Case Study: Rapidly Progressive Interstitial Lung Disease with Right Ventricular Failure

Young father with a devastating diagnosis gets a second chance after receiving a heart and double-lung transplant

A 40-year-old male never-smoker with a past medical history of non-ischemic heart failure, beta thalassemia, sickle-cell trait, and interstitial lung disease presented to the TUH emergency room with chest pain. He was found to have a large left pneumothorax that was treated with tube (8Fr) thoracostomy. A right pneumothorax, a week prior to this presentation, was treated at another hospital. His first abnormal chest X-ray result was found about two years ago at a different emergency room. Since that time, his shortness of breath progressed rapidly.


Image 1: Non-enhanced high-resolution CT scan of thorax demonstrating interstitial lung disease from suspected NSIP, paraseptal emphysema, and left pneumothorax with 8Fr tube.
Image 2: CTA thorax demonstrating a linear defect in the right atrium consistent with clot in transit. Also visualized are dilated right atrium and ventricle with right heart strain, underlying pulmonary fibrosis, and thoracostomy tube.

Diagnostic Findings

CT scan of the thorax demonstrated interstitial lung disease with paraseptal emphysema. Evaluation for connective tissue diseases revealed a positive ANA as well as positive SS-A and SS-B antibodies, which can be found in lung diseases secondary to connective tissue disorders.

Echocardiogram showed right ventricular dilation with reduced function. Prior records also revealed normal coronaries and normal pulmonary pressures via right heart catheterization one month ago. The non-ischemic cardiomyopathy was believed to be an isolated yet advanced right ventricular cardiomyopathy.

Clinical Course

  • The patient developed an acute left-lower-extremity deep vein thrombosis and pulmonary embolism with clot visualized in his right atrium via echo and CT chest.
  • He underwent surgical embolectomy requiring insertion of a centrally placed right ventricular assist device.
  • Despite a number of interventions to stabilize his cardiopulmonary status and liberate him from mechanical support, his condition did not improve.

Further evaluation with CT scans and cardiac imaging confirmed that recent insults had resulted in irrecoverable cardiopulmonary failure.

Treatment

While fully supported by a centrally cannulated mechanical assist device, the patient actively participated in physical therapy. After multidisciplinary evaluation, he was listed for heart and double-lung transplantation. Within the week, he underwent an uneventful transplant.

Outcomes

He was discharged to home, breathing room air, 44 days after his initial presentation. He reports feeling well and has been enjoying getting back to being an active father for his three children.

Discussion
The patient’s pulmonary embolism unmasked the severity and lethal nature of his rapidly progressive interstitial lung disease and chronic cardiomyopathy. Yet, with a dedicated multidisciplinary team, as well as hard work by the patient and family, even such seriously ill patients may have a second chance at survival.


James C Brown MD, MBA
Assistant Professor of Pulmonary and Critical Care Medicine
Lewis Katz School of Medicine at Temple University