Pulmonary vein stenosis

Jaya Menon1*, Umarani S2, Hemalatha R3, Shalini4

1Nursing Superintendent, Kauvery Heart city, Trichy, India

2Assitant Nursing Superintendent, Kauvery Heart city, Trichy, India

3 Nursing Supervisor, Kauvery Heart city, Trichy, India

4 Nursing Non Critical Ward Senior Staff, Kauvery Heart city, Trichy, India

Abstract

Pulmonary vein stenosis is a challenging condition requiring a high degree of suspicion appropriate diagnostics imaging and a tailored treatment approach that may involve surgery catheter based intervention and potentially novel therapies like imagine.

Diagnosis often involves imaging techniques like echocardiography CT scans or MRI and treatment may include surgery balloon angioplasty or standing.

Definition

Pulmonary vein stenosis (PVS) is a rare condition where the pulmonary veins which carry oxygen rich blood from the lungs to the heart become narrowed or blocked.  The narrowing or blockage restricts blood flow potentially leading to complications like pulmonary hypertension and heart failure.  PVS can be congenital (present at birth) or acquired.

Causes

  • Congenital heart defects
  • Genetic Condition
  • Noonau syndrome
  • Williams’s syndrome
  • Maternal factors.

Acquired Risk Factor

  • Rheumatic fever
  • Endocarditis
  • Radiation therapy
  • Valve Calcification

Symptoms

  • Dyspnea
  • Cough (may be bloody)
  • Fatigue
  • Poor feeding (or) growth failure
  • Lung infection
  • Pulmonary hypertension
  • Cyanosis (Bluish lips / Skin)
  • Palpitaiotn – due to associated arrhythmias or atrial fibrillation.

Past History

  • Pulmonary vein stenosis
  • Coaractation of aorta
  • Severe pulmonary arterial hypertension

Past Surgical History

  • Coarctation of aorta repair with pulmonary vein repair (28.02.24)
  • Pulmonary vein dilatation (10.07.25)

Present History

Child have a complaints of dypnea breathlessness and palpitaiton intially came to hospital child active and alert. Echo showed diagnosed as pulmonary vein stenosis and severe PAH (Pulmonary Artgery Hypertension)

Echo

  • Mild TR/Mild PAH
  • LLPV Gradient – 8/4 mmhg
  • RUPV gradient – 4/2 nngg
  • Bi pericardial effusion/clot
  • Good biventricular function
  • Small ASD with left to right shunt
  • S/P pulmonary vein balloon dilatation

Case presentation

A 5 years boy known case of pulmonary vein stenosis coarctationof aorta, sever pulmonary arterial hypertension, patient had complaints of not gaining weight breathing difficulty and palpitation. During his OPD evaluation he was suggested cardiac catheterization and pulmonary vein dilatation done (3.7.25) with successful results.

Present procedure

S NVesselPre Dilatation

Mean Pressure
Post Dialtation

Mean pressure
1Left Atrium8 mm Hg8 mm Hg
2LLPV18 mm Hg13 mm Hg
3RUPV14 mm Hg9 mm Hg
4LA78

Cardiac catheterization + pulmonary vein dilatation

Clinical Signs and Symptoms

  • Respiratory distress – difficulty in breathing especially in infants and young children.
  • Tachypnea – Rapid breathing
  • Tachycardia – Rapid heart rate
  • Cyanosis – Bluish discolouration of skin lips and nails due to low oxygen.
  • Failure to thrive – Poor weight gain in infant
  • Haemoptysis – coughing up blood
  • Pulmonary hypertension – increased pressure in the pulmonary arteries.
  • Respiratory infection – impaired lung function
  • Heart failure – peripheral edema

Diagnostic Evaluation

1. ECG

2. Chest X-ray: Pulmonary congestion or edema enlarged heart

3. Echo Cardiogram: (Trans thoracic or Trans esophageal).

  • Elevated pulmonary artery pressure.
  • Colour Doppler helps visualize abnormal flow.

4. CT Angiography

  • Cold standard for non-invasive anatomical assessment
  • Provide clear visualization of pulmonary assessment

  • Cardiac MRI: Can measure blood flow and detect stenosis severity.

