Left Sternal Border Research Articles

P1321 A case of multiple levels of ventricular obstruction

Abstract Subaortic stenosis (SAS) is a rare entity in adults with unclear etiology and variable presentation. SAS may be presented with symptoms mimicking Hypertrophic Cardiomyopathy (HCM). Often a combination of imaging modalities is needed to distinguish SAS from HCM with obstruction. A 53 years old man, smoker, was referred to our medical center suffering from shortness of breath on exertion. He first presented at another facility with a 2 month history of shortness of breath and chest discomfort during brisk physical activity and the possible diagnosis of HCM was made. On physical examination, a 3/6 systolic murmur was audible along the left sternal border, that became louder with standing and the Valsalva maneuver. The patient had non distended jugular veins, clear lung fields and no ankle edema. The results of laboratory exams did not reveal any pathological sign. The transthoracic echocardiogram revealed significant left ventricular hypertrophy (Interventricular septum 21 mm, Posterior wall 16 mm) with normal left ventricular systolic performance (ejection fraction >70%). The aortic valve was tricuspid and calcified whereas mitral valve was morphologically normal, with systolic anterior motion and mild posterolaterally directed regurgitation. Two systolic gradients, one dynamic, late peaking of 85mmHg and another fixed of 70mmHg were detected in left ventricular outflow track (LVOT). Transesophageal echocardiography was performed for the better evaluation of aortic valve and showed a three level obstruction caused by the systolic motion of the mitral valve towards the hypertrophic septum at LVOT, the presence of a membranous subaortic membrane and the calcified aortic valve respectively. The aortic valve was calcified with a moderate stenosis (0.8cm2 / m2) from 3D planimetry. A Cardiac Magnetic Resonance exam was ordered and confirmed the significance of hypertrophy and the presence of circumferential subaortic membrane. No late enhancement after the administration of Gadolinium was observed. Coronary angiography was performed and demonstrated normal coronary arteries. We hypothesized that the presence of subaortic membrane led to marked myocardial wall thickness and to the destruction of the aortic valve due to turbulent flow in the LVOT. The patient was referred for surgical management Extended septal myectomy combined with complete resection of orbital subaortic membrane were performed. he calcified aortic valve was replaced by bioprosthetic valve No 23mm. The patient tolerated the procedure well with significant symptomatic improvement. TTE performed 1 month postoperatively showed no remarkable LVOT gradient. The results of histopathology and genes investigations are pending. Subaortic membrane is a rare cause of symptoms that can mimic hypertrophic cardiomyopathy. A combination of imaging modalities is needed to distinguish subaortic stenosis from aortic valve stenosis and hypertrophic obstructive cardiomyopathy. Abstract P1321 Figure. Three levels obstruction

P876 I may still steal you : huge coronary cameral fistula (non-dominant right coronary artery to right ventricle) in a neonate causing heart failure

Abstract Funding Acknowledgements Not applicable OnBehalf Not applicable A 25 days old neonate, weighing 2.8kg was brought with complaints of feeding difficulties, not gaining birth weight and rapid breathing. The parents had not noticed any cyanosis. He was further referred to us as the treating paediatrician heard a pansystolic murmur over the left sternal border. When examined, baby was afebrile, tachypnoea (70 per minute), regularly tachycardia(180bpm). We did not appreciate any dysmorphism. On cardiovascular examination we found a mild cardiomegaly, RV S3, mild hepatomegaly with no signs of active infection. Systemic saturations were 98% in all limbs. Other systemic examinations were essentially normal. 12 lead ECG showed, physiological RV dominance, with no signs of LV volume overload. Chest radiograph showed cardiomegaly of RV type with adequate PBF. 2 D echocardiogram : The intra-cardiac structures were found to be normal. A huge coronary-cameral fistula originating from RCA, traversing in right AV groove and draining into the RV with mild RV enlargement. No signs of PAH. Biventricular function was preserved. CT coronary angiogram : CT confirmed the presence and anatomy of the RCA—RV fistulous tract seen on echocardiography. The fistula measured ∼ 6mm at the origin and 3.5mm at the point of entry into RV. As we could not see any PDA arising from RCA and a twig from LCX traversing in interventricular groove, we thought it’s a left dominant coronary circulation. Hemodynamics in the index case The symptoms of heart failure in the index neonate is due to the volume overload on the RV. The non-dominance of RCA is probably a blessing in disguise for this baby in preventing coronary steal. The "steal phenomenon" will be apparent once the physiological (high) PVR comes down. Plan Baby is on diuretics and nutritional support, under weekly follow up. We will be performing catheter study (around 12 weeks of life or as per clinical indications) to look for dominance and need/feasibility of device closure. Abstract P876 Figure.

