International Journal of Cardiology 171 (2014) e107–e109
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
Isolated giant congenital diverticulum of the left ventricle in adulthood Matthias Lachaud a,b,c,⁎, Adriano Tivane a, Alda Menete a, Kumar Narayanan d, Daniel Sidi a,e, Beatriz Ferreira a a
Instituto do Coração, Maputo Mozambique Institut du Thorax, Nantes France c Inserm, UMR 970, Hôpital Europeen George Pompidou, Paris, France d Heart Institute, Cedars-Sinai Medical Center, Los Angeles, USA e Pediatric Cardiology, Hôpital Necker-Enfants Malades, Paris, France b
a r t i c l e
i n f o
Article history: Received 15 September 2013 Received in revised form 9 December 2013 Accepted 14 December 2013 Available online 22 December 2013 Keywords: Congenital Surgery Ventricular Syncope Heart failure
To the Editor A 26 year-old patient with no specific personal or family history of heart disease or history of premature death in the family consulted our center for effort related dyspnea associated with episodes of palpitations, atypical chest pain as well as three episodes of syncope over the past 4 months. Clinical examination revealed a harsh pan-systolic murmur (5/6) at the apex and was otherwise unremarkable. The chest X-ray showed a normal cardiothoracic index with a discrete unusual prominence of the left lower border and apex. The electrocardiogram showed sinus rhythm with no features of ventricular hypertrophy or enlargement and no premature beats. Echocardiography demonstrated the presence of a voluminous horseshoe-shaped outpouching at the left ventricular (LV) apex with posterior, but no infradiaphragmatic extension (Fig. 1). It exhibited paradoxical contraction, probably because it filled out of the LV, and hence expanded during LV systole. A slight degree of compression of the right ventricle could be observed in some views. The outpouching had a thrombus at its distal end (Fig. 2). It communicated with the LV apex through a narrow, 8 mm neck. No other cardiac abnormalities, were found, including ventricular septal defect; however LV filling pressure was elevated. A computed tomographic (CT) scan ⁎ Corresponding author at: Instituto do Coração, Av. Kenneth Kaunda No. 1111, Maputo, Mozambique. Tel.: +258 1 41 63 47; fax: +258 1 41 43 85. E-mail address: [email protected] (M. Lachaud).
0167-5273/$ – see front matter © 2013 Elsevier Ireland. Ltd All rights reserved. http://dx.doi.org/10.1016/j.ijcard.2013.12.027
confirmed the diagnosis, and allowed precise measurement of the outpouching (9 cm × 7 cm) and delineation of morphology (Fig. 3). Coronary angiography ruled out any associated coronary artery disease. Work-up did not reveal any evidence for an acquired etiology (tuberculosis, traumatic, myocardial inflammation, chronic inflammatory diseases). The patient's condition was stabilized first by institution of medical therapy (angiotensin converting enzyme inhibitor, diuretic, beta-blocker, amiodarone, anticoagulant). Surgical excision was decided upon because of the size, the presence of the thrombus, symptoms of congestive heart failure as well as the syncope presumed to be related to ventricular arrhythmias. This surgery was performed during a humanitarian mission carried out by a Swiss non-governmental organization. The patient's symptoms quickly resolved after surgery and medication was stopped. Histopathology showed the presence of all the three usual myocardial layers, confirming that this giant ventricular outpouching was a diverticulum. Apical diverticulum and aneurysm are classified as congenital in origin when no causes for an acquired defect are discovered. Fetal echocardiography may help to support a congenital etiology when the diagnosis is made during the prenatal period with no evidence of an acquired abnormality. When first detected late in childhood or adulthood, it is more difficult to determine whether an intrathoracic outpouching of the ventricle is congenital or acquired. In this situation, cardiac catheterization and angiography are usually performed to rule out coronary lesions [1–4]. CT scan may be of interest to characterize the nature of the outpouching, evaluate the coronary arteries as well as to detect other associated cardiac defects [5]. Apical congenital diverticulum and aneurysm are two distinct entities with different morphologic and histologic characteristics and outcomes. Apical congenital aneurysm is defined by a large akinetic or dyskinetic pouch with a wide connection to the ventricle and endocardial or transmural fibrosis on histologic examination, whereas apical congenital diverticulum usually demonstrates synchronous contraction with 3 well defined myocardial layers on pathology. The outcome of aneurysms has been described as being particularly more severe compared to diverticulums. The outpouching presented here is a congenital ventricular diverticulum, according to the pathology examination, and the dysysnchony appears to be related to the very large size of the outpouching with a normal wall, unable to accommodate the large volumes during cardiac contraction. Congenital diverticulum of the LV is a very uncommon pathology [1–3]. Apical congenital diverticulum is most usually a part of the
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M. Lachaud et al. / International Journal of Cardiology 171 (2014) e107–e109
Fig. 1. Echocardiographic four-chamber view. Diverticulum arising from the LV apex with a narrow 8 mm communication and causing some compression of the RV apex. D: diverticulum; LV: left ventricle; RV: right ventricle.
