Ann Thorac Surg 2001;71:722-723
© 2001 The Society of Thoracic Surgeons
Case report
Congenital pulmonary vein stenosis associated with cor triatriatum
Masayoshi Ito, MDa,
Seiya Kikuchi, MDa,
Yoshikazu Hachiro, MDa,
Tomio Abe, MDa
a Department of Thoracic and Cardiovascular Surgery, Sapporo Medical University School of Medicine, Sapporo, Japan
Accepted for publication April 13, 2000.
Address reprints requests to Dr Ito, Department of Thoracic and Cardiovascular Surgery, Sapporo Medical University School of Medicine, South 1, West 16, Chuou-ku, Sapporo 060-8543, Japan
e-mail: shinwa{at}seagreen.ne.jp
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Abstract
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We report an 11-year-old boy who underwent surgical correction for stenosis of the right and left lower pulmonary veins at their junction with the left atrium and associated cor triatriatum. The embryology and therapeutic approaches to congenital pulmonary veins stenosis are discussed.
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Introduction
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Congenital pulmonary veins (PV) stenosis is a rare anomaly. Edwards [1] found 2 patients (0.4%) among 711 patients with major cardiac and thoracic vascular anomalies. Congenital PV stenosis is often an isolated lesion, but it may coexist with total anomalous pulmonary vein connection, complete transposition of the great artery, ventricular septal defect, and other anomalies [2]. Despite its embryologic relation [3], congenital PV stenosis associated with cor triatriatum is extremely rare. We present such a rare case of PV stenosis associated with cor triatriatum and discuss the embryology and therapeutic approaches to congenital PV stenosis.
The patient, a 2-month-old male infant, was taken to another hospital with tachypnea. He was diagnosed with complex cor triatriatum (Lucus-Schmit type IB1, Niwayama type IIA1), and was immediately operated on to remove the abnormal septum between the common pulmonary venous chamber (CPVC) and the left atrium and to close the septal defect between the CPVC and the right atrium. His condition improved in the weeks after the operation, and postoperative cardiac catheterization revealed a satisfactory result.
He continued to be healthy and without limitation to activities until 11 years of age when he presented with a moderate pulmonary hypertension and recurrence of cor triatriatum by the cardiac echocardiogram. He was referred to our hospital. Physical examination on admission showed a well-developed boy (weight, 40.5 kg; height, 157.5 cm). The heart sounds were almost normal. Chest roentgenogram showed a slightly prominent main pulmonary artery segment; the cardiothoracic ratio was 51%. Preoperative cardiac catheterization showed moderate pulmonary hypertension (35 mm Hg), and a pressure gradient between pulmonary wedge pressure and left atrium pressure (11 mm Hg). He was diagnosed with a recurrence of cor triatriatum and was referred to our department for the possibility of a surgical repair. At this point, the pulmonary vein stenosis was not recognized.
He underwent a repair of the recurrent cor triatriatum. After instituting cardiopulmonary bypass using direct cannulation of the superior and inferior venae cava, moderate hypothermia was achieved. The aorta was cross-clamped and cold blood cardioplegia was administered through the aortic root. The CPVC was opened through a vertical incision anterior to the right PVs. An abnormal septum was noted between the CPVC and the left atrium, with a hole of about 2 cm. A membranous diaphragm obstructed the entrances of the right and left lower PVs into the CPVC, and the openings were about 2 mm and 5 mm, respectively. The right and left upper PVs were connected with the CPVC without stenosis. The abnormal septum was excised to make an opening as large as possible. The membranous diaphragm at the junction of the right and left lower PVs and the CPVC was then removed. Post repair cardiac function was good and after cardiopulmonary bypass was discontinued, the pulmonary pressure was 20/14 mm Hg, whereas pressure in the radial artery was 110/62.
The postoperative course was uneventful. The postoperative cardiac echocardiogram and cardiac catheterization revealed satisfactory results. The patient was discharged from the hospital 13 days after the operation and at present (ie, 8 months after surgery), he is doing well.