P1242 Giant right atrium aneurysm presenting as right heart failure

Abstract Introduction Idiopathic aneurysmal dilatations of the right atrium are rare anomalies. It can be diagnosed at any time between foetal and adult life. This exceptional condition can be confused with other conditions that involve enlargement of right atrium. We report a clinical case of a symptomatic adult who was diagnosed with giant right atrium aneurysm. Case report An 83-year-old female presented with complaints of fatigue, paroxysmal nocturnal dyspnoea, exertional dyspnoea, orthopnoea and cough since last week. There were no history of syncope, convulsions or evidence of thromboembolism. There were a medical history of diverticulosis and atrial fibrillation (warfarin therapy). The principal findings on physical examination included holosystolic murmur at the left middle sternal border, pulmonary rales, jugular venous distension, enlarged liver and peripheral oedema. An electrocardiogram showed an atrial fibrillation with a controlled heart rate response, right axis deviation, right bundle-branch block. A chest radiography posteroanterior view showed a markedly enlarged cardiac silhouette, increased pulmonary vascular congestion, and bilateral pleural effusions. Computed tomography (CT) scan showed aneurysmal dilated right atrium communicating with right ventricle. Right ventricle (RV) and RV outflow tract were dilated with normal pulmonary arteries. Two-dimensional transthoracic echocardiography revealed aneurysmal dilated right atrium measuring 398mL/m2. The tricuspid valve was no displaced. There was severe tricuspid regurgitation and no stenosis. The right atrium was kinetic without any intracavitary thrombus. The intertrial and interventricular septa were intact. The right ventricle and outflow tract were mildly dilated with preserved systolic function. The left atrium and left ventricle were normal. The patient was admitted to the cardiology department with the diagnosis of right heart failure. Conclusion Aneurysm of right atrium is an uncommon condition. It is diagnosed as a disproportionately enlarged right atrium compared to the other cardiac chambers in the absence of other cardiac or hemodynamic abnormalities and must be distinguished from other anomalies causing structural pathology of the right atrium. Approximately, one-half of the patients have no symptoms. Others presented with arrhythmia, palpitations, chest pain, shortness of breath, and fatigue. The major rhythm abnormality is atrial fibrillation or atrial flutter. Our patient presented with symptoms of right heart failure and atrial fibrillation. The right enlargement is usually associated with tricuspid annular dilatation responsible for functional regurgitation, which can be severe in some cases. The diagnosis of right atrium malformation can be established by echocardiography, CT or magnetic resonance imaging. Literature reports various ways to manage these patients. Treatment ranges from conservative to surgical resection specially in the presence of arrhythmias. Abstract P1242 Figure. Aneurysm of right atrium

P1727 Large coronary artery to pulmonary artery fistula as primary source of pulmonary blood supply in tetralogy of fallot with pulmonary atresia

Abstract Funding Acknowledgements Not Applicable OnBehalf Not Applicable Introduction Pulmonary blood supply in patients of Tetralogy of Fallot with pulmonary atresia is usually from patent arterial duct or major aortopulmonary collaterals (MAPCAs) arising from descending thoracic aorta. We describe a case in which large coronary to pulmonary artery fistula was the primary source of pulmonary blood supply. Case Report A 17 years old female was referred to our hospital for diagnostic workup of suspected congenital heart disease. She was previously undiagnosed and now complains of progressive shortness of breath for last few months. On physical examination she was non-dysmorphic with oxygen saturation of ∼ 77 % in room air, blood pressure of ∼ 117/72 mmHg, pulse rate of ∼ 89 beats per minute and respiratory rate of ∼ 24 breaths per minute. She was clinically cyanosed with grade 3 clubbing and polycythemic. Cardiovascular examination revealed quiet precordium with normally placed apex beat, grade 2 parasternal heave with single second heart sound and grade 3/6 continuous murmur along left mid sternal border. Twelve lead electrocardiogram (ECG) showed normal sinus rhythm, right axis deviation and right ventricular hypertrophy. There was no evidence of ischemia. Chest X-ray revealed "boat shaped heart" with oligaemic lung fields. Transthoracic echocardiography showed large conoventricular ventricular septal defect with bidirectional flow. There was aortic over-ride with dilated left main coronary artery. No forward flow was seen across right ventricular outflow tract. Considering hugely dilated left main coronary artery, suspicion of coronary to pulmonary artery fistula was made and cardiac computed tomography followed by conventional angiography was done, both confirmed the diagnosis of Tetralogy of Fallot with pulmonary atresia and large coronary artery to main pulmonary artery fistula as a primary pulmonary blood supply. Two small collaterals (MAPCAs) were also identified supplying small part of right and left lungs. Conclusion This case highlights unusual source of pulmonary blood supply in Tetralogy of Fallot with pulmonary atresia. Correct pre-operative diagnosis is essential for appropriate surgical planning and better outcome. Abstract P1727 Figure. TOF-PA with CA to PA Fistula

94 When the gut affects the heart

Abstract A 59-year-old man came to our attention with progressive shortness of breath associated with lower limb oedema. He complained also of recurrent malar flushing, diarrhea and weight loss (14 kg in the last 8 months). At physical examination, a holosystolic murmur best heard over the fourth left sternal border, intensified upon inspiration, and a palpable tender liver with irregular surface were noted. Transthoracic and transesophageal echocardiogram revealed dilated right chambers. The tricuspid annulus was dilated and the leaflets thickened, shortened and retracted with restricted mobility and incomplete coaptation, leading to severe tricuspid regurgitation (Panels A-B). Moderate pulmonary regurgitation was also detected (Panel C). Cardiac MR confirmed the morphology of the tricuspid valve (Panels D-E, arrows) and the severity of the tricuspid regurgitation (regurgitant fraction 66%). The pulmonary valve leaflets were also thickened and fixed (Panel F, arrow), with moderate pulmonary regurgitation (regurgitant fraction 21%). With the suspicion of carcinoid syndrome, 5-hydroxyindoleacetic acid (5-HITT) was tested by 24-hour urine sample and a value of 929.5 µmol/24h was found (n.v. 10.5-47.1). Whole body 18-F DOPA PET was performed. Axial views and reconstructed images revealed a short segment of mural thickening (2.4 cm and 1.1 cm) in the jejunum with a moderate/high uptake (Panels G, arrow). Adjacent to this lesion, a mesenteric spiculated mass (4.0 x 2.8 cm) with moderate uptake was visible (Panels H, arrow). Multiple enhancing nodules with high uptake were present in the liver (Panel I). One of the liver nodules was biopsied. The biopsy revealed extensive infiltration by well differentiated neuroendocrine tumor (Panel J, blue arrow). The tumor cells were arranged in nests and were composed of cells with round to oval nuclei and eosinophilic cytoplasm (Panel K). The neuroendocrine nature of the tumor was confirmed by strong diffuse positivity with synaptophysin (Panel L), chromogranin and CD 56. Ki-67 showed a proliferative index of less than 3%. The tumor morphology, combined with the immunohistochemical features, was in keeping with a well differentiated neuroendocrine tumor. Carcinoid heart disease is a rare cause of primary tricuspid and pulmonary valve disease leading to significant morbidity and mortality caused by right heart failure. The characteristic pathological findings are endocardial plaques of fibrous tissue that may involve the tricuspid valve and, rarely, the pulmonary valve. The fibrous tissue in the plaques results in distortion of the valves leading to either stenosis, regurgitation, or both. Abstract 94 Figure.

