Ann Thorac Surg 2007;84:666-668
© 2007 The Society of Thoracic Surgeons
Case Reports
Conversion to Total Cavopulmonary Connection After Failed One and One-Half Ventricular Repair
Hideki Uemura, MD, FRCSa,
Toshikatsu Yagihara, MDb,*,
Iki Adachi, MDa,
Koji Kagisaki, MDb,
Fumiaki Shikata, MDb
a Department of Cardiothoracic Surgery, Royal Brompton Hospital, London, United Kingdom
b Department of Cardiovascular Surgery, National Cardiovascular Center, Osaka, Japan
Accepted for publication February 23, 2007.
* Address correspondence to Dr Yagihara, National Cardiovascular Center, Japan, 5-7-1 Fujishirodai, Suita, Osaka, 565-8565, Japan (Email: yagihara{at}hsp.ncvc.go.jp).
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Abstract
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In 3 patients previously undergoing one and one-half ventricular repair, right ventricular dysfunction progressed for more than 10 years. Their clinical features resembled those seen in patients undergoing the atriopulmonary Fontan procedure, and reoperation was carried out for conversion to total cavopulmonary connection. Hemodynamics improved subsequent to the circulatory renewal. In 2 patients having atrial arrhythmia before conversion, the resected right atrial wall illustrated grossly abnormal histopathology. These patients suffered from persistent sinus nodal dysfunction and eventually needed pacemaker implantation. The third patient died of sepsis 4 months later.
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Introduction
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The so-called one and one-half ventricular repair (1.5 VR) is used when the pulmonary ventricle seems to be insufficient for usual biventricular physiology. Creating a cavopulmonary anastomosis, blood draining through the superior caval vein is forwarded directly into the pulmonary artery with inferior caval venous flow ejected through the pulmonary ventricle. This physiology remains a good alternative to total cavopulmonary connection (TCPC), but it could provide a less than ideal outcome in the long term [1]. In 3 of 14 patients who previously underwent this eclectic definitive repair, such a downside required reoperation for conversion to TCPC; clinical features before reoperation being quite similar to those in patients undergoing conversion from atriopulmonary connection to TCPC [2, 3].
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Case Reports
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The 3 patients underwent 1.5 VR between 1988 and 1990 (Table 1). The cavopulmonary anastomosis was achieved in a conventional Glenn fashion (2 patients) or in a bidirectional fashion (1 patient). The atrial septal defect was entirely closed. The right atrium became markedly dilatated over 10 years. Exercise tolerance gradually got worse. In 2 patients, atrial arrhythmia was progressive and symptomatic. In the other, a serous albumin level stayed below the normal range with sporadic edema. Because of these clinical features, conversion to TCPC was used.
The renewal was established by extracardiac grafting using a 20-mm expanded polytetrafluoroethylene tube. The pulmonary trunk was divided. The atrial septum was removed. The right atrium was extensively reduced in size and cryoablated to minimize possible circuits for electric re-entry. The pulmonary artery (PA) previously divided in patient 1 was made confluent with a space being provided behind the ascending aorta by plicating the aortic wall to accommodate a 16-mm ringed expanded polytetrafluoroethylene tube. In patient 2, the inferior caval venous channel was forwarded exclusively to the left lung. The aortic valve was concomitantly repaired in patients 1 and 3 because moderate regurgitation had been noted. In all patients, warfarin was given orally for 1 year after surgery according to our routine management.
Patients 1 and 2
Patients 1 and 2, both with preoperative atrial arrhythmia, eventually underwent permanent pacemaker implantation 1 month later (Table 1). Patient 2 needed another reoperation 1 year after TCPC conversion for occlusion of the left PA. As used in patient 1, the PA was made confluent using a ringed expanded polytetrafluoroethylene tube with the ascending aorta replaced using a 24-mm prosthesis to allow more space behind for the PA reconstructed. Catheterization 1 month after this revision demonstrated the PA in good shape. However, the left PA was found thrombosed again 1 year later. This patient has been followed regularly with the unilateral lung Fontan circulation.
