Ann Thorac Surg 2007;83:2222-2224
© 2007 The Society of Thoracic Surgeons
Case Reports
Localized Pericardial Constriction Resulting in a "Dumbbell" Heart
Zainul A. Hamdulay, MCha,
Pawan Kumar, MCha,*,
Mohammed Ali, MCha,
Shilpa S. Bhojraj, MDb,
Sanjesh B. Jain, MDb,
Anil M. Patwardhan, MChc
a Department of Cardiovascular Surgery, Prince Aly Khan Hospital, Mumbai, India
b Department of Cardiac Anaesthesia, Prince Aly Khan Hospital, Mumbai, India
c Department of Cardiovascular Surgery, King Edward Memorial Hospital, Mumbai, India
Accepted for publication December 29, 2006.
* Address correspondence to Dr Kumar, Department of Cardiovascular Surgery, Prince Aly Khan Hospital, Nesbit Rd, Mazagaon, Mumbai, 400 010, India (Email: pawkum73{at}yahoo.com).
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Abstract
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We describe an unusual case of a young man presenting with calcific constrictive pericarditis. The patient had a history of restrictive cardiomyopathy and pericardial effusion during infancy and received antituberculous treatment. Investigations revealed the presence of thickened pericardium and a thickened calcific constrictive band around the atrioventricular groove posteriorly and over the infundibulum anteriorly. Intraoperatively, the band caused the heart to have a "dumbbell" appearance. A pericardiectomy was performed along with excision of the constricting band. The patient had an uneventful recovery.
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Introduction
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Constrictive pericarditis is an uncommon but potentially curable pathology. Localized pericardial constrictions are very rare. We report a case of a patient presenting with constrictive pericarditis, with an accompanying tight constrictive ring along the atrioventricular groove posteriorly and crossing the infundibulum anteriorly, resulting in a dumbbell-shaped heart.
A 23-year old man was admitted to Prince Aly Khan Hospital with complaints of class II dyspnea, easy fatigability, and pitting pedal edema. When the patient was 2 years old, he was admitted with fever, cough, and breathlessness. He was then investigated and was diagnosed as a case of restrictive cardiomyopathy and pericardial effusion. The patient was empirically started on antituberculous treatment for a period of 2 years. He remained asymptomatic for next 15 years.
Five years ago, he developed symptoms of congestive heart failure that had progressed during the last 2 years. On examination, he appeared undernourished. The pulse rate was 70 beats/min and of low volume. Pulsus paradoxus was present. No pericardial knock or rub could be auscultated. There was tender hepatomegaly, ascites, and lower limb pitting edema.
A chest roentgenogram showed calcification of the pericardium. An echocardiogram revealed a markedly thickened and calcific pericardium (10 mm) causing constriction at the atrioventricular groove. The left ventricular posterior wall showed sudden diastolic cessation with septal bounce. The inferior vena cava was dilated, with absent respiratory variation. Left ventricular size and function were normal.
A cardiac magnetic resonance imaging study showed changes of constrictive pericarditis. A ring of calcification, encircling the right and left ventricles and distal to the atrioventricular groove was seen at the junction of the basal and mid cavitary regions. An altered shape of left ventricle was seen as a result (Fig 1).
The operation was performed through a median sternotomy. The heart had a dumbbell shape because of a tight constricting band seen just around and immediately below the atrioventricular groove (posteriorly and inferiorly). Anteriorly, the band crossed the infundibulum (Fig 2). The band was heavily calcified. The pericardium was dissected of the ventricles beyond the band. The adhesions were flimsy in quality. The constricting band was then incised and areas of calcification debrided using a rongeur. The entire visible area of the band was excised. Next, the right atrium and the vena cavae were freed. Central venous pressure decreased from 25 mm of Hg to 16 mm of Hg. The chest was closed after achieving hemostasis.
The patient had an uneventful postoperative recovery. He was extubated on the first postoperative day and was discharged on day 10. The patient has now been following up for 1 year and is in New York Heart Association functional class I. He has no signs of congestive cardiac failure. He is currently being continued on minimal diuretics. His follow-up echocardiography study shows an essentially normal heart.
Histopathology of the excised pericardium showed fibrocollagenous thickening of pericardium with areas of hemorrhage and heavy calcific deposits. No areas of active inflammation or granuloma were seen. No vasculitis was identified, and there was no evidence of primary or metastatic malignancy.
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Comment
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Constrictive pericarditis is an important diagnosis because of its potential cure. It usually develops after an episode of acute pericarditis, with subsequent organization and fibrous scarring of the pericardium, resulting in obliteration of the pericardial space and restricted filling of the cardiac chambers. Tuberculosis is the most common cause in developing countries [1], such as India. In Western countries, tuberculous constrictive pericarditis is rare. The other causes for constrictive pericarditis include idiopathic, after radiotherapy or cardiac surgery, related to connective tissue disease, neoplastic, uremic, and sarcoidosis.
Localized constrictive pericarditis is rare [2–5]. Localized pericardial constriction has been reported after previous pericardiectomy [2, 5] and along with congenital heart disease [3]. The most common presentation reported with localized constrictions involves the atrioventricular groove, along with obstruction to the right ventricular outflow tract [3, 5]. This patient had a similar picture. Although calcification may not necessarily cause clinical constriction, extensive calcification is the most notable characteristic of chronic constrictive pericarditis [4].
Constrictive pericarditis is uncommon in pediatric patients [6]. The patient discussed here had a presentation suggestive of constrictive pericarditis when he was 2 years old; however, he recovered with medical management and antituberculosis treatment. Although the cause could not be identified, he was empirically treated as a case of tuberculous pericarditis owing to the high incidence of the disease in the country. Whether antituberculous treatment prevents constrictive pericarditis is debatable [7].
Constrictive pericarditis subsequently developed in this patient after many years, an observation that has been shared by others [5]. Localized constrictions are most common around the atrioventricular groove posteriorly and over the infundibulum anteriorly. The reason has not been discussed. We believe that the atrioventricular groove and right ventricle body–infundibulum junction is the transition gutter-like area between the relatively fixed (and less moving) structures such as the atria and infundibulum and the dynamic ventricles. This would logically result in stasis of an organizing collection in this particular region. This collection, over years, is replaced with cicatrix fibrocalcific tissue.
Total pericardiectomy is the treatment of choice for constrictive pericarditis and has good long-term results [8]. Our patient underwent pericardiectomy and had an uneventful postoperative recovery. The histopathology of the excised pericardium did not reveal evidence of active or healed tuberculosis. We believe, however, that the primary cause was tuberculosis during infancy, with subsequent cicatrization that led to chronic constrictive pericarditis.
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References
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- Spondnick DH. Chronic and constrictive pericarditis. New York, NY: Grune and Stratton; 1964. pp. 81-100.
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- Nataf P, Cacoub P, Dorent R, et al. Results of subtotal pericardiectomy for constrictive pericarditis Eur J Cardiothorac Surg 1993;7:252-255.[Abstract]
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Abstract
Introduction
