Images in cardiovascular medicine
Left ventricular noncompaction presenting like a doublechambered left ventricle Giuseppina Novoa, Gregory Dendramisa, Gianluca Marroneb, Salvatore Novoa and Gaetano Thienec J Cardiovasc Med 2015, 16:522–524 a Chair and Division of Cardiology, University of Palermo, bDiagnostic and Interventional Radiology, Mediterranean Institute of Transplantation and High Specialization Therapy (ISMETT), Palermo and cDepartment of Medical Diagnostic Sciences and Special Therapies, University of Padua Medical School, Padua, Italy
Correspondence to Giuseppina Novo, MD, PhD, U.O.C. di Cardiologia, A.O.U.P. ‘P. Giaccone’, Via Del Vespro, 127, CAP 90127, Palermo, Italy Tel: +0916554303; fax: +0916554304; e-mail: [email protected] Received 13 September 2014 Revised 10 November 2014 Accepted 7 December 2014
Left ventricular noncompaction (LVNC) is a rare cardiomyopathy with a variable prognosis. The patient may develop severe heart failure requiring heart transplant, life-threatening arrhythmias and cardiac thromboembolism. However, according to more recent evidence the
prognosis is actually considered less severe than before, and the main adverse prognostic determinants are poor left ventricular ejection fraction and a diagnosis occurring for symptoms in young age.1 When detected in an asymptomatic adult and not associated with other cardiovascular abnormalities, the disorder is generally believed to be at little risk. However, today data regarding left ventricular accessory chamber are few in the literature and prognosis is not well defined.2 A 24-year-old woman was referred to our echocardiography laboratory for the detection of a cardiac murmur. The patient reported a state of well-being and her previous clinical history was negative. Transthoracic echocardiogram showed mildly dilated and hypertrabeculated left
Fig. 1
Panel a. Transthoracic 2D echocardiogram: short axis view at basal level showing the outpouching of the interventricular septum. Panel b. Transthoracic 2D echocardiogram: apical four-chamber view showing the prominent trabecula, which courses parallel to the interventricular septum and subdivides the left ventricle into main and accessory chambers, communicating each other at basal level. Panel c, d. Cardiac MRI (Cine-FIESTA sequences, white blood): image in short axis view with sections at middle and apical levels. The asterisks show the accessory ventricular chamber.
1558-2027 Copyright ß 2015 Wolters Kluwer Health, Inc. All rights reserved.
DOI:10.2459/JCM.0000000000000264
Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
Left ventricular noncompaction presenting like a double-chambered left ventricle Giuseppina et al. 523
Fig. 2
Panels a–d. Cardiac MRI (T2-weighted triple-inversion black blood, FAT-SAT): images in short axis view with sections at different levels base-apex showing the presence of hypertrabeculated LV and of the accessory ventricular chamber (see asterisks). Fig. 3
Cardiac MRI: long axis (panels a and b) and short axis (panels c and d) steady-state free precession images through the apical portion of the left ventricle show extensive trabecluations. The ratio of trabeculated myocardium to compact myocardium by far exceeds the threshold of 2.3.
Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
524 Journal of Cardiovascular Medicine 2015, Vol 16 No 7
ventricle (LV) with preserved systolic function. A very prominent trabecula, originating from the inferior apex and coursing parallel to the interventricular septum, subdivided the LV into a main and an accessory chamber, communicating with each other at basal level (Fig. 1a and 1b). The patient underwent cardiac magnetic resonance (CMR) that confirmed the presence in the LV of deep recesses, that communicated with the left ventricular cavity. The recesses were separated by myocardial trabeculae with the same signal intensity of the remaining myocardium. The short axis section showed the presence of hypertrabeculated LV, with a ratio between spongy and compacted layers that met the diagnostic criteria of LVNC (>2.3)3 in which a giant trabecula delineated an accessory ventricular chamber (Figs 1C, D; 2 and 3). Neither defects of myocardial perfusion nor areas of delayed enhancement and congenital midline defects were detected. Other diagnoses that should be taken into consideration are aneurysms, pseudoaneurysms, diverticula of the LV, or isolated double-chambered left ventricle (DCLV). Distinguishing among these conditions is challenging but of great clinical importance given the consequent different treatment modalities and prognosis. Double-chambered ventricle is a rare congenital cardiac anomaly, characterized by the division of the ventricular chamber into two chambers by normal muscular tissue and may be associated with congenital midline defects. This diagnosis was excluded because the muscular tissue separating the two chambers presented a two-layered structure; moreover, double-chambered right ventricle is more common than DCLV.4 Furthermore, a precise evaluation of wall motion and of the width of the two chambers communication was helpful to exclude the diagnosis of diverticulum, aneurysms, and pseudoaneurysms. The
diagnosis of LV diverticulum was excluded because this communicates with the main chamber through a narrow neck, not noticeable in our case. The diagnosis of left ventricular aneurysm was also excluded because this entity lacks the complete layering of the ventricular wall and has dyskinetic motion, expanding during systole; moreover in LV aneurysm the presence of delayed enhancement due to fibrosis is detectable at CMR. These characteristics also apply to pseudoaneurysm, which is a contained rupture of LV free wall and is devoid of any component of the cardiac wall.5 On the basis of the presence of an altered ratio between noncompacted and compacted layers (> 2.3), the presence of all the ventricular wall layers and normal systodiastolic movements of the accessory chamber, the findings reported were considered consistent with the diagnosis of LVNC in which a giant trabecula, parallel to the interventricular septum, subdivided the LV, thus simulating an accessory ventricular chamber. This association suggests that LVNC during embryonic development may result in subdivided LV. The patient is currently in good health and she is followed-up in our institution without any therapy.
