bluuebunny/crossref_metadata_2025 huggingface dataset:
A RARE GUCH AND PULMONARY HYPERTENSION: A SHORT STORY
Authors: Saraullo S , Palermi A , Ricci F , Renda G , Gallina S
Published: May 2024

A 44–year–old woman presented to emergency department with dyspnea and anasarca. No past history of cardiologic diseases. Physical exam was notable for jugular turgor, hepatomegaly, rales and tachy–arrhythmic tones. Laboratory tests showed severe anemia (Hb 3.2 g/dl) and a severe mixed acidosis. ECG showed atrial fibrillation, 150/min. On echocardiogram left ventricle had normal size and systolic function while right chambers were dilated, hypokinetic (TAPSE 14 mm, RVFAC 30%), systo–diastolic D–shape, severe tricuspid regurgitation; high probability of pulmonary hypertension (VRT 3.2 m/s, and accessory signs). Angio–CT chest was performed, ruling out pulmonary embolism. She was admitted with a working diagnosis of right heart failure complicated by respiratory failure, intercurrent undated AF and severe pulmonary hypertension. On a routinal echo aorto–pulmonary shunt and ASD and coronary venous sinus dilatation were reavaled. A cardiac–CT was performed, for suspected congenital heart disease, and showed ASD sinus venosus (Fig.1), patency of Botallo‘s duct (PDA), aortic coarctation (Fig.2) and persistence of left superior vena cava (PLSVC, type III) draining into coronary sinus (Fig. 3). The patient‘s clinical condition deteriorated progressively until she died from cardiac arrest on the fifth day from the admission, preventing further investigations. Our patient was a so called GUCH with ASD and PDA resulting in group I (WHO) pulmonary hypertension due to a prevalent left – right shunt (Simonneau et al). The clinical presentation, on the other hand, could be due to the onset of atrial fibrillation leading to hemodynamic instability and thus reversal to right–to–left shunt (Eisenmerger‘s Syndrome) with multiple organ involvment facilitated by anemia. In our case, it is unclear, as well as in the literature, whether PLSVC play a role in the development of pulmonary hypertension; further investigation (MRI, autopsy) would have allowed a better anatomo–functional characterisation excluding a possible unroofed coronaric sinus with left atrial drainage. In our opinion, it is important to recognize PLSVC not only for its implications during interventional procedures but as markers of associated congenital heart disease.
NEW ONSET OF PULMONARY HYPERTENSION IN A PATIENT AFFECTED BY RECURRENT LUNG CANCER
Authors: Pezzullo E , Del Giudice C , Cappelli M , Natale F , Cimmino G , Ciaramella F , Golino P , Loffredo F
Published: May 2024

39 years old non–smoker female patient came to our observation in July 2023 complaining dyspnea on mild exertion. She had a history of lung adenocarcinoma with epidermal growth factor receptor (EGFR) positive for exon 19 deletion treated with left pneumonectomy and ipsilateral hilar mediastinal lymphadenectomy 5 years earlier, and contralateral disease progression currently treated with chemotherapy. Transthoracic echocardiogram showed circumferential prepericardial effusion requiring pericardiocentesis with transient clinical benefit. Indeed, after a month, she presented with worsening dyspnea and echocardiographic evidence of dilated and hypokinetic right ventricle and severe pulmonary hypertension associated with distal right pulmonary artery stenosis secondary to extrinsic compression by the neoplastic mass as shown by the chest CT scan (fig 1). A right heart catheterization confirmed this finding (minimum diameter of right pulmonary artery of 6 mm, maximum systolic pulmonary blood pressure of 99 mmHg) and a palliative stenting of the distal portion of the right pulmonary artery was performed with resolution of the stenosis, reduction of the pulmonary gradient (fig 2 and 3) and resolution of the dyspnea.
PROMINENT CRISTA TERMINALIS AND ATRIAL FLUTTER: CASE REPORT
Authors: Cariello F , Bencardino G , Ventura G
Published: May 2024

Introduction

Crista terminalis is a fibromuscular ridge at the posterolateral region of the right atrium (RA). Prominent crista terminalis can mimic pathologic RA mass on transthoracic echocardiograms. Transesophageal echocardiography can be used to differentiate non pathologic structures from pathologic ones.

Case Report

A 51–year–old men was referred to our clinic with typical atrial flutter. Transthoracic echocardiogram (TTE) revealed a RA mass. It was round, 15 mm in diameter, immobile, not calcified, and located at the posterior region of RA. Transesophageal echocardiography (TEE) showed prominent crista terminalis superiorly located beneath superior vena cava in the RA at 91 degrees midesophageal position. The patient undergoes catheter ablation for typical atrial flutter.

Discussion

Several congenital structures and normal variants such as Chiari’s network, eustachian and thebesian valves, and atrial septal aneurysms may simulate pathologic RA masses such as RA thrombus or myxomas. However, in this case, an anatomical variant is associated with an arrhythmia that requires treatment.

