UNMASKING THE SILENT THREAT: A CASE OF HYPERTROPHIC CARDIOMYOPATHY ELUDING ECHOCARDIOGRAPHIC DETECTION Prajwal Kumsi Sreedhar, Shriya Vudari, Jagan Beedupalli, Kathryn Gayle; Willis-Knighton Health, Shreveport, LA.
Introduction: Hypertrophic cardiomyopathy (HCM) is an autosomal dominant disorder encompassing several patterns of left ventricular hypertrophy (LVH), such as asymmetric septal (“classic” HCM), concentric, reverse septal, neutral, and apical (ApHCM). ApHCM is a rare subtype of HCM with reported incidences ranging from 1-2% in Western countries and is characterized by localized hypertrophy of apical segments of the left ventricle. ApHCM has less frequent sarcomere mutations, and fewer patients have a positive family history compared to classic HCM. Recent data suggest annual cardiac death rates approaching those of classic HCM, at 0.5-4%. Case: A 62-year-old male patient with no past medical or family history presented after a syncopal episode. Over the past year, he had recurrent episodes of syncope and non-specific chest pain prompting multiple heart catheterizations that were unrevealing. The electrocardiogram (ECG) on presentation revealed sinus bradycardia, voltage criteria for left ventricular hypertrophy (LVH), and curved ST- segment elevation with deep T-wave inversions in the precordial leads concerning for acute myocardial
ischemia but was unchanged from his prior ECGs. Echocardiography showed moderate concentric LVH with an ejection fraction of 60-65%. Since he had unexplained syncope associated with ECG changes and unexplained LVH on echo it was decided to pursue cardiac MRI (CMR). Subsequent CMR revealed ApHCM with a spade-shaped left ventricle cavity and apical aneurysm, which was initially missed on echocardiography. With the patient being symptomatic and at high risk for sudden cardiac death (SCD), he underwent implantable cardioverter defibrillator placement for primary prevention. Conclusion: ApHCM poses a specific diagnostic challenge with echocardiography missing up to 40% of the cases with apical hypertrophy. CMR can identify 25 to 43% of those missed by echocardiography and is especially more sensitive for detecting early ApHCM phenotypes. Currently, no ApHCM-specific recommendations to guide the diagnosis exist. This case outlines the understanding and facilitates the recognition of ApHCM while also highlighting the knowledge gaps.
UNRAVELLING DIFFERENTIATION SYNDROME Tanmayi Gadre, John Godke; Baton Rouge General Hospital, Baton Rouge, LA.
Introduction: Differentiation syndrome (DS) is a rare complication of ATRA (all-trans retinoic acid) therapy employed in patients with acute promyelocytic leukemia. It is characterized by an increasing white blood cell count, fever, dyspnea, pulmonary infiltrates and is associated with severe complications and mortality if not treated properly. Early detection, prophylactic initiation of corticosteroids or crucial aspects to prevent this syndrome. It is one of the elusive entities that can often be overlooked, and its pathophysiology remains largely unknown. Case: A 52-year-old male presents with generalized weakness and worsening shortness of breath. He is hypotensive, hypoxic with a significant leukocytosis of 261cells/mm3 with 82% blasts and is admitted for suspected acute myeloid leukemia (AML)/ chronic
lymphocytic leukemia (CLL). Upon the oncologist’s review of the peripheral smear, he is diagnosed with suspected acute promyelocytic leukemia (APML) with blast cell crisis and promptly started on all-trans retinoic acid (ATRA) as well as dexamethasone to prevent differentiation syndrome. In a race against time to provide supportive care for tumor lysis syndrome, disseminated invtravascular coagulation as well as multi-organ dysfunction, he continues to deteriorate. The patient is now febrile with a worsening white count and worsening respiratory status eventually requiring intubation. He then goes on to develop refractory shock and acute respiratory distress syndrome and is started on steroids, titrated to four pressors along with a paralytic agent and epoprostenol. Once induction was achieved with ATRA, the plan was to start chemotherapy however, 57
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