Risultati per: La gestione del paziente depresso da parte del medico di medicina generale: case reports

Circulation, Volume 150, Issue Suppl_1, Page A4145146-A4145146, November 12, 2024. INTRODUCTION:Familial Hypercholesterolemia (FH) is an underdiagnosed hereditary disorder of Low-density lipoprotein cholesterol (LDL-C) metabolism ,strongly associated with atherosclerotic cardiovascular disease (ASCVD). We present an FH case which shows the importance of early management.CASE REPORT:A 40 year old female with a history of Diabetes Mellitus, Hypertension (HTN), Non ST Elevation Myocardial Infarction (NSTEMI) came for angina like chest pain for 6 months. Her Coronary Angiography (CAG) showed Triple Vessel Coronary Artery Disease with 90% Left Main, 90% Left Anterior Descending, 80% Left Circumflex (LCX), 95% Right Coronary (RCA) arteries occlusion; Coronary Artery Bypass Grafting (CABG) was done. Her medical records showed poorly managed systolic blood pressure readings up to 180-200 mm Hg and hypercholesterolemia despite being on 3 antihypertensives and statins. On physical examination BMI 22 kg/m2, xanthelasmas, corneal arcus, elbow and achilles tendon xanthoma(figure.1),B/L Renal and carotid bruit was present. Her brother had premature CAD, daughters had hypercholesterolemia signs. Lab results indicated high cholesterol with, LDL 372.8 mg/dl; LDL/HDL 11.65. Multi-slice CT showed diffuse atheromatous changes of descending aorta, mesenteric arterial system and > 60% bilateral (L >R) renal artery stenosis(RAS). By Dutch Lipid Clinic Network criteria phenotypic FH was diagnosed and sent to lipid clinic for FH management.DISCUSSION:FH is an Autosomal Dominant disorder that leads to high LDL-C, escalating CAD risk by 20-fold. High intensity Statins cut LDL-C by 50%, pivotal in FH management. In our case late diagnosis of FH led to uncontrolled HTN due to RAS alongside CAD; Early diagnosis and medical management could have prevented the outcome as early initiation of statin in FH reduces their risk to age-matched population levels.Conclusion:FH is a systemic atherosclerosis often at subclinical level that needs to be investigated, also patients with premature ASCVD(male

Circulation, Volume 150, Issue Suppl_1, Page A4141052-A4141052, November 12, 2024. Introduction:Cardio-cerebral infarction, a rare clinical presentation involving simultaneous acute ischemic stroke and acute myocardial infarction, poses significant therapeutic challenges. The incidence of this dual infarction is currently unknown due to its rarity. Delaying intervention for one condition to address the other can lead to permanent morbidity, disability, or even death. Coronary artery fistulas are uncommon with estimated incidence of 0.3%. Among these, a fistula between the left anterior descending artery and the pulmonary artery is the rarest variant, comprising about 17% of all coronary artery fistula cases.Case:A 54-year-old male, with a known history of atrial fibrillation and hypertension, presented to our emergency department with non-rotatory dizziness. Physical examination was unremarkable, but neurological examination revealed medial rectus palsy and left facial asymmetry. A cranial MRI indicated a hyperacute infarction in the left cerebellum. Laboratory tests showed markedly elevated troponin I levels ( >50 ng/ml) and atrial fibrillation, along with inferior wall ST elevation on the electrocardiogram. Due to the high risk of hemorrhagic conversion, the loading of antiplatelets was deferred. Instead, the patient was treated with Aspirin 80 mg once daily, Clopidogrel 75 mg once daily, and Enoxaparin 0.4 ml subcutaneously once daily. A 2D echocardiogram revealed an ejection fraction of 43%, hypokinesia of the anterior and intraventricular septum from base to apex, and severe mitral stenosis. Cardiac catheterization identified a coronary artery fistula from the left anterior descending coronary artery to the main pulmonary artery. Treatment for acute coronary syndrome and acute cerebellar infarct continued. An open-heart surgery was considered. However, during his hospital stay, the patient experienced hemorrhagic conversion and altered sensorium. His condition further deteriorated, necessitating a tracheostomy and long-term care.Conclusion:Cardio-cerebral infarction is an extremely rare and poorly studied syndrome that presents significant treatment challenges and carries a grave prognosis if not addressed immediately. The medical conundrum of deciding which condition to treat first underscores the need for further research. Both interventional cardiologists and interventional neuroradiologists play crucial roles in the effective management of this emergency condition.

