bluuebunny/crossref_metadata_2025_split huggingface dataset:
MULTIFOCAL ATRIAL TACHYCARDIA AND HYPERTROPHIC CARDIOMYOPATHY IN THREE PATIENTS WITH COSTELLO SYNDROME
Authors: Catucci S , Lombardi M , Massari E , Maiorano A , Meliota G , Pirolo T 
Published: May 2024

Abstract

Introduction
Costello syndrome is a rare disease, caused by mutation of the HRAS gene, associated with intellectual disability, growth retardation, facial dimorphisms. Cardiac abnormalities, mainly cardiac hypertrophy and multifocal atrial tachycardia, are present in 1/3 of patients.

Clinical Cases
From 2019 to 2023, 3 newborns with Costello syndrome came under our observation. M.M. showed respiratory distress, hypotonia and dysmorphic notes; genetic analysis highlighted the pathogenic variant c.64C&gt;A (p.Gln22Lys). On the echocardiogram, septal hypertrophic cardiomyopathy was found with left outflow obstruction and mitral insufficiency. Therapy with propranolol was undertaken. Holter ECG never revealed arrhythmias. At the last check–up, SIV hypertrophy, continent mitral valve, free left ventricular outflow. The second patient, O.L., arrived at 10 days of age following a run of supraventricular tachycardia. He had bilateral clubfoot and facial dimorphisms. The echocardiogram was normal. On Holter ECG atrial tachycardia interrupted by rare sinus beats. Therapy with flecainide was undertaken, without benefit. Subsequently, was undertaken amiodarone, then associated with digitalis. She was discharged with satisfactory control of the average ventricular rate, but later due to the appearance of chaotic atrial arrhythmia, propranolol was added; on the Holter ECG in triple therapy, periods of sinus rhythm alternating with short episodes of atrial tachycardia were observed. Genetic analysis revealed the pathogenic variant c.34G&gt;A, p.(Gly12Ser) of the HRAS gene. The third patient, F.A., presented atrial tachycardia. Flecainide therapy had transient benefit. Subsequently propranolol was added, without benefit. Therapy with amiodarone was initiated. Transesophageal electrophysiological study demonstrated multifocal atrial tachycardia, unresponsive to overdrive pacing. Partial control of the average ventricular rate was achieved in triple therapy. On the echocardiogram, mild concentric hypertrophy and dysplastic pulmonary valve. Genetic testing detected the c.35G&gt;C, p(Gly12Ala) variant of the HRAS gene.

Conclusions
Costello syndrome requires cardiac monitoring for the possible presence of multifocal atrial tachycardia and hypertrophic cardiomyopathy. Arrhythmia, which can also occur independently of hypertrophy, can be self–limited in the first months of life with aggressive pharmacological treatment but can persist or worsen in ¼ of patients.
THE CLINICAL IMPACT OF IMPLANTABLE LOOP RECORDER IN CHANGING MEDICAL DECISION: A SINGLE CENTER EXPERIENCE
Authors: Zaccaria C , Bernardini A , Paoletti Perini A , Padeletti M , Ciliberti D , Giomi A , Milli M 
Published: May 2024

Abstract

Background
Implantable Loop Recorder (ILR) has various indications for monitoring patients in different clinical settings. Most studies focus on a single indication without comprehensively evaluating the clinical impact of ILR. This study aims to retrospectively review a single experience regarding the actual role of ILR.

Methods
Patients were enrolled from March 2015 to April 2023 due to: syncope of unknown etiology, embolic stroke of undetermined source (ESUS), palpitation, monitoring of arrhythmias in Brugada syndrome (BrS), and following a pulmonary vein isolation (PVI). A change in the clinical course (cardiac implantable electrical devices (CIEDs) implantation, start of anticoagulation therapy, execution of an electrophysiologic study, ablation for tachyarrhythmias or PVI ablation) due to an ILR finding was considered as the primary endpoint. An unexpected result was defined as a different clinical finding from the expected for the cause of ILR implantation (ex. the finding of an atrioventricular block in a patient implanted for ESUS).

