HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Eric N. Mendeloff Thomas L. Spray Charles B. Huddleston Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Mendeloff, E. N. Articles by Mallory, G. B. Search for Related Content PubMed PubMed Citation Articles by Mendeloff, E. N. Articles by Mallory, G. B., Jr Related Collections Related Article

Ann Thorac Surg 1995;60:903-906
© 1995 The Society of Thoracic Surgeons

---

Original Articles: General Thoracic

Lung Transplantation for Congenital Pulmonary Vein Stenosis

Divisions of Cardiothoracic Surgery and Pediatric Cardiology, Washington University School of Medicine and St. Louis Children's Hospital, St. Louis, Missouri

	Abstract

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Background. Congenital pulmonary vein stenosis is a uniformly fatal disease when left untreated. Transcatheter techniques (for example, balloon dilation and stent placement) have proved to be only temporizing measures, and previous surgical attempts at treatment of this entity have provided little improvement and few survivors.

Methods. Over the last 4 years, 6 patients with congenital pulmonary vein stenosis have been treated at our institution, 3 of whom underwent bilateral sequential lung transplantation.

Results. The 3 patients who underwent bilateral lung transplantation are alive and well 6 to 24 months after transplantation. The other 3 died of complications of the disease before donor lungs became available.

Conclusions. Making the diagnosis of congenital pulmonary vein stenosis requires a high index of suspicion, and referral for lung transplantation should be made as soon as the diagnosis is reached. Lung transplantation has resulted in good-quality short to medium-term survival for 3 patients with this otherwise untreatable disease.

	Introduction

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
See also page 907.

Congenital stenosis of the pulmonary veins is a rare condition that is progressive and virtually always fatal [1]. Neither surgical nor transcatheter therapies have resulted in long-term relief of pulmonary vein stenosis or the associated pulmonary hypertension [1–4]. Here we review our experience with this entity and present the results of lung transplantation as treatment of pulmonary vein stenosis.

	Material and Methods

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Between July 1990 and August 1994, 6 children with congenital pulmonary vein stenosis were treated at our institution. The children ranged in age from 2 months to 2 years. Pulmonary artery pressures ranged from nearly systemic to suprasystemic at the time of evaluation, and all 6 patients had clinical and radiographic evidence of pulmonary congestion secondary to progressive pulmonary venous obstruction. Prior procedures included attempted repair of total anomalous pulmonary venous return with stenosis of the individual pulmonary veins in 1 infant and pulmonary vein stenting in 2 children (by an open procedure in 1 and by a transcatheter procedure in the other). Five of the 6 patients required critical levels of support at the time of evaluation at our institution, including 2 infants who were supported with extracorporeal membrane oxygenation (ECMO); 5 were listed for lung transplantation, and 3 underwent successful bilateral sequential lung transplantation.

Clinical Summaries
Clinical data on the 6 patients are summarized in Table 1.

PATIENT 2.
Patient 2 was seen at age 3 months with congestive heart failure. Echocardiography and cardiac catheterization suggested the diagnosis of partial anomalous pulmonary venous return with pulmonary vein stenosis. At operation, the infant was found to have total anomalous pulmonary venous return with all four veins draining to the right atrium, and stenosis of the right and left upper pulmonary veins. There was, in addition, a small atrial septal defect, which was enlarged to allow the pulmonary venous return to be baffled to the left atrium. The right upper pulmonary vein was dilated with Hegar's dilators, and the left upper pulmonary vein was incised longitudinally and then closed transversely at the venoatrial junction.

The patient was discharged home after an uneventful postoperative course but returned 1 month later with tachypnea, cyanosis, and poor perfusion. An echocardiogram demonstrated a dilated, hypertensive right ventricle, and cardiac catheterization confirmed pulmonary hypertension and recurrent stenosis of the upper pulmonary veins. After cardiac catheterization, the patient had persistent hemodynamic instability and recurrent pulmonary hypertensive crises, one of which resulted in cardiac arrest from which she could not be resuscitated. At postmortem examination, she was found to have complete occlusion of the left upper pulmonary vein and hypoplasia of the right upper pulmonary vein as well as chronic hypertensive changes in the pulmonary vascular bed.

