Dilated Ventricle Research Articles

P277 MULTIFACTORIAL RECURRENT CHYLOUS ASCITES: THE CASE REPORT OF A PATIENT WITH HEART FAILURE WITH PRESERVED EJECTION FRACTION (HFPEF), LIVER DISEASE AND PAST ABDOMINAL SURGERY

Abstract Chylous ascites is the accumulation of triglyceride–rich fluid in abdominal cavity. It is caused by lymphatic ducts injury, usually as consequence of surgery, neoplasia, or infection. Rarely, it can also occur from heart failure, pericarditis, or cirrhosis. We report the case of a 66–year–old man electively hospitalized for acute renal failure and anasarca state. The cardiological history was significant due to acute myocardial infarction treated with CABG, PM implantation for complete AV block, atrial fibrillation, and type 2 diabetes mellitus. The entero–hepatological history started in 2019 when, two months after abdominal surgery for an incarcerated hernia, he developed chylous ascites and chylotorax. Starting from 2021, the patient presented recurrent episodes of tense ascites, requiring periodic paracentesis. Haematological and infectious disease evaluation resulted negative, liver biopsy showed congestive hepatopathy with fibrosis, while venous catheterization found high right filling pressure with not portal hypertension. Patient was treated with Apixaban 2.5 mg b.i.d., Furosemide 50 mg b.i.d., Metformin 1500 mg, Enalapril 20 mg, and Simvastatin 20 mg. During the hospitalization other paracentesis confirmed the chylous nature of the effusion, echocardiography (despite very bad acoustic windows) showed preserved left ventricle systolic function (LVEF 55%), 3rd degree LV diastolic disfunction, and dilatation of right ventricle, atria, and inferior Vena Cava. Right heart catheterization confirmed the presence of LV restrictive physiology, combined pulmonary hypertension, and ruled out the diagnosis of constrictive pericarditis. In hospital treatment focused on resolving acute renal failure and hypervolemia through intravenous diuretic and Albumin and on optimizing cardiological therapy. The patient was discharged with the following treatment: Apixaban 2.5 mg b.i.d., Furosemide 50 mg b.i.d., Canrenone 100 mg b.i.d., Empagliflozin 10 mg, Simvastatin 20 mg, and indication to hypo–lipidic diet. This case highlights how the coexistence of different predisposing factors of chylous ascites (past abdominal surgery, heart failure and liver disease) can amplify each other. Recognizing the single role of each of them is of pivotal importance to elaborate targeted treatment. Moreover, in literature, there are some cases of chylous ascites associated with constrictive pericarditis and with severe heart failure, but to our knowledge not with coexistence of HFpEF.

C15 PERCUTANEOUS EDGE–TO–EDGE REPAIR USING EDWARDS PASCAL SYSTEM FOR SEVERE TRICUSPID REGURGITATION IN CONGENITALLY CORRECTED TRANSPOSITION OF THE GREAT ARTERIES

Abstract Introduction Congenitally corrected transposition of the great arteries (ccTGA) is a rare cardiac malformation characterized by ‘double’ discordance of atrioventricular and ventriculo–arterial connections, resulting in a systemic tricuspid atrioventricular valve and a systemic right ventricle. Clinical case We describe a case report of 54 years–old male patient with ccTGA, diagnosed when he was 3 years–old. Patient performed regular follow–up. Since December 2019, the patient reported onset of effort dyspnoea (NYHA III), fatigue and exercise intolerance. Despite medication optimization, his symptoms persisted. He was referred at our Centre for specialist evaluation. Transthoracic echocardiography showed mild dilatation and dysfunction of right ventricle, massive systemic tricuspid valve regurgitation (EROA 42 mm2), due to multiple jets and systolic pulmonary artery pressure of 45 mmHg. As reported by most recent guidelines, indication was given for surgical replacement of the systemic tricuspid valve. However, the patient refused the cardiac surgery option. Transesophageal echocardiography (TEE) screening showed feasibility of percutaneous approach to tricuspid regurgitation. Being the jet central, the strategy was to place one device implant between the septal and the anterior leaflet (9–3 oriented), as central as possible. Following simultaneous leaflet capture and further optimization of leaflet, the result was excellent. Post–implant deployment TEE revealed only mild–to–moderate residual tricuspid regurgitation, with no tricuspid stenosis (mean gradient 2 mm Hg). The result was a percutaneous bicuspidization of a tricuspid systemic valve. The patient recovered quickly and was discharged to home 2 days after the procedure. Patient experienced significant improvement in functional status (NYHA I), exercise capacity and quality of life at 6 months follow–up. Conclusions In ccTGA patients, treatment of severe tricuspid regurgitation and improvement of the systemic ventricle’s systolic function is critical since progression of dysfunction of the RV and tricuspid regurgitation are considered key determinants for morbidity and mortality in these patients. Based on the results obtained in our case, percutaneous edge–to edge repair seems to be a reasonable alternative to the surgical approach and three–dimensional transesophageal echocardiography is imperative for the proper visualization of the leaflets outcome of the procedure and his durability.