Conclusion

In this case we want to emphasize that prominent crista terminalis can mimic RA mass and may lead to atrial arrhythmias. TEE can be used to differentiate normal structures from pathologic.
CAN A “BROKEN HEART” SUCCESSFULLY BE TRANSPLANTED? A CASE REPORT
Authors: Mazzone F , Giovannico L , Fischetti G , Parigino D , Savino L , Silva A , Di Bari N , Bottio T
Published: May 2024

In January 2023 a 59–year–old woman with dilated ischemic cardiomyopathy, was admitted to our Cardiac Surgery Center for a heart transplant eligibility study. She presented symptoms of dyspnea and in the last 6 months was admitted four times to the hospital for heart failure. An echocardiogram showed a severely biventricular hypocontractility (LVEF: 22%; TAPSE 11 mm). After one month, the patient was urgently admitted to our Intensive Care Unit for cardiogenic shock. ECMO VA was implanted percutaneously. Therefore, the patient was placed on the national emergency waitlist for transplantation. A potential donation was identified ten days later. The donor was a 58–year–old woman with no cardiovascular risk factors, had died of a rupture of a cerebral aneurysm. During hospitalization, echocardiography showed LVEF of 30% with akinesia of the middle and apical wall and hyperkinesia of the basal with a mild improvement in contractility (LVEF: 40%) two days later. Coronary angiography was normal. The patient‘s condition resembled Takotsubo syndrome. Virtual and subsequently cytotoxic crossmatch was negative. We proceeded with orthotopic heart transplant. No noteworthy complications occurred during the operation. She received anti–thymocyte immunoglobulin for immunosuppression induction and prednisone, tacrolimus and mycophenolate mofetil for maintenance therapy. The echocardiogram, performed few days later, revealed preserved contractile function in both ventricles (LVEF: 58%). The patient was discharged after the first four endomyocardial biopsies displaying no signs of rejection (ISHLT’04 0R). At ten–month follow–up, the patient is in good clinical condition with no substantial changes during the follow–up echocardiogram. Brain injuries, including subarachnoid haemorrhage, traumatic brain injury and stroke, have been extensively documented in the literature to cause a surge in catecholamines, with a direct effect on the heart, resulting in a transient myocardial dysfunction commonly referred to as ‘Takotsubo cardiomyopathy‘ or ‘broken heart syndrome‘. Studies have shown that dysfunctional hearts at the start of transplant screening have normal contractile function at the time of organ retrieval have similar outcomes to those without dysfunction. This knowledge may help to increase the pool of potential donor hearts and improve the availability of hearts for transplantation, which is crucial given the shortage of suitable donor organs.
“OPS… I DID IT AGAIN”: ANOTHER CASE OF COEXISTENCE OF CORONARY ARTERY DISEASE AND CORONARY ARTERY BRIDGING IN A PATIENT WITH EXERCISE–INDUCED ISCHEMIA. WHEN A SINGLE TEST MIGHT NOT BE ENOUGH
Authors: Campana N , Mapelli M , Nava A , Pezzuto B , Vignati C , Campodonico J , Teruzzi G , Giulia S , Mani N , Montisci R , Cadeddu Dessalvi C , Agostoni P
Published: May 2024

Introduction

To investigate the presence of significant coronary artery disease (sCAD), the clinician may resort to the use of anatomical or functional tests. When multiple factors contribute to the genesis of ischemia, a single test may not be enough, and an integrated approach is essential.

Clinical Case

74–year–old man, athlete, has been complaining dyspnoea after intense exertion for 3 months. Rest ECG and echocardiogram were unremarkable. A cardiopulmonary exercise test (CPET) showed excellent functional capacity (peakVO2 24 ml/Kg/min, 113% of predicted value) without signs of ischemia; however, the test was stopped early due to high blood pressure (250/120 mmHg) and therefore submaximal (peak RQ 0.95). Antihypertensive therapy was set. A coronary CT showed sCAD. The coronary angiography revealed significant stenosis of the middle circumflex artery and significant stenosis of the distal right coronary artery; both lesions were treated with angioplasty and stent implantation. Due to symptoms persistence, we performed a new CPET. The test was maximal (peak RQ 1.13), confirming an even better functional capacity, but highlighting the presence of inducible ischemia (O2–pulse downsloping and VO2/W flattening). A new coronarography showed good angiographic result. The results prompted our team to revise the anatomic tests, leading to the finding of intramyocardial course of anterior interventricular artery. Verapamil 80mg t.d.s. was set. Before discharge, a CPET was repeated: the test was maximal (RQ 1.1) and showed attenuation of signs of ischemia at high load. Following discharge, the patient reported well–being during regular follow–up.

Conclusions

our case highlights how the integration of anatomic and functional diagnostic tests (performed during a proper physical exercise), plays a key role in the management of suspected myocardial ischemia. The clinical case also shows how CPET is a valuable tool for the detection of myocardial ischemia only if performed reaching the maximality criteria.
STRESS ANGINA IN A PATIENT WITH ANOMALOUS ORIGIN OF THE LEFT CORONARY ARTERY AND INTRAMYOCARDIAL COURSE: A RARE DANGER
Authors: Fiorella A , Burattini O , Ganimede M , D‘Alessandro G , Rodio G , Luzzi G
Published: May 2024

Background

The anomalous origin of coronary artery (AOC) still represents one of the most intriguing conundrumsin cardiology. It is generally diagnosed during coronary angiography performed for other reasons and 85% of the cases are benign. Nonetheless, they can sometimes cause angina, coronary syndrome, and sudden cardiac death.

Clinical Case

We describe a rare case of a 50–year–old dyslipidemic man, overweight, with arterial hypertension (AH) reporting episodes of stable angina when performing physical activities for several months. On the EKG a negative T wave in the lateral site was noticed, therefore an echocardiogram was ordered, which showed heart disease with moderate left ventricular hypertrophy, without obstruction of the left ventricular outflow tract. Notably, the patient already underwent cardiac magnetic resonance imaging (CMR) in another center, in the suspicion of initial hypertrophic cardiomyopathy. However, to us, the hypertrophy seemed to be rather related to the previously unknown AH, which probably remained undiagnosed for years. Coronary angiography showed an anomalous origin of the left coronary artery from the right sinus of Valsalva. On CT coronary angiogram the anterior descending artery (ADA), after its anomalous origin, described an interventricular, subpulmonary course, becoming intramyocardial for a stretch of approximately 39 mm, consequently undergoing a severe narrowing of caliber. Hence, stress CMR was performed with dobutamine and resulted positive for inducible ischemia. We proceed with interseptal cardiac surgical correction, cautiously releasing the ADA from the endocardium and overlying myocardium (coronary debridging).

