Personalised stroke evaluation and management: tailoring individualised patient care for hereditary haemorrhagic telangiectasia.

SummaryHereditary haemorrhagic telangiectasia (HHT) has an estimated prevalence of 1 in 5000-8000 individuals globally with pulmonary arteriovenous malformations (PAVMs) affecting approximately 15%-50% of HHT patients. Ischaemic stroke is a known complication of PAVMs that affects ≤30% of patients with PAVMs. Studies have shown that patients with PAVMs have ischaemic stroke a decade earlier than routine stroke. The predominant mechanism of ischaemic stroke in HHT patients is paradoxical embolism due to PAVMs, but most HHT-related PAVMs are asymptomatic. Additionally, HHT is often underdiagnosed in patients and poses a challenge to physicians due to its rarity. We present a case of a patient with ischaemic stroke who was subsequently diagnosed with HHT and found to have a PAVM on further evaluation. This case highlights the importance of using an individualised patient-centred stroke evaluation and screening for PAVMs in patients who had a stroke with possible or suspected HHT and definite HHT.

A pulmonary artery was embolized in a patient with an occluded pulmonary vein to manage massive hemoptysis.

BACKGROUND: Stenosis and obliteration of the pulmonary vein can be developed by multiple diseases and might cause hemoptysis. Traditional therapy including surgical procedure and conservative treatments might be inappropriate choices to manage massive hemoptysis. CASE PRESENTATION: A 64-year-old man, diagnosed with advanced stage IVA lung squamous cell carcinoma, presented with dyspnea and recurrent, massive hemoptysis. An initial contrast-enhanced computed tomography revealed a giant tumor in the left lung hilus and occlusion of the left superior pulmonary vein. Despite immediate selective bronchial artery embolization and simultaneous embolization of an anomalous branch of the internal thoracic artery, the massive hemoptysis continued. Subsequently, embolization of the left superior pulmonary artery was performed, achieving functional pulmonary lobectomy, which successfully treated the hemoptysis without relapse during a six-month follow-up. The patient continues to undergo cancer therapy and remains stable. CONCLUSIONS: This case successfully managed massive hemoptysis associated with lung cancer invasion into the pulmonary vein through functional pulmonary lobectomy via embolization of the corresponding pulmonary artery.

[Resected Right Upper Lobe Lung Cancer with Displaced Bronchus and Anomalous Pulmonary Vein].

The patient is a 77-year-old man. He was referred to our hospital after a chest computed tomography (CT) scan revealed a 6.5 cm-sized mass in the right lung apex. Bronchoscopy revealed adenocarcinoma, clinical stageⅡB, and the patient was referred for surgery. Preoperative 3D-CT revealed the presence of a displaced bronchus, probably B1a, branching from the right main bronchus centrally from the upper lobe bronchus, and an abnormal vessel (V2) running dorsal to the upper lobe bronchus and the right main bronchus, and returning directly to the left atrium. Surgery was performed by resectioning the right upper lobe through a posterolateral incision, combined resection of the wall pleura, and lymph node dissection (ND2a-2). Because lung cancer surgery is sometimes accompanied by abnormal bronchial and pulmonary vascular branches, it is essential to thoroughly examine the patient before surgery for checking abnormal branches by bronchoscopy and 3D-CT.

Rare case of total anomalous pulmonary venous return into the right atrium in situs solitus.

We report an uncommon case report of total anomalous pulmonary venous returns into the right atrium at the base of the superior caval vein's ostium without a sinus venosus defect, in situs solitus, without vertical vein or a posterior pulmonary venous confluence.

Right S3 segmentectomy for lung cancer with partial anomalous pulmonary venous return in the right upper pulmonary vein: A case report.

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital malformation where the pulmonary vein partially refluxes into the venous system. Here, we present the first robotic-assisted right S3 segmentectomy in a 70-year-old male with early-stage lung cancer and PAPVR in the right upper pulmonary vein. The patient, with suspected primary lung cancer (11 mm diameter, pure solid appearance in right S3 segment), exhibited clinical stage T1bN0M0 stage IA2. Preoperative computed tomography revealed severe lung emphysema, and right V1-3 returned directly to the superior vena cava. However, no signs of right-sided heart failure were observed, and echocardiogram was normal with a pulmonary-to-systemic blood flow ratio of 1.4. Successful robot-assisted right S3 segmentectomy with hilar nodal dissection was performed, and the patient was discharged on the sixth postoperative day without complications. One year postoperatively, there has been no recurrence of lung cancer or respiratory/right-sided heart failure symptoms.

The chief culprit of intractable hypoxemia: a case report of rare pulmonary arteriovenous fistula complicated with giant hemangioma.

