bluuebunny/crossref_metadata_2025 huggingface dataset:
USING THE 6MWT FOR SETTING AEROBIC LOADS AFTER CARDIAC SURGERY AN INPATIENT SETTING
Authors: Romano N , Monina E , Longoni P , Forni G , Mazza A
Published: May 2024

Background

In the field of Preventive and Rehabilitative Cardiology (CPR), aerobic–type training turns out to be one of the cornerstones for intensive rehabilitation of patients undergoing cardiac surgery. One of the most critical issues appears to be the method by which the patient‘s initial workload is set. In this research project, an attempt is made to implement in clinical practice, an equation to identify the workload to be set at the first rehabilitation session. The formula in question is a modified version of Luxton‘s formula used to predict maximal workload using anthropometric/clinical data and results at the Six Minute Walk Test as indicators. The primary objective is to demonstrate that patients who trained from a load set through the use of the formula under consideration walk a greater distance at the end of hospitalization at the 6MWT than patients who did not use the equation for initial load setting.

Materials and Methods

Two small groups of patients (45 IG and CG 38 and 8 drop out) who underwent the same rehabilitation program at the O.U. of Rehabilitation Cardiology at ICS Maugeri in Pavia were compared, having as their only difference the mode of initial workload setting. Patients were recruited during the period April 2023– November 2023.

Results

At the final observation time, GI patients traveled more meters at the 6MWT than GC patients; they also finished the program with a higher workload.

Conclusions

It was shown that the workload determined by the formula is well tolerated by the patient and has brought benefits on functional capacity and perception of health status by the patient with cardiac surgery outcomes.
INTRODUCTION OF LUNG ULTRASONOGRAPHY (LUS) AS A DIAGNOSTIC TOOL IN THE REHABILITATION OF POST–CARDIAC SURGERY PATIENTS WITH PLEURAL EFFUSION
Authors: Lovagnini M , Rossi D , Caporotondi A , Guazzotti G , Forni G , Pinna G
Published: May 2024

Background

Pleural effusion (PE) is a common complication after cardiac surgery and is associated with postoperative heart failure and adverse outcomes. Lung ultrasound (LU) has been shown to be more accurate than chest x–ray (CXR) in the diagnosis and evaluation of PE. Other advantages include bedside applicability, no radiation exposure, and low cost. However, the use of LU in the rehabilitation setting of post–cardiac surgery patients is still limited.

Objective

To determine the extent of the use of LU compared with CXR in the evaluation of PE in post–cardiac surgery follow–up (FUP) in an Italian rehabilitation hospital.

Methods

Fifty consecutive patients (pts) (mean age: 67+9 years, 28 males, 22 females) admitted to our rehabilitation center 9+2 days after cardiac surgery (25 valve surgery, 16 coronary artery bypass graft (CABG), 6 valve surgery + CABG, 3 pulmonary endarterectomy) were included in the study and underwent baseline LU + CXR (time T0). Based on the clinical condition of each patient, the treating cardiologist decided whether to perform follow–up assessment by LU alone (GROUP1, less severe patients) or by CXR + LU (GROUP2, more severe patients) (time T1). The severity of PE was graded by the radiologist/sonographer using a 4–point scale (0=none, 1=mild, 2=moderate, 3=severe PE). The presence of pulmonary atelectasis on LUS was reported to the physician. Patients performed a 6MWT at baseline and at discharge.

Results

Figure 1 shows the study flow chart and Table 1 shows the main patient characteristics in both Groups. Table 2 shows the comparison between the two methods of PE assessment. At T0, the severity of PE as assessed by LU was similar in both groups and was underestimated by CXR. Of the 18 patients who underwent CXR+LU at T1, 9 (50%) required CXR evaluation for the following pulmonary complications: 5 suspected pneumonia (2 confirmed), 2 congestive heart failure, 1 hypoxemia, 1 parenchymal nodule. Medical therapy and the percentage of patients with bilateral atelectasis were similar in the two groups.

Conclusions

Our data show that CXR underestimate the severity of PE. CXR is still unnecessarily ordered by the treating cardiologist in approximately one third of patients with moderate/severe PE and no other pulmonary complications.
LONG–TERM FUNCTIONAL LIMITATIONS ON CARDIOPULMONARY EXERCISE TESTING IN A COHORT OF PATIENTS WITH EMOTIONS–TRIGGERED TAKOTSUBO SYNDROME
Authors: Maggio E , Jabbour J , Arcari L , Cacciotti L , Magrì D , Recchioni T , Valeri L , Vizza C , Badagliacca R , Papa S
Published: May 2024

Background

In patients with prior Takotsubo syndrome (TTS), long lasting functional cardiac limitations were described as compared with normal subjects. Emotions–triggered Takotsubo syndrome (E–TTS) has more favorable outcomes than TTS preceded by a physical trigger or by no identifiable factors. The aim of the present study was to assess long–term cardiac functional limitations in a cohort of asymptomatic E–TTS patients.

Methods

We enrolled n=39 asymptomatic patients with a diagnosis of E–TTS. Cardiopulmonary exercise tests (CPET) were performed at 30 [12–40] months median follow–up from the acute event. A cohort of n=39 individuals matched for age, sex, body mass index and comorbidities served as control.

