bluuebunny/crossref_metadata_2025_split huggingface dataset:
603 Endothelial dysfunction is associated with hypertensive disorder during pregnancy
Authors: Avallone Carlo Maria , Nuzzo Martina , Rota Irene , Persico Nicola , Carugo Stefano , Bollati Valentina , Vicenzi Marco 
Published: December 2021

Abstract

Aims
Arterial hypertension (AH) is one of the main determinants of clinical disorders during pregnancy affecting 2% to 10% of pregnancies with a substantial public health impact. Both endothelial injury and increased vascular reactivity have been reported to be involved in the pathogenesis of pre-eclampsia syndrome. Abnormal patterns in brachial artery Doppler velocities have been shown to be predictive of pre-eclampsia in first trimester. The aim of this study is to investigate whether flow-mediated dilation (FMD) and Doppler flow derived-parameters can predict the occurrence of AH.

Methods and results
The study population consisted of pregnant women (mean age 32 years) who had been referred to the IRCCS Fondazione Ca’ Granda Policlinico of Milan. None of them had any medical issues and was taking any medications at the time of pregnancy. FMD was performed on left brachial artery according to expert recommendation. Measurements of brachial artery diameter and flow have been collected at rest, shortly before cuff release and then 5-, 15-, 30-, 60-, and 90-s during hyperaemia phase. Among Doppler measurements, systolic and diastolic velocity (Vs and Vd, respectively) as well as mean velocity (mean V) were considered. In addition, the pulsatility index (PI) and resistance index (RI) were calculated. A 3-months follow-up was planned in order to detect the presence of AH. All data were expressed as the median. U-test (Mann–Whitney analysis) was performed to test difference among hypertensives and non-hypertensives We recruited 48 women (median age 32 yeas) whose 4 (8.5%) developed AH during pregnancy. These latter had statistically significant higher systolic velocity measured at 5 s after the release of distal occlusion (126 cm/s vs. 173 cm/s; P &lt; 0.05). No other velocity Doppler data [diastolic velocity (Vd), mean velocity (mean V), PI, RI, TAMAX, and TAMEAN] showed a statistical significant association with AH development.

Conclusions
The present study suggests that the vascular assessment through Doppler during FMD procedure may foresee the development of hypertensive disorder in pregnancy. Our result provides the first evidence that the peak systolic velocity of brachial artery may represent a marker of early endothelial activation or damage, that can be directly involved in the pathophysiological mechanisms of the hypertensive disorders in pregnancy.
158 Racial differences in val122Ile associated transthyretin cardiac amyloidosis
Authors: Argirò Alessia , Zampieri Mattia , Batra Jaya , Rosenblum Hannah , Burkhoff Daniel , Maurer Mathew , Olivotto Iacopo , Cappelli Francesco 
Published: December 2021

Abstract

Aims
The valine-to-isoleucine substitution (Val122Ile) is the most common variant of transthyretin (TTR) amyloidosis in the USA, primarily affecting individuals of African descent and leading to a restrictive cardiomyopathy. This variant has recently been identified in a cluster of White individuals in Italy. In this study we aimed to investigate differences in the clinical phenotype of Val122Ile associated TTR cardiac amyloidosis (ATTR-CA) between Black and White individuals.

Methods and results
In this retrospective study of 70 patients (mean age 72 years) with Val122Ile associated TTR ATTR-CA, cardiac chamber performance was compared using noninvasive pressure-volume analysis. Compared to White patients (n = 17), Black individuals (n = 53) had lower systolic blood pressures (110 vs. 131 mmHg, P &lt; 0.001), reduced pulse pressures (41 vs. 58 mmHg, P &lt; 0.001), and impaired renal function (eGFR 46 vs. 67 mL/min/1.73 m2, P &lt; 0.001) at presentation. Systolic properties and arterial elastance were similar. Black patients had an end-diastolic pressure-volume relationship shifted upward and leftward relative to White patients, indicating reduced left ventricular chamber capacitance. Pressure-volume area at a left ventricular end-diastolic pressure of 30 mmHg was lower in Black compared to White individuals (8055 mmHg*ml vs. 11 538 mmHg*ml, P = 0.008).

