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Ann Thorac Surg 1995;60:144-150
© 1995 The Society of Thoracic Surgeons
Divisions of Thoracic and Cardiovascular Surgery and Anatomic Pathology and Section of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota
Accepted for publication March 15, 1995.
| Abstract |
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Methods. Eight patients (age range, 3 months to 43 years; median age, 1.5 years) underwent surgical relief of pulmonary vein stenosis. Two had congenital pulmonary vein stenosis, 5 had pulmonary vein stenosis that was acquired after surgical treatment of total anomalous pulmonary venous connection, and 1 had pulmonaryvein stenosis associated with idiopathic mediastinal fibrosis and calcification.
Results. One infant died 2 months after correction of acquired pulmonary vein stenosis. At follow-up extending to 16 years (median follow-up, 6.5 years), 6 patients are in New York Heart Association functional class I, and 1 patient is in class II.
Conclusions. In view of the dismal natural history of untreated pulmonary vein stenosis, prompt surgical relief of the stenosis may be a rewarding undertaking.
| Introduction |
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| Material and Methods |
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Symptoms consisted of dyspnea (n = 8) and increased pulmonary infections (n = 5). In all patients, the diagnosis of pulmonary vein stenosis was made by cardiac catheterization (Figs 1, 2![]()
). In 2 recent patients, transesophageal echocardiography confirmed the diagnosis. Patients with pulmonary venous obstruction after a previous Mustard, Senning, or Fontan operation were excluded from this series.
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In 7 patients (patients 1 through 6 and 8), the pulmonary vein stenoses were approached through a right atriotomy and incision of the atrial septum. In 1 patient (patient 7), the stenotic right pulmonary veins were approached through a left atriotomy at the right interatrial groove.
Pulmonary Venous Obstruction After Correction of TAPVC
SUPRACARDIAC TYPE (PATIENT 1).
In this patient at age 3 months, a left vertical pulmonary vein was ligated, and the common pulmonary vein was anastomosed side-to-side to the left atrium; the ASD was closed with a Dacron patch. At reoperation 3 months later, the Dacron patch was covered with a thick layer of neointima and was contracted. In addition, excessive fibrous tissue was present at the right and left common pulmonary vein orifices. The right orifice measured only 2 mm and the left orifice, 5 mm. The Dacron patch was excised, the fibrous tissue at both common pulmonary vein orifices was resected, and lateral incisions were made from both orifices to the individual superior and inferior pulmonary veins. The ASD, atrial septum, and right atriotomy were closed with a generous autologous pericardial patch (Fig 3
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Five months later, increasing dyspnea developed. Angiography demonstrated severe stenoses of the orifices of both right pulmonary veins. At reoperation at 7 months, there was dense fibrosis of the mediastinum. The previously inserted pericardial baffle had contracted, and the flow pathway between the right pulmonary veins and the left atrium was atretic. Stenotic orifices of the right pulmonary veins were dilated from 12 to 18 mm. A generous patch of preserved dura mater was used to construct a new baffle from the right pulmonary veins to the ASD and left atrium. An 18-mm probe could be passed beneath the baffle. A second generous patch of dura mater was used to enlarge the superior vena cava at its entrance into the right atrium.
In patient 3, the left lower pulmonary vein and the right pulmonary veins drained into the coronary sinus, and the left upper pulmonary vein drained into a left vertical vein. At the primary operation, the roof of the coronary sinus was excised to connect the coronary sinus to the left atrium. A pericardial patch was then sewn over the mouth of the coronary sinus and ASD so as to direct the coronary sinus and pulmonary venous blood to the left atrium [25]. At reoperation, there was a stenosis at the common pulmonary vein entrance to the left atrium. The ostium of the common pulmonary vein was incised toward the right to the level of entry of the individual right superior and inferior pulmonary veins; both veins then admitted 6-mm probes. The left lower lobe vein was freed so that it also admitted a 6-mm probe. The atrial septum was closed with an autologous pericardial patch.
INFRACARDIAC TYPE (PATIENTS 4 AND 5).
In patient 4, the primary operation consisted of anastomosis of the vertical segment of the common pulmonary vein to the left atrium and closure of the ASD. At reoperation, the anastomosis was enlarged by incising it to the right and closing the atrial septum with an autologous pericardial patch. In addition, there was a stenosis at the orifice of the left upper pulmonary vein; this was dilated. The descending vein that communicated with the portal vein was still patent; therefore it was ligated.