Histological sections of the resected atrial wall and septum illustrated remarkable abnormalities (Table 1, Fig 1).
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Fig 1. Histologic sections in patient 1 showed grossly abnormal architecture of the right atrial wall. (a) Interstitial fibrosis and endocardial thickening were obvious. (Masson’s trichrome stain; x20). (b) Myocytes possessed abnormalities including hypertrophy, vacuolation or peri-nuclear halo, and degeneration of myofibril. (Hematoxylin-eosin stain; x200).
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Patient 3
In patient 3, a serous albumin level improved from a preoperative value of 2.6 g/dL to postoperative 4.6 g/dL without supplement. This patient was discharged home and was doing well, but died 4 months after TCPC conversion at a local hospital because of sepsis.
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Comment
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It was suggested that 1.5 VR could contain broad spectrum in terms of right ventricular size or function [4]. Our indication for 1.5 VR was an alternative to the Fontan procedure, rather than backing off from biventricular repair [1]. The hypoplastic right ventricle did not grow, but it even regressed in its configuration subsequent to 1.5 VR establishment. It failed to function efficiently enough in the long term as a chamber for pumping blood returning through the inferior caval vein. It was interesting to find that the right atrium can be over-dilated even with half of the systemic venous flow.
Indication criteria for conversion from the 1.5 VR physiology to the Fontan circulation certainly remain contentious. We regarded right atrial pressure being higher than that of the superior caval vein as one sensible reason. This situation indicates that the hypoplastic right ventricle is not working ideally, and the cavity can be excluded without worsening congestion of the lower body organs. There is little possibility of a raise in PA pressure after the conversion because the total amount of the pulmonary perfusion does not change. Another factor favoring conversion is atrial arrhythmia. Although a permanent pacemaker could be needed in a high incidence [3], stable cardiac rhythm improved functional status considerably [2]. This was also the case in the present series.
Not surprisingly, histologic changes in the atrial wall were raised as a crucial background for manifestation of atrial arrhythmia [5]. Our current microscopic findings did not discord with this opinion. Need for pacemaker implantation after TCPC conversion may partly reflect such irreversible histopathologic changes.
As for the operative procedure, we followed our previous policy for TCPC conversion including reduction of the right atrial size [2]. We maintain the policy of concomitant anti-arrhythmic maneuver so as to settle arrhythmic problems as much as possible. In addition, we now also pay attention to the method for reconstructing the PA once divided for the conventional Glenn procedure.
In conclusion, a failing right heart after 1.5 VR can be treated by conversion to TCPC for better hemodynamic and functional status. Concomitant pacemaker implantation will be among the surgical choices for further rhythm disturbance even after a successful conversion. Considerable pathologic changes presented in the atrial wall were derived from patients with atrial arrhythmia.
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Acknowledgments
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We express a special thanks to Dr Hatsue Ishibashi-Ueda, Department of Pathology at the National Cardiovascular Center, for his histologic investigations.
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References
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- Numata S, Uemura H, Yagihara T, Kagisaki K, Takahashi M, Ohuchi H. Long-term functional results of the one and one half ventricular repair for the spectrum of patients with pulmonary atresia/stenosis with intact ventricular septum Eur J Cardiothoracic Surg 2003;24:516-520.[Abstract/Free Full Text]
- Kawahira Y, Uemura H, Yagihara T, Yoshikawa Y, Kitamura S. Renewal of the Fontan circulation with concomitant surgical intervention for atrial arrhythmia Ann Thorac Surg 2001;71:919-921.[Abstract/Free Full Text]
- Mavroudis C, Backer CL, Deal BJ, Johnsrude CL. Fontan conversion to cavopulmonary connection and arrhythmia circuit cryoablation J Thorac Cardiovasc Surg 1999;115:547-556.
- Hanley FL. The one and a half ventricular repair—we can do it, but should we do it? J Thorac Cardiovasc Surg 1999;117:659-664.[Free Full Text]
- Becker AE. How structurally normal are human atria in patients with atrial fibrillation? Heart Rhythm 2004;1:627-631.[Medline]
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Abstract
Introduction
