Year           First Author

1894           Probyn-Williams               

Potential right pulmonary vein atresia

The gross pathological findings of potential right pulmonary venous atresia and potential anomalous left pulmonary venous connection were observed in an infant.

1937           Mallory                             

Intimal fibrosis of left atrial wall and pulmonary veins

A pathological process of pulmonary venous obstruction secondary to extension of intimal fibrosis of the left atrial wall extending into the pulmonary veins was observed in a male of 10 years.

1940          Hyndman                         

Experimental unilateral pulmonary venous occlusion is compatible with long-term survival

A canine pulmonary venous ligation model was used to study the effects of congestion on pulmonary tuberculosis. The authors observed that complete unilateral pulmonary venous occlusion was compatible with life in dogs. In 1965, Mudd and associates subsequently studied the bronchomorphometric effects of unilateral ligation of the pulmonary veins in dogs. Many dogs survived for 10 months after the intervention without developing abnormally increased pulmonary arterial pressures.

1942           Brody                                

Comprehensive review of anomalous pulmonary venous connections

All known reports of patients with partial and total anomalous pulmonary venous connections were reviewed and presented. No cases with potential intraluminal pulmonary venous obstruction were reported other than the case of Probyn-Williams.

1948           Lawrence                          

Pulmonary vein stenosis associated with constrictive pericarditis

The first known pediatric case of pulmonary vein stenosis associated with constrictive pericarditis, confirmed with a post-mortem examination, was report in a male of 14 years.

1951            Edwards                           

Pulmonary arterial changes associated with pulmonary vein stenosis

A female of 26 years with multi-lobar pulmonary venous obstruction due to an inflammatory mass and mediastinal process was reported. The pulmonary vascular histology showed proliferative thickening of the small arterial and arteriolar walls of the lobes with pulmonary venous obstruction. The lobe without venous obstruction had nearly normal small arterial and arteriolar walls. Only the lobe without venous obstruction showed radiographic evidence of pulmonary edema. The authors postulated that the occlusive arterial and arteriolar lesions in the lobes with severe venous obstruction protected the pulmonary capillaries from excessive passive congestion and from elevated pressure.

1951            Reye                                 

Primary stenosis of pulmonary veins with normal left atrial connections

A female of 8 years who died following a long history of cardiovascular symptoms was reported. Her autopsy showed severe ostial narrowing of the left upper, left lower, and right lower pulmonary veins. The right upper pulmonary vein was present but did not connect with the left atrial wall. There was evidence of intraluminal stenosis of the left upper extraparenchymal pulmonary vein. The extraparenchymal right lower and left lower pulmonary veins were normal in size. Reye proposed the pulmonary venous narrowing was present since birth, or congenital.

1955           Emslie-Smith                    

Heart catheterization performed before the death of an adolescent with pulmonary vein stenosis

A female of 14 years who died with occlusion of the right pulmonary veins and a moderate patent ductus arteriosus (5 mm diameter) with a right to left shunt was reported. She had problems with cyanosis since an age of 2 months. She underwent heart catheterization shortly before her death. The pulmonary arterial pressures were slightly greater than the aortic pressure; however, reliable pulmonary arterial wedge pressures were not measured. Her autopsy showed a normal common left pulmonary vein. There was membranous obstruction of the right upper pulmonary vein with two tiny fenestrations. The right lower pulmonary vein had no connection with the left atrium. Both extraparenchymal right pulmonary veins had a normal outer diameter but felt hard and their walls were 2-3 times thicker than the left extraparenchymal veins.

1957           Andrews                           

Pulmonary vein stenosis associated with pulmonary interstitial fibrosis

Histological evidence of pulmonary interstitial fibrosis in 4 adults and one child with various causes of pulmonary venous obstruction was observed. Left-sided pulmonary interstitial fibrosis was observed in a female of 3 years with a functional single ventricle. Her right pulmonary veins were normal. The left pulmonary veins had an appearance of fibrous cords.

1958           Diamond                          

Pulmonary vein stenosis associated with pulmonary interstitial fibrosis

A child was followed from 1 to nearly 4 years of age with pulmonary interstitial fibrosis of the lung, right greater than left (potential Hamman-Rich syndrome). The right pulmonary veins were narrow with no opening into the left atrium. It is unclear whether interstitial fibrosis or pulmonary venous obstruction was the primary process.

1958           Sherman                          

Proposal that surgical correction of primary pulmonary vein stenosis may be feasible

An infant male of 8 months with congenital stenosis of the right upper, right lower and left lower pulmonary veins at their junction with the left atrium was reported. The right middle and left upper pulmonary veins were widely patent. The authors proposed that surgical correction might be feasible.

1959           Bernstein                          

Angiography of the right heart and pulmonary circulation performed before death

A male of 6 years with occlusion of the right upper pulmonary vein and stenosis of the right lower and left upper pulmonary veins was reported. An angiogram showing the right heart and pulmonary circulation was performed before the child’s death.

1962           Shone                               

Angiography and hemodynamic measurements before death

Angiography and hemodynamic measurements were performed in a male infant of 5 months with ostial narrowing of all pulmonary veins. The right pulmonary arterial pressure was 170/80 mmHg. A reliable pulmonary arterial wedge pressure could not be measured. The areas of pulmonary vein stenosis appeared to result from fibrous thickening of the intima.

1967           Contis                               

Intimal hyperplasia observed in the upstream intrapulmonary veins

A male of 11 years who had ostial narrowing of the right upper, left upper and left lower pulmonary veins was reported. The authors compared the size of the child’s extraparenchymal veins to the size of veins of 20 other children who died without pulmonary vein obstruction. They reported the presence of thrombus in two of the child’s narrow pulmonary veins. They reported hyperplasia of elastic tissue and intimal hyperplasia in the upstream intrapulmonary veins.

1967           Levine                               

Atresia of the common pulmonary vein

An infant male of 3 weeks with atresia of the common pulmonary vein was reported. There was an atretic strand between the left atrium and the confluence of pulmonary veins.

1968           Rywlin                               

Pulmonary lymphangiectasia associated with pulmonary vein atresia

The presence of pulmonary lymphangiectasia was observed in a newborn female with a blind common pulmonary vein.