Patient 5 had primary repair of infracardiac TAPVC and closure of the ASD at 12 days of age elsewhere. At reoperation, the severely stenotic anastomosis between the common pulmonary vein and the left atrium was enlarged by excising the common septum between the left atrium and the right pulmonary veins. A residual ASD was closed with a Dacron patch.
Congenital Pulmonary Vein Stenosis
Patient 6 had stenosis of the left upper pulmonary vein (see Fig 1
), a small ASD, and a small perimembranous VSD. At operation when he was 10 months of age, the stenotic orifice of the left upper pulmonary vein was dilated until it was the same size as the other pulmonary vein orifices. The ASD and VSD were closed by sutures.
Patient 7 had transposition of the great arteries, subarterial VSD, patent ductus arteriosus, and an ASD. At another institution when she was 2 weeks of age, the patient had undergone ligation of the patent ductus arteriosus through a left thoracotomy and subsequently a Blalock-Hanlon atrial septectomy and creation of a right modified Blalock-Taussig shunt through a right thoracotomy. At reoperation at our institution when she was 1 year of age, the orifices of both right pulmonary veins had fibrotic stenoses (see Fig 2
). The fibrous tissue was resected, and the orifices of both right pulmonary veins were incised and enlarged with an autologous pericardial patch (Fig 4
). An arterial switch operation with a Lecompte maneuver, patch closure of the VSD, and suture closure of the ASD were then performed.
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A right atriotomy was made with a T extension posteriorly into the left atrium and another extension medially into the atrial septum. The atrial septal incision was extended laterally across the right lower pulmonary vein stenosis and distally to the lower pulmonary vein bifurcation, which received blood from the middle and right lower lobes. The entire area was enlarged with an autologous pericardial patch. The initial diameter of the right lower pulmonary vein was 13.5 mm (143 mm2), and the diameter after patch enlargement was 16.5 mm (214 mm2), a 50% increase in cross-sectional area. The patch was carried up to the level of the atrial septum, the atrial septal incision was closed with a second patch of autologous pericardium, and the first patch was then continued up to close the T extension of the right atriotomy.
Histologic examination of the right middle lobe biopsy sample showed severe, widespread pulmonary venous and arterial obstructions caused by intimal smooth muscle proliferation, fibrosis, and old, partially recanalized thrombi (Fig 5
). These findings are consistent with constriction of pulmonary vessels by mediastinal or pulmonary hilar fibrosis.
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| Results |
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Follow-up for all 8 patients was complete and ranged from 2 months to 16 years (median follow-up, 6.5 years; mean follow-up, 7.5 years). One Eskimo patient (patient 3) died at home at the age of 5 months, 2 months after operation. A postmortem examination was not performed.
One patient (patient 2) who underwent incomplete repair of TAPVC elsewhere at the age of 1 year and who, 25 years later, had completion of the repair at our institution, at last follow-up had severe pulmonary hypertension with a pulmonary artery pressure of 125/55 mm Hg, a right ventricular pressure of 125/25 mm Hg, and a pulmonary capillary wedge pressure of 29 mm Hg. Angiography showed adequate patency of the pulmonary veins. The patient is being treated with calcium-channel blockers and digoxin and is currently in New York Heart Association functional class II. The other 6 patients are in functional class I. Four of these patients had echocardiographic follow-up (3 by transesophageal echocardiography), which showed adequate patency of the pulmonary veins.
| Comment |
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The reported incidence of pulmonary venous obstruction after repair of TAPVC ranges up to 18% [1124]. Symptomatic obstruction usually occurs within the first 6 months after repair of TAPVC, although 3 of the 5 patients in our series were seen late (at 2, 8, and 25 years). Pulmonary venous obstruction after repair of TAPVC can be caused by anastomotic stenosis secondary to technical failure, differential growth of the venous anastomosis and the pulmonary vein involved, neointimal tissue overgrowth on the surface of an artificial patch, or intimal hyperplasia within the individual pulmonary veins unrelated to the operative site [14, 24]. Jenkins and colleagues [26] reviewed the size of the individual pulmonary veins and related this to outcome in infants less than 4 months old at presentation. They found that the sum of the diameters of the individual pulmonary veins indexed for body surface area was significantly larger in survivors without obstruction than in patients who died without operation, those in whom obstruction developed, or those who died late. They concluded that size of the pulmonary veins is an important determinant of outcome in patients with TAPVC.