Conclusions
Despite presenting at a similar age to White patients, Black individuals with Val122Ile associated ATTR-CA have a greater degree of cardiac chamber dysfunction at the time of diagnosis due to impaired ventricular capacitance. Whether these differences are attributable to amyloidosis or other cardiovascular disease requires further study.
587 Exercise induced pulmonary hypertension in hypertrophic cardiomyopathy: a combined cardiopulmonary exercise test-echocardiographic study
Authors: Re Federica , Halasz Geza , Moroni Francesco , Beltrami Matteo , Baratta Pasquale , Avella Andrea , Zachara Elisabetta , Olivotto Iacopo 
Published: December 2021

Abstract

Aims
Pulmonary arterial hypertension (PAH) has been described in patients with hypertrophic cardiomyopathy (HCM) and it was associated with a worse prognosis. Nevertheless in most HCM patients, despite normal pulmonary pressures at rest, congestive symptoms are elicited by exercise. In the present study, combining cardiopulmonary exercise test (CPET) with echocardiography, we aimed to evaluate the presence of exercise-induced pulmonary hypertension (EiPAH) its role in functional limitation and its prognostic significance in a cohort of patients with obstructive and non-obstructive HCM.

Methods and results
182 HCM patients (35% females, mean age 47.5 ± 15.9) undergoing CPET. During CPET, LVOT velocities and trans-tricuspid gradient were measured. Thirty-seven patients (20%) developed sPAP &gt; 40 mmHg at peak exercise (EiPAH). EiPAH was associated with an lower exercise performance, larger left atrial volumes, higher left ventricular gradient and higher VE/VCO2 slope .At multivariable model baseline sPAP (P &lt; 0.0001) and baseline left ventricular obstruction (LVOT) (P = 0.028) were significantly associated with EiPAH .Kaplan-Meier curve analysis showed EiPAH was a significant predictor of HCM –related morbidity (hazards ratio: 6.21, 95% CI: 1.47–26.19; P = 0.05; 4.21, 95% CI: 1.94–9.12; P &lt; 0.001) for the primary and the secondary endpoint respectively.

Conclusions
EiPAH was present in about one fifth of HCM patients without evidence of elevated pulmonary pressures at rest, and was associated with adverse clinical outcome. Diagnosing EiPAH by exercise echo/CPET may help physicians to detect early stage of PAH requiring a closer clinical monitoring and individualized treatment strategies.
641 Left ventricular non-compaction as a distinct cardiomyopathy or a phenotypic feature: a case report
Authors: De Zan Giulia , Marconetto Camilla , Carli Alessandro , Parodi Alessandro , De Vecchi Simona , Patti Giuseppe 
Published: December 2021

