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Ann Thorac Surg 1999;68:1845-1848
© 1999 The Society of Thoracic Surgeons
a Cardiothoracic Surgical Unit, Green Lane Hospital, Auckland, New Zealand
b Department of Cardiology, Green Lane Hospital, Auckland, New Zealand
Address reprint requests to Dr Kerr, Cardiothoracic Surgical Unit, Green Lane Hospital, Green Lane West, Auckland 3, New Zealand;
e-mail: fnomura{at}kure-nh.go.jp
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Abstract |
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Introduction |
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The incidence of atrial arrhythmias reported after a classic Fontan (atriopulmonary connection) was from 4% to 35% at 5-year follow-up, and up to 40% at 10-year follow-up [1–3]. The modification to a total cavopulmonary connection (TCPC), which reduces the amount of atrial wall involved on the systemic pathway, was reported to reduce the incidence of postoperative atrial arrhythmias down to 14% at 5 years [3]. However, there is still a high incidence of atrial arrhythmias in the long term. Gandhi and associates [4] and Rodefeld and associates [5] have reported that in animal experiments, atrial flutter may occur in the presence of typical atrial suture lines, both for the classic Fontan and the TCPC, without high atrial pressure.
In this paper we present 2 patients with problems late (12 and 20 years) after a Fontan operation. Each patient had complications necessitating reoperation (1 with conduit obstruction, the other with giant right atrium and thrombus) and we required a procedure to both improve hemodynamics and also to improve control of their persistent arrhythmias.
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Case reports |
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With bicaval cannulae and normothermic cardiopulmonary bypass, the atriopulmonary connection was divided, and the atrium was opened along the previous suture line (Fig 1A). The right atrial thrombus was excised. With the heart still beating in atrial flutter, an incision was made commencing at the midpoint of the sulcus terminalis and extending vertically forward to the tricuspid annulus. During completion of this incision the heart reverted to sinus rhythm. Cryoablation was added from the medial end of this incision to the tricuspid. The inferior vena cava was detached from the right atrium and a wide section of atrial wall was excised in the region of the original right atriotomy. An atrial septectomy was performed. A 24-mm Gore-Tex (W.L. Gore & Assoc, Flagstaff, AZ) conduit was then anastomosed between the inferior vena cava and the right pulmonary artery. The superior vena cava was also connected to the superior aspect of right pulmonary artery. Three days postoperation, she continued in a slow nodal or sinus rhythm that needed atrial pacing.
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Patient 2
The second patient was a 28-year-old female with tricuspid atresia who had a modified Fontan operation using a homograft from the right atrium to the right ventricle at the age of 8. Her conduit was replaced because of calcification and stenosis at the age of 23. At 12 years after the initial operation, she developed intermittent rapid atrial flutter and recently she had suffered from atrial fibrillation. Various antiarrhythmic medications have been tried, including amiodarone, which resulted in impaired pulmonary function. Mechanical cardioversion has been necessary on several occasions. Multiple circuits of atrial flutter were observed by 24-hour electrocardiogram as well as electrophysiologic study. An echocardiogram showed mild conduit stenosis and incompetence with a giant right atrium. Her clinical status was NYHA II or III. Again, we believed an extracardiac Fontan with atrial pathway division would offer the best chance of arrhythmia control.
Under cardioplegic arrest and circulatory arrest, bilateral superior vena cavas were anastomosed to bilateral pulmonary arteries with each cardiac end closed (Fig 1B). The right atrium was opened along the previous incision and the old homograft (right atrium to right ventricle) was resected. An incision was made commencing at the midpoint of the sulcus terminalis and extending vertically forward to the tricuspid annulus. Cryoablation was added to complete this line of ablation as far as the tricuspid valve annulus. An atrial septectomy was done excising the floor of the fossa ovalis and unroofing the coronary sinus back towards the atrial septal defect. The inferior vena cava was detached from the right atrium. A wide section of atrial wall was excised in the region of original incision. A 24-mm Gore-Tex conduit was placed between inferior vena cava and right pulmonary artery as an extracardiac conduit.
On postoperative day 26, she developed a slow rhythm that required DDD pacemaker insertion. After 5 months, electrocardiogram shows that her basic rhythm is sinus rhythm with bradycardia (heart rate of 40 bpm) and occasional atrial fibrillation (heart rate of 110 bpm), which necessitates DDD pacing and digoxin. Atrial flutter was eliminated on a 24-hour electrocardiogram done at 4 months after operation. Now she is back to work with improved clinical status (NYHA I).
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Comment |
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A typical suspected flutter cycle can be shown in both post-Fontan and post-TCPC patients [4, 5] (Fig 2). Marcelletti’s extracardiac Fontan series [6] has shown only a 7.2% of incidence of atrial flutter at 5 years with excellent conduit patency. In addition, Danielson describes a right-sided maze procedure for right atrial arrhythmias in congenital heart disease effective in eliminating or reducing the incidence of atrial arrhythmias [7]. The extracardiac conduit revision with a limited division of atrial pathways (Fig 2) is a valid surgical option for classic Fontan patients requiring surgical revision and troubled by atrial flutter.
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This procedure did not appear to eliminate atrial fibrillation, although it became easier to control. Follow-up is only 9 and 6 months respectively, but there have been no episodes of atrial flutter or requirement for cardioversion and both patients are able to be paced atrially. We hope they have less chance of developing atrial flutter in the future. Both patients are improved clinically and back to school or work.
In conclusion, we have presented two successful case reports in which atrial flutter has been eliminated and clinical status has improved for patients with a classic Fontan operation performed 12 and 20 years previously. The operation involved converting them to an extracardiac conduit and performing a limited atrial pathway division with a combination of incision and cryoablation. Clearly the follow-up so far is short-term only and we have yet to know whether the early benefit is maintained in the long-term.
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Related Article
Ann. Thorac. Surg. 1999 68: 1848.
This article has been cited by other articles:
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  M. Koh, T. Yagihara, H. Uemura, K. Kagisaki, I. Hagino, T. Ishizaka, and S. Kitamura Optimal timing of the Fontan conversion: Change in the P-wave characteristics precedes the onset of atrial tachyarrhythmias in patients with atriopulmonary connection J. Thorac. Cardiovasc. Surg., May 1, 2007; 133(5): 1295 - 1302. [Abstract] [Full Text] [PDF]
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 S. P. Setty, K. Finucane, J. R. Skinner, and A. R. Kerr Extracardiac conduit with a limited maze procedure for the failing Fontan with atrial tachycardias Ann. Thorac. Surg., December 1, 2002; 74(6): 1992 - 1997. [Abstract] [Full Text] [PDF]
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