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J Thorac Cardiovasc Surg 2004;127:281-283
© 2004 The American Association for Thoracic Surgery
Brief communication |
a Departments of Cardiac Surgery and Cardiology, Children's Hospital Boston, Harvard Medical School, Boston, Mass, USA
Received for publication March 17, 2003; accepted for publication June 18, 2003.
* Address for reprints: Pedro J. del Nido, MD, Department of Cardiac Surgery, Children's Hospital Boston, 300 Longwood Ave, Boston, MA 02115, USA
pedro.delnido{at}tch.harvard.edu
Long-term outcome of patients after the Fontan procedure is currently excellent.1 Despite initial reports with high incidence of postoperative obstruction of pulmonary venous pathway,2 this complication has become rare.3,4 When it does occur, it can cause severe deterioration of the cavopulmonary circulation,2,3 and reoperation is usually necessary. We report our experience with 3 patients presenting with this lesion late after cavopulmonary connection, with the aim of better understanding the potential causes of this complication. We also discuss methods of prevention and treatment.
Patients and methods
Between January 1984 and November 2002, a total of 1092 patients with single-ventricle physiology underwent Fontan procedures at Children's Hospital Boston. Among these patients, 268 were admitted for reoperation, including 3 patients with the diagnosis of isolated pulmonary venous pathway obstruction 8, 23, and 42 months after the Fontan procedure. Patients 1 and 3 had a diagnosis of hypoplastic left heart syndrome, whereas patient 2 had diagnosis of heterotaxy syndrome with situs inversus, double-outlet right ventricle, and unbalanced atrioventricular canal with pulmonary stenosis. None of the patients had obstructive pulmonary venous drainage at initial diagnosis. All had undergone lateral tunnel cavopulmonary connection with a 4-mm fenestration. Patient 2 underwent closure of the fenestration with a percutaneous device (CardioSEAL, NMT Medical, Boston, Mass) 4 months after the Fontan operation (Table 1).
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Symptoms at readmission included peripheral edema and protein-losing enteropathy in 2 cases and easy fatigability and sporadic cyanotic episodes in the other. Two-dimensional echocardiography demonstrated restrictive interatrial communication in all cases (mean transatrial pressure gradient 8.6 mm Hg). Patient 2 had a discrete left atrial membrane, resulting in additional obstruction to pulmonary venous pathway (Figure 1) . Hemodynamic studies showed transatrial pressure gradients of 6 and 5 mm Hg in patients 1 and 3, whereas in patient 2 a pressure gradient of 10 mm Hg was measured across the left atrial membrane (Table 1).
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At short-term follow-up (17, 2, and 13 months, respectively), all patients have improved level of activity. There has been complete resolution of protein-losing enteropathy in patients 1 and 3. Two-dimensional echocardiography in patients 1 and 3 has shown an unobstructed pulmonary venous pathway.
Discussion
Long-term results after lateral tunnel-type Fontan connection are reported to be excellent, with an overall survival of 91% and freedom from failure of 92% at 10 years.1 Obstructed pulmonary venous pathway was described as an early complication of the lateral tunnel cavopulmonary connection as a result of improper baffle creation.2 Technical improvements have reduced this complication, and in our experience it is rare. Previous reports3,4 underline the relative greater frequency of this complication in heterotaxy syndrome when associated with anomalous pulmonary and systemic venous connections, which can make surgical repair complicated with the need to create a tortuous and potentially obstructive pathway. Despite this concern, a recent report from our institution5 has shown excellent outcome with cavopulmonary connection in patients with heterotaxy syndrome and anomalous pulmonary venous return. This is likely due to improved surgical technique and use of extracardiac conduit in cases with complex atrial and venous anatomy (18%).
None of the 3 patients had evidence of obstruction at the pulmonary venous pathway early after the Fontan procedure, as determined by 2-dimensional echocardiography. Pulmonary venous obstruction developed slowly through a period of several months to years. In patient 2, pulmonary venous obstruction developed 4 months after device closure of the fenestration, suggesting that the additional scarring from device implantation contributed to the obstruction. The obstruction was due to fibrotic tissue at either the interatrial septum or in the interatrial course of the pulmonary veins (patient 2).
To prevent this complication, and to ensure a wide open pulmonary venous outflow, particularly in patients with mitral atresia or stenosis, we currently enlarge the communication between the pulmonary veins and the right atrium by unroofing the coronary sinus posteriorly into the left atrium, in addition to resection of the interatrial septum.
In conclusion, late onset obstruction of pulmonary venous pathway after lateral tunnel cavopulmonary connection is a rare but serious complication that may be prevented by unroofing the coronary sinus at the time of Fontan procedure.
References
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