1968           Warren                             

Role of measuring pulmonary arterial wedge pressure in diagnosing pulmonary venous obstruction

A male of 5 years who developed stenosis of the anastomosis between the pulmonary veins and the left atrium following repair of total anomalous pulmonary venous return to a persistent left superior vena cava was reported. A diagnosis of pulmonary venous obstruction was based upon a large difference between the measured pulmonary arterial wedge pressure and the left atrial pressure.

1969           Stevens                            

Types of pulmonary venous obstruction after experimental lung transplantation

Three causes of pulmonary venous obstruction following lung transplantation in dogs were described. Acute and intermediate venous obstruction were associated with thrombosis of the vessels. Chronic venous obstruction was associated with atrial cuff fibrosis or pulmonary venous stenosis.

1971            Kawashima                       

First successful repair of bilateral primary pulmonary venous obstruction

An adolescent male underwent the first successful repair of bilateral pulmonary venous obstruction involving the right upper and left common pulmonary veins using cardiopulmonary bypass. The right upper venous membrane was resected, and the opening of the left common pulmonary vein was widened by incising the thickening at two opposing points. Atrial and ventricular septal defects were also closed surgically. The report included a follow up period of 7 months.

1971            Friedli                               

Intraluminal pulmonary vein stenosis observed after repair of total anomalous pulmonary venous connection

The initial case of potential intraluminal pulmonary vein stenosis following repair of total anomalous return below the diaphragm with obstruction was reported. At the time of emergent surgical repair following birth, the pulmonary venous confluence appeared thick, narrow and somewhat inflamed. The anastomosis to the left atrium was revised at 2 months of age due to nearly complete obstruction by fibrosis. During a second operation for pulmonary venous obstruction at 3 months of age, dense scarring was noted around the ostia of common right and common left pulmonary veins measuring approximately 1.5 mm in diameter. The ostia were incised, the scarring was resected and a new anastomosis of each pulmonary vein to the left atrium was created. The report included a follow up period of approximately 3 months.

1971            Becker                              

Collateral bronchial veins associated with pulmonary vein stenosis

An evaluation of 13 infants with congenital stenosis of the pulmonary veins identified dilation of the bronchial veins within the lung parenchyma, potentially serving as collaterals to pulmonary venous obstruction.

1972           Binet                                 

Repair of pulmonary vein stenosis without cardiac bypass

A female child underwent successful repair of pulmonary vein stenosis without cardiac bypass. Membranes obstructing the upper and lower right veins were excised. The venous branches and right common pulmonary vein were augmented with a patch of autologous pericardium. The report included a follow up period of 6 months.

1976           Anderson                         

Potential intraluminal pulmonary vein stenosis after repair of partial anomalous pulmonary venous return

The initial case of potential intraluminal pulmonary vein stenosis following repair of partial anomalous return of a right common pulmonary vein to the right atrium using a pericardial tunnel was reported in a male of 7 years. Right pulmonary venous obstruction developed within 6 months. At the time of additional corrective surgery, the entrance of the right pulmonary vein into the pericardial tunnel was found to be reduced to a 3 mm opening.

1978           Whight                             

Additional cases of potential intraluminal stenosis of pulmonary veins after repair of anomalous pulmonary venous return

Two cases of potential intraluminal stenosis of pulmonary veins following repair of total anomalous pulmonary venous return to the coronary sinus were reported. In 1978, Katz also reported a case of potential intraluminal pulmonary vein stenosis following repair of total anomalous pulmonary venous return. The patient required reoperation to excise thickened endocardium that had developed at the ostia of the pulmonary veins but not at the anastomosis itself. In 1980, Turley reported 2 patients who developed potential intraluminal pulmonary vein stenosis following repair of total anomalous pulmonary venous return below the diaphragm. Intimal fibroplasia of the pulmonary veins was noted proximal to the site of atrial anastomosis. Both patients developed recurrent stenosis and died after attempted patch augmentation of the affected veins. Including the reports of Friedli, Anderson, Whight, and Katz, 7 patients had now been reported with potential intraluminal pulmonary vein stenosis following repair of anomalous pulmonary venous return. These reports helped confirm that intraluminal pulmonary vein stenosis is a genuine postoperative complication of surgery.

1981           Massumi                           

Recurrent stenosis after balloon dilation of pulmonary vein stenosis

A female of 35 years with stenosis of the right upper, right lower, and left upper or common pulmonary veins attributed to sclerosing mediastinitis was reported. The veins were treated with balloon dilation. She died after a period of 9 months with clinical evidence of recurrent pulmonary vein stenosis.

1982           Khonsari                           

First successful repair of common pulmonary vein atresia

The successful repair of common pulmonary vein atresia was performed in a newborn male at 7 days of age. The report included a follow up period of 15 months without symptoms and normal pulmonary arterial pressure confirmed by heart catheterization.

1982           Bini

Role of pulmonary wedge angiograms in the diagnosis of pulmonary vein stenosis

A female of several years who developed severe stenosis of the left pulmonary vein following repair of total anomalous pulmonary venous return during infancy was reported. A pulmonary arterial wedge angiogram defined the site of pulmonary venous obstruction better than a pulmonary arterial angiogram.

1982           Haworth

The pulmonary venous pathology of 6 newborns with obstructed return of anomalous pulmonary veins during the first week of life was evaluated. She observed changes in the extraparenchymal and intrapulmonary veins including, increased vein wall thickness, generally small extrapulmonary veins, and intimal proliferation. She proposed that these changes may predispose to the later development of pulmonary vein stenosis despite a successful surgical repair.

1984           Lock

Unsuccessful balloon dilation of pulmonary vein stenosis

Unsuccessful attempts at balloon dilation were performed in 5 children congenital, acquired or postoperative pulmonary vein stenosis.

1984           Warden

Warden procedure

A method for repair of partial anomalous pulmonary venous connection to the superior vena cava was described. The Warden procedure has potentially decreased the risk of postoperative stenosis of the anomalous veins.

1984           Bini

Poor results associated with surgical repair of congenital pulmonary vein stenosis

Eight children underwent surgical repair of congenital pulmonary vein stenosis. Despite some relief of the obstruction, all patients developed relentless progression of pulmonary vein stenosis and died.

1985           Pacifico

Use of living autologous atrial tissue in the repair of congenital pulmonary vein stenosis

The successful use of living autologous atrial tissue in the repair of congenital stenosis of the pulmonary veins in two infants was reported with a follow up period of 3 months for both infants.

