Severe Heart Failure Due to Noncompaction Cardiomyopathy: A Case Report E.M. Cantero-Pérez, J.M. Sobrino-Márquez, A. Adsuar-Gómez, E. Lage-Gallé, A. Grande-Trillo, and A. Martínez-Martínez ABSTRACT Noncompaction cardiomyopathy involves an infrequent pathology whose diagnosis has been rising in recent years owing to a better understanding of the disease and, therefore, better diagnosis. Today, there are no well established protocols for its treatment. For this reason, it is necessary to adapt the therapy of choice to each patient. We report the case of a 35-year-old man with no past medical history of interest who was admitted due to acute pulmonary edema. Diagnostic tests revealed noncompaction cardiomyopathy with biventricular severe systolic dysfunction. In this situation, heart transplantation was considered to be the best therapeutic option, which was carried out with an uneventful postoperative course. This is one of the few cases reported in the literature for this issue.

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ONCOMPACTION CARDIOMYOPATHY is a rare congenital heart disease. During the past decades, an increase in its prevalence has been reported, probably owing to underdiagnosis in the past.1 It is rarely diagnosed during adulthood and it has a wide spectrum of clinical features. Patients can remain asymptomatic or develop progressive heart failure. In advanced stages, the treatment is based on control of symptomatic complications (heart failure, embolic events, and/or arrhythmias), not differing from that performed in other processes.2e9 A poor prognosis has been described in this situation.6e9 Several therapies have been used in these patients, such as cardioverter defibrillator6,8,9 and even cardiac transplantation.6,7 However, we still do not know what the best treatment is according to clinical evidence.2,4 There are only 2 cases in the literature that have shown an acutepresentation noncompaction cardiomyopathy requiring heart transplantation.6,7

dilated left ventricle with an ejection fraction of 20%. After stabilization, he was referred to our hospital for a full pretransplantation study. New morphologic studies (transthoracic echocardiography, nuclear magnetic resonance) showed deep trabeculations in the ventricular wall, mainly in the apex and lateroapical wall (Fig 1). In systole, the noncompacted-compacted ratio was 2:1, and in diastole the ratio noncompacted/(compacted þ noncompacted) was found to be >0.5. The right ventricle was dilated and dysfunctional (tricuspid annular plane systolic excursion 13 mm). Ventricular filling was restrictive. Both atrioventricular valves showed grade II/IV regurgitation, and the PA pressure was estimated to be 50 mm Hg. Continuous monitoring during admission recorded an episode of nonsustained ventricular tachycardia and frequent ventricular extrasystoles. Because the patient had poor short-term prognostic data and because of the limited clinical evidence of the best therapeutic management, we decided to include the patient on the waiting list for a heart transplantation, which took place 3 weeks later.

CASE REPORT We report the case of a 35-year-old man with congenital sensorineural hearing loss as the only personal history of interest. Three relatives also had hearing loss and 1 sister had undergone surgery for an atrial septal defect closure. Being previously asymptomatic, he was admitted to the Intensive care unit because of 1-week progressive dyspnea leading to pulmonary edema, requiring noninvasive mechanical ventilation and inotropic support. The echocardiogram showed a severely

From the Department of Cardiology (E.M.C.-P., J.M.S.-M., E.L.-G., A.G.-T., A.M.-M.) and Department of Cardiac Surgery (A.A.G.), Heart Area, Clinical Management Unit, Virgen del Rocío University Hospital, Seville, Spain. Address reprint requests to Eva María Cantero Pérez, Heart Area, Clinical Management Unit, Virgen del Rocío University Hospital, Manuel Siurot Street, S/N 41012, Seville, Spain. E-mail: [email protected]

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SEVERE HEART FAILURE

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Fig 1. Magnetic resonance imaging: sagittal view showing compacted and noncompacted myocardial ventricular wall.

Fig 2. Explanted heart: hypertrabeculation of the left ventricular lateral wall.

Anatomic-pathologic studies confirmed the diagnosis (Fig 2). The transplantation was uneventful, and the patient was discharged on postoperative day 10. At the time of writing, the patient had remained asymptomatic.

are needed to determine the prognosis and establish clinical guidelines.

REFERENCES DISCUSSION

Noncompaction cardiomyopathy is a cardiac pathology that was first described in 1984.10 Diagnostic criteria are based on echocardiographic studies. Its clinical presentation is very heterogeneous, and the prognosis depends on the presence and degree of any of its 3 main clinical manifestations (heart failure, tachyarrhythmia, and/or thromboembolic events).2e4,6,7 Owing to this wide variability, it is difficult to establish rigid practice guidelines. In this context, the best therapeutic option is selected according to general clinical practice recommendations. Ventricular assist devices and heart transplantations are indicated for patients with heart failure and symptoms refractory to conventional therapies.11 Therefore, although their use is not well defined, we must keep them in mind in any patient with noncompaction cardiomyopathy with no response to other therapies. Nevertheless, further studies

1. Wald R, Gruschen V, Golding F, Kirsh J, McCrindle B, Benson L. Determinants of outcome in isolated ventricular noncompaction in childhood. Am J Cardiol. 2004;94: 1581e1584. 2. Vieira L, Cury VM, Machado L, Mady C. Noncompaction cardiomyopathy: a current view. Arq Bras Cardiol. 2011;97(1): e13ee19. 3. Sarma RJ, Chana A, Elkayam U. Left ventricular noncompaction. Prog Cardiovasc Dis. 2010;52:264e273. 4. Captur G, Nihoyannpoulos P. Left vantricular noncompactation: Genetic heterogeneity, diagnosis and clinical course. Int J Cardiol. 2010;140:145e153. 5. Van der Starre P, Deuse T, Pritts C, et al. Late profound muscle weakness following heart transplantation due to Danon disease. Muscle Nerve. 2013;47(1):135e137. 6. Amuchastegui M, Amuchastegui T, Contreras AE, et al. Isolated left ventricular noncompaction: a review of three different presentations for a rare disease. Conn Med. 2013;77(1):11e18.  7. Glaveckaite_ S, Rucinskas K, Celutkien e_ J, et al. Heart transplantation in an adult patient with isolated noncompaction of the left ventricular myocardium. Medicina (Kaunas). 2010;46(3): 193e199.

3674 8. Ozben B, Mutlu B, Etdogan O. ICD implantation in left ventricular noncompaction: a case report and review of the literature. Cardiol J. 2011;18(6):691e694. 9. Kobza R, Jenni R, Erne P, et al. Implantable cardioverterdefibrillators in patients with left ventricular noncompaction. Pacing Clin Electrophysiol. 2008;31(4):461e467.

CANTERO-PÉREZ, SOBRINO-MÁRQUEZ, ADSUAR-GÓMEZ ET AL 10. Engberding R, Bender F. Identification of a rare congenital anomaly of the myocardium by two-dimensional echocardiography: persistence of isolated myocardial sinusoids. Am J Cardiol. 1984;53: 1733e1734. 11. Jessup M, Brozena S. Heart failure. N Engl J Med. 2003;348(20):2007e2018.

Severe heart failure due to noncompaction cardiomyopathy: a case report.

Noncompaction cardiomyopathy involves an infrequent pathology whose diagnosis has been rising in recent years owing to a better understanding of the d...
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