Results

Despite recovery of left ventricular ejection fraction, patients with prior E–TTS had lower peak VO2 and percentage of predicted peak VO2 (17.8 ± 3.6 vs 22.5 ± 6.5; P < 0.001 and 75.2 ± 14.1 % vs 100.6 ± 17.1%, P <0.001), VO2 at anaerobic threshold (AT) (11.1 [10.1–12.9] vs 14.4 [12.5–18.7]; P <0.001), peak O2 pulse (9.7 ± 2.5 vs 13.1 ± 3.5; P <0.001) and higher VE/VCO2 slope (30.4 ± 3.7 vs 27.2 ± 3.5; P <0.001) compared with matched controls. We found no statistically significant differences in heart rate reserve (HRR), respiratory equivalent ratio (RER), mean blood pressure and peak PetCO2 between patients and controls.

Conclusions

Despite their overall favorable outcome, asymptomatic patients with E–TTS were found to have long–term subclinical functional cardiac impairments. CPET proved to be a useful tool in the long–term evaluation of E–TTS patients after recovery of LVEF. Further studies are needed to fully clarify the origin of the detected CPET abnormalities as well as their prognostic relevance in patience with recovered E–TTS.
A TAVI VALV IN VALV AT ALMOST 90 YEARS
Authors: Salucci C , Orso F , Verga F , Corti G , Graziani M , Alfano F , Poletti E , Cagnoni C , Ungar A , Baldasseroni S
Published: May 2024

Degeneration of the biologic aortic valve prosthesis represents an increasingly important issue. This process often affects frail elderly patients who are not candidates for cardiac re–intervention. TAVI valve–in–valve implantation therefore probably remains the only viable option in such patients.We report the clinical case of Mrs. Luciana F., currently 94 years old, who underwent biological aortic valve replacement in 2016. In charge of the Elderly Patient‘s Heart Failure Outpatient Clinic (SOD Geriatrics–UTIG) since January 2023.At first evaluation,89 years old, widowed, living alone; MMSE 29/30. SPPB 7/12. Totally autonomous in BADL, and partially in IADL.She complained of decreased exercise tolerance (NYHA Class III). No alterations in left ventricular kinetics. Calcified and degenerated aortic valve prosthesis with markedly reduced box opening and severe stenosis (Vel 4.7 m/s; Grad max/med (86/55 mmHg) mild insufficiency. May 2023 access to DEA for syncopal episode, complicated by fall down stairs and head injury; on echocardiogram relief of further degeneration of aortic bioprosthesis (moderate–grade intra–prosthetic insufficiency at 2 jets with PTH 300 msec and severe stenosis, Gmax/Gmed 88/55 mmHg, DVI 0.20 June 2023 worsening of dyspnea that appeared even at rest NYHA IV. At echocolordoppler confirmed severe aortic stenosis severe bioprosthesis degeneration. She was evaluated in Heart Team: low overall risk (MPI 0.31) therefore percutaneous implantation of Medtronic Evolut R aortic bioprosthesis has been performed. At discharge asymptomatic, eupnoic, walking independently without aids for short stretches. At subsequent follow–up.–1 month: substantial clinical well–being in the absence of dyspnea on ordinary exertion (NYHA II), no orthopnea or paroxysmal nocturnal dyspnea; denies angor, heart palp or syncopal episodes. She has resumed household chores and leaves the house almost every day –6 months: clinical stability. She walks long distances every day in the absence of symptomatology. –1 years (turned 90 years old): clinical stability with dyspnea for moderate exertion continues active life. Trans prosthetic gradient max/medium 22/12 mmHg, VD–AD 30 mmHg. In conclusion TAVI procedure on degenerated biological prosthesis should be an option to be considered in the elderly patient selected and evaluated by a multidisciplinary Heart Team to achieve improvement of cardiovascular symptoms and concomitantly recovery of functional autonomy.
LOCK PROTOCOL: A NEW ROBUST WORKFLOW FOR A VERY HIGH FIRST PASS PULMONARY VEIN ISOLATION DURING ATRIAL FIBRILLATION ABLATION
Authors: Zarra S
Published: May 2024

Introduction

Pulmonary vein isolation (PVI) is the gold standard for atrial fibrillation (AF) ablation. First pass PVI is often demanding when a raw geometry reconstruction is used in combination with an inaccurate automarks annotation, accounting for discontinuous or non–transmural lesions and related AF recurrences. We hypothesized that a magnetic–post processing of left atrial geometry in combination with an LSI–guided high power short duration (HPSD) automarks annotation could improve procedural efficiency.

Purpose

Aim of the study was to determine the impact on procedural outcomes of the new “Lock PVI protocol” during AF ablation with the new voxel mode EnSite X mapping system.

Methods

We included in the study consecutive patients with AF scheduled for pulmonary vein isolation (PVI) at our Departement between January 2021 and December 2022. Patients were divided in two Groups, the Lock–PVI Group and the control No Lock–PVI Group. The “Lock PVI” Group was ablated with the new Ensite X System using the new protocol, which consisted in (1) performing a road map with contact–force annotation at potential PVI sites after High Density reconstruction, (2) shaving the magnetic geometry reconstruction to unearth true 3D location of contact points, (3) Use of 3D–Automarks color–coded with a Lesion Index (LSI) target of 5.5 (anterior) and 4.5 (posterior) (4) Inter–lesion distance £6 mm (5) High Power Short Duration Approach (50 W). The No–Lock PVI was represented by patients ablated with and HPSD ablation with the previous version of the EnSite Precision mapping suit as per local standard practice. Groups were compared in term of procedural duration, radiological exposure and “first pass” isolation.

Results

Forty–four patients (22 “Lock PVI” and 22 controls mean age 63,3±8 years, 65,9% male, 47,7% persistent AF), for a total of 88 PVI circles were enrolled. Procedural duration was similar between approaches (121,1±43,6 vs 125,3±30,1 min; P=0,72) but RF time was shorter in the “Lock PVI” group (19,6±4 vs 29,55±9,1 min; P<0,001), even if with a longer fluoroscopy (19,5±10,1 vs 13,4±4,9 min; P=0,01). Of note, first pass isolation was significantly higher in the “Lock PVI” group: 40/44 (90,9%) vs 28/44 (63,6%) circles (P=0,002).

