Conference: 2011 PH Professional Network Symposium
Release Date: 09.22.2011
Presentation Type: Abstracts
A. Esan, MD, A. Monson, RN, S. Raoof, MD, S. Sethi, MD
Division of Pulmonary and Critical Care Medicine, Department of Medicine, New York Methodist Hospital, Brooklyn, NY
PURPOSE: Presentation of an interesting case of pulmonary veno-occlusive disease (PVOD) and pulmonary aneurysm with iloprost-induced respiratory failure. Radiology, bronchoscopy and biopsy findings discussed.
BACKGROUND: Pulmonary veno-occlusive disease is an uncommon and often difficult-to-diagnose cause of pulmonary hypertension (PH). Its true incidence is unknown, however it accounts for 5 - 10% of histological forms of cases initially thought to be idiopathic pulmonary arterial hypertension (PAH). One year mortality rate is reported as high as 72%, with most patients dying within 2 years of diagnosis. Timely diagnosis is therefore crucial as treatment options are unfortunately limited.
METHODOLOGY: 34 year old female with a history of Factor V Leiden mutation presents with exertional dyspnea and nonproductive cough of 4 days. She denied any other symptoms. She was a nonsmoker and took no medications. The rest of her history was noncontributory. Positive findings on examination were tachypnea and hypoxia resulting in the need for oxygen supplementation with 3L/min. She was admitted for respiratory insufficiency and her initial labs were normal while an echo performed revealed an EF of 65% and RVSP of 80 mmHg suggestive of PH. Her connective tissue disease work-up was negative. A right heart catherization revealed a mean PAP of 36mmHg with a PAOP of 16mmHg. A V/Q scan revealed multiple large segmental and sub-segmental perfusion defects in both lungs, though a subsequent CT-chest demonstrated hilar and mediastinal lymphadenopathy as well as bilateral diffuse ground glass opacities (GGO) but no evidence of acute or chronic PE. She later had a pulmonary angiogram that showed normal right main pulmonary artery but ectasia of two middle lobe branches of right PA. She underwent a bronchoscopy with BAL and endo-bronchial ultrasound guided with lymph node biopsy in the OR which revealed hyperemia of lobar and segmental bronchi with vascular engorgement in her right middle lobe (RML) and lingular regions that was absent in the trachea and main bronchi. The BAL from her RML was bloody and consistent with alveolar hemorrhage. Her EBUS also demonstrated very vascular lymph nodes (station 7) producing a bloody aspirate. She tolerated the procedure well, however she was prophylactically given inhaled iloprost post- procedure for PH. Approximately 30 minutes after iloprost administration, she desaturated to 89%. She initially improved on 100% non-rebreather mask and was later given another dose of inhaled iloprost to decrease her pulmonary pressures. Over the next 4 hours she developed severe hypoxemic respiratory failure requiring ventilator support, and hemodynamic instability requiring vasopressor therapy and remained in a critical state for 48 hours. She improved over the next 5 days and was successfully extubated.
FINDINGS: A surgical biopsy that was performed 1 month later confirmed our clinical suspicion of PVOD.
IMPLICATIONS: The treatment of PH is based on its classification. PVOD (group 1') presents very similar to and hence is often misdiagnosed as PAH (group1), however, there are a number of important clinical signs that differentiate them such as lung crackles, pleural effusions, ground glass opacities, septal thickening, mediastinal adenopathy and occult pulmonary hemorrhage. Treatment with PAH- specific therapy can be very detrimental in PVOD, occasionally resulting in death. If PVOD is suspected careful consideration should be taken before administering these medications. Patients should be referred early to lung transplantation programs as is at present the only therapy that significantly prolongs life.