bluuebunny/crossref_metadata_2025_split huggingface dataset:
PLATYPNEA–ORTHODEOXIA SYNDROME SECONDARY TO TRAUMATIC TRICUSPID VALVE RUPTURE: A CASE REPORT
Authors: Della Torre M , Freschini M , Del Pinto M 
Published: May 2024

Abstract

Introduction
Platypnea–Orthodeoxia Syndrome (POS) is a rare clinical condition characterized by dyspnea and arterial desaturation exacerbated in an upright position and alleviated in the supine position. The main pathophysiological mechanism involves an intra– or extracardiac right–to–left (R–L) shunt. We report an acute POS presentation secondary to traumatic tricuspid valve rupture.

Clinical Case
A 42–years–old man presented to the ED with new–onset worsening dyspnea. A week prior, he was involved in a car crash with airbag deployment and blunt chest trauma, confining him to bed. In medical history: double surgical aortic valve replacement for endocarditis caused by intravenous drug use. At presentation: BP was 100/65 mmHg, sO2 was 94% on room air. Transthoracic echocardiography revealed a LVEF of 40% with akinesis of the apex and the distal segments. Coronary angiography ruled out LAD artery traumatic injuries. After thorough clinical evaluation, an abnormal drop in sO2 (&lt;80% on room air) was observed in the seated position. A microbubble test demonstrated a massive R–L shunt through the interatrial sept (IAS). Transesophageal echocardiography revealed atrial septal aneurysm, patent foramen ovale (PFO) with R–L shunt, severe tricuspid regurgitation (TR) with the jet directed to the IAS due to a chordae tendineae rupture and posterior leaflet flail. Heart Team discussion: high surgical risk, only the percutaneous closure of the PFO was recommended. The procedure was performed with the deployment of Amplatzer device and resulted free of complications. In the subsequent days, the patient was cautiously mobilized. A 6MWT showed no evidence of desaturation and good effort tolerance. Due to the high surgical risk, a conservative strategy with close clinical and echocardiographic follow–up was adopted regarding the TR. The patient was discharged in clinical compensation and in good general condition.

Conclusion
This case of POS is categorized among those secondary to intracardiac shunting without an increase in pressures in the right heart. The direction of flows within the right cardiac chambers, determined by the TR jet in this instance, dictates the shunt. This report expands our knowledge of potential POS causes, highlighting the importance of considering rarer etiologies based on the patient‘s medical history and presentation. Furthermore, this case emphasizes the necessity for a multidisciplinary approach to assess optimal treatment options.
A RARE MANIFESTATION OF Q FEVER
Authors: Rossi D , Scollo C , D‘Andria R , Magnano R , Pezzi L , D‘Alleva A , Corazzini A , Primavera M , Fulgenzi F , Forlani D , Vitulli P , Paloscia L , Gallina S , Genovesi E , Di Marco M 
Published: May 2024

Abstract
A 19–year–old male with no significant medical history presented to the Emergency Department with persistent chest pain and worsening fever over five days. The 12–lead electrocardiogram (ECG) showed sinus rhythm, elevation of the ST segment in the inferior leads, and slight depression in V1–V2. Laboratory tests revealed elevated cardiac enzymes (hs–TnI 11698 pg/ml) and a mild increase in inflammatory markers. Echocardiography demonstrated normal morpho–functional parameters and a mild pericardial effusion. The patient was admitted to the Cardiovascular Intensive Care Unite with a diagnosis of myopericarditis. Treatment with ibuprofen and colchicine led to a gradual improvement in cardiac enzymes and inflammatory markers. Microbiological exams showed IgM positivity for Coxiella burnetii, suggesting a possible zoonotic origin. Cardiac Magnetic Resonance Imaging (MRI) revealed signs of myocardial edema and non–ischemic late gadolinium enhancement (LGE). As the myopericarditis was uncomplicated, no immunosuppressive/modulatory treatments were administered. Coronary CT confirmed the integrity of the epicardial coronary circulation. One month later, the patient was asymptomatic with no recurrence of fever or chest pain. Pericardial effusion resolved, and laboratory and instrumental findings were normal. The cause of myopericarditis was attributed to Coxiella burnetii, an intracellular bacterium with reservoirs in domestic animals. Transmission occurs through contact with urine, feces, unpasteurized milk, or infected animal placentas. Clinical presentation varies from asymptomatic to flu–like symptoms or organ involvement. The heart can be affected in both endocarditic and, rarely, myocarditic forms. In conclusion, this case represents an infectious myocarditis caused by Coxiella burnetii, confirmed by antibody positivity and a favorable response to anti–inflammatory and antibiotic therapy. In the literature, few cases of myocarditis due to Coxiella burnetii are reported, and it is a rare complication of Q fever. However, it is an entity that exists and should be considered in the appropriate clinical–anamnestic context.
HEART FAILURE WITH PRESERVED EJECTION FRACTION (HFPEF) IN A PATIENT WITH RECENT TAVI AND NUMEROUS CORONARY FISTULAS OF THE LEFT CORONARY ARTERY DRAINING INTO THE VENTRICULAR CAVITY
Authors: Maffei S , Tarsi G , Paolini F 
Published: May 2024