Conclusion

The new “Lock PVI” approach allowed a consistently better first pass isolation (>90%) with less RF time when compared to standard of care PVI.
CONTINUOUS LEFT STELLATE GANGLION BLOCK DURING VT ABLATION TO SLOW VT AND IMPROVE ABLATION OUTCOME
Authors: Morena A , Matta M , Ferraris F , Frea S , Anselmino M , Berruquier P , Gravinese C , Giannino G , Angelini F , Saglietto A , De Ferrari G , Dusi V
Published: May 2024

Introduction

Perioperative management of advanced patients with indication to ventricular tachycardia (VT) ablation is complex. A PAINESD score ≥ 15 was proposed to identify those who might benefit from pre–emptive mechanical cardiopulmonary support (MCS). We present a case successfully managed with continuous percutaneous left stellate ganglion block (C–PLSGB).

Case

A 71–year–old diabetic man with severe post–ischemic cardiomyopathy (LVEF 24%, NYHA Class III) and chronic pulmonary disease was admitted for electrical storm with multiple ICD shocks due to poorly tolerated monomorphic VT (MMVT) at 176 bpm resistant to anti–tachycardia pacing (ATP), despite ongoing chronic oral therapy with amiodarone and carvedilol. Poorly tolerated and ATP–resistant MMVTs at 170–180 bpm recurred despite intravenous (iv) amiodarone and lidocaine (20 mcg/kg/min) and oral propranolol. Ultrasound guided C–PLSGB was therefore started; lidocaine was chosen for the continuous infusion due to the short half–life, at the initial dose of 10 mg/h; 6 hours later, his poorly tolerated MMVT recurred at 167 bpm. Therefore, at first we increased iv lidocaine to 30 mcg/kg/min, without benefit, then we increased C–PLSGB at 37.5 mg/h and decreased iv lidocaine to 20 mcg/kg/min due to neurological symptoms. With this regimen, the patient stayed VT free for 2 hours, then the clinical VT recurred at 130 bpm, with good hemodynamic tolerability and ATP–response. C–PLSGB infusion was than increased to 75 mg/h, with no more VT. Endocardial VT ablation was planned. Despite a very high PAINESD score (31) we decided to avoid pre–emptive MCS. We stopped lidocaine (both iv and as C–PLSGB) 2 hours before ablation. As soon as the patient came in the EP room (before instrumentation), the clinical MMVT recurred at 133 bpm. C–PLSGB was then resumed (initially at 100 mg/h for 30 minutes, then at 37.5 mg/h). The MMVT then spontaneously recurred at 133 bpm: it was mapped and ablated in the medio–apical anterior wall, with acute VT interruption. A second VT morphology then appeared, that was mapped and ablated at a more basal site, despite a partial epicardial component of the circuit was identified. C–PLSGB was then stopped and no more VT occurred nor were induced 1 hour later. At 8 months the patient is still alive with no more VT.

Conclusion

C–PLSGB with individually tailored infusion rates could be used during VT ablation as a mean to reduce VT cycle length and allow for mapping without MCS.
MISDIAGNOSED PARTIAL ANOMALOUS PULMONARY VENOUS RETURN AS A CAUSE OF RIGHT VENTRICULAR DILATION IN TRICUSPID VALVE DYSPLASIA
Authors: Pierfelice F , Rende G , Gallina S , Balducci A
Published: May 2024

A 38–year–old woman comes to our attention for regular follow–up of a known tricuspid valve dysplasia, diagnosed since she was 19 years old. The patient has been persistently asymptomatic and hemodynamically compensated. In previous cardiac assessments, there was moderate and stable tricuspid regurgitation (TR) that never reached the diagnostic criteria for Ebstein‘s anomaly (7 mm). The right atrium was dilated, and the right ventricle was at the upper limits of normal dimensions. However, our evaluation revealed a worsening of TR to a severe degree, associated with valvular annulus dilation and severe dilation of the right ventricle, which remained normokinetic, and evident diastolic overload of the interventricular septum. Therefore, a cardiac surgery intervention was indicated to correct the valvulopathy. During hospitalization, a transthoracic echocardiogram was repeated to investigate a possible cause of right ventricular overload, not fully explained by the relatively short duration of severe TR. During the exam, suspecting Partial Anomalous Pulmonary Venous Return (PAPVR) as one of the possible determinants, a vessel compatible with left superior pulmonary venous return was visualized, which converged into the anonymous vein and then into the right superior vena cava. Further diagnostic investigation with magnetic resonance angiography and computed tomography angiography confirmed the severe dilation of the right cardiac chambers and the presence of left upper pulmonary venous return and part of the left lower lobe into the left anonymous vein, with a Qp/Qs of 1.7 in relation to the pre–tricuspidal shunt. Therefore, a cardiac surgery intervention was indicated for PAPVR correction. However, previously unnoticed thrombocytosis was revealed in the hematological exams, leading to the initiation of a pertinent hematological workup and the cardiac surgery intervention was postponed to reduce the risk of perioperative complications. In conclusion, PAPVR is a rare congenital condition that can remain asymptomatic until adulthood due to a chronic adaptation of the right heart to volume overload and should, therefore, be considered in the differential diagnosis of right cardiac chambers dilation, especially when other causes do not justify its pathophysiology.
A 20–YEAR FOLLOW–UP OF SUCCESSFUL SURGICAL MANAGEMENT OF DOUBLE–OUTLET RIGHT VENTRICLE WITH PULMONARY STENOSIS: A CASE REPORT
Authors: Giordano M , Molinari L , Mansour D , Ricci F , Renda G , Gallina S , Paloscia L , Di Marco M
Published: May 2024

