Asian Cardiovasc Thorac Ann 2007;15:435-437
© 2007 Asia Publishing EXchange Ltd
Atrial Septal Defect with Ascites and Pleural Effusion
Hee-Hwa Ho, MRCP,
Elaine Chau, FRCP,
Alex Chiu, MRCP,
On-Hing Kwok, FRCP
Division of Cardiology, University Department of Medicine, Grantham Hospital, Hong Kong, China
For reprint information contact: On-Hing Kwok, FRCP, Tel: 852 2835 7899, Fax: 852 2892 7517, Email: vohkwok{at}hksh.com, Cardiac Catheterization & Intervention Center, Cardiology Center, 7/F Central block, Hong Kong Sanatorium & Hospital, 2 Village Road, Happy Valley, Hong Kong, China.
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ABSTRACT
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A case of a lady referred for repair of an atrial septal defect is described. She presented with an insidious onset of recurrent ascites and pleural effusion. Cardiac catheterization showed constrictive physiology. The patient subsequently underwent surgical closure of the atrial septal defect and pericardiectomy
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INTRODUCTION
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We report a case of which a young lady with atrial septal defect presented with recurrent pleural effusion and ascites. It is a rather unusual presentation for atrial septal defect per se. Only after extensive investigations and repeated cardiac catheterisation, a concomitant diagnosis of constrictive pericarditis was made. The article will discuss about how we arrived at the final diagnoses and the result after surgery.
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CASE REPORT
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A 38-year-old lady who previously enjoyed good health, presented to a community hospital with insidious onset of exertional dyspnea and abdominal distension. On physical examination, the jugular venous pressure was elevated and the abdomen was distended with hepatomegaly and ascites. The electrocardiogram showed sinus rhythm with partial right bundle branch block. Chest X-Ray revealed mild cardiomegaly and right pleural effusion. Apparently, the initial focus of the physicians was to rule out liver pathology and autoimmune disease, but the investigations did not suggest either diagnosis. The patient subsequently underwent exhaustive investigations to unravel the cause of her pleural effusion, which included pleurocentesis, pleural biopsy, bronchoscopy, and cardiothoracic (CT) scan. Again, there were no positive findings.
Transthoracic echocardiogram showed normal left ventricular function with the suspicion of a secundum atrial septal defect. The right atrium and ventricle were mildly dilated with mild tricuspid regurgitation. The right ventricular systolic pressure was 37 mm Hg. Transesophageal echocardiogram confirmed the diagnosis of a large secundum atrial septal defect. The maximum diameter across the atrial septal defect was 3 cm. After exhaustive investigations, it was decided that the atrial septal defect was the cause of the symptoms and the patient underwent cardiac catheterization. Right and left heart catheterization further confirmed the presence of the atrial septal defect with left-to-right shunt ratio of 2.46. The patient was then referred to us for consideration of surgical closure of the defect.
Hemodynamic data of the cardiac catheterization was critically reviewed. It was noted that both right and left atrial pressures, right and left ventricular end diastolic pressures were all equally elevated up to 28 mm Hg (Figure 1
). On further examination of the atrial tracing (Figures 1A & B), blunted x and exaggerated y descent (‘M’ pattern) was noted. The ventricular diastolic filling followed a "dip and plateau" pattern. Transthoracic echocardiogram was repeated. There was, however, very subtle respiratory discordance. The fall in mitral inflow velocity and the increase in tricuspid inflow velocity during inspiration was less than 25% compared with those during expiration. Cardiac catheterization was repeated. Simultaneous right and left heart catheterization showed equalization and elevation of the right and left ventricular end diastolic pressure with the presence of the "dip and plateau" sign (Figures 1C, D & E). On peak inspiration, the left ventricular pressure decreased to the nadir but there was no significant increase in the right ventricular pressure (Figure 2). Presumably, the respiratory discordance might have been blunted by the large left-to-right shunt through the atrial septal defect. Temporary occlusion of the septal defect with a balloon might have been able to demonstrate the constrictive physiology with typical respiratory discordance. Unfortunately, an appropriate balloon was not available at that juncture. Subsequent magnetic resonance imaging (MRI) of the heart, however, showed normal thickness pericardium and no pericardial calcification.
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Figure 1. Hemodynamic tracings. A. Right atrial pressure tracing, showing elevated filling pressure with "M pattern". B. Left atrial pressure tracing, showing elevated filling pressure with "M pattern". C. Right ventricular pressure tracing, showing elevated diastolic filling pressure with "dip and plateau" pattern. D. Left ventricular pressure tracing, showing elevated diastolic filling pressure with "dip and plateau" pattern. E. Simultaneous left ventricular and right ventricular pressure recording, showing equalization of end-diastolic pressures.