Results
130 patients of which 58 (44%) were females with a mean age of 58 ± 6 years were enrolled. The indication for implant resulted to be ESUS (25/130, 19%), syncope (38/130, 29%), palpitation (18/130 14%), BrS (24/130 28%), or after PVI follow–up (25/130 20%). A change in clinical course was reached in 44 (33%) patients after 12 ± 8 months of follow–up. Ten (8%) patients had a double event. The change of the clinical course was more frequent in female sex than in males (26/58 [45%] vs. 18/72 [25%] patients, p =0.025). In patients with syncope, a CIED was implanted in 7 (24 %) patients but interestingly 7 tachyarrhythmias events (5 AF and 2 SVT) were incidentally detected. In the stroke group, 6 (24%) started anticoagulation therapy after AF detection, and 3 (12%) CIEDs were implanted due to bradyarrhythmia. ILR allowed the diagnosis of tachyarrhythmia in 4 (22%) patients implanted for palpitation. In the syncope and ESUS group, no difference between expected diagnosis and unexpected events was found (syncope 7/38 [18%] vs. 9/38 [24%], p= 0.32; ESUS 3/25 [12%] vs. 6/25 [24%], p = 0.55).

Conclusions
ILR impacted on clinical pathway in 33% of patients during 12 ± 8 months of follow–up. Despite the reason for its implantation, it remains a useful tool for diagnosing other arrhythmias and the consequent changing of therapeutical approach. ILR had more impact in changing the clinical course in females than in males.
A CATASTROPHIC COMPLICATION OF A CHEST TRAUMA: A CASE REPORT
Authors: Bianco S , Piscione M , Polito D , Crispino S , Pugliese M , Ussia G , Grigioni F , Gabrielli D 
Published: May 2024

Abstract

A 43 year old patient came to the Emergency Department of our hospital for a cardiology visit. The EKG revealed polymorfic ventricular tachycardia with a heart rate of 160 bpm so that he was admitted to the Intensive Care Unit. In 2005, he had a post traumatic myocardial infarction after a car accident treated with the implantation of one drug eluted stent on the descending artery. Three years later, a single–chamber ICD was placed. On admission in Intensive Care Unit, cardiac auscultation revealed normal heart sounds without murmurs. No lower extremity edema was observed. A transthoracic echocardiogram showed general hypokinesis with reduced global left ventricle systolic function and no pericardial effusion (EF=20%). The patient was haemodynamically stable, the blood pressure was of 130/80 mmHg. The device was also interrogated and it showed six ineffective attempts of antitachycardia pacing. After that, the patient was given an effective intracavitary shock. Moreover, the patient underwent coronary angiography which showed no disease progression and patency of the stent previously implanted on the proximal descending artery. The electrophysiological study was performed and subsequently 3 ventricular ectopic focuses were effectively ablated. Multiple DC shocks were delivered during the procedure and the atrioventricular bundle was damaged. In consideration of the clinical features and the left ventricular reduced ejection fraction, an indication to the implantation of CRT–D was given.

Conclusions
This case report describes a rare complication of a blunt chest trauma. A young patient with no significant past medical history suffered a dissection of the coronary descending artery resulting in ST–elevation myocardial infarction. The mechanism of coronary occlusion following blunt chest injury may be due to: shear force applied to the coronary arteries leading to intraluminal thrombosis; vascular rupture; embolism to the coronary arteries; fissuring of an atherosclerotic plaque with dislodgment of plaque material; vascular spasm at the site of the injury. Anyway, severe left ventricular dysfunction caused the developing of polymorfic ventricular tachycardia which was unresponsive to medical treatment and was treated with ablation.
ACQUIRED LQTS WITH VENTRICULAR ARRHYTMIAS IN METHADONE AND CARDIOMYOPATHY AT ONSET
Authors: Roagna E , Angelini F , Gallone G , Vairo A , Bellettini M , Bocchino P , Saglietto A , Anselmino M , Giustetto C , De Ferrari G , Dusi V 
Published: May 2024

Abstract

Introduction
Methadone is associated with QT interval prolongation and increased risk of ventricular arrhythmias such as torsade de pointes (TdP), particularly if associated with other contributing factors. The management is complex, considering that in the majority of cases methadone suspension is impossible.