88 Severe tricuspid regurgitation due to papillary muscle rupture: A rare complication of anterior myocardial infarction and ventricular septal perforation

Abstract INTRODUCTION Papillary muscle rupture (PMR) of the mitral valve is a fatal complication of acute myocardial infarction (MI). As a complication of anterior MI, PMR of the tricuspid valve is extremely rare. We experienced a case of acute anterior MI complicated with tricuspid PMR and ventricular septal perforation (VSP). REPORT An 85-year-old woman was admitted with general fatigue and appetite loss. Her consciousness was alert, but she was pale, and had cold extremities. Her blood pressure was 74/62 mmHg, and pulse was 99/min. There was no leg edema. Pan systolic regurgitant murmur (Levine III/VI) was audible at the lower left sternal border. Her oxygen saturation was 86% under room air. Electrocardiogram revealed ST segment elevation and QS pattern in V1 to V3. Transthoracic echocardiography revealed dyskinesis of the apical anterior septum, VSP with bidirectional shunt, and severe pulmonary hypertension. Left ventricular ejection fraction was preserved (58%). The anterior tricuspid leaflet was flail due to PMR, resulting in severe tricuspid regurgitation and right ventricular dilatation. Coronary angiography revealed a single vessel disease of the left anterior descending artery (LAD). We recommended surgical treatment, and transferred her to another hospital. DISCUSSION Common causes of tricuspid PMR are infective endocarditis and chest trauma. Right ventricular infarction, usually caused by right coronary artery (RCA) occlusion, may cause tricuspid PMR. In our case, however, tricuspid PMR was complicated with anterior MI. The tricuspid anterior, posterior, and septal leaflets are attached to anterolateral RV wall, inferior septum, and infundibular septum, respectively, via papillary muscles. Right ventricular branches and septal branches of the RCA usually supply these papillary muscles. Anterior leaflet PMR in this case was possibly due to anomalous blood supply of anterolateral RV wall by the right ventricular branches of the LAD. Right ventricular overload due to large VSP shunt elevated RV diastolic pressure and right atrial pressure, and might worsen ischemia of the RV wall. Abstract 88 Figure. severe TR & shunt flow through VSP

P1299 Right sided infective endocarditis with paradoxical embolization

Abstract Background Right sided infective endocarditis (IE) accounts for 5-10% of IE cases, systemic embolization is uncommon and if present it is linked to the presence of shunt or concomitant left sided IE. Clinical presentation A 35-years old gentleman with history of heroin intravenous drug abuse (IV), presented with a history of unexplained fever for two weeks along with exertional dyspnea, productive cough, chest pain and severe left hypochondria pain. On examination he had a blood pressure of 130/80 mmHg, a heart rate of 130 bpm, a temperature of 40oC, elevated jugular venous pressure and a harsh pansystolic murmur over the lower left sternal border. Laboratory results revealed anaemia, leukocytosis elevated ESR and CRP and blood cultures were positive for methicillin-resistant staphylococcus aureus (MRSA), electrocardiography showed sinus tachycardia and abdomen computed tomography scan revealed multiple splenic infarctions. Methods and results 2D&3DTrans-Thoracic Echocardiography (TTE) revealed the presence of an echogenic elongated highly mobile mass measures 2.0 cm in maximum dimension attached to the atrial surface of the anterior tricuspid valve leaflet a long with severe valvular regurgitation. Patent foramen ovale (PFO) was visualized by Color Doppler and right to left shunt was confirmed by contrast study with a complete opacification of the left side. The left ventricle dimensions were normal , there was an evidence of hyokinesis of inter-ventricular septum (IVS) and inferior wall and function was reduced, estimated LVEF = 45%. Hence, coronary angiography was done and revealed normal coronaries. 3D Trans-esophageal Echocardiography(TEE) was done for better visualization of the interatrial septum (IAS), vegetation and to rule out complications. The study confirmed the presence of PFO, there was no concomitant IAS defects, the vegetation is highly mobile and facing the IAS. Accordingly, patient was diagnosed with tricuspid infective endocarditis complicated with paradoxical embolization, anti-biotics were commenced and patient underwent successful tricuspid valve replacement and PFO closure. Discussion Tricuspid valve endocarditis has been linked to IV drug abuse and staphylococcus aureus has been recognized as the most commonly implicated organism. While systemic emboli are rare in right sided IE, our patient represent this uncommon complication. He had multiple splenic infractions and TTE contrast study showed PFO with a high degree of right to left shunt. Coronary embolization was a suspect in our patient as well given the presence of regional wall motion abnormalities involving the left ventricle inferior wall and IVS. Conclusion Echocardiography is a crucial imaging modality in patient with long standing fever and history of IV drug abuse to rule out infective endocarditis. 3D-TEE is of added value along with TTE in better definition of vegetations and detection of infective endocarditis complication. Abstract P1299 Figure. Tricuspid valve infective endocarditis

