New treatment offers hope for patients with devastating lung disease

• First time in South Africa that a minimally invasive surgical procedure successfully used balloons to dilate blocked arteries in the lungs.
• Procedure offers hope for patients with so-called “orphan disease” who generally have a very poor quality of life. 

In a first for South Africa, and a watershed moment for patients with a devastating lung disease known as chronic thromboembolic pulmonary hypertension (CTEPH), a team of cardiologists from Stellenbosch University’s (SU) Faculty of Medicine and Health Sciences (FMHS) and Tygerberg Hospital recently performed a new, minimally invasive surgical procedure known as balloon pulmonary angioplasty (BPA) on three patients. 

During this procedure, the team led by Drs Hellmuth Weich and Lloyd Joubert of the FMHS, used small balloons to dilate blocked arteries in the lungs of patients affected by CTEPH. Weich and Joubert were supported by Prof Irene Lang from Vienna, Austria, who is a world leader in this field.
 

CTEPH is a rare and progressive form of pulmonary hypertension and is caused by recurrent blood clots that prevent blood from flowing through the lungs. Patients become increasingly short of breath, and most of them wait more than two years before the correct diagnosis is made, as the disease is often misdiagnosed in its early stages. If left untreated, heart failure will eventually result as the blockage to blood flow increases beyond the heart’s ability to pump.

Patients with CTEPH frequently have a very poor quality of life in South Africa, with limited treatment options. Because of this, it has traditionally been considered an “orphan disease” and many patients have been left without hope.

Improvement in the treatment of CTEPH patients at Tygerberg Hospital commenced some time ago, when Prof Brian Allwood from the FMHS’ Division of Pulmonology was trained in the diagnosis and management of such patients at Massachusetts General Hospital and Harvard University in Boston, USA.

For some patients, surgical removal of larger clots is an alternative and potentially curative treatment option. However, this is not possible for many patients due to the location of clots in the lungs, as well as the poor general health of patients with advanced disease. 

BPA is a less invasive approach, and sometimes the only option for patients, but until now it has not been performed with success in South Africa.
 

The team of Tygerberg’s Division of Cardiology has gained significant experience in life-saving interventions for major bleeding from the lungs due to tuberculosis and its complications. Although this is a very different condition to CTEPH, the approach to these interventions in the lung arteries is not dissimilar to BPA and this was a first step in the launching of a BPA programme.

However, because there was no expertise with this procedure in South Africa and having a number of patients needing BPA, the team approached Lang, who runs one of the biggest BPA programmes in Europe, for assistance.

All three patients who received the minimal invasive BPA treatment, had suffered from extreme, long-term shortness of breath which has been resistant to medication. Although this was the first in a series of BPA treatments for each of them, it is likely that they will all return to a much-improved level of functioning and enjoy a better quality of life.
 

As part of a workshop in the management of CTEPH, the team also invited Prof Michael Madani, Chief of Cardiovascular and Thoracic Surgery at the University of California, San Diego to Cape Town and Tygerberg Hospital. Chronic blood clots can be removed surgically by means of a surgical procedure known as pulmonary thromboendarterectomy (PTE), as the more conventional alternative to BPA. The team of cardiothoracic surgeons at Tygerberg is experienced at performing PTE, but were fortunate to have Madani, a world leader in the procedure, to assist Prof Jacques Janson, Head of the Division of Cardiothoracic Surgery, with such a procedure on 3 May.