BACKGROUND: Pulmonary arteriovenous fistula (PAVF) is a rare disease, which can lead to the direct return of unoxidized venous blood to pulmonary veins and left heart, resulting in right-to-left shunt leading to hypoxia. Long term, the right-to-left shunt will cause severe pathophysiological changes in the patient's body and pulmonary circulation, and the prognosis will be poor if PAVF is not treated timely. CASE PRESENTATION: Here, we report the case of a 71-year-old man who presented with chest tightness and shortness of breath. After a series of examinations, PAVF and giant hemangioma were diagnosed, which are difficult to operate.Transcatheter interventional therapy was initiated. The patient recovered on the third day after operation and was discharged smoothly. During the long-term follow-up of nearly 4 years after discharge, the general condition and quality of life of the patient basically returned to normal. CONCLUSIONS: PAVF is rare but very important clinical problem. When the clinical manifestations of persistent unexplained hypoxia appear, it is necessary to fully consider the possibility of PAVF. Once the diagnosis of PAVF is clear, timely treatment is recommended to avoid deterioration of the disease and affecting the prognosis.

Use of Polyurethane-Covered Stents for Exclusion of Pulmonary Arteriovenous Malformations.

PURPOSE: To evaluate the safety, technical success and short-term effectiveness of polyurethane-covered stent (PK Papyrus, BioTronik, Berlin) in the treatment of pulmonary arteriovenous malformations (PAVMs) that are not amenable to embolotherapy. MATERIALS AND METHODS: In this IRB-approved, retrospective study, data from patients who received polyurethane-covered stents for exclusion of PAVMs were analyzed. The study included 5 patients (all women) with a median age of 40 years (range 25-60). Patients presented with hypoxemia, TIAs, and/or epistaxis; 4 were confirmed to have HHT. All had multiple PAVMs diagnosed on chest CT and underwent embolization with other devices in addition to the polyurethane-covered stent. The indication for stent placement in all cases was a short and/or tortuous feeding artery. Safety was assessed by immediate or short-term complications, e.g., migration, stent thrombosis, and fracture. Technical success was defined as the ability to accurately place the stent at the intended location. Effectiveness was defined as successful exclusion of PAVM with no perfusion across the AVM. RESULTS: Technical success of stent placement was 100%. AVM exclusion rate was 80% after single stent deployment; in the case of incomplete exclusion, success was achieved using an overlapping stent to completely cover a second feeding artery. During the median follow-up period of 5 months (range 2-10), all stents remained patent, and AVMs were excluded without other complications. CONCLUSION: Exclusion of PAVMs with polyurethane-covered stents is technically feasible, safe, and shows short-term effectiveness for PAVMs with a short/tortuous feeding artery when traditional embolization techniques are not possible.

A rare combination: a single common pulmonary vein with polysplenia syndrome.

A single common pulmonary vein is a rare anomaly, mostly occurring with asplenia. We wanted to report our case because it co-exists with polysplenia and ciliary dyskinesia.A baby girl about 2 months old with respiratory arrest was admitted to the emergency room of our hospital. She responded to pulmonary resuscitation. We ascertained from her medical history that she had been hospitalised twice because of recurrent lung infections. After the initial check and treatment, we applied imaging modalities to detect underlying problems of recurrent lung infection. Echocardiographic evaluation was suboptimal because of the widespread pulmonary infiltration. Still, we noticed a single common pulmonary vein without obstruction at the junction of the left atrium. Subsequently, a chest CT scan with contrast was performed. It revealed a single common pulmonary vein entering the left atrium with a single orifice, atrial septal defect, two lobes in both lungs, hepatic veins draining directly into the right atrium, and polysplenia. When the flow of a single common pulmonary vein goes directly to the right atrium through the defect, it may cause right heart dilatation as it did in our case. Therefore, patients may need to undergo surgery at an earlier age.

Transarterial Embolization of Simple Pulmonary Arteriovenous Malformations: Long-Term Outcomes of 0.018-Inch Coils versus Vascular Plugs.

PURPOSE: To compare the safety, effectiveness, and persistence rates of 0.018-inch coils with those of Amplatzer vascular plugs (AVPs; Abbott Vascular, Abbott Park, Illinois) for the treatment of pulmonary arteriovenous malformations (PAVMs) in response to a growing concern that 0.018-inch coil embolization would increase the long-term persistence rate. MATERIALS AND METHODS: This is a retrospective, single-center study of a database (2002-2020) of 633 PAVM embolizations. Complex PAVMs and those not embolized with 0.018-inch coils or plugs were excluded. PAVM embolization material was classified into 4 groups: (a) 0.018-inch nonfibered coils (NFCs), (b) 0.018-inch fibered coils (FCs), (c) NFCs and FCs, or (d) plugs. Persistence was defined as flow through the PAVM on digital subtraction angiography (DSA) or as <30% diameter reduction of the aneurysmal sac on unenhanced computed tomography (CT). Kaplan-Meier analysis and Cox regression were used to assess PAVM's persistence-free survival. RESULTS: A total of 312 PAVM embolizations with NFCs (43 PAVMs), FCs (127 PAVMs), NFCs and FCs (12 PAVMs), or plugs (130 PAVMs) in 109 patients (28% men; mean age = 49 years) were included. All PAVM embolizations were technically successful without any major adverse events. PAVM persistence-free survival rates at 10 years' follow-up were 40.8% versus 44.7% in the NFC and FC groups (P = .22) and 47.3% versus 81.0% in the 0.018-inch coil (NFC or FC) and plug groups (P < .0001), respectively. There were 0.43 (79/182) and 0.08 (10/130) re-embolization procedures per PAVM in the 0.018-inch coil and plug groups, respectively (P < .001). CONCLUSIONS: PAVM embolization with 0.018-inch coils was safe, but persistence rate with PAVM embolization was significantly higher than that with plugs, with no significant differences between FCs and NFCs.