PATIENT 3.
Patient 3 was seen at 5 months of age with severe respiratory distress and diffuse interstitial infiltrates on the chest roentgenogram. She was intubated for respiratory failure and treated for pneumonia. As her condition continued to deteriorate, an open-lung biopsy was performed, which was not diagnostic. Cardiac catheterization revealed a stenosed left lower pulmonary vein with the remainder of the pulmonary venous return draining to the left atrium through a plexus of uniformly small veins. A stent was placed in the left lower pulmonary vein using transcatheter technique, which resulted in enough improvement to allow temporary extubation. However, the patient was reintubated for respiratory failure and hemodynamic instability and at that point was referred for lung transplantation evaluation. Her condition continued to be extremely unstable after she was transferred to our hospital despite attempts at treatment with pressor agents and mechanical ventilation; 1 week after referral, she had a pulmonary hypertensive crisis leading to cardiac arrest and death.

PATIENT 4.
Patient 4 was seen at 10 weeks of age with weight loss and progressive respiratory distress. A chest roentgenogram revealed diffuse pulmonary opacities. Echocardiographic and Doppler evaluation revealed a dilated, hypertrophied right ventricle, an atrial septal defect with right-to-left flow, and normally connected pulmonary veins with high-velocity flow. The patient was transferred to St. Louis Children's Hospital where the diagnosis of pulmonary vein stenosis involving all four pulmonary veins was confirmed by cardiac catheterization. Angiography demonstrated uniformly small pulmonary veins with an additional discrete stenosis at the venoatrial junction. He was listed for lung transplantation and supported with mechanical ventilation and pressor agents over a period of several days, during which time cyanosis and hemodynamic instability increased. Palliative transcatheter pulmonary vein dilation was performed as a temporizing measure while suitable organs were sought.

PATIENT 5.
Patient 5 was seen at age 8 months with episodic cyanosis and radiographic evidence of pulmonary venous congestion. Echocardiographic evaluation followed by cardiac catheterization at an outside institution revealed pulmonary vein stenosis involving all four pulmonary veins. Pulmonary vein stenting was performed as an open procedure at 11, 12, and 16 months of age, with transient symptomatic improvement. However, by age 19 months, the patient had progressively more frequent and more severe episodes of cyanosis, and cardiac catheterization confirmed progression of the pulmonary vein stenosis. At age 2 years, she was referred for evaluation for lung transplantation and was found to be a suitable candidate.

PATIENT 6.
Patient 6 was seen at age 7 weeks with respiratory distress, mixed metabolic and respiratory acidosis, and impending circulatory collapse. The chest roentgenogram showed diffuse pulmonary opacities. The patient was resuscitated and underwent an extensive cardiac and pulmonary evaluation. An echocardiogram revealed a dilated, hypertensive right ventricle with right-to-left shunting across a patent foramen ovale and normally connecting pulmonary veins. The results of open-lung biopsy were consistent with pulmonary venous hypertension, and cardiac catheterization confirmed the diagnosis of pulmonary vein stenosis involving all four pulmonary veins. The veins were uniformly small throughout their length.

The patient was listed for lung transplantation; she required critical levels of support (neuromuscular blockade, mechanical ventilation, and pressor agents), and after one brief episode of severe hypotension, cyanosis, and bradycardia from which she was successfully resuscitated, she was supported with ECMO for a total of 24 days without incident. During this waiting period, she did not require neuromuscular blockade or aggressive support with pressor agents (Fig 1).

View larger version (268K): [in this window] [in a new window]   Fig 1. . (Patient 6.) (A) When seen, this patient had progressive dyspnea, profound hypoxemia, and acidosis requiring intubation. Chest roentgenogram is remarkable for bilateral interstitial infiltrates and a right-sided effusion. (B) After 24 days on extracorporeal membrane oxygenation, bilateral sequential lung transplantation was performed. This chest roentgenogram was obtained shortly after hospital discharge.

In the 1 child who had undergone previous stenting of the pulmonary veins, the presence of these stents precluded placement of a clamp on the adjacent left atrium. Removal of these intravascular metallic stents required the use of cardioplegic arrest of the heart to perform an open excision of the pulmonary venous entrances into the left atrium. The anastomoses were then created to direct openings in the left atrium using absorbable suture.

