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Ann Thorac Surg 1996;62:1261-1266
© 1996 The Society of Thoracic Surgeons
Division of Cardiothoracic Surgery and Department of Pediatric Cardiology, Johns Hopkins Medical Institutions, and Department of Pediatric Cardiology, University of Maryland School of Medicine, Baltimore, Maryland
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Abstract |
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 TopFootnotes Abstract IntroductionPatients and MethodsResultsCommentReferences |
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Methods. Extracardiac lateral tunnels (n = 9) were constructed using a polytetrafluoroethylene patch (n = 7), pericardial patch (n = 1), or in situ pericardial flap (n = 1). Extracardiac lateral conduits (n = 6) were constructed using nonvalved homografts (n = 2) or polytetrafluoroethylene tube grafts (n = 4). Fenestrations were created in 4 patients (2 each in extracardiac lateral tunnel and extracardiac lateral conduit patients). Aortic cross-clamping was completely avoided in 12/15 patients (aortic cross-clamping in 2 patients for atrial septal defect enlargement and 1 for Damus-Kaye-Stansel procedure).
Results. There have been no operative deaths. Prolonged postoperative chest tube drainage (>2 weeks) has been rare (n = 1). At follow-up (range, 6 to 54 months; mean, 27.5 months), all patients are in New York Heart Association class I or II and remain in normal sinus rhythm. Late protein-losing enteropathy was seen in 1 patient and was successfully treated by percutaneous creation of a stented fenestration from the extracardiac tunnel to the systemic atrium. Late catheterizations reveal unobstructed extracardiac lateral tunnel function and low pulmonary pressures (range, 11 to 13 mm Hg). Advantages of the extracardiac Fontan include (1) avoidance of aortic cross-clamping in most patients, (2) the hemodynamic benefits of total cavopulmonary connection, (3) avoidance of atriotomy and intraatrial suture lines, (4) preservation of sinus rhythm and no arrhythmias at 2 year follow-up, (5) drainage of the coronary sinus to low pressure atrium, (6) allowance for early/late fenestrations, (7) prevention of baffle leaks and intraatrial obstruction, and (8) allowance for growth (tunnel procedures only).
Conclusions. We recommend this extracardiac procedure for all suitable patients undergoing surgical conversion to the Fontan circulation.
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Introduction |
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TopFootnotesAbstractIntroductionPatients and MethodsResultsCommentReferences |
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In 1993, we reported our results and early experience with the extracardiac Fontan procedure, a modification of the total cavopulmonary connection (TCC) that combines the bidirectional Glenn shunt with an extracardiac lateral tunnel (ELT) to carry inferior vena caval flow to the pulmonary arteries [1]. We proposed that this operation would preserve the hemodynamic benefits ascribed to the TCC, yet avoid the potential disadvantages of aortic cross-clamping and complications related to the intraatrial placement of baffles or tunnels, including leaks, obstruction, or dysrhythmias [2]. Since that time, following favorable reports from other centers [3–7], we have also begun to employ extracardiac lateral conduits (ELCs) between the inferior vena cava and pulmonary artery to achieve the same end. The intermediate results of the ELT and ELC procedures are the focus of this report.
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Patients and Methods |
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TopFootnotesAbstractIntroductionPatients and MethodsResultsCommentReferences |
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. Mean age at operation for the 15 patients was 6.4 years (range, 15 months to 32 years). Eleven of 15 patients (5/9 ELT, 5/6 ELC) had previously placed bidirectional Glenn shunt(s). Five bidirectional Glenn shunt(s) were performed concomitantly in 4 patients undergoing the ELT Fontan procedure, whereas all patients undergoing ELC conversion to the Fontan circulation had undergone staged bidirectional Glenn shunts during previous operations. Four patients had undergone pulmonary artery (PA) banding as neonates. In 2 patients who had undergone staged bidirectional Glenn shunts in preparation for an eventual extracardiac Fontan procedure, marked enlargement of the head developed due to presumed venous engorgement when a banded PA (60 mm Hg gradient) or tightly stenotic subpulmonic outflow tract was left patent. Both have returned to normal size after either closure of the main PA followed shortly thereafter by definitive conversion to the ELT Fontan (patient 7) or early conversion to the ELC Fontan (patient 12). One patient with pulmonary atresia and intact ventricular septum had previously undergone a standard Fontan-Kreutzer operation. This was converted to an ELC to effect arterialization of right ventricular (sinusoidal) blood to treat significant myocardial ischemia.
