History

Patients usually present with exercise intolerance, fatigue, and progressive dyspnea. Because these symptoms are nonspecific, they are typically attributed to more common diseases such as obstructive lung disease, cardiac abnormalities, obesity, and deconditioning. When pulmonary pressures continue to increase and right ventricular failure develops, chest discomfort, syncope, hemoptysis, light-headedness, and peripheral edema can occur.^[29]

The symptoms arise from limitations in cardiac output caused by an increased pulmonary vascular resistance (PVR) and increased minute ventilation requirements secondary to increased dead space ventilation. It is essential that clinicians take a careful history in order to recognize previous events consistent with venous thromboembolism and other risk factors (see Risk Factors). Although most patients with chronic thromboembolic pulmonary hypertension (CTEPH) have a history of at least one previous acute thromboembolic event, approximately 30-40% have no such history, thereby making the diagnosis of CTEPH more challenging.^[13]

Physical Examination

Findings of CTEPH on physical examination may include a diminished or fixed splitting of the second heart sound (S2), accentuation of the pulmonic closure sound (P2), a palpable right ventricular heave, jugular venous distention, a right-side third heart sound (S3), tricuspid regurgitation, hepatomegaly, ascites, and peripheral edema. Bruits may be heard on auscultation over the peripheral lung fields; they rise from turbulent flow through partially obstructed pulmonary arteries. Cyanosis may be a sign of right-to-left shunting through a patent foramen ovale.

Complications

If not appropriately diagnosed and treated, CTEPH results in right ventricular failure, severe hypoxemic respiratory failure with need for mechanical ventilation, cardiogenic shock, and death. If CTEPH is diagnosed in a timely manner, treatment options can also carry potential complications. Different medical and surgical therapies for CTEPH and complications thereof are discussed in Complications in the Treatment section.

Differential Diagnoses

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Media Gallery

Ventilation perfusion scan showing bilateral large wedge-shaped mismatched perfusion defects and areas of gray indicating decreased perfusion. Right-sided heart catheterization for this patient showed combined precapillary and postcapillary pulmonary hypertension and the following: right atrial pressure 10 mm Hg, right ventricular pressure 82/8 mm Hg, pulmonary artery wedge pressure 22 mm Hg, and pulmonary artery pressure 83/27 mm Hg with a mean of 49 mm Hg. He was referred to an expert center for pulmonary endarterectomy evaluation, where he underwent pulmonary angiography. Findings from the right side showed an occluded upper lobe anterior segment, a proximal web in the upper lobe, and disease in all lower segments. Findings from the left side showed an occluded superior segment of the lower lobe with disease in basal segments, proximal web in lingula, and intact upper lobe vessels. He underwent pulmonary endarterectomy with intraoperative University of California San Diego classification of thrombus as right 1 and left 2. Postoperatively, he has had dramatic improvement in his symptoms and is off all pulmonary arterial hypertension therapy.
Extensive pulmonary emboli are seen in both lungs, with a dominant, eccentric thrombus related to the left main pulmonary artery. More distal emboli were noted in the subsegmental pulmonary arteries (not shown). This is a 42-year-old woman with a history of multiple pulmonary emboli and protein S deficiency who initially presented with severe dyspnea (New York Heart Association functional class IV) and was initiated on multiple pulmonary arterial hypertension medications, including intravenous prostacyclin. She underwent pulmonary endarterectomy, and the images of the clots that were removed intraoperatively are shown in another image.
The heart is enlarged, with right atrial and ventricular enlargement in addition to reflux of contrast into the intrahepatic inferior vena cava. The main pulmonary artery itself measures approximately 3 cm. There was mosaic attenuation of both lungs, most pronounced related to the right-middle and upper lobes (not shown).
Mosaic pattern seen on expiratory images of a chest CT scan in a patient with chronic thromboembolic pulmonary hypertension. The same patient’s ventilation/perfusion scan is also shown. The areas of hypovascularity in blood vessels with clots cause areas of relative hypoperfusion, which appear darker than normal lung and give rise to the differential opacities seen on this image.
Intraoperative clot during pulmonary endarterectomy. This is a 42-year-old woman with a history of multiple pulmonary emboli and protein S deficiency who initially presented with severe dyspnea (New York Heart Association functional class IV) and was initiated on multiple pulmonary arterial hypertension medications, including intravenous prostacyclin. Preoperative right-sided heart catheterization showed right atrial pressure of 16 mm Hg, pulmonary artery pressure of 124/29 mm Hg with a mean of 67 mm Hg, cardiac output of 4.53, and pulmonary vascular resistance of 12 Wood units. Immediate postoperative right-sided heart catheterization showed right atrial pressure of 9 mm Hg, pulmonary artery pressure of 40/15 mm Hg with a mean of 25 mm Hg, cardiac output of 5.15, and pulmonary vascular resistance of 2.9 Wood units. She came off all her pulmonary arterial hypertension therapy immediately postoperatively and continues to do well.

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Group 4 Pulmonary Hypertension Clinical Presentation

History

Physical Examination

Complications

References

Tables

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