Abstract
A 71-year-old male with no cardiovascular risk factors and a history of two uninvestigated episodes of loss of consciousness one year before, presented in our department to undergo a treadmill exercise stress test in relation to inverted T waves in V5-V6 on his basal ECG and frequent ventricular extrasystoles (PVCs). The stress test was positive for inducible ischemia, with a significant depression of the ST-segment in the lateral lead. A transthoracic echocardiography revealed normal thicknesses and volumes of the left ventricle (LV) with a dyskinesia of the lateral wall and a mildly reduced ejection fraction (EF) up to 46%. Epicardial surface was unremarkable. Next, a coronary angiography showed no critical obstruction in the epicardial coronary arteries. In order to further investigate the frequent PVCs and the reduction of the LV systolic function, a cardiac magnetic resonance (CMR) with paramagnetic contrast agent was performed. The images outlined the presence of non-compaction in the distal lateral wall and apex with a late gadolinium enhancement (LGE) in the epicardial portion of the lateral wall (Figure 1). Moreover, the CMR showed an adipose infiltration of the interventricular septum and thickened right ventricular trabeculae, suggesting an overlap with arrhythmogenic left ventricular dysplasia. During the subsequent follow-up, the patient was implanted with an implantable cardiac defibrillator (ICD), after two reported episodes of loss of consciousness and an electrophysiology study (EPS) resulting in the induction of a symptomatic ventricular tachycardia. Non-compaction cardiomyopathy is a rare and still relatively novel nosological entity, which can sometimes be observed in the same patient with other forms of cardiomyopathy. In the context of an unexplained reduction of LVEF and frequent PVCs, the accuracy of CMR in describing cardiac morphology and myocardial layer remains an indispensable tool to detect otherwise underdiagnosed potentially fatal cardiomyopathies.
406 A 50 years old man with a late presentation of arrhythmogenic cardiomyopathy
Authors: Dellino Carlo Maria , Bariani Riccardo , Cipriani Alberto , Marra Martina Perazzolo , Pilichou Kalliopi , Iliceto Sabino , Corrado Domenico , Basso Cristina , Rigato Ilaria , Bauce Barbara 
Published: December 2021

Abstract
In January 2014 a 50 years old man without previous medical history experienced a syncopal episode. During evaluation at Emergency department (ED) a sustained ventricular tachycardia (VT) with haemodynamic compromise was found and successfully treated with DC shock. The patient was admitted to the Coronary Care Unit (CCU) where the ECG showed diffuse low QRS voltages and flattened T waves. Coronary angiography showed normal coronary arteries. 2-D echocardiogram documented the presence of a mildly dilated left ventricle (LV) with a mildly decreased systolic function (EF : 41%); the right ventricle (RV) was severely dilated (REDV : 41 cmq/mq) with a severe systolic dysfunction (fractional area change: 21%) with diffuse hypokinesia and akinesia of subtricuspid region. Cardiac magnetic resonance (CMR) confirmed ventricular dimensional and kinetic abnormalities and tissue characterization sequences demonstrated the presence of fatty infiltration of the epicardial segments of LV lateral wall and of RV free wall. After gadolinium injection, late gadolinium enhancement (LGE) presented the same distribution of the fatty infiltration. A diagnosis of arrhythmogenic cardiomyopathy (AC) was made and ICD in secondary prevention was implanted. The patient was treated with Sotalol (240 mg/daily) and remained asymptomatic and free from sustained ventricular arrhythmias for five years. In January 2019 he started to complain asthenia, dyspnoea (NYHA II) and anorexia and he was admitted to ED where a persistent slow VT was detected. Echocardiogram showed a severely dilated LV with severe systolic dysfunction (EF: 30%) with substantially unchanged RV features. One year later he experienced an heart failure (HF) episode with further reduction of LV systolic function (EF: 21%). Cardiopulmonary test documented a severe ventilation/perfusion mismatch (VE/VCO2 slope 50.6) and severe reduction of the exercise tolerance (VO2 peak 9.2 ml/kg/min). In March 2021 the patient started heart transplantation check list. Three weeks after the discharge he was transplanted. In conclusion, this clinical case highlights an infrequent late presentation of AC, with an initial high arrhythmic burden and a following rapid progression to refractory HF requiring heart transplantation.
188 Disarming the bomb in AL amyloidosis: a case report
Authors: Licchelli Luca , De Michieli Laura , Sinigiani Giulio , Da Pozzo Stefano , Barbera Mila Della , Rizzo Stefania , Cecchin Diego , Berno Tamara , Cipriani Alberto 
Published: December 2021

Abstract

Aims
Light-chain amyloidosis (AL) is a rapidly progressive systemic disease commonly involving also the myocardium, with poor outcome if lately diagnosed and treated. Clinical presentation can be widely varied, ranging from unapparent disease with soft symptoms to acute heart failure syndromes, requiring urgent therapies and supports.

