Ann Thorac Surg 2003;75:275-277
© 2003 The Society of Thoracic Surgeons
Case report
Acquired pulmonary vein stenosis as a cause of life-threatening pulmonary hypertension
Sophie M. Jaillard, MDa*,
François R. Godart, MDb,
Thameur Rakza, MDc,
Arnaud Chanez, MDa,
Pierre Lequien, MDc,
Alain J. Wurtz, MDa,
Laurent Storme, MDc
a Department of Thoracic Surgery, University Hospital of Lille, Lille, France
b Department of Cardiology, University Hospital of Lille, Lille, France
c Department of Neonatology, University Hospital of Lille, Lille, France
Accepted for publication July 10, 2002.
* Address reprint requests to Dr Jaillard, Clinique de Chirurgie Thoracique, Hôpital Calmette, Centre Hôpitalier Régional et Universitaire de Lille, 59037 Lille, France.
e-mail: sjaillard{at}chru-lille.fr
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Abstract
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We report the case of an infant born prematurely at 27 weeks gestational age with life-threatening pulmonary hypertension crisis as a result of left upper pulmonary vein stenosis. Surgical treatment consisted of a lobectomy, which is a safe and effective procedure. Evidence strongly suggests that the venous stenosis may have resulted from hypertonic drugs infused through an umbilical catheter facing the upper left venous–atrial junction.
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Introduction
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Pulmonary vein stenosis (PVS) is a morphologic and functional narrowing of the junctional area between one or more pulmonary veins and the left atrium that causes obstruction of pulmonary blood flow and pulmonary hypertension. Pulmonary vein stenosis is either congenital, and usually associated with heart disease, or acquired, resulting from surgical treatment of total anomalous pulmonary venous connection or from venoocclusive disease or sclerosing mediastinitis. We describe an uncommon case of isolated PVS observed in a child.
The patient was a severely intrauterine growth-restricted premature newborn female infant (birth weight, 580 g; gestational age, 27 weeks), delivered by caesarean section because of maternal preeclampsia. Respiratory distress syndrome required nasal continuous positive airway pressure for 10 days. Echocardiogram at day two documented a left-to-right shunting through an atrial septal defect (3 to 4 mm) and a patent ductus arteriosus. No pulmonary hypertension was noted. The ductus arteriosus closed spontaneously at day 8. Weaning from oxygen was obtained at day 11. At day 24, the infant, weighing 605 g, was transferred to a referring hospital.
Two months later, cyanotic crisis and dyspnea were observed. Chest radiography showed mild cardiomegaly. Echocardiography showed right ventricular and pulmonary artery enlargement and right-to-left shunting at the level of the atrial septal defect. A tricuspid regurgitation was noted (gradient between right ventricle and right atrium, 92 mm Hg). Left ventricular ejection fraction was normal. Treatment with digoxin (15 µg/kg per day) and aldactazin (1.5 mg/kg per day) was started. Continuous oxygen therapy was required.
At age 5 months, echocardiography revealed an acceleration of the blood flow at the level of the left superior pulmonary vein, suggesting existence of a stenosis of this vessel. A subsequent cardiac catheterization was performed. The pulmonary artery pressure ranged from 70 to 150 mm Hg depending on the infant’s state of agitation. Pulmonary artery angiography revealed a decreased vascularization of the left upper lobe. On the selective left pulmonary artery opacification, contrast injection delineated the left pulmonary artery before being redirected retrogradely toward the right pulmonary artery. A venous angiogram did not clearly opacify the left upper vein (Fig 1A).
Isotope scan and cardiac magnetic resonance imaging further supported the diagnosis of stenosis of the superior pulmonary vein (Figs 1B, 1C).
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Fig 1. (A) Pulmonary venous angiogram: superior and inferior right and inferior left pulmonary vein opacification was immediate, although the superior left pulmonary vein opacification was slow, showing the small size of this vessel. (B) Perfusion scan demonstrated decreased perfusion of the left upper lobe. (C) Cardiac magnetic resonance imaging showed pulmonary artery (PA) diameter three times larger than aorta (Ao) diameter; the right superior pulmonary vein (PV) was clearly visualized, although the left superior vein was not visualized. Magnetic resonance imaging was performed without anesthetizing the infant and without cardiac gating. (LA = left atrium; LV = left ventricle.)
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At age 8 months (weight, 4470 g), an upper left lobectomy was performed because of life-threatening pulmonary hypertension crisis. At thoracotomy, the left upper lobe was enlarged and appeared heavy and congested. The anterior aspect of the left hilum and bronchial wall were covered with dilated lymphatic vessels. Gross and microscopic examination of the extrapericardial part of the vein was normal. Histologic examination revealed diffuse lymphangiectasis and hemosiderin deposition associated with venous distension in the pulmonary parenchyma. Inhaled nitric oxide, mechanical ventilation, and medical treatment were stopped at postoperative days 2, 3, and 7, respectively. At postoperative day 7, pulmonary artery pressure was decreased (gradient between right ventricle and right atrium, 22 mm Hg).