P226 A STRANGE CASE OF ISOLATED RIGHT VENTRICULAR DYSFUNCTION

Abstract Introduction In Europe, approximately 29% of the population aged between 15 and 64 have used illicit drugs. Italy is at the top of this ranking. Description 45–year–old female, no previous cardiology events. She enters the Emergency Department for typical chest pain following alcohol and cocaine abuse. EKG (see figure 1) detects sinus tachycardia and mild non–specific changes in ventricular repolarization. Blood tests reveal an increase in Troponin T levels (305 ng/L, NV < 14 ng/L, then decreased with subsequent measurements) and D–dimer (902 mg/mL, NV < 500 mg/mL). Echocardiography reveals a non–dilated right ventricle with severe systolic dysfunction (TAPSE 11 mm). No signs of pulmonary hypertension but a dilated and hypocollapsing inferior vena cava is noted. Abnormal motion of the interventricular septum with systolic d–shape is present (see figure 2). Left ventricle appears normal. Pulmonary angio–Tc was negative for embolic phenomena and coronary angiography detects no lesions on coronary arteries. At discharge, right ventricular systolic dysfunction and septal d–shape were completely regressed (see Figure 3). Discussion Cocaine is a strong systemic adrenergic agonist, which can cause vasospasm of small arterioles of the cardiac microcirculation with possible formation of intracoronary thrombi. Peculiarity of this case is the selective and reversible involvement of the right ventricle, an extremely rare presentation as evidenced by the almost absence of scientific literature on the subject (there is only a similar case report published on the American Journal of Emergency Medicine in 2015). Reversible damage from vasospasm and/or intracoronary thrombosis localized to the marginal branch of the right coronary artery, induced by cocaine use, is conceivable. D–shape of the interventricular septum associated with right ventricular dilatation is likely caused by acute right ventricular failure causing volume overload in the right sections. Take home message Drugs abuse is a widespread problem among the population. Cocaine intake is associated with important adverse cardiovascular effects. A rare presentation can be isolated right ventricular ischemia associated with acute right heart failure.

P162 ARRHYTHMIC MITRAL VALVE PROLAPSE OCCURRING AS ABORTED SUDDEN CARDIAC DEATH

Abstract Mitral valve prolapse (MVP) is the common valvular heart disease, with a prevalence about 1–3% in the population. MVP is characterized on echocardiography by an abnormal systolic displacement of mitral valve leaflets into left atrium (LA) ≥ 2 mm above the mitral annulus. We described a clinical case of a 15 years–old boy with a first clinical presentation of out–of–hospital cardiac arrest related to ventricular fibrillation (VF) with a successful resuscitation after 2 DC shocks. Retrospectively, an episode of loss of awareness reported as post–minational. No familiar history of sudden cardiac death (SCD) or MVP was noticed. In the emergency room, the patient was hemodynamically unstable and was sedated and intubated. The surface electrocardiogram (ECG) showed sinus rhythm, QRS with fragmentation, and frequent monomorphic isolated premature ventricular contractions (PVCs) with RBBB/LAFB configuration.Frequent monomorphic PVCs with bigeminy was observed during continuous ECG monitoring, so the patient was treated with magnesium sulphate and metoprolol. The patient was subsequently weaned from ventilation support, presenting good gas exchange and without neurological sequalae. The 24h Holter monitoring showed an average of 53 bpm, extremely rare supraventricular extrasystoles, high incidence of monomorphic PVCs (23% of the total beats) with 4 couplets. Transthoracic echocardiography documented normal size and thickness with systolic function (GLS –21%, mild asynchrony of posterior basal segments), bi–leaflet prolapse associated to mild–moderate mitral regurgitation, and mitral annular disjunction (MAD) of 6.2 mm. The late gadolinium enhancement cardiac magnetic resonance showed bi–leaflet prolapse with mild insufficiency and maximum MAD 9 mm, mild LA dilatation, and slightly dilated both ventricles, without regional wall motion abnormalities; no scar or fibrosis at the late gadolinium enhancement was observed. According to guidelines, the patient received a subcutaneous implantable cardiac defibrillator for the secondary prevention of SCD and maintained under betablockers in order to control the arrhythmic burden. The genetic sampling did not show any pathogenetic mutation potentially related to cardiomyopathies or channelopathies. This case show an arrhythmic MVP complex with probably posterior papillary muscle source. The early PVCs with R/T constituted a possible trigger of VF. The betablocker therapy exhibited a full suppression of extrasystolic burden.

