How Do We Determine If an SSc Patient Needs to Be Sent to a PH Specialty Center?

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Dr. Krasuski:
How do we determine if a systemic sclerosis patient needs to be sent to a PH specialty center? Here is the rationale for the two steps of the DETECT algorithm. Again, in step one, this is non echocardiographic data. These are six variables that can be easily obtained by a rheumatologist to then determine the need for echocardiography. In step two, again, if an echo is indicated, we look at two specific variables and then a cardiologist determines whether the next step, a heart catheterization is necessary. So, by using the two steps to determine referral for right heart catheterization, DETECT optimizes resource usage and reduces the burden on particular medical departments.

Here is the DETECT protocol. And the first step, again, clinical information is looked at if, in fact, this score that's calculated is elevated here, an echo is then recommended. Then this echo is looked at specifically for these characteristics including right atrial area TR velocity. And then if this then is highly scores, again the patient should proceed to a heart catheterization. A smartphone app has been developed for this to make this easier for the physicians in the community to identify the patients that need to proceed through the scoring system. Collaborative assessment of the pH patient. This is data to collect and where testing should occur. There are a number of different variables that have been shown to predict outcome in patients with pulmonary arterial hypertension. In yellow here you can see the variety of different clinical characteristics that can predict survival and other outcomes in patients with pulmonary arterial hypertension. Some of this testing should occur in the community level or the PH center. And you can see these are the characteristics that are reasonable to consider early on in the diagnostic process. And then when the patient reaches the specialty center, certain things such as echocardiography, if it hasn't already been performed a VQ scan often is best performed in these centers. And finally hemodynamic evaluation.

The revised diagnostic algorithm must be seen in two parts. Step one, triage and diagnosis of common conditions are done in the community. So again, you have patients with a history that's suggestive, they have some symptoms and or a laboratory test that are suggestive of pulmonary hypertension. They undergo an echocardiogram. If that echocardiogram is a low-risk echocardiogram consider other causes, there are many different causes of dyspnea, chest pain, other of the symptoms that these patients can present with. If they have high or intermediate risk of pulmonary hypertension, for some of these patients a fast-track referral should occur. And other of these patients proceeding with their additional workup, including a VQ scan to screen for CTEPH, considering a VQ scan to screen for CTEPH. If left heart disease is a high probability, again, this should be looked at, some patients may have intrinsic lung disease as well. If there's no clinically significant left heart disease or lung disease ideally these are patients that net then need to be sent to a PH expert center. With echo, there's a variety different criteria here but if you're just looking at the TR velocity if the TR velocity is 2.9 or higher, this is someone you should be very concerned has pulmonary hypertension and even possibly pulmonary arterial hypertension. But it's important to recognize that PH often has many different ideologies, particularly left heart disease.

Now in the revised diagnostic algorithm step two is the role of the PH expert center. Ideally, these are patients that probably need to have their VQ scan performed at the specialty center but at the least that VQ scan should be reviewed at the specialty center. If there's any mismatch, perfusion defects the patients should be carefully evaluated for the presence of possible chronic thromboembolic pulmonary hypertension. Some of these patients will in fact not have pulmonary hypertension. Some of these may have CTEPH that is known as CTED. In which case, it becomes a little more challenging to know whether interventions are indicated or not. In some cases, basically, CTEPH is confirmed. And in that case, classifying how to approach these patients and making sure that surgery is considered is very important. But review by a multidisciplinary PH team is particularly important in the patient with CTEPH and assessing that patient and ensuring they get rapid medical therapy and potentially surgery and or balloon pulmonary angioplasty.

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