	Results

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
All but 1 of these 6 patients required critical levels of support in an intensive care unit, and 2 were supported with ECMO while waiting for organs after being listed for lung transplantation. Of the 6, 3 died without undergoing transplantation, 1 of sequelae of hemodynamic compromise and complications of ECMO (patient 1) and 2 of pulmonary hypertensive crises (patients 2 and 3). The average time from diagnosis to death was 43 days.

The remaining 3 patients, 1 of whom was supported with ECMO for 24 days while waiting for organs, had bilateral sequential lung transplantation. Details of the clinical courses of these patients have been previously reported [5]. Size-matched donors were found for 2 of them, who thus received two whole lungs. The remaining patient received bilateral lower lobes only from a larger donor; the chest could not be closed at the time of transplantation but was closed on the tenth postoperative day. The average hospital stay after transplantation was 42 days. All of the patients who underwent transplantation are alive and well 0.5 to 1.8 years after transplantation. None have echocardiographic evidence of recurrent pulmonary hypertension; 1 patient (patient 6) has mild bilateral stenosis of the bronchial anastomoses.

	Comment

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Congenital pulmonary vein stenosis presenting in infancy is a rare entity, accounting for 0.4% to 0.6% of cardiac anomalies in autopsy series [2, 5, 6]. Typically, infants are seen in the second or third month of life and have rapid and inexorable progression of the disease with death before 1 year of age. The disease is virtually always fatal when untreated [1]. Attempts at surgical correction have yielded poor results. A variety of surgical options aimed at treatment of this entity have been associated with a high rate of early postoperative recurrence of stenosis and eventual death from progressive pulmonary hypertension [2, 4].

The diagnosis of congenital pulmonary vein stenosis can be difficult, especially when there is normal pulmonary venous connection to the left atrium; one must have a high index of suspicion for the diagnosis if it is to be made in a timely fashion. Persistent respiratory symptoms in an infant unresponsive to appropriate antibiotic treatment, failure to identify an organism, and absence of left heart obstruction on echocardiograms suggest the possibility of pulmonary vein stenosis. The diagnosis is confirmed at cardiac catheterization by the following findings: pulmonary hypertension with or without elevated pulmonary capillary wedge pressure; absence of mitral stenosis (verified by direct measurement of left atrial pressure simultaneously with left ventricular end-diastolic pressure) or left ventricular outflow tract obstruction; presence of a gradient on pullback between one or more pulmonary veins and the left atrium; and angiographic evidence of pulmonary vein stenosis, hypoplasia, or both. Severe pulmonary hypertension with structural changes in the pulmonary arteries of all lobes can occur even when the pulmonary vein stenosis includes only one or two veins [7, 8]. The failure of attempts at localized surgical repair suggests that although the diagnosis is made on the basis of local morphologic findings, the disease process may involve the entire pulmonary venous system. Given the rapidly progressive nature of the disease and the uncertainty regarding the time in which a suitable donor may be identified, early diagnosis is crucial.

In some cases (as illustrated by patient 1), timely diagnosis and aggressive support are not sufficient to allow a patient to survive to lung transplantation. For others, early diagnosis and listing for transplantation may allow survival. Use of ECMO to support critically ill patients prior to the onset of severe hemodynamic compromise, as in the case of patient 6, can provide a relatively long period of hemodynamic stability while suitable organs are sought.

The 3 patients in this series who survived until organs became available were critically ill during that period; 1 of them spent 24 days on ECMO while awaiting organs. Nevertheless, in the short term after lung transplantation, these infants have done quite well. All of them are currently at home without the need of supplemental oxygen and without major neurologic impairment. Obviously, the long-term outcome remains uncertain, as it is for all patients who currently undergo lung transplantation.

In conclusion, lung transplantation has been an effective therapy for 3 of 6 infants and children with congenital pulmonary vein stenosis seen by us. The need of critical levels of support preoperatively did not preclude a good outcome in these patients, all of whom are well at short-term follow-up. Although long-term outcome from lung transplantation at a young age remains uncertain, the rapidly progressive and uniformly fatal course of the disease combined with the temporary and palliative nature of other forms of therapy leads us to recommend referral for lung transplantation as soon as the diagnosis of congenital pulmonary vein stenosis is made.

	Footnotes

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Presented at the Thirty-first Annual Meeting of The Society of Thoracic Surgeons, Palm Springs, CA, Jan 30--Feb 1, 1995.

Address reprint requests to Dr Mendeloff, St. Louis Children's Hospital, Suite 5W24, One Children's Place, St. Louis, MO 63110.

