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Assessing Nutritional Risk Can Predict PAH Prognosis At First Hospitalization

Adjunct nutritional therapies may be a simple way to improve the prognosis for patients with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension who are hospitalized.

Using the Geriatric Nutritional Risk Index (GNRI) when patients with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH) are hospitalized can help predict their prognosis, according to a study published in Circulation Reports.

PAH and CTEPH are considered precapillary pulmonary hypertension (PH). Among patients with precapillary PH, "undernutrition may trigger or aggravate disease progression. However, it is unclear whether undernutrition at the point of diagnosis is significantly associated with prognoses in patients with precapillary PH," the authors explained.

The study included 104 patients with either PAH or CTEPH who were treated at the Kagoshima University Hospital in Japan. The majority of patients (84.6%) were female and 68.3% were World Health Organization functional class III or IV; the mean age was 60 years.

A score of < 92 was considered a low GNRI and ≤ 92 was considered a high GNRI. Patients with a low GNRI were more likely to be younger (54 years) compared with the patients with a high GNRI (61 years). Patients in the low-GNRI group also had significantly lower body mass index, serum albumin levels, and hemoglobin.

The median follow-up period was 24 months, during which 16 (15.4%) patients died. The composite outcome of either rehospitalization or all-cause death occurred in nearly half (45.5%) of patients in the low-GNRI group compared with 20.7% of patients in the high-GNRI group. After adjusting for potentially confounding covariates, the researchers still found a higher likelihood of all-cause death or rehospitalization for patients with low GNRI.

"Taken together, these results suggest that undernutrition at the time of diagnosis is a potent prognosticator in patients with PH," the authors wrote.

They did list a number of limitations, including the relatively small sample size and the retrospective nature of the study. The study only evaluated GNRI scores once, so they could not assess changes in GNRI. In addition, they admitted there may have been unmeasured confounding factors that were not excluded.

The authors suggested that further studies evaluate the effectiveness of nutritional interventions in patients with PAH and CTEPH since undernutrition and weight loss are frequent among them.

"The present study suggests that the GNRI score at first hospitalization may be a simple predictor of prognosis in patients with PAH and CTEPH," they concluded. "Adjunct nutritional therapies may improve outcomes in such patients."

Reference

Kubota K, Miyanaga S, Iwatani N, et al. Geriatric nutritional risk index is associated with prognosis in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Circ Rep. 2020;2(7):372-377. Doi:10.1253/circrep.CR-20-0046

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Identifying Subpopulations Of PH And PAH

Considerations for the identification of specific subpopulations of PH and PAH, as well as their impact on outcomes and treatment goals.

Transcript

Hilary M. DuBrock, MD: Chronic thromboembolic pulmonary hypertension [CTEPH] is Group 4 PH [pulmonary hypertension]. These patients have pulmonary hypertension that develops due to chronic thrombi and organized thrombus that develop in the pulmonary blood vessels. This, over time, can lead to right heart failure and even death if not treated. CTEPH is characterized by precapillary pulmonary hypertension with an elevated mean PA [pulmonary artery] pressure, pulmonary vascular resistance, and a normal pulmonary artery wedge pressure. However, we categorize it differently than idiopathic Group 1 pulmonary arterial hypertension [PAH] because there's a different approach to treatment of chronic thromboembolic pulmonary hypertension.

Patients with CTEPH need to first and foremost be evaluated for surgical intervention, which is pulmonary thromboendarterectomy. In patients who are not candidates for surgery, either because they have distal disease or because they have comorbidities that would preclude surgical intervention, we consider other options for treatment such as balloon pulmonary angioplasty or medical therapy with medications such as riociguat, a soluble guanylate cyclase stimulator.

Connective tissue disease—associated pulmonary arterial hypertension is a subgroup of Group 1 PAH. Patients with connective tissue disease–associated pulmonary arterial hypertension have PAH that develops in the context of different types of connective tissue disease, such as scleroderma, lupus, mixed connective tissue disease, polymyositis, and dermatomyositis, among other disorders. Patients with connective tissue disease–associated pulmonary arterial hypertension tend to have a worse prognosis and respond less well to PAH-directed therapy when compared to patients with idiopathic pulmonary arterial hypertension. Among patients with connective tissue disease pulmonary arterial hypertension, scleroderma is the most common cause of connective tissue disease that is associated with PAH.

Portopulmonary hypertension refers to a subgroup of Group 1 pulmonary arterial hypertension, in which pulmonary arterial hypertension develops in patients with either cirrhotic or non-cirrhotic portal hypertension. These patients tend to have a worse prognosis compared to idiopathic or connective tissue disease—associated pulmonary arterial hypertension, despite having better hemodynamics at the time of diagnosis. Additionally, a mainstay of treatment goals in patients with portopulmonary hypertension is to improve their eligibility for a procedure such as liver transplantation, if that is needed to treat their underlying liver disease. The treatment goals in portopulmonary hypertension tend to be different from goals in other forms of PAH.