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Figure 2. Simultaneous right and left ventricular pressure recording during the respiratory cycle. An exaggerated increase in left ventricular pressure during expiration was noted. Respiratory variation of right ventricular pressure appeared "blunted". Subtle respiratory discordance was evident. RA = Right atrium, LA = Left atrium, RV = Right ventricle, LV = Left ventricle.
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The patient underwent surgical closure of the atrial septal defect and exploration of the pericardium. During the operation, both the parietal and visceral pericardium were found to be thickened and densely adherent to each other. A pericardiectomy was performed and the atrial septal defect closed. Histological examination of the pericardium showed densely fibrotic tissue with capillary ingrowth. There was no granuloma or inflammatory infiltrate. The patient made an uneventful recovery and remained symptom-free for over 2 years.
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DISCUSSION
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The co-existence of atrial septal defect and constrictive pericarditis is rather uncommon and creates a diagnostic challenge.1 Recurrent pleural effusion and ascites in a young adult is an unusual presentation of atrial septal defect per se. A high index of suspicion is mandatory in making the complete diagnosis. Meticulous hemodynamic study during cardiac catheterization is indispensable in this scenario.
The hemodynamic data can be difficult to interpret in this situation. Examination of dynamic respiratory changes, which demonstrates exaggerated ventricular interdependence, is quite specific in demonstrating the presence of constrictive physiology.2,3,4 The diastolic filling pattern and partial respiratory discordance observed in our patient was suggestive of, but not conclusive for, constrictive pericarditis. Perhaps, temporary occlusion of the atrial septal defect with a balloon might have assisted in unmasking the dynamic respiratory variation.
Calcified pericardium on lateral chest X-Ray occurs only in less than half of the patients with chronic constrictive pericarditis.5–7 Tuberculosis used to be the leading cause of constrictive pericarditis. Nowadays, the more common etiologies are idiopathic (probably viral in origin) and post-irradiation therapy.5–7 Echocardiographic examination often shows pericardial thickening but cannot definitely exclude the diagnosis. Magnetic resonance imaging is more precise in elucidating the presence of thickened pericardium. Pericardial thickening and pericardial calcification, however, are not synonymous with constrictive pericarditis since they may be present without impairing ventricular filling. In general, normal pericardial thickness on MRI has a strong negative predictive value. Nevertheless, there are exceptions to the rule. This is the second case we have seen of constrictive pericarditis with normal thickness pericardium on MRI or Ultra-fast CT scan. Both patients were young adults suffering from disabling symptoms and frequent hospital admissions. Both patients recovered completely after pericardiectomy. Intraoperatively the parietal and visceral pericardia were found densely "glued" to each other. This form of non-calcific constrictive pericarditis has been described by Hancock and is characterized by an adherent fluid-fibrin layer in the process of organization rather than a rigid, scarred shell.8
Differentiation from restrictive cardiomyopathy at the bedside and even after cardiac catheterization may be difficult, but the distinction is important as constrictive pericarditis is curable. Meticulous study of the hemodynamic pattern during respiratory cycle, fluid challenge or exercise may help the differentiation. Sometimes, endomyocardial biopsy may be necessary if the diagnosis is still uncertain.
Given such a clinical scenario, careful search for clues of constrictive pericarditis is probably warranted and may disclose a curable or remediable heart disease.
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REFERENCES
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- Kouvaras G, Goudevenos J, Chronopoulos G, Sofronas G, Reed P, Adams P, et al. Atrial septal defect and constrictive pericarditis. An unusual combination. Acta Cardiol 1989;44:341–9.[Medline]
- Hurrell DG, Nishimura RA, Higano ST, Appleton CP, Danielson GK, Holmes DR Jr, et al. Value of dynamic respiratory changes in left and right ventricular pressures for the diagnosis of constrictive pericarditis. Circulation 1996;93:2007–13.[Abstract/Free Full Text]
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- Azrak E, Kern MJ. Hemodynamic rounds series II:evaluating new hemodynamic criteria of constrictive physiology: respiratory dynamics. Cathet Cardiovasc Interv 1999;47:80–4.[Medline]
- Spodick, DH. The normal and diseased pericardium:current concepts of pericardial physiology, diagnosis and treatment. J Am Coll Cardiol 1983;1:240–51.[Medline]
- Ling LH, Oh JK, Breen JF, Schaff HV, Danielson GK, Mahoney DW, et al. Calcific constrictive pericarditis: is it still with us? Ann Intern Med 2000;132:444–50.[Abstract/Free Full Text]
- Donald S. Baim, William Grossman. Grossman’s Cardiac Catheterization, Angiography, and Intervention. In: Beverly H. Lorell, William Grossman, editors. Profiles in Constrictive pericarditis, restrictive cardiomyopathy, and cardiac tamponade. 6th Edition. P829–51.
- Hancock EW. On the elastic and rigid forms of constrictive pericarditis. Am Heart J 1980;100(6 Pt 1):917–23.[Medline]
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ABSTRACT
INTRODUCTION
