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Cardiovascular Disease |

Acute Heart Failure in a Young Female: A Case of Noncompaction

Weyman Lam, MD; Gbolahan Ogunbayo, MD; David Lobo, MD
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Rochester General Hospital, Rochester, NY


Chest. 2015;148(4_MeetingAbstracts):72A. doi:10.1378/chest.2269978
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Abstract

SESSION TITLE: Cardiovascular Disease Student/Resident Case Report Posters

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Acute onset congestive heart failure is a rare event in a young patient with minimal cardiac risk factors. We present an uncommon cause of heart failure in an otherwise healthy young female.

CASE PRESENTATION: A 27 year-old Asian American female with a history of hypertension presented with generalized edema that began a week prior to admission. She reported worsening dyspnea on exertion, but denied chest pain, palpitations, light-headedness or dizziness. She was adopted and did not know her biological parents’ medical history. Physical examination revealed a blood pressure of 155/120 mmHg, jugular venous distention, rhonchi in right middle and lower lung field and evidence of ascites. Her lower extremities were notable for significant edema with serous weeping. Laboratory findings were significant for BNP of 4128, and troponin of 0.24. A transthoracic echocardiogram showed an ejection fraction of less than 20%. There was also evidence of right-sided heart failure, severe tricuspid regurgitation, an apical thrombus in the left ventricle, and anatomy suggestive of non-compaction syndrome. A cardiac MRI was obtained which showed pericardial effusion with increased trabeculae in the lateral wall, which are also consistent with mild non-compaction. She was diuresed and started on anticoagulation. A loculated right pleural effusion, requiring video-assisted thoracotomy and decortication, complicated her hospital course. She had an uncomplicated recovery and was discharged twelve days postoperatively.

DISCUSSION: Left ventricular noncompaction is a rare condition with an incidence of less than 0.3%. It can be genetically sporadic or familial. There is an arrest of the normal compaction process in the myocardium during embryogenesis. It is characterized by abnormal two-layered myocardium (compacted and non compacted) and thickened myocardial wall with prominent trabeculae. Although this condition may be asymptomatic, it can present as heart failure, fatal arrhythmias, thromboembolic events, or even sudden cardiac death. Between studies, prognosis is variable with mortality at follow-up from 2% to 47%.

CONCLUSIONS: Left ventricular noncompaction should be considered in the differential diagnosis in young patients without cardiac risk factors with decompensated congestive heart failure. Making the correct diagnosis is important for the long-term management of patients, as well as screening of their relatives when necessary.

Reference #1: Stanton C, Bruce C, Connolly H MD, Brady P, Syed I, Hodge D, Asirvatham S, Friedman P. Isolated Left Ventricular Noncompaction Syndrome. Am J Cardiol. 2009;104:1135-1138).

Reference #2: Oechslin EN, Attenhofer Jost CH, Rojas JR, Kaufmann PA, Jenni R. Long-Term Follow-up of 34 Adults With Isolated Left Ventricular Noncompaction: A Distinct Cardiomyopathy With Poor Prognosis. JACC. 2000. 36(2):493-500.

DISCLOSURE: The following authors have nothing to disclose: Weyman Lam, Gbolahan Ogunbayo, David Lobo

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