Chronic thromboembolic pulmonary hypertension or CTEPH is a rare but fatal disease that affects the lungs. It occurs when blood clots obstruct the pulmonary arteries, leading to increased blood pressure in the lungs.

Over time, this can lead to heart failure and death. The incidence of CTEPH is estimated to be around 5-40 per million individuals per year. While medical therapy is available for CTEPH, it is often inadequate, leading to a poor prognosis.

However, a lifesaving surgical procedure known as pulmonary endarterectomy or PEA can improve the symptoms and prognosis of CTEPH.

What is Pulmonary Endarterectomy?

Pulmonary endarterectomy or PEA is a surgical procedure that involves removing organized blood clots or thromboembolic material from the pulmonary arteries.

The surgery is performed under general anesthesia, and the patient’s heart and lungs are temporarily stopped to allow the surgeon to remove the clots effectively. The procedure typically lasts 4-6 hours. After surgery, the patient is placed on mechanical ventilation to help with breathing. The patient is also given anticoagulant therapy to prevent further clot formation.

Can All Patients with CTEPH Benefit from Pulmonary Endarterectomy?

Not all patients with CTEPH are suitable candidates for pulmonary endarterectomy. The surgery is only recommended for patients with proximal or central pulmonary artery obstruction and a favorable hemodynamic assessment.

The hemodynamic assessment involves measuring the blood flow and pressure in the lungs to assess if the surgery is likely to succeed. The evaluation also considers the patient’s general health, presence of comorbidities, and support systems for postoperative care. Unfortunately, many patients with CTEPH are not diagnosed early enough to be eligible for pulmonary endarterectomy.

Case Series of Eight Patients with CTEPH Who Underwent Pulmonary Endarterectomy

A recent case series published in the Journal of Cardiothoracic Surgery documented the experiences of eight patients with CTEPH who underwent pulmonary endarterectomy. The patients, four men and four women, were aged between 25 and 67 years.

They all presented with symptoms such as shortness of breath, fatigue, and chest pain. The time between symptom onset and diagnosis ranged from 6 months to 7 years.

Before surgery, all patients underwent a thorough medical evaluation, including a ventilation-perfusion scan and right heart catheterization, to assess the extent and severity of their CTEPH.

The hemodynamic assessment showed that all patients had proximal or central artery involvement and a favorable prognosis for surgery. The patients underwent surgery between 2013 and 2018 at a single center specialized in pulmonary endarterectomy.

The results were encouraging. All eight patients survived surgery, and none required extracorporeal membrane oxygenation or mechanical circulatory support. The average length of stay in the ICU was two days, and the average hospital stay was 14 days.

Postoperatively, all eight patients reported an improvement in their symptoms and quality of life, including reduced fatigue and shortness of breath. Follow-up evaluations over a period of 1-2 years showed sustained improvements in hemodynamic parameters, including mean pulmonary artery pressure and pulmonary vascular resistance.

Conclusion

Pulmonary endarterectomy is a surgical procedure that offers a chance of cure for patients with CTEPH. Early diagnosis and referral to specialized centers with experience in the surgery are crucial for optimal outcomes.

The case series of eight patients with CTEPH who underwent pulmonary endarterectomy showed favorable outcomes, with all patients surviving surgery and experiencing improvements in their symptoms and hemodynamic parameters. Further studies are needed to determine the long-term outcomes of the surgery and the factors that predict success.