In an autopsy study, Haworth and Reid [27] examined in detail the pulmonary vasculature in infants with TAPVC and pulmonary venous obstruction. They observed increased wall thickness (intimal and medial hypertrophy) of arteries and veins, arterialization of veins, and abnormal extension of smooth muscle cells into the small intra-acinar arteries. These findings have been confirmed by other studies [2830] and are identical to the classic histologic findings of chronic pulmonary venous hypertension (as present, eg, in mitral stenosis) [31]. Although it appears that the pulmonary venous changes that occur with TAPVC are reversible if corrected at an early age and pulmonary artery pressure can normalize [32], the thick-walled extrapulmonary veins may not grow and may undergo further intimal proliferation and fibrosis [28]. Presumably, such stenoses are becoming more apparent now that more infants are surviving primary repair of TAPVC [2123].
One patient in this report had a different form of acquired pulmonary vein stenosis, ie, idiopathic mediastinal fibrosis, which is known to be capable of involving pulmonary veins as well as any other mediastinal structure [33, 34]. The disease had progressed rapidly, and the only source of pulmonary venous return to the left atrium was through a stenotic right lower lobe pulmonary vein. Although the etiology of mediastinal fibrosis is frequently unknown, at one time, tuberculosis and syphilis were suspected causes [33]. It is also a well-documented sequela of exposure to Histoplasma capsulatum [34].
Generally, the prognosis for patients with congenital or acquired pulmonary vein stenosis is poor, particularly if all veins are affected [110, 14, 23, 24, 34]. Although progression of symptoms may be less rapid if only one or two pulmonary veins are involved [10], progression to bilateral pulmonary vascular disease and death is usually the rule, even in initially less severe cases [19, 24].
The incidence of anastomotic stenoses after repair of TAPVC can be reduced by the creation of generous anastomoses between the pulmonary venous structures and the left atrium; in this regard, the open technique of anastomosis is preferable to the use of clamps [12]. In the infracardiac type of TAPVC, incision of the anterior surface of the vertical segment of the common pulmonary vein with extension through the confluence of the pulmonary veins (Y incision) allows a wide anastomosis to the left atrium, up to the tip of the atrial appendage [35]; in some cases, patching the atrial septum with autologous pericardium may be useful to increase the size of the left atrium. The use of absorbable suture (polyglyconate or polydioxanone) [36] or an interrupted suture technique [37] may decrease the incidence of anastomotic obstruction owing to less limitation of growth of the low-pressure anastomosis. Maneuvers that increase pulmonary venous pressure during operation for TAPVC, such as snaring of pulmonary veins, are best avoided [38]. No measures are currently available to prevent excessive fibrous tissue formation at the surgical sites, a cause of late pulmonary venous stenosis in some patients.
Two-dimensional echocardiography with pulsed Doppler examination and color-flow imaging provides an accurate method of identifying and localizing pulmonary venous obstruction and quantifying its degree [23]. Percutaneous balloon dilation and placement of currently available stents in stenotic pulmonary veins have usually provided only temporary relief [10, 39], but improved intravascular stents may offer better palliation in the future. The first reported successful surgical repair of congenital pulmonary vein stenosis was by Kawashima and co-workers [40] in 1971. In subsequent reports of surgical treatment of congenital pulmonary vein stenosis, the early and late mortality has been high [9, 10, 41, 42], and only sporadically have good early and intermediate-term results been reported [37, 43]. Both patients with congenital pulmonary vein stenosis in our series currently are in New York Heart Association functional class I with follow-up of 5 years and 10 years.
In mild pulmonary vein stenosis, intraoperative dilation may be all that is needed. We believe that it is preferable to use autologous pericardial tissue or atrial tissue for patch enlargement of severe pulmonary vein stenoses. Whenever possible, the use of artificial or allograft patches should be avoided, as they may induce neointimal formation with increased need of reoperation [20]. The techniques described by Pacifico and associates [37] for relief of pulmonary vein stenosis with the use of atrial wall or atrial septal tissue may be useful in select cases. If relief of pulmonary venous obstruction is not possible, it may be necessary to excise undrained pulmonary tissue to prevent irreversible pulmonary vascular disease [42]. In the case of congenital or acquired pulmonary vein stenosis that is beyond repair and that is associated with end-stage parenchymal lung disease, single- or double-lung transplantation may be indicated [44].
It summary, the results of this study suggest that prompt surgical relief of pulmonary venous obstruction, whether congenital in origin, caused by anastomotic stenosis or pulmonary vein hyperplasia after repair of TAPVC, or associated with mediastinal fibrosis, may lead to good long-term results.
| Acknowledgments |
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| Footnotes |
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| References |
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