A 25–year–old man, suffering from worsening exertional dyspnea for 4 months, was referred to our Cardiology Unit. The patient was previously diagnosed with a late diagnosis of double outlet right ventricle (DORV), as well as pulmonary stenosis, VSD, and right–sided aortic arch for which he underwent a first surgical procedure around the age of 2 involving an intersternal anastomosis through a left lateral thoracotomy approach. A second intervention took place 2 years later for a radical correction of the anomaly through a median sternotomy. The patient had sporadic follow–up since then. A baseline ECG was performed, showing evidence of sinus rhythm, normal AV with a bifid P wave in lead II, slight RAD, incomplete RBBB, and asymmetric negative T waves from V1 to V3 and in aVL, and biphasic T waves in V4–V5. We performed an echocardiogram which showed: a dilated RA, a normal–sized LA, an apparently intact interatrial septum without evident shunts; a normal sized LV; EF 60%; a moderately dilated, hypertrophic and hypertrabeculated RV; FAC 33%; normal aortic flow; a minimal MR; a massive residual pulmonary insufficiency; a moderate TR; a dilatated pulmonary trunk; a normal–sized and collapsible IVC upon inspiration; PAPs of 70 mmHg; no pericardial effusion. A maximal exercise stress test appeared to be normal throughout. However, due to the severe pulmonary hypertension he was referred to our PH centre. While these patients are commonly affected by residual early or late left ventricular outflow tract obstruction, fortunately our patient was not. DORV is a relatively common congenital heart defect characterized by an abnormal connection between the ventricles and arteries, in which both the aorta and the pulmonary trunk are connected completely or predominantly to the right ventricle. The anatomical spectrum of this anomaly is quite large, and it can be associated with the presence of a VSD that often extends towards one or both of the arterial valves, allowing the flow of blood from the left ventricle to the aorta. An accurate diagnosis through echocardiography or CMR is essential in order to guide the surgical repair and the appropriate therapeutic approach for individuals affected by this anomaly. A successful surgical correction of DORV is essential, but it is important to be aware of the potential complications of post–operative PH (especially in patients with preoperative PH), which necessitates close surveillance and appropriate therapeutic management.
A RARE CAUSE OF PULMONARY EDEMA, INCREASED AORTIC GRADIENTS AND DECOMPENSATED HEART FAILURE: A CASE REPORT
Authors: Desalvo P , Giordana F , Coppini L , Borzillo I , Ferraro I , Ruffino E , Maiellaro F , De Benedetto Fabrizi M , Cinconze S , Baralis G , Annoni G , Rossini R
Published: May 2024

A 58–year–old male consulted our emergency department (ED) in May 2023 for new–onset severe dyspnea and ankle swelling. Patient history included previous aortic valve replacement (STJ 23 mechanical aortic valve) in 2004 for sub–valvular stenosis due to subaortic membrane. ED transthoracic echocardiogram (TTE) showed a dilated left ventricle (LV) with severely depressed ejection fraction (EF: 25%), LV non–compaction (LVNC), aortic mechanic prosthesis with increased gradients (49/32 mmHg), no perivalvular leaks, dilated pulmonary artery. Increased aortic gradients raised suspicion of aortic prosthetic thrombosis as the cause of acute pulmonary edema. However, urgent fluoroscopic investigation proved normal mobility of the hemidisks (Fig.1). Patient was admitted to cardiac intensive care unit. TTE also showed increased LVOT gradients, suggesting a hyperdynamic condition. Lab tests ruled out hyperthyroidism, sepsis and anemia. RV stroke volume was measured: QpQs was 0.7. Careful search for intra/extra–cardiac shunts with TTE ruled out intra–cardiac shunts, but highlighted a patent ductus arteriosus (PDA). PDA represents a peculiar case in which, despite being a left to right shunt, echo–measured QpQs results <1 as pulmonary flow is measured in the RVOT, upstream of the shunt. CT ruled out pulmonary embolism and coronary artery disease and confirmed the presence of PDA and pulmonary artery dilation. Transesophageal echo confirmed the PDA (Fig.2) and highlighted coronary sinus dilation due to persistence of left superior vena cava (PLSVC). Right heart catheterization (RHC) showed a significant shunt (Qp/Qs 1,57) with increased pulmonary pressure (mPAP 33 mmHg) and pulmonary vascular resistance (PVR, 4,03 WU). Cardiac MRI confirmed LVNC with late gadolinium enhancement in the basal septum. LV function improved (EF 36%, Fig.3) after optimal unloading. Patient was discharged with optimal medical therapy for HFrEF (ARNI, SGLT2i, MRA, BB) and was referred to hub congenital heart disease Center (Regina Margherita Hospital, Torino) for percutaneous duct closure after balloon closure test. Percutaneous closure was successfully performed without any complications. RHC three months after closure showed normalization of PVR (0,9 WU) and pulmonary pressure (mPAP 19 mmHg). To our knowledge, this is the first reported case of subaortic membrane, LVNC, PDA and PLSVC. Patient was successfully treated with percutaneous PDA closure and is currently asymptomatic, class NYHA 1.
OVERCOMING EXTREME MEDICAL–SURGICAL EVENTS, IN CARRIERS OF CRTD AND CCM. CLINICAL STABILIZATION
Authors: Incampo G , Rizzo D , Mallardi A , Bux F , Campagna M , Ruggeri R , Cuonzo M , Valecce R , Moramarco M , Montemurro M , Giannoccaro V , Marino F , Bonfantino M , Pirozzi M
Published: May 2024