Cantrell syndrome and is related to a common defect in embryologic development of the midline thoracoabdominal formation. This condition may present with a large midline thoracoabdominal defect which is symptomatic with an obvious pulsatile epigastric diverticulum (omphalocele), or a small dehiscence which is often discovered only at surgery [2,3]. Though congenital LV diverticulum can be asymptomatic, it is sometimes complicated by congestive heart failure, ventricular arrhythmia, arterial thromboembolic incidents or sudden death by rupture [1–4]. Management is usually based on the treatment of the congestive heart failure and arrhythmia, as well as the prevention of
thomboembolism by anticoagulation [3]. While no standard indications exist for surgery, the decision depends on parameters such as diverticulum size, the presumed risk of rupture, the occurrence of a thromboembolic incident or serious ventricular arrhythmia [1,2]. Congenital heart disease in Africa is usually discovered late, often with a dramatic presentation [6,7]. The lack of regular access to heart surgery, and dependence on charitable humanitarian missions due to scarcity of resources [8], makes the management of such situations challenging. This case report has been written in accordance with the international guidelines for publication.
Fig. 2. Horseshoe-shaped left ventricular diverticulum with posterior extension and thrombus. T: thrombus; D: diverticulum.
M. Lachaud et al. / International Journal of Cardiology 171 (2014) e107–e109
e109
Fig. 3. Contrast enhanced computed tomographic (CT) scan, transverse view showing the diverticulum (9.25 × 7.02 cm) with a heterogeneous and trabeculated wall without intra abdominal extension. D: diverticulum.
References [1] Ohlow MA. Congenital left ventricular aneurysms and diverticula: definition, pathophysiology, clinical relevance and treatment. Cardiology 2006;106:63–72. [2] Marijon E, Ou P, Fermont L, et al. Diagnosis and outcome in congenital ventricular diverticulum and aneurysm. J Thorac Cardiovasc Surg 2006;131:433–7. [3] Marijon E, Hausse-Mocumbi AO, Ferreira B. Cantrell's syndrome. Cardiol Young 2006;16:95–6. [4] Haegeli LM, Ercin E, Wolber T, et al. Arrhythmic manifestations in patients with congenital left ventricular aneurysms and diverticula. Am J Cardiol 2011;108:1826–30.
[5] Gobal F, Sachdeva R, Uretsky BF. Computed tomography and angiography of left ventricular diverticulum. J Am Coll Cardiol 2011;58:e17. [6] Celermajer DS, Chow CK, Marijon E, Anstey NM, Woo KS. Cardiovascular disease in the developing world: prevalences, patterns, and the potential of early disease detection. J Am Coll Cardiol 2012;60:1207–16. [7] Zühlke L, Mirabel M, Marijon E. Congenital heart disease and rheumatic heart disease in Africa: recent advances and current priorities. Heart 2013;99:1554–61. [8] Ferratini M, Marianeschi S, Santoro F, et al. Valvulopathies in sub-Saharan African children: patterns, humanitarian interventions and cardiac surgical problems. Int J Cardiol 2013;165:237–41.
