Cardiovascular Disease |

Noncompaction Cardiomyopathy and Idiopathic Retroperitoneal Fibrosis: A Rare Occurrence FREE TO VIEW

Prithvi Kukkadapu, MBBS; Mangalore Amith Shenoy, MBBS; Joy Victor, MD; Gerald Hollander, MD; Jacob Shani, MD
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Maimonides Medical Center, Brooklyn, NY

Chest. 2014;146(4_MeetingAbstracts):110A. doi:10.1378/chest.1990136
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SESSION TITLE: Cardiovascular Student/Resident Case Report Posters I

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Non-compaction of the left ventricle (LVNC) is a rare disorder, classified as primary genetic cardiomyopathy. LVNC was previously named spongy myocardium or hyper-trabeculation syndrome1. A review from Switzerland identified 34 cases within 15 years, which represented 0.014% of echocardiograms that were performed2. Retroperitoneal fibrosis is a rare entity characterized by inflammation and fibrosis that affects retroperitoneal structures3. We report a case of refractory congestive heart failure in a patient with LVNC complicated by progressive renal failure due to retroperitoneal fibrosis.

CASE PRESENTATION: 48 year old African American male with heart failure with AICD and chronic kidney disease secondary to retroperitoneal fibrosis presented to the hospital with complaints of progressive dyspnea, fatigue and leg swelling. On physical exam elevated jugular venous pressure, bibasilar crackles and pitting edema were appreciated. Echocardiogram showed prominent trabeculations in the apex, consistent with LVNC with severe LV systolic dysfunction, EF 10-15% and moderately reduced RV systolic function.The patient was admitted to the cardiac ICU and started on diuretics, beta-blockers, also requiring intravenous inotropes. The hospital course was complicated by worsening renal failure requiring intermittent hemodialysis and an IVC thrombus.

DISCUSSION: LVNC is a rare congenital cardiomyopathy that appears to result from intrauterine arrest of endomyocardial morphogenesis1. Two forms have been described: an isolated form and a non isolated form associated with other congenital cardiac defects. The classic clinical manifestations include heart failure, arrhythmias and thromboembolic events. Treatment includes following the standard guidelines for heart failure, AICD for primary prevention of sudden cardiac death and eventual heart transplant. Retro-peritoneal fibrosis ( RF) is a progressive disorder,often presenting with systemic hypertension, left ventricular hypertrophy and deep venous thrombosis3. This patient’s management of LVNC was complicated by the presence of ESRD due to progressive RF.

CONCLUSIONS: Left ventricular non-compaction (LVNC) is a rare sporadic or familial cardiomyopathy. The clinical presentations and prognosis of such patients are variable; including heart failure, chest pain, thromboembolic events and atrial and ventricular arrhythmias. LVNC patients who have end-stage heart failure are candidates for cardiac transplantation evaluation.

Reference #1: Almeida, Ana G., and Fausto J. Pinto. "Non-compaction cardiomyopathy."Heart 99.20 (2013): 1535-1542.

Reference #2: Oechslin, Erwin N., et al. "Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis."Journal of the American College of Cardiology 36.2 (2000): 493-500.

Reference #3: Vaglio A, et al. “Retroperitoneal fibrosis” Lancet 2006; 367: 241-51

DISCLOSURE: The following authors have nothing to disclose: Prithvi Kukkadapu, Mangalore Amith Shenoy, Joy Victor, Gerald Hollander, Jacob Shani

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