CLOT ME IF YOU CAN: A CASE OF ANTIPHOSPHOLIPID ANTIBODY SYNDROME

SESSION TITLE: Monday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Non-bacterial thrombotic endocarditis (NBTE) is a disease characterized by the presence of vegetations on cardiac valves. NBTE has increasingly been recognized as a condition associated with numerous diseases and a potentially life-threatening source of thromboembolism. Antiphospholipid antibody syndrome (APS) is a systemic autoimmune disease defined by thrombotic or obstetrical events that occur in patients with persistent antiphospholipid antibodies. We present a case of NBTE who was found to have valvular regurgitation and thrombotic vegetations, an atypical manifestation of APS. CASE PRESENTATION: A 29 y/o female presented with substernal chest pain, fatigue and shortness of breath with exertion for 2 weeks duration. Review of system was negative. Vital signs were stable. Exam revealed crackles in lung bases, diastolic murmur heard along the left sternal border. She had no significant PMH. She smokes a pack of cigarettes every day. She denied any history of illicit drug use. CXR showed increased pulmonary vascular congestion in lung bases. Labs revealed elevated BNP (>7000). Echo showed normal LVEF, severe AR and moderate MR. TEE confirmed severe AR and severe MR with mass like vegetations and perforation of the aortic and mitral valve. Blood cultures were negative. Work up for culture negative bacterial endocarditis revealed positive antiphospholipid antibody profile with elevated Anti Beta-2 glycoprotein antibody as well as anti-cardiolipin antibody and positive lupus anticoagulant. Anti-smith Ab and anti-ds DNA Ab were negative. Further history revealed patient had 1 still birth and 3 pre-term deliveries, complicated by pre-eclampsia. Based on these findings patient was diagnosed with primary antiphospholipid antibody syndrome. Patient underwent aortic and mitral valve replacements. She clinically improved and was discharged home on coumadin. DISCUSSION: APS can occur either as a primary condition or in association with a systemic autoimmune condition like SLE. Patients can present with a broad spectrum of thrombotic and non thrombotic clinical manifestations. Diagnosis should be suspected when there is occurrence of one or more otherwise unexplained venous or arterial thrombotic events, especially in young patients. Clinical manifestations are thought to be secondary to the effects of antiphospholipid antibody on the pathways of anticoagulation. Our patient presented with atypical manifestations of APS with cardiac involvement and responded well to the valve surgery and anticoagulation. CONCLUSIONS: APS is a rare condition and clinicians should have a high index of suspicion in young patients presenting with thromboembolic manifestations or with signs of NBTE to prevent further life threatening complications. Reference #1: Giannakopoulos, B., and Krilis, S. A. (2013). The pathogenesis of the anti-phospholipid syndrome. N. Engl. J. Med. 368, 1033–1044. doi:10.1056 Reference #2: Crowther MA, Ginsberg JS, Julian J, Denburg J, Hirsh J, Douketis J, Laskin C, Fortin P, Anderson D, Kearon C, Clarke A, Geerts W, Forgie M, Green D, Costantini L, Yacura W, Wilson S, Gent M, Kovacs MJ. A comparison of two intensities of warfarin for the prevention of recurrent thrombosis in patients with the antiphospholipid antibody syndrome. N Engl J Med. 2003; 349: 1133–1138. Reference #3: Arnaud L Mathian A Devilliers Het al. Patient-level analysis of five international cohorts further confirms the efficacy of aspirin for the primary prevention of thrombosis in patients with antiphospholipid antibodies. Autoimmun Rev 2014; 14: 192–200. DISCLOSURES: No relevant relationships by Deepthi Gudivada, source=Web Response No relevant relationships by Venkat Rajasurya, source=Web Response

PULMONARY HYPERTENSION IN ESSENTIAL THROMBOCYTOSIS: IS HYDROXYUREA A POTENTIAL TREATMENT? A CASE REPORT

SESSION TITLE: Monday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Essential thrombocytosis is a myeloproliferative disorder which is associated with group 5 pulmonary hypertension (PH). PH is uncommonly prevalent in patients with myeloproliferative disease. Here we present a case of essential thrombocytosis with elevated right ventricular systolic pressures that showed significant improvement with initiation of hydroxyurea. CASE PRESENTATION: 79 year old Caucasian female with past medical history of chronic obstructive pulmonary disease , paroxysmal atrial fibrillation, essential thrombocytosis who initially presented with shortness of breath. Physical examination revealed decreased bilateral air entry, with a loud pulmonary component of the second heart sound and a grade 2 systolic ejection murmur in the left lower sternal border. Laboratory studies revealed a platelet count of 1,368 x 103/cumm. Initial admission echocardiogram confirmed the physical findings revealing severe tricuspid regurgitation and severe pulmonary hypertension with right ventricular systolic pressure(RSVP) of 94 mm Hg. The interventricular septum was flattened , consistent with right heart pressure overload. A previous echocardiogram done 5 years ago demonstrated mild pulmonary hypertension with RVSP of 45 mmHg and mild tricuspid regurgitation. Computed tomography pulmonary angiogram performed during the admission did not show any significant pulmonary vascular pathology. Patient had discontinued hydroxyurea as an outpatient on her own due to a non-healing ankle ulcer. She was restarted on hydroxyurea and discharged on the same. Patient was readmitted two months later with cough and worsening expectoration, low-grade fever and was treated for an exacerbation of her underlying chronic obstructive pulmonary disease. A repeat echocardiogram performed during this admission for concerns of worsening pulmonary hypertension showed a significant improvement in pulmonary hypertension with right ventricular systolic pressure of 60 mmHg. Platelet count also improved to 389 x 103/cumm. DISCUSSION: We suspect pulmonary hypertension can have a significant impact on morbidity and mortality in patients with myeloproliferative disorder. One of the studies revealed an incidence of pulmonary hypertension in about 47.8%(1 )in patients with essential thrombocytosis. Pulmonary hypertension is often asymptomatic (1,2,3 )in these patients and treatment modalities have not been identified and established. The beneficial effect of hydroxyurea could be just due to its effect on underlying myeloproliferative disease by inactivating ribonucleotide reductase and inhibiting DNA synthesis causing cell death or via a alternate mechanism which needs to be studied at this point(4). CONCLUSIONS: The case report emphasizes the need for early evaluation of PH in myeloproliferative disorder. There is also a need for further studies regarding the use of hydroxyurea as a treatment modality for such cases. Reference #1: 1. Altintas A, Karahan Z, Pasa S, et al. Pulmonary hypertension in patients with essential thrombocythemia and reactive thrombocytosis. Leuk Lymphoma 2007;48(10):1981–1987. Reference #2: 2. Kadikoylu G, Onbasili A, Tekten T, Barutca S, Bolaman Z. Functional and morphological cardiac changes in myeloproliferative disorders (clinical study). Int J Cardiol 2004;97(2):213–220. Reference #3: 3. Dingli D, Utz JP, Krowka MJ, Oberg AL, Tefferi A. Unexplained Pulmonary Hypertension in Chronic Myeloproliferative Disorders. CHEST 2001;120(3):801–808. 4. Yarbro JW. Mechanism of action of hydroxyurea. Semin Oncol 1992 Jun;19(3 Suppl 9):1-10 DISCLOSURES: No relevant relationships by Shaylika Chauhan, source=Web Response No relevant relationships by Akhilesh Kumar Khuttan, source=Web Response No relevant relationships by Arjun Madhavan, source=Web Response No relevant relationships by Shalini Tripathi, source=Web Response