Circulation, Volume 150, Issue Suppl_1, Page A4146404-A4146404, November 12, 2024. Background:Pregnancy poses physiologic changes that can precipitate severe or fatal cardiovascular events among women with hypertension (HTN). Understanding mechanisms by which delivery might contribute to these events in women with HTN is critical for prevention.Aims:To examine trends in markers of cardiac wall strain or inflammation measured via N-terminal pro b-type natriuretic peptide (NTproBNP) and high-sensitivity C-reactive protein (hs-CRP) in pregnancies with and without HTN.Methods:In a prospective, 1:1 case-control design, we enrolled pregnant women with and without HTN between 24-32 weeks gestation (2019-2022). HTN was defined by a clinician diagnosis of chronic or gestational HTN or a baseline blood pressure (BP) ≥140/90 mm Hg. The control group (no HTN) had a systolic BP

Circulation, Volume 150, Issue Suppl_1, Page A4143294-A4143294, November 12, 2024. Introduction:Cardiac pacing devices, including Permanent Pacemakers (PPM) and Implanted Cardioverter Defibrillators (ICD), are essential in managing various cardiac conditions. Exercise capacity in patients hinges on both cardiac function enhancement and an appropriate chronotropic response. This case report underscores the strategic use of Cardiopulmonary Exercise Testing (CPET) to determine the activation of the Rate Response to Exercise (RRE) function in a patient with Heart Failure with reduced ejection fraction (HFrEF).Case Report:Our patient is a 64-year-old male with HFrEF due to hypertensive heart disease and ICD placement. He was undergoing therapy optimization at our advanced heart diseases clinic. Initially presenting with poor functional capacity, his condition improved significantly with ongoing therapy adjustments, as reflected by his KCCQ scores. This improvement enabled a comprehensive assessment using CPET. The initial CPET trial revealed moderately reduced exercise capacity and an absent chronotropic response, with a heart rate at 65 bpm and a peak VO2 of 67% predicted, categorizing him as Weber Class B. Based on these findings, we activated the RRE function and conducted a follow-up CPET. The re-evaluation demonstrated notable improvements: extended exercise duration post-anaerobic threshold, a peak VO2 of 72% predicted, and an upgraded Weber Class A status.Summary:This case exemplifies how tailored programming of pacing devices can significantly enhance exercise capacity in HF patients. Activating the RRE function in the patient’s PPM led to marked improvements in exercise capacity and prognostic indicators derived from CPET parameters, corroborating the limited existing data on this approach. Although specific guidelines are lacking, our findings highlight the utility of CPET in customizing PPM settings, particularly for HFrEF patients. This report underscores the importance of individualized treatment and invites further research into optimizing device settings to maximize patient outcomes. Leveraging CPET in routine clinical practice could revolutionize heart failure management, offering a precise and patient-centric approach.

Circulation, Volume 150, Issue Suppl_1, Page A4140084-A4140084, November 12, 2024. Description of case:A 55 yo man with no PMHx presented with a few hours of intermittent left-sided chest tightness.Serial EKG revealed dynamic ST-T wave changes in lateral leads—Figure 1.His lipid panel was abnormal, and the High-Sensitive Troponin T trended up—table 1.Based on the hemoglobin A1C result of 10.6, he was newly diagnosed with DM.Coronary angiography shows two vessel diseases. The left circumflex artery’s first obtuse marginal artery branch (LCX-OM1) received a stent in the first instance.During the planned PTCA for treatment of LAD 48 hours after, he developed sudden bilateral loss of vision while on the table just before stent deployment.In this case, with the emergence of the symptom of vision loss, PTCA was halted to allow the operator to rule out an acute cerebrovascular accident (CVA) through urgent CT head and MRI brain scans.He consented to complete the LAD PTCA the day after, which achieved the intended restenosis without complications.Discussion:Bilateral transient cortical blindness (TCB) is a reversible syndrome of vision loss affecting both eyes in the presence of normal visual pathways due to a brief disturbance in the occipital cortices. TCB is more commonly associated with cerebral angiography but has been rarely reported after coronary angiography.Angiographic contrast toxicity is one of the proposed pathophysiologic mechanisms. During our case, approximately 30 mL of iohexol was used before TCB, while about 120 mL of iodixanol was used for the repeat and conclusive procedure.We are not aware of a previous report in which PTCA was recommenced hours after being halted due to TCB in a patient suffering acute coronary syndrome.The absence of symptom recurrence when the PTCA was recommenced, which underscored the benign nature of TCB, enabled the patient to receive conclusive, definitive treatment for his acute coronary condition.

