130 Chronic Thromboembolic Disease Pulmonary Hypertension (CTEPH)

CASE 130

image Clinical Presentation

72-year-old man with progressive dyspnea, repeated syncopal episodes, and medically refractory chronic pulmonary thromboembolic disease

image Radiologic Findings

PA (Fig. 130.1A) and lateral (Fig. 130.1B) chest radiographs demonstrate marked enlargement of the central pulmonary and interlobar arteries with sharply tapered or pruned peripheral vessels. The cardiac silhouette is enlarged. The enlarged right heart encroaches upon the retrosternal clear space on the lateral exam (Fig. 130.1B). Subsequent catheter pulmonary angiography (not illustrated) confirmed CPTE.

image Diagnosis

Chronic Thromboembolic Disease Pulmonary Hypertension (CTEPH)

image Differential Diagnosis

• Idiopathic Pulmonary Artery Dilatation

image Young women

image Unilateral enlargement of main and left pulmonary artery

image No pressure gradient

• Pulmonary Valve Stenosis

image Unilateral enlargement of main and left pulmonary artery

image Left upper lobe vessels larger than counterpart right upper lobe vessels

• Pulmonary Artery Sarcoma (see Case 129)


Fig. 130.1

image Discussion


Pulmonary artery hypertension (PAH) is defined by an elevated mPAP >25 mm Hg at rest (>30 mm Hg during exercise) and is classified by vascular changes affecting either the precapillary (arterial) or postcapillary (venous) pulmonary circulation. The latter occurs between the capillary bed and the left atrium.


The vascular changes may be idiopathic, as in primary pulmonary hypertension (PPH) (i.e., precapillary level in the absence of an identifiable cause), but more commonly represent a secondary response to alterations in pulmonary blood flow. The postcapillary counterpart to PPH is pulmonary venoocclusive disease (PVOD), a rare idiopathic condition that diffusely affects the postcapillary pulmonary circulation but is characterized by normal pulmonary capillary wedge pressures (PCWP). Both precapillary and postcapillary pulmonary hypertension are regarded as secondary (SPH) when a cause can be established (Table 130.1).

Clinical Findings

Pulmonary Artery Hypertension

Precapillary hypertension creates a hemodynamic pattern of elevated right atrial pressure, increased mPAP, increased pulmonary vascular resistance, normal PCWP, and decreased cardiac output. Postcapillary hypertension is characterized by elevated right atrial pressure, increased resistance, and normal or elevated PCWP. Patients present with progressive dyspnea (60%), fatigue, angina, syncope, cor pulmonale, and Raynaud phenomenon. Women are affected three times as often as men. Onset of symptoms is typically in youth or middle age. Patients with portal hypertension (with or without liver disease), collagen vascular disease, HIV-AIDS, or a history of aminorex fumarate (an appetite suppressant) ingestion have an increased risk. Additionally, pregnant or postpartum patients and those using oral contraceptives may also be at increased risk.

Pulmonary Venoocclusive Disease (PVOD)

In addition to the hemodynamic derangements of PAH discussed above, PVOD is associated with normal or variably elevated PCWP. Normal left atrial and left ventricular pressures are characteristic and help exclude cardiac disease as the cause of venous hypertension. Patients present with progressive dyspnea, hemoptysis, and antecedent flulike symptoms. One-third of cases occur in children. There is a slight male predominance in adult patients. PVOD may be associated with pregnancy, bone marrow transplantation, or drug toxicity (e.g., carmustine, bleomycin, mitomycin). The clinical presentation and radiographic findings are suggestive of interstitial lung disease often leading to a delay in diagnosis.

Table 130.1 Potential Causes of Pulmonary Artery Hypertension

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Jan 14, 2016 | Posted by in RESPIRATORY IMAGING | Comments Off on 130 Chronic Thromboembolic Disease Pulmonary Hypertension (CTEPH)

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Primary (Precapillary or Arterial) Pulmonary Hypertension

Primary (Postcapillary or Venous) Pulmonary Hypertension


Pulmonary venoocclusive disease (PVOD)

Secondary (precapillary or arterial) pulmonary hypertension

Secondary (postcapillary or venous) pulmonary hypertension

Congenital left-to-right cardiac shunt (e.g,, ASD, eisenmenger physiology)

Pulmonary capillary hemangiomatosis


Focal venous constriction (e.g., congenital venous stenosis, fibrosing mediastinitis)

Tumor embolism (e.g., breast cancer, gastric cancer, renal cell, right atrial sarcoma, hepatoma)

Obstructive left atrial mass (e.g., left atrial neoplasia, myxoma, mitral stenosis, left ventricular failure)

Infection (e.g., HIV-AIDS, schistosomiasis)

Compromised pulmonary venous drainage (e.g., anomalous pulmonary venous connections)

IVDA (e.g., talc and other foreign body embolization)