Atrial Septal Defect Coexisting With Constrictive Pericarditis-A Diagnostic Conundrum

10/1/13

Atrial Septal Defect Coexisting With Constrictive Pericarditis - A Diagnostic Conundrum - ISPUB

The Internet Journal of Thoracic and Cardiovascular Surgery ISSN: 1524-0274

Atrial Septal Defect Coexisting With Constrictive Pericarditis - A Diagnostic Conundrum Frank Edwin FWAC S National C ardiothoracic C entre (NC TC ), Korle Bu Teaching Hospital Accra Ghana Mark Tettey FWAC S National C ardiothoracic C entre (NC TC ), Korle Bu Teaching Hospital Accra Ghana Lawrence Sereboe FWAC S National C ardiothoracic C entre (NC TC ), Korle Bu Teaching Hospital Accra Ghana Ernest Aniteye FRC A National C ardiothoracic C entre (NC TC ), Korle Bu Teaching Hospital Accra Ghana Martin Tamatey FWAC S National C ardiothoracic C entre (NC TC ), Korle Bu Teaching Hospital Accra Ghana Kow Entsua-Mensah FWAC S National C ardiothoracic C entre (NC TC ), Korle Bu Teaching Hospital Accra Ghana David Kotei MD National C ardiothoracic C entre (NC TC ), Korle Bu Teaching Hospital Accra Ghana Kwabena Frimpong-Boateng FWAC S National C ardiothoracic C entre (NC TC ), Korle Bu Teaching Hospital Accra Ghana Citation: F. Edwin, M. Tettey, L. Sereboe, E. Aniteye, M. Tamatey, K. Entsua-Mensah, D. Kotei, K. Frimpong-Boateng: Atrial Septal Defect C oexisting With C onstrictive Pericarditis - A Diagnostic C onundrum. The Internet Journal of Thoracic and Cardiovascular Surgery. 2009 Volume 14 Number 1. DOI: 10.5580/2e6 Keywords: Atrial septal defect, Secundum, Diagnosis, C onstrictive Pericarditis, Echocardiography, Intraoperative.

Abstract The combination atrial septal defect and chronic constrictive pericarditis is very rare and poses diagnostic challenges because one tends to mask the clinical features of the other. We report such a case in which the initial diagnosis was suspected to be chronic constrictive pericarditis. This diagnosis was later changed to secundum atrial septal defect after transthoracic echocardiography. The complete diagnosis was made intra-operatively and the patient was relieved of both pathologies surgically.

Case Report A 15 year-old schoolboy was referred to our institution for specialist evaluation on account of anasarca and cough of 6 weeks’ duration. The main symptoms at presentation were progressive dypnea on effort, a dry cough and generalised body swelling. Physical examination revealed anasarca with engorged neck veins. The pulse was 110/minute, regular with fair volume with a blood pressure of 110/60mmHg. The jugular venous pressure was estimated to be 4cm above the sternal angle. The apex beat was neither visible nor palpable. The second heart sound was split (judged to be physiologic) and there was a grade II/VI ejection systolic murmur loudest at the upper left sternal edge. No pericardial rub was heard. The lung fields were clear to auscultation. Ascites was present and the liver was ballotable. Lower extremity edema was present up to mid-leg level. An abdominal ultrasound examination showed congestive hepatosplenomegaly and ascites. The liver function tests were essentially normal. C hest x-ray showed a cardiothoracic ratio of 0.55. No pericardial calcification was noted. C hronic constrictive pericarditis was suspected to be the cause of the patient’s symptoms. Decongestive therapy was begun with intravenous frusemide and spirinolactone with good effect. However, a transthoracic echocardiogram (TTE) showed dilated right–sided chambers and a secundum atrial septal defect (1.8cm) with a archive.ispub.com/journal/the-internet-journal-of-thoracic-and-cardiovascular-surgery/volume-14-number-1/atrial-septal-defect-coexisting-with-constrictive-pericarditis-a-diagnostic-conundrum.html#sthash.aaTmrk…