References 1
2
3
4 5
Fazio G, D’angelo L, Visconti C, et al. Isolated left ventricular noncompaction: a larger part of submerged iceberg with criteria for diagnosis to the limits of standard. Int J Cardiol 2010; 145:e1–e2. Correia AS, Madureira AJ, Gonc¸alves A, Almeida PB, Arau´jo V. Doublechambered left ventricle plus left ventricular noncompaction: report of an abnormal association. Eur Heart J Cardiovasc Imaging 2013; 14:127. Petersen SE, Selvanayagam JB, Wiesmann F, et al. Left ventricular noncompaction: insights from cardiovascular magnetic resonance imaging. J Am Coll Cardiol 2005; 46:101–105. Harikrishnan S, Sivasankaran S, Tharakan J. Double chambered left ventricle. Int J Cardiol 2002; 82:59–61. Kilner PJ, Geva T, Kaemmerer H, Trindade PT, Schwitter J, Webb GD. Recommendations for cardiovascular magnetic resonance in adults with congenital heart disease from the respective working groups of the European Society of Cardiology. Eur Heart J 2010; 31:794–805.
Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
Left ventricular noncompaction presenting like a doublechambered left ventricle Giuseppina Novoa, Gregory Dendramisa, Gianluca Marroneb, Salvatore Novoa and Gaetano Thienec J Cardiovasc Med 2015, 16:522–524 a Chair and Division of Cardiology, University of Palermo, bDiagnostic and Interventional Radiology, Mediterranean Institute of Transplantation and High Specialization Therapy (ISMETT), Palermo and cDepartment of Medical Diagnostic Sciences and Special Therapies, University of Padua Medical School, Padua, Italy
Correspondence to Giuseppina Novo, MD, PhD, U.O.C. di Cardiologia, A.O.U.P. ‘P. Giaccone’, Via Del Vespro, 127, CAP 90127, Palermo, Italy Tel: +0916554303; fax: +0916554304; e-mail: [email protected] Received 13 September 2014 Revised 10 November 2014 Accepted 7 December 2014
Left ventricular noncompaction (LVNC) is a rare cardiomyopathy with a variable prognosis. The patient may develop severe heart failure requiring heart transplant, life-threatening arrhythmias and cardiac thromboembolism. However, according to more recent evidence the
prognosis is actually considered less severe than before, and the main adverse prognostic determinants are poor left ventricular ejection fraction and a diagnosis occurring for symptoms in young age.1 When detected in an asymptomatic adult and not associated with other cardiovascular abnormalities, the disorder is generally believed to be at little risk. However, today data regarding left ventricular accessory chamber are few in the literature and prognosis is not well defined.2 A 24-year-old woman was referred to our echocardiography laboratory for the detection of a cardiac murmur. The patient reported a state of well-being and her previous clinical history was negative. Transthoracic echocardiogram showed mildly dilated and hypertrabeculated left
Fig. 1
Panel a. Transthoracic 2D echocardiogram: short axis view at basal level showing the outpouching of the interventricular septum. Panel b. Transthoracic 2D echocardiogram: apical four-chamber view showing the prominent trabecula, which courses parallel to the interventricular septum and subdivides the left ventricle into main and accessory chambers, communicating each other at basal level. Panel c, d. Cardiac MRI (Cine-FIESTA sequences, white blood): image in short axis view with sections at middle and apical levels. The asterisks show the accessory ventricular chamber.