A 63–year–old male patient suffering from chronic dilated ischemic heart disease, with severe depression of contractility and estimated EF of 16%, carrier of CRT–D and CCM, in follow up at our center. Extreme events of medical/surgical interest have occurred in the last period: the patient underwent strangulated hernia surgery with ileostomy and subsequent stay in intensive care. Transferred spontaneously to cardiology where he was treated for heart failure also with norepinephrine and levosimendan. Once the CCM was reprogrammed with sensing recalibration and optimal delivery of therapy for 7 hours a day, interspersed with regular breaks, the patient showed a gradual improvement in vital parameters and in general clinical conditions, with more adequate blood pressure and diuresis regimes. The presence of the double device (CRT–D and CCM), together with medical therapy, allowed the patient‘s stabilization and clinical remission, with discharge and transfer to a cardiovascular rehabilitation center. The Cardiac Rhythm Management guaranteed by the CRTD, with optimization of medical therapy, together with the positive inotropic action of cardiac contractility modulation (CCM), allowed the patient to overcome high intensity surgical and medical events.
“A BROKEN TRANSPLANTED HEART”: A CASE OF TAKOTSUBO SYNDROME IN A HEART TRANSPLANT RECIPIENT
Authors: Delle Femine F , Liccardo B , Loffredo F , Pezzullo E , D‘Arienzo D , Amarelli C , Golino P
Published: May 2024

75–year–old female patient went to the emergency department for oppressive chest pain radiating to the left arm and dyspnea, developed after an argument with family members. The patient underwent heart transplant in 2010 because of chronic ischemic heart disease. In history: systemic arterial hypertension, former smoking, dyslipidemia, and chronic renal failure. At blood tests: TnIHS 78.4 ng/L (<29 ng/L), CK–MB 1.9 microg/L, PROBNP–NT 43,000 pg/ml, creatinine 2 mg/dL. ECG showed inverted T waves in DI, AVL and V4 to V6 (fig 1). Transthoracic echocardiography was remarkable for a moderate reduction in systolic function (EF 38%), akinesia of apical portion, mid–segment of the IVS and mid–segment of posterior wall. Coronarography showed epicardial coronaries free of angiographically significant lesions. We decided to not perform ventriculography to avoid worsening renal function. At clinical stabilization, the patient underwent cardiac MRI with evidence of hypokinesia of the mid–ventricular segments with preserved global systolic function and hypo/akinesia of right ventricular apex with endocavitary thrombosis. Tissue characterization images were compatible with the presence of diffuse phlogistic involvement of the left ventricular myocardium (most evident at the mid–ventricular segments), in the absence of areas of myocardial necrosis. On pre–dimission echocardiogram, global left ventricular systolic function appeared markedly improved (auto EF 52%). DISCUSSION Our case is a rare case of Takotsubo syndrome (TS) in a transplanted heart. While TS accounts for 1–2% of all ACS in the general population, there is no precise data on its prevalence in heart transplant recipients. Although the pathophysiological mechanism underlying TS is still debated, there is evidence of hyperactivation of the sympathetic system with excessive catecholamine release. What is particular in our clinical case is that transplanted heart is denervated (because of resection of the post–ganglionic neural axons), although in some patients there is progressive reinnervation starting around the second year. Myocardial scintigraphy with iodine–123 meta iodobenzylguanidine can be used to study reinnervation. In our case, the presence of typical angor may be a clinical indicator of reinnervation, as many transplant patients present with CAV in the absence of chest pain.
SURGICAL REPAIR AND PERCUTANEOUS CLOSURE FOR POSTINFARCTION VENTRICULAR RUPTURE
Authors: Gattari B , Betta D , Gasparetto N
Published: May 2024

Case Report

A 44–year–old male was admitted to ICU for inferior STEMI in cardiogenic shock. The echocardiogram documented pericardial effusion in the right ventricle with initial signs of tamponade and the left ventricle with akinesia of the inferior–posterior wall and thinning of a small lateral portion, as from a pseudoaneurysm. Pericardiocentesis was performed with subsequent restoration of haemodynamic stability. The following emergency coronary angiography showed subocclusive stenosis of a thin postero–lateral branch (culprit lesion), critical stenosis at the origin of the anterior descending artery and occlusive stenosis of a developed diagonal branch. A CT scan documented a parietal breach in the middle of the left ventricle lateral wall with a pseudoaneurysm in close contiguity with the pericardium, in a setting compatible with contained cardiac rupture [Fig.1]. Hence, the patient underwent emergency cardiac surgery: a bovine pericardium patch was placed in the infarcted area, without complications. Later, percutaneous revascularisation on the left main–left anterior descending artery was performed. A few months later the patient was asymptomatic at a cardiological check–up, nonetheless, the echocardiogram revealed aneurysmal dilatation in the middle of the left ventricle lateral wall [Fig.2]; heart CT scan and ventriculography confirmed a pseudo–aneurysmal formation at the site of previous patch placement. Cardiac surgery was unfeasible due to high intra–operative risk, therefore pseudoaneurysm percutaneous closure under radioscopic and transthoracic echocardiographic monitoring was performed, with good final echocardiographic result [Fig.3].