P131 ACUTE RIGHT HEART FAILURE: MASSIVE PULMONARY EMBOLISM OR RIGHT INFARCTION?

Abstract Right ventricular infarction is often unrecognized. Rarely isolated, it complicates up to 50% of inferior infarction, conditioning morbidity and mortality. Clinical presentation, characterized by hypotension, and echocardiografic features, with right ventricular involvement, can often mimic high risk pulmonary embolism. This case report focuses on an 85–years–old woman who called 118 for chest pain and asthenia. The patient is in shock, when accepted into the emergency room. ECG reveals a third–degree AV block with escape rhythm at rate of 35bpm and ST segment elevation in inferior leads, requiring temporary transvenous pacemaker placement. Bedside echo shows a borderline LV systolic function (EF 50%) with inferior wall akinesis and a dilated and hypokinetic right ventricle (TAPSE 15mm). In emergency we perform a coronary angiogram, that reveals a total occlusion of the ostial right coronary artery, treated by PCI 2 DES. During clinical course, no complications occur. At hospital discharge, the patient is hemodynamically compensated, without neurological sequelae and with cardiac enzymes in decrease (Trp I HS peak 94 000pg/ml). The aim of this case report is to focus cardiologist’s attention on right ventricular infarction, knowing its clinical presentation and its laboratory, electrocardiographic and echocardiographic features.

P177 COR TRIATRIATUM DEXTER: THE ROLE OF TRANSTHORACIC ECHOCARDIOGRAPHY

Abstract Introduction Cor triatriatum dexter (CTD) is an extremely rare congenital cardiac abnormality and its prompt recognition by transthoracic echocardiography (TTE) is essential for a correct diagnosis. Case presentation An 82–years–old woman was admitted to our cardiologic ward for worsening dyspnoea. The physical examination revealed a bilateral lower extremity oedema with elevated jugular venous pressure and hepatomegaly. The ECG showed atrial fibrillation with a heart rate of 70 bpm already treated with beta–blockers and direct–acting oral anticoagulant. TTE revealed a left ventricle with normal dimensions and function, a dilated right ventricle with a mild systolic dysfunction and increased wall thickness, a marked dilation of the right atrium and a tricuspid severe regurgitation. A deeper analysis of the right atrium revealed the presence of membrane partitioning the chamber into two compartments (Figure 1). The separation was incomplete as demonstrated by the flow across the membrane with Color–Doppler technique (Figure 2). The patient was treated using high doses of diuretics with a subsequent clinical improvement. A percutaneous intervention on the tricuspid valve was planned by the Heart Time. Discussion CTD derives from the persistence of the right valve of the sinus venosus resulting in a complete or incomplete fibromuscular band which separates the right atrium into two cavities. The spectrum of clinical manifestations is broad, based upon the degree of separation. In older patients CTD is often asymptomatic and incidentally found during diagnostic imaging examinations. TTE plays a key role in the identification of CTD, as a first–line diagnostic imaging tool. It, also, allows a differential diagnosis with a prominent eustachian valve, chiari network, and thebesian valve. However, in difficult cases a multiparametric approach is required including transoesophageal echocardiogram or cardiac magnetic resonance. Echocardiography has a pivotal role in the prompt recognition of other cardiac abnormalities associated with CTD such as atrial septal defect or patent foramen ovale, right ventricular hypoplasia and Ebstein’s anomaly. In our case, none of these were documented. Atrial fibrillation should be investigated due to severe atrial chamber dilation. A multiplane evaluation of the right atrium and the Color–Doppler technique are helpful to visualize the configuration of the membrane and evaluate the absence of flow disturbances.

