Hiroyuki Fujii

Chronic thromboembolic pulmonary hypertension (CTEPH) is rare in adolescents, and evidence regarding balloon pulmonary angioplasty (BPA) in young patients with hereditary thrombophilia remains limited. A 16year‐old male was diagnosed with CTEPH and protein S deficiency, characterized by markedly low activity and antigen levels. Genetic analysis revealed a novel PROS1 frameshift variant (c.53del, p.Val18GlyfsTer69) predicted to cause loss of function. Despite anticoagulation and riociguat therapy, pulmonary hypertension persisted, and pulmonary angiography demonstrated predominantly peripheral organized thrombotic lesions. The patient underwent staged BPA at ages 16 and 17, with an additional session at 22. All procedures were completed without complications and were associated with sustained improvement in hemodynamics and exercise capacity during long‐term follow‐up. This case highlights the importance of considering hereditary thrombophilia in adolescent patients with CTEPH and suggests that staged BPA may be a safe and effective treatment option in selected patients with predominantly peripheral disease.

Background: Qing-Dai (indigo naturalis), which is used to treat inflammatory bowel disease in Japan, has been linked to pulmonary arterial hypertension (PAH) and is recognised in the 2022 European Society of Cardiology/European Respiratory Society guidelines as a drug associated with PAH. However, the long-term clinical course of Qing-Dai-induced PAH has not been studied. Methods: This retrospective multicentre study included 20 patients diagnosed with PAH after Qing-Dai use in Japan between 2011 and 2024. Haemodynamic data were collected when PAH was diagnosed, within 1 year after Qing-Dai discontinuation and at the final follow-up evaluation. To elucidate the underlying mechanisms, we analysed the composition of Qing-Dai and examined its effects on vascular endothelial cells. Results: The study cohort comprised 11 female and nine male patients (median age: 40 years; range: 19–70 years). The median duration of Qing-Dai exposure before PAH was diagnosed was 4.8 years (range: 0.5–15 years). Discontinuation of Qing-Dai and the use of PAH medications were associated with significant improvement in haemodynamic parameters. The mean pulmonary arterial pressure decreased from 52.5 to 25.0 mmHg, and the median pulmonary vascular resistance decreased from 10.8 to 2.6 Wood units. Six (30%) patients discontinued PAH medication and remained recurrence-free. One patient experienced PAH relapse after discontinuation of PAH medications. Seven (35%) patients exhibited persistent PAH despite treatment. A mass spectrometry analysis of Qing-Dai identified indirubin, which was found to induce endothelial apoptosis. Conclusion: Discontinuation of Qing-Dai is important to PAH management. Routine follow-up is necessary to monitor potential recurrence.

The management of chronic thromboembolic pulmonary hypertension (CTEPH) has changed dramatically over the past two decades, with improved prognosis owing to the availability of pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA) and approved medications. Recently, treatment strategies combining these options have been determined by a multidisciplinary team according to localisation, characteristics, haemodynamics and comorbidities. This review summarises recent advances in hybrid therapy, combining PEA and BPA as a treatment approach for CTEPH, and discusses future perspectives.

Background: Perivascular inflammation contributes to the development of atherosclerosis and microcirculatory dysfunction. Pericoronary adipose tissue (PCAT) attenuation, measured by coronary computed tomography angiography, is a potential indicator of coronary inflammation. However, the relationship between PCAT attenuation, microcirculatory dysfunction, and periprocedural myocardial injury (PMI) remains unclear. Methods and Results: Patients with chronic coronary syndrome who underwent coronary computed tomography angiography before percutaneous coronary intervention were retrospectively identified. PCAT attenuation and adverse plaque characteristics were assessed using coronary computed tomography angiography. The extent of microcirculatory dysfunction was evaluated using the angio‐based index of microcirculatory resistance before and after percutaneous coronary intervention. Overall, 125 consecutive patients were included, with 50 experiencing PMI (PMI group) and 75 without PMI (non‐PMI group). Multivariable analysis showed that older age, higher angio‐based index of microcirculatory resistance, presence of adverse plaque characteristics, and higher lesion‐based PCAT attenuation were independently associated with PMI occurrence (odds ratio [OR], 1.07 [95% CI, 1.01–1.13]; P =0.02; OR, 1.06 [95% CI, 1.00–1.12]; P =0.04; OR, 6.62 [95% CI, 2.13–20.6]; P =0.001; and OR, 2.89 [95% CI, 1.63–5.11]; P <0.001, respectively). High PCAT attenuation was correlated with microcirculatory dysfunction before and after percutaneous coronary intervention and its exacerbation during percutaneous coronary intervention. Adding lesion‐based PCAT attenuation to the presence of adverse plaque characteristics improved the discriminatory and reclassification ability in predicting PMI. Conclusions: Adding PCAT attenuation at the culprit lesion level to coronary computed tomography angiography‐derived adverse plaque characteristics may provide incremental benefit in identifying patients at risk of PMI. Our results highlight the importance of microcirculatory dysfunction in PMI development, particularly in the presence of lesions with high PCAT attenuation. Registration: URL: https://center6.umin.ac.jp/cgi‐open‐bin/ctr/ctr_view.cgi?recptno=R000057722 ; Unique identifier: UMIN000050662.

Background: Balloon pulmonary angioplasty (BPA) is a promising treatment modality for nonoperable chronic thromboembolic pulmonary hypertension (CTEPH). However, BPA for atypical CTEPH with concurrent chronic obstructive pulmonary disease (COPD) remains controversial owing to the risk of exacerbation of ventilation‐perfusion mismatch. We aimed to evaluate the efficacy and safety of BPA for CTEPH with moderate or severe COPD. Methods and Results: Data from 149 patients with CTEPH, who underwent BPA from March 2011 to June 2021, were retrospectively analyzed. Patients were divided based on COPD comorbidity: the COPD group (n=32, defined as forced expiratory volume in 1 second/forced vital capacity P =nonsignificant. Respiratory function and oxygenation improved in the COPD group (forced expiratory volume in 1 second/forced vital capacity [61.8±7.0% to 66.5±10.2%, P =0.02] and arterial oxygen partial pressure [60.9±10.6 mm Hg to 69.3±13.6 mm Hg, P P =0.024) and higher diffusing capacity for lung carbon monoxide ( P =0.028) at baseline were associated with greater improvement in oxygenation in the multivariable linear analysis. Lung injury per BPA session was 1.6% in the COPD group. Conclusions: The efficacy and safety of BPA for nonoperable CTEPH in patients with comorbid COPD were similar to those in patients without COPD. Oxygenation and forced expiratory volume in 1 second/forced vital capacity improved in patients with COPD. BPA should be considered in patients with CTEPH with concurrent COPD.

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