Circulation, Volume 150, Issue Suppl_1, Page A4145402-A4145402, November 12, 2024. INTRODUCTION:Peripartum cardiomyopathy (PPCM) is a form of systolic heart failure, which occurs late in pregnancy or within the first 5 months postpartum and it is the most common cause of death related to heart failure in pregnant women. To establish the diagnosis, other causes of heart failure must be ruled out and a left ventricular ejection fraction (LVEF)

Circulation, Volume 150, Issue Suppl_1, Page A4146391-A4146391, November 12, 2024. Background:Accurate ECG interpretation is critical for triaging, diagnosis, and managing patients with cardiovascular conditions. Current computerized methods have limited accuracy and are often proprietary algorithms based on raw signal data. We sought to evaluate ECG-GPT, a novel vision-text transformer model capable of generating free-text diagnosis statements from images of ECGs.Methods:ECG-GPT was developed with 3 million ECGs at Yale and validated in 2 large and geographically distinct populations: (1) 1.4 million ECG images with diagnosis statements from Mount Sinai Health System (MSHS), NY, and (2) 45,389 ECG images with 6 diagnostic labels from UK Biobank (UKB). We employed 2 metrics: (A) a rule-based approach to evaluate diagnostic accuracy for 19 rhythm and conduction disorders on MSHS ECGs and 6 key conditions on UKB ECGs, and (B) semantic similarity (similar meaning) using a fine-tuned DistilBERT model. Using semantic similarity methods, we assessed the quality of generated text across the full range of ECG diagnoses by computing similarity both for all model-generated and reference statements and for subsets specific to ECGs flagged for each of the 19 extracted conditions. To establish baseline similarities, we also computed semantic similarities between random combinations of model-generated and reference statements within each of these sets.Results:The model performed well across multiple key labels, spanning rhythm and conduction disorders, including an AUROC of 0.92 for atrial fibrillation, 0.95 for left bundle branch block, and 0.90 for atrioventricular block (Table A). More importantly, in addition to the labels, ECG-GPT identified the full context of the diagnosis statements with allied conditions. It had a median pairwise cosine similarity of 0.86 (IQR 0.78-0.94), significantly greater than the median baseline similarity of 0.73 (IQR 0.66-0.79, p

Circulation, Volume 150, Issue Suppl_1, Page A4134594-A4134594, November 12, 2024. Description of Cases:Case 1: A 72 year old male with atrial fibrillation (AF) and tachy-brady syndrome underwent Micra implantation. On post-procedure day 2, pacing thresholds were unacceptably elevated. A new Micra device was deployed; subsequently, a microsnare was placed through the Micra delivery catheter and the docking button of the old Micra was successfully snared (Figure A). The old Micra was then captured over the delivery catheter and removed from the body.Case 2:A 76 year old male underwent Micra implantation and AV node ablation for AF with persistent tachycardia. One month later, he presented with heart failure and interval decrease in ejection fraction from 66% to 33%. After biventricular leads were implanted, an Agilis catheter was advanced towards the Micra. The docking button was successfully snared with a gooseneck snare (Figure B), and the Micra was extracted with gentle traction.Case 3:A 76 year old male with complete heart block and longstanding persistent AF received a Micra. Nine months later, he presented with heart failure and interval decrease in ejection fraction from 55% to 25%. A defibrillator lead was implanted but no suitable CS branch was identified. The Aveir retrieval catheter was advanced and the Micra docking button was captured with the triloop snare (Figure C). The protective sleeve was advanced over the Micra and it was successfully removed. Subsequently, a left bundle area lead was placed.Discussion:Although uncommon, extraction of the tined leadless pacemaker (Micra) may be indicated at times. As rates of implantation continue to increase, establishing uniform procedures for extraction becomes increasingly important. These three cases highlight unique options for Micra extraction, and the feasibility of each approach. However, a dedicated tool and protocol would be ideal for patient safety and consistency. No complications occurred with the above cases.

