Eur J Cardiothorac Surg 2004;26:848-849
© 2004 Elsevier Science NL
Tricuspid valve repair in a case with congenital absence of left thoracic pericardium
Wolfgang A. Goetza*,
Andreas Lieboldb,
Ferdinand Vogta,
Dietrich E. Birnbauma
a Department of Cardiothoracic Surgery, University Hospital, 93042 Regensburg, Germany
b Department of Cardiothoracic Surgery, University of Rostock, Rostock, Germany
Received 11 April 2004;
received in revised form 17 May 2004;
accepted 7 June 2004.
* Corresponding author. Address: Klinikum der Universitaet Regensburg, Herz-, Thorax- und herznahe Gefaesschirurgie, Universität Regensburg, Franz-Josef-Strauss-Allee 11, 93042 Regensburg, Germany. Tel.: +49-941-944-9801; fax: +49-941-944-9802
e-mail: wolfgang.goetz{at}klinik.uni-regensburg.de
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Abstract
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We present a case of severe tricuspid valve insufficiency because of disruption of the anterior tricuspid leaflet with congenital absence of left thoracic pericardium. Findings suggest that tricuspid valve disruption was a result of distorted right ventricular geometry because of luxation of the heart into left thoracic cavity. Tricuspid valve could be repaired by reinsertion of anterior tricuspid leaflet and De-Vega annuloplasty. Normal hemodynamic was obtained and weaning from cardio pulmonary bypass was possible by lifting the heart in orthotopic position using increased positive end expiratory pressure. Postoperative course was uneventful.
Key Words: Tricuspid insufficiency • Absence of pericard • Tricuspid valve repair
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1. Introduction
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Absence of pericardium is a very rarely seen congenital defect. Predominantly pericardial defects appear partially on the left thoracic pleuropericardium [1–3].
Most patients with congenital absence of the pericardium are asymptomatic. Subsequent experience has shown that pericardial defects are rarely of clinical importance [2] and that persons with pericardial defects have the same life expectation as those of the general population [4]. There are only a few cases published where pericardial defects were associated with chronic chest pain and strangulation of the heart [2,3,5] or chordal rupture of the anterior tricuspid leaflet [6]. We report of a patient with congenital absence of left thoracic pericardium resulting in displacement of the heart and severe tricuspid valve insufficiency because of disruption of the anterior tricuspid leaflet.
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2. Case report
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A 44-year-old woman was admitted because of severe tricuspid regurgitation. She suffered since a year from increasing exertion dyspnoea (NYHA III). Patient had recent right heart decompensation with subsequent liver insufficiency. Biopsy of liver showed stasis induced cirrhosis. In ergospirometry maximal oxygen uptake was 10.1 ml/kg/min with unimpaired pulmonary function. Right and left heart catheter revealed a reduced cardiac output of 3.3 l/min (cardiac index 1.9) with normal left ventricular ejection (EF 73%) but severely dilated right atrium and ventricle with altered function and severe tricuspid insufficiency. Pulmonary pressure was at systole 16 mmHg and at diastole 2 mmHg, middle 7 mmHg. Additionally an atrial septal defect with a left right shunt of 0.3 l/min was found.
Transthoracic echocardiography showed severe tricuspid regurgitation with a jet filling more than half of the dilated right atrium. During diastole a flail of the anterior tricuspid leaflet into right ventricle was seen. Tricuspid annulus was dilated (48 mm) and both, the right atrium and ventricle were heavily dilated (RVEDD 85 mm).
Cardio-magnetic-resonance-imaging [7] showed a dilated right atrium and ventricle. The left sided pericardium was absent. The heart was rotated to the left to such an extent that the apex of the heart did touch the left dorsal thoracic wall (Fig. 1) . Right ventricular volume at end diastole was 411 ml at end systole 265 ml with stroke volume 145 ml and a tricuspid regurgitation of 95 ml (65%).
At operation the right atrium and ventricle were remarkely enlarged, the complete left sided pericardium was absent and the heart was lying entirely in the left chest with the apex touching the dorsal thoracic wall. After cardiopulmonary bypass had been installed the right atrium was opened and the tricuspid valve inspected. A widely dilated tricuspid annulus was found. The anterior tricuspid leaflet was completely disrupted at the line of insertion along on the tricuspid annulus. The other two leaflets and the papillary muscles revealed no pathology. The right ventricular anterior wall and the outflow tract were dilated. The disrupted rim on the tricuspid annulus was identified and the anterior tricuspid leaflet was reinserted to the tricuspid annulus with a running 4.0 prolene suture. After De-Vega annuloplasty [8] (31 mm diameter) with a double pledgeded 3.0 prolene suture, testing revealed a competent valve. Atrial septal defect was closed by a running 3.0 prolene suture. After reperfusion, weaning from cardio-pulmonary bypass was not possible because of luxation of the entire heart into the left thoracic cavity, resulting in a kinking of the base and insufficient diastolic filling of the heart. Increasing the positive end expiratory pressure from 3 to 10 mm H2O lifted the heart into orthotropic position which allowed uncompromised diastolic filling of the right ventricle and made weaning from cardio pulmonary bypass possible. Intraoperative and postoperative echocardiography revealed a competent tricuspid valve with normal function. Postoperative course was uneventful and heart function was unimpaired. The patient could be discharged eight days postoperatively. Three month after operation patient had no symptoms and was in NYHA functional class I.
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3. Discussion
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Many surgical procedures have been suggested for the management of symptomatic small or moderate pericardial defects with herniation of the heart, like primary closure, pleural flaps and pericardioplasty with patches of polytetrafluorethylene, Dacron or xeno-pericardium. But in large size defects it is recommended to extend the existing defect to prevent strangulation of the heart or its appendages through the partial pericardial defect [2,6]. There has been no evidence that life is shortened by absence of left sided pericardium neither there has been an evidence that repair of such a defect led to improvement of heart function. Therefore pericardioplasty is not recommended in complete absence of the left sided pericardium [1,3,6]. Only a few cases are known where absence of left pericardium was associated with chronic chest pain [2,3,5] or chordal rupture of the anterior tricuspid leaflet [6].
In this case, absence of left sided pericardium resulted in a displacement of the heart into left thoracic cavity and consequent stretching of the anterior right ventricular wall. Findings suggest that the structural changes caused abnormal strain and stress on the anterior tricuspid leaflet which resulted in a disruption of this leaflet from the annulus with subsequent tricuspid regurgitation and right ventricular failure. Tricuspid valve repair could restore normal tricuspid valve function. Initial weaning from cardio pulmonary bypass was not possible because of kinking of the base of the displaced heart and consecutive compromised diastolic filling. Increasing positive end expiratory pressure in order to lift the heart allowed normal diastolic filling and made weaning from cardio pulmonary bypass possible.
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References
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- De Vega N.G., De Rabago G., Castillon L., Moreno T., Azpitarte J. A new tricuspid repair. Short-term clinical results in 23 cases. J Cardiovasc Surg (Torino) 1973.
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Abstract
1. Introduction
