A DISEASE WHICH LOOKS LIKE HEART FAILURE BUT IS NOT

PULMONARY VENO-OCCLUSIVE DISEASE

Epidemiology• True incidence of PVOD is unknown.• Usually misclassified as PAH.• Postulated to be 0.1 to 0.2 cases per million persons in the general population.• Pathologic hallmark of PVOD is extensive and diffuse occlusion of the pulmonary veins by fibrous tissue.

Etiology • Genetic : BMPR-2 gene which also accounts for 25% of the idiopathic PAH cases.• Infection : Toxoplasma, Cytomegalovirus, EBV, HIV and Measles.• Exposure to chemotherapeutic agents like Bleomycin, Mitomycin and Carmustine etc.• Also associated with auto-immune diseases like SLE, RA, Sjogrens etc.

Clinical features• Symptoms of dyspnea following an infection associated with cough, chest pain, hemoptysis etc.• Pleural effusions on Xray, invariable finding.• All else is suggestive of heart failure and superimposed secondary Pulmonary hypertension.• Overall the clinical picture looks very much like heart failure.• Patient’s have an elevated JVD, parasternal heave, pedal edema, appear fluid overloaded, Xray will show bilateral effusions with pulmonary edema like picture.• Diuresis helps initially by improving right heart failure but not efficacious as the main problem is not fixed.

Cardiac catheterization• Classically has normal or low wedge pressure (Pulmonary artery occlusion presssure)• Has demonstrable elevated pressures on right side.• Low or minimally elevated Left sided pressures.

LUNG BIOPSY is definitive diagnostic test but in view of high risk and lack of intervention after diagnosis the value is questionable.

Cardiac catheterization example pressures• Example patient numbers :• RA pressure : Mean 13• RV pressure 69/12• PA pressure 66/30 (mean 44)• PCWP 14.• LV EDP 16• Cardiac output : 8.84• CI : 3.88

Treatment • Nothing has been proved to be efficacious.• Vasodilator therapy which is efficacious in PAH is dangerous and can be fatal in these patients.• Anticoagulation.• Minimal role for steroids and immunosuppressants.• Oxygen and lung transplantation.