Methods and results
A 52-year-old women came to our outpatient cardiomyopathy clinic because of hypertrophic cardiomyopathy (HCM) suspicion. Family history included a paternal cousin with diagnosis of unspecified cardiomyopathy and cardiac arrest. Her medical history was unremarkable until few months ago, when she started to complain with palpitations and asthenia. Given that both electrocardiogram and echocardiogram had previously showed signs of left ventricular (LV) hypertrophy, she was referred to us for HCM evaluation. Our physical examination was unremarkable, in particular there were no signs of central or peripheral venous congestion. Electrocardiogram showed a diffuse strain pattern with inferolateral ST-depression and T-wave inversion. Echocardiogram showed a thicked interventricular septum (17 mm), a pseudo-normal transmitral filling pattern with mild increase of LV filling pressure (E/E′ 11), a severely dilated left atrium (51 ml/mq). To complete the diagnostic path for HCM, we asked for a cardiac magnetic resonance (CMR), which two months later gave to us the diagnosis of myocardial amyloid infiltration. The diagnosis was quite surprising, because the patient was fine, 6 min walking test assessed a good functional capacity (500 m), no heart failure signs were recorded. So, we sent the patient to perform bone scintigraphy, which showed Perugini 0 uptake, and blood exam, showing, instead, rise of lambda free light chains, cardiac troponin (41.5 ng/l) and NTproBNP (5318 ng/l). Patient was urgently referred to haematologists, who using bone marrow and fat pad biopsy diagnosed a multiple myeloma with stage IV sec. Mayo AL amyloidosis. (Cy)BorD therapy was started, reaching a complete response in 4 months.

Conclusions
Diagnosis of AL amyloidosis is tricky due to heterogeneous clinical onset and multi-organ involvement. Cardiologist community should be aware of this condition, phenotypically mimicking HCM, but with very different management, in order to favour early diagnosis, prompt referral and treatment initiation. This case teaches that only 1. clinical awareness and 2. multidisciplinary approach can lead to disarm the bomb in AL amyloidosis.
180 Cognitive disorders in takotsubo syndrome: incidence, short, and long term outcome. Results from a prospective multi-centre registry
Authors: Mallardi Adriana , Santoro Francesco , Leopizzi Alessandra , Vitale Enrica , Iacoviello Massimo , Brunetti Natale Daniele 
Published: December 2021

Abstract

Aims
Takotsubo syndrome (TTS) is an acute and reversible left ventricular dysfunction, whose pathophysiological mechanisms are not completely known. There are evidence suggesting a possible link between neurological disease and TTS. Aim of the study was to evaluate incidence and prognostic value of cognitive neurological disorders among TTS patients.

Methods and results
379 consecutive patients were enrolled in a prospective multicentre registry. History, clinical features, echocardiographic parameters, in-hospital complications and long-term Follow-up events of all patients were recorded. Cognitive neurological disorders included Alzheimer disease, old age dementia and cognitive impairment for other causes. Prevalence of cognitive neurological disorders among TTS patients was 5.5% (num = 21). Among this subset of patients 48% (num = 10) had Alzheimer syndrome, 24% (num = 5) old age dementia and 28% (num = 6) cognitive impairment for other causes. Compared to the control group, these patients were older (81 ± 5 vs. 71 ± 12, P = 0.01) and predominantly men (24% vs. 9%, P = 0.01). No differences in term of cardiovascular risk factors and left ventricular ejection fraction at admission and discharge were found among the two groups. TTS patients with cognitive neurological disorders experienced higher rate of in-hospital complications (62% vs. 28%, P = 0.01), that were mainly driven by higher rate of pulmonary oedema (14% vs. 9%, P = 0.01), cardiogenic shock (29% vs. 8%, P = 0.01), death (24% vs. 4% P = 0.01), ischaemic stroke (10% vs. 4%, P = 0.01), and left ventricular thrombi (10% vs. 3%, P = 0.01). At long-term follow-up patients with cognitive neurological disorders when compared to those without, experienced higher rate of mayor cardiovascular events (48% vs. 16%, P = 0.01), cardiovascular re-hospitalization (14% vs. 10%, P = 0.01) and death (43% vs. 9%, P = 0.01).