Clinical Case
Man, 39y, history of past cocaine and heroin abuse, in chronic methadone therapy, cames in ED for palpitation and recurrent syncope, after increasing methadone dose from 50 to 130 mg/die. At The ECG sinus rhythm, 83 bpm, wide monomorfic ventricular extrasystolia bigemina, of epicardical origin from the inferior–lateral wall (morphology like BBDx, positive concordance in the precordial leads, negative in DIII and aVF); QTc likely markedly prolonged. During observation recurrent episodes of TdP with external defibrillation; administred ev potassium (K 3.1), MgSO4 and β blocker ev. During hospitalization started nadolol, titrated until 1 mg/kg, and gradually reduced methadone up to 50 mg/die. Executed Holter ECG and ergometric test with evidence of flat and notched T waves with QTc markedly prolonged (&gt; 550 ms) and poor adjustment to chronotropic variations. The echocardiogram shows LVFE 50%, GLS –13% (more reduce at inferior and infero–lateral walls); strongly negative electromechanical window (EMW –232 ms) and pronounced mechanical dispersion (+73 ms). The CMR evidenced late gadolinium enhancement (LGE) of non ischemic pattern, in the same segments with alterated kinesis. Mexiletine oral load test (6 mg/kg) was performed with contemporary ECG and Echo, without significant QTc, EMM or mechanical dispertion variation, suggesting unusefulness of chronic administration. After collective discussion, and considering patient choise, a bicameral ICD was implanted. A wide spectrum genetic test for channellopathies and cardiomyopathies was performed, (NGS and MLPA tecnique), resulted negative. At 1 year follow up (methadone 40 mg/die) persist prolonged QTc (580 ms), without arrhythmic episodes.

Conclusions
Our clinical case illustrates methadone proarrhytmic effect, associated with hypokalaemia and cardiomyopathy at onset, in a subset of prolonged QTc, with elevated electro–mechanical dispersion. Permanent ventricular bigeminism, rare in other acquired or congenital LQTS, is decribed linked with methadone use and its molecular effect of IKr and IK1 block; in our patient there’s correlation between site of origin and site of fibrosis (LGE).
OUTCOME OF OUT OF HOSPITAL CARDIAC ARREST: ARE THERE GENDER DIFFERENCES?
Authors: Bolognesi M , Rossi L , Pelizzoni V , Seghini P , Biagi A , Bricoli S , Ferraro S , Pisati M , Aschieri D 
Published: May 2024

Abstract

Background
Despite advance in resuscitation science, out of hospital cardiac arrest (OHCA) has an average survival rate of only 10 %. Ventricular fibrillation (FV) is amenable to defibrillation, but deteriorates to non shockable rhythms over time. It’Known that immediate cardiopulmonary resuscitation and eraly defibrillation increase survival after OHCA. It remains unclear whether there is an association between gender and survival. It seems that women OHCA patients are older, are less likely to have a witness arrest and they had fewer arrest in pubblic location than men . However these data are controversial.

Methods
We considered consecutively 6995 OHCA survivors resuscitated from emergency medical System (EMS) or bystander (BS) In Piacenza between june 1999 to decembre 2022 and we evaluated the outcome, considering sex and gender disparities

Results
Out of 6995 suffered from OHCA, 2983 (43%) were females with average age 81 ±13 years and 4012 (57%) males with average age of 73 ±15 years (p&lt;0,00001).We observed with advanced age an incresead rate of woman that suffered from OHCA in comparison to man (&lt; 44 years 177 m vs 63 w; 45–60 y 594 m vs 152 w, 61–75 y 1261m vs 530w; &gt; 75 y 1980 m vs 2239 w).The survival of our population was 270 patients At EMS arrival 6150 patients (88%), 2779 (45%) female and 3371 (55%) male presented a non schockable rhythms (NSR):asystole or pulseless electrical activity (PEA) whilst 845 patients(12%), 170(75%) males and 56 (25%) females, presented FV .In the group with FV as initial rhythm, the survival was 26% (56/206 f 27% vs 107/647 m 26% p= 0,06). Also in this group women were slightly older (73 years f vs 67 m, p&lt;0,00001). In all population survival decreased in females vs males (66/2984 w 2% vs 200/4012 5%f vp&lt;0,00001).Females appeared with more non schockable rhythms (93 % f vs 84 % m, p&lt; 0,00001)Women suffered from OHCA were found more frequently at home (2785 w 93% vs 3379 84%m p &lt; 0,0001).Furthermore 97% males and 98% female were treated by EMS and 3 % males and 1,3% females by BS(survival males 3,6% EMS vs 51% BS and females 2 EMS%vs 26%BS).

Conclusions
In our OHCA population, the survival was 4% with better results in man Female were older and they had less survival, because initial rhythm registered were non shockable rhythms. They were found at home,with a delay in the first aid. This can be due to a gender disparities with advanced age.
LYME AND DENGUE FEVER: FROM BANGLADESH TO ITALY
Authors: Massara M , Zappelli L , Lanari A , Pergolini M , Giovagnoli A , Refi G , Di Noto G 
Published: May 2024

Abstract

Background
Dengue fever (Flavivirus) and Lyme disease (Borrelia burgdorferi) are rare tropical infectious diseases transmitted by hematophagous vectors (Aedes mosquitoes and ticks, respectively), and they are extremely uncommon in Italy. In European countries, both represent a public health concern as they manifest as imported diseases.