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
Isolated giant congenital diverticulum of the left ventricle in adulthood Matthias Lachaud a,b,c,⁎, Adriano Tivane a, Alda Menete a, Kumar Narayanan d, Daniel Sidi a,e, Beatriz Ferreira a a
Instituto do Coração, Maputo Mozambique Institut du Thorax, Nantes France c Inserm, UMR 970, Hôpital Europeen George Pompidou, Paris, France d Heart Institute, Cedars-Sinai Medical Center, Los Angeles, USA e Pediatric Cardiology, Hôpital Necker-Enfants Malades, Paris, France b
a r t i c l e
i n f o
Article history: Received 15 September 2013 Received in revised form 9 December 2013 Accepted 14 December 2013 Available online 22 December 2013 Keywords: Congenital Surgery Ventricular Syncope Heart failure
To the Editor A 26 year-old patient with no specific personal or family history of heart disease or history of premature death in the family consulted our center for effort related dyspnea associated with episodes of palpitations, atypical chest pain as well as three episodes of syncope over the past 4 months. Clinical examination revealed a harsh pan-systolic murmur (5/6) at the apex and was otherwise unremarkable. The chest X-ray showed a normal cardiothoracic index with a discrete unusual prominence of the left lower border and apex. The electrocardiogram showed sinus rhythm with no features of ventricular hypertrophy or enlargement and no premature beats. Echocardiography demonstrated the presence of a voluminous horseshoe-shaped outpouching at the left ventricular (LV) apex with posterior, but no infradiaphragmatic extension (Fig. 1). It exhibited paradoxical contraction, probably because it filled out of the LV, and hence expanded during LV systole. A slight degree of compression of the right ventricle could be observed in some views. The outpouching had a thrombus at its distal end (Fig. 2). It communicated with the LV apex through a narrow, 8 mm neck. No other cardiac abnormalities, were found, including ventricular septal defect; however LV filling pressure was elevated. A computed tomographic (CT) scan ⁎ Corresponding author at: Instituto do Coração, Av. Kenneth Kaunda No. 1111, Maputo, Mozambique. Tel.: +258 1 41 63 47; fax: +258 1 41 43 85. E-mail address: [email protected] (M. Lachaud).
0167-5273/$ – see front matter © 2013 Elsevier Ireland. Ltd All rights reserved. http://dx.doi.org/10.1016/j.ijcard.2013.12.027
confirmed the diagnosis, and allowed precise measurement of the outpouching (9 cm × 7 cm) and delineation of morphology (Fig. 3). Coronary angiography ruled out any associated coronary artery disease. Work-up did not reveal any evidence for an acquired etiology (tuberculosis, traumatic, myocardial inflammation, chronic inflammatory diseases). The patient's condition was stabilized first by institution of medical therapy (angiotensin converting enzyme inhibitor, diuretic, beta-blocker, amiodarone, anticoagulant). Surgical excision was decided upon because of the size, the presence of the thrombus, symptoms of congestive heart failure as well as the syncope presumed to be related to ventricular arrhythmias. This surgery was performed during a humanitarian mission carried out by a Swiss non-governmental organization. The patient's symptoms quickly resolved after surgery and medication was stopped. Histopathology showed the presence of all the three usual myocardial layers, confirming that this giant ventricular outpouching was a diverticulum. Apical diverticulum and aneurysm are classified as congenital in origin when no causes for an acquired defect are discovered. Fetal echocardiography may help to support a congenital etiology when the diagnosis is made during the prenatal period with no evidence of an acquired abnormality. When first detected late in childhood or adulthood, it is more difficult to determine whether an intrathoracic outpouching of the ventricle is congenital or acquired. In this situation, cardiac catheterization and angiography are usually performed to rule out coronary lesions [1–4]. CT scan may be of interest to characterize the nature of the outpouching, evaluate the coronary arteries as well as to detect other associated cardiac defects [5]. Apical congenital diverticulum and aneurysm are two distinct entities with different morphologic and histologic characteristics and outcomes. Apical congenital aneurysm is defined by a large akinetic or dyskinetic pouch with a wide connection to the ventricle and endocardial or transmural fibrosis on histologic examination, whereas apical congenital diverticulum usually demonstrates synchronous contraction with 3 well defined myocardial layers on pathology. The outcome of aneurysms has been described as being particularly more severe compared to diverticulums. The outpouching presented here is a congenital ventricular diverticulum, according to the pathology examination, and the dysysnchony appears to be related to the very large size of the outpouching with a normal wall, unable to accommodate the large volumes during cardiac contraction. Congenital diverticulum of the LV is a very uncommon pathology [1–3]. Apical congenital diverticulum is most usually a part of the
e108
M. Lachaud et al. / International Journal of Cardiology 171 (2014) e107–e109
Fig. 1. Echocardiographic four-chamber view. Diverticulum arising from the LV apex with a narrow 8 mm communication and causing some compression of the RV apex. D: diverticulum; LV: left ventricle; RV: right ventricle.