Discussion and Conclusions

In clinical practice, it is difficult to establish a causal relationship between symptoms and AOC, as this represents a rare finding. In our case, an intramyocardial course was also identifiedand was responsible for both systolic and diastolic compression by the hypertrophic myocardium. The symptoms had begun to appear when myocardial hypertrophy due to untreated AH had become significant. Following the European guidelines, the treatment of AOC with evidence of ischemia should be surgical. We believe it is of fundamental importance in the presence of AOC to always suspect and exclude the presence of any coronary compressions, responsible for ischemia and arrhythmic events.
NOT JUST A SIMPLE BIRTH: SPONTANEOUS CORONARY ARTERY DISSECTION IN A YOUNG MOTHER
Authors: Caprio M , Ottaviani A , Ricci F , Renda G , Gallina S
Published: May 2024

A 33–year–old Caucasian woman with no cardiovascular history was admitted to the gynecology department due to a threat of preterm birth. During hospitalization, the patient experienced chest pain complicated by ventricular fibrillation. Resuscitation maneuvers were performed, followed by an emergency cesarean section with fetal extraction and DC shock, resulting in return of spontaneous circulation. Coronary angiography revealed spontaneous dissection of the first marginal branch, treated with conservative strategy. After 3 days, another episode of chest pain occurred with evidence of inferolateral STEMI. Emergency coronary angiography showed severe stenosis in the proximal segment of the circumflex branch and the mid–segment of the anterior descending branch; OCT confirmed spontaneous coronary dissection (SCAD) in these vessels. A conservative strategy was adopted, with medical therapy including DAPT for a year, ACE inhibitor and beta–blocker. After 1 month, a follow–up OCT of the coronary arteries showed almost complete resolution of the previously observed dissections. 7 years later, the patient presented to the emergency room with chest pain similar to the previous episode. A diagnosis of NSTEMI was made and the patient was admitted to the cardiac intensive care unit. Coronary angiography revealed spontaneous dissection of the right coronary artery causing occlusion in the mid–segment. Coronary angioplasty guided by IVUS was performed with a drug–coated balloon achieving TIMI 3 flow. Given the patient‘s medical history and suspicion of fibromuscular dysplasia, further diagnostic evaluation was conducted with CT angiography of the intracranial circulation, revealing double focal dissections in the both carotid arteries asymptomatic and without flow obstruction. The patient was discharged, prescribed SAPT and scheduled for follow–up with coronary and intracranial circulation CT angiography. SCAD is defined as an epicardial coronary artery dissection that is not associated with atherosclerosis. It commonly occurs in patients with few traditional cardiovascular risk factors. Its cause is hypothesized to be multifactorial with contributions from underlying arteriopathies, hormonal influences or systemic inflammatory diseases, often compounded by environmental precipitants or stressors. The abstract emphasizes the challenges of diagnosis, limited understanding of pathophysiology and the absence of well–defined guidelines regarding the treatment of SCAD.
MACARONI SIGN AND TAKAYASU ARTERITIS IN A PATIENT WITH TUBERCOLOSIS: AN EASY TOOL FOR DIAGNOSIS
Authors: Mazzella F , Weisz S , Guarino A , Verde R , Sorbelli C , Fele N , Severino S
Published: May 2024

Takayasu arteritis (TKA) is a chronic systemic autoimmune disease characterised by inflammation of the aorta and major branch vessels. Carotid Artery (CA) lesions in TKA, known as the ‘macaroni sign’, are characterised by global thickening of the common CA wall and lumen narrowing. There has been speculation that there is a link between TKA and tuberculosis (TB). Both illnesses cause arterial wall changes. We describe a TKA case diagnosed based on the presence of the ‘macaroni sign’ on CU in a young Asian women 30 years old with a diagnosis of latent TB. She has not previous histiry of disease and visited the hospital with complaints dyspnoea and carotidynia. At admission, body temperature, blood pressure and heart rate were 36.8°C, 112/60mm Hg on her right arm (left, 84/60 mmHg) and 76 beats/min, respectively. The pulse in her left radial artery was not palpable. Physical examination revealed spontaneous pain and tenderness on both sides of the patient’s neck. Blood test results demonstrated an elevated inflammatory response C–reactive protein, 8.0 mg/dL (normal range: less than 0.5 mg/L); erythrocyte sedimentation rate (ESR), 120 mm/hour. ANA, ENA and ASMA profile were negative. Quantiferon TB gold was positive. We suspected TKA and performed a CU, which revealed marked thickening of the intima/media of the bilateral common CAs and a positive macaroni sign (figure 1, Panel A and B). After a detailed evaluation of the systemic vessels the patient was diagnosed with TKA Type I limited to Branches from the aortic arch. The FDG–PET/CT demonstrated an increase in the glucose uptake in thoracic aorta, subclavian and right CA (SUV 4.5). The patient was treated with oral steroids at a dose of 0.8mg/kg and Isoniazid. After 6 months she showed a disappearance of carotidynia and dyspnoea, with a reduction in C–reactive protein, 1.5 mg/ dL and ESR=39 mm/hour. The FDG–PET/CT demonstrated a reduction in the glucose uptake in thoracic aorta, subclavian and right CA (SUV 3.5). Although MRI is usually recommended for the definitive diagnosis, the ‘macaroni sign’ identified by CU is a low–cost, simple and non–invasive method that can be easily performed during initial hospital visits. In addition, a clear correlation between MRI and CT angiography findings and disease activity or progression has not been found for TKA yet. Therefore, methods using ultrasound in combination with ESR should be considered easy and useful for diagnosis and clinical follow–up.
CONSTRICTIVE PERICARDITIS DURING SARS-COV-2 INFECTION: A CASE REPORT
Authors: De Maio J , Caputi A , Martelli A , Campisano M , Ceresoli P , Bagni E
Published: May 2024