Discussion

Cardiac rupture is a relevant high–mortality complication of acute myocardial infarction which needs to be treated promptly. Early diagnosis plays a crucial role in changing the patient‘s prognosis and is made possible by clinical, echocardiographic, haemodynamic and radiological criteria. The effectiveness of different treatment modalities is still controversial. In the presented case a surgical approach was preferred under emergency settings, while a less invasive percutaneous approach was chosen in the following and more stable situation.
HALF DOSE THROMBOLYSIS IN INTERMEDIATE – HIGH RISK PULMONARY EMBOLISM WITH TRICUSPID VALVE MOBILE MASS: A BAIL OUT STRATEGY
Authors: Roccabruna A , Giatti S , Pastore G , D’Elia K , Carraro M , Conte L , Marcantoni L , Aggio S , Ribichini F , Picariello C , Zanon F
Published: May 2024

A 82–year–old previously healthy male was referred to the emergency department complaining sudden onset dyspnea and witnessed syncope complicated by head trauma. Recurrent dizziness occurred previously. No history or drug consumption noted. On admission: heart rate 90 beats/min, blood pressure 110/60 mmHg, respiratory rate 25 breaths/min, oxygen saturation 87% in room air, normal body temperature. No lower extremities tenderness or swelling reported. Consistent with peripheral blood oxygen saturation levels, arterial blood gas analysis showed hypoxemia and respiratory alkalosis. Laboratory exams were significant for thrombocytopenia (89.000 cells/L), elevated cardiac troponin T levels (222 ng/L) and natriuretic peptide (NT pro–BNP 3944 pg/mL). Head CT excluded acute cerebral hemorrhage. At the electrocardiogram, signs of RV strain were present (biphasic T–waves in V1–V3 leads, S1–Q3–T3). Transthoracic echocardiography (TTE) showed dilated RV with hypokinetic free wall, “D–shaped” LV, “60/60” sign and a mobile filiform mass crossing the tricuspid valve plan in the right atrium (fig. 1). The patient did not present structural valvular abnormalities or endocarditis–related risk factors. CT pulmonary angiography detected a “saddle” PE and extensive thrombosis in inferior vena cava. After unfractioned heparin (UFH) bolus, the patient was transferred to our intensive care unit and balancing risks and benefits the chosen treatment option consisted in half dose systemic thrombolysis (50 mg rtPA in 60’’; 10 mg as loading dose in 2’). Because of potential harmful effects in thrombocytopenia and recent head trauma, UFH was temporarily withheld and started again after 1 hour. The day after, a beside TTE showed complete tricuspid thrombus resolution, a significant decrease in RV/LV ratio and systolic pulmonary artery pressure (fig. 2). Apart from small hematomas, no bleedings were reported. The patient was shifted to the ward on the next day and following course was uneventful. On day 4 a direct oral anticoagulant (Dabigatran 110 mg) was started. Abdominal and inferior limb–deep–venous ultrasound were both negative for pathological reports. A total body CT was scheduled to exclude silent malignancies at discharge. Take home message: in intermediate–high risk PE, half dose systemic fibrinolysis strategy is controversial since its potential benefit is counterbalanced by concerns about major bleedings, but increasing data suggest its efficacy with low risk profile.
FEAR OF SUDDEN DEATH ‘INTERRUPTED‘ BY WEARABLE DEFIBRILLATOR: CASE REPORT
Authors: Valecce R , Carretta D , D‘Agostino C , Bonfantino M
Published: May 2024

A male patient (pt) 49y,grade II familiarity for sudden cardiac death (SCD).He arrived in the emergency department for worsening dyspnea and he underwent ECG:sinus rhythm and left bundle branch block(QRS duration 170 msec);color Doppler echocardiogram (CDE)showing:Dilated left ventricle, severely and globally hypocontractile(EF30%).Negative cardiac enzymes on laboratory tests.The coronary arteries were normal at angiographic control.The pt did not undergo to cardiac MRI because of claustrophobia.Blood samples were sent for genetic screening for cardiomyopathy.The pt started drug therapy following ESC guidelines.He was transferred to cardiopulmonary rehabilitation department.At the end of the 2 weeks of the rehabilitation cycle, left ventricular systolic dysfunction persisted.The pt was discharged with a wearable defibrillator (WCD) to continue therapy optimization following ESC heart failure guidelines.At the end of 3 months,the pt underwent an echocardiographic recheck which confirmed dilated,severely,and globally hypocontractile left ventricle(EF35%).ICD implantation was indicated, but the pt refused it.Therefore,was prescribed the continuation of SCD protection by WCD.The pt underwent monthly followups,for the next 6 months,reporting no improvement in left ventricular contractile capacity measured by CDE and continuing to refuse biventricular ICD implantation.We noted a correct and consistent wearing of the WCD.After 177 days of wearing,the pt had an episode of loss of consciousness and WCD shock.A remote device check documented ventricular fibrillation at 200 bpm,which was correctly recognized and discontinued.The pt accepted biventricular ICD implantation.At follow–up at 1 month CDE documented improvement in left ventricular contractile capacity(EF 40%),and no arrhythmic events in the absence of symptoms.WCDs have been used extensively in Italy since 2015.This technology provides temporary protection from SCD for pts with an evolving risk profile who are not yet eligible for an ICD.WCD is an effective therapy for the treatment of SCD with a very low complication rate and high pt compliance over time.Our case demonstrates how WCD enabled optimal titration of drug therapy following guidelines and prevented sudden death in young high–risk pts.In addition, our case shows that reduced compliance for definitive ICD implantation due to non–acceptance of the disease,fear of surgery,or subsequent lifestyle changes are not barriers to saving patients from SCD.
PERCUTANEOUS CORRECTION OF "BADLY ACCOMPANIED" MASSIVE TRICUSPID REGURGITATION
Authors: Mancini G , Lucarelli K , Bellomo V , Troisi F , Casamassima V , Lombardi F , Inchingolo R , Grimaldi M
Published: May 2024

Background

Tricuspid regurgitation (TR) is a common valvular pathology that impairs quality of life and survival. Tricuspid transcatheter edge–to–edge repair (t–TEER) has shown promising potential in the TR treatment. There remains several uncertainties such as timing of intervention in relation to clinical status, RV function, pulmonary artery pressure and many comorbidities that are often overlooked.