Circulation, Volume 150, Issue Suppl_1, Page A4139444-A4139444, November 12, 2024. Case Presentation:A 39-year-old Southeast Asian female presented with fatigue and exertional dyspnea. She underwent a myomectomy four years ago. Examination showed vital signs within normal limits, except for an SpO2 of 87%. Blood tests indicated microcytic hypochromic anemia and elevated D-Dimer levels of 1479 ng/mL.A transthoracic echocardiogram revealed a large mass occupying almost the entire right atrium, extending into the inferior vena cava. A CT scan showed a large, low attenuation, and minimally enhanced mass extending from the right atrium into the inferior vena cava and right hepatic vein, possibly invading segments VII-VIII of the liver (Figure 1). Abdominal CT and MR images revealed multiple degenerating uterine fibroids, bilateral dilated uterine tubes, and mild right hydronephrosis (Figure 2).The patient underwent surgical removal of the cardiac mass under general anesthesia with extracorporeal circulation and hypothermia. Pathological examination confirmed a benign tumor characterized by bundles of smooth muscle cells (Figure 3). Immunohistochemical staining with Actin (+), Progesterone receptor (+), Desmin (+), Ki67 (+) was consistent with a diagnosis of benign metastasizing leiomyoma (BML) of the heart. Subsequently, elective hysterectomy and bilateral salpingo-oophorectomy were performed.Discussion:Although rare, uterine leiomyoma can exhibit metastatic behavior, disseminating to extraneous anatomical sites. BML should be considered in the differential diagnosis when patients present with an atypical cardiac mass and have a history of uterine leiomyoma, myomectomy, or hysterectomy.BML of the heart is an exceptionally rare condition, predominantly affecting middle-aged women who have undergone hysterectomy or myomectomy. While BML commonly occurs in pre-menopausal patients and shares a potentially similar mechanism with endometriosis, as indicated by its response to estrogen-blocking agents, the occurrence of BML in post-menopausal women suggests the involvement of other contributing factors.Multiple imaging modalities play a significant role in the diagnosis and preparation for surgical management.

Circulation, Volume 150, Issue Suppl_1, Page A4140250-A4140250, November 12, 2024. Background:Papillary fibroelastomas (PFE) arising between the left upper pulmonary vein and left atrial appendage, commonly referred to as the coumadin ridge or Q-tip, are extremely rare and best detected by transesophageal echocardiography (TEE). Although PFEs are benign, they have been associated with a range of serious embolic events.Objective:To analyze the characteristics, clinical findings, and diagnostic techniques of PFEs originating from the coumadin ridge.Methods:We describe 13 cases of pathology proven papillary fibroelastomas uniquely attached to the Q-tip. Patient data, including demographics, mass characteristics and diagnostic imaging were collected and analyzed.Results:Thirteen patients with pathology-proven PFEs attached to the coumadin ridge were included in the study. The median age of the cohort is 73 (IQR 67-77), and 54% of the patients were male. Tumor sizes ranged from 2mm to 20mm with a median of 5mm (IQR 3.5-8.5). Twelve (92%) of the PFEs were initially visualized on TEE, and one case was diagnosed postoperatively by pathologists after the excision of the left atrial appendage. The majority of cases were incidental diagnoses, with 46% of cases incidentally discovered intraoperatively by routine TEE, and 30% visualized on TEE done for diverse reasons including valvular assessment, exclusion of infective endocarditis, and coronary artery disease (CAD) assessment. One case was incidentally detected on CMR performed as part of a metastatic tumor workup. Four (30%) patients presented with symptoms of stroke/TIA, of which 2 had a previous history of stroke. One patient had symptoms of both TIA and angina pectoris. Cardiovascular disease risk factors were prevalent: 69% had hyperlipidemia, 46% had atrial fibrillation (Afib), 31% had CAD, 54% had hypertension, and 46% were former smokers.Conclusions:Masses on the Q-tip are rare but significant intracardiac sources of embolism. Accurate identification and categorization of these masses are challenging, yet critical, to guide appropriate surgical intervention and improve patient outcomes. Further studies are needed to establish standardized diagnostic and treatment protocols for this unique presentation of PFEs.