UNNATURAL HISTORY OF A RARE VASCULAR DISEASE

SESSION TITLE: Wednesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/23/2019 09:45 AM - 10:45 AM INTRODUCTION: The reported incidence of this rare genetic vascular disease is 1-2 cases per 100,000 people in North America and 1:5000 to 1:8000 around the world each year. This disease causes vascular dysplasia throughout the body, which in turn cause telangiectasias and arteriovenous malformations (AVMs). This increases the tendency for bleeding, especially in the lungs. CASE PRESENTATION: A 69-year-old female with past medical history of breast cancer status post mastectomy and iron deficiency anemia presented to the Emergency Department (ED) brought in by EMS for massive hemoptysis. The history was limited because the patient was non-arousable. The patient was intubated in the ED due to hypoxia of 50% on room air upon EMS arrival at her home. Physical exam showed tachycardia, oxygen saturation of 84% on 15L non-rebreather, 2/6 systolic murmur at left lower sternal border, and diminished breath sounds at the bases with crackles at the right base. Chest X-ray showed multiple coils in bilateral lung bases. CT chest re-demonstrated coils, areas of ground glass, residual AVMs, and fluid collection concerning for pulmonary hemorrhage. Emergent bronchoscopy was performed to identify source of blood and cause of respiratory failure. It showed active bleeding in the right middle lobe, thought to be due to ruptured AVMs. Patient was taken to Interventional Radiology (IR) for embolization of 2 intercostal/bronchial arteries that were supplying the right middle lobe. They found multiple AVMs in left base and right middle lobe with previous coils in bilateral lung bases. IR was able to coil a remaining middle lobe AVM. Medical records obtained from the Naval hospital noted a history of Osler-Weber-Rendu syndrome with multiple coils and complications, with disease also in the son and grandson. She also developed demand ischemia from pulmonary hemorrhage secondary to AVM rupture causing acute hypoxic respiratory failure secondary to Right-to-Left shunting. She was given home oxygen to maintain oxygenation greater than 86% to prevent more cardiac ischemia and altered mentation. DISCUSSION: Osler-Weber-Rendu syndrome (Hereditary Hemorrhagic Telangiectasia) is an underreported Autosomal Dominant disease. The most common presenting symptom (90%) is epistaxis. They can develop AVMs in the lungs and brain, mucocutaneous and gastrointestinal telangiectasias, and other areas as they increase in age. Pulmonary AVMs cause right-to-left shunt leading to paradoxical embolic strokes and migraines. Pulmonary hemorrhages can be life threatening if treatment is delayed or the vessels causing hemorrhage can’t be found/embolized with mortality at 10%. Life expectancy is in 50s or 60-80s. CONCLUSIONS: All AVMs in this patient’s lungs should be coiled but it was difficult due to the number of AVMs with previous coils. This patient likely had oxygen saturations in 80s but needed a higher oxygen saturation to prevent cardiac ischemia. Reference #1: Lessnau, Klaus-Deter, et al. “Osler-Weber-Rendu Disease (Hereditary Hemorrhagic Telangiectasia).” Background, Pathophysiology, Etiology, 5 July 2018, emedicine.medscape.com/article/2048472-overview. Reference #2: Shovlin, C. “Clinical manifestations and diagnosis of hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome).” 2018 March 30, www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-hereditary-hemorrhagic-telangiectasia-osler-weber-rendu-syndrome Reference #3: Shovlin, C. “Management of hereditary hemorrhagic telangiectasia.” 2018 April 03, www.uptodate.com/contents/management-of-hereditary-hemorrhagic-telangiectasia DISCLOSURES: No relevant relationships by Tracy Lee, source=Web Response

