Respiratory · Year 1 · from Respiratory

Case 3: Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Clinical Image

Source: Radiopaedia - CTEPH - Educational use

Patient Presentation

A 62-year-old man is referred to a pulmonary hypertension center with progressive dyspnea over 2 years. He had a documented pulmonary embolism 4 years ago following a long flight, for which he received 6 months of anticoagulation. He improved initially but has had slowly worsening exercise tolerance for the past 2 years. His primary care physician recently detected elevated pulmonary pressures on echocardiography.

Demographics

  • Age: 62 years
  • Sex: Male
  • Past Medical History: Pulmonary embolism 4 years ago, obesity, former smoker
  • Medications: None currently (completed anticoagulation 3.5 years ago)
  • Social History: Retired, sedentary lifestyle

Chief Complaint

Progressive exertional dyspnea for 2 years, worsening despite initial recovery from PE

Physical Examination

  • Blood pressure: 132/78 mmHg
  • Heart rate: 88 bpm
  • Respiratory rate: 20/min
  • Oxygen saturation: 92% on room air
  • General: Obese, appears comfortable at rest
  • Neck: Elevated JVP
  • Cardiovascular: Loud P2, RV heave, holosystolic murmur at left lower sternal border
  • Respiratory: Clear
  • Extremities: Moderate bilateral lower extremity edema

Workup

  • Echocardiography: Estimated RVSP 68 mmHg, RV dilation, TR moderate
  • BNP: 380 pg/mL
  • V/Q scan: Multiple large, bilateral segmental and subsegmental mismatched perfusion defects (high probability)
  • CT pulmonary angiography: Chronic organized thrombus with webs and bands in lobar and segmental arteries, mosaic attenuation in lung parenchyma
  • Pulmonary angiography: Confirms chronic thromboembolic disease, surgically accessible
  • Right heart catheterization:
  • Mean PAP: 48 mmHg
  • PCWP: 12 mmHg
  • PVR: 8 Wood units

Diagnosis

Chronic Thromboembolic Pulmonary Hypertension (WHO Group 4)

Treatment

  1. Lifelong anticoagulation (regardless of surgical intervention)
  2. Pulmonary thromboendarterectomy (PTE) - potentially curative:
  • Surgery to remove organized, fibrotic thrombus from pulmonary arteries
  • Performed at specialized centers on cardiopulmonary bypass with deep hypothermic circulatory arrest
  • Cure rate >85% in appropriately selected patients
  1. If inoperable or residual PH post-surgery:
  • Riociguat (FDA-approved for inoperable/persistent CTEPH)
  • Balloon pulmonary angioplasty (BPA) for distal disease
  1. Diuretics and oxygen as needed
  2. IVC filter not routinely indicated if anticoagulated

Physiological Principles Demonstrated

  • CTEPH pathogenesis: 2-4% of acute PE patients develop CTEPH when thrombus does not resolve but organizes into fibrous tissue, causing mechanical obstruction and secondary arteriopathy.
  • V/Q scan sensitivity: V/Q scan is more sensitive than CTPA for detecting chronic organized thrombus and is essential in CTEPH workup; normal V/Q effectively excludes CTEPH.
  • Surgical cure potential: Unlike PAH, CTEPH is potentially curable because the obstruction is mechanical and can be surgically removed if disease is proximal.
  • Progression despite initial PE treatment: CTEPH can develop even after "adequate" anticoagulation; ongoing symptoms after PE should prompt evaluation.

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