Case Summary

A 80–year–old man with dilated cardiomyopathy, persistent atrial fibrillation and biventricular defibrillator (ICD), was admitted to our centre for congestive heart failure (CHF). Echocardiography showed massive TR, severe right heart dilatation, increased systolic pulmonary artery pressure, severely dilated inferior vena cava (IVC) and hepatic veins with systolic flow reversal. We also found an uncommonly high blood flow coming to the right atrium from the IVC, due to an anomalous vascular communication between an intrahepatic portal branch and a hepatic vein, a so–called intrahepatic portosystemic venous shunt (IPSVS). Although these relatively rare shunts are usually not treated unless symptomatic, after a collective careful evaluation, we decided to proceed to the embolization of the IPSVS with a vascular plug, to make the t–TEER less challenging, safer and more durable. In better general conditions, after 2 months, the patient underwent the transcatheter tricuspid procedure with a single XTW TriClip™ (Abbott). At 3 months follow–up, the patient showed noticeable improvements in QoL, CHF signs and symptoms, echocardiographic parameters and exercise capacity.

Discussion

Appropriate timing of t–TEER is crucial not only to avoid irreversible RV damage and organ failure, but also to increase the success rate of the procedures. In this case we observed that the IPSVS treatment contributed significatively to improve the patient’s clinical status and to reduce the right heart volume overload before attempting the t–TEER. This combined approach facilitated the technical success of tricuspid procedure, that was itself challenging due to the ICD leads in the right chambers.

Conclusion

Patients’ selection and timing of percutaneous TR treatment are not clearly defined. This case shows that a winning strategy to approach challenging TR is to solve, before the tricuspid treatment, all the potentially treatable comorbidities that could negatively interfere with the technical success of the procedure itself and with the durability of TR reduction.
DESMOPLAKIN ASSOCIATED NON DILATED LEFT VENTRICULAR CARDIOMYOPATHY TREATED WITH ANAKINRA
Authors: Pezzullo E , Caputo A , Catapano D , Alfieri R , Covino S , D‘Elia S , Golino P , Loffredo F
Published: May 2024

A 24 year old female patient experienced,from 2010 to November 2023,12 myocarditis–like episodes, often related to emotional stress, characterized by chest pain,increased markers of myocardial necrosis, normal C–reactive Protein values,normal left ventricular systolic function on echocardiogram.She was admitted to our division for the first time in July 2019 because of recurrent acute myocarditis, extensive non–ischemic fibrosis and myocardial edema on magnetic resonance imaging (MRI) and interstitial edema with monocytic lymphocytic infiltrates (CD3 +>7/mm2) and cardiomyocyte necrosis at endomyocardial biopsy.A genetic test was performed leading to the identification of a novel frameshift mutation in the tail C domain of desmoplakin (DSP) producing a truncated protein.Destabilization of desmosome can be associated,to massive troponin release,innate immune activation resulting in autoantibody formation to intracellular cardiomyocyte protein.Mutations in DSP can link to arrhythmogenic cardiomyopathy,characterized by intermittent myocardial inflammatory episodes that appear clinically similar to myocarditis,leading to left ventricular fibrosis and to high incidence of ventricular arrhythmias.Our patient was initially treated with intravenous high dose corticosteroid therapy with oral tapering at discharge but two relapses(December 2020 and April 2021)occurred at corticosteroid withdrawal.In April 2021 immunosuppressive therapy with azathioprine was initiated in association with colchicine and oral corticosteroids,however after three months a new recurrence occurred.In July 2021 off–label Anakinra 100 mg subcutaneously daily was started.Anakinra is an interleukin (IL)–1 beta recombinant receptor antagonist used to treat idiopathic recurrent pericarditis.A number of studies have stressed the possible role IL–1 in the pathogenesis of myocardial inflammation involving inflammasome activation,cardiomyocytes loss ultimately leading to heart failure and arrhythmias.Anakinra showed a good response and tolerability and allowed corticosteroid withdrawal.The patient did not experience any further recurrence until November 2023.This episode was however characterized by milder and shorter chest pain,lower release of troponin in absence of edema on magnetic resonance imaging and inflammation on cardiac (18)F–fluoro–2–deoxyglucose positron emission tomography suggesting that modulation of cardiac inflammation may be crucial also when associated with pathogenic mutations.
PREGNANCY IN DANON’S DISEASE: WHAT WE KNOW ABOUT THERAPY IN THIS PARTICULAR SUBGROUP
Authors: Lampus M , Sanna S
Published: May 2024

