暂无摘要。

BACKGROUND: Knowledge of the anatomy of pulmonary arteries is essential in many invasive procedures concerning pulmonary circulation. In the diagnosis and treatment of chronic thromboembolic pulmonary hypertension (CTEPH), two-dimensional (2D) pulmonary angiography is used. Recognizing the topographic course of the pulmonary arteries and understanding the status in three dimensions (3D) is paramount. This study aimed to evaluate and describe the branching variant of pulmonary arteries in a single case, as well as morphological parameters of the segmental arteries, like length, diameter and branching angles.
METHODS: One pair of embalmed human cadaver lungs was dissected by a scalpel and surgical forceps and was measured up to the subsegmental arteries.
RESULTS: The diameters (ranging from 3.04 to 9.29 mm) and lengths (ranging from 9.09 to 53.91 mm) of the pulmonary segmental arteries varied. The proximal branching angles were wide and close to perpendicular, while distally, the angles between the segmental and subsegmental arteries were narrower (30-45°). Upon evaluating the branching, rare variations were identified and delineated, notably in the lower lobes of both lungs.
CONCLUSIONS: Utilizing knowledge and data in clinical settings is instrumental for effectively diagnosing and treating CTEPH. Further research is required to explore the complications in invasive procedures related to various anatomical variations.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease resulting from impaired patency of the pulmonary arteries by a clot, and the treatment method of choice is pulmonary endarterectomy (PEA). In inoperable patients, balloon pulmonary angioplasty (BPA) is recommended, but we need to implement pharmacological bridge therapy to BPA in some cases. We report a case of a 38-year-old male diagnosed with CTEPH, disqualified from PEA due to comorbidity, who developed right ventricular (RV) failure. The case shows a complex pharmacological treatment method that can be successfully used as an effective bridge therapy to BPA in patients with CTEPH and severe RV dysfunction, disqualified from surgery.

Hughes-Stovin syndrome (HSS) is a rare vasculitis of unknown etiology. The disease is characterized by pronounced inflammation and damage to the vessel walls, with subsequent widespread vascular thrombosis and the formation of pulmonary artery aneurysms that can lead to fatal hemoptysis. This disorder can be mistaken for other conditions, such as chronic thromboembolic pulmonary disease (CTEPD) without or with pulmonary hypertension at rest (CTEPH).We report the case of a 20-year-old female with HSS, which was misdiagnosed as CTEPH and subsequently treated with anticoagulants, which led to severe hemoptysis and eventually death of the patient. This case highlights the challenges of diagnosing HSS at early stages of the disease.HSS should be considered in young patients with signs of large vessel vasculitis in combination with thrombotic occlusions of pulmonary arteries, with or without aneurysms of the pulmonary arteries, and particularly, if there are no risk factors for thromboembolic disease.

We encountered a case of frequent nonsustained polymorphic ventricular tachycardia (NSPVT) due to hemodynamically unstable chronic thromboembolic pulmonary hypertension (CTEPH). A 78-year-old woman was taking anticoagulants for CTEPH. She had refused specific treatment for CTEPH, including pulmonary vasodilators, because she was then asymptomatic. She fell and sustained a femoral neck fracture, and she was referred to our hospital in anticipation of a surgical repair. Her condition on admission was complicated by respiratory failure, and electrocardiogram monitoring showed frequent NSPVT. A right heart catheterization revealed high mean pulmonary artery pressure with severely reduced cardiac output. Pulmonary angiography showed bilateral stenosis and multiple obstructions. Because NSPVT was attributed to low cardiac output syndrome caused by CTEPH, rescue balloon pulmonary angioplasty (BPA) was performed, and riociguat treatment was initiated. Afterward, the NSPVT resolved. This case suggests that the combination of rescue BPA with riociguat therapy might be an immediate and effective treatment for patients with inoperable CTEPH and severe hemodynamic instability.