Abstract
Coronary fistula is a rare form of congenital heart disease and is defined as an abnormal connection between a coronary branch and a cardiac chamber or a large intrathoracic vessel without the interposition of the capillary circulation. The right coronary artery is the most common site of origin for coronary fistula. Other sites include the left anterior descending artery and left circumflex artery. The clinical presentation and the natural history can be extremely variable depending on the patient‘s age, fistula size, and anatomy. Small to medium–sized coronary fistulas may be asymptomatic. Large fistulas can manifest with angina pectoris due to myocardial ischemia secondary to the coronary steal phenomenon. Shunting through large fistulas can have hemodynamic consequences secondary to volume overload in the pulmonary or systemic circulation, leading to symptoms and signs of heart failure. We describe the case of an 83–year–old woman recently undergoing TAVI and suffering from primary myelofibrosis requiring periodic transfusions. Pre–TAVI coronary angiography showed coronary vessels free from significant hemodynamic lesions and multiple coronary–ventricular fistulas draining into the left ventricle. Total body aortic CT angiography did not reveal other abnormal vascular connections. One month after the aortic valve correction procedure, the patient presented with heart failure (HfpEF). At a recent follow–up visit, diuretic therapy was discontinued due to the successful correction of aortic valve disease and normal ejection fraction. Due to further anemization from the underlying hematologic condition, the patient underwent blood transfusion with worsening of dyspnea despite correction of hemoglobin levels. Discontinuation of diuretic therapy and transfusion of red blood cell units resulted in volume overload with worsening clinical condition. The presence of multiple coronary fistulas precluded an interventional approach, but therapeutic optimization and reintroduction of diuretic therapy restored the patient‘s hemodynamic compensation. Usually fistulas drain into the low–pressure venous circulation like right ventricle and right atrium. This case represents a rare instance of multiple left coronary fistulas draining into the left ventricle (only 3% of cases). Aortic valve correction may have further reduced left ventricular telediastolic pressures, worsening the shunt of coronary fistulas resulting in heart failure due to volume overload.
HCM AT THE CROSSROAD
Authors: Bosso A 
Published: May 2024

Abstract

Introduction
Treatment of oHCM frequently requires difficult choices, especially when it is superimposed on other urgent clinical conditions,such as AMI or mechanical complications.