P375 ACUTE PULMONARY EMBOLISM IN A STABLE PATIENT PRESENTING WITH RIGHT CARDIAC CHAMBERS THROMBOSIS: A TICKING CLOCK SCENARIO

Abstract A 73–year–old male presented to the emergency department with a 1–week history of asthenia and exertional dyspnoea. He reported a recent finding of a not yet characterized renal mass in a not relevant past medical history. At the admission, he was haemodynamically stable. Electrocardiogram showed sinus rhythm without relevant abnormalities. Blood results evidenced only a slight increase of hs–troponin levels and D–dimer. An early point–of–care ultrasound (POCUS) demonstrated normal chambers dimensions and biventricular function but in the right atrium an elongated and mobile mass was documented. The echo–features were very suggestive of a large thrombus and a computed tomography pulmonary angiogram revealed several emboli in the left pulmonary artery. At the admission to our cardiac intensive care unit, according to pulmonary embolism (PE) ESC guidelines1, systemic anticoagulation was started with an aPTT–guided unfractioned heparin (UFH) infusion. Intensive monitoring was set to permit early detection of haemodynamic impairment. Multidisciplinary discussion on management was instituted but, soon after, the patient had a PEA arrest. POCUS demonstrated an extremely dilated right ventricle so rescue fibrinolytic therapy (FT) was attempted during cardiopulmonary resuscitation but the patient died. The intermediate–high–risk profile of PE encompasses a very heterogeneous group of patients, in whom controversy still exists regarding the net clinical benefit of using FT compared to anticoagulation alone. In the largest PEIHTO trial, FT compared to UFH alone prevented death and hemodynamic decompensation but increased major bleedings and strokes2. Data meta–analysis confirmed that in these patients, FT was associated with a lower mortality at the cost of more bleedings3. Current guidelines endorse FT only at the moment of clinical deterioration. Nevertheless, stable patients with “thrombus in transit” represent a niche of potentially rapidly deteriorating clinical scenario with not–predictable timing. In this setting we believe that, waiting for data deriving from interventional approaches, an aggressive therapeutical strategy should be considered, offering more liberally an early FT, especially when absolute contraindications are lacking. Data from registry or trials are strongly requested. 1.Konstantinides SV et al. EHJ 2020;41:543–603. 2.Meyer G et al. NEJM 2014;370:1402–11. 3.Chatterjee et al. JAMA 2014;311:2414–21.

Comparative evaluation of echocardiographic and morphometric characteristics of the rat heart left ventricle

Purpose of the study. Comparative evaluation of echocardiographic and morphometric dimensions of the rat heart left ventricle. Materials and methods. The study included 10 outbred male rats weighing 160–180 g. The size of the heart left ventricle was assessed using echocardiography and morphometry. The measurements were carried out according to standard protocols. To perform morphometric measurements, hearts were stopped in systole with a lethal dose (1.0 mg/kg) of 0.025 % strophanthin K solution, and in diastole, by immersing the hearts in a chilled physiological solution (calcium-free medium). Results. It was shown that the systolic size of the left ventricle of the heart according to echocardiography is 1.79Ѓ}0.10 mm, and morphometry 1.64Ѓ}0.09 mm (p = 0,302); diastolic size of the left ventricle, respectively, 3.42Ѓ}0.16 mm and 3.66Ѓ}0.17 mm (р = 0,318). The detected discrepancies do not exceed 10 % and, apparently, are due to the fact that the physiological dimensions of the heart left ventricle are measured by a non-invasive echocardiographic method, and for morphometric measurements of the dimensions in the systole, the heart was stopped by injection of strophanthin K, which entails an overload of heart cells with Ca2+ ions and as a result, contracture of cardiomyocytes; post-mortem cardiac arrest in diastole, in a calcium-free environment, is accompanied by a loss of cardiac muscle tone and, therefore, dilatation of the left ventricle will naturally be greater than physiological. Conclusion. Noninvasive echocardiographic measurement of the size of the heart left ventricle fully reflects the actual size of the left ventricle of the rat heart (the difference does not exceed 10 %), i. e. echocardiographic measurements are valid.

Identification of a novel path for cerebrospinal fluid (CSF) drainage of the human brain.

How cerebrospinal fluid (CSF) drains from the human brain is of paramount importance to cerebral health and physiology. Obstructed CSF drainage results in increased intra-cranial pressure and a predictable cascade of events including dilated cerebral ventricles and ultimately cell death. The current and accepted model of CSF drainage in humans suggests CSF drains from the subarachnoid space into the sagittal sinus vein. Here we identify a new structure in the sagittal sinus of the human brain by anatomic cadaver dissection. The CSF canalicular system is a series of channels on either side of the sagittal sinus vein that communicate with subarachnoid cerebrospinal fluid via Virchow-Robin spaces. Fluorescent injection confirms that these channels are patent and that flow occurs independent of the venous system. Fluoroscopy identified flow from the sagittal sinus to the cranial base. We verify our previous identification of CSF channels in the neck that travel from the cranial base to the subclavian vein. Together, this information suggests a novel path for CSF drainage of the human brain that may represent the primary route for CSF recirculation. These findings have implications for basic anatomy, surgery, and neuroscience, and highlight the continued importance of gross anatomy to medical research and discovery.