At age 2 years, clinical examination was normal. Control cardiac catheterization was performed. The pulmonary artery and mean pulmonary capillary pressures were 29/10 (18) mm Hg (systolic/diastolic/mean) and 8 mm Hg, respectively. Later follow-up was uneventful.
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Comment
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Pulmonary vein stenosis is a rare condition with poor prognosis, particularly if all veins are affected [1]. Hemoptysis or recurrent unilateral chest infections are typical presenting features during the first years of life [2]. Pulmonary vein stenosis frequently results in pulmonary artery hypertension [1], and in this case pulmonary hypertension signs were in the foreground. Congenital PVS is usually associated with heart disease [3], and acquired PVS may occur after surgical treatment of total anomalous pulmonary venous connection or may result from venoocclusive disease or sclerosing mediastinitis [3, 4]. The mechanism of pulmonary hypertension in PVS remains incompletely elucidated and is probably multifactorial [2, 5].
In our patient, the origin of the upper left PVS remains unclear. Congenital PVS cannot be ruled out. However, lack of pulmonary hypertension during the first few weeks of life does not support this hypothesis. Furthermore, no previous case of congenital stenosis of an isolated pulmonary vein revealed by pulmonary hypertension signs has been described in the literature. Some evidence suggests that the upper left PVS in our case may result from an iatrogenic complication. During the first day of life, resuscitation was performed using a double-lumen umbilical venous catheter (polyurethane, Arrow, Reading, PA) to transfuse red blood cells and infuse half-molar bicarbonate (4.2%). Retrospective analysis of the chest radiography revealed a misplaced catheter close to the superior pulmonary vein for 24 hours (Fig 2).
We speculate that infusion of hypertonic solution facing the venous–atrial junction may have injured the vessel wall and induced either a thrombotic lesion or an inflammatory response, causing fibrous thickening of the superior vein. Timing of the occurrence of pulmonary hypertension suggests that the PVS was acquired as observed after surgical treatment of the total anomalous pulmonary venous connection [3]. Location of the lesion (upper left PVS) strongly suggests that the PVS may result from hypertonic drug infusion facing the upper left venous–atrial junction.
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Fig 2. Chest radiography of the premature infant at day 1. The umbilical venous double-lumen catheter was introduced through the atrial septal defect into the left atrium, with its end close to the venous–atrial junction.
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Treatment is based on excision of undrained pulmonary tissue for the following reasons: (1) direct approach on the pulmonary veins is usually difficult, and (2) relentless progression of vein stenosis after surgical relief or endovascular stent implantation is common [1, 6, 7]. A sutureless in situ pericardial repair of the vein was a viable option, but this surgical procedure is more difficult to achieve [8].
This case further supports the hypothesis that a single PVS may induce life-threatening pulmonary hypertension. In this case, lobectomy is a safe and effective procedure.
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References
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- Bini R.M., Cleveland D.C., Ceballos R., et al. Congenital pulmonary vein stenosis. Am J Cardiol 1984;54:369-375.[Medline]
- Cullen S., Deasy P.F., Tempany E., Duff D.F. Isolated pulmonary vein atresia. Br Heart J 1990;63:350-354.[Abstract/Free Full Text]
- van Son J.A.M., Danielson G.K.D., Puga F.J., Edwards W.D., Driscoll D.J. Repair of congenital and acquired pulmonary vein stenosis. Ann Thorac Surg 1995;60:144-150.[Abstract/Free Full Text]
- Dye T.E., Saab S.B., Almond C.H., Watson L. Sclerosing mediastinitis with occlusion of pulmonary veins. Manifestations and management. J Thorac Cardiovasc Surg 1977;74:137-141.[Abstract]
- Holcomb R.G., Tyson W.R., Ivy D.D., Abman S.H., Kinsella J.P. Congenital pulmonary venous stenosis presenting as persistent pulmonary hypertension of the newborn. Pediatr Pulmonol 1999;28:301-306.[Medline]
- Coles J.G., Yemets I., Najm H.K., et al. Experience with repair of congenital heart defects using adjunctive endovascular devices. J Thorac Cardiovasc Surg 1995;110:1513-1520.[Abstract/Free Full Text]
- Sadr I.M., Tan P.E., Kieran M.W., Jenkins K.J. Mechanism of pulmonary vein stenosis in infants with normally connected veins. Am J Cardiol 2000;86:577-579.[Medline]
- Lacour-Gayet F., Zoghbi J., Serraf A.E., et al. Surgical management of progressive pulmonary venous obstruction after repair of total pulmonary venous connection. J Thorac Cardiovasc Surg 1999;117:679-687.[Abstract/Free Full Text]
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Abstract
Introduction