In our Department we witnessed numerous cases of pericarditis at the end of summer 2023, including that of a 44-year-old patient suffering from rheumatoid arthritis with breath-taking chest pain and fever. Objectively no rubbing, but mild-moderate pericardial effusion. On the EKG diffuse anomalies of ventricular repolarization; increased inflammation indices with negative troponin; positive Sars-Cov-2 PCR. During hospitalization, despite the improvement of clinical parameters, dyspnea persisted. On the control echocardiogram, disappearance of the pericardial effusion, but thickening of the pericardial sheets associated with a "notch" of the interventricular septum and an anomalous Doppler profile in the suprahepatic vein. These findings were subsequently confirmed by cardiac MRI, with diagnostic criteria for constrictive pericarditis. On the basis of these data, therapy with high doses of NSAIDs and colchicine was established. The patient is currently followed periodically by our clinics and feels well. In the literature, constrictive pericarditis is generally secondary to chronic conditions; in this case the contribution of Sars-Cov-2 infection and rheumatoid arthritis cannot be excluded.
ARRHYTHMOGENIC RIGHT VENTRICULAR CARDIOMYOPATHY AND CARDIAC SARCOIDOSIS: THE GREY ZONE
Authors: Gualini E , Palazzini M , Giannattasio C , Garascia A , Ossola P , Gentile P , Ghidini S
Published: May 2024

Cardiac sarcoidosis (CS) is a rare multisystem disorder characterized by granulomatous infiltration of the myocardium, whose clinical manifestations can be extremely heterogeneus. We report the case of a 47–year–old man with a previous diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVD). In history he had a marked arrhythmic pattern with multiple episodes of sustained ventricular arrhythmias, biventricular disfunction (LVEF 37%, RVEF 32%) and extensive biventricular transmural late–gadolinium enhancement (greater than 50%) at cardiac RMN. At EKG epsilon wave mainly seen in precordial leads. In November 2022 he was implanted with bicameral defibrillator and in March 2023 he underwent ablation procedure for ventricular tachycardia with RF with endo–epicardial approach. In May 2023 he arrived at our Centre for acute decompensated heart failure. At restored hemodynamic stability, screening for the possible inclusion in transplant list was started. Right heart catheterization (RHC) showed low cardiac index with no pulmonary hypertension. Chest CT unexpectedly showed the presence of multiple micronodules with perilymphatic distribution with confluent appearance compatible with pulmonary sarcoidosis. In consideration of this result, the transplant program was temporarily postponed and investigations for possible cardiac involvement of an inflammatory nature were started. 18F–FDG PET–CT showed pulmonary, splenic, lymphonodal and especially cardiac uptake in the mid–distal segment of the lateral wall of the left ventricle and in the inferior–apical site suspicious for inflammatory pathology. Unfortunately, both lymphonodal and cardiac biopsies were inconclusive because of insufficient material. Nevertheless, the clinical scenario satisfied the Japanese criteria for possible CS; therefore, treatment with prednisone 1 mg/kg was started and a 18F–FDG PET–CT was planned after 3 months of steroid therapy. CS can disguise as a phenocopy of arrhythmogenic right ventricular cardiomyopathy because of several common characteristics, especially arrhythmic presentation. Due to these similarities, a careful overall evaluation of each patient with ARVD is mandatory as several transplant centers have reported cases of CS in explanted heart with a pretransplant misdiagnosis of ARVD or dilated cardiomiopathy. Performing a chest CT in all ARVD patients as part of transplant screening may be considered.
A RARE CASE OF TYPE A AORTIC DISSECTION PRESENTING AS ACUTE STROKE: A DIAGNOSTIC CHALLENGE
Authors: Saraullo S , Rizzuto M , Costantini D , Ruffo F , Corazzini A , Di Marco M , Renda G , Ricci F , Gallina S
Published: May 2024

A 60–year–old woman presented to the emergency department with acute left hemiplegia, not chest pains. Past medical history was significant for family history of aortic aneurysm, arterial hypertension. At brain–CT was reported hypodensity areas in posterior arm of the right internal capsule and in right tempero–polar lobe, confirmed on brain–MRI, and compatible with acute ischemic injury. Echocolor–dopper of epiaortic vessels documentated ulcerated plaque into the right common carotid artery (CCA). Selective catheterization found focal dissection obstructing the flow, treated with self–expanding stent. On echocardiography evalutation for suspiction of TAAD: dilatation of ascending aorta with flap oscillation, severe arotic regurgitation, pericardial effusion (Fig. 1). CT–Angiography was performed with evidence of type A dissection extending superiorly to the right CCA and inferiorly to the iliac bifurcation. For coma state, the patient was mechanically ventilated, and seriate CTs showed new ischemic lesions and hemorrhagic infarction of previous. Because of contraindication for cardiac surgery due neurological state, the patient was referred at medical therapy as bridge to hybrid aortic arc substitution at resolution of intracranial hemorrhages. In the next days there was progressive improvement neurological status with restoration of sensory and autonomic breathing with hemodynamic stability. Aortic dissection (AD) is defined as a disruption of the medial layer leading to the formation of two lumens separated by an intimomedial flap. AD is a critical diagnosis to make due to life–threatening outcomes and its non–specific presentation as in the case of our patient where the management of AD was delayed due to an unusual initial clinical presentation. Multimodality imaging approach has been fundamental for correct diagnosis and risk stratification. A limited number of studies have reported cases with stroke as the first symptom with concomitant TAAD. Concomitant intracerebral hemorrhage and TAAD is a very rare presentation and the optimal timing of surgery in these patients is unknown. In our opinion, patients who presents acute stroke should be carefully evaluated using multimodal imaging to exclude TAAD before administering reperfusion therapy. In addition, performing CAS before surgery for TAAD is challenging but might be a valid treatment option to maintain good brain perfusion and improve prognosis, but further investigations are needed.
SEVERE MITRAL REGURGITATION TREATED WITH TRANSCATHETER AORTIC VALVE IMPLANTATION IN MITRAL RING (TMVIR): A CASE REPORT
Authors: Vitale C , Giannini C , Costa G , Petronio A , De Carlo M
Published: May 2024

