Asian Cardiovasc Thorac Ann 2005;13:79-81
© 2005 Asia Publishing EXchange Ltd
Congenital Giant Diverticulum of The Left Ventricle in an Adult
Sai S Oruganti, DM,
Dharma R Ayapati, MCh1,
Milton A James, MD,
Jagan MR Jinna, MD2,
Seshagiri R Damera, DM,
Sundaram Challa, MD3
Department of Cardiology
1 Department of Cardiothoracic Surgery
2 Department of Radiology
3 Department of Pathology, Nizam’s Institute Of Medical Sciences, Hyderabad, India
For reprint information contact: Sai S Oruganti, DM Tel: 91 40 2339 6538 Fax: 91 40 2331 0076 Email: osaisatish{at}yahoo.com, Department of Cardiology, Nizam’s Institute Of Medical Sciences, Punjagutta, Hyderabad 500 082, Andhra Pradesh, India.
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ABSTRACT
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Congenital diverticulum of the left ventricle is a rare anomaly. Echocardiography, cardiac catheterization, and magnetic resonance imaging of the heart diagnosed a giant left ventricular diverticulum in a 20-year-old male with a history of dyspnea and palpitations. He underwent successful surgical correction of the left ventricular diverticulum.
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INTRODUCTION
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Congenital diverticula of the left ventricle (LV) are extremely uncommon; the actual incidence is unknown. They are reported either as an isolated entity or as part of the syndrome of muscular diverticula of the heart arising from either or both ventricles, associated with other congenital cardiac and/or somatic defects.1,2 The following is an interesting case of an isolated congenital giant LV diverticulum in an adult with subsequent successful surgical correction. Available literature on congenital LV diverticulum is reviewed.
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CASE REPORT
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A 20-year-old male patient was admitted to the University hospital with complaints of breathlessness on exertion (New York Heart Association Class II) and palpitations. General physical examination was unremarkable. Cardiovascular examination revealed diffuse pulsations in the left 3rd to 5th space. A prominent fourth heart sound and an ejection systolic murmur of Gr II/VI were heard at the left sternal border. Chest X-Ray showed cardiomegaly of LV configuration with a cardiothoracic ratio of 65%. Electrocardiogram showed normal sinus rhythm and prominent Q waves with ST elevation in leads II, III, aVF.
Transthoracic echocardiogram revealed a globular accessory chamber of 10 x 8 cm size with septations. This was in communication with the LV via a narrow neck at the inferolateral wall. The accessory chamber showed diffuse hypokinesia with blood flow to and fro from the LV with cardiac contractions. There was mild mitral regurgitation with a LV ejection fraction of 45%. Transesophageal echocardiogram delineated the morphology of the accessory chamber much more clearly with a large thrombus in-between the septa (Figure 1
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Figure 1. Transesophageal echocardiogram showing a large left ventricular diverticulum with septations and thrombus (arrow).
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Magnetic resonance imaging (MRI) of the heart with spin echo T1 weighted images showed a large out-pouching with a narrow neck, which was in communication with the LV at the inferolateral wall. This was abutting the left dome of the diaphragm (Figure 2).
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Figure 2. MRI of the heart in the coronal plane showing a large diverticulum with a narrow neck (arrow) which is in communication with the left ventricle.
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The patient underwent cardiac catheterization, which showed normal right heart pressures and an elevated LV end diastolic pressure (LVEDP) of 28 mm Hg. Coronary angiography revealed normal coronary arteries. LV angiography was omitted in view of elevated LVEDP and thrombus in the diverticulum, to avoid possible embolic complications during the procedure.
With a diagnosis of congenital LV diverticulum, the patient was admitted for surgical correction. The heart was approached through median sternotomy and the patient was connected to cardiopulmonary bypass using bicaval connection and ascending aortic arterial return, under moderate hypothermia. The whole procedure was performed on the fibrillating heart without cross clamping. There was a large diverticulum (10 x 8 cm) with a narrow neck about 2.5 cm in diameter which was in connection with the LV at the inferolateral wall. Multiple fibrous septa were traversing the cavity with a thrombus inside the sac. The neck was closed using a Gore-Tex patch (WLGore, Flagstaff, AZ, USA) with continuous sutures of proline and reinforced with the redundant sac in two layers. The heart was defibrillated and it gained sinus rhythm. The patient was re-warmed, de-aired, and weaned off smoothly from cardiopulmonary bypass. He had an uneventful recovery.
Histopathological examination of the wall of the diverticulum showed the endocardial surface lined by flattened cells and myocardial fibers in the sub-endocardial surface and adventitia. There was thrombus formation in the lumen and lympho-mononuclear aggregates in the media and adventitia (Figure 3). These findings were suggestive of LV diverticulum. Postoperative MRI of the heart showed totally resected diverticulum with complete obliteration of its communication with the LV (Figure 4).
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Figure 3. Photomicrograph showing the wall of the left ventricular diverticulum with intimal proliferation and cardiac muscle fibers (hematoxylin and eosin stain, original magnification x 10).
