Ann Thorac Surg 2007;84:312-313
© 2007 The Society of Thoracic Surgeons
How To Do It
Modified Superior Repair for Supracardiac Total Anomalous Pulmonary Venous Connection in the Adult
John J. Nigro, MD, MSa,*,
Steven Choi, MDa,
Joseph Graziano, MDa,
Kimberly Lynn Gandy, MD, PhDb,
Lourdes M. Guerrero-Tiro, MDa,
David C. Cleveland, MDa
a The Congenital Heart Center, Heart & Lung Institute, Saint Joseph’s Hospital and Medical Center, Phoenix, Arizona
b Department of Cardiothoracic Surgery, University of Arizona, Tuscon, Arizona
Accepted for publication October 16, 2006.
* Address correspondence to Dr Nigro, The Congenital Heart Center, Heart & Lung Institute, Saint Joseph’s Hospital and Medical Center, 500 W Thomas Rd, Suite 500, Phoenix, AZ 85013 (Email: john.nigro{at}chw.edu).
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Abstract
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Repair of total anomalous pulmonary venous connection has been well described and is associated with good long-term outcomes for neonates and infants. Although rare, some patients initially present at adolescence or during adulthood and repair is complicated by compensatory changes related to chronic volume overload. Transection of the superior vena cava facilitates both exposure of the venous confluence and repair in these patients.
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Introduction
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Total anomalous pulmonary venous connection is a well-described cardiac anomaly that accounts for 1% to 5% of all congenital heart defects [1]. Four basic anatomic types of anomalous venous connection have been defined based on the level of the pulmonary-systemic (venous) connection relative to the heart and include the following: supracardiac (type I), intracardiac (type II), infracardiac (type III), and mixed drainage (type IV) [1]. Pathophysiology of these lesions is dependent on the integrity of venous drainage and can manifest as pulmonary venous obstruction or as congestive heart failure. Most of these lesions provide significant symptoms in the neonate or infant and require early repair [1].
Although uncommon, patients without obstruction and without significant heart failure can present later in life and even during adulthood [2–6]. Longstanding volume overload in these patients can cause severe right heart and major venous dilatation. These compensatory changes complicate traditional repair techniques that were developed for use in neonates and infants. Two adult patients with unrepaired supracardiac total anomalous pulmonary venous connection (type I) presented to our medical center for surgical correction. The right heart, innominate vein, and superior vena cava were markedly enlarged in both of these patients (Fig 1), and complicated the traditional (repair) approach to the pulmonary venous confluence and the left atrium. A modification of the superior approach with transection of the superior vena cava provided excellent exposure and facilitated repair in both patients.
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Fig 1. Magnetic resonance image of adult patient with supracardiac total anomalous pulmonary venous return demonstrates engorgement of the innominate vein and superior vena cava. (* = aorta; IV = innominate vein; SVC = superior vena cava; VV = vertical vein.)
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Technique
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Median sternotomy and pericardiotomy are performed. The superior vena cava is circumferentially mobilized up to the caval innominate junction and off of the pulmonary artery and pericardium. The ascending aorta is circumferentially mobilized and encircled at the level of the transverse sinus. The vertical vein is identified and encircled inferior to its junction with the innominate vein. A stay suture is placed on the tip of the left atrial appendage and is retracted to the patient’s left to ensure that the atrium and appendage are properly positioned for subsequent repair. The patient is heparinized and bi-caval cannulation is established with the upper (venous) cannula placed at the caval innominate junction. Carbon dioxide is infused into the thoracic space at a high enough rate to ensure a nitrogen depleted operative field. Cardiopulmonary bypass is initiated, the heart is arrested, and the right atrium is isolated. The cava is transected at least 2 cm above the cava-atrial junction to avoid the sinus node and at least 3 cm distal to the superior caval cannula to ensure ease at caval reconstruction. The roof of the left atrium is then exposed by leftward retraction of the ascending aorta and inferior retraction of the atrial portion of the (distal) superior vena cava (Fig 2). This provides excellent exposure to the pulmonary venous confluence and the roof of the left atrium. Both of these structures are incised to create a connection that is at least as large as the left ventricular inflow area, and the repair is completed with running monofilament suture. The transected caval orifice is usually quite large and the interatrial septal defect can be closed through it without an atriotomy (Fig 3). Caval continuity can be restored after removal of air and cross clamp removal using a running monofilament suture. The vertical vein is usually ligated prior to separation from cardiopulmonary bypass.
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Fig 2. Transection and inferior retraction of the distal superior vena cava combined with aortic retraction (away from surgeon) facilitates exposure (A) to the pulmonary venous confluence, and repair (B) of supracardiac total anomalous pulmonary venous connection. (Ao = aorta; PA = pulmonary artery; SVC = superior vena cava.)
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Comment
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Although presentation at an adult age is rare, we have recently treated 2 adult patients who had unrepaired supracardiac total anomalous pulmonary venous return. Traditional repair in these patients was complicated by compensatory changes (severe enlargement of the right heart and systemic veins) and depth of the adult chest. A superior approach modified with caval transection provided excellent exposure of both the pulmonary venous confluence and the roof of the left atrium. Caval reconstitution was required, but atriotomy was avoided.
Caval transection is a useful technique to aid exposure of the left atrial dome and mitral valve. This technique can also facilitate repair of supracardiac total anomalous pulmonary venous connection in the rare patient who initially presents as an adult or adolescent.
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References
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- Kirshbom PM, Jaggers J, Ungerleider RM. Total anomalous pulmonary venous connectionIn: Mavroudis C, Backer CL, editors. Pediatric Cardiac Surgery. Philadelphia, PA: Mosby; 2003. pp. 612-623.
- Melki J, Kovarsky S, Redonnet M, et al. Total anomalous pulmonary venous return in a 61 year-old adultin French Ann Chir 1992;46:722-724.[Medline]
- Mycinski C, Doll G, Chantepie A, et al. Total abnormal pulmonary venous return surgically treated in an adult Arch Mal Coeur Vaiss 1989;82:815-817.[Medline]
- Chaudron JM, Chalant C, Kremer R. Total anomalous pulmonary venous return in adults Arch Mal Coeur Vaiss 1977;70:309-314.[Medline]
- Billig DM, Peguero FA. Total anomalous pulmonary venous returnSuccessful total correction in a 44-year-old man with subtotal absence of interatrial septum, tricuspid insufficiency, and cardiac dextroversion. Chest 1976;69:687-690.[Medline]
- Singh R, Weisinger B, Carpenter M, Dammann Jr JF, McGuire LB. Total anomalous pulmonary venous return, surgically corrected in two patients beyond 40 years of age Chest 1971;60:38-43.[Medline]
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Abstract
Introduction