Introduction

The increasing prevalence of mitral regurgitation (MR), combined with a growing population of elderly patients at risk of being unsuitable for surgical options, has led to a greater interest in the use of less invasive treatments such as transcatheter heart valve (THV) implantation. This case involves the use of transcatheter aortic valve implantation in a patient with a previous mitral annuloplasty (TMViR).

Case Presentation

A 77–year–old woman had previously undergone mitral annuloplasty (Physio Ring II 32 mm, Medtronic), Neocord implantation and closure of the posterior commissure for severe MR due to anterior leaflet prolapse. She presented to our attention with worsening dyspnea (NYHA Class III) and a sudden weight gain. Transesophageal echocardiogram (TEE) revealed findings indicative of a previous mitral annuloplasty failure, with residual severe MR along with severe tricuspid regurgitation (TR). The case was discussed in the Heart Team and given the elevated surgical risk and the patient‘s anatomical unsuitability for edge–to–edge therapy, it was decided to perform a TMViR implantation. Under echocardiographic guidance and general anesthesia, right common right femoral vein access was obtained. After transseptal puncture, predilation of the interatrial septum was performed with balloon (Evercross 12 mm, Medtronic). Subsequent access to the left ventricle was achieved with a 6F PigTail catheter, and a stiff exchange guide (Safari S, Boston Scientific) was used to advance the 16Fr Delivery system. With the assistance of TEE, aortic valve implantation on the mitral ring was performed (Sapien 29 mm, Edwards Lifesciences) with rapid pacing via transvenous temporary PM placed in right ventricle (Spikeflow, Fiab). At the end of the procedure, two closure systems (Proglide, Abbott) were used for right femoral vein access. Post–implantation TEE showed a trivial paravalvular leak external to the annulus and a mean transvalvular gradient of 5 mmHg. The postoperative course proceeded without complications, facilitating the patient‘s discharge on the third postoperative day. Currently no further hospitalization in the last 6 months.

Conclusion

Despite surgical intervention remaining the gold standard in treating severe MR in patients who have undergone a previous annuloplasty, in patients at high surgical risk for a re–operation, who are not eligible for other percutaneous options, TMViR could be a safe and effective option for treatment.
ACUTE MYOCARDIAL INJURY IN A PATIENT WITH TYPE–1 NEUROFIBROMATOSIS: A CASE OF SUSPECTED PHEOCROMOCYTOMA–ASSOCIATED MYOCARDITIS
Authors: Cingolani M , Cannillo M , Suppo U , Almohani A , Renon F , Troiano F
Published: May 2024

A 54–year–old patient with an history of type–1 neurofibromatosis presented to the emergency department (ED) due to recurrent abdominal pain with diarrhea. Clinical examination didn’t show anything remarkable. She was mildly hypertensive, apyretic. During her staying in the ED, she complained about chest pain and an electrocardiogram (ECG) was performed. ECG showed sinus rhythm, normal heart rate, left ventricular (LV) hypertrophy with marked horizontal ST segment depression in the precordial leads and bimorphic ventricular ectopic beats with prevalent right bundle branch block superior axis morphology, likely from the medio–basal inferior LV. Troponin T (peak 958 ng/L, normal <15 ng/L) and NTproBNP (peak 2357 ng/L, normal <180 ng/L) were elevated, hemoglobin, creatinine and liver function were normal, C–reactive protein peaked at 120 mg/dL. Echocardiogram showed mildly reduced left ventricular ejection fraction (LVEF) with inferior and inferolateral hypokinesia and moderate mitral regurgitation (MR). She was admitted to cardiac intensive care unit with the suspected diagnosis of an acute coronary syndrome and was started on acetylsalicylic acid, metoprolol, furosemide and potassium canrenoate. Amiodarone was added due to frequent ventricular tachycardias. Due to the symptoms and the history of neurofibromatosis a whole body computed tomography (CT) was done. CT showed a 53 mm mass on the left adrenal gland with intense enhancement, suspected for a neoplastic origin (Figure 2). Plasmatic and urinary cathecolamines and metanephrines were increased. Positron–emission tomography (PET)/CT with 68Ga showed left adrenal uptake, compatible with a neuroendocrine tumor. Coronary arteriography showed normal coronary arteries. The patient underwent a cardiac magnetic resonance (CMR) study in the suspect of acute myocarditis. CMR showed normal biventricular function, an inferior basal hypokinesia with focal intramyocardial late gadolinium enhancement (LGE) suspected for a post–inflammatory origin and mild pericardial effusion. After pharmacological preparation a laparoscopic left adrenalectomy was performed, and histology confirmed the diagnosis of a composite pheocromocytoma. Three months after, the patient was asymptomatic, biochemistry showed normalization of both troponin T and endocrinological biomarkers, the echocardiogram showed preserved LVEF without areas of hypokinesia and mild MR. A follow–up CMR at six months from the acute injury has been scheduled.
PAPILLARY FIBROELASTOMA: AN UNUSUAL CAUSE OF TRANSIENT ISCHEMIC ATTACK
Authors: Lorenzini B , Palazzolo M , Carlà A , Occhiuzzi E , Sciarra L , Romano S
Published: May 2024