1985           Smallhorn

Role of Doppler echocardiography in the assessment of pulmonary venous obstruction

The role of pulsed Doppler echocardiography in the assessment of pulmonary venous obstruction was described.

1988           Fong

An early classification system of pulmonary vein stenosis

An early classification system of pulmonary vein stenosis was proposed. The authors identified patients with tubular hypoplasia of the extrapulmonary veins and hourglass narrowing of the extrapulmonary veins at the veno-atrial junction.

1990           Miki

Use of pedicled flaps of right atrium and atrial septum to repair total anomalous pulmonary venous connection to the right atrium

Pedicled flaps of right atrium and atrial septum were used to repair a female of 3 months with total anomalous pulmonary venous connection to the right atrium. The authors proposed that this method may decrease the risk of late pulmonary venous obstruction. In 1998, Aeba reported a similar technique in 4 infants with no mortality and no postoperative pulmonary venous obstruction.

1990           LaBourene

Pulmonary vascular pathophysiology associated with banding the pulmonary veins of young pigs

Hemodynamic and histological findings were reported in a piglet model of pulmonary vein stenosis. Three weeks after banding pulmonary veins, an increased pulmonary arterial pressure occurred, unaccompanied by a rise in pulmonary arterial wedge pressure. There was breakdown of the internal elastic lamina in the banded pulmonary veins with apparent migration of smooth muscle cells from media to subendothelium. After 6 weeks, an increase in pulmonary arterial wedge pressure and a further increase in pulmonary arterial pressure were observed. The authors also observed mild intimal thickening of banded pulmonary veins, complete degradation of elastic laminae, and an increase in collagen.

1991           O’Laughlin

Transient improvement pulmonary vein stenosis after placement of stents

The use of endovascular stents in 30 patients with congenital heart disease was reported. One of the patients had pulmonary vein stenosis following repair of total anomalous pulmonary venous return. Stents were placed in right and left pulmonary veins. The infant transiently improved; however, developed stenosis in the upstream intrapulmonary veins within a follow up period of 3 months. Similar reports of stent placement in pulmonary veins and restenosis in children were reported by other institutions in 1993 and 1995.

1992           Wilson

Use of interrupted sutures and pericardial patch augmentation in the repair of total anomalous pulmonary venous connections

The use of interrupted sutures and a patch of pericardium to augment the anastomosis to decrease mortality and postoperative pulmonary vein stenosis when repairing total anomalous pulmonary venous connections was reported.

1993           Kim

Use of magnetic resonance imaging in pulmonary vein stenosis

The use of magnetic resonance imaging in two children was reported. This modality complimented the findings of lung perfusion scans, heart catheterization, and invasive angiography.

1993           Jenkins

Mortality of total anomalous pulmonary venous connection repair associated with small sum of individual vein diameters

A retrospectively study was performed to evaluate the size of individual pulmonary veins and venous confluences in the preoperative echocardiograms of children with total anomalous pulmonary venous connection. Seventy-five percent of late deaths were associated with stenosis of pulmonary veins remote from the anastomosis site. They observed that a small sum of individual vein diameters was associated with mortality. This observation was validated by a subsequent report by Bando in 1996. In 2004, Ando reported that patients with total anomalous pulmonary venous connections with dysmorphic pulmonary venous confluence or tributary veins had a higher incidence of postoperative pulmonary venous obstruction. 

1993           Dudell

Use of extracorporeal membrane oxygenation in the management of common pulmonary vein atresia

Extracorporeal membrane oxygenation was used in the management of 5 patients with atresia of the common pulmonary vein. The authors cited a dismal probability of survival for this lesion based upon past publications. Two of their 3 patients who were managed with extracorporeal membrane oxygenation survived repair of common pulmonary vein atresia. The report included a follow up period of 8 to 18 months.

1994           Spray

Treatment of congenital pulmonary vein stenosis with lung transplantation

Lung transplantation was performed in 33 children. Two of the patients were infants with congenital pulmonary vein stenosis. This institution subsequently published their mid-term experience in these patients and a third infant in 1995 with a follow up period of 6 to 22 months.

1996           Lacour-Gayet

Initial report of a sutureless repair for right pulmonary vein stenosis

The first “sutureless repair” of right pulmonary vein stenosis in a male of 2 years using in situ pericardium was reported. The child developed stenosis of the right upper, right lower and left lower pulmonary veins following urgent repair of infradiaphragmatic total anomalous pulmonary venous connection. The stenosis recurred following enlargement of each ostium by endarterectomy. The length of right extrapulmonary vein narrowing was approximately 10 mm. Each right pulmonary vein was opened from the ostium upstream to healthy tissue. The in situ pericardium was then folded over the atrial mass and sutured to the lower edges of the pulmonary veins and right atrium analogous to the Senning procedure. The left lower pulmonary vein was again treated by endarterectomy. Six months following the procedure, there was no right pulmonary venous obstruction and moderate left lower pulmonary venous obstruction.

1998           Najm

Initial report of sutureless repair for right and left pulmonary vein stenosis

The repair of right and left pulmonary vein stenosis using a sutureless technique with in-situ pericardium was reported in two infants who developed bilateral pulmonary vein stenosis following repair of total anomalous pulmonary venous connections. The method included an incision in the left atrium and extended into the pulmonary venous ostia separately, which can be carried into the secondary, and if necessary, the tertiary branches of the veins to a level at which the intima appears grossly normal. The pericardium overlying the entrance of the veins was used to create an enlarged communication between the opened veins and the left atrium. The neoatrium was created by suturing the pericardium to the epicardium of the left atrium, completely circumscribing the opening in the left atrium and veins so that the pulmonary venous effluent was contained by pericardium. There was only mild residual or recurrent pulmonary vein stenosis in each child with follow up periods of 12 to 15 months.

1998           Caldarone

Worse outcome of pulmonary vein stenosis in the setting of bilateral disease

A series of patients who developed pulmonary veins stenosis following repair of total anomalous pulmonary venous connections was reported. Survival was worse in patients who developed bilateral pulmonary vein stenosis.

