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Ann Thorac Surg 2004;77:1334-1340
© 2004 The Society of Thoracic Surgeons
a PediatricsSection of Pediatric Cardiology, Baylor College of Medicine, Texas Children's Hospital, Houston, Texas, USA
b SurgeryDivision of Congenital Heart Surgery, Baylor College of Medicine, Texas Children's Hospital, Houston, Texas, USA
c AnesthesiologySection of Pediatric Cardiovascular Anesthesia, Baylor College of Medicine, Texas Children's Hospital, Houston, Texas, USA
Accepted for publication October 2, 2003.
* Address reprint requests to Dr Mott, Section of Pediatric Cardiology, Texas Children's Hospital, 6621 Fannin, MC# 19345C, Houston, TX 77030, USA
e-mail: amott{at}bcm.tmc.edu
| Abstract |
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METHODS: Between July 1995 and April 2003, 23 patients (>18 years old) had Fontan operations. We retrospectively reviewed their perioperative courses.
RESULTS: Twenty-three Fontan operations (first-time [n = 8] and redo [n = 15]) were performed with no early or late deaths. No patient has required reoperation. One patient has been listed for orthotopic heart transplantation. The overall mean age is 23 years (18 to 41 years); mean follow-up, 30 months; median postoperative hospital stay, 8 days (4 to 34 days); and median duration of chest tube drainage, 4 days (2 to 12 days). The postoperative New York Heart Association (NYHA) functional class was improved in 22 of 23 patients. Eight first-time Fontan operations (7 of 8 nonfenestrated) were performed; lateral tunnel (n = 7) and extracardiac conduit (n = 1). Two patients had preoperative arrhythmias. New onset arrhythmias (ventricular tachycardia and sinus node dysfunction), requiring treatment, occurred in two patients. Fifteen redo-Fontan operations (all nonfenestrated) were performed; lateral tunnel (n = 5) and extracardiac conduit (n = 10). Fifteen patients had preoperative arrhythmias, thirteen of which had intraatrial reentry tachycardia (IART) and required antiarrhythmic medications. Concomitant intraoperative radiofrequency ablation (RFA) (n = 11) and cryoablation (n = 1) procedures were performed. In the immediate postoperative period, there was IART recurrence in five patients (post-RFA [n = 4] and postcryoablation [n = 1]). At latest follow-up, no patient is being treated with antiarrhythmic medications. Two patients had new onset atrial arrhythmias that required treatment.
CONCLUSIONS: The Fontan operation can be performed in adults with minimal morbidity and improved NYHA functional class. New onset arrhythmias requiring treatment are sources of perioperative morbidity. Complete arrhythmia resolution of the preoperative arrhythmia may not be achieved in the immediate postoperative period in redo-Fontan patients. However, modification (intraoperative radiofrequency ablation-right atrial debulking) of the atrial tachycardia circuits in the redo-Fontan patients can result in complete resolution of preoperative atrial tachyarrhythmias at early follow-up.
| Introduction |
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Since the mid to late 1990s, the reported operative mortality risk after the Fontan operation has decreased to less than 5% [36]. Several management strategies have been incorporated in order to achieve the improved mortality: universal risk factors or "guidelines" that have resulted in better patient selection; a "staged" approach to the Fontan operation with an interim superior cavopulmonary connection; and modification of the surgical technique to utilize a lateral atrial tunnel with a fenestration. However, prolonged pleural effusions and arrhythmias continue to be sources of postoperative morbidity and prolonged hospital stays [79].
Unlike the management of children with a functional single ventricle, the management guidelines have yet to be established for the adult who is a candidate for the Fontan operation. Adults with functional single ventricle usually present the following: (1) as having a "failed" Fontan (chronic arrhythmias, protein losing enteropathy, pleural effusions, ventricular dysfunction, limited exercise capacity); (2) after long-term surgical palliation; or (3) as uncorrected. The purpose of this report is to describe the clinical presentation and early perioperative course in adults who have undergone modifications of the Fontan operation at our institution.
| Material and methods |
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Data collected included demographic data (age at surgery, cardiac diagnosis, surgical date, and cardiac operation). Preoperative echocardiographic and catheterization data were reviewed. Other data included total hospital length of stay, postoperative hospital length of stay, total days in the cardiac intensive care unit, duration of mechanical ventilation, and outcome. Overall mortality was defined as death occurring from the time of surgery to the most recent follow-up. Early postoperative death was defined as that occurring in hospital or less than 30 days following surgery.