LEGIONELLA MICDADEI: A CASE OF CULTURE NEGATIVE NATIVE MITRAL VALVE ENDOCARDITIS

SESSION TITLE: Monday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Legionella is a Gram-negative bacteria that is most commonly associated with pneumonia. We describe a rare case of native valve endocarditis caused by a Legionella isolated from mitral valve tissue. CASE PRESENTATION: A 29-year-old male with a history of stroke with no residual deficits and Mitral valve (MV) endocarditis presented with slurred speech and left facial droop which lasted for 30 seconds after which it resolved completely. Prior to admission, he reported fevers, chills, and night sweats. Medical history was notable for lupus nephritis diagnosed one year ago on azathioprine and mycophenolate. He denied any intravenous drug use. A grade 2/6 holosystolic murmur was heard at the left lower sternal border to the apex. He was febrile to 101.2 F, heart rate of 114 bpm, blood pressure 106/62 mm Hg. Labs were significant for a small troponin leak. Blood cultures were sterile. CT head showed mild encephalomalacia on the left insula. Transthoracic echocardiogram showed severely dilated left atrium and new mobile echo density located laterally on the mitral annulus and transesophageal echocardiogram showed MV dehiscence. He was started on empirical coverage for culture-negative endocarditis and was transferred to surgery. He underwent Redo sternotomy and redo mitral valve replacement with a St. Jude’s mechanical prosthesis. The valve tissue appeared very friable, possibly infected and it was debrided and sent to microbiology for culture and polymerase chain reaction [PCR]. PCR was positive for Legionella micdadei. He was discharged with appropriate antibiotics. DISCUSSION: This case is another example of culture-negative legionella endocarditis diagnosed using infected valve tissue. Extrapulmonary manifestations of Legionella infections (septic arthritis, myocarditis, pericarditis, and endocarditis) often occur in immunocompromised patients and are most often treated with fluoroquinolones. Diagnostic tests for culture-negative endocarditis include special culturing techniques, molecular techniques e.g., PCR and serologic assays, and histopathology evaluation of valvular tissue when surgical excision is performed. CONCLUSIONS: Legionella species should be considered in the differential diagnosis of culture-negative endocarditis in both immunocompetent and immunocompromised patients. Reference #1: Pearce, M. M., Theodoropoulos, N., Noskin, G. A., Flaherty, J. P., Stemper, M. E., Aspeslet, T., ... Reed, K. D. (2011). Native valve endocarditis due to a novel strain of Legionella. Journal of Clinical Microbiology, 49(9), 3340-3342. Reference #2: Park D, Pugliese A, Cunha BA. Legionella micdadei prosthetic valve endocarditis. Infection. 1994;22(3):213–215. Reference #3: Chen TT, Schapiro JM, Loutit J. Prosthetic valve endocarditis due to Legionella pneumophila. J Cardiovasc Surg. 1996;37(6):631–633 DISCLOSURES: No relevant relationships by Mohammed Essa, source=Web Response No relevant relationships by Karthik Gonuguntla, source=Web Response No relevant relationships by Gaurav Manek, source=Web Response No relevant relationships by Shivaraj Patil, source=Web Response

Congenital arteriovenous fistula between descending aorta and the left innominate vein

Thoracic aortocaval fistulae are rare entities where a direct shunt between thoracic arteries and systemic veins is seen. They can be traumatic or congenital in origin. Congenital thoracic aortocaval fistulae usually involve descending aorta and azygos, hemiazygos systems. Presenting symptoms range from continuous murmur to signs of congestive heart failure. In this case report, imaging findings of a 3-year-old girl referred for continuous murmur over the left sternal border are presented. Computed tomography angiography revealed multiple tortuous vessels along the descending aorta with a course toward the left brachiocephalic vein, and was suspicious for an aorta-venous fistula. Subsequent digital subtraction angiography for treatment planning showed a fistula originating at the level of the left 6th intercostal artery, with direct drainage into the left brachiocephalic vein without involvement of the azygos/hemiazygos system.

MON-253 Severe Infantile Hypercalcemia in Williams-Beuren Syndrome

Background: Williams-Beuren Syndrome (WBS) is due to a microdeletion on chromosome 7q11, and is associated with facial dysmorphisms, supravalvular aortic stenosis, a sociable personality and infantile hypercalcemia that is clasically mild and transient. Here we describe an infant found to have incidental severe hypercalcemia that led to a diagnosis of WBS. Case: A 9 month old girl, born at 36 weeks, developed failure to thrive (FTT) thought to be due to poor appetite. She failed to gain weight despite fortification of formula to 24 kcal/oz three months ago. Her medical history included delayed motor milestones, transverse hypoplastic aortic arch, persistent pulmonary stenosis and constipation managed with prune juice. On exam, heart rate was 134 bpm, BP 100/48 (>95th%ile for height). Her weight was 5.9 kg (<1st%ile) and length 66.4 cm (3rd%ile). She was noted to have facial dysmorphisms (stellate iris, epicanthal folds, broad nasal tip, long philtrum, thick vermilion of upper lip, and micrognathia). She was able to sit briefly without support, and was generally hypotonic. She also had a grade II murmur at the left lower sternal border (LLSB). Due to persistent FTT, labs were obtained and showed serum calcium (Ca) of 17.3 mg/dL (9.0-11.0), which was repeated and was 18.6 mg/dL, prompting admission. On admission: iCa 2.60 mmol/L (1.08-1.30), Mg 2.1 mg/dL (1.6-2.2), ALP 164 (32-117 U/L), Phos 3.1 mg/dL (3.7-6.5), BUN 21 mg/dL (5-18), Cr 0.51 mg/dL(0.38-0.96), PTH 12 pg/mL (15-65), urine Ca:Cr ratio 0.56 (<0.60), 25-vitD 43 ng/mL (31-80) and 1,25-vitD 5.3 pg/mL (15-60), with abnormal values bolded. An EKG showed normal sinus rhythm and normal QTc. Renal US showed bilateral medullary nephrocalcinosis. She was given IV fluids (NS D5) at 1.5x maintenance, switched to a low-calcium diet (CalciloTM) and daily vit-D supplementation was discontinued. Despite IV hydration for 36 hours, she remained hypercalcemic (Ca 15.6 mg/dL; iCa 1.96 mmol/L) and thus received two doses of IV furosemide to aid diuresis. She was weaned off IV fluids after calcium was <13mg/dL, and iCa 1.29 mmol/L. A genetic micro-array was sent and showed the deletion of chr7.q11.23, consistent with WBS. She was discharged on a low calcium diet and calcium levels have ranged between 10.0-10.8 mg/dL. Her appetite improved, and her weight increased to the 20th%ile without any other dietary changes, by her 15 month visit. Conclusion: Hypercalcemia in WBS can be severe, with the noted calcium level being the highest reported in the literature. The etiology of hypercalcemia in WBS remains unknown and can typically be managed with a low calcium diet and minimizing vitamin D intake. In severe hypercalcemia, IV hydration is the mainstay for acute management, with furosemide, bisphosphonates and other methods reserved for resistant hypercalcemia.