Papillary fibroelastomas (PFEs) are small, avascular benign tumors. Despite that, PFEs could lead to important clinical consequences such as transient ischemic attack and myocardial infarction. A 65–year–old female patient was admitted to our ER presenting a transient ischemic attack characterized by retrograde amnesia lasting about an hour. She had a history of systemic scleroderma and left breast cancer treated with local radiotherapy. In our hospital she underwent a neurological examination and a head CT which were negative for ongoing acute pathologies. An electrocardiogram was performed which showed sinus rhythm at 65 bpm. A transthoracic echocardiogram was also performed revealing a floating mass arising from left ventricular outflow tract between aortic and mitral valves. The transesophageal echocardiogram confirmed a solitary lesion approximately 2 centimeters long localized between A2 and A3 mitral valve scallops. The patient was therefore referred for surgery. During surgery the mass was found to arise from anterior mitral annulus and was resected. The histopathological examination of the mass confirmed the morphologic diagnosis of papillary fibroelastoma. Histologically PFEs are made up of a single layer of endocardial cells covering a matrix of collagen. The main risk factors for the development of PFEs are iatrogenic causes, such as radiotherapy, and autoimmune diseases such as systemic scleroderma. In 54% of cases PFEs are asymptomatic at the time of diagnosis. Since over 95% of PFEs present on the left side of the heart, systemic embolism is the most frequent presentation typically as transient ischemic attacks. Initial evaluation is accomplished with echocardiography. PFEs appear as echo–dense and pedunculated masses. Cardiac computed tomography and magnetic resonance imaging are second line modalities in PFEs evaluation. Definitive diagnosis of PFE requires pathological confirmation. Surgical resection remains the treatment of choice for all symptomatic patients. For symptomatic patients who are not surgical candidate, long term antiplatelets or anticoagulation therapy should be initiated. Asymptomatic patients should have surgical resection if the size of the tumor is > 1 cm. PFEs are associated with significant morbidity and mortality. Echocardiography remains the most utilized diagnostic modality. Surgical treatment should always be considered. Antithrombotic therapy should be considered in non–surgical candidates.
UNUSUAL PATIENT IN CATH LAB: A CASE REPORT
Authors: Picardi G , Viceconte N
Published: May 2024

Background

Acute coronary syndromes occur in daily cardiologist’s practice. Atherosclerosis is doubtless the main etiology; however, it exists an alternative physiopathological mechanism: spontaneous coronary artery dissection (SCAD). Atherosclerosis and SCAD do not share the same risk factors: typical SCAD epidemiology comprehends young women often in pregnancy. SCAD is caused by sudden disruption of the coronary artery wall, resulting in separation of the inner intimal lining from the outer vessel wall.

Case Description

F.C. a 37–years–old, female, entered to the Emergency Department (ED) referring typical chest discomfort radiating to jaw and upper limbs lasting two hours. EKG presented ST segment elevation in V2–V4. Diagnosis of ACS–STEMI was performed. Urgent coronary angiography showed long dissection involving LDA. At first due to clinical relief, the physicians suggested to a conservative treatment and a watchful waiting. Soon the patient experimented malignant arrhythmias and percutaneous revascularization was necessary. True lumen engagement with guidewire was ensured by IVUS technique; Resolute Onyx 2.5 mmx34 mm was deployed in LDA mid tract, protecting the side brunch with further guidewire. Because of retrograde squeezing of intramural hematoma, a further Resolute Onyx 3 mmx8 mm was released proximally overlapped the first stent. Good result TIMI 3. After six months, the patient was asymptomatic and Coro CT confirmed the stents patency and absents of further dissections. Discussion: Randomized control trial about best treatment option in patient with SCAD are unavailable. Conservative therapy is often the first line, especially in dissections involving mid–distal tracts of main branch. The reason of this stands in a high percentage of spontaneous healing of dissections within 3–6 months, From data available in medical literature, PCI performed in atherosclerosis coronaropathy has better outcomes than PCI performed in SCAD. The latter is associated with a larger incidence in periprocedural complications. False lumen engagement with guidewire and intramural hematoma ‘squeezing’ after stent dilatation are the typical procedure complications.

Conclusions

Because of exiguous numbers, SCAD is a topic of large gap in evidence. Nowadays, lots of our knowledge about SCAD results from case reports or small meta–analysis. Indeed, the present case report offers it contribute to increase the state of art on this theme.
ANOTHER BRICK IN THE WALL: A STRANGE CASE OF A BIG HUG
Authors: Chiorazzo L , Gambioli V , Trambaiolo P , D‘Ambrosi A , Fina P , Miraldi F , Saade W , Granatelli A
Published: May 2024

Background

Left ventricular pseudoaneurysm (LVP) is a rare complication of myocardial infarction MI (less than 0,1% of all patients with MI) and develops when cardiac rupture is contained by adherent pericardium or scar tissue, thus contains no endocardium or myocardium, unlike a true left ventricular aneurysm (LVA).