Preliminary Experience with a Low-Profile High-Density Braid Occluder for Transcatheter Embolization of Pulmonary Arteriovenous Malformations.

This brief report describes safety, technical feasibility, and early treatment effectiveness of the low-profile braided occluder (LOBO; Okami Medical, San Diego, Caliornia) for embolization of 9 pulmonary arteriovenous malformations (PAVMs) in 4 patients (3 female and 1 male; age range: 33 to 63 years; 3 patients showed positive results for hereditary hemorrhagic telangiectasia genes). A total of 10 occluders were deployed in 10 vessels (median treated vessel diameters, 3 and 4 mm for LOBO-3 and LOBO-5 groups, respectively). All devices were successfully deployed into the feeder pulmonary arteries, achieving complete cessation of flow. There were no severe adverse events or device migrations. Available short-term follow-up computed tomography (6 PAVMs: median, 7 months; range, 1.5-7 months) demonstrated complete occlusion without persistence or recanalization. The early experience of embolization of PAVMs using a low-profile braided occluder showed it to be safe and effective. Further studies with larger cohorts and longer follow-up periods are warranted.

STIC-HD live flow technology in the antenatal diagnosis of scimitar syndrome: A case report.

Scimitar syndrome (SS) is a rare entity with an incidence of approximately 1-3 in 200 000 people. It is typically characterized by complete or partial anomalous pulmonary venous drainage from the right lung into the systemic venous circulation, most commonly the inferior vena cava (IVC). For the first time, we report the diagnosis of SS in a fetus in utero using four-dimensional (4D) spatiotemporal image correlation combined with high-definition live flow rendering mode (STIC-HD live flow).

Feasibility of Breath-Hold Zero TE Magnetic Resonance Imaging Sequence for Evaluation of Pulmonary Arteriovenous Malformations After Embolotherapy.

ABSTRACT: We obtained breath-hold zero TE (ZTE) magnetic resonance imaging for the evaluation of pulmonary arteriovenous malformations before and after embolotherapy. To the best of our knowledge, there have been no reports of ZTE for the entire lung imaging in single breath-hold scan time such as 20 seconds. Breath-hold ZTE magnetic resonance imaging can be a useful technique for magnetic resonance-based follow-up of vascular lung diseases without using contrast media, reducing the undesired artifacts from metallic devices.

Prospective Study of Polytetrafluoroethylene-Covered Microplugs and Detachable Coils for Embolization of Pulmonary Arteriovenous Malformations: Technical Results, Procedure Times, and Costs.

PURPOSE: To determine time to occlusion and procedure costs of embolization of pulmonary arteriovenous malformations (PAVMs) using a polytetrafluoroethylene-covered microplug compared with embolization using detachable coils. MATERIALS AND METHODS: In this prospective study, 37 patients (mean age, 39.1 years [SD ± 17.6]) with 82 PAVMs underwent embolization with microplug or detachable coils between April 2019 and January 2023. Technical success, procedure time intervals, and costs were analyzed. RESULTS: In 37 patients, 82 PAVMs and 101 feeding arteries were successfully treated (microplug, 64; microplug + another device, 5; detachable coils alone, 32). Time from embolic device inserted into the catheter to device deployed and time to occlusion differed significantly between microplug and detachable coil cohorts (P < .0001 for both). Embolization with ≥1 microplug had a significantly shorter occlusion time than embolization with detachable coils (median, 10.0 minutes saved per feeding artery) (P < .0001). Compared with detachable coil embolization, microplug embolization saved a median of 9.0 minutes per feeding artery (P < .0001) and reduced room cost by a median of $429 per feeding artery (P < .0001). Device costs per feeding artery did not differ significantly between microplug ($2,790) and detachable coil embolization ($3,147) (P = .87). CONCLUSIONS: Compared with coils, microplugs had an equally high technical success rate but significant time to occlusion and room costs savings per feeding artery. Total room cost and device cost together did not differ significantly between microplugs and coils. Microplugs may be considered technically effective and at least cost-neutral for PAVM embolization where clinically appropriate.

Transcatheter correction of sinus venosus defect in a patient with a challenging anatomical configuration: From bench testing to clinical success.

We report successful transcatheter correction of a sinus venosus defect in a 72-year-old woman with anomalous pulmonary venous return in a challenging anatomical configuration. The procedure was facilitated by hands-on simulation training on a newly developed, perfused, 3D-printed model.