LIBMAN-SACKS ENDOCARDITIS IN A SYSTEMIC LUPUS ERYTHEMATOSUS MALE PATIENT

ABSTRACTPreliminary: Libman-Sacks endocarditis (verrucous vegetations, marantic, or nonbacterial thrombotic endocarditis) is a rare cardiac manifestation in SLE. The objective was to describe Libman-Sacks endocarditis in a SLE male patient.Case: A 21-year-old male was admitted to the hospital with shortness of breath, epigastric pain, oral ulcer, discoid lesions, and knee joints pain since 3 months before. He worked as a paper mill employee and was exposed to instruments radiating UV rays for 2 years. Antibiotics initiation was given due to an infective endocarditis suspicion. Vital signs were as follows: temperature 37oC, heart rate 110beats/minute, respiratory rate 30breaths/minute, blood pressure 100/80mmHg, and SaO2 99% with nasal oxygenation. Cardiac examination showed regular rate and rhythm with diastolic murmur at left ICS III and upper left sternal border. Lower extremities showed pitting edema. Laboratory results: hemoglobin 9.6g/dl, RBC 3.6x106/μl, hematocrit 30.8%, WBC 0.88x103/μl, platelet count 22x103/μl, BUN 74.0mg/dl, serum creatinine 1.6mg/dl, ESR 24mm/h, CRP 1.2mg/dl, C3 &lt;16.4mg/dl, C4 8mg/dL, ANA test indeterminate (23.84), and anti dsDNA negative (7.4WHOunits/mL). Chest X-Ray showed mitral heart configuration with right ventricle and left atrium enlargement, pneumonia, and right pleural effusion. ECG showed normal sinus rhythm, left ventricular hypertrophy with repolarization abnormality, and prolonged QT wave. A 2-D echocardiogram showed an evidence of vegetation on aortic and pulmonary valve.Discussion: Based on the American College of Rheumatology SLE Criteria, low complement level, and evidence of vegetation on aortic and pulmonary valve, the patient was diagnosed as Libman-Sacks Endocarditis.Conclusion: The diagnosis should be confirmed by antiphospholipid antibodies examination.Key Words:

Ischemic Stroke In infant With Tetralogy of Fallot

Objectives: Although stroke is often viewed as occurring primarily in the elderly, it also strikes infants, children, young adults. Stroke in children are rare but increasingly important conditions due to the severity of their complications and diverse diagnostic differentials. It is not yet clearly understood and with multifactorial etiologies. When it comes to stroke, children are not just little adults. Stroke risk factors, symptoms, prevention efforts, and treatment are often different in children than in adults. Up to 80 % of children with ischemic stroke have cardiovascular disease. Case report: RS, An infant, aged two and half years old was admitted to DR. M. Djamil hospital Padang, with right hemiplegy and developed aphasia after activity. One year ago, he had history of cyanotic skin but neglected by his parents and never sought medical treatment. Physical findings on this patient are cyanosis and clubbing fingers. Cardiac auscultation revealed a pansystolic murmur grade III-IV best heard at the upper left sternal border. Chest radiograph showed a characteristic “bootshaped” heart, which is a reflection of Right Ventricular (RV) hypertrophy. The ECG showed right axis deviation. Brain CT Scan revealed a large hypodense lesion on the left cerebral hemisphere suggested as large brain infarction. Echocardiogram revealed tetralogy of fallot with a larged malaligned ventricular septal defect (VSD) with 60 % aortic override. Patient was managed conservatively. After two weeks of admission, patient was discharged with improvement.Conclusion : Ischemic stroke in this infant with tetralogy of fallot occurred via paradoxical embolism mechanism.

A chance encounter with a case of hypertrophic cardiomyopathy.

A chance encounter with a case of hypertrophic cardiomyopathy.

Case 1: The Impact of Methylergometrin Maleate Toxicity on Neonates

A full-term male neonate of 2.6 kg birthweight is born via a spontaneous vaginal delivery to a mother who denies any unfavorable obstetric or medical history. The delivery occurs at a peripheral hospital without any labor challenges. Approximately 20 minutes after delivery, the neonate develops central cyanosis and respiratory distress, for which he receives respiratory support in the form of oxygen through a nasal cannula. A decision is made to refer him to a tertiary NICU. On arrival at the NICU, lung auscultation reveals bilateral equal air entry, no adventitious sounds, subcostal and intercostal recessions, a respiratory rate of 70 breaths/min, and oxygen saturation above 95% in room air. Normal first and second heart sounds are heard, plus a systolic 3/6 murmur over the left upper sternal border; the heart rate is 155 beats/min, mean blood pressure is 60 mm Hg (75/50 mm Hg), and echocardiography shows an insignificant patent ductus arteriosus and small atrial septal defect secundum. The abdomen is soft and lax with no organomegaly detected. Neurologic examination shows lethargy, hypotonia, hyporeflexia of the deep tendon reflexes, and diminished primitive reflexes; the pupils are 2 mm in diameter with sluggish reaction to light bilaterally. Cranial ultrasonography depicts partially effaced ventricles as well as cerebrospinal fluid spaces denoting the possibility of …