Clinical Case

A 41–year–old man without remote cardiac history was admitted to the Emergency Department with chest pain that had started 7 days before after a referred ‘big hug’ from a friend. Results of the physical examination were unremarkable. Electrocardiogram: T waves negative in the inferior and lateral leads. Blood exams: normal troponins, C–reactive protein and D–dimer values. Chest computed tomography: no sign of pulmonary embolism. Transthoracic echocardiography (TE): ejection fraction 45%, voluminous aneurismal chamber communicating with the infero–posterior wall of the LV, stratified thrombus and mild mitral valve regurgitation. Coronary angiography: three vessel coronary artery disease. The last diagnosis was established by cardiac magnetic resonance imaging (MRI) which revealed a voluminous pseudoaneurysm of the inferior wall (56 mm x 50 mm with a 51 mm neck) and transmural late gadolinium enhancement (LGE) of the wall adjacent to the pseudoaneurysm with fibroadipose replacement and calcific metaplasia. The patient was transferred to the Cardiac Surgery Department. Intraoperatively the LVP was resected and the defect of the LV was repaired with a patch in bovine pericardium. In addition, coronary artery bypass grafting and mitral valve repair were performed.

Discussion

LVP develops when pericardium, in combination with thrombus and inflammation, surrounds a cardiac rupture. Inferior and posterolateral myocardial infarction (MI), due to occlusion of the Circumflex artery, are responsible for 82% of the LVP. LVP can be also found in the apical region due to anterior MI. In conclusion symptoms, signs, electrocardiographic abnormalities and radiographic findings seen in patients with LVP can be indistinguishable from those in patients with coronary disease alone. TE is the first non–invasive method for early diagnosis of LVA, but MRI is the gold standard for differential diagnosis between true aneurysm vs pseudoaneurysm. LGE of the pericardium is a new specific marker of pseudoaneurysm as opposed to true aneurysm.
EOSINOPHILIC MYOPERICARDITIS: A CASE SERIES
Authors: Laiso L , Bocchino P , Angelini F , Claudia R , Pidello S , Frea S , Gallone G , Gaetano Maria D
Published: May 2024

Background

Eosinophilic myocarditis is characterized by acute myocardial inflammation due to eosinophilic tissue infiltration.

Methods

Diagnosis was based on laboratory tests, Echo and CMR; EMB was performed in uncertain cases. Treatment was performed according to the etiology of EM. All patients underwent regular follow–up at our Center.

Results

This study includes 9 patients (89% Caucasian, 78% women, mean age 48.2±13.6 years), admitted in our Center from February 2021 to November 2023. 66.67% cases occurred in the setting of eosinophilic granulomatosis with polyangiitis, 11% was secondary to B–cell lymphoblastic leukemia, and 22% were labelled as idiopathic EM. All patients presented with high eosinophil count (6133±4893/mcL), elevated CRP (57±66 mg/L) and hs–troponin I (3610±5166 ng/L) levels, and 67% cases NT–proBNP (10751±4038 ng/L) was elevated. 67% patients presented with dyspnea, 56% with chest pain, and 56% with fever. On ECG, sinus tachycardia, low QRS voltages and ST segment depression with negative T–waves were common. Thickening of the myocardial walls with reduction of GLS was seen in all cases; in 44% cases, the EF was below 50%; 56% cases had mild pericardial effusion. 1 patient had intraventricular thrombi at CMR, another one had bilateral pulmonary embolism. On CMR, all patients had areas of hypokinesia, myocardial edema, and myo–pericardial late gadolinium enhancement. EMB was performed in 5 cases, confirming the presence of intramyocardial eosinophilic infiltrates. 7 patients required CICU, and 1 needed inotrope (dobutamine) use. The patient with ALL was treated with combined chemotherapy. The other EM cases were treated with immunosuppressive therapy consisting of corticosteroids and either cyclophosphamide or rituximab or mepolizumab. All patients received cardioprotective therapy with BBs and ACE–inhibitors; in patients with pericardial involvement, colchicine was administered. All patients showed rapid clinical improvement following immunosuppressive therapy initiation, with prompt reduction of circulating eosinophils and hs–troponin I values and normalization of the ejection fraction. On CMR imaging, edema disappeared at follow–up, with LGE replacement in one case. All patients were alive and in good conditions at the last follow–up.

Conclusions

EM is an underdiagnosed life–threatening disease. A careful assessment of the etiology, as prompt therapy, is mandatory to achieve clinical amelioration and good mid–term prognosis.
SEVERE HEART DYSFUNCTION AND INTRA–CARDIAC THROMBOSIS: WHAT ELSE?
Authors: Mastroberardino S , Segreti A , Frau L , D‘Antonio L , Appetecchia A , Ricciardi D , Grigioni F , Ussia G
Published: May 2024

Introduction

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly Churg–Strauss syndrome, is a rare vasculitis of small–medium blood vessels. We report a case of a young woman with severe cardiovascular, neurological and respiratory problems caused by the syndrome.

