(PDF) Unilateral pulmonary edema: A case report and review of the literature
Chronic Thromboembolic Pulmonary Hypertension
Chronic thromboembolic pulmonary hypertension (CTEPH) is high blood pressure in the arteries in your lungs. You can get it after you've had a pulmonary embolism (PE) -- a blood clot in your lung. There are about 5,000 new cases a year. Around 1% to 5% of people who've had a PE will get it.
Doctors often find it hard to diagnose CTEPH because the symptoms are a lot like ones they see in other diseases. Typically, symptoms come on slowly. They may include:
Rarely, CTEPH can also make you cough up blood.
Scarring from blood clots is behind CTEPH. When you get blood clots in your lungs, they can turn into scar tissue. Scar tissue makes blood vessels narrower, or can even block them. Blocked or narrowed arteries make it harder for your heart to pump blood through them. This raises your blood pressure. Your heart muscle can also get weaker over time.
Things that raise your chances of a blood clot in your lungs include:
If you already have pulmonary hypertension before you get a blood clot in your lung, you're more likely to get CTEPH. Your risk also goes up if you have one or more of the following:
Certain types of cancers, blood diseases, or inflammatory diseases can raise the chances that you'll get it, too.
CTEPH can be hard to diagnose. There are tests that can help:
The most common way to treat CTEPH is through surgery. A pulmonary thromboendarterectomy (PTE), also called a pulmonary endarterectomy, cures the condition for over 90% of people who have it.
During the surgery, a surgeon stops your heart, and a heart-lung bypass machine takes over to move your blood. The surgeon then removes the clots from your lungs.
Since the surgery is tricky, it's best to have it done by a surgeon who's familiar with it. If you don't have access to one, or have other medical conditions that make the surgery risky, there are other treatments. A balloon pulmonary angioplasty is a procedure where doctors inflate a tiny balloon inside your lung arteries. This helps widen them to lower the pressure. There are also medications your doctor can prescribe to help treat it if you can't have surgery.
To help keep your risk of CTEPH low, your doctor will likely put you on anticoagulants, or blood thinners. These medicines help stop clots from forming in your blood. A low-salt diet can also help lower your blood pressure. Doctor-approved exercise is also key to helping you manage your blood pressure and keep your heart and lungs healthy.
Promising Findings For The Treatment Of Patients With Pulmonary Arterial Hypertension With A High Risk Of Mortality
Pulmonary arterial hypertension (PAH) is a rare and severe lung disease with a life-threatening prognosis. After several positive trials, a recent study has confirmed the efficacy of a biotherapy, sotatercept. These promising results for patients were presented at the American College of Cardiology 2025 Annual Scientific Session (ACC.25) by Marc Humbert, professor at Université Paris-Saclay, head of the Pneumology Department at Bicêtre Hospital (AP-HP), director of the joint research unit for Pulmonary Hypertension: Pathophysiology and Novel Therapies (UMR 999 – Univ. Paris-Saclay/Inserm) and coordinator of the French Pulmonary Hypertension reference centre.
Pulmonary arterial hypertension (PAH) causes gradual shortness of breath at exercise and then at rest, fainting or even syncope, and can be life-threatening. It is caused by an abnormal increase in blood pressure in the arteries running from the right side of the heart to the lungs. Over time, the small pulmonary arteries thicken and become blocked due to a progressive accumulation of cells in the vascular wall. Sotatercept works as an "activin receptor ligand trap" and aims to restore pulmonary vascular homeostasis by preventing the excessive activation of vascular proliferation pathways.
A previous phase 3 trial (which allowed for a potential market launch to be considered) had already produced positive results, but a new trial, named ZENITH, offers new hope for high-risk patients despite receiving maximal background therapy for PAH. It has just demonstrated the efficacy of sotatercept, a drug marketed by Merck, in significantly reducing the risk of death, lung transplantation or prolonged hospitalization for more than 24 hours compared with placebo. As early as the interim analysis, which was scheduled after 50% of the events planned within the framework of the trial, it was demonstrated that patients receiving sotatercept had a 76% relative risk reduction in morbidity and mortality events compared with patients taking placebo. At a median follow-up of less than one year, 17.4% of patients treated with sotatercept experienced at least one of these events, compared with 54.7% in the placebo group (p<0.0001).
The trial has shown that adding sotatercept to the maximum-tolerated standard treatment for PAH reduces the risk of death or clinical worsening events and improves exercise capacity and functional class compared with standard PAH treatments alone."
Prof Marc Humbert, Director of the Pulmonary Hypertension Reference Centre, AP-HP, Université Paris-Saclay
Based on these results and for ethical reasons, the study was stopped early by an independent monitoring committee to give patients receiving placebo the opportunity to replace it with sotatercept, to avoid compromising their chances of benefiting from effective treatment. This is the first time in the field of PAH treatment trials that an interim analysis has required a study to be stopped due to efficacy.
The findings were presented at the American College of Cardiology 2025 Annual Scientific Session (ACC.25) and published simultaneously in The New England Journal of Medicine.
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