CRYPTOCOCCAL PERICARDITIS IN THE HAART ERA

SESSION TITLE: Chest Infections 3 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Cryptococcus neoformans is an opportunistic fungal pathogen most frequently encountered in the Human immunodeficiency virus (HIV) infected host. However, due to advances in anti-retroviral therapy, HIV is no longer the most common predisposing factor for cryptococcal infections.1 Cryptococcus mainly affects the central nervous system, but cardiac manifestations like endocarditis, myocarditis, and rarely pericarditis, have been reported. CASE PRESENTATION: A 79 year old farmer presented with a non-productive cough, dyspnea and sore throat. His past medical history was significant for Crohn’s disease, hepatocellular carcinoma and cirrhosis secondary to untreated Hepatitis C and chronic kidney disease. He was initially treated with azithromycin for presumed bronchitis but subsequently admitted for progressive dyspnea. On examination, he had a pericardial rub and a holosystolic murmur over the left sternal border with basal crackles. In addition, pulsus paradoxus was appreciated with 20 mm Hg variation. EKG revealed atrial flutter with variable heart block. Laboratory workup showed pancytopenia and negative HIV antigen/antibody. The echocardiogram revealed large circumferential pericardial effusion, measuring 3.26 cm at its largest dimension with pre-tamponade physiology. Emergent pericardiocenthesis was performed with removal of one liter of fluid. Cryptococcal antigen was negative, but culture returned positive for Cryptococcus Neoformans. The patient was started on fluconazole 400 mg daily with good clinical response. A repeat echocardiogram in 2 weeks showed resolution of effusion and pre-tamponade physiology and normalization of ejection fraction. His pulmonary symptoms resolved, and he was discharged with close follow-up. DISCUSSION: Cryptococcal pericarditis in an uncommonly reported entity; prognosis and optimal treatment is therefore uncertain. Under-recognition of cryptococcal pericarditis may lead to significant morbidity and mortality, specially in those who are HIV negative. Comorbidities such as malignancy and end-stage liver disease (ESLD) should be considered important risk factors as well. In fact, Cryptococcal infections in patients with ESLD has been associated with higher mortality rates, even when compared to that of HIV-positive patients and HIV-negative patients without ESLD2. One rationale for this is liver’s role in clearing cytokines, bacteria, and endotoxins from the circulation.3 Furthermore, Cryptococcal antigen has been considered to be highly sensitive, but if negative and clinical suspicion is high, complementary cultures and histopathological examination should be pursued as well. CONCLUSIONS: Cryptococcosis is a globally distributed infection, affecting mainly immunocompromised hosts including those with ESLD, HIV and malignancy. In comparison to central nervous system, cardiovascular involvement including pericardial effusion and tamponade is rarely reported. Reference #1: Pappas PG, Perfect JR, Cloud GA, et al. Cryptococcosis in human immunodeficiency virus-negative patients in the era of effective azole therapy. Clin Infect Dis. 2001;33(5):690–9 Reference #2: Spec A, Raval K, Powderly WG. End-Stage Liver Disease Is a Strong Predictor of Early Mortality in Cryptococcosis. Open Forum Infect Dis. 2015 Dec 15;3(1):ofv197. Reference #3: Bonnel AR, Bunchorntavakul C, Reddy KR. Immune dysfunction and infections in patients with cirrhosis. Clin Gastroenterol Hepatol. 2011 Sep;9(9):727-38. DISCLOSURES: No relevant relationships by Kamran Manzoor, source=Web Response No relevant relationships by Amanda Noska, source=Web Response No relevant relationships by Paulette Pinargote Cornejo, source=Web Response No relevant relationships by Wilmer Salazar Morales, source=Web Response

Case 262.

History A 17-year-old white male adolescent was re-evaluated for a withheld cardiac condition initially detected during prenatal imaging at an outside institution. He had previously experienced intermittent episodes of shortness of breath, chest pain, and palpitations with exertion, but more recently he had been asymptomatic. He had no other comorbidities and no family history of congenital heart disease, cardiomyopathy, arrhythmia, or sudden cardiac death. At physical examination, the patient had a grade II/IV rumbling systolic murmur best heard at the left upper sternal border. Otherwise, cardiovascular and other physical examination findings were normal. An electrocardiogram showed sinus bradycardia and nonspecific T wave changes. A graded exercise stress test was normal. Cardiac MRI was performed without and with gadolinium-based contrast material ( Figs 1 - 3 ). Multiple echocardiograms obtained since birth, cardiac MR images obtained 2 and 4 years earlier ( Fig 4 ), and nongated CT images obtained with iodinated contrast material to evaluate trauma 8 years prior ( Fig 5 ) showed findings similar to those of the current examination. Prior chest radiographs (not shown) were normal, and angiography performed when the patient was 4 years old revealed normal coronary arteries. [Figure: see text][Figure: see text][Figure: see text][Figure: see text][Figure: see text][Figure: see text][Figure: see text].

Siesmocardiographic Changes with HF Status Change: Observations from a Pilot Study

Introduction Many strategies and technologies have been developed to minimize readmissions and manage HF. These strategies are often either labor intensive, invasive and/or expensive. New methodologies for accurate, rapid and low-cost ambulatory metrics of HF status are needed, of which seismocardiography (SCG) may be particularly useful. SCG uses accelerometer sensors to detect both audible, and sub-audible (i.e., below the frequencies detectable by the human ear) vibration signals. Although described decades ago, new sensor and digital processing capabilities are expanding the possible utility of this approach. Objective To investigate the use of SCG for HF monitoring. Methods Twenty patients (ages 51 to 75, 30 % female) admitted for HF were studied with a contact SCG sensor on the left lower sternal border of the chest wall at the 4thintercostal space for 1 min at two points in time with an interval of 2 to 48 hrs between assessments. The collected data contained 34 SCG waveform pairs: 17 pairs for patients with a NYHA class change from IV to III and 17 pairs for patients who stayed in class III. Results SCG signal waveform time-amplitude features were analyzed and the waveform variation quantified between the recording pairs. Results showed that the waveform variation was significantly different for those w/ and w/o NYHA class change (p Conclusions SCG changes may be a useful, safe and inexpensive modality for outpatient HF monitoring, enabling more timely intervention. These preliminary findings are supportive of an ability to differentiate HF functional status change. Further prospective studies are warranted.