1999           Breinholt

Association of pulmonary vein stenosis with congenital heart defects

Stenosis of pulmonary veins with normal left atrial connections was associated with congenital heart defects. The authors also observed a higher mortality in patients with more than 2 stenotic pulmonary veins.

2000          Sadr

Histological evaluation indicating that recurrent obstruction appears to be due to myofibroblastic proliferation

Microscopy, immunohistochemistry, and cell culture to explore the mechanism of restenosis in 4 infants with isolated pulmonary vein stenosis. The authors reported that recurrent obstruction appears to be due to myofibroblastic proliferation. Light microscopy identified intimal spindle cell proliferation leading to luminal obstruction in all specimens. The spindle-shaped proliferative cell identified on light microscopy contained ultrastructural elements usually found in myocytes and fibroblasts. Myofibroblasts are a progenitor cell for myocytes and fibroblasts. This report potentially led to subsequent use of the term “intraluminal pulmonary vein stenosis” in subsequent publications beginning with Goff and associates in 2009.

2001           Heyneman

Use of CT angiography to image pulmonary vein stenosis

Cross-sectional imaging with CT angiography was used to identify unilateral pulmonary vein atresia in 3 adult patients.

2001           Ungerleider

Successful alleviation of pulmonary vein stenosis in 1 child using intraoperatively placed stents

Successful alleviation of pulmonary vein stenosis was observed in 1 of 5 children using intraoperatively placed stents.

2001           Videlefsky

Use of magnetic resonance phase-shift velocity mapping in pulmonary venous obstruction

Magnetic resonance phase-shift velocity mapping was used in 7 children and adolescents with pulmonary venous obstruction. In the absence of any associated left-to-right shunt lesions, pulmonary venous blood flow velocities of greater than or equal to 100 cm/s indicated significant obstruction.

2001           Shiraishi

Use of helical computed tomographic angiography to reliably image pulmonary vein stenosis

Helical computed tomographic angiography was used to reliably image pulmonary vein stenosis in an infant following repair of total anomalous pulmonary venous drainage.

2002          Sacco

Successful treatment of unilateral pulmonary vein atresia with implantation of a stent

The first successful treatment of unilateral pulmonary vein atresia with implantation of an endovascular stent was reported. The atretic obstruction was perforated with the stiff end of the guidewire. The report includes a follow up period of 21 months including angiography to confirm a patent stent.

2002          Greil

Gadolinium-enhanced 3-dimensional magnetic resonance angiography in pulmonary vein stenosis

Gadolinium-enhanced 3-dimensional magnetic resonance angiography was able to rapidly and accurately diagnose a wide spectrum of pulmonary and systemic venous anomalies in children and adults. The report included 15 patients with pulmonary vein stenosis.

2003          Ricci

Impact of early presentation and the presence of postoperative pulmonary hypertension

Early presentation and the presence of postoperative pulmonary hypertension had the greatest adverse impact on outcome for pulmonary vein stenosis following repair of total anomalous pulmonary venous connections.

2003          Tomita

First group to report the favorable outcome of stent implantation and the role of repeat dilation

A favorable outcome of stent implantation and subsequent re-dilation in 7 veins of 4 children with stenosis of normal atrial connections or anomalous connections following repair was reported. Lesion diameters increased from 0.8 mm to 3.6 mm to 3.6 mm to 8.4 mm when stents were implanted. Re-stenosis occurred in all stents except for the vessel dilated to a diameter of 8.4 mm. Re-dilation resulted in a decrease in intimal thickness. Long-term patency was achieved in 2 veins dilated to a diameter of 5.6 mm and 8,4 mm. The authors proposed the final stent diameter within a vessel may determine long-term patency, and aggressive re-dilatation may be crucial for successful therapy. Subsequent reports by Bonello in 2015, Cory in 2017, and Kurita in 2019 also observed that reintervention was associated with improved survival and/or vein patency.

2003          McMahon

Sonotherapy to prevent re-stenosis

Sonotherapy was used in a male of 2 years who developed recurrent neointimal thickening following placement of stents to alleviate bilateral pulmonary vein stenosis following repair of total anomalous pulmonary venous return. Patency improved with a follow up period of 3 months when the stented lesions were treated with ultrasound sonotherapy to prevent re-stenosis.

2005          Roman

Use of phase-contrast magnetic resonance imaging to evaluate distribution of pulmonary blood flow

Phase-contrast magnetic resonance imaging was used to determine how pulmonary arterial blood flow is affected by pulmonary venous obstruction in children. The authors observed redistribution of pulmonary arterial flow and recommended that magnetic resonance imaging be used to assess the functional importance of an obstruction when investigating pulmonary vein stenosis.

2005          Yun

Outcomes with conventional and sutureless techniques for alleviating pulmonary vein stenosis in patients who have undergone repair of anomalous pulmonary venous drainage and normal venous connections

Outcomes of conventional and sutureless techniques for alleviating pulmonary vein stenosis were compared in patients who have undergone repair of anomalous pulmonary venous drainage and normal venous connections. When adjusted for pulmonary vein stenosis score, the sutureless repair was associated with a nonsignificant trend toward greater freedom from reoperation or death.

2006          Seale and McMahon

Cutting balloon dilation of pulmonary veins stenosis

At separate institutions, Seale and McMahon reported that cutting balloon dilation of pulmonary veins stenosis provided immediate, but limited sustained, relief of obstruction.

2006          Devaney

Outcomes of sutureless pericardial marsupialization and conventional techniques for pulmonary vein stenosis

Sutureless pericardial marsupialization was associated with satisfactory midterm results and appeared superior to other conventional techniques in children undergoing repair of pulmonary vein stenosis.

2006          Riedlinger

Histology of pulmonary vein stenosis lesions demonstrating intimal myofibroblast-like cells and expression of various receptor tyrosine kinases

The histology of pulmonary vein stenosis lesions was studied in seven infants. Lesional cells stained strongly and diffusely with smooth muscle actin and muscle-specific actin. Receptor tyrosine kinase expression was strong and diffuse for PDGFR-alpha and -beta, FGFR, and VEGFR-2. Lesional cells stained for VEGF and PDGF beta receptor was phosphorylated. The authors concluded: 1) the histologic appearance, and the strong diffuse immunoreactivity for smooth muscle markers, indicates that the intimal lesional cells are myofibroblast-like, and 2) expression of various receptor tyrosine kinases and some ligands suggest an autocrine or paracrine role of these proteins in the pathogenesis of the intimal occlusive lesion in pulmonary vein stenosis.