Case Report
A 54–year–old man from Bangladesh was admitted with symptoms of fever, dyspnea, and pericarditic chest pain. Laboratory and imaging examinations revealed severe acute respiratory failure secondary to left lower lobe pneumonia and pleuropericardial effusion. Broad–spectrum antibiotic therapy and anti–inflammatory treatment were initiated, suspecting para–pneumonic pleuropericarditis. During hospitalization, there was a gradual improvement with a reduction in inflammatory indices and resolution of lung consolidation. The patient was discharged after 14 days with anti–inflammatory therapy (ibuprofen and colchicine). Approximately 7 days later, the patient returned to the emergency department complaining of a recurrence of symptoms and fatigue. The EKG showed phases of 2:1 AV block, EAS and EPS, complete de novo left bundle branch block (LBBB), alternating with an escape atrial rhythm, not evident in previous EKG. During hospitalization, complete AV block emerged with prolonged asystolic phases, requiring urgent placement of a temporary pacemaker. Concurrently, blood tests revealed anemia and increased levels of inflammatory markers, troponin, creatinine, transaminases, cholestatic indices, LDH, and lipase, indicative of multiple organ failure (MOF). Acute pathologies or active bleeding were ruled out by total body CT with contrast. Blood cultures and serological tests were performed to exclude infections by Trichinella, Mycoplasma, Borrelia, Dengue, and other rare infectious diseases. Due to progressive deterioration, the patient was transferred to a tertiary care hospital. In the following days, laboratory results showed positive IgG and IgM for Dengue (without serological evidence of viral RNA) and positive IgG and IgM antibodies for Borrelia burgdorferi.

Conclusion
In Italy, the incidence of these diseases is low, and their coexistence in the same host is even rarer (if not anecdotal). However, coinfection cannot be excluded in patients from areas where these diseases are endemic, such as Bangladesh.
AN UNCOMMON CASE OF SUPERIOR VENA CAVA SYNDROME FOLLOWING CARDIAC SURGERY
Authors: Michele C , Asta A , Maio E , Giovanella E , Vera Mondazzi L , Trunfio D , Abbas Ahmed M , Rocchi D , Portella G , Masini F 
Published: May 2024

Abstract
Superior vena cava (SVC) syndrome (SVCS) is due to the obstruction of the SVC leading to swelling of the upper body and to low cardiac output. Non tumoral causes, mainly thrombosis of endovascular devices, account for 30% of cases. We report a case of acute iatrogenic SVCS. A 7 years old 16 kg boy underwent mitral valve surgery in severe rheumatic mitral regurgitation. After induction a 15 cm 5.5 Fr 3 lumens central venous catheter (CVC) was inserted in the right internal jugular vein (IJV) under ultrasound guidance without issues. After achieving proper anticoagulation ascending aorta (18 Fr) and bicaval cannulation were performed (20 Fr single stage cannula with right–angled metal tip in the SVC and 26 Fr single stage cannula in the inferior vena cava). Cardiopulmonary bypass (CPB) was performed with pediatric circuit and the mitral valve was repaired. Despite apparently good result of the repair and uneventful weaning from CPB a more accurate transesophageal echo (TEE) showed moderate iatrogenic mitral stenosis. Cannulation was re–performed with same size cannulas and mitral valve replacement (St.Jude 25 mm) was provided. After weaning from CPB with mild cardiovascular support (adrenaline 0.03 mcg/kg/min and noradrenaline 0.05 mcg/kg/min) the TEE showed no abnormalities and the patient was transferred to the ICU in stable conditions. The central venous pressure (CVP) never raised over 10 mmHg. In the ICU the patient developed sinus tachicardia and hypotension, unresponsive to fluids and vasopressors, and swelling of the face with no increase in CVP. The chest Xray showed enlargement of the right upper mediastinum with TEE evidence of accellerated flow in the SVC around the CVC (fig.1). The patient was taken to the operating room and a too tigh pursue string suture was found on the SVC. After optimization of the suture the flow through the SVC was restored with immediate clinical improvement and de–escalation of the cardiovascular support. The face oedema quickly decreased allowing extubation on the day after surgery. The following course was uneventful. SVCS is a life threatening complication of cardiac surgery. A too tight pledgetted pursue–string suture is an uncommon cause compared to clot compression. The CVP measured by a IJV CVC is generally elevated in SVCS, but in our case the tip of the CVC was downstream to the stenosis so showing normal values. Multimodal monitoring is mandatory to lead to emergency surgical treatment in case of need.
EARLY RESULTS OF MINIMALLY INVASIVE CORONARY ARTERY BYPASS GRAFTING
Authors: Squiccimarro E , Margari V , Kounakis G , Visicchio G , Pascarella C , Carbone C , Paparella D 
Published: May 2024

