田邉 信宏

BACKGROUND: Recent guidelines discourage the use of pulmonary arterial hypertension (PAH)-targeted therapies in patients with pulmonary hypertension (PH) associated with respiratory diseases. Therefore, stratifications of the effectiveness of PAH-targeted therapies are important for this group. OBJECTIVES: The authors aimed to identify phenotypes that might benefit from initial PAH-targeted therapies in patients with PH associated with interstitial pneumonia and combined pulmonary fibrosis and emphysema. METHODS: We categorized 270 patients with precapillary PH (192 interstitial pneumonia, 78 combined pulmonary fibrosis and emphysema) into severe and mild PH using a pulmonary vascular resistance of 5 WU. We investigated the prognostic factors and compared the prognoses of initial (within 2 months after diagnosis) and noninitial treatment groups, as well as responders (improvements in World Health Organization functional class, pulmonary vascular resistance, and 6-minute walk distance) and nonresponders. RESULTS: Among 239 treatment-naive patients, 46.0% had severe PH, 51.8% had mild ventilatory impairment (VI), and 40.6% received initial treatment. In the severe PH with mild VI subgroup, the initial treatment group had a favorable prognosis compared with the noninitial treatment group. The response rate in this group was significantly higher than the others (48.2% vs 21.8%, ratio 2.21 [95% CI: 1.17-4.16]). In multivariate analysis, initial treatment was a better prognostic factor for severe PH but not for mild PH. Within the severe PH subgroup, responders had a favorable prognosis. CONCLUSIONS: This study demonstrated an increased number of responders to initial PAH-targeted therapy, with a favorable prognosis in severe PH cases with mild VI. A survival benefit was not observed in mild PH cases. (Multi-institutional Prospective Registry in Pulmonary Hypertension associated with Respiratory Disease; UMIN000011541).

BACKGROUND: Pulmonary arterial hypertension is a serious medical condition. However, the condition is often misdiagnosed or a rather long delay occurs from symptom onset to diagnosis, associated with decreased 5-year survival. In this study, we developed and tested a deep-learning algorithm to detect pulmonary arterial hypertension using chest X-ray (CXR) images. METHODS: From the image archive of Chiba University Hospital, 259 CXR images from 145 patients with pulmonary arterial hypertension and 260 CXR images from 260 control patients were identified; of which 418 were used for training and 101 were used for testing. Using the testing dataset for each image, the algorithm outputted a numerical value from 0 to 1 (the probability of the pulmonary arterial hypertension score). The training process employed a binary cross-entropy loss function with stochastic gradient descent optimization (learning rate parameter, α = 0.01). In addition, using the same testing dataset, the algorithm's ability to identify pulmonary arterial hypertension was compared with that of experienced doctors. RESULTS: The area under the curve (AUC) of the receiver operating characteristic curve for the detection ability of the algorithm was 0.988. Using an AUC threshold of 0.69, the sensitivity and specificity of the algorithm were 0.933 and 0.982, respectively. The AUC of the algorithm's detection ability was superior to that of the doctors. CONCLUSION: The CXR image-derived deep-learning algorithm had superior pulmonary arterial hypertension detection capability compared with that of experienced doctors.

Background Peripheral pulmonary artery stenosis (PPS) refers to the stenosis of the pulmonary artery from the trunk to the peripheral arteries. Although pediatric PPS is well described, the clinical characteristics of adult-onset idiopathic PPS have not been established. Objectives We characterized the disease profile of adult-onset PPS. Methods We collected data in Japanese centers. This cohort included patients underwent pulmonary angiography (PAG) and excluded patients with chronic thromboembolic pulmonary hypertension or Takayasu arteritis. Patient backgrounds, right heart catheterization findings, imaging findings, and treatment profiles were collected. Results Forty-four patients (median age: 39 years [Q1–Q3:29–57]; 29 females [65.9%]) with PPS were enrolled from 20 centers. In PAG, stenosis of segmental and peripheral pulmonary arteries was observed in 41 (93.2%) and 36 patients (81.8%), respectively. Thirty-five patients (79.5%) received medications approved for pulmonary arterial hypertension (PAH-drugs) and 22 patients (50.0%) received combination therapy. Twenty-five patients (56.8%) underwent transcatheter pulmonary angioplasty. Right heart catheterization data showed improvements in both the mean pulmonary artery pressure (44versus40 mmHg; p<0.001) and pulmonary vascular resistance (760versus514 dyn·s·cm⁻⁵; p<0.001) from baseline to the final follow-up. The 3-, 5-, and 10-year survival rates of patients with PPS were 97.5% (95% confidence interval [CI]:83.5–99.6), 89.0% [95% CI:68.9–96.4), and 67.0% (95% CI:41.4–83.3), respectively. Conclusions In this study, the patients with adult-onset idiopathic PPS presented with segmental and peripheral pulmonary artery stenosis. Although patients had severe pulmonary hypertension at baseline, they showed a favorable treatment response to the PAH-drugs combined with transcatheter pulmonary angioplasty.

The percentage cross-sectional area of the lung under five (%CSA<5) is the percentage of pulmonary vessels with <5 mm2 area relative to the total lung area on computed tomography (CT). The extent that %CSA<5 is related to pulmonary hemodynamics in patients with chronic thromboembolic pulmonary hypertension (CTEPH) is unclear, as is the effect of pulmonary endarterectomy (PEA) on %CSA<5. Therefore, we aimed to evaluate the clinical significance of %CSA<5 in patients with CTEPH. We studied 98 patients (64 females, mean age 62.5 ± 11.9 years), who underwent CT with %CSA<5 measurement and right heart catheterization (RHC). Patients were classified into groups based on eligibility for PEA. We compared the %CSA<5 with pulmonary hemodynamics measured by RHC in various groups. In 38 patients who underwent PEA, the relationship between %CSA<5 and pulmonary hemodynamics was also evaluated before and after PEA. Significant correlations between %CSA<5 and pulmonary vascular resistance, and compliance, and pulmonary artery pulse pressure were observed in all patients. Pulmonary hemodynamics in the patients who underwent or were eligible for PEA showed a significant correlation with %CSA<5. Additionally, %CSA<5 was significantly lower in the postoperative than in the preoperative group. There was no correlation between changes in %CSA<5 and pulmonary hemodynamics before and after PEA. Furthermore, %CSA<5 did not correlate significantly with prognosis. %CSA<5 may reflect pulmonary hemodynamics in CTEPH with central thrombosis. Furthermore, %CSA<5 was reduced by PEA postoperatively. However, %CSA<5 is not a prognostic indicator, its clinical usefulness in CTEPH patients is limited, and further validation is required.