	References

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 

1. Driscoll DJ, Hesslein PS, Mullins CE. Congenital stenosis of individual pulmonary veins: clinical spectrum and unsuccessful treatment by transvenous balloon dilation. Am J Cardiol 1982;49:1767–72.[Medline]
2. Park SC, Neches WH, Zuberbuhler JR, Siewers RD, Bahnson HT. Diagnosis and surgical management of bilateral pulmonary vein stenosis. J Thorac Cardiovasc Surg 1974;67:755–61.[Medline]
3. Castaneda AR, Jonas RA, Mayer JE Jr, Hanley FL. In: Anomalies of the pulmonary veins. Cardiac surgery of the neonate and infant. Philadelphia: Saunders, 1994:157--66.
4. Bini RM, Cleveland D, Ceballos R, Bargeron LM, Pacifico AD, Kirklin JW. Congenital pulmonary vein stenosis. Am J Cardiol 1984;54:369–75.[Medline]
5. Bridges ND, Mallory GB Jr, Huddleston CB, Canter CE, Sweet SC, Spray TL. Lung transplantation in children and young adults with cardiovascular disease. Ann Thorac Surg 1995;59:813–21.[Abstract/Free Full Text]
6. Edwards JE. Congenital stenosis of the pulmonary veins: pathologic and developmental considerations. Lab Invest 1960;9:46–50.[Medline]
7. Geggel RL, Fried R, Tuuri DT, Gyler DC, Reid LM. Congenital pulmonary vein stenosis: structural changes in a patient with normal pulmonary artery wedge pressure. J Am Coll Cardiol 1984;3:193–9.[Abstract]
8. Reid JM, Jamieson MPG, Cowan DC. Unilateral pulmonary vein stenosis. Br Heart J 1986;55:599–601.[Abstract/Free Full Text]

This article has been cited by other articles:

				U. K. Chowdhury, B. Airan, A. Malhotra, A. K. Bisoi, A. Saxena, S. S. Kothari, M. Kalaivani, and P. Venugopal Mixed total anomalous pulmonary venous connection: Anatomic variations, surgical approach, techniques, and results J. Thorac. Cardiovasc. Surg., January 1, 2008; 135(1): 106 - 116. [Abstract] [Full Text] [PDF]

				U. K. Chowdhury, K. G. Subramaniam, K. Joshi, S. Varshney, G. Kumar, R. Singh, and P. Venugopal Rechanneling of total anomalous pulmonary venous connection with or without vertical vein ligation: Results and guidelines for candidate selection J. Thorac. Cardiovasc. Surg., May 1, 2007; 133(5): 1286 - 1294. [Abstract] [Full Text] [PDF]

				L. A. Latson and L. R. Prieto Congenital and Acquired Pulmonary Vein Stenosis Circulation, January 2, 2007; 115(1): 103 - 108. [Full Text] [PDF]

				E. J. Devaney, A. C. Chang, R. G. Ohye, and E. L. Bove Management of Congenital and Acquired Pulmonary Vein Stenosis Ann. Thorac. Surg., March 1, 2006; 81(3): 992 - 996. [Abstract] [Full Text] [PDF]

				J. P. Breinholt, J. A. Hawkins, L. Minich, L. Y. Tani, G. S. Orsmond, S. Ritter, and R. E. Shaddy Pulmonary vein stenosis with normal connection: associated cardiac abnormalities and variable outcome Ann. Thorac. Surg., July 1, 1999; 68(1): 164 - 168. [Abstract] [Full Text] [PDF]

				F. Lacour-Gayet, J. Zoghbi, A. E. Serraf, E. Belli, D. Piot, C. Rey, F. Marcon, J. Bruniaux, and C. Planche SURGICAL MANAGEMENT OF PROGRESSIVE PULMONARY VENOUS OBSTRUCTION AFTER REPAIR OF TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION J. Thorac. Cardiovasc. Surg., April 1, 1999; 117(4): 679 - 687. [Abstract] [Full Text] [PDF]

This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Eric N. Mendeloff Thomas L. Spray Charles B. Huddleston Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Mendeloff, E. N. Articles by Mallory, G. B. Search for Related Content PubMed PubMed Citation Articles by Mendeloff, E. N. Articles by Mallory, G. B., Jr Related Collections Related Article