Clinical Case
A 52–year–old man with known oHCM, who spontaneously quickly discontinued recommended therapy, presents to theED with a sudden episode of dyspnea and presyncope. A firstECG was essentially normal for pathology. After 1h, the patient experiences a sudden episode of chest pain with rapid hemodynamic deterioration and cardiogenic shock(CS). The ECG shows changes compatible with inferolateral MI. The TTE shows severe LVOTO, SAM, severe MR and a suspicious oval image attached to the anterior mitral leaflet. The patient underwent orotracheal intubation, coronary angiography and TEE. Coronary angiography shows a thrombotic occlusion of the proximal circumflex artery, treated with pPCI. TEE confirms a partial rupture of the papillary muscle head. Treatment:The patient‘s CS can be caused by 2different conditions that require treatments with opposing pathophysiological principles. SevereMR due to papillary muscle rupture, as mechanical complication of MI, requires afterload reduction, hemodynamic stabilization with IABP and emergency transfer to a cardiac surgery center. In contrast, the treatment of the underlying pathology(severe LVOTO and severe rMR due to SAM)requires pharmacological stabilization with depression of cardiac contractility and increase in afterload. In the context of cardiogenic shock a rapid choice was required. To help us make the right choice, an Esmolol test was performed. After iv administration of 2mg Esmolol, heart rate was reduced without reduction in BP. The patient was then treated with esmolol in continuous infusion and norepinephrine. In oHCM the ventriculo–arterial coupling is reduced, less than 1. This treatment favored better V–A coupling, ensuring an increase in afterload and a reduction in myocardial contractility. The patient‘s clinical condition rapidly improved, allowing extubation at48h and early mobilization at96h. Myectomy and mitral repair surgery was scheduled 1 month afterPCI.

Conclusions
Through the use of a short–lived drug such as Esmolol, it was possible to trace the prevailing mechanism of the patient‘s CS. Differently from other cases of CS, to rebalance the V–Acoupling, it was crucial to reduce myocardial contractility and increase afterload. A pathophysiological pharmacological approach ensured a good outcome.
AN UNUSUAL CASE OF VENTRICULAR DYSFUNCTION
Authors: Minopoli T , Petrone A , Di Mario C 
Published: May 2024

Abstract
Sarcoidosis is a multisystemic inflammatory disorder characterized by non–caseating granulomas and rarely involves the heart. While frequently presenting without symptoms, cardiac sarcoidosis can lead to arrhythmias, heart failure, and sudden death, with symptoms mimicking other cardiac conditions. We present a case of a 61–year–old woman admitted to our emergency department in February 2021 with resting dyspnea and atypical chest pain. Elevated hs–troponin and NT–proBNP levels, a new–onset left bundle branch block on ECG, and signs of pulmonary congestion on chest X–ray prompted further investigation. Echocardiography revealed diffuse hypokinesis with akinesia of the mid–apical inferior wall and a significant ventricular dysfunction (LVEF 30%). Coronary angiography excluded lesions, diagnosing MINOCA. Cardiac MRI showed transmural fibrosis without a typical cardiomyopathic pattern. An FDG–PET scan indicated uptake in the heart and lymph nodes. A biopsy of a left supraclavicular lymph node confirmed non–necrotizing granulomas, consolidating the diagnosis of cardiac sarcoidosis. Treatment with Prednisone 25 mg was initiated and gradually tapered to 6.5 mg, leading to a progressive normalization of systolic function (LVEF 56% at 12–month follow–up) and resolution of lymphadenopathies. Unexplained isolated ventricular dysfunction presents a wide range of potential causes. Despite its rarity, sarcoidosis should be considered as a possible etiology. While characteristic patterns may emerge in cardiac MRI, atypical presentations underscore the need for a comprehensive diagnostic approach. Timely diagnosis and effective treatment can significantly enhance prognosis, emphasizing the importance of prompt intervention for optimal outcomes.
ACUTE PERICARDITIS IN A PATIENT WITH PRIMARY HEART LYMPHOMA
Authors: Lanati G , Di Spigno F , Caraffini A , Sabatino M , Covini D , Turchio P , Rusticali G , Febo O , Aschieri D 
Published: May 2024