Histologically, the membranous diaphragm removed from the orifices of the PVs showed intimal fibrous thickening and there was no cell infiltration suggestive of active inflammation.
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Comment
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Congenital PV stenosis has an embryologic relation with total anomalous pulmonary vein connection and cor triatriatum [3]. By the 3 mm stage, a diverticulum has formed in the future left atrial portion of the sinoatrial region. The diverticulum, or common PV, grows toward the developing lungs and ultimately connects with the PVs, which have already formed. When this drainage pathway is established, the early connections between the splanchnic plexus and the umbilicovitelline and cardinal venous systems become of secondary importance and are largely lost. As the left atrium grows rapidly, the common PV is absorbed into its posterior wall and the PVs enter the left atrium individually. A variety of obstructive lesions of the pulmonary venous drainage may occur, depending upon the stage at which normal embryogenesis is interrupted. If atresia of the common PV occurs very early, a major drainage route can be established through either the umbilicovitelline or cardinal venous system total anomalous pulmonary vein connection with infracardiac or supracardiac type. If stenosis occurs at a later stage, the result may be stenosis of the common PV (cor triatriatum). If stenosis occurs at a very late stage, after absorption of the common PV into the left atrium, the result will be stenosis or atresia of individual PVs. Stenosis may have occurred over two stages in our patient. Furthermore, PV stenosis may occur on a physiologic basis in patients with cor triatriatum due to restriction of flow from the chamber containing PVs to the chamber containing the mitral valve.
The clinical picture of PV stenosis resembles that seen with any lesion that produces pulmonary venous hypertension. The prognosis is poor, particularly if all veins are affected. Although progression of symptoms may be less rapid if only one or two PVs are involved, progression to bilateral pulmonary vascular disease and death is usually the rule, even in initially less severe cases [1–6]. Two-dimensional echocardiogram with pulse Doppler examination and color-flow imaging provide an accurate method for identifying and localizing pulmonary venous obstruction and qualifying its degree [2].
Kawashima and coworkers [4] performed the first reported successful surgical repair of PV stenosis in 1971. In subsequent reports, the early and late mortality rates have been high, and early and intermediate results have been reported only sporadically [5, 6]. The surgical approach to PV stenosis must be flexible. The technique of repair will depend upon the local anatomy of each lesion. Those of the diaphragmatic type, as described in this report, can be treated effectively by excision of the obstructing membrane [4]. Increase of the caliber of the vein can be effectively achieved by patch angioplasty. Whenever possible, as van Son [2] previously reported, the use of artificial or allograft patches should be avoided, as they may induce neointimal formation with increased need for reoperation. When segmental stenosis or atresia is present, the technique described by Pacifico and associates [6] for relief of pulmonary venous obstruction with the use of atrial wall or atrial septal tissue may be useful.
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References
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Edwards J. Congenital stenosis of pulmonary veins. Lab Invest 1960;9:46-66.[Medline]
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Van Son J.A.M., Danielson G.K., Puga F.J., et al. Repair of congenital and acquired pulmonary vein stenosis. Ann Thorac Surg 1995;60:144-150.[Abstract/Free Full Text]
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Sade R.M., Freed M.D., Matthews E.C., et al. Stenosis of individual pulmonary veins. J Thorac Cardiovasc Surg 1974;67:953-962.[Medline]
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Kawashima Y., Ueda T., Naito Y., et al. Stenosis of pulmonary veins: report of a patient corrected surgically. Ann Thorac Surg 1971;12:196-202.[Medline]
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Binet J.P., Bouchard F., Langlos J., et al. Unilateral congenital stenosis of the pulmonary veins. A very rare cause of pulmonary hypertension. J Thorac Cardiovasc Surg 1972;63:397-402.[Medline]
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Pacifico A.D., Mandke N.V., McGrath L.B., et al. Repair of congenital pulmonary venous stenosis with living autologous atrial tissue. J Thorac Cardiovasc Surg 1985;89:604-609.[Abstract]
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Abstract
Introduction