A twenty–seven year old pregnant woman in her twenty–eighth week, suffering from Danon‘s disease, goes to the emergency room due to inappropriate shock of the ICD on high frequency atrial tachyarrhythmia. Three days before admission, Nadolol was replaced with Metoprolol due to difficulties related to obtaining the drug; since then the patient develops episodes of palpitations and chest pain.The medical history includes Hashimoto‘s thyroiditis, obesity and a family history of hypertrophic cardiomyopathy and CAD. The 12–lead ECG shows arrhythmia due to atrial fibrillation at a rate of 100 bpm, LBBB with secondary repolarization changes. Device interrogation revealed episodes of high frequency atrial fibrillation and atrial flutter. The ECOCG shows a non–dilated left ventricle with slightly increased thicknesses at the level of the interventricular septum and apex, with slight apical hypokinesia and preserved systolic function indices. During hospitalization, the patient‘s condition is stabilized and she always presents with good hemodynamic compensation. Nadolol is reintroduced together with anti–coagulant therapy with low molecular weight heparins. Danon‘s disease represents a rare phenocopy of hypertrophic cardiomyopathy and atrial fibrillation is of particular interest in this population subgroup. Pregnancy is a hypercoagulable state and AF is an arrhythmia associated with significant thromboembolic complications. Patients with hypertrophic cardiomyopathy that develop atrial fibrillation are especially at a higher risk of thrombosis. Furthermore, exposure to beta–blockers during pregnancy has been associated with intrauterine fetal growth restriction, preterm birth and perinatal mortality. There is a non–negligible risk connected to poor fetal growth, but the risk/benefit ratio leans in favor of maternal electrical stability, which, if lacking, would still cause damage to the fetus due to hypoperfusion phenomena.
A CASE OF SYNCOPE
Authors: Romano G , Carmina M , Bonni E , Minà C , Bellavia D , Vaccaro P , Panzarella G , Pieri M , Polizzi V
Published: May 2024

A male patient aged 20 years old who presented exertional syncope was admitted to the hospital.

At presentation patient was awake and cooperative. Hemodynamic was stable with normal gas exchange and respiratory pattern. No focal neurological signs were found. Physical exam and blood tests were normal. Electrocardiogram showed normal sinus rhythm, short PR intervals with signs of ventricular preexcitation, left ventricular hypertrophy (LV), like a saddleback ST elevation in leads V1–V2 and several ST–T changes in multiple–lead, T wave inversion. No extracardiac features were found. The echocardiography showed features of hypertrophic LV, without LV out flow track obstruction and normal systolic function. There was “ground glass’ appearance of the myocardium and global longitudinal strain reduction and "apical sparing". Analysis of dry blood drops ruled out Fabry disease. Cardiac magnetic resonance showed marked asymmetric septal hypertrophy with late gadolinium enhancement septal impregnation. Waiting for the results of genetic tests, the diagnosis of hypertrophic cardiomyopathy (HCM) has been made. Electrophysiological studies were performed and a ventricular tachycardia was induced; accordingly, a subcutaneous defibrillator was implanted. A heterozygous ALPK3 mutations (del_14bp, rs1466450842) and a heterozygous SCN5A (missense variation (p.Arg 225 Trp)), inherited from the mother and father respectively, were detected in the proband. The clinical management was very complex because progressive myocardial hypertrophy is one of the main characteristics of cardiomyopathy related to the ALPK3 gene and furthermore, mutations in SCN5A are associated with severe cardiac conduction disorders and degenerative alterations of the conduction system. To the best of our knowledge, this is the first reported case simultaneously reporting both mutations.

Conclusions

Diagnosis of cardiomyopathy with hypertrophic phenotype is still a challenge. Precise diagnosis depends on multiparametric approach including molecular genetic techniques.
A LATE PRIMARY CARNITINE DEFICIENCY DIAGNOSIS IN ADULTHOOD DURING ARRHYTHMIC RISK REASSESSMENT IN HYPERTROPHIC CARDIOMYOPATHY
Authors: Ciampi C , Abete R , Vittori C , Sebastiani R , Iorio A , Mancinelli A , De Filippo P , Terzi A , Senni M , Iacovoni A
Published: May 2024

Background

Hypertrophic cardiomyopathy (HCM) is the most common inherited heart disease, typically caused by mutations in cardiac sarcomere protein genes. Inborn errors of metabolism are a rare cause of this condition, usually diagnosed in infancy due to severe heart failure and concurrent extracardiac manifestations.

Clinical Case

A 36–year–old female patient, diagnosed with non–obstructive HCM at the age of 9 due to heart failure, was treated with beta–blockers and ACE inhibitors. During the follow up she remained stable in NYHA class I, family screening was positive but genetic analysis has never been performed due to parents and patient’s refusal. At the latest echocardiographic evaluation (August 2023), a slight reduction in ejection fraction (from 50% to 40%) was observed, with akinesia of the IVS and the inferior wall, in the presence of known asymmetric hypertrophy. Based on this finding, a CMR was performed, which compared to the previous one conducted 3 years earlier, confirmed the reduction in ejection fraction and showed an increase in left ventricular volumes and a significant worsening of LGE areas. Additionally, a cardiopulmonary exercise test was performed, revealing a reduced VO2max of 15 l/min. To assess the risk of sudden cardiac death, an HCM Risk–SCD calculation was performed, yielding a result indicating a mild risk (3.19%). Considering the rapid disease progression, a family history of sudden cardiac death, and increased isolated ventricular extrasystoles from recent Holter monitoring, a S–ICD was implanted. Simultaneously, the patient agreed to genetic testing, revealing an exon 5 SLC22A5 gene mutation, suggesting autosomal recessive primary carnitine deficiency, a rare metabolic disorder usually causing early heart failure and several extracardiac manifestation, none of which the patient exhibited. In our case, uncovering this finding leads to two significant therapeutic implications: a major appropriateness of implanting an ICD, as the HCM Risk–SCD is not applicable in metabolic forms of HCM, and the need to initiate oral carnitine supplementation therapy to halt the progression of the disease. This evidence underscores the importance of personalised arrhythmic risk assessment in HCM patient evaluations and the necessity to exclude atypical metabolic causes of HCM, even if unlikely, given the significant therapeutic implications that may arise.