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We have previously described the extracardiac Fontan procedure in detail [1]. All patients were placed on aortobicaval (tricaval in patients with bilateral superior venae cavae) cardiopulmonary bypass to facilitate the operation. A summary of the procedure for each patient is reported in Table 2. Nine of the 15 patients underwent fashioning of an ELT. In 7 of these patients PTFE was used to construct the tunnel (Fig 1); in 1 patient pericardium was employed, and in 1 patient it was feasible to use an in situ pericardial flap for creation of the tunnel. Extracardiac lateral conduits between the divided inferior vena cava and PA were constructed in the remaining 6 patients. In 2 of these patients a nonvalved homograft was used; in the other 4 patients, PTFE tube grafts were employed.
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Patients undergoing the ELT procedure were placed on a lifelong aspirin regimen, whereas those receiving the ELC were anticoagulated with warfarin for 6 months and then placed on a lifelong aspirin regimen.
The mean (± standard deviation) cardiopulmonary bypass time for patients undergoing the ELT procedure was 164.7 ± 50 minutes, which was significantly longer than for patients receiving the ELC (104.7 ± 38 minutes; p < 0.05 by Mann-Whitney test of significance).
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Results |
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TopFootnotesAbstractIntroductionPatients and MethodsResultsCommentReferences |
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. There were no operative deaths. Normal sinus rhythm was preserved in all patients in the immediate postoperative period. Fourteen of 15 patients were extubated within the first 24 hours postoperatively. One patient remained ventilated for 70 hours to facilitate delayed sternal closure. One patient had prolonged chest tube drainage (>2 weeks), and 2 other patients required treatment for chylothoraces, which resolved with medical therapy and sclerosis (1 patient). A third patient was readmitted after discharge with a late chylothorax, which did not respond to medical therapy and required eventual thoracic duct ligation. One patient required continuous venovenous hemodialysis (duration, 48 hours) for acute renal failure despite hemodynamic stability and early extubation. Creatinine clearance returned to normal in this patient before discharge. One patient sustained a perioperative stroke, manifested by seizures and documented by computed tomography. Follow-up was obtained in all 15 patients. The median follow-up period was 26 months (range, 6 to 54 months). Twenty-four–hour Holter monitoring has confirmed that all patients remain in normal sinus rhythm. Fourteen of 15 patients (93.3%) are in New York Heart Association class I; 1 patient is in New York Heart Association class II and requires angiotensin-converting enzyme inhibition for afterload reduction. Arterial oxygen was in excess of 92% on room air in 14 of 15 patients (93.3%).
Late protein-losing enteropathy (PLE) occurred in the same patient who had required postoperative thoracic duct ligation for treatment of a delayed chylothorax (patient 6). The PLE was unresponsive to intense medical therapy, and heart transplantation was considered. As a bridge to transplantation, a stented fenestration was made between the ELT and native atrium in the common atrial wall (Fig 2). Albumin requirements ceased within 48 hours, and the patient has been free of all manifestations of PLE since this intervention. Due to the size of the stented fenestration (8 mm), the patient remains moderately desaturated (oxygen saturations of 75% on room air); this has been well tolerated, however, and the patient demonstrates normal activity and neurologic development for age. He remains on warfarin therapy due to the presence of his stent and has had one episode of temporary neurologic deficit during a period of subtherapeutic (international normalized ratio < 2.5) anticoagulation, presumably from an embolus. This has completely resolved without residua.
