Case Study

Halting Disease Progression in its Tracks

Advanced Diagnosis and Therapy for Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

A 62-year-old male presented with shortness of breath. Patient had a known history of pulmonary embolism dating back to 1978, and had another pulmonary embolism in the mid-1990s. He was diagnosed with a blood clotting disorder called protein S deficiency. He reported passing out twice while either coughing or exerting himself in the past three months. Patient was referred to the Temple Pulmonary Hypertension, Right Heart Failure and Pulmonary Thromboendarterectomy Program for further evaluation.

DIAGNOSTIC FINDINGS

Case_study_One

  • NYHA class III symptoms
  • Left ventricular ejection fraction of 65%
  • Mild to moderate RV dilation
  • Mild RV dysfunction
  • Pulmonary artery pressure by right heart cath: 60/20 PVR Wood units

A CT pulmonary angiogram was performed. CT found complete occlusion of the right main pulmonary artery by chronic thrombosis. Partial occlusion of the proximal left pulmonary artery. (Image right.)

DIAGNOSIS

Patient was diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH). He was deemed an appropriate candidate for pulmonary thromboendarterectomy (PTE).

THERAPY

Case_study_twoPatient underwent successful PTE surgery, with extensive chronic clot removed from both lungs. (Image right.)

OUTCOMES

  • Patient’s pulmonary hypertension has resolved.
  • Patient’s right ventricular size and function have returned to normal.
  • The patient has had a complete resolution of his shortness of breath and a return to normal quality of life

KEY POINTS

  • CTEPH should always be considered in patients who are short of breath with a history of pulmonary embolism.
  • CTEPH is an important and potentially curable form of pulmonary hypertension.
  • CTEPH diagnosis evaluation and PTE surgery should only be done at a center with expertise in this condition. The Temple Heart & Vascular Institute is one of the only expert centers in the United States.

LEAD PHYSICIANS

Paul Forfia, MD
Director, Pulmonary Hypertension, Right Heart Failure and Pulmonary Thromboendarterectomy Program, Temple University Hospital

Yoshiya Toyoda, MD, PhD
Chief, Cardiovascular Surgery, Temple University Hospital