Charles D. Burger, MD: Physicians always love to talk about diagnosis. That's one of the reasons we get into the field: to figure out what's going on with the patient to determine the diagnosis and the best treatment recommendations. Really, it starts with your interview with the patient. For example, if you're looking for the possibility of connective tissue disease like scleroderma with the patient, you may be asking them about symptoms of scleroderma, which include joint pain, changes in their fingers where the skin thickens, or when the hands will turn different colors when exposed to cold. That's called Raynaud phenomenon, and you're asking about that.

In a similar way, you're looking for these findings on physical examination. One of the hallmarks of disease are tiny spider-type reddenings of the skin that are referred to as telangiectasias. If these are prevalent or if they've been increasing in incidence recently, you worry about the patient possibly having pulmonary arterial hypertension. Your examination might show signs of this, but you often have to move forward, as you might imagine, to diagnostic evaluation.

Generally, you screen for pulmonary hypertension in someone with scleroderma with an echocardiogram. That might show you elevated right heart pressures. It might show you strain on the right heart. And then, you need to move forward with the confirmatory test, which is a right heart catheterization, regardless of the type of pulmonary hypertension. That will show you a profile that would confirm the presence of Group 1 PAH in a patient with connective tissue disease.

Similarly, if you're worried about liver disease, there might be a history of risk factors for liver disease, such as having chronic hepatitis. You're looking for signs on examination that might indicate the liver isn't functioning properly, such as fluid that will accumulate in the abdomen, or what's referred to as ascites. As you move forward with screening for pulmonary hypertension, you will use an echocardiogram and a right heart catheterization just as with connective tissue diseases, but it also involves some assessment of liver function, which involves blood tests, imaging of the liver, and generally, consultation with a gastroenterologist with a specific interest in liver disease.

Group 4 chronic thromboembolic disease pulmonary hypertension is a little different, and it can be subtle because not all patients have a history of having a blood clot in their legs or their lungs. They may not be aware that this is the cause. As you're moving through your diagnostic evaluation, it's very important for providers to get a ventilation/perfusion lung scan. This is the screening test of choice based on guideline recommendations that would help you uncover the possibility of chronic thromboembolic disease pulmonary hypertension. It will be abnormal in those patients. Additional testing might be required, such as a pulmonary angiogram or a CT [computed tomography] scan with contrast, to further delineate the scope of the problem.

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COVID-19 Leads To High In-hospital Mortality For Patients With Pulmonary Hypertension

September 29, 2022

2 min read

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In a cohort of patients with chronic precapillary pulmonary hypertension, the rates of in-hospital mortality for those who contracted COVID-19 were more than 40%, according to a study.

Study investigator David Montani, MD, PhD, explained that SARS-CoV2 has a predilection for causing pulmonary vascular injury and that patients with pre-existing pulmonary vascular disease could therefore be at increased risk for adverse outcomes from COVID-19. "Conversely, pulmonary arterial hypertension therapies, which improve pulmonary endothelial function, could protect from SARS-CoV2 effects on the pulmonary endothelium of PAH patients," Montani, who is professor of respiratory medicine at the Bicêtre Hospital in Paris, told Healio. "There is limited data available on the outcomes of COVID-19 in patients with pulmonary hypertension (PH)."

Source: Adobe Stock.

For the study, which was published in the American Journal of Respiratory and Critical Care Medicine, Montani and colleagues prospectively collected characteristics, management and outcomes of adults with precapillary PH in the French PH registry. Patients had contracted COVID-19 during the first year of the COVID-19 pandemic.

The study included 211 patients with PH — 123 with PAH, 47 with chronic thromboembolic PH and 41 with other types of PH — who experienced COVID-19; among them, 40.3% were outpatients, 32.2% were hospitalized in a conventional ward and 27.5% were in an ICU.

Treatment for hospitalized patients (n = 126) included corticosteroids (54%), high-flow oxygen (37.3%) and invasive ventilation (11.1%).

Researchers observed an overall mortality of 24.6% (95% CI, 18.8-30.5) and an in-hospital mortality of 41.3% (95% CI, 32.7-49.9). Overall, non-survivors were older than survivors (median age, 69.4 years vs. 61.8 years; P <.001) and a greater proportion were men (67.3% vs. 38.4%). Also, more non-survivors vs. Survivors had comorbidities — including chronic respiratory disease (61.5% vs. 26.4%; P < .001) systemic hypertension (53.8% vs. 32.7%; P < .01) diabetes (30.8% vs. 16.4%; P = .02) and chronic renal failure (51.9% vs. 19.5%; P < .001) —and more severe PH at their most recent evaluation preceding COVID-19 diagnosis.

In addition, findings revealed that the use of PAH therapy was comparable between survivors and non-survivors, although a smaller proportion of deceased patients had been receiving anticoagulation therapy (42.3% vs. 59.1%; P = .03).

"This study demonstrates that patients with pulmonary hypertension are at high risk of COVID-19 mortality, illustrating the importance of vaccination, early treatment and other preventative measures in this population," Montani said.

Sources/DisclosuresCollapse Disclosures: Montani reports receiving grants or contracts to the institution from Acceleron, Janssen and Merck; consulting fees from Acceleron; and honoraria from Bayer, Janssen and Merck. Please see the study for all the other authors' relevant financial disclosures.

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