Conclusions
TTS patients with cognitive neurological disorders had an increased risk of in and out of hospital mayor cardiac adverse events and mortality at short and long-term follow-up.
84 Left atrial reverse remodelling in dilated cardiomyopathy
Authors: Nuzzi Vincenzo , Raafs Anne , Manca Paolo , Henkens Michiel T.h.M. , Gregorio Caterina , Boscutti Andrea , Verdonschot Job , Hazebroek Mark , Knackstedt Christian , Merlo Marco , Stolfo Davide , Sinagra Gianfranco , Heymans Stephane R.B. 
Published: December 2021

Abstract

Aims
Left atrial (LA) dilation is associated a with worse prognosis in several cardiovascular settings but therapies can promote LA reverse remodelling. Characterizing and defining the prognostic implications of LA volume (LAVI) reduction in dilated cardiomyopathy (DCM).

Methods and results
Consecutive DCM patients from two tertiary care centres, with available echocardiography at baseline and at 1 year follow-up, were analysed. LA dilation was defined as LAVI &gt;34 ml/m2, Delta (Δ)LAVI was defined as the 1 year relative LAVI reduction. The outcome was a composite of death/heart transplantation/heart failure hospitalization (D/HTx/HFH). Five hundred sixty patients were included [age 52 ± 13 years; left ventricular ejection fraction (LVEF) 31 ± 10%, LAVI 45 ± 18 ml/m2]. Baseline LAVI had a non-linear association with the risk of D/HTx/HFH, independently from LVEF (P &lt; 0.001). At 1 year follow-up, LAVI decreased in 374 patients (67%, median ΔLAVI 24%, interquartile range 37% 11%). Factors independently associated with ΔLAVI were higher baseline LAVI and lower baseline LVEF. After adjustment for confounders, ΔLAVI showed a linear association with the risk of D/HTx/HHF (HR: 0.92, 95% CI: 0.86 0.99 per 5% decrease, P &lt; 0.001). At 1 year Follow-up, patients with a ≥ 15% reduction in ΔLAVI or LAVI normalization (i.e. Follow-up LAVI ≤34ml/m2) (42% of the cohort) were at lower risk of D/HTx/HFH (HR: 0.49, 95% CI: 0.33 0.74, P &lt; 0.001).

Conclusions
In a large cohort of DCM, 1 year reduction in LAVI is observed in the majority of patients. The association between reduction in LAVI and D/HTx/HHF candidates LA reverse remodelling as complementary early therapeutic goal in DCM.
711 A rare case of takotsubo syndrome induced by pacemaker implantation
Authors: Strangio Antonio , Leo Isabella , Sabatino Jolanda , Romano Letizia Rosa , Bella Sabrina La , Spaccarotella Carmen , Mongiardo Annalisa , Pasceri Eugenia , Aquila Iolanda , Bellomusto Rita , Signorelli Adriano , Santarpia Giuseppe , Curcio Antonio , De Rosa Salvatore , Indolfi Ciro 
Published: December 2021

Abstract

Aims
Takotsubo syndrome (TTS) is an acute cardiac condition characterized by a temporary wall motion abnormality of the left ventricle that mimics an acute coronary syndrome (ACS). TTS usually occurs following emotionally or physically triggering event. We report a rare case of Takotsubo syndrome following a pacemaker implantation.