2007          Holt

Conditions associated with stenosis of pulmonary veins with normal left atrial connections

Conditions associated with stenosis of pulmonary veins with normal left atrial connections were reported. In addition to the presence of congenital heart disease, which was previously reported, these conditions included genetic variants, prematurity and bronchopulmonary dysplasia. In 2008, Drossner reported similar findings and noted that the left pulmonary veins were much more likely to be stenotic than the right pulmonary veins. These findings were also corroborated in a multi-center study in 2009 by Seale.

2008          Buitrago

Sutureless technique for primary repair of inferior total anomalous pulmonary venous connection

A sutureless technique for primary repair of inferior total anomalous pulmonary venous connection was reported.

2009          van de Laar

Potential cause of pulmonary vein stenosis identified on a locus of chromosome 2q

A consanguineous family with four affected siblings with primary pulmonary vein stenosis in association with prenatal lymphatic abnormalities was described. The authors identified a locus on chromosome 2q for potential disease using genome-wide linkage analysis.

2009          Grady

Results of lung transplantation for pulmonary vein stenosis

The results of lung transplantation for 17 children with pulmonary vein stenosis were reported. The actuarial survival was approximately 80% at 5 years and 40% at 10 years.

2010           Jing-Jing

Use of 3-dimensional transthoracic echocardiography for imaging pulmonary vein stenosis

A girl of 3 years with a stenosis of the left upper pulmonary vein was imaged rapidly with real time 3-dimensional transthoracic echocardiography.

2010           Peng

Comparison between conventional and cutting balloon dilation of pulmonary vein stenosis

A large study comparing conventional and cutting balloon dilation of congenital and postoperative pulmonary vein stenosis in children was reported. Both conventional and cutting balloon angioplasty were effective at decreasing gradient and increasing lumen size acutely, but both methods provided limited benefit long-term.

2010           Gordon

Treatment of pulmonary vein stenosis with polytetrafluoroethylene covered stents

The treatment of pulmonary vein stenosis with expanded polytetrafluoroethylene covered stents was reported in 3 children. Stents remained patent during a follow up period of 3-6 months. In 2016, Hilbert emergently used a covered stent to manage rupture of a pulmonary vein after balloon dilation for postinterventional pulmonary vein stenosis.

2010           Mueller

Paclitaxel-eluting balloons in the treatment of pulmonary vein stenosis

Paclitaxel-eluting balloons were used in the treatment of congenital pulmonary vein stenosis. There was no restenosis of the treated veins; however, follow up was limited to a couple weeks due to the infant’s death.

2010           Furukawa

Comparison in-stent stenosis using bare metal stents and sirolimus-eluting stents in an animal model

In-stent stenosis 8 weeks following placement of bare metal stents and sirolimus-eluting stents was studied in young pigs. The degree of in-stent stenosis was significantly reduced in the sirolimus-eluting stent group.

2010           Honjo

Trends toward improved survival and freedom from reintervention by using a sutureless technique for pulmonary vein stenosis

Trends toward improved survival and freedom from reintervention were observed when using a sutureless technique for the primary repair of mixed total anomalous pulmonary venous drainage.

2011           Rehman

Phase II clinical trial of vinblastine and methotrexate in multivessel intraluminal pulmonary vein stenosis

A phase II clinical trial of vinblastine and methotrexate was performed in infants and children with multivessel intraluminal pulmonary vein stenosis. The toxicity profile resulted in numerous treatment delays and interruptions that, combined with limited information on the natural history of this patient population, hampered the authors’ ability to determine the true efficacy of this approach.

2011           Viola

Impact of the sutureless technique on mortality following repair of primary pulmonary vein stenosis

The impact of the sutureless technique on mortality following repair of primary pulmonary vein stenosis in children was reported. The authors observed the mortality and restenosis rates remained high despite the adoption of a sutureless technique. A preoperative pulmonary vein stenosis score of greater than 4 was a strong predictor of poor prognosis.

2012           Balasubramanian

Stent implantation diameter ≥7 mm was associated with longer freedom from reintervention

Outcomes after stent implantation for the treatment of congenital or postoperative pulmonary vein stenosis in children was evaluated. A stent implantation diameter ≥7 mm was associated with longer freedom from reintervention and from significant in-stent stenosis.

2012           Bingler

Use of cryo-balloon dilation of pulmonary vein stenosis

Cryo-balloon dilation of pulmonary vein stenosis was used in children and adolescents with pulmonary vein stenosis. Cryo-balloon dilation of pulmonary vein stenosis resulted in acute relief of stenosis; however, appeared minimally effective as the sole therapy.

2013           Matsuhisa

Anterior translocation of the atrial septum as a modification of the sutureless technique

The anterior translocation of the atrial septum as a modification of the sutureless technique may decrease the risk of pulmonary vein stenosis following primary repair of total anomalous pulmonary venous connection.

2014           Heching

High incidence of necrotizing enterocolitis with pulmonary vein stenosis

A high incidence of necrotizing enterocolitis in premature infants who are diagnosed with acquired pulmonary vein stenosis was reported. In 2022, Duchon used a case-control study to further demonstrate the association between pulmonary vein stenosis and intestinal pathology. The authors proposed that the presence of gastrointestinal pathology should lead to early surveillance and intervention.

2014           Kato

Endothelial to mesenchymal transformation observed in an animal model of neonatal pulmonary vein banding

Neonatal pulmonary vein banding in piglets recapitulated the critical aspects of clinical pulmonary vein stenosis. Endothelial to mesenchymal transformation was observed in upstream vessels. After stent implantation, decompression of a pulmonary vein was associated with reappearance of endothelial marker expression, suggesting the potential for plasticity in the observed pathologic changes. Though rapid in-stent restenosis occurred.

2014           Zhu

Losartan ameliorates the upstream pulmonary vein vasculopathy

Losartan ameliorated the upstream pulmonary vein vasculopathy in a piglet model of pulmonary vein stenosis. Losartan treatment improved the severity of pulmonary hypertension associated with pulmonary vein stenosis and pulmonary venous intimal hyperplasia.