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Figure 4. Postoperative MRI of the heart showing the totally excised diverticulum and its communication with the left ventricle.
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DISCUSSION
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True LV aneurysm, congenital or acquired, may be defined as an abnormal bulge or out-pouching of a portion of the LV wall secondary to a mechanical weakness caused by a developmental defect or an acquired abnormality. Usually, LV aneurysm occurs in adults secondary to myocardial infarction. In contrast congenital aneurysm of the LV is rare. A ventricular aneurysm is classified as congenital in origin when no known causes of acquired aneurysm are found.3 A diverticulum, on the other hand, is usually defined as a pouch or sac created by herniation of the internal layer of a tubular organ through a defect in its muscular coat. Both of these definitions are independent of the shape of the dilated area, although diverticula are often tubular or sacular and have a narrow orifice, whereas aneurysms have a wide mouth. The neck is defined as narrow if its diameter is less than 40% of the maximal diameter of the sac.3 In our patient the neck diameter was only 2.5 cm whereas the maximal diameter of the sac was 10 cm. In very rare instances, an aneurysm of the LV may have a narrow neck opening into the LV cavity. Congenital diverticulum of the LV can occur at the cardiac base in sub-mitral or sub-aortic locations. It is rarely found at an apical location, or associated with developmental defects of the diaphragm, pericardium and abdominal wall.2 In our case the diverticulum was at the inferolateral wall of the LV.
Congenital diverticulum of the LV is usually asymptomatic. If symptomatic, patients may present with congestive heart failure, arrhythmia and thromboembolism. Our patient had recurrent ventricular tachycardia, dyspnea on effort and non-anginal chest pain. The cause and effect relation of chest pain in these patients is not conclusively proven.4 Mitral regurgitation may be observed secondary to anatomic distortion of the mitral valve apparatus. Our patient had mild mitral regurgitation, which totally disappeared following surgical repair of the diverticulum.
Sudden death due to rupture of the diverticulum has been described. Lowe and colleagues suggested that rupture might be the result of excessive pressure in the lumen of the diverticulum caused by the contraction of the LV against the closed orifice of the diverticulum.5 Some authors have suggested contractility of the diverticulum as a differentiating feature from congenital aneurysm.6 However there is disagreement over this.
Singh and colleagues characterized diverticula as being non-contractile and congenital aneurysms as contractile.7 The patient under discussion showed diffuse hypokinesia of the diverticular wall probably causing blood stasis in-between the septa and at some places causing formation of thrombi. Several authors have attempted to differentiate congenital aneurysm from diverticulum on different criteria.4 However, presenting features, investigation modalities and therapeutic options are similar for both entities. A diagnosis of LV aneurysm or diverticulum may be suggested by clinical examination, electrocardiogram and chest X-Ray. Echocardiogram, MRI of the heart and cardiac catheterization will confirm the diagnosis.
The natural history of congenital LV diverticulum is not clearly known. As mentioned in the literature,8 as well as in our own experience, early surgical correction may be beneficial for optimal outcome and to prevent sudden rupture and other catastrophic sequelae.
We conclude that congenital LV diverticulum is a disease of diverse manifestations and for diagnosis a high index of suspicion is required during interpretation of chest X-Ray and echocardiogram. Early diagnosis using invasive and non-invasive cardiac imaging techniques is essential to adequately differentiate congenital LV diverticulum from acquired true aneurysm and pseudoaneurysm in order to avert its adverse natural history.
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REFERENCES
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- Hoeffel JC, Henry M, Pernot C. Heart diverticula in children: radiological aspects. Ann Radiol
1974;17:411–5.
- Cantrell JR, Haller JA, Ravitch MM. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium and heart. Surg Gynaecol Obstet
1958;107:602–14.
- Tecklenberg PL, Alderman EL, Billingham ME, Shumway NE. Diverticulum of the left ventricle in hypertrophic cardiomyopathy. Am J Med
1978;64:707–14.[Medline]
- Vaidiyanathan D, Prabhakar D, Selvam K, Alagesan R, Thirunavukarasu N, Muthukumar D. Isolated congenital left ventricular diverticulum in adults. Indian Heart J
2001;53:211–3.[Medline]
- Lowe JB, Williams JC, Robb D, Cole D. Congenital diverticulum of the left ventricle. Br Heart J
1959;21:101–6.
- Baltaxe HA, Wilson WJ, Amiel M. Diverticulosis of the left ventricle. Am J Roentgenol
1979;133:257–61.[Abstract]
- Singh A, Katkov H, Zavoral JH, Sane SM, McLoed JD. Congenital aneurysms of the left ventricle. Am Heart J
1980;99:25–32.[Medline]
- Meyersohn J, Schiffer J. Rupture of a congenital aneurysm of the left ventricular apex. Chest
1973;63:838–40.[Abstract/Free Full Text]
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ABSTRACT
INTRODUCTION