Abstract
A 51–year–old male patient was admitted to the emergency room for stabbing chest pain associated with dyspnea on mild exertion. Past medical history: obesity, previous HCV infection, previous intravenous substance abuse. Physical examination: no audible cardiac murmurs or rubs, no wet lung sounds, mild edema of the face and upper limbs. ECG: sinus rhythm, no repolarization alterations. Lab tests: elevation of troponin I (93 ng/l) and CRP (2 mg/dl). Echocardiogram (poor visualization): non–dilated and normokinetic left ventricle (EF 0.55), no significant valvular disease, mild pericardial effusion. Coronary angiography: no obstructive coronary disease. A diagnosis of acute pericarditis was made, and anti–inflammatory therapy with ibuprofen and colchicine was started. Cardiac MRI showed a large right atrial mass, which infiltrates the interatrial septum and causes obstruction of the superior vena cava; the mass showed hypointensity on T1, clear hyperintensity on T2 and modest and non–homogeneous late gadolinium enhancement; mediastinal lymphadenopathy was also detected; overall, the exam raised the suspicion of malignant primary cardiac tumor (cardiac lymphoma versus angiosarcoma). The patient was referred to a tertiary center for endomyocardial biopsy, which showed the presence of large B–cell lymphoma, and the patient was started on chemotherapy. Discussion Cardiac masses are rare and usually benign. Malignant tumors are more frequently secondary, while primary ones are very rare. Lymphomas represent 1–2% of all cardiac tumors, are more frequent in male adults and are associated with immunodeficiency conditions. 70% are diffuse large B–cell lymphomas. Transthoracic echocardiogram is the main first–level test but has poor sensitivity, especially in cases with high acoustic impedance. Cardiac MRI is the main test for accurate anatomical definition and tissue characterization. In this specific case, the hyperintensity in the T2 sequences, the presence of inhomogeneous LGE and the mediastinal lymphadenopathy suggested the possible lymphoproliferative nature of the mass. Angiosarcoma appears more heterogeneous on T2 sequences and presents a ring of peripheral enhancement with central hypopointesity in post–contrast sequences. Endomyocardial biopsy allows for a definitive diagnosis, but is performed only when the benefits outweigh the risks. In this case, it proved to be essential for the histological characterization and the subsequent therapeutic approach.
USE OF SGLT2–INHIBITORS IN THE TREATMENT OF ANTHRACICLINE CARDIOTOXICITY
Authors: Pezzi L , Magnano R , D‘Alleva A , Vitulli P , Forlani D , Primavera M , Verrengia E , Corazzini A , Duronio G , Genovesi E , Paloscia L , Di Marco M 
Published: May 2024

Abstract
The survival rate of cancer patients has significantly increased in the last 25 years thanks to the extraordinary advances in anti-tumor therapies. However, many chemotherapeutic agents are potentially cardiotoxic. In particular, anthracycline-induced cardiotoxicity is a clinical condition caused by chemotherapy drugs that lead to morpho-functional alterations and cell death of cardiomyocytes; the most typical clinical manifestation of this cardiotoxicity is the development of left ventricular dysfunction, which over time can evolve towards full-blown heart failure. It is therefore characterized by a reversible asymptomatic stage that progresses to an irreversible stage, often difficult to treat. For an early individualization of this toxicity, a dedicated clinic has been established in our center for the monitoring of patients undergoing treatment with cardiotoxic chemotherapy. We describe the case of a 53-year-old patient with preserved biventricular systolic function undergoing neoadjuvant treatment for breast cancer, who at the end of the second infusion of anthracyclines presented with mild exertional dyspnoea associated with radiological signs of pulmonary congestion and an increase in the indices of myocardionecrosis and BNP. The echocardiogram revealed a sudden worsening of the left ventricular systolic function which was 48%. It was therefore decided to hospitalize the patient and initiate infusional diuretic therapy with a rapid clinical and instrumental response. An SGLT2 inhibitor with indication for the treatment of heart failure with moderately reduced ejection fraction was also added to the therapy. At the one-month check-up, there was an almost complete recovery of left ventricular function and a clear symptomatic benefit with associated normalization of laboratory markers of myocardial damage and heart failure, with the possibility for the patient to undergo surgery to remove the left ventricle. tumor mass. This clinical case could represent a starting point to start considering therapy with SGLT2 inhibitors for the prevention and early treatment of cardiac toxicities from some chemotherapeutics, in order to avoid the process of irreversibility of myocardial damage with subsequent poor response to anti-inflammatory therapies. – conventional compensation.
A CASE REPORT OF MICROVASCULAR ANGINA WITH ATYPICAL ECG FINDINGS
Authors: Todisco S , Federico D , Cappannoli L , Buffon A , Fracassi F , Burzotta F , Crea F 
Published: May 2024