2014           Zheng

Low mortality after repair of total anomalous pulmonary venous connections

A 2.3% mortality was reported for their techniques to repair of total anomalous pulmonary venous connections. There was no stenosis of the anastomosis between the pulmonary venous confluence. Only 2.3% of surviving patients had increased Doppler flow velocities in pulmonary veins during a follow up period of 6 months to 3 years.

2015           Esch

A 7.6% prevalence of stroke with catheter-medicated interventions for pulmonary vein stenosis

A 7.6% prevalence of clinically evident stroke was observed among a large number of patients who underwent catheter-medicated interventions for pulmonary vein stenosis.

2015           Kotani

Left-sided pulmonary venous obstruction between left atrium and descending aorta

The combination of relative cardiomegaly within the context of the thoracic cavity at the level of the pulmonary veins and antero-lateral displacement of the aorta was associated with left-sided pulmonary venous obstruction.

2016           Lo Rito

Outcome of pulmonary vein stenosis repair influenced by size of vessels

Smaller upstream or downstream total cross-sectional area indexed for body surface area negatively influenced survival in patients undergoing repair of pulmonary vein stenosis.

2016           Pogoriler

Lung pathology of pulmonary vein stenosis

An evaluation of the lung pathology of 33 patients with pulmonary vein stenosis was performed. The authors evaluated relatively large number of patients compared to previous reports. They reported that intrapulmonary pulmonary veins commonly show muscular thickening consistent with venous hypertensive remodeling.

2016           Jing

Potential benefit of sutureless repair for total anomalous pulmonary venous drainage and small pulmonary veins

Individual pulmonary veins outgrew somatic growth in surviving patients after primary sutureless repair of total anomalous pulmonary venous drainage. The authors proposed that primary sutureless repair may be a useful measure in patients with total anomalous pulmonary venous drainage and small pulmonary veins.

2016           Jean-St-Michel

Stenosis of left pulmonary veins more common after heart transplantation

The surgical approach and the post-transplant clinical course of a cohort of pediatric heart transplant recipients with a pre-transplant diagnosis of PV stenosis or atresia was reviewed. The left pulmonary veins were most commonly affected. Seven of 12 patients survived.

2016           Zhang

Use of the sutureless technique associated with decreased mortality and post-repair pulmonary vein stenosis

The use of the sutureless technique was associated with decreased mortality and diminished post-repair pulmonary vein stenosis in patients presenting with preoperative obstruction when compared with a conventional surgical approach.

2017           Kalfa

A pulmonary vein stenosis severity score based on the assessment of each pulmonary vein

The results of repair of primary pulmonary vein stenosis in a multi-center study was performed. A specific pulmonary vein stenosis severity score was developed based on the assessment of each pulmonary vein. The authors observed a guarded prognosis despite adoption of the sutureless technique. Postoperative pulmonary hypertension and severity of disease evaluated by the new severity score were independent prognostic factors regardless of surgical technique.

2017           Kovach

A clinicopathologic study of 214 sites of pulmonary vein stenosis

A clinicopathologic study of 214 sites of pulmonary vein stenosis in 97 patients was performed. Histologically, all sites showed sparsely cellular intimal expansion composed of haphazardly arranged fibroblasts with slender nuclei in myxoid matrix, or a paucicellular fibrointimal proliferation consistent with a form of reactive hyperplasia. 

2017           Suntharos

Superimposed imaging to guide interventions

Superimposed real-time 3-dimensional CT or magnetic resonance images helped to guide interventions for acquired pulmonary vein stenosis better than 2-dimensional angiography in adult patients.

2017           Yamauchi

Selective pulmonary artery occlusion to effectively treat hemoptysis

Selective pulmonary artery occlusion with a vascular plug effectively treated hemoptysis associated with pulmonary venous obstruction.

2018           Sykes

Reduced right ventricular function is a potential predictor of survival

Reduced right ventricular function and increased ratio of the pulmonary artery and aortic diameters appeared to be predictors of decreased survival. In 2025, Takajo reported further concerning the role of right ventricular systolic pressure and right ventricular dysfunction as independent predictors of mortality in children with pulmonary vein stenosis.

2018           Domingo

Pulmonary vasodilators are safe and effective for selected patients with pulmonary vein stenosis

Acute vasodilator testing can be used to identify patients with pulmonary vein stenosis who are reactive to oxygen, oxygen with nitric oxide, and diltiazem. Further, clinical improvement was temporally associated with subsequent pulmonary vasodilator therapy in some patients with few adverse effects. Pulmonary edema or an increased need for oxygen did not occur in 7 patients with 2 narrow veins and 5 patients with 3 veins. The authors proposed that the risk of developing edema in the setting of segmental disease may actually decrease by lowering the afferent driving pressure into segments of lung with pulmonary venous obstruction.

2018           Backes

A systematic review, meta-analysis, and meta-regression of infants with pulmonary vein stenosis

A systematic review, meta-analysis, and meta-regression of infants with pulmonary vein stenosis was performed. The authors reported a greater mortality in infants with 3 or 4 stenotic veins than in those with 1 or 2 veins. They also observed a greater mortality in infants with bilateral than unilateral disease.

2018           Callahan

Imatinib mesylate with or without bevacizumab for multivessel intraluminal pulmonary vein stenosis

The 48- and 72- week outcomes among patients receiving imatinib mesylate with or without bevacizumab for multivessel intraluminal pulmonary vein stenosis were reported. After the 72-week follow-up, 16 patients had stabilized, 27 had recurred locally without stabilization, and 5 had progressed. Stabilization was associated with the absence of lung disease and a higher percentage of eligible drug doses received. Patients were also treated surgical or catheter-mediated interventions during the study.

2018           Sughimoto

No postoperative pulmonary venous obstruction using conventional repair of total anomalous pulmonary venous drainage
No postoperative pulmonary venous obstruction using conventional repair of total anomalous pulmonary venous drainage. The authors provided 4 recommendations: (1) mark incision lines between 2 chambers to gain anatomically natural alignment, (2) place precise stitches by “intima-to-intima” using monofilament suture, (3) adequate orifice size should be guaranteed in greater than expected mitral valve size, (4) do not hesitate to undertake a redo additional anastomosis by a different approach when an echocardiography shows the velocity more than 1.5 m/s.