Case Report

A 45–year–old woman, affected by bronchial asthma and nasal polyposis, experiencing dyspnea and paresthesia on the left lower limb, underwent a total body PET–CT which demonstrated left segmental pulmonary thromboembolism, lung pseudo–nodular infiltration, and pericardial and pleural effusions. Thus, she entered Emergency Department. Hepatomegaly, left lower limb hot and edematous were observed. The ECG revealed low QRS voltages in the peripheral leads and poor r–wave progression in the precordial leads. The trans–thoracic echocardiography showed dilatated and hypokinetic right (RV) and left ventricle (LV) with severe reduction of global systolic function (LV ejection fraction, LVEF = 20%), bi–atrial dilation, circumferential pericardial effusion and thrombotic stratification at the apex. Blood test examination displayed severe eosinophilia, raised NT–Pro BNP and troponin I levels. A heart/coronary CT was performed, documenting epicardial coronaries free from significant atheromatous pathology. In addition, a cardiac MRI showed late gadolinium enhancement in the sub–endocardial area with diffuse involvement of the mid–apical ventricular segments and intraventricular septum, as in fibrotic outcomes. The subsequent total body CT scan confirmed the known thromboembolism, pulmonary lesions and pleuro–pericardial effusion; cardiogenic liver cirrhosis, and thrombosis of the right uterine vein and left gonadal vein were also reported. The auto–antibodies blood testing showed negativity for ANCA. In the presence of bronchial asthma, nasal polyposis, motoneuritis and hypereosinophilia, we diagnosed ANCA–negative eosinophilic vasculitis with cardiac involvement. Genetic testing was negative for FIP1L1–PDGFRA, excluding treatment with imatinib. Thus, we started immunosuppression therapy. However, albeit optimal medical treatment for ventricular dysfunction, the implantation of a subcutaneous cardiac defibrillator was required.

Conclusion

The possible cardiac manifestations in the context of EGPA may be several. Therefore, a prompt diagnosis results in a better prognosis for the patients.
MYOCARDITIS, CORONARY ARTERY DISSECTION OR BOTH: A JUVENILE ENIGMA
Authors: Sala E , Cereda A , Di Giovine G , Conconi B , Lucreziotti S
Published: May 2024

A 26–year–old man was admitted to the Coronary Care Unit for severe, non–irradiated, oppressive anterothoracic pain. He had no relevant pathological history, but reported an episode of gastroenteritis the week before. EKG showed sinus rhythm and accelerated idioventricular rhythm phases. Blood pressure and 2D echo were normal. Inflammation indeces were negative, hsTnT reached a peak of 2026 ng/L. Despite the absence of inflammation signs, our first guess was myocarditis. Cardiac magnetic resonance (CMR) was performed: T2w–STIR images showed edema of the middle inferior wall and of the mid–basal anterior and anterolateral wall, while T1–weighted post gadolinium images showed transmural enhancement of the mid–basal anterior, antero lateral and inferior walls with a dubious origin from the sub endocardium in the anterior and anterolateral segments; LVEF was 55%. Invasive coronary angiography (ICA) was performed to rule out ischemic etiology: LAD showed a very thin lumen in its periphery and two obtuse marginal branches had an amputated appearance: it made us think of healing dissections. Then it turned out that the patient have been working out with heavy weights the day before: we therefore considered spontaneous coronary artery dissection. Such hypothesis was consistent with the absence of inflammations indices, but didn’t explain the basal and middle inferior involvement that CMR had shown. On the other hand, the angiographic finding may be consistent with peripheral vascular suffering linked to myocarditis induced inflammation. To date, we haven’t found univocal interpretation. Anti–remodeling therapy with IACE and beta blockers was administered, in addition to acetylsalicylic acid. CMR will be performed in two months and may provide further results once the edema will be reabsorbed. ICA will be repeated to make sure that distal coronary blood flow is improved.

Take Home Messages

Myocarditis can also occur with no signs of inflammation. In myocarditis it has been demonstrated that slower distal coronary blood flow may occur, due to the increase in peripheral vascular resistance linked to the presence of edema. In the acute phase, especially if CMR is performed very early, the large amount of edema can make the enhancement transmural, masking the site of origin of the damage.
GIANT RIGHT ATRIAL MYXOMA CAUSING HEART FAILURE SYMPTOMS
Authors: Maffè S , Paffoni P , Bergamasco L , Facchini E , Ariotti S , Prenna E , Ticozzi S , Paino A , Dellavesa P
Published: May 2024

Background

Cardiac myxoma is the most common benign tumor of the heart. About 75% of myxomas occur in the left atrial cavity, but they can also appear in the right atrium (18%) as unusual location. It presents with a variety of clinical signs and symptomatology making diagnosis frequently quite a challenge.

Objective

The aim of this article is to present a case report of giant right atrial myxoma with symptoms of heart failure in adult patient.

Case Report

We present a case of large right atrial myxoma, discovered incidentally in a female patient 88–year–old who came to our attention for worsening dyspnea, asthenia, peripheral edema,dizziness and palpitations. The transthoracic and transesophageal echocardiogram show a voluminous hyperechogenic formation in the right atrium, measuring 82 x 42mm (Figure 1) which almost entirely occupies the atrium and was found to prolapse through the tricuspid valve into the right ventricle, causing obstruction during diastole. The right cardiac chambers appear dilated, with increased pressures causing compression on the left cardiac chambers, with D–shape of the interventricular septum and fixed left convexity of the interatrial septum. The patient presents paroxism of atrial fibrillation, and a massive left pleural effusion requiring drainage. Cardiac magnetic resonance imaging confirms the mass and the tissue characterization (isointensity of the T1 signal, increased intensity in T2, absence of contrast uptake in the perfusion sequences and presence of heterogeneous late enhancement) support the diagnosis of atrial myxoma (Figure 2). The patient underwent surgery with removal of the mass (Figure 3), subsequent regular clinical course with normalization of the right chambers and of hemodynamic compensation. The histological diagnosis of myxoma was confirmed. Cardiac myxomas can produce three types of clinical manifestations: constitutional symptoms, embolization and intracardiac obstruction. Most of the myxomas are asymptomatic; the right atrial myxomas present with obstructive features like dyspnoea, syncope, right side heart failure; rarely it can cause left ventricular overload and biventricular heart failure, as in our case. Transesophageal echocardiography is superior for precise evaluation of cardiac tumors, but our case demonstrates how cardiac MRI has become the gold standard exam, allowing a perfect morphological evaluation and overall a tissue characterization of cardiac masses before histology.