5. Cardiac catheterization

  • Measures pressure gradients across pulmonary vein
  • Can perform angiography to visualize stenosis directly.

6. Pulmonary function test

7. Oxygen saturation

Management

Medical Management

  • Antithrombotic Therapy: Anticoagulant with warfarin or dual antiplatelet therapy

(Tab. Aspitin 75mg OD)

  • Targeted Medical therapy: (Tab.Imatinib)

Surgical Management: Cardiac catheterization + pulmonary vein dilatation.

Nursing Management

Pre-Operative Care

  • Vital signs monitoring
  • Respiratory status monitoring (ex: shortness of breath, cough and wheezing)
  • Cardiac status monitoring (such as edema, jugular venous distension)
  • Growth and development (Monitor height and weight)
  • Administered the medication regular follow up (antiplatelet)
  • Pain assessment (Chest pain or chest discomfort evaluation)

Post Procedure care

  • Check the vital signs and monitoring
  • Surgical intervention (Bleeding, hematoma) catheter based intervention procedure site any infection.

Pain Management: Provide pain relief measure including analgesics and non-pharmacological intervention.

Fluid Management:

  • Monitor fluid intake and output chart maintained.
  • Provided the comfortable positioning
  • Closed monitoring the procedure site and watch for any oozing and bleeding.

Anatomy and Physiology

Pulmonary vein Stenosis

Pulmonary vein stenosis (PVS) is a condition where the pulmonary vein which carry oxygenated blood form the lungs to the heart narrow become obstructed.  This can be caused by congenital defect, acquired condition life infection or injuries complication of medical procedure like ablation for atrial fibrillation.

Anatomy of Pulmonary vein

  • Normal Anatomy – Typically three are four pulmonary vein (two on each side) that drain oxygenated blood from the lungs into the left atrium of the heart.
  • Left pulmonary Veins – The left superior and inferior pulmonary veins drain the left lung and enter the left atrium.
  • Right Pulmonary Veins – The right superior and inferior pulmonary veins drain the right lungs and enter the left atrium.
  • Location – These veins are posterior structure and can be challenging to visualize with some imaging techniques.
  • Variation – There can be variation in the anatomy such as a common left sided trunk where the left superior and inferior veins merge before entering the left atrium.
  • Pulmonary vein Stenosis:
  • Narrowing – In PVS, the pulmonary vein or their connection to the left atrium become narrowed or obstructed.

Etiology

  • Exact cause in unknown
  • Congenital heart defect
  • Peior surgeries (like radio frequency ablation for atrial fibrillation)
  • Other lung or heart condition

Clinical Presentation

  • Respiratory distress (Rapid & laboured breath)
  • Cough
  • Haemoptysis (coughing up blood)
  • Pulmonary hypertension

Diagnostic tests

  • Echo cardiography (TEE included)
  • CT angiography
  • MRI to visualize the pulmonary veins and flow
  • Cardiac catheterization

Physiology of Pulmonary Vein

Pulmonary veins are blood vessels that carry oxygen blood from your lungs to your heart.  Your pulmonary veins are part of your body’s pulmonary circuit.  This is a system of blood vessels that moves blood between your heart and lungs.  This circuit also includes your pulmonary arteries.

Reference

  • Latson LA, Prieto LR. Congenital and acquired pulmonary vein stenosis. Circulation. 2007; 115:103–108. doi: 10.1161/CIRCULATIONAHA.106.646166.
  • Maan A, Shaikh AY, Mansour M, Ruskin JN, Heist EK. Complications from catheter ablation of atrial fibrillation: a systematic review. Crit Pathw Cardiol.
  • Porres DV, Morenza OP, Pallisa E, Roque A, Andreu J, Martínez M. Learning from the pulmonary veins. Radiographics.
  • Edwards JE. Congenital stenosis of pulmonary veins. Pathologic and developmental considerations. Lab Invest. 1960; 9:46–66.
  • Rostamian A, Narayan SM, Thomson L, Fishbein M, Siegel RJ. The incidence, diagnosis, and management of pulmonary vein stenosis as a complication of atrial fibrillation ablation. J Interv Card Electrophysiol. 2014; 40:63–74. doi: 10.1007/s10840-014-9885-z.z

 

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