Left ventricular non-compaction with congenital diaphragmatic hernia causing cardiac dextroposition
A 45-year-old man with a 2-month history of breathlessness, fatigue and irregular heart rate was referred to our centre after first-ever cardiopulmonary decompensation. At presentation he was stable with heart rate of 120/min, blood pressure of 140/90 mmHg, in NYHA class II. 12-lead ECG showed atrial fi brillation, low R-waves voltage and deep S-waves with non specific ST changes in V1-V6. A posterior-anterior chest X-ray showed an enlarged cardiac silhouette, whereas on lateral picture an unusual structure was identified in the thorax cavity. An echocardiogram showed an enlarged, hypertrophied left ventricle (LV) with severe global systolic impairment. A computed tomography revealed an intestinal loop, characterized as the anterior Morgagni type congenital diaphragm hernia, in the anterior mediastinum resulting in the heart dislocation to the right side. Cardiac magnetic resonance revealed a thinned apex coupled with increased trabeculation at the apex, posterior, lateral and anterior wall. The measurements of double-layered LV wall, confi rmed the diagnosis of LV non-compaction (LVNC). This case is an illustrate of a constellation of rare anomalies of LVNC, persistent left superior vena cava with a congenital anterior Morgagni-type diaphragm hernia, causing heart dextroposition. On multidisciplinary team meeting the general decision of conservative treatment was made with typical systolic heart failure pharmacotherapy, restoration of sinus rhythm, considering referral to ICD and/or CRT-D, and regular clinical and echocardiographic follow-up of both patient and patient’s pedigree. JRCD 2012; 1: 18–23
Key words: Cardiac malposition; Dextroposition; Diaphragmatic hernia; Left ventricular non-compaction
Maron BJ, Towbin JA, Thiene G, et al. Contemporary defintions and classsification of the cardiomyopathies. Circulation 2006; 113: 1807–1816.
Murphy RT, Thaman R, Blanes JG, et al. Natural history and familial characteristics of isolated left ventricular non-compaction. Eur Heart J 2005; 26: 187–192.
Elliot P, Andersson B, Arbustini E, et al. Classification of the cardiomyopathies: a position statement from the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J 2007; 29: 270–277.
Tsai SF, Ebenroth ES, Hurwitz RA, et al. Is left ventricular non-compaction in children truly an isolated lesion? Pediatr Cardiol 2009; 30: 597–602.
Stollberger C, Finsterer J, Blazek G. Left ventricular hypertrabeculation/non-compaction and association with additional cardiac abnormalities and neuromuscular disorders. Am J Cardiol 2002; 90: 899–902.
Oechslin EN, Attenhofer-Jost CH, Rojas JR, et al. Long-term follow-up of 34 adults with isolated left ventricular non-compaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol 2000; 36: 493–500.
Kovacevic - Preradovic T, Jenni R, Oechslin EN, et al. Isolated left ventricular non-compaction as a cause of heart failure and heart transplantation: a single centre experience. Cardiology 2009; 112: 158–164.
Xing Y, Ichida F, Matsuoka T, et al. Genetic analysis in patients with left ventricular non - compaction and evidence for genetic hetrogeneity. Mol Genet Metab 2006; 88: 71–77.
Charron P, Arad M, Arbustini E, et al. Genetic counselling and testing in cardiomyopathies: a position statemnt of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J 2010; 31: 2715–2726.
Hershberger RE, Lindenfeld J, Mestroni L, et al. Genetic evaluation of cardiomyopathy – a Heart Failure Society of America practice guidelines. J Cardiac Fail 2009; 15: 83–97.
Ritter M, Oechslin E, Sutych G, et al. Isolated non-compaction of the myocardium in adults. Mayo Clin Proc 1997; 72: 26–31.
Jenni R, Oechslin E, Schneider J, et al. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart 2001; 86: 666–671.
Chin TK, Perloff JK, Williams RG, et al. Isolated non-compaction of left ventricular myocardium. A study of eight cases. Circulation 1990; 82: 507–513.
Thuny F, Jacquier A, Jop B, et al. Assessment of left ventricular non-compaction in adults: side-by-side comparison of cardiac magnetic resonance imaging with echocardiography. Arch Cardiovasc Dis 2010; 103: 150–159.
Kohli SK, Pantazis AA, Shah JS, et al. Diagnosis of left ventricular non-compaction in patients with left ventricular systolic dysfunction: time of a reappraisal of diagnostic criteria? Eur Hear J 2008; 29: 89–95.
Petersem SE, Selvanayagam JB, Wiesmann F, et al. Left ventricular non-compaction: insights from cardiovascular magnetic resonance imaging. J Am Coll Cardiol 2005; 46: 101–105.
Jacquier A, Thuny F, Jop B, et al. Measuremt of trabeculated left ventricular mass using cardiac magentic resonance imaging in the diagnosis of left ventricular non-compaction. Eur Heart J 2010; 31: 1098–1104.
van Dalen BM, Caliskan K, Soliman Ol, et al. Diagnostic value of rigid body rotation in noncompaction cardiomyopathy. J Am Soc Echocardiogr 2011; 24: 548–555.
Task Force for Diagnosis and Treatment of Acute and Chronic Heart Failure 2008 of European Society of Cardiology, Dickstein K, Cohen-Solal A, Filippatos G, et al. The European Society of Cardiology Guidelines for the diagnosis and treatment of acute and chronic heart failure 2008. Developed in Collaboration with the Heart Failure Association of the ESC (HFA) and endorsed by the European Society if Intensive Care Medicine (ESICM). Eur Heart J 2008; 29: 2388–2442.
Jacobs JP, Anderson RH, Weinberg PM, et al. The nomenclature, definition and classification of cardiac structures in the setting of hetrotaxy. Cardiol Young 2007; 17 (Suppl. 2): 1–28.
Maldjian PD, Saric M. Approach to dextrocardia in adults: review. AJR 2007; 188: 39 – 49.
Evans WN, Aucherman RJ, Collazos JC, et al. Dextrocardia: practical points and comments on terminology. Pediatr Cardiol 2010; 31: 1–6.
Gedik E, Tuncer MC, Onat S, et al. A review of Morgagni and Bochdalek hernias in adults. Folia Morphol 2011; 70: 5–12.
Friedman MA, Wiseman S, Haramati L, et al. Non-compaction of the left ventricle in a patient with dextroversion. Eur J Echocardiogr 2007; 8: 70–73.
Grattan MJ, Buffo - Sequeira I, Fortier M, et al. Biventricular non - compaction in a patient with dextrocardia/dextroversion diagnosed with cardiac magnetic resonance imaging. Can J Cardiol 2009; 25: 335–336.
Baskurt M, Kalyoncuoglu M, Kucukoglu S. Noncompaction cardiomyopathy and dextroversion of the heart. Herz 2010; 36
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