Lack of consensus on the definition of severe congenital heart defects for research: A challenge for comparing international studies.

Lack of consensus on the definition of severe congenital heart defects for research: A challenge for comparing international studies.

Evaluation of normal measurements of fourth ventricle by computed tomography

Background: Size of fourth ventricle varies in different pathologies, in the context of enlarged brain ventricles size observed in routine clinical practices, knowledge of the usual range of exact measurement is required. Aims and Objectives: This study is hospital-based observational study to derive normal measurement range of fourth ventricle and correlating it with measurements of cerebrum and age of patients. Materials and Methods: Hospital-based prospective study was conducted in the department of radiodiagnosis in a teaching medical college. Patients with virtually normal study on unenhanced head CT scan of both sex and age groups from 2 year to 60 years, were included in the study. Patients with CT scans showing gross pathological changes affecting the normal anatomy of ventricles were excluded from the study. Results: The mean age of the cases was 37.0±16.88 years. Mean fourth ventricle anterioposterior dimension is 7.5±2.5 mm and transverse dimension is 12±3.1 mm. Anteroposterior diameter of fourth ventricle showed non-significant correlation (r=0.06, P=0.56) with anteroposterior diameter of skull and no significant correlation with (r=0.07, P=0.45) transverse diameter (TD) of skull. AP diameter of fourth ventricle showed a positive correlation with age, which was statistically significant (r2=0.698, P=0.01). TD of fourth ventricle showed slight positive correlation (r=0.202 P=0.048) with anteroposterior diameter of skull and (r=0.142, P=0.168) TD of skull whereas nil with. TD of fourth ventricle showed a positive correlation with age, which was statistically non-significant (r2=0.659, P=0.01). It increases with age. Conclusion: Knowledge of normal measurements helps in assessing, dilated fourth ventricle in various pathologies.

PO-04-015 BIVENTRICULAR ARRHYTHMOGENIC CARDIOMYOPATHY ASSOCIATED WITH A NOVEL HETEROZYGOUS PLAKOPHILIN-2 EARLY TRUNCATING VARIANT

N/A N/A N/A A 31-year-old male patient suffered from palpitations, which occurred particularly during and shortly after physical activity. The patient was very athletic and took part in marathons. His family history was unremarkable. The electrocardiogram showed T wave inversions in V1-V3 (Figure 1A). During bicycle ergometry monomorphic premature ventricular complexes (PVC) and non-sustained ventricular tachycardia episodes (left-bundle branch block morphology, inferior axis) evolved (Figure 1B, black arrow). Of note, under maximal physical stress a second PVC with a right-bundle branch block morphology occurred (Figure 1B, red arrow) suggesting left ventricular (LV) involvement. Echocardiographic evaluation showed a dilated right ventricle (RV) with reduced systolic function, a subtricuspid aneurysm and multiple sacculations of the RV free wall (Figure 1C-D, white markers). Cardiac magnetic resonance imaging (MRI) confirmed the right ventricular findings (Figure 1E, red arrows). In addition, late gadolinium enhancement and fatty deposits were visible in the LV lateral wall (Figure 1F-G, yellow arrows and blue asterix). Genetic testing was performed using a panel covering 173 genes (Agilent, SureSelectQXTTarget Enrichment) and next generation sequencing (NGS) (Illumina MiSeq). It revealed a novel heterozygous nonsense variant in the plakophilin-2 (PKP2) gene (808C>T p. (Gln270Ter) in exon 3) that causes an early truncation, which was confirmed by Sanger sequencing. It was classified as pathogenic (class V). Cascade screening of the asymptomatic patient’s mother (64-y-old), father (70-y-old) and sister (33-y-old) showed that the patient's father and sister harbored the same PKP-2 variant. Both did not report a history of participating in competitive sports, and thorough cardiac evaluation of these two genotype positive family members showed normal findings. Previous studies have indicated that early truncation of the PKP2 C-terminus likely causes arrhythmogenic right ventricular cardiomyopathy (ARVC) irrespective of transcript position. Although desmosomal and non-desmosomal variants create certain genetic potential for the development of ARVC, exercise has an important role in determining the development, severity, and pattern of phenotypic expression. The fact that the only index patient, as a marathon runner, developed ARVC, suggests that the novel PKP2 variant poses a genetic risk, but is not sufficient for inducing an ARVC phenotype by itself.