Circulation, Volume 150, Issue Suppl_1, Page A4120854-A4120854, November 12, 2024. Introduction:Familial hyper-alpha-lipoproteinemia (HALP) is a heterogenous genetic lipid disorder that is found in only 8% of the population and manifests as elevated HDL levels above the 90thpercentile. HALP is due to mutations in various genes including cholesteryl ester transfer protein (CETP), hepatic lipase, or apolipoprotein C-III (APOC3). While epidemiological studies have noted an inverse relationship between high HDL and the development of coronary artery disease, recent data have shown a lack of causal atheroprotective effects. We present a case of a patient with significantly elevated HDL and peripheral vascular disease.Case Description:Patient is a 64-year-old female with past medical history of peripheral artery disease with occlusion of the left femoral artery and popliteal arteries status post angioplasty, hypertension, type 2 diabetes, alcohol use, and CKD Stage 4 who presented to the advanced lipid clinic for management of elevated lipoproteins. Patient’s laboratory data was significant for total cholesterol (TC) of 375 mg/dL, a high-density lipoprotein (HDL) of >200 mg/dL, triglycerides (TG) of 66 mg/dL, and a low-density lipoprotein (LDL) of 175 mg/dL. Further testing revealed elevated apolipoprotein A-I of 231 mg/dL. Patient was subsequently initiated on a high intensity statin with improvement in her lipid panel with a TC of 247 mg/dL, HDL of 133 mg/dL, TG of 50 mg/dL, and LDL of 106 mg/dL, with plan for further uptitration of lipid therapy to target LDL

Circulation, Volume 150, Issue Suppl_1, Page A4145414-A4145414, November 12, 2024. Background:Kawasaki disease (KD) is a systemic vasculitis that is rare and underdiagnosed in adults given its atypical presentation, especially in non-Asian patients. Incomplete KD is characterized by less than 4 of the classic clinical findings of mucocutaneous inflammation and is more common in adults. Kawasaki shock syndrome (KSS) is a rare complication that has features of both cardiogenic and vasoplegic shock.Case:A 48-year-old Black male was admitted for subjective fevers, abdominal pain, cervical adenopathy, and jaundice. Labs include WBC count 13320/cmm with 85% neutrophils, total bilirubin 13.1 mg/dL, direct bilirubin 9.3 mg/dL, AST 64 U/L, ALT 191 U/L, ALP 143 U/L, GGT 201 U/L, and urinalysis with pyuria. Microbiological workup and abdominal imaging were unremarkable. Subsequently, he developed chest pain with hypotension, diffuse ST elevations on ECG, and hsTropI of 638 ng/L. Cardiac catheterization revealed no obstructive CAD but elevated filling pressures with cardiac index of 1.8 L/min/m2, suggestive of myopericarditis with cardiogenic shock. IABP was inserted. TTE revealed EF of 20-25% with global hypokinesis, reduced RV systolic function, mild MR, and mild pericardial effusion. Significant labs include ESR 102 mm/hr, CRP 22 mg/L, ferritin 775 ng/mL, albumin 2.3 gm/dL, hemoglobin 9.3 gm/dL, and platelets 703000/cmm. High-dose aspirin and colchicine were given with significant improvement. IABP was removed 2 days post-placement. Cardiac MRI at 1-week post admission revealed reduced LV systolic function and diffuse pericardial enhancement. Aspirin, colchicine, and GDMT were prescribed at discharge. At 2-week follow-up, symptoms had resolved with normalization of labs. Clinical findings, lab abnormalities, and resolution with high-dose aspirin were suggestive of incomplete KD. The patient was subsequently lost to follow-up.Discussion:Incomplete KD should be considered in an adult patient with myopericarditis and evidence of severe systemic inflammation when all classic findings are not present as current criteria are intended for children. Cervical adenopathy and hepatitis are more common in adults while coronary artery aneurysms are rarer. Early mechanical support in KSS may be beneficial.

