Ashwini Arjuna, MD
Faculty Physician - Advanced Pulmonology
St. Joseph's Hospital and Medical Center, United States
Disclosure information not submitted.
Rajat Walia, MD
Division of Pulmonary Medicine
Norton Thoracic Institute, St. Joseph's Hospital and Medical Center, Phoenix, AZ, United States
Disclosure information not submitted.
Michael Olson, BS
Medical Student
Norton Thoracic Institute, St. Joseph's Hospital and Medical Center, Phoenix, AZ, United States
Disclosure information not submitted.
Title: A case of missed pulmonary veno-occlusive disease in a patient with nodular emphysema
Case Report Body:
Introduction: Pulmonary veno-occlusive disease (PVOD) is a rare condition which presents in adults with pulmonary arterial hypertension. The diagnosis is often missed as the symptoms are nonspecific and presentation varies. We present a case of missed PVOD in a gentleman with history of significant tobacco use recently diagnosed with smoking-related emphysema and heart failure.
Description: The patient is a 65-year-old male who was referred to our advanced lung disease center for lung transplant evaluation in the setting of pulmonary hypertension refractory to triple therapy. He was recently diagnosed with heart failure given uncontrolled hypertension with worsening dyspnea, weight loss, lower extremity edema, and supportive physical exam findings. Work-up at our center included a CT scan of the chest showing multiple pulmonary nodules, hilar lymphadenopathy, bullous changes, and prominent pulmonary arteries. An ABG revealed severe hypoxemia on 6L nasal cannula. Right heart catheterization revealed severe pulmonary hypertension (mPAP 45 mmHg). Given this constellation of findings and degree of pulmonary arterial hypertension poorly improved by triple therapy, a diagnosis of nodular PVOD was suspected, and the patient completed the lung transplant evaluation. Unfortunately, a rectal malignancy was identified in the work-up, and thus he was not considered a viable transplant candidate. During the evaluation, it also came to our attention that records from >10 years ago showed a remote history of severely reduced DLCO of 11%. Had this patient received better follow-up and earlier intervention for progressive heart failure symptoms, he may have benefitted from earlier diagnosis and treatment with lung transplantation.
Discussion: The diagnosis of PVOD is made retrospectively after autopsy of explanted tissue. Particularly when the presentation is atypical, the diagnosis may be missed. Features which provide early diagnostic evidence for PVOD include pulmonary hypertension with venous congestion on imaging.