Case of acute recurrent myocardial infarction in a child

Information on the essential aspects of prevalence, etiology and pathogenesis of coronary heart disease, which is the leading cause of death in the world, is given in this article. It is emphasized that the incidence of ischemic coronary events is the result of a complex sequence of pathogenetic links, which involves the formation of atherosclerotic lesions and destabilization as the primary provoking factor. Purpose - the attraction of the medical community attention to the problem of ischemic heart disease in children. A clinical case of recurrent myocardial infarction in a child who was examined and treated at the MNPE «Ivano-Frankivsk Regional Children’s Clinical Hospital of the Ivano-Frankivsk Regional Council», is described here. The key points of the patient’s complaints, anamnesis of life and this disease are described, the data of the objective condition at the hospitalization and during dynamic observation of the child, which showed the signs of left ventricular overload, coronary blood flow disorder, dilatation of the left heart ventricles, decreased left ventricular contractility. The data of the main indices of laboratory and instrumental methods of research, including X-ray diagnostic methods, performed not only on the basis of the department, but also in the Scientific-Practical Medical Center of Pediatric Cardiology and Cardiac Surgery of the Ministry of Health of Ukraine (Kyiv). The treatment of this clinical case in the child is described in this article. The data of dynamic observation are given. The discussion and conclusions emphasize the problem of coronary insufficiency in pediatrics, which requires clinical understanding, the choice of the correct diagnostic algorithm and qualified medical care. The study was performed in accordance with the principles of the Declaration of Helsinki. Informed consent of the child’s parents was obtained for the research conduction. No conflict of interests was declared by the authors.

Right ventricle dysfunction does not predict mortality in patients with SARS-CoV-2-related acute respiratory distress syndrome on extracorporeal membrane oxygenation support

The prognostic role of right ventricle dilatation and dysfunction (RVDD) has not been elucidated in patients with coronavirus disease (COVID)-related respiratory failure refractory to standard treatment needing extracorporeal membrane oxygenation (ECMO) support. To assess whether pre veno-venous (VV) ECMO RVDD were related to in-intensive care unit (ICU) mortality. We enrolled 61 patients with COVID-related acute respiratory distress syndrome refractory to conventional treatment submitted to VV ECMO and consecutively admitted to our ICU (an ECMO referral center) from 31th March 2020 to 31th August 2021. An echocardiographic exam was performed immediately before VV ECMO implantation. Males were prevalent (73.8%) and patients with a body mass index > 30 kg/m2 were the majority (46/61, 75%). The overall in-ICU mortality rate was 54.1% (33/61). RVDD was detectable in more than half of the population (34/61, 55.7%) and associated with higher simplified organ functional assessment (SOFA) values (P = 0.029) and a longer mechanical ventilation duration prior to ECMO support (P = 0.046). Renal replacement therapy was more frequently needed in RVDD patients (P = 0.002). A higher in-ICU mortality (P = 0.024) was observed in RVDD patients. No echo variables were independent predictors of in-ICU death. In patients with COVID-related respiratory failure on ECMO support, RVDD (dilatation and dysfunction) is a common finding and identifies a subset of patients characterized by a more severe disease (as indicated by higher SOFA values and need of renal replacement therapy) and by a higher in-ICU mortality. RVDD (also when considered separately) did not result independently associated with in-ICU mortality in these patients.

An unusual case of a Puerto Rican patient with involuntary pelvic movements, behavioral changes and mutism associated to VSP13A gene (P3-4.001)