Complications reviewed included postoperative issues such as "prolonged" pleural effusions (in-dwelling pleural catheter >7 days or readmission for pleural effusions), pericardial effusions, significant arrhythmias (arrhythmias requiring treatment), neurologic sequelae, postoperative bleeding requiring mediastinal-thoracic exploration, and thromboembolic events.
| Results |
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First-time Fontan operation
Eight patients underwent first-time operations: lateral tunnel (n = 7) and extracardiac conduit (n = 1). The indications for surgery included cyanosis (n = 8), decreased exercise tolerance (n = 8), and intractable tachyarrhythmia (n = 1). One patient had a fenestration of the lateral tunnel and this patient also sustained a cerebral vascular accident with a left hemiparesis, which is resolving. The mean cardiopulmonary bypass time was 221 (± 92) minutes, mean age was 23 years (18 to 41 years), and mean follow-up is 43 months.
The underlying cardiac diagnoses were L-transposition of the great arteries-straddling atrioventricular valve (n = 2), double inlet left ventricle-transposition of the great arteries-pulmonary valve stenosis (n = 2), complete atrioventricular canal defect right ventricular dominant (n = 2), tricuspid atresia-pulmonary atresia (n = 1), and double outlet right ventricle-transposed great arteries (n = 1). Prior operations included: aortopulmonary shunt (n = 6), pulmonary artery band (n = 4), and atrial septectomy (n = 1).
Ten secondary operations were performed, which included pulmonary arterioplasty (n = 5), atrial septectomy (n = 2), aortovalvuloplasty (n = 1), and tricuspid valvuloplasty/annuloplasty (n = 2). Other details of the preoperative and perioperative courses are described in Table 1.
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ICU = intensive care unit..
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The underlying cardiac diagnoses were tricuspid atresia with or without normally related great arteries (n = 8), double inlet left ventricle/transposed great arteries (TGA) (n = 2), tetralogy of Fallot-mitral valve atresia (n = 1), heterotaxy with complete atrioventricular canal defect (n = 1), ventricular inversion with L-TGA (n = 1), double outlet right ventricle-mitral valve atresia (n = 1), and pulmonary atresia-intact ventricular septum (n = 1).
Each patient presented with decreased exercise tolerance. Two patients had undergone evaluation for OHT before the redo-Fontan operation. In addition to their first Fontan modification, eight patients had undergone previous placement of an aortopulmonary shunt and atrial septectomy (n = 2).
At the time of surgery, there were 27 secondary operative procedures performed, which included epicardial pacemaker placement (n = 13), right atrial debulking in patients with previous atriopulmonary Fontans (n = 12), radiofrequency ablation (n = 11), and implantable cardioverter defibrillator placement (n = 1). Other details of the preoperative and perioperative courses are described in Table 3.
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Two patients were diagnosed with new onset atrial tachycardia in the early postoperative period; each is well controlled with ß-blocker therapy. A summary of the perioperative arrhythmias and treatment is in Table 2. Each patient had improved NYHA functional classes. Figure 2 describes the preoperative and postoperative NYHA functional class.
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| Comment |
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In this study, we report the early operative results in 23 adult patients who have undergone the Fontan operation at our institution. Our overall survival at early follow-up compares favorably with reported studies [6, 13, 15, 16]. An improved postoperative NYHA functional class was achieved in the majority of patients despite some having decreased cardiac indices and elevated ventricular end-diastolic pressures. In those who underwent first-time Fontan operation, improved functional class is possibly secondary to the decreased ventricular volume load. In the redo-Fontan patients, we theorize that obviating coronary sinus hypertension by allowing it to drain into the lower pressure pulmonary venous atrium allows for improved myocardial oxygen delivery and myocardial systolic function.