Circulation, Volume 150, Issue Suppl_1, Page A4138112-A4138112, November 12, 2024. Background:Mitral annular calcification (MAC) is a common incidental finding associated with advanced renal dysfunction, hyperlipidemia, hypertension, or abnormal calcium metabolism. Caseous mitral annular calcification (CMAC) is a less common MAC variant involving central liquefaction necrosis, which results in a paste-like substance consisting of calcium, fatty acids, and cholesterol.Case description:A 64-year-old female presented to clinic with chronic shortness of breath and fatigue that started after COVID-19 pneumonia in 2021. As part of the initial evaluation, a transthoracic echocardiogram (TTE) was done, which revealed moderate annular calcification, 3+ mitral valve regurgitation, and a 1.8×1.5 cm echogenic mass on the posterior mitral annulus, concerning for possible fungal vegetation. Further multimodality imaging was pursued. Transesophageal echocardiogram confirmed the presence of a 2.0×1.7 cm cystic mass but demonstrated normal mitral valve function. Cardiac MR further identified a 1.2×0.8×1.5 cm hypointense mass, with surrounding late gadolinium enhancement, suggesting an associated inflammatory/degenerative process. Gated cardiac CT scan showed a mass with ring-like dense peripheral calcification and low central attenuation. Overall, findings from multimodality imaging were most consistent with CMAC. In the present case, the patient was monitored conservatively, as her symptoms of shortness of breath improved, and she did not experience any associated complications.Discussion:The present case demonstrates an incidental finding of an echogenic mass on the posterior mitral annulus, later identified as CMAC. Initial imaging with TTE helps evaluate for CMAC. However, on TTE alone, CMAC is often misdiagnosed with other conditions, including intracardiac tumors, abscesses, vegetations, or thrombi, and more advanced imaging is often recommended. On cardiac MR, CMAC is usually hypointense due to the elevated calcium content and may have surrounding late gadolinium enhancement due to associated inflammation. On gated cardiac CT, CMAC presents with a hyperintense rim due to calcification and a hypointense center consistent with central necrosis. As in this patient, CMAC is usually a benign condition that can be monitored conservatively. Indications for invasive intervention include significant valvular dysfunction, embolization, or conduction abnormalities. Multimodal imaging can aid in appropriate diagnosis to mitigate unnecessary interventions.

Circulation, Volume 150, Issue Suppl_1, Page A4112944-A4112944, November 12, 2024. Case:A 62-year old male with a history of chronic kidney disease and paroxysmal atrial fibrillation presented with several months of fatigue, a progressive decline in functional status (NYHA IIIb-IV), weight loss, and intermittent lower extremity swelling. Blood pressure was 92/72 mmHg on presentation, and heart rate was 90 bpm. Physical examination revealed a jugular venous pressure of 12-14 cm H2O and 2+ bilateral lower extremity edema with mild wheezing.Methods/workup:Laboratories are shown in Table 1 and were notable for a serum free light chain ratio of 0.14. Transthoracic echocardiogram (figure 1) revealed a left ventricular (LV) ejection fraction of 33% with increased LV wall thickness and severe bi-atrial enlargement. Bone marrow biopsy revealed 2% lambda restricted plasma cells without evidence of amyloid deposits; metastatic bone survey was negative. A fat pad biopsy and endomyocardial biopsy stained negative for amyloid with Congo red. Cardiac MRI showed diffuse, circumferential subendocardial late gadolinium enhancement, most compatible with cardiac amyloidosis (figure 2). The patient also had a chest CT scan that demonstrated a few patchy nodular pulmonary infiltrates. Given his extensive negative workup, he was referred to pulmonary and endobronchial biopsy was positive for amyloid on Congo red staining (figure 3). Biopsy tissue typing revealed Lambda subtype AL amyloidosis.Management:The patient was started on Daratumumab, Cyclophosphamide, Bortezomib, and Dexamethasone by Hematology. Unfortunately, about a month after his diagnosis, he developed worsening heart failure despite treatment. He was admitted to an outside hospital with refractory cardiogenic shock and passed away during that admission.Discussion:The diagnosis of AL amyloidosis requires a high index of suspicion for prompt diagnosis and treatment. We present a case in which cardiac amyloidosis was suspected based on the patient’s clinical picture and cardiac imaging; however, multiple initial biopsies were negative. An expedited, multimodal and interdisciplinary diagnostic assessment is imperative as prognosis with chemotherapy is highly dependent on extent of AL disease, patient functional status, and presence of cardiac compromise. Elevated natriuretic peptides, troponins, autonomic dysfunction, and hypotension are negative prognostic features.