<h3>Objective:</h3> To present a case of a Puerto Rican woman with Chorea-Acanthocytosis positive for VSP13A gene variant presenting with severe pelvic movements. <h3>Background:</h3> Chorea-Acanthocytosis is an autosomal recessive inherited neurodegenerative disorder originated from variants of the VPS13A gene. It is characterized by clinical features that may include chorea, tics, parkinsonism, dystonia, speech disturbances, and epilepsy. Most case reports of Puerto Rican descent have been reported in the United States. <h3>Design/Methods:</h3> NA <h3>Results:</h3> We present the case of a 41-year-old Puerto Rican woman with unusual involuntary movements and mutism. At onset she developed irritability and generalized choreiform movements that worsened over the years. There is family history of undiagnosed involuntary movements and family consanguinity. Clinical symptoms worsened to non-verbal communication, dysphagia, additional behavioral changes, and involuntary movements. Neurological examination showed choreiform movements, orofacial movements and blepharospasm. She presented forceful, brisk and disabling movements of the pelvis and neck present while lying down and worsened with activity. Genetic test for Huntington disease was negative, repeated blood smear did not show acanthocytes. CPK was mildly elevated, liver enzymes and serum ceruloplasmin levels were normal. CSF analysis was unremarkable. Autoimmune encephalitis was suspected and she received a course of IVIG without benefit. EEG was normal. Brain MRI showed abnormal signal intensity of bilateral gray matter involving putamen and caudate nuclei with associated ex vacuo dilation of frontal horns and lateral ventricles. Genetic testing was positive for autosomal recessive homozygous VSP13A gene variant. <h3>Conclusions:</h3> This case illustrates the variable presentation of chorea-acanthocytosis and emphasizes the importance of early recognition and timely genetic testing among Hispanic patients presenting with involuntary movements. Our patient had an uncommon initial presentation with predominant severe pelvic and neck movements making the diagnosis challenging and delayed. An accurate and early diagnosis is important to offer symptomatic treatment, identify potential future complications, and provide genetic counseling. <b>Disclosure:</b> Dr. Enriquez-Gonzalez has nothing to disclose. The institution of Dr. Serrano has received research support from Eli Lilly. The institution of Dr. Serrano has received research support from Abbvie.

Pulmonary Hypertension and Patent Foramen Ovale: A Rare Risk Factor for Stroke (P12-5.022)

<h3>Objective:</h3> To illustrate the importance of a comprehensive and individualized stroke evaluation in young patients with pulmonary hypertension presenting with ischemic stroke. <h3>Background:</h3> Approximately 45% of strokes in the United States are cryptogenic. A common etiology of cryptogenic stroke is a paradoxical embolism through a patent foramen ovale (PFO). Additionally, pulmonary artery hypertension (PAH) is independently associated with 1.5 times higher odds of stroke. It is unclear the compounded risk of stroke in patients with both a PFO and PAH. <h3>Design/Methods:</h3> Case Report and Literature Review. <h3>Results:</h3> A 35-year-old woman with history of PAH presented with vertigo, nausea and headache. Her neurological assessment revealed left-sided dysmetria and truncal ataxia. Brain MRI demonstrated a large left superior cerebellar non-hemorrhagic infarct with partial effacement of the fourth ventricle. She was not a candidate for reperfusion therapy. She required external ventricular drainage. Transthoracic echocardiogram (TTE) showed a dilated right ventricle with reduced systolic function but was otherwise unrevealing. Bilateral lower extremity duplex ultrasound showed no evidence of deep vein thrombosis. The patient was seen in our outpatient multidisciplinary cryptogenic stroke clinic, where repeat TTE with agitated saline injection showed evidence of a PFO. Due to severe pulmonary hypertension, PFO closure was contraindicated, due to potential for worsening right heart function. The patient was started on apixaban for secondary stroke prevention without recurrence of embolic events. <h3>Conclusions:</h3> This unique case report highlights the importance of PFO screening in patients with PAH. Patients with PAH have an increased incidence of stroke, the mechanisms of which are not well understood. In this case, PFO was identified in a patient with severe PAH in our outpatient clinic. Right-to-left shunting in this setting may increase risk of paradoxical embolism. A multidisciplinary approach to cryptogenic stroke patients may facilitate identification of rare mechanisms of stroke as well as improve and personalize secondary stroke prevention. <b>Disclosure:</b> Mr. Arkin has nothing to disclose. Dr. Then has nothing to disclose. An immediate family member of Dr. Ragupathi has received personal compensation for serving as an employee of Merck.

Propylthiouracil Induced Neurotoxicity in Swiss Albino Mice Fetus

Introduction: Anti-thyroid drugs are used to treat hyperthyroidism in pregnancy, as pregnancy-associated hyperthyroidism causes fetal and maternal complications. Propylthiouracil is recommended for the treatment of hyperthyroidism in the first trimester, though limited reports of congenital anomalies are associated with its antenatal usage.Aim: The present study aims to reveal the teratogenicity of propylthiouracil by studying its neurotoxic effect in mice fetuses.Material and Methods: Propylthiouracil was orally administered in a dose of 100 mg/kg body weight/day to pregnant Swiss albino mice during the period of organogenesis (treated group), while pregnant dams of control group were given distilled water orally (same volume and for same duration). The pregnancy was terminatedon the 18th day of conception after sacrificing the pregnant dams and fetuses were collected. The fetal brains (from both, control and treated groups) were dissected out, examined and processed for further histological study.Results: The brains of the treated group shows hemorrhagic spots on gross examination, while microscopic examination shows degeneration in the hippocampal area, degeneratedchoroid plexus and dilatation of lateral and fourth ventricles along with the destruction of ependymal lining of these ventricles. No such findings are present in brain of controlgroup.Conclusion: The safety of propylthiouracil must be re-evaluated through large randomised clinical trials, as it is observed to have neurotoxic effect on mice fetus in the present study

Unrecognized Patent Foramen Ovale in Patient With Sinus Venosus-Type Atrial Septal Defect With Partial Anomalous Pulmonary Venous Return.

