Results From the United States Chronic Thromboembolic Pulmonary Hypertension Registry: Enrollment Characteristics and 1-Year Follow-up.

Background: The United States Chronic Thromboembolic Pulmonary Hypertension Registry (US-CTEPH-R) was designed to characterize the demographic characteristics, evaluation, clinical course, and outcomes of surgical and nonsurgical therapies for patients with chronic thromboembolic pulmonary hypertension.

Research Question: What are the differences in baseline characteristics and 1-year outcomes between operated and nonoperated subjects?

Study Design And Methods: This study describes a multicenter, prospective, longitudinal, observational registry of patients newly diagnosed (< 6 months) with CTEPH. Inclusion criteria required a mean pulmonary artery pressure ≥ 25 mm Hg documented by right heart catheterization and radiologic confirmation of CTEPH.

Choriocarcinoma presenting with thyrotoxicosis.

We describe a 26-year-old man with metastatic choriocarcinoma who presented with hyperthyroidism associated with elevated β-human chorionic gonadotropin (B-HCG) and respiratory failure secondary to diffuse lung metastasis. After the first cycle of chemotherapy, the concentration of B-HCG dramatically decreased and the patient became euthyroid, allowing us to discontinue antithyroid medications. The patient's hyperthyroidism was caused by stimulation of the thyroid gland by high B-HCG levels, as shown by the marked improvement of the patient's thyroid function panel after chemotherapy.

Efficacy of a Low-Tidal Volume Ventilation Strategy to Prevent Reperfusion Lung Injury after Pulmonary Thromboendarterectomy.

Rationale: Reperfusion lung injury is a postoperative complication of pulmonary thromboendarterectomy that can significantly affect morbidity and mortality. Studies in other postoperative patient populations have demonstrated a reduction in acute lung injury with the use of a low-tidal volume (Vt) ventilation strategy. Whether this approach benefits patients undergoing thromboendarterectomy is unknown.

Pilot study comparing SPECT perfusion scintigraphy with CT pulmonary angiography in chronic thromboembolic pulmonary hypertension.

Background And Objective: The management of chronic thromboembolic pulmonary hypertension (CTEPH) is dependent on the extent of pulmonary artery obstruction, which is usually evaluated by planar perfusion scanning and CT pulmonary angiography (CTPA). We previously reported that SPECT perfusion scanning is more sensitive than planar scanning for detecting vascular obstruction in CTEPH. The purpose of this study is to compare SPECT with CTPA for detecting segmental pulmonary artery obstruction in CTEPH.

Single photon emission computed tomography in chronic thromboembolic pulmonary hypertension.

Background And Objective: The management of chronic thromboembolic pulmonary hypertension (CTEPH) is largely dependent on the extent of obstruction in the pulmonary arteries. Planar perfusion scans are commonly used to quantify perfusion defects in CTEPH patients. However, planar scans typically under-represent the extent of vascular obstruction in CTEPH.

Chronic thromboembolic pulmonary hypertension in pediatric patients.

Objective: Chronic thromboembolic pulmonary hypertension is a rare form of pulmonary hypertension that can lead to progressive right heart failure and death. Pulmonary thromboendarterectomy surgery is the treatment of choice resulting in significant improvements in functional status, cardiopulmonary hemodynamics, and survival. This study reports the largest case series of pediatric patients with chronic thromboembolic pulmonary hypertension who underwent pulmonary thromboendarterectomy surgery at one institution.

Pulmonary hypertensive medical therapy in chronic thromboembolic pulmonary hypertension before pulmonary thromboendarterectomy.

Background: The currently recommended treatment for chronic thromboembolic pulmonary hypertension is pulmonary thromboendarterectomy (PTE). No convincing evidence for the use of pulmonary hypertensive medical therapy (PHT) exists in operable candidates. We sought to determine the prevalence of the use of PHT on referral for PTE and the effects on pre-PTE hemodynamics and post-PTE outcomes/hemodynamics.

Chronic thromboembolic pulmonary hypertension.

The description of organized thrombus in major pulmonary arteries can be found in autopsy reports dating back to the late nineteenth and early twentieth centuries. Not until the 1950s was the antemortem diagnosis and clinical syndrome of chronic thrombotic obstruction of the major pulmonary arteries better characterized. The first surgical attempt to remove the adherent thrombus from the vessel wall occurred in 1958.