Cantrell syndrome and is related to a common defect in embryologic development of the midline thoracoabdominal formation. This condition may present with a large midline thoracoabdominal defect which is symptomatic with an obvious pulsatile epigastric diverticulum (omphalocele), or a small dehiscence which is often discovered only at surgery [2,3]. Though congenital LV diverticulum can be asymptomatic, it is sometimes complicated by congestive heart failure, ventricular arrhythmia, arterial thromboembolic incidents or sudden death by rupture [1–4]. Management is usually based on the treatment of the congestive heart failure and arrhythmia, as well as the prevention of
thomboembolism by anticoagulation [3]. While no standard indications exist for surgery, the decision depends on parameters such as diverticulum size, the presumed risk of rupture, the occurrence of a thromboembolic incident or serious ventricular arrhythmia [1,2]. Congenital heart disease in Africa is usually discovered late, often with a dramatic presentation [6,7]. The lack of regular access to heart surgery, and dependence on charitable humanitarian missions due to scarcity of resources [8], makes the management of such situations challenging. This case report has been written in accordance with the international guidelines for publication.
Fig. 2. Horseshoe-shaped left ventricular diverticulum with posterior extension and thrombus. T: thrombus; D: diverticulum.
M. Lachaud et al. / International Journal of Cardiology 171 (2014) e107–e109
e109
Fig. 3. Contrast enhanced computed tomographic (CT) scan, transverse view showing the diverticulum (9.25 × 7.02 cm) with a heterogeneous and trabeculated wall without intra abdominal extension. D: diverticulum.
References [1] Ohlow MA. Congenital left ventricular aneurysms and diverticula: definition, pathophysiology, clinical relevance and treatment. Cardiology 2006;106:63–72. [2] Marijon E, Ou P, Fermont L, et al. Diagnosis and outcome in congenital ventricular diverticulum and aneurysm. J Thorac Cardiovasc Surg 2006;131:433–7. [3] Marijon E, Hausse-Mocumbi AO, Ferreira B. Cantrell's syndrome. Cardiol Young 2006;16:95–6. [4] Haegeli LM, Ercin E, Wolber T, et al. Arrhythmic manifestations in patients with congenital left ventricular aneurysms and diverticula. Am J Cardiol 2011;108:1826–30.
[5] Gobal F, Sachdeva R, Uretsky BF. Computed tomography and angiography of left ventricular diverticulum. J Am Coll Cardiol 2011;58:e17. [6] Celermajer DS, Chow CK, Marijon E, Anstey NM, Woo KS. Cardiovascular disease in the developing world: prevalences, patterns, and the potential of early disease detection. J Am Coll Cardiol 2012;60:1207–16. [7] Zühlke L, Mirabel M, Marijon E. Congenital heart disease and rheumatic heart disease in Africa: recent advances and current priorities. Heart 2013;99:1554–61. [8] Ferratini M, Marianeschi S, Santoro F, et al. Valvulopathies in sub-Saharan African children: patterns, humanitarian interventions and cardiac surgical problems. Int J Cardiol 2013;165:237–41.
