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Ann Thorac Surg 1997;63:1175-1177
© 1997 The Society of Thoracic Surgeons
Divisions of Cardiovascular Surgery, and Cardiology, Miami Children's Hospital, Miami, Florida
Accepted for publication November 14, 1996.
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Abstract |
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Introduction |
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The Fontan procedure, first described in 1971 [1], continues to evolve. Total cavopulmonary connection developed as an alternative to atriopulmonary connection in an effort to create more efficient flow dynamics [2]. This type of right heart bypass is currently most often achieved by using an intraatrial lateral tunnel or an intraatrial conduit [3]. The lateral tunnel technique has gained favor by streamlining the venous flow pattern and providing growth potential, but postoperative sinoatrial conduction disturbances related to multiple atrial suture lines still can occur [4]. Recent technical modifications using extracardiac conduits to connect the inferior vena cava (IVC) to the pulmonary artery have reduced the incidence of atrial arrhythmias [5, 6]; however, the long-term performance of these conduits remains uncertain.
Choreoathetosis is a known complication of cardiopulmonary bypass. Reoperation on cardiopulmonary bypass may lead to progression or exacerbation of this neurologic injury [7]. To minimize the risk of exacerbation of this condition in a patient with choreoathetosis after prior bilateral bidirectional cavopulmonary anastomosis, we designed a procedure to complete the Fontan connection without using cardiopulmonary bypass.
A 6-month-old boy with mitral atresia, double-outlet single right ventricle, subpulmonary stenosis, and bilateral superior venae cavae (SVCs) underwent a modified Blalock-Taussig shunt. Then, at the age of 17 months, the child underwent a bilateral bidirectional cavopulmonary anastomosis and atrial septectomy. Postoperatively, choreoathetosis developed. The patient's neurologic status gradually improved so that by discharge he had only moderate choreoathetoid movements including tongue thrusting, facial grimacing, and arm movements.
At the age of 7 years, the child was referred for a Fontan procedure because of worsening cyanosis. His neurologic status had improved so that he could now walk, self-feed, attend school, and speak in short phrases with mild speech difficulties. The preoperative neurologic examination demonstrated moderate choreoathetoid movements centered in the face and arms. Preoperative echocardiographic findings were consistent with mitral atresia, double-outlet right ventricle, subpulmonary stenosis, and an unrestrictive atrial septal defect. Preoperative cardiac catheterization confirmed patent bilateral bidirectional cavopulmonary anastomoses with good distal pulmonary artery anatomy. The transpulmonary gradient was low, with bilateral mean pulmonary artery pressures of 12 mm Hg and atrial pressures of 9 mm Hg. The pulmonary-to-systemic flow ratio was 0.8, with a pulmonary vascular resistance index of 0.8 Wood units per meter squared. Resting arterial oxygen saturation was 75% with a hemoglobin level of 16 g/dL.
At reoperation, under general anesthesia, the median sternotomy was reopened and the right SVC was exposed. The main pulmonary artery and left SVC were encased in dense scar tissue. The child was heparinized (4 mg/kg). A test occlusion of the right SVC was well tolerated with no increase in jugular venous pressure and no change in oxygen saturation or blood pressure. Therefore, flow through the left SVC to the left lung was adequate to provide oxygenation and support the circulation. A side-biting clamp was applied to the junction of the right SVC and the right pulmonary artery. An incision was made on the anterior wall of the right SVC, extending across the previous cavopulmonary anastomosis onto the right pulmonary artery. A beveled 16-mm ribbed expanded polytetrafluoroethylene (W.L. Gore and Associates, Inc, Flagstaff, AZ) tube graft was anastomosed to this incision. The vascular clamp was then removed and the tube graft was clamped, restoring flow from the right SVC to the pulmonary artery.
The IVC was dissected free from the diaphragm and test occluded. The blood pressure dropped steadily and the clamp was removed. The left femoral vein was then exposed and cannulated with a 14F Biomedicus (Medtronic Inc, Anaheim, CA) venous cannula. A 16F right-angled venous cannula was placed in the right atrium and connected to the femoral venous cannula with a short segment of pump tubing (Fig 1
). With this venous shunt open, the IVC was again test occluded, producing no change in blood pressure or oxygen saturation. The IVC pressure stabilized at 20 mm Hg. The IVC was divided just above the diaphragm, and the cardiac end was oversewn. The tube graft was then trimmed to the appropriate length and anastomosed end-to-end to the IVC. The graft was deaired and opened. The venous shunt was removed and the femoral vein was repaired. The child returned to the cardiac intensive care unit and was extubated 4 hours after the operation.
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Comment |
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TopFootnotesAbstractIntroductionCommentAcknowledgmentsReferences |
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The prior bilateral bidirectional cavopulmonary anastomosis made this patient a perfect candidate for this procedure because it allowed the maintenance of pulmonary blood flow to the left lung during the upper anastomosis. Because cerebral venous hypertension would be undesirable and represent a threat to the brain, test occlusion of the right SVC was performed with monitoring of right internal jugular pressure.
In the optimal Fontan connection, flow energy loss due to turbulence in the venous pathway should be minimized [2]. A symmetric extracardiac conduit for cavopulmonary connection may reduce turbulence between the venae cavae and the pulmonary arteries, thereby improving hydrodynamic flow performance. The risk for atrial arrhythmias may also be decreased by avoiding extensive right atrial suture lines [5, 6].
Polytetrafluoroethylene grafts have been successfully used as venous conduits in a variety of settings [8]. Externally supported conduit may preserve laminar flow by maintaining uniform cross-sectional geometry. These ribbed conduits may also resist external compression by the right atrium and lung. Moreover, this patient's size allowed placement of a large conduit, which increases flow and decreases the likelihood of conduit thrombosis.
In this patient, the presence of a prior bilateral bidirectional cavopulmonary anastomosis allowed performance of an extracardiac Fontan operation without cardiopulmonary bypass. In the future, techniques for extracardiac Fontan completion without cardiopulmonary bypass in patients with single bidirectional cavopulmonary connections may be developed as well.
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Acknowledgments |
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was prepared by Jeffrey A. White, MS. |
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