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Atrial Septal Defect Coexisting With Constrictive Pericarditis - A Diagnostic Conundrum - ISPUB

left-to-right shunt. The initial working diagnosis was therefore changed according ly and the patient was subsequently referred to the surgeon as a case of congestive cardiac failure secondary to atrial septal defect (ASD). The patient remained on oral medication while preparing to undergo ASD closure. There was a relapse of congestive symptoms after a year in-spite of oral decongestive treatment. A repeat TTE was performed but no ASD was seen. A transesophageal echocardiogram (TEE) was later performed which demonstrated a secundum ASD (Figure 1) but no colour flow could be detected across the defect.

Figure 1. Pre-operative TEE showing the ASD.

A right heart catheterization was done and showed a mean central venous pressure of 15mmHg, mean right ventricular pressure of 22mmHg (33/7mmHg), mean pulmonary artery pressure of 23mmHg (32/16mmHg) and pulmonary capillary wedge pressure of 17mmHg. The mixed venous oxygen tension was 45.6mmHg with a mixed venous oxygen saturation of 82.4% on room air. There was no change in the oxygen saturation after inhalation of 100% oxygen. The features did not clearly confirm the presence of an ASD. Nevertheless, a decision was taken to proceed with surgery with the diagnosis of secundum ASD based on clinical grounds and the TEE findings. After performing a median sternotomy, the pericardium was found to be grossly thickened and densely adherent to the myocardium with areas of calcification. A formal pericardiectomy was performed without cardiopulmonary bypass. After pericardial decortication of both leftand right-sided chambers, progressive right-sided chamber distension was noted. An intra-operative TEE was performed to evaluate the archive.ispub.com/journal/the-internet-journal-of-thoracic-and-cardiovascular-surgery/volume-14-number-1/atrial-septal-defect-coexisting-with-constrictive-pericarditis-a-diagnostic-conundrum.html#sthash.aaTmrk…

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interatrial septum. This clearly demonstrated a secundum ASD with colour flow indicating a left-to-right shunt at atrial level. Aorto-bicaval cannulation was performed and cold crystalloid antegrade cardioplegia was admistered through the root of the cross-clamped ascending aorta to achieve cardiac arrest. Total cardiopulmonary bypass with a membrane oxygenator was established. The ASD (2.5cm x 1.5cm) was closed primarily through a right atriotomy. Reperfusion and weaning from bypass was accomplished uneventfully. The post-operative course proceeded smoothly with marked hemodynamic improvement. The patient was discharged home on the 10th post-operative day and remains asymptomatic after 23 months’ follow-up. Histology of the excised pericardium showed a thickened pericardium with extensive fibrosis and infiltration by chronic inflammatory cells extending into the adjacent fatty tissue. No evidence of tuberculosis or specific inflammation was found.

