubo Syndrome with apical akinesia and basal hypercontractility, different variants have been described. The mid-ventricular variant, seen in our patient was found in only 14.6% of the cases in the International Takotsubo Registry, followed by basal “reverse” and focal variants (2.2% and 1.5%, respectively).
439 TRANSIENT SEVERE MITRAL REGURGITATION IN REVERSED TAKOTSUBO SYNDROME
Authors: Leopizzi Alessandra , Santoro Francesco , Costantino Roberto , Giorda Gianpaolo , Passero Tommaso , Ignone Gianfranco , Brunetti Natale Daniele
Published: December 2022

A 65-years old Caucasian woman was referred to the emergency department for chest pain, dyspnoea and high systolic blood pressure. In the previous days she complained a constant state of anxiety and worries due to family troubles.

At the admission 12 – lead EKG showed sinus rhythm, ST-T segment depression >1 mm in the lateral and anterior leads (from V2 to V6). Laboratory exams showed elevated values of high sensivity troponin levels (high-sensitive troponin: 41 pg/mL – maximum laboratory cut off value: 12 pg/mL).

Bedside echocardiogram showed an LVEF of 40% due to apical hyperkinesis, akynesis of the basal segments and hypokinesis of the mid segments of the left ventricle; a severe double jet MR (Fig.1); a type II diastolic dysfunction (E/A: 1,5; E/e’: 14). Several B lines (> 3) were present in all pulmonary regions assessed by lung POCUS.

Cardiac angiography demonstrated non obstructive coronary artery disease. Left ventriculography showed an hyperdynamic apex and severe basal hypokinesis.

Clinical and functional status rapidly improved. Daily EKGs were registered with a progressive resolution of the ST-T segment depression. She was discharged on the 7th days and the TTE in pre-discharge showed an LVEF of 60%, without wall motion abnormalities and complete recovery of mitral regurgitation.

We report a case of reversible severe mitral regurgitation in a patient with basal ballooning TTS. Mital regurgitation is generally described in the contest of classical apical TTS. The potential mechanisms for severe MR are similar to those of acute myocardial infarction. Admission and daily echocardiographic evaluation is crucial in this subset of patients, with a tailored therapy following the severity of mitral valve disease.

Fig. 1
115 DIURETIC DOSE TRAJECTORIES IN DILATED CARDIOMYOPATHY: PROGNOSTIC IMPLICATIONS
Authors: Nuzzi Vincenzo , Cannatà Antonio , Pellicori Pierpaolo , Manca Paolo , Stolfo Davide , Gregorio Caterina , Barbati Giulia , Bromage Daniel I , Mcdonagh Theresa , Cleland John Gf , Merlo Marco , Sinagra Gianfranco
Published: December 2022

Background

For patients with heart failure, prescription of loop diuretics (LD) and higher doses are associated with adverse prognosis. We investigated LD dose trajectories and their associations with outcomes in patients with dilated cardiomyopathy (DCM).

Methods

Associations between outcomes and both furosemide equivalent dose (FED) at enrolment and change in FED in the subsequent 24 months were evaluated. According to FED trajectory, patients were divided in i) dose↑ (FED increase by ≥50% or newly initiated); ii) dose↓ (FED decrease by ≥50%); iii) stable dose (change in FED by <50%); iv) never-users. Primary outcome was all-cause-death/heart transplantation/ventricular-assist-device/heart failure hospitalization. Secondary outcome was all-cause-death/heart transplantation/ventricular-assist-device.

Results

Of 1,131 patients enrolled, 738 (65%) were prescribed LD at baseline. Baseline FED was independently associated with outcome (HR per 20mg increase: 1.12 [95% CI 1.04-1.22, p=0.003]. Of the 908 with information on FED within 24 months from enrolment, 31% were never-users; 29% dose↓; 26% stable dose and 14% dose↑. In adjusted models, compared to never-users, stable dose had a higher risk of primary outcome (HR 2.42 [95% CI: 1.19-4.93], p=0.015), while dose↑ had the worst prognosis (HR 2.76 [95% C.I. 1.27-6.03], p=0.011). The results were consistent for the secondary outcome. Compared to patients who remained on LD, discontinuation of LD (143, 24%) was associated with improved outcome (HR 0.43 [95% C.I. 0.28-0.65], p<0.001).

Conclusions

In a large cohort of patients with DCM, LD use and increasing FED are powerful markers of adverse outcomes. Patients who never require LD have an excellent prognosis.