Asian Cardiovasc Thorac Ann 2002;10:351-353
© 2002 Asia Publishing EXchange Pte Ltd
Bidirectional Glenn Shunt For Right Ventricular Endomyocardial Fibrosis
Amit Mishra, MS,
Soman Rema Krishna Manohar, MCh,
Ramalingam Sankar Kumar, MCh,
Marthandavarma Sankaran Valiathan, FRCS
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Department of Cardiovascular Thoracic Surgery Sree Chitra Tirunal Institute for Medical Sciences Technology Thiruvananthapuram, Kerala India
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For reprint information contact: Soman Rema Krishna Manohar, MCh Tel: 91 471 52 4648 Fax: 91 471 44 6433 email: manohar{at}sctimst.ker.nic.in Department of Cardiovascular and Thoracic Surgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India.
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ABSTRACT
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A 25-year-old man in New York Heart Association functional class IV with right ventricular endomyocardial fibrosis received a palliative bidirectional Glenn shunt. Despite a stormy postoperative convalescence the bidirectional Glenn shunt provided good long-term palliation.
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INTRODUCTION
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Endomyocardial fibrosis is a restrictive cardiomyopathy, often bilateral, of uncertain etiology. It is commonly reported from tropical countries, and the Kerala state of India has a high prevalence of the disease. There is predominant involvement of the right ventricle with minimal involvement of the left ventricle, presenting with gross cardiomegaly, ascites, and multiorgan dysfunction. As medical treatment does not afford significant long-term palliation, various surgical treatments have been attempted, including a Fontan-type procedure.
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CASE REPORT
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A 25-year-old male shopkeeper, symptomatic for 5 years, presented with exertional palpitations of 3 years duration and exertional dyspnea (New York Heart Association functional class IV) for the previous 6 months. Clinical examination revealed a heart rate of 70 beats per minute with occasional arrhythmia, blood pressure of 110/70 mm Hg, and an elevated jugular venous pulse. The apex beat was in the left 6th intercostal space 2 cm lateral to the mid clavicular line. On auscultation, a grade 2/6 short systolic murmur was heard over the right sternal border. The liver was palpable as soft and pulsatile 5 cm below the costal margin, and there was tense ascites. Chest radiography revealed a large globular heart and a cardiothoracic ratio of 0.75 (Figure 1 
). Electrocardiography showed atrial fibrillation, QRS axis at + 90º, and a controlled ventricular rate. Two-dimensional transthoracic echocardiography with Doppler studies displayed features of severe right ventricular endomyocardial fibrosis with severe obliteration of the right ventricular cavity and apex, severe tricuspid regurgitation, and gross enlargement of the right atrium. The left ventricle appeared normal, but there was mild mitral regurgitation. Cardiac catheterization demonstrated elevated right atrial (25 mm Hg) and right ventricular end-diastolic (24 mm Hg) pressures. On right ventriculography, there was severe obliteration of the ventricular cavity and apex, suggestive of endomyocardial fibrosis. Left ventriculography showed a smooth rounded configuration of the ventricular apex (bald appearance) and mild mitral regurgitation, suggesting mild disease of the left ventricle.1
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Figure 1. Anteroposterior chest radiograph showing a large globular heart and a cardiothoracic ratio of 0.75.
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Surgery was performed under standard cardiopulmonary bypass with moderate hypothermia and cold crystalloid antegrade cardioplegia for myocardial protection. The superior vena cava was divided between clamps, and the superior end was anastomosed end-to-side with the superior surface of the right pulmonary artery; the lower end was suture closed. The heart resumed function in atrial fibrillation, and cardiopulmonary bypass was discontinued with heavy inotropic support (intravenous infusion of isoprenaline 0.2 µg·kg-1·min-1 and epinephrine 0.2 µg·kg-1·min-1). The postoperative recovery was stormy. The patient was re-explored for cardiac tamponade on the 5th postoperative day, and required ventilatory support until the 8th postoperative day. The inotropics were tapered over a period of 3 weeks. A tense pericardial collection developed, which needed subxiphoid drainage on the 26th day. However, he made a gradual but satisfactory recovery on diuretics and decongestants, and was discharged after 2 months. Cardiac catheterization and angiography just before discharge showed a functioning Glenn shunt with reduced right atrial pressure (from 25 to 16 mm Hg) and right ventricular end-diastolic pressure (from 24 to 14 mm Hg). After 8 years of follow-up, he was in New York Heart Association functional class II and back at work. Transthoracic echocardiography showed an ejection fraction of 71%, no progression of disease in either the right or left ventricle, and mild mitral regurgitation. Digital subtraction angiography demonstrated a patent Glenn shunt with good bidirectional flow (Figure 2 ).