2019           Kirkpatrick

Metakaryotic cells in biopsy specimens of pulmonary vein stenosis

Metakaryotic cells were identified in biopsy specimens in pediatric patients with pulmonary vein stenosis. The authors proposed that these cells may be the precursor to myofibroblasts.

2019           van Duin

Pulmonary vascular effects of cGMP pathway agents in an animal model of pulmonary vein stenosis

The pulmonary vascular effects of vasoactive agents that act on the cGMP pathway of signal transduction in an animal model (swine) of pulmonary vein stenosis was studied. The authors observed that some vasodilators differentially effect vessels in banded and unbanded regions of the lung.

2019           Khan

Outcomes of bare metal stents and drug-eluting stents in pulmonary vein stenosis

Outcomes using bare metal stents and drug-eluting stents in pulmonary vein stenosis were compared. The authors reported a significantly lowered lumen loss rate with drug-eluting stents at medium term follow-up. Further, drug-eluting stents can be fractured to enable larger diameters.

2020          Kirkpatrick

Metformin therapy for pulmonary vein stenosis

Two patients who developed pulmonary vein stenosis following repair of total anomalous pulmonary venous return were treated with metformin. Treatment potentially slowed the progression of disease.

2020          Callahan

Use of systemic sirolimus to prevent the progression of in‑stent stenosis

The use of systemic sirolimus to prevent the progression of in‑stent stenosis in pediatric pulmonary vein stenosis was reported. The authors observed that sirolimus slowed the growth rate of in-stent stenosis following stent implantation in comparison to pre-treatment rates.

2020          Geggel

A multi-patch procedure for repair of scimitar syndrome

A multi-patch procedure was used for the repair of scimitar syndrome. Five of 11 patients who underwent baffling or re-implantation procedures developed postoperative pulmonary vein stenosis. None of the 11 patients who underwent the multi-patch procedure developed postoperative stenosis with a follow up period of 2 months to 3 years. 

2020          Zartner

Bioabsorbable magnesium scaffolds for left upper pulmonary vein stenosis

Bioabsorbable magnesium scaffolds were used in 3 infants with left upper pulmonary vein stenosis. Restenosis occurred after degradation and made further interventions necessary, but neither vessel growth nor further interventions were hindered by stent material.

2020          Masaki

Rapamycin therapy in an animal model of left lower pulmonary vein stenosis

An animal model of left lower pulmonary vein stenosis in infant pigs was developed. The authors reported of evidence on the dedifferentiation of smooth muscle-like cells and mammalian target of rapamycin pathway activation in the pathogenesis of pulmonary venous obstruction. Delivery of rapamycin to the anastomotic site from the external side delayed pulmonary venous anastomotic stenosis.

2020          Feng

Outcomes of the modified L-shaped incision technique and the posterior technique for supracardiac total anomalous pulmonary venous repair

The outcomes of the modified L-shaped incision technique and the posterior technique of supracardiac total anomalous pulmonary venous repair were compared. The modified L-shaped incision technique was significantly associated with decreased death and postoperative pulmonary venous obstruction.

2021           Callahan

Imaging of pulmonary vein stenosis with intravascular ultrasound

Intravascular ultrasound images of stenotic pulmonary veins and histology were compared. Intravascular ultrasound demonstrated wall thickening with up to two layers of variable echogenicity, often with indistinct borders, which corresponded with a layer of hyperplasia of myofibroblast-like cells in abundant myxoid matrix and a less myxoid layer with more collagen, smooth muscle and elastic fibers. Additional experience with intravascular ultrasound in pulmonary vein stenosis was reported by Callahan this year. Also in 2021, Heyden and Kops used intravascular ultrasound in the evaluation and management of children with pulmonary vein stenosis. The authors reported that intravascular ultrasound provided detailed imaging of stenotic lesions, which might help to guide therapy.

2021           Hammer

Elevated wall shear stress at discrete pulmonary venous sites

The role of elevated wall shear stress at discrete pulmonary venous sites in triggering stenosis was studied by retrospectively analyzing cardiac catheterization, lung scan, and X-ray computed tomography data to estimate wall shear stress in the pulmonary veins at multiple time points during disease progression in two patients. The results were consistent with the existence of a level of elevated wall shear stress above which the disease is progressive and below which progression is halted. Their analysis also suggested the possibility of predicting the target lumen size necessary for a given vein to reduce wall shear stress to normal levels and remove the trigger for stenosis progression.

2021           Liufu

Outcomes for conventional and sutureless repairs of infracardiac total anomalous pulmonary venous connection

The outcomes for conventional and sutureless repair of infracardiac total anomalous pulmonary venous connection were compared in 63 infants. The sutureless repair was associated with lower mortality and lower incidence of restenosis in pulmonary veins and anastomosis.

2021           Patel

Survival benefit associated with systemic sirolimus therapy in moderate to severe pulmonary vein stenosis

Investigators sought to determine whether mTOR inhibition with sirolimus as a primary medical therapy would improve outcomes in high-risk infants and children with pulmonary vein stenosis. The authors reported a survival benefit associated with systemic sirolimus therapy in infants and children with moderate to severe pulmonary vein stenosis.

2021           Niccum

Association between aspiration and poor treatment response to imatinib and multimodal interventions

The association between aspiration and poor treatment response to imatinib and multimodal interventions was evaluated in patients with intraluminal pulmonary vein stenosis. Multivariable analysis revealed that patients with clinical aspiration had nearly five times higher odds of poor treatment response than patients without aspiration.

2021           Kuo

Multidisciplinary care

Due to the complexities of imaging, medical therapy and anatomical therapies, the authors proposed that a multidisciplinary approach to treating pulmonary vein stenosis is critical.

2021           Zettler

Two phenotypes of pulmonary vein stenosis in premature infants

Two phenotypes of pulmonary vein stenosis in premature infants were identified, those with disease stabilization and those with disease progression. The presence of severe pulmonary hypertension at diagnosis was increased, and survival was decreased in the group with disease progression.

2021           Choi

Posterior aortopexy used to alleviate pulmonary vein compression

The use of posterior aortopexy in five patients was reported. The authors concluded that this may be a good surgical option for left pulmonary venous obstruction caused by compression between the left atrium and the anteriorly positioned descending aorta, unless there was diffuse narrowing.