1558-2027 Copyright ß 2015 Wolters Kluwer Health, Inc. All rights reserved.
DOI:10.2459/JCM.0000000000000264
Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
Left ventricular noncompaction presenting like a double-chambered left ventricle Giuseppina et al. 523
Fig. 2
Panels a–d. Cardiac MRI (T2-weighted triple-inversion black blood, FAT-SAT): images in short axis view with sections at different levels base-apex showing the presence of hypertrabeculated LV and of the accessory ventricular chamber (see asterisks). Fig. 3
Cardiac MRI: long axis (panels a and b) and short axis (panels c and d) steady-state free precession images through the apical portion of the left ventricle show extensive trabecluations. The ratio of trabeculated myocardium to compact myocardium by far exceeds the threshold of 2.3.
Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
524 Journal of Cardiovascular Medicine 2015, Vol 16 No 7
ventricle (LV) with preserved systolic function. A very prominent trabecula, originating from the inferior apex and coursing parallel to the interventricular septum, subdivided the LV into a main and an accessory chamber, communicating with each other at basal level (Fig. 1a and 1b). The patient underwent cardiac magnetic resonance (CMR) that confirmed the presence in the LV of deep recesses, that communicated with the left ventricular cavity. The recesses were separated by myocardial trabeculae with the same signal intensity of the remaining myocardium. The short axis section showed the presence of hypertrabeculated LV, with a ratio between spongy and compacted layers that met the diagnostic criteria of LVNC (>2.3)3 in which a giant trabecula delineated an accessory ventricular chamber (Figs 1C, D; 2 and 3). Neither defects of myocardial perfusion nor areas of delayed enhancement and congenital midline defects were detected. Other diagnoses that should be taken into consideration are aneurysms, pseudoaneurysms, diverticula of the LV, or isolated double-chambered left ventricle (DCLV). Distinguishing among these conditions is challenging but of great clinical importance given the consequent different treatment modalities and prognosis. Double-chambered ventricle is a rare congenital cardiac anomaly, characterized by the division of the ventricular chamber into two chambers by normal muscular tissue and may be associated with congenital midline defects. This diagnosis was excluded because the muscular tissue separating the two chambers presented a two-layered structure; moreover, double-chambered right ventricle is more common than DCLV.4 Furthermore, a precise evaluation of wall motion and of the width of the two chambers communication was helpful to exclude the diagnosis of diverticulum, aneurysms, and pseudoaneurysms. The
diagnosis of LV diverticulum was excluded because this communicates with the main chamber through a narrow neck, not noticeable in our case. The diagnosis of left ventricular aneurysm was also excluded because this entity lacks the complete layering of the ventricular wall and has dyskinetic motion, expanding during systole; moreover in LV aneurysm the presence of delayed enhancement due to fibrosis is detectable at CMR. These characteristics also apply to pseudoaneurysm, which is a contained rupture of LV free wall and is devoid of any component of the cardiac wall.5 On the basis of the presence of an altered ratio between noncompacted and compacted layers (> 2.3), the presence of all the ventricular wall layers and normal systodiastolic movements of the accessory chamber, the findings reported were considered consistent with the diagnosis of LVNC in which a giant trabecula, parallel to the interventricular septum, subdivided the LV, thus simulating an accessory ventricular chamber. This association suggests that LVNC during embryonic development may result in subdivided LV. The patient is currently in good health and she is followed-up in our institution without any therapy.
References 1
2
3
4 5
Fazio G, D’angelo L, Visconti C, et al. Isolated left ventricular noncompaction: a larger part of submerged iceberg with criteria for diagnosis to the limits of standard. Int J Cardiol 2010; 145:e1–e2. Correia AS, Madureira AJ, Gonc¸alves A, Almeida PB, Arau´jo V. Doublechambered left ventricle plus left ventricular noncompaction: report of an abnormal association. Eur Heart J Cardiovasc Imaging 2013; 14:127. Petersen SE, Selvanayagam JB, Wiesmann F, et al. Left ventricular noncompaction: insights from cardiovascular magnetic resonance imaging. J Am Coll Cardiol 2005; 46:101–105. Harikrishnan S, Sivasankaran S, Tharakan J. Double chambered left ventricle. Int J Cardiol 2002; 82:59–61. Kilner PJ, Geva T, Kaemmerer H, Trindade PT, Schwitter J, Webb GD. Recommendations for cardiovascular magnetic resonance in adults with congenital heart disease from the respective working groups of the European Society of Cardiology. Eur Heart J 2010; 31:794–805.
Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