Circulation, Volume 150, Issue Suppl_1, Page A4120434-A4120434, November 12, 2024. Introduction:Mitral annular disjunction (MAD) is an abnormality of the mitral valve where a portion of the mitral annulus and valve leaflets insert into the left atrium away from the fibrous annulus. Since its identification in 1981 its diagnosis has increased due to the advancements in cardiac imaging. It’s association with ventricular arrythmia (VA) and sudden cardiac death (SCD) underscores the importance of its identification. We present a case of ventricular tachycardia (VT) that is attributed to MAD with associated sub-mitral valve left ventricular aneurysm (SMA) formation.Case:A 51-year-old African American male with history of posterior papillary muscle premature ventricular contractions (PVC), PVC induced cardiomyopathy, and family history of sudden cardiac death in his mother who presented to the emergency department due to palpitations. Electrocardiogram (ECG) showed sustained monomorphic VT at a rate of 160 bpm. This converted to normal sinus rhythm with intravenous amiodarone and metoprolol. Further work up included cardiac magnetic resonance which identified a 4 x 1.8 x 1.4 cm SMA on the basal inferior and inferolateral walls. There was near transmural late gadolinium enhancement (LGE) of the aneurysm and an associated 7 mm of MAD with posterior mitral valve prolapse (MVP). He underwent left heart catheterization which was normal. ECG showed a superior right axis with associated right bundle branch block which correlated to a basal inferolateral exit and to the location of the SMA. Ultimately the patient underwent implantation of secondary prevention implantable cardioverter-defibrillator and initiation of sotalol.Discussion:The prevalence of MAD is approximately 30% in those with MVP and 8% in the general population. Those patients with MAD and MVP appear to be at higher risk for VA and SCD. The most likely mechanism for this association is the fibrotic changes that occur in the basal inferolateral left ventricle because of mechanical stretch on the papillary muscles by the prolapsing leaflets. The scar and aneurysm that develop can serve as substrate for reentrant VA. Treatment for this involves surgical repair of the mitral valve annulus and correction of the MAD distance which has been shown to improve arrythmia.Conclusion:This case illustrates an important but poorly understood arrhythmogenic entity that will increase in incidence as cardiac imaging advances. Its implications in VA and SCD warrant further research.

Circulation, Volume 150, Issue Suppl_1, Page A4124313-A4124313, November 12, 2024. A 30-year-old male electrician presented with two days of fever and positional left shoulder pain and was found to have elevated inflammatory markers and a large pericardial effusion with echocardiographic evidence of tamponade for which he underwent pericardiocentesis. He was discharged on a course of anti-inflammatory therapy with presumed diagnosis of idiopathic pericarditis based on negative cytology. He returned 6 months later with several weeks of upper respiratory infection symptoms as well as new abdominal discomfort and emesis.On presentation, his vital signs were notable for tachycardia to 113 and cardiac exam was notable for tachycardia, regular rhythm, no rubs or murmurs, nondisplaced precordial impulse, normal jugular venous pressure with Kussmaul sign, and pulsus paradoxus of 6. An electrocardiogram showed sinus tachycardia with diffuse ST segment changes (Figure A). His cardiac biomarkers were unremarkable (troponin 21). His labs revealed normocytic anemia with hemoglobin of 11 and erythrocyte sediment rate above assay. Echocardiogram demonstrated a circumferential complex pericardial effusion with echocardiographic evidence of early tamponade (Figure B, C). A pericardiocentesis was attempted with inability to advance wire within pericardial space. A malignancy workup was initiated. CT demonstrated multi-station thoracic and lower cervical lymphadenopathy, moderate left pleural effusion, and an intrapericardial mass with associated pericardial effusion (Figure D). Cardiac magnetic resonance imaging demonstrated a circumferential intrapericardial non-mobile mass measuring up to 22 mm in thickness posteriorly and the mass was isointense to myocardium indicating low-fat content (Figure E). A supraclavicular lymph node biopsy was obtained with immunohistochemical staining positive for WT1 and calretinin, consistent with metastatic epithelioid mesothelioma of pericardial versus pleural etiology (Figure F). A PET-CT subsequently showed a FDG-avid circumferential anterior pericardial mass and multiple pleural-based lesions concerning for metastatic mesothelioma. He was initiated on pemetrexed/carboplatin systemic chemotherapy. With systemic therapy, his disease has been stable for 6 months. Pericardial mesothelioma is a rare malignancy. This case underscores the diagnostic challenges associated with pericardial mesothelioma and the importance of cancer workup as part of the pericardial effusion workup.