Chronic thromboembolic pulmonary hypertension is a rare but life-threatening complication of long-term central venous catheters (CVC) in children. However, evidence in terms of potential treatment strategies and outcome data remains scarce. We describe two cases of CVC-related thrombosis (Hickman-catheter) complicated by recurrent pulmonary emboli. One patient experienced a complete thromboembolic obstruction of the right pulmonary artery with normal pulmonary pressures and the second patient suffered from a central thromboembolic obstruction of both pulmonary arteries associated with severe pulmonary hypertension. Both patients successfully underwent surgical thromboendarterectomy with deep hypothermic circulatory arrest.

We present unusual coronary-pulmonary collaterals in a 65-year-old CTEPH patient. Perfusion mapping of a dual-energy computed tomography (DECT) study revealed areas of right lung that were minimally perfused despite unilateral occlusion of the right pulmonary artery, leading to the discovery of coronary-pulmonary collaterals via invasive coronary angiography. Pulmonary thromboendarterectomy removed the clot en-bloc. Post-surgery DECT and catheterization confirmed restoration of pulmonary arterial circulation and excellent hemodynamic response. Here, suggestion of perfusion to a proximally obstructed lung with DECT helped to document the presence of rarely documented coronary-pulmonary artery collaterals.

UNASSIGNED: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare thromboembolic disease, with occasional critical consequences. Essential thrombocythaemia (ET) is associated with an increased incidence of venous and arterial thrombotic events. In addition, the JAK2-V617F mutation increases the risk of thrombosis. Few reports have evaluated the utility of balloon pulmonary angioplasty (BPA) for worsening CTEPH with ET and the JAK2-V617F mutation.
UNASSIGNED: A 76-year-old woman, diagnosed with ET and the JAK2-V617F mutation, presented with dyspnoea. Echocardiography showed severe tricuspid regurgitation with a flattened interventricular septum. Contrast-enhanced computed tomography showed an eccentric thrombus in the right main pulmonary artery (PA) and thrombi in bilateral peripheral PAs. Acute pulmonary embolism (PE) was initially diagnosed, and heparinization was initiated; however, her oxygen saturation gradually worsened despite continued anticoagulation therapy. Her oxygen saturation level decreased to 90% (under a reservoir mask of 10 L). Her haemodynamics suggested CTEPH comorbidity. We decided to perform emergency right heart catheterization (RHC) and pulmonary angiography (PAG). RHC showed severe pulmonary hypertension. PAG showed fresh and organized thrombi and web regions in several segmental PAs. These findings indicated a combination of acute PE and CTEPH. Rescue BPA was performed on the right A1, A3, A8, and A9 segments. After BPA, the patient\'s oxygen saturation showed marked improvement. The patient was discharged 18 days after hospitalization without complications.
UNASSIGNED: Rescue BPA could be an effective treatment for worsening CTEPH in severely impaired conditions, even with ET and the JAK2-V617F mutation.

A 77-year-old man presented with dyspnoea and was diagnosed with chronic thromboembolic pulmonary hypertension. Balloon pulmonary angioplasty was attempted; however, the balloons could not be advanced to the total occlusion lesion in the right A3 segment. The obstruction was overcome using a microcatheter Corsair (AsahiKASEI). This technique may be useful in managing a total occlusion lesion.

Pulmonary thromboendarterectomy is a potentially curative option for most patients with chronic thromboembolic pulmonary hypertension (CTEPH). However, a special group of patients with CTEPH requires simultaneous cardiac procedures. We report a rare case of successful surgical treatment of a CTEPH patient with a left anterior descending artery myocardial bridge. Despite the complexity of performing pulmonary thromboendarterectomy (PTE), the issue concerning the method of revascularization of the artery in the case of the left anterior descending artery myocardial bridge is controversial. PTE and supracoronary myotomy were performed. In our case, the optimal surgery method for the left anterior descending artery myocardial bridge was chosen intraoperatively based on the depth and length of the myocardial bridge. The patient\'s significant functional improvement after surgery and hemodynamic normalization were confirmed at the follow-up assessment. This case demonstrates rare but potentially dangerous pathologies that can be treated with minimal adverse effects.