Pulmonary vein stenosis

# Overview
Pulmonary vein stenosis is extremely rare, progressive and usually fatal, if left untreated.
Isolated stenosis of pulmonary veins, as they enter into the left atrium, is a rare congenital cardiac abnormality that has significant impact on clinical outcome.
Diagnosis is difficult to suspect, especially in the absence of other cardiac abnormalities.
Symptoms and clinical signs of pulmonary vein stenosis are very similar to those of chronic lung disease and are very difficult to differentiate.

# Historical Perspective
-  was first discovered by , a , in  during/following .
- In ,  mutations were first identified in the pathogenesis of .
- In , the first  was developed by  to treat/diagnose .

# Classification
-  may be classified according to  into  subtypes/groups:
- Other variants of  include , , and .

# Pathophysiology

## Normal Pulmonary Vein (Lateral)
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## Normal Pulmonary Vein (PA)
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## Left Upper Pulmonary Vein Stenosis Pulsed Wave Echo

## Left Upper Pulmonary Vein Stenosis post Atrial Fibrillation Ablation
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# Pathological Findings
Images courtesy of Professor Peter Anderson DVM PhD and published with permission  PEIR, University of Alabama at Birmingham, Department of Pathology
- Pulmonary vein stenosis: Gross, natural color, close-up view of two veins entering atrium
- Pulmonary vein stenosis: Gross, natural color, pulmonary outlet showing thickened pulmonary arteries
- Pulmonary vein stenosis: Gross, natural color, unopened veins and atrium dorsal view
- Pulmonary vein stenosis: Gross, natural color. cut surface. little to show except congestion and probable intrapulmonary hemorrhage
- Pulmonary vein stenosis: Micro low mag, H&E, junction of pulmonary vein and atrium
- There is a patchy interstitial widening (A) due to proliferation of thin-walled capillaries in the alveolar walls (B, arrows). Secondary intimal thickening in a pulmonary vein is present (C, elastic tissue stain) indicative of secondary veno-occlusive disease and associated pulmonary hypertension.
- There is a patchy interstitial widening (A) due to proliferation of thin-walled capillaries in the alveolar walls (B, arrows). Secondary intimal thickening in a pulmonary vein is present (C, elastic tissue stain) indicative of secondary veno-occlusive disease and associated pulmonary hypertension.
- There is a patchy interstitial widening (A) due to proliferation of thin-walled capillaries in the alveolar walls (B, arrows). Secondary intimal thickening in a pulmonary vein is present (C, elastic tissue stain) indicative of secondary veno-occlusive disease and associated pulmonary hypertension.

# Causes
-  may be caused by either , , or .
-  is caused by a mutation in the , , or  gene.
- There are no established causes for .

# Differentiating  from other Diseases
-  must be differentiated from other diseases that cause , , and , such as:

# Epidemiology and Demographics
- The prevalence of  is approximately  per 100,000 individuals worldwide.
- In , the incidence of  was estimated to be  cases per 100,000 individuals in .

## Age
- Patients of all age groups may develop .
-  is more commonly observed among patients aged  years old.
-  is more commonly observed among .

## Gender
-  affects men and women equally.
-  are more commonly affected with  than .
- The  to  ratio is approximately  to 1.

## Race
- There is no racial predilection for .
-  usually affects individuals of the  race.
-  individuals are less likely to develop .

# Risk Factors
- Common risk factors in the development of  are , , , and .

# Natural History, Complications and Prognosis
- The majority of patients with  remain asymptomatic for .
- Early clinical features include , , and .
- If left untreated,  of patients with  may progress to develop , , and .
- Common complications of  include , , and .
- Prognosis is generally , and the  of patients with  is approximately .

# Diagnosis

## Diagnostic Criteria
- The diagnosis of  is made when at least  of the following  diagnostic criteria are met:

## Symptoms
-  is usually asymptomatic.
- Symptoms of  may include the following:

## Physical Examination
- Patients with  usually appear .
- Physical examination may be remarkable for:

## Laboratory Findings
- There are no specific laboratory findings associated with .
- A    is diagnostic of .
- An  concentration of   is diagnostic of .
- Other laboratory findings consistent with the diagnosis of  include , , and .

## Imaging Findings
- There are no  findings associated with .
-  is the imaging modality of choice for .
- On ,  is characterized by , , and .
-  may demonstrate , , and .

## Other Diagnostic Studies
-  may also be diagnosed using .
- Findings on  include , , and .

# Treatment

## Medical Therapy
- There is no treatment for ; the mainstay of therapy is supportive care.
- The mainstay of therapy for  is  and .
-  acts by .
- Response to  can be monitored with  every .

## Surgery
- Surgery is the mainstay of therapy for .
-  in conjunction with  is the most common approach to the treatment of .
-  can only be performed for patients with  .

## Prevention
- There are no primary preventive measures available for .
- Effective measures for the primary prevention of  include , , and .
- Once diagnosed and successfully treated, patients with  are followed-up every . Follow-up testing includes , , and .