2021           Najm

Use of an in situ autologous pericardial roll to decrease the need for vessel mobilization

Use of an in situ autologous pericardial roll may abrogate the need for mobilization of distant anomalous pulmonary veins with direct anastomosis or complex intracardiac baffles.

2022          Góreczny

Use of fusion imaging and 2-dimensional angiography in interventions for pulmonary vein stenosis

The use of fusion imaging and 2-dimensional angiography in interventions for pulmonary vein stenosis were compared. Their results suggest a possibility for lower contrast utilization, decreased radiation exposure, and shorter fluoroscopy and study times with the fusion imaging guidance compared to 2-dimensional angiography.

2022          Zablah

Use of optical coherence tomography in pulmonary venous stenosis

The technical feasibility of using optical coherence tomography in the evaluation of pediatric pulmonary venous stenosis was reported. It provided intraluminal anatomy in children with both native and treated pulmonary vein stenosis when vessel size was less than 5 mm.

2022          Feins

A new anatomically focused repair strategy to alleviate pulmonary vein angulation and minimize turbulence

Use of a new anatomically focused repair strategy to alleviate pulmonary vein angulation and minimize turbulence was reported with promising early outcomes. The authors also reported that antiproliferative chemotherapy was associated with decreased mortality.

2022          Kise

Use of a half-uncovered deployment technique to secure larger stents

Use of a half-uncovered deployment technique to secure larger stents for postoperative pulmonary vein stenosis was evaluated based upon reports that larger stent diameters decrease the risk of restenosis. All stents were precisely placed and successfully resolved the stenosis.

2023          Lisheng

Neonatal Rat model of pulmonary vein stenosis

A neonatal rat model of pulmonary vein stenosis was developed, which may be a useful platform for understanding mechanisms and treatments for the progression of disease.

2023          Aggarwal

Percutaneous recanalization of pulmonary vein atresia

Percutaneous recanalization of pulmonary vein atresia was safe and feasible. A high acute success rate and results in growth of the distal pulmonary veins occurred with placement of drug-eluting stents. 

2023          Li

No statistical difference in postoperative pulmonary vein flow velocity when a primary sutureless repair was used for the 4 subgroups of total anomalous pulmonary venous connection

No statistical difference in postoperative pulmonary vein flow velocity was observed when a primary sutureless repair was used for the 4 subgroups of total anomalous pulmonary venous connection. The authors proposed that the primary sutureless technique may decrease the postoperative rate of pulmonary venous obstruction and may be used for any subtype of total anomalous pulmonary venous connection.

2023          Sengupta

Risk prediction model proposed for late reintervention

A risk prediction model was proposed for late reintervention that may guide prognostication of high-risk patients after repair of total anomalous pulmonary venous return. Higher risk scores were significantly associated with worse freedom from reintervention.

2024          Ning

A novel in vitro model of pulmonary vein stenosis using bioprinted bifurcated pulmonary venous constructs

A novel in vitro model of pulmonary vein stenosis was developed. Bioprinted bifurcated pulmonary venous constructs were seeded with endothelial cells and perfused, demonstrating the formation of a uniform and viable endothelium. Computational modeling identified the bifurcation point at high risk of endothelial cell overgrowth. Application of an external magnetic field enabled targeting of the rapamycin-loaded superparamagnetic iron oxide nanoparticles at the bifurcation site, leading to a significant reduction in endothelial cell proliferation with no adverse side effects.

2024          Devlin

3-dimensional printed reconstructions of pulmonary vein stenosis for computational fluid dynamic analysis

Three-dimensional printed reconstructions of pulmonary vein stenosis in patients were developed for computational fluid dynamic analysis utilizing Doppler echocardiography to represent in vivo flow conditions. The authors identified areas of undesirable alterations in wall shear stress before and after catheterization. They proposed that patient-specific 3-dimensional in vitro models demonstrate great promise to refine interventional approaches and mitigate complications in treating patients with primary pulmonary vein stenosis.

2024          Butto

Pulmonary vein stenosis following heart transplantation

A relatively large retrospective study was performed to describe the characteristics of pulmonary vein stenosis following heart transplantation in children. The incidence of pulmonary vein stenosis was 4.5%, and underlying congenital heart disease was a strong risk factor. Multiple vessels can be involved and may require catheter-based or surgical interventions.

2024          Fuller

Stress and coping in caregivers of children with pulmonary vein stenosis

Stress and coping were explored in caregivers of children with pulmonary vein stenosis. In a mixed-method study, the authors found high levels of illness-related parental stress among caregivers of children. Stress evolved over time from what caregivers described as ‘survival mode’ to a future-oriented outlook. Caregivers indicated that communication and parental role functioning were coping strategies that could be better supported by providers and health systems.

2024          Nijres

An anchoring guide catheter to facilitate recanalizing occluded pulmonary veins

A novel technique using an anchoring guide catheter to facilitate recanalizing occluded pulmonary veins in 2 small children was described. This technique enhanced the catheter’s alignment and stability during the recanalization process. 

2024          Stidham

Use of Doppler echocardiography to approximate bilateral lung perfusion

The use of Doppler echocardiography to approximate bilateral lung perfusion was compared to nuclear medicine perfusion scans. The authors proposed that this method provides a safer and more accessible alternative method of quantifying pulmonary perfusion abnormalities in some patients with pulmonary vein stenosis.

2024          Kalustian

Systemic sirolimus therapy is associated with a reduced intervention frequency

The use of systemic sirolimus therapy was associated with a reduced intervention frequency for pulmonary vein stenosis (5.0 pre-therapy and 1.7 on-therapy).

2025          Shentu

Valsartan therapy potentially decreases the progression of postoperative pulmonary vein stenosis

Early and sustained valsartan therapy of infants following repair of total anomalous pulmonary venous connection did not prevent the incidence of postoperative pulmonary vein stenosis requiring an intervention. However, disease progression in additional vessels and upstream vessels was less likely to occur. Death occurred in 11.4% of the control group patients versus none of the valsartan group patients.

2025          Zablah

Use of electrical impedance tomography to evaluate lung perfusion

Electrical impedance tomography was used to noninvasively evaluate lung ventilation and perfusion in patients with congenital pulmonary vein stenosis before and after catheter-based interventions.  Electrical impedance tomography correctly depicted perfusion in comparison to angiography.