Ann Thorac Surg 2003;75:271-273
© 2003 The Society of Thoracic Surgeons
Case report
The fenestrated Kawashima operation for single ventricle with interrupted inferior vena cava
Robert L. Hannan, MD*a,
Anthony F. Rossi, MDa,
David G. Nykanen, MDa,
Leo Lopez, MDa,
Francisco Alonso, RNFAa,
Jeffrey A. White, MSa,
Redmond P. Burke, MDa
a Division of Cardiovascular Surgery, Miami Children ’s Hospital, Miami, Florida, USA
Accepted for publication August 9, 2002.
* Address reprint requests to Dr Hannan, Division of Cardiovascular Surgery, 3200 SW 60th Court, Suite 102, Miami, FL 33155, USA
e-mail: rhannan001{at}aol.com
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Abstract
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An 8-month-old boy with double outlet right ventricle with hypoplastic left ventricle, heterotaxy, left atrial isomerism, bilateral superior vena cavae without bridging vein, and interruption of the inferior vena cava with azygous continuation to the left superior cava underwent a bilateral bidirectional cavopulmonary anastomosis. A calibrated 3-mm connection between the right pulmonary artery and the common atrium was constructed with the proximal right superior vena cava to allow right to left shunting, analogous to a fenestration in a Fontan operation. We hypothesize that in small young patients undergoing the Kawashima operation a fenestration may improve postoperative hemodynamics.
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Introduction
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Children with single ventricle, heterotaxy, polysplenia and interrupted inferior vena cava (IVC) with azygous continuation are often palliated with a bidirectional cavopulmonary anastomosis (in this setting referred to as the Kawashima operation [1]). The interrupted IVC with azygous continuation creates a postoperative circulation similar to that in the Fontan procedure, less hepatic blood flow. The creation of Fontan-like physiology in infants has been sporadically reported with limited morbidity and mortality data [2–4]. The development of pulmonary arteriovenous malformations and the effect of including hepatic blood flow in the pulmonary circulation have been well described and is the source for most of the data on patients less than 1 year of age. We hypothesized that fenestration of the Kawashima circulation with the potential for a controlled right to left shunt would be technically possible and would be of theoretical benefit postoperatively in infants, especially if additional procedures beyond cavopulmonary anastomosis were required. We also hypothesized that a fenestration might be of benefit if flow through the pulmonary valve was preserved in an attempt to decrease the possibility of the development of pulmonary arteriovenous malformations.
A 3,500 g newborn boy was diagnosed with complex single ventricle (double outlet right ventricle with hypoplastic left ventricle) heterotaxy, left atrial isomerism, bilateral superior vena cavae (SVC) with no bridging vein, interruption of the inferior vena cava with continuation to the left SVC, pulmonic stenosis, patent ductus arteriosus to the left pulmonary artery, and a right aortic arch with mirror image branching. He was prostaglandin dependent and underwent a 3.5-mm central shunt operation in the newborn period. Evaluation at 8 months of age (weight 5.2 kg) revealed a restrictive atrial septum, hypoplastic central pulmonary arteries, and an oxygen saturation of 86%. His atrial septum did not appear to be treatable in the cardiac catheterization laboratory. He was brought to the operating room and atrial septectomy, extensive central pulmonary arterioplasty, and bilateral bidirectional cavopulmonary anastomoses were performed.
The proximal right superior SVC was anastamosed to the right pulmonary artery and constricted with a silk ligature tied tightly around a 3-mm coronary probe (Fig 1).
Forward flow from the right ventricle through the stenotic pulmonary valve was preserved with no effect on SVC pressure. SVC pressure was 16 mm Hg with a common atrial pressure of 7 mm Hg after separation from cardiopulmonary bypass. Postoperative echocardiography revealed decompression of the pulmonary artery through the fenestration into the common atrium (Fig 2).
The patient underwent an uneventful postoperative course with oxygen saturations in the high 80% range. At 18 months of age he underwent elective cardiac catherization to evaluate his central pulmonary artery reconstruction. Catheterization demonstrated spontaneous closure of the fenestration, an unrestrictive atrial septum, and no significant pulmonary artery stenosis (Fig 3).
He remains well at 2 years of age with an oxygen saturation of 90%.
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Fig 1. Creation of the connection between the common atrium and the right pulmonary artery (RPA) with a constriction in the proximal RPA created over a 3-mm coronary dilator. The left superior vena cava and azygous continuation of the inferior vena cava to the left superior vena cava are not shown. (AO= aorta; RSVC = right superior vena cava.)
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Fig 2. Echocardiogram demonstrating flow from the pulmonary artery through fenestration to the common atrium (arrow).
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Comment
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The acute risk associated with the creation of this near-Fontan physiology in small infants is not well documented in the literature. Kawashima and associates [2] reviewed their 15-year results in 1994 and there were no patients under 1 year of age. There are several reports of a small number of patients under 1 year of age undergoing the Kawashima operation [3, 4]. We believe that the addition of one calibrated SVC to pulmonary artery anastomosis to create a potential right to left shunt was warranted in this small infant who required additional procedures beyond straightforward bidirectional cavopulmonary anastomosis. We believe this is the first report of a fenestration being created in a Kawashima operation to allow for potential right to left shunting. In addition preserving limited flow from the RV may reduce the late complication of pulmonary arteriovenous malformations in this patient.
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References
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- Kawashima Y., Kitamura S., Matsuda H., et al. Total cavopulmonary shunt operation in complex cardiac anomalies. A new operation. J Thorac Cardiovasc Surg 1984;87:74-81.[Abstract]
- Kawashima Y., Matsuki O., Yagihara T., et al. Total cavopulmonary shunt operation. Semin Thorac Cardiovasc Surg 1994;6:17-20.[Medline]
- McElhinney D.B., Reddy V.M., Moore P., et al. Bidirectional cavopulmonary shunt in patients with anomalies of systemic and pulmonary venous drainage. Ann Thorac Surg 1997;63:1676-1684.[Abstract/Free Full Text]
- Shah M.J., Rychik J., Fogel M.A., et al. Pulmonary AV malformations after superior cavopulmonary connection: resolution after inclusion of hepatic veins in the pulmonary circulation. Ann Thorac Surg 1997;63:960-963.[Abstract/Free Full Text]
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Abstract
Introduction