Methods and results
A 77-year-old woman was admitted to our hospital with a third-degree atrioventricular block. She was asymptomatic with a history of hypertension, diabetes, hyperlipidaemia and extrapyramidal syndrome. Laboratory tests reported High-Sensitive cardiac Troponin T (HS-cTnT) 32.9 ng/L (ULN &lt;14), creatine kinase muscle and brain (CK-MB) 4.1 ng/mL (ULN &lt; 4.94) and NT-proB-type Natriuretic Peptide (NT-proBNP) 1465 pg/ml (ULN &lt; 125). Echocardiography showed a normal left ventricular ejection fraction (EF = 58%) (Figure 2A). The patient underwent dual chamber pacemaker implantation without immediate complications. Three days later, a routine ECG showed new T wave inversions (Figure 1), in absence of symptoms. Echocardiography revealed apical akinesia, with ‘apical ballooning’ (EF 30%) (Figure 2B). Serum cardiac markers were increased (CK-MB 8.2 ng/ml, HS-cTnT 189.7 pg/ml, NT-proBNP 15 005 pg/ml. A coronary angiography excluded obstructive coronary artery disease (Figure 3). Given the impossibility of carrying out a cardiac RMI for the recent pacemaker implantation and after exclusion of other diagnoses, pacemaker implantation induced Takotsubo syndrome was suspected.

Conclusions
The trigger of this case of TTS was the pacemaker implantation, a relatively brief and painless procedure that, in her case could have constitute a considerable emotional and physical stress. To the best of our knowledge 13 cases of TTS after pacemaker implantation have been described to date. Of those cases, four had an asymptomatic course. Therefore, the real incidence of TTS following pacemaker implantation may be underestimated and ECG and echocardiography should always be performed after pacemaker implantation.
131 Cancer and takotsubo syndrome: clinical features, outcome, and inflammatory patterns. Results from a multicentre prospective registry
Authors: Vitale Enrica , Santoro Francesco , Leopizza Alessandra , Mallardi Adriana , Iacoviello Massimo , Brunetti Natale Daniele 
Published: December 2021

Abstract

Aims
Cancer represents one of the major determinants of prognosis in patients with Takotsubo Syndrome (TTS). Aim of this study was therefore to compare clinical features, short- and long-term outcome and inflammatory pattern among TTS patients with history of cancer.

Methods and results
412 consecutive patients with TTS were enrolled in a multicentre prospective registry from July 2007 to February 2021. Clinical features, in and out of hospital MACE, laboratory and imaging data were collected. A sub-analysis evaluating serum interleukins levels among 12 patients with cancer vs. a propensity score matched cohort was performed. Prevalence of history of cancer was 12% (N = 51 pts). Patients with history of cancer were older (77 vs. 72 years, P = 0.01), with a higher percentage of male (23.5% vs. 8.8%, P = 0.01). Diabetes, dyslipidemia, and obesity were more prevalent in patients with cancer (29% vs. 22%, 49% vs. 42%, 25.5% vs. 18.5%, P = 0.01 respectively), while a similar prevalence was found for hypertension and smoke. Left ventricular ejection fraction (LVEF) at admission and discharge was lower in patients with history of cancer (33% vs. 37%, 44% vs. 50%. P = 0.03 respectively). Patients with cancer showed higher incidence of in hospital events (41% vs. 33%, P = 0.01) mainly driven by cardiogenic shock (21.5% vs. 8.5% P = 0.01), in hospital death (13.7% vs. 4.7%, P = 0.01), left ventricular thrombi (9.8% vs. 3.3%, P = 0.01) and ventricular arrhythmias (13.7% vs. 7.4%, P = 0.01). The long-term mortality was higher in patients with history of cancer (31.3% vs. 11.3%, P = 0.01). A distinct inflammatory pattern was found in cancer patients: at admission there were higher levels of IL 2 and VEGF levels (IL-2 3.3 vs. 0.7 pg/ml, P = 0.05, VEGF 476.3 vs. 249.5 pg/ml, P = 0.03); at discharge IL-4 was lower (1.17 pg/ml vs. 2.49 pg/ml, P = 0.04) while VEGF remained higher in subjects with TTS and cancer (406 vs. 128 pg/ml, P = 0.03).

Conclusions
Cancer patients with TTS are characterized by different clinical features, epidemiological characteristics, worse prognosis and higher long-term mortality when compared to patients with TTS without history of malignancy. A distinct inflammatory pattern can be found in this subset of TTS patients.