Abstract
Coronary microvascular dysfunction (CMD) relates to a broad range of clinical settings in which cardiac microcirculation is morphologically and/or functionally affected, leading to myocardial ischemia and causing anginal symptoms (microvascular angina). Based on the pathogenetic mechanism, there are two possible endotypes of CMD: 1) coronary microvascular spasm and 2) reduced coronary flow reserve (CFR). The gold standard for CMD diagnosis is represented by the coronary angiography with functional tests: 1) the acetylcholine provocative test for the diagnosis of microvascular spasm (endothelium–dependent dysfunction); 2) the determination of CFR and of the index of microvascular resistance (IMR) thorough a flow guidewire after endothelium–independent vasodilation (usually obtained with intracoronary adenosine injection). We report an emblematic case of CMD due to microvascular spasm in a young woman with episodes of typical angina at rest, especially during the night, associated with tachycardia episodes. The patient was admitted to the cardiology unit and underwent coronary angiography, which revealed mild coronary atherosclerosis without any significant stenosis. Due to the very suggestive clinical presentation, provocative test by intracoronary acetylcholine infusion at increasing doses (20–50–100 micrograms) was also performed: from the beginning of the second dose (50 micrograms) the patient started suffering from her typical angor (symptomatology similar to that reported at home), and diffuse ST–T alterations (ST depression and T waves inversion in precordial leads) occurred. At the beginning of the third dose (100 micrograms) a lateral ST–segment elevation (mostly in aVL) was surprisingly observed. The ST–T modifications (including the elevation) were interestingly associated with only diffuse moderate vasoconstriction of the left anterior descending artery, not fulfilling the Covadis criteria for epicardial vasospasm, thus suggesting the presence of a vasospasm of the coronary microcirculation. Symptoms and ECG changes both regressed after intracoronary nitrates administration. As a consequence of the diagnosis of microvascular angina with microvascular vasospasm, medical therapy with calcium antagonists was administered (withdrawing beta–blockers).
CORONARY ARTERY ANEURYSM IN IGG4–RELATED DISEASE
Authors: Mingoia G , Astuti G , Orlando E , Comparato F , Farina C , D’Angelo F , Leggio G , Evola S , Novo G , Corrado E , Puccio D 
Published: May 2024

Abstract

Introduction
Coronary artery ectasia is a rare disease defined as a local or diffuse dilatation of the coronary artery more than 1.5 times the diameter of the adiacent normal segment. The etiology is diverse and can be rarely associated with immunoglobulin G4 (IgG4)–related disease. The high levels of serum IgG4 may promote the development of low–density plaques, intimal thickening due to pericoronitis and subsequent coronary ectasia.

Case Report
We present the case of a 72–year–old male patient with arterial hypertension, dyslipidemia, chronic renal insufficiency, and a family history of cardiovascular diseases. In the previous year, he suffered a cerebral hemorrhage attributed to an unspecified cerebral vascular malformation, which was managed with an endovascular approach. The patient was admitted for Non–ST–segment Elevation Myocardial Infarction (NSTEMI). Coronary angiography revealed widespread ectasia of the coronary vessels, a chronically occluded marginal branch, and an aneurysmatic proximal right coronary artery with the presence of a thrombus on the aneurysmal segment, dissolved following administration of intracoronaric heparin. Furthermore a critical stenosis at the crux involving the posterior interventricular branch was detected and angioplasty with drug–eluting stent implantation was performed on the affected vessel. Given the coronary artery ectasia, aortic ectasia revealed by echocardiography, renal insufficiency and persistently elevated amylase and lipase levels, further diagnostic investigation was conducted suspecting IgG4–related disease. Hematological tests revealed elevated IgG and IgG4 subclass levels (882 mg/dL and 1438 mg/L, respectively), C3 117 mg/dL, C4 30 mg/dL, and total IgE 130 KU/L. The diagnosis of IgG4–related disease was confirmed and the patient was advised to continue cardiological and rheumatological follow–up.

Conclusion
When the coronary arteries are involved in the IgG4–related disease, risk of acute events such as heart attack may increase and the prognosis is poor. Coronary thickening and ectasia, typical imaging features, other aortic involvement and inflammation indicators, like elevated serum IgG4, should be considered as red flags for an early investigation and subsequent early intervention.