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DISCUSSION
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Surgical techniques reported for the treatment of isolated right ventricular endomyocardial fibrosis include right ventricular endocardiectomy with replacement of the tricuspid valve, a palliative classic Glenn shunt, and total cavopulmonary connection.2–4 From 1981 to 1994, 110 patients with endomyocardial fibrosis underwent surgical treatment at our institute, which included biventricular, right ventricular, and left ventricular types. Forty-three patients had isolated right ventricular disease and underwent right ventricular endocardiectomy and tricuspid valve replacement with a low-profile tilting disc valve. The overall hospital mortality was 30% with 5 operative deaths and 8 postoperative deaths. Among the 30 survivors, 20 patients showed prosthetic valve dysfunction during follow-up. There were 8 late deaths, and 7 patients were lost to follow-up; all of these had documented prosthetic valve dysfunction during their initial follow-up. The postoperative functional improvement in an earlier report was attributed to correction of tricuspid regurgitation.5
Following endocardiectomy, the small ventricular cavity would not accommodate a tissue valve and hence we were forced to use a low-profile mechanical valve in all our patients. Mechanical valves in the tricuspid position are extremely difficult to manage and prone to thrombosis despite adequate anticoagulation.4 This was our experience and 65% of our patients showed prosthetic valve dysfunction during postoperative follow-up.6 As the left ventricle in this case showed early disease and these lesions are often bilateral, it was considered prudent to perform a less radical procedure than total cavopulmonary connection, thus a bidirectional Glenn shunt was chosen.7 Although good long-term palliation has been achieved by the classic Glenn shunt, it was decided to institute a pulsatile bidirectional cavopulmonary shunt maintaining prograde flow through the main pulmonary artery because of its advantages including prevention of late arteriovenous malformation in the lung.3,8 The stormy postoperative course in this patient could be attributed to postoperative ventricular dysfunction requiring inotropic support. The large pericardial cavity in the presence of elevated right-sided filling pressures can explain the pericardial collection and late tamponade.
A Fontan type of operation to bypass the severely diseased right ventricle seems attractive.4 In an earlier report, we described regular progression of the disease towards involvement of the previously unaffected ventricle.7 However, this patient showed no evidence of progression of the disease even after 8 years, and the right ventricle was able to cope with venous return from the inferior vena cava. If the disease process remains static, a bidirectional Glenn shunt may provide long-term palliation as partial diversion of systemic venous return to the pulmonary artery would reduce the volume load on the diseased right ventricle. In the event of progression of disease in the left ventricle, endocardiectomy and mitral valve replacement may be considered.
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REFERENCES
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- Tharakan JM, Venkitachalam CG, Balakrishnan KG. Angiographic features of endomyocardial fibrosis. In: Valiathan MS, Somers K, Kartha CC, editors. Endomyocardial fibrosis. New Delhi: Oxford University Press, 1993:169.
- Dubost C. The surgical treatment of constrictive fibrous endocarditis. J Cardiovasc Surg
1978;19:581–4.[Medline]
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1972;24:291–4.[Medline]
- Kumar N, Prabhakar G, Fawzy ME, Al Halees Z, Duran CMG. Total cavopulmonary connection for right ventricular endomyocardial fibrosis. Eur J Cardio-thoracic Surg
1992;6:391–2.[Abstract]
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1987;43:68–73.[Abstract]
- Valiathan S, Shyamkrishnan KG. Surgical treatment of endomyocardial fibrosis: Kerala experience. In: Valiathan MS, Somers K, Kartha CC, editors. Endomyocardial fibrosis. New Delhi: Oxford University Press, 1993:220–7.
- Gupta PN, Valiathan MS, Balakrishnan KG, Kartha CC, Ghosh MK. Clinical course of endomyocardial fibrosis. Br Heart J
1989;62:450–4.[Abstract/Free Full Text]
- Srivastava D, Preminger T, Lock JE, Mandell V, Keane JF, Mayer JE, et al. Hepatic venous blood and the development of pulmonary arteriovenous malformation in congenital heart disease. Circulation
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ABSTRACT
INTRODUCTION