Adult patients present for the Fontan operation with very unique anatomic and hemodynamic profiles such that the preoperative, intraoperative, and perioperative management strategies must be individualized. Some patients in our population presented with more favorable anatomic and hemodynamic profiles. Still others presented as clinical dilemmas, without any clear-cut favorable anatomic and hemodynamic characteristics or a clear-cut operative strategy. Orthotopic heart transplantation is often considered the best option for this group of patients. Because of this heterogeneity, the proper and decisive timing for operative intervention must be determined, albeit in most cases, nonelective.
Certain aspects of our operative and postoperative strategies warrant discussion. In our series, the Fontan with an extracardiac conduit is used more commonly in the redo-operations, but not exclusively. A Fontan with a lateral tunnel is preferred and is performed in the first-time Fontan operation if the anatomy is favorable. However, in the presence of an anatomic substrate that includes complex systemic or pulmonary venous anatomy, a Fontan with an extracardiac conduit is performed.
The Fontan fenestration was rarely used in our cohort of adults. Fontan fenestration has been shown to decrease early perioperative morbidity in the form of prolonged pleural effusions and increased hospital stay, although the long-term advantages are not known [17]. One patient with preoperative moderate atrioventricular valve regurgitation, moderate ventricular systolic dysfunction, and increased pulmonary vascular resistance had a Fontan modification that incorporated a fenestration. We incorporate the Fontan fenestration in selected younger patients but have tried to avoid the fenestration in adults, secondary to the deleterious effects of acute cyanosis. Also, some of our patients have had previous neurologic insults.
The median durations of chest tube drainage and postoperative hospital stay in our series were low, which compares favorably with adult series in which a Fontan fenestration was not used [6, 13] and with those studies in which a fenestration was used more liberally [10, 14, 15].
Another significant source for perioperative morbidity and mortality is the recurrence of atrial tachyarrhythmias. The indication for redo-Fontan surgery in many of our patients, as in other studies, was refractory IART, with its resultant negative effects on the Fontan circulation [1316, 18, 19]. While centers report excellent operative and hemodynamic results in children and adults who undergo redo-Fontan surgery, the persistence of preoperative arrhythmias and the occurrence of new onset arrhythmias continue to be sources of postoperative morbidity [14, 15, 2022].
In our series, 13 of 15 patients presented for redo-Fontan operation with IART and were being treated with oral antiarrhythmic medications. Although complete resolution of the preoperative arrhythmia may not be achieved in the early postoperative period (5 of 13 patients had arrhythmia recurrence in the early postoperative period), perhaps modifications of the tachycardia circuits with concomitant intraoperative radiofrequency ablation (as previously described by and similar to Cox and colleagues [23]) and right atrial debulking in patients with previous atriopulmonary connections have resulted in complete resolution of the IART at last follow-up. A right atrial RFA procedure is preferred in the patient with atrial flutter or intraatrial reentry tachycardia. The RFA lesions are placed between the (1) tricuspid valve and coronary sinus (2), the fossa ovalis and the superior vena cava atriotomy, and (3) the fossa ovalis and the inferior vena cava.
Our results also compare favorably with those of Mavroudis and colleagues [13] who, in a larger cohort of children and adults, reported success with antiarrhythmic surgery concomitant with extensive right-sided and left-sided atrial modification in those patients presenting with failed Fontan circulations. They noted a low incidence of recurrent arrhythmias.
Conclusion
In adult patients presenting for the Fontan operation a comprehensive preoperative management strategy, which includes good patient selection and timely and decisive surgical intervention, can result in improved early outcome with minimal morbidity and improved functional capacity. Complete resolution of the preoperative arrhythmia may not be achieved in the immediate postoperative period in the redo-Fontan patients. However, modifications of the tachycardia circuits with atrial ablation and atrial debulking can result in complete resolution of refractory atrial tachyarrhythmias at early follow-up. New-onset arrhythmias continue to be a source of perioperative morbidity.
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