Abstract
Coronary artery bypass grafting (CABG) is the benchmark in elective revascularization for Coronary Artery Disease (CAD), particularly for complex or multivessel CAD, left main coronary involvement, diabetes, or impaired heart function. Traditional CABG, via median sternotomy using cardiopulmonary bypass (CPB), has limitations like CPB–related morbidity and sternal complications. Alternatives like Minimally Invasive Cardiac Surgery (MICS CABG) and Hybrid Revascularization (HR) aim to address these drawbacks. We present a series of 215 consecutive patients (mean age 67±9 years, 27.9% females, EuroSCORE II 1.5±1.3%) between 2017–2023 who underwent elective minimally invasive surgical revascularization. Among the cohort, 164 (76.3%) had Minimally Invasive Direct Coronary Artery Bypass (MIDCAB) via anterolateral left mini–thoracotomy (ALMT) at the fifth intercostal space (IS), with 140 (85.4%) receiving a single left internal thoracic artery (LITA) graft to the left anterior descending (LAD) artery and 24 (14.6%) an additional distal anastomosis. Thirty–four (20.7%) had HR via percutaneous coronary intervention (PCI) on the right coronary artery (RCA). Of the total cohort, 35/215 patients (16.3%) underwent multivessel MICS CABG via ALMT, with mean 2.3±0.5 distal anastomoses using LITA, right ITA, radial artery, or saphenous vein. Heart manipulation was via a subxiphoid device. Four MICS CABG patients (11.4%) had HR. More than half of MICS CABG cases were performed with planned peripheral CPB, without cross–clamping the Aorta. Of the total cohort, 16/215 patients (7.4%) received MICS CABG in an endoscopic fashion, using 3D thoracoscopy for LITA and RITA harvesting via bilateral ports in the 2nd, 3rd, and 4th IS, employing peripheral CPB and aortic clamping by Chitwood clamp at the second IS, with cardioplegia delivery via a 4 cm right anterior MT. Distal anastomoses were performed via left anterior MT, with easy subxiphoid manipulation of the emptied heart. Transit time flowmetry was used in all cases. Conversion to sternotomy was needed in 3 cases, all were MIDCAB. Thirty–day mortality was 1.4%, median ventilation was 4 [2–5] hours, and Intensive Care Unit–stay was 1 [1–2] days. All patients received graft evaluation via computerized tomography before hospital discharge. Mean follow–up was 2.9±1.9 years (max 5 years), with no deaths and 4 (1.9%) requiring follow–up PCI. MICS CABG is a promising future technique for heart revascularization by dedicated teams.
IATROGENIC ACUTE MITRAL INSUFFICIENCY FOLLOWING IMPELLA–ASSISTED INTERVENTIONAL PROCEDURE
Authors: Totaro P , Pelenghi S 
Published: May 2024

Abstract

Background
Short–term left ventricle mechanical assistance device could be useful in assist interventional cardiologist during high–risk procedures. Abiomed Impella is one of the most frequently used device in this respect due to the high flow available with a low rate of reported complications. Here we report a rare but potentially catastrophic complication related to the Impella device.

Case Description
A 78 years old patient was referred to our Division by a peripheral hospital for a severe, symptomatic (NYHA III) mitral regurgitation (MR) due to posterior mitral leaflet prolapse (with focal eversion of P3) and chordal rupture (Fig 1a). Medical history included a previous admission in the some hospital due to a scheduled Impella–assisted PTCA (left main/left anterior descending artery) . Pre–procedural trans–thoracic ECHO (TTE) did not show significant MR (Fig 1b). Results Patient underwent urgent surgical procedure. At operation postero–medial mitral commissure was seen to be completely everted with multiple rupture of P3 and A3 chordae. Surgical procedure (Fig 1c) included: P3 plication + posterior–medial commissure edge–to–edge plasty + complete mitral semi–rigid ring (Simulus) n.30. Left atrial appendage was also closed using an external Atriclip. Postoperative course was without significant complications except a superficial wound dehiscence which delayed hospital discharge till 20th postoperative day.

Conclusion
Despite being a feasible and attractive option for short–term mechanical circulation assistance during high–risk interventional procedure, Impella device insertion could cause significant mitral valve damage leading to clinical deterioration. Such potentially catastrophic complication should be, therefore, mandatory excluded before patient discharge following interventional procedure.