Discussion The tortuous route to complete diagnosis in this patient highlights William Osler’s observation in 1892 - “Probably no serious disease is so frequently overlooked by the practitioner. Post-mortem experience shows how often pericarditis is not recognized or goes on to resolution and adhesion without attracting notice”. The combination of ASD and chronic constrictive pericarditis (C P) is very rare. Diagnosis of this clinical entity is compounded by the fact that the features of the ASD may be masked by those of C P. Tanaka et al1 report a case of recurrent C P and ASD in which the ASD was missed at the time of the first pericardiectomy. In our patient, although the initial working diagnosis was C P, the possibility of coexistence with ASD was not considered even after the index echocardiography. It is important to make the diagnosis of C P because surgical treatment is often curative. C onversely, without treatment, there is progressive hemodynamic and physical deterioration with a reduced life expectancy. No single investigative tool suffices to make a foolproof diagnosis of C P, the anatomical hallmark of which is pericardial thickening. The presence of pericardial thickening on imaging studies does not necessarily indicate constrictive physiology. C onversely, patients with C P may not have pericardial thickening on imaging studies or even histologically following pericardiectomy2. Non-invasive imaging modalities such as chest x-ray examination, echocardiography, computerized tomography (C T) scans and magnetic resonance imaging (MRI) are often used alone or in combination to demonstrate pericardial thickening in C P. C ardiac catheterization may confirm a suspicion of constrictive physiology but it is invasive and not often used unless the diagnosis remains in doubt after non-invasive methods are exhausted. As in the case reported, echocardiography does not reliably detect pericardial thickening but it has gained increased usefulness in the evaluation of C P based on information provided by Hatle3 and colleagues who reported dynamic changes with respiration occurring in patients with C P. These changes - the dissociation of intrathoracic and intracardiac pressures as well as enhanced ventricular interaction, can be demonstrated echocardiographically and increases the diagnostic yield in cases of C P. As challenging as the diagnosis of C P is when it exists alone, coexistence with an ASD can prove clinically misleading; sometimes, a complete diagnosis is made only at autopsy4-6. In our patient, the clinical features were arguably dominated by those of C P but this was overlooked when echocardiography failed to demonstrate pericardial thickening and suggested a diagnosis that was thought to account for the clinical features of right-sided heart failure. There was no credible explanation for a supposedly large ASD without Doppler evidence shunting across the defect. A complete catheterization was not done in this patient due to equipment problems but the right heart catheterization was not suggestive of a left-to-right atrial level shunt. In retrospect, we postulated that this was so because of possible equalization of left and right atrial pressures imposed by the constrictive physiology; intra-operative TEE showed a large left-to-right shunt archive.ispub.com/journal/the-internet-journal-of-thoracic-and-cardiovascular-surgery/volume-14-number-1/atrial-septal-defect-coexisting-with-constrictive-pericarditis-a-diagnostic-conundrum.html#sthash.aaTmrk…

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across the ASD after surgical relief of the constriction. The cause of this unusual combination of lesions, if indeed there is a common etiological factor, remains unknown7. It is probable that the coexistence of these lesions is purely coincidental.

Conclusion It is extremely important to recognize this association of lesions during life because both are amenable to surgical correction. The diagnosis of C P requires a high index of suspicion and a multi-modality approach to clinical investigation. Echocardiographic evaluation of C P must not be limited to assessment of pericardial thickening but should include assessment of dynamic changes in cardiac physiology with respiration. C linicians should bear in mind that the coexistence of C P and ASD may give rise to no demonstrable shunt echocardiographically across the ASD.

References 1. Tanaka K, Murota Y, Andoh T, Asano K. A case of recurrent constrictive pericarditis complicated with atrial septal defect. Nippon Kyobu Geka Gakkai Zasshi. 1995;43(8):1195-1197. 2. Talreja DR, Edwards WD, Danielson GK, Schaff HV, Tajik AJ, Tazelaar HD, Breen JF, and Oh JK. C onstrictive Pericarditis in 26 Patients With Histologically Normal Pericardial Thickness. C irculation 2003; 108(15): 1852-1857. 3. Hatle LK, Appleton C P, Popp RL. Differentiation of constrictive pericarditis and restrictive cardiomyopathy by Doppler echocardiography. C irculation 1989;79:357–370. 4. Kumar S, Szatkowski J, Thomas W. Atrial septal defect complicated by constrictive pericarditis. C hest. 1971; 60(1):101-105. 5. Kouvaras G, Goudevenos J, C hronopoulos G, Sofronas G, Reed P, Adams P, C okkinos DV. Atrial septal defect and constrictive pericarditis. An unusual combination. Acta C ardiol. 1989;44(4):341-349. 6. Tanoue Y, Tomita Y, Kajiwara T, Tominaga R. C onstrictive pericarditis with atrial septal defect. Ann Thorac C ardiovasc Surg. 2006; 12(5):373-375. Generated at: Mon, 30 Sep 2013 18:07:14 -0500 (000002e6) — http://archive.ispub.com:80/journal/the-internet-journal-of-thoracic-andcardiovascular-surgery/volume-14-number-1/atrial-septal-defect-coexisting-with-constrictive-pericarditis-a-diagnostic-conundrum.html

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