Unrecognized Patent Foramen Ovale in Patient With Sinus Venosus-Type Atrial Septal Defect With Partial Anomalous Pulmonary Venous Return.

Improvement of left ventricular ejection fraction in patients with heart failure with reduced ejection fraction: Predictors and clinical impact

BackgroundA percentage of patients with heart failure with reduced ejection fraction (HFrEF) improve left ventricular ejection fraction (LVEF) in the evolution. This entity, defined for the first time in an international consensus as heart failure with improved ejection fraction (HFimpEF), could have a different clinical profile and prognosis than HFrEF. Our main aim was to analyze the differential clinical profile between the two entities, as well as the mid-term prognosis. Material-methodsProspective study of a cohort of patients with HFrEF who had echocardiographic data at baseline and follow-up. A comparative analysis of patients who improved LVEF with those who did not was made. Clinical, echocardiographic and therapeutic variables were analyzed, and the mid-term impact in terms of mortality and hospital readmissions for HF was assessed. ResultsNinety patients were analyzed. Mean age was 66.5(10.4) years, with a male predominance (72.2%). Forty five patients (50%) improved LVEF (Group-1,HFimpEF) and forty five patients (50%) sustained reduced LVEF (Group-2,HFsrEF). The mean time to LVEF improvement in Group-1 was 12.6(5.7) months. Group-1 had a more favorable clinical profile: lower prevalence of cardiovascular risk factors, higher prevalence of de novo HF (75.6% vs. 42.2%; p<0.05), lower prevalence of ischemic etiology (22.2% vs. 42.2%; p<0.05), with less basal dilatation of the left ventricle. At the end of follow-up (mean 19(1) months) Group-1 had a lower hospital readmission rate (3.1% vs. 26.7%; p<0.01), as well as lower mortality (0% vs. 24.4%; p<0.01). ConclusionPatients with HFimpEF seem to have a better mid-term prognosis in terms of reduced mortality and hospital admissions. This improvement could be conditioned by the clinical profile of patients HFimpEF.

The Impact of COVID-19 upon Intracardiac Hemodynamics and Heart Rate Variability in Stable Coronary Artery Disease Patients

The aim. To study the impact of COVID-19 upon intracardiac hemodynamics and heart rate variability (HRV) in stable coronary artery disease (SCAD) patients.&#x0D; Materials and methods. In this cross-sectional study we analyzed clinical and instrumental data obtained from a sample of 80 patients. The patients were divided into three groups: group 1 included patients with SCAD without COVID-19 (n=30), group 2 included patients with SCAD and COVID-19 (n=25), and group 3 included patients with COVID-19 without SCAD (n=25). The control group included 30 relatively healthy volunteers.&#x0D; Results. The changes in intracardiac hemodynamics and HRV in group 2 were characterized by the impaired left ventricular systolic and diastolic function, dilation of both ventricles and elevated systolic pulmonary artery pressure. Left ventricular end-diastolic volume was higher in group 2 (205±21 ml) than that in group 1 (176±33 ml; р&lt;0.001) and group 3 (130±21 ml; р&lt;0.001). Patients in the groups 1–3, compared to controls, presented with the decrease in the overall HRV (by standard deviation [SD] of all NN intervals [SDNN]; SD of the averages of NN intervals in all 5 min segments of the entire recording; and mean of the SDs of all NN intervals for all 5 min segments of the entire recording) and parasympathetic activity (root-mean-square difference of successive NN intervals; the proportion derived by dividing the number of interval differences of successive NN intervals greater than 50 ms [NN50] by the total number of NN intervals [pNN50], and high frequency spectral component), along with QT interval prolongation and increase in its variability. Group 2 demonstrated the most advanced changes in HRV (by SDNN and pNN50) and both QT interval characteristics.&#x0D; Conclusions. The patients with SCAD and concomitant COVID-19, along with both ventricles dilation and intracardiac hemodynamics impairment, presented with the sings of autonomic dysfunction, QT interval prolongation and increase in its variability. The heart rate variability and QT interval characteristics should be additionally considered in the management of such patients.