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Comment |
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TopFootnotesAbstractIntroductionPatients and MethodsResultsCommentReferences |
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The construction of the TCC has several drawbacks. It requires prolonged aortic cross-clamping, with mean intervals of 88 minutes having been reported in large series [13]. Due to the intraatrial position of the conduit, there is the potential for baffle obstruction of the pulmonary veins or atrioventricular valves in addition to early or late baffle leaks resulting in cyanosis and failure of palliation. Dysrhythmias are frequently encountered and are likely a direct result of the need for atriotomy and extensive intraatrial suture lines [14]. As mortality for TCC procedures has diminished, the significance of these factors has increased as they have become the prime determinants of long-term operative results and functional status.
Because of the location of the lateral tunnel in the extracardiac Fontan procedure, complications due to intraatrial techniques are avoided, yet the hemodynamic and hydrodynamic effects of the TCC are preserved. We believe that the most important advantages are the avoidance of aortic cross-clamping and atrial incisions/suture lines. These may be a crucial advantage in patients with preexisting systolic diastolic dysfunction caused by chronic hypoxemia and volume overload. Late function may also be favorably affected, as ventricular performance and maintenance of normal sinus rhythm are the critical determinants of overall optimal circulation after total right heart bypass procedures [14].
In constructing the ELT, the lateral atrial wall comprises between 30% and 50% of the tunnel circumference; this preserves the potential for growth obviating the need for oversizing the conduit between the inferior vena cava and PA. The area of atrial tissue exposed to high venous pressures is also minimized. As with the standard intracardiac TCC, an adjustable atrial septal defect or fenestration between the systemic circulation and the atrium (or atria) can be incorporated into the atrial wall component of the ELT at operation. The ELT preserves the option to add a fenestration percutaneously later in the postoperative period, if needed. However, construction of the ELT does require placement of epicardial atrial suture lines. Although no atrial arrhythmias have been observed in up to 4.5 years of follow-up, the long-term effects of epicardial suture lines on atrial arrhythmogenicity is unknown. The main advantage of the ELC is that it is technically more simple than ELT construction, thus further reducing the cardiopulmonary bypass time. In a small series, patients undergoing the ELC had a lower combined incidence of persistent perfusions and chylothoraces (0%) when compared with patients undergoing the ELT (33%). Although a fenestration can be fashioned at operation, the options for adjustable or late percutaneous fenestrations are forfeited when the ELC technique is used.
In this review of a consecutive series of 15 children undergoing conversion to the Fontan circulation, we have demonstrated that the extracardiac Fontan procedure, either as a lateral tunnel or lateral conduit, can be applied to a variety of complex congenital heart anomalies. At median follow-up of 27 months, it is important to note that all patients remain in normal sinus rhythm, supporting our belief that avoidance of atrial incisions and intraatrial suture lines as well as prevention of chronic exposure of the atria to high venous pressures may reduce the incidence of early and late dysrhythmias. Avoidance of ischemic arrest of the heart would also appear to be beneficial in terms of preservation of ventricular function, as 14 of 15 patients continue to be in New York Heart Association class I.
Late follow-up is required to confirm both the relative prevention of late dysrhythmias using the extracardiac technique and the growth potential of the lateral tunnel. For now, however, we recommend the adoption of the extracardiac technique for the conversion of suitable candidates to the Fontan circulation.
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Footnotes |
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TopFootnotesAbstractIntroductionPatients and MethodsResultsCommentReferences |
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Address reprint requests to Dr Laschinger, O'Dea Medical Arts Bldg, 7505 Osler Dr, Suite 304, Towson, MD 21204
This article has been selected for the discussion forum on the STS Web site:http://www.sts.org/annals
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TopFootnotesAbstractIntroductionPatients and MethodsResultsCommentReferences |
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of the circumference of the extracardiac lateral tunnel. The stent has been expanded to 8 mm in size. (C) Result of the percutaneous fenestration procedure. Note the majority of inferior vena caval blood is now diverted to the native atrium. Protein-losing enteropathy is resolved.