Jin Ueda

Efficacy and safety of balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension guided by cone-beam computed tomography and electrocardiogram-gated area detector computed tomography

BACKGROUND Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease characterized by chronic obstructive thrombus and pulmonary hypertension. Balloon pulmonary angioplasty (BPA), an emerging alternative catheter-based treatment for inoperable patients with CTEPH, has not yet been standardised, especially for lesion assessment in distal pulmonary arteries. Recent advancement in computed tomography enables distal CTEPH lesions to be visualized. METHODS We retrospectively studied 80 consecutive patients with inoperable CTEPH who received BPA guided by cone-beam computed tomography (CT) (CBCT) or electrocardiogram (ECG)-gated area detector CT (ADCT) for target lesion assessment. We collected clinical and hemodynamic data, including procedural complications, before BPA and at 3 months and 1year after BPA. RESULTS Three hundred eight-five BPA sessions (4.8 sessions/patient) were performed for the lesions of subsegmental arteries (1155 lesions), segmental arteries (738 lesions), and lobar arteries (4 lesions) identified by CBCT or ECG-gated ADCT. Significant improvements in the symptoms, 6-min walk distance, brain natriuretic peptide level, exercise capacity, and haemodynamics were observed 3 months and 1year after BPA. No cases of death or cardiogenic shock with a low rate of severe wire perforation (0.3%) and severe reperfusion oedema (0.3%) were observed. CONCLUSIONS BPA guided by CBCT or ECG-gated ADCT is effective and remarkably safe in patients with CTEPH . These new advanced CT techniques may be useful in pre-BPA target lesion assessment.

Efficacy of cardiac rehabilitation after balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension

Objective To determine safety and efficacy of cardiac rehabilitation (CR) initiated immediately following balloon pulmonary angioplasty (BPA) in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH) who presented with continuing exercise intolerance and symptoms on effort even after a course of BPA; 2–8 sessions/patient. Methods Forty-one consecutive patients with inoperable CTEPH who underwent their final BPA with improved resting mean pulmonary arterial pressure of 24.7±5.5 mm Hg and who suffered remaining exercise intolerance were prospectively studied. Participants were divided into two groups just after the final BPA (6.8±2.3 days): patients with (CR group, n=17) or without (non-CR group, n=24) participation in a 12-week CR of 1-week inhospital training followed by an 11-week outpatient programme. Cardiopulmonary exercise testing, haemodynamics, and quality of life (QOL) were assessed before and after CR. Results No significant between-group differences were found for any baseline characteristics. At week 12, peak oxygen uptake (VO2), per cent predicted peak VO2 (70.7±9.4% to 78.2±12.8%, p<0.01), peak workload, and oxygen pulse significantly improved in the CR group compared with the non-CR group, with a tendency towards improvement in mental health-related QOL. Quadriceps strength and heart failure (HF) symptoms (WHO functional class, 2.2–1.8, p=0.01) significantly improved within the CR group. During the CR, no patient experienced adverse events or deterioration of right-sided HF or haemodynamics as confirmed via catheterisation. Conclusions The combination of BPA and subsequent CR is a new treatment strategy for inoperable CTEPH to improve exercise capacity to near-normal levels and HF symptoms, with a good safety profile.

Congenitally Corrected Transposition of the Great Arteries and Situs Inversus in an Octogenarian With Systemic Right Ventricular Failure

Systemic right ventricular (RV) failure in patients with congenitally corrected transposition of the great arteries (ccTGA), a major cause of mortality in the long-term follow-up, is usually induced by concomitant severe morphologically tricuspid regurgitation (TR) with/without Ebsteins anomaly or progressive conduction tissue disturbances. However, whether or not myocardial fibrosis is a common cause of systemic RV failure in patients with ccTGA remains unclear. Here, we describe an 82-year-old man who had been diagnosed previously as having uncomplicated ccTGA and situs inversus and recently developed systemic RV failure, which was neither associated with severe TR nor advanced conduction tissue abnormalities. Cardiovascular magnetic resonance (CMR) with delayed-enhancement imaging clearly detected extensive myocardial scars (presumably fibrosis) in the RV wall as well as prominent dilatation, hypertrophy, and systolic dysfunction of the systemic RV. These findings suggest that myocardial fibrosis can cause systemic RV failure in elderly patients with uncomplicated ccTGA despite the absence of severe TR or advanced conduction tissue abnormalities and that CMR may be a useful examination to accurately detect systemic RV failure associated with myocardial fibrosis and to subsequently clarify the prognosis in these patients.

Thalidomide for Hereditary Hemorrhagic Telangiectasia With Pulmonary Arterial Hypertension

initiate thalidomide, which increases platelet-derived growth factor-B expression and downregulates vascular endothelial growth factor in endothelial cells, stimulating mural cell coverage and leading to normal vascular maturation.1,2 After the initiation of thalidomide (50 mg daily), the anemia was dramatically improved without blood transfusion (Figure H) and telangiectatic lesions in the tongue were no longer notable (Figure A-2). Although intensive therapy with pulmonary vasodilators might induce bleeding, we were able to add tadalafil 10 mg daily and increase ambrisentan to 7.5 mg daily without any bleeding side-effects. Twelve months after thalidomide treatment, right heart failure developed with deteriorating pulmonary hemodynamics (mean PAP, 90 mmHg; cardiac index, 1.88 L/min/m2; Table, January 2014). Wedged distal pulmonary angiography showed markedly decreased peripheral vessels (Figure I-2) as compared with that before thalidomide therapy (Figure I-1). After the discontinuation of thalidomide, the bleeding recurred and PAH did not improve, as reflected by serial changes in B-type natriuretic peptide and tricuspid regurgitation pressure gradient (Figure G,H). Finally, the patient died due to right heart failure. Thalidomide was beneficial against mucocutaneous bleeding,1–7 but careful consideration is required with regard to its initiation in HHT patients with PAH.

Development of Pulmonary Arterial Hypertension in a Patient Treated with Qing-Dai (Chinese Herbal Medicine)

Pulmonary arterial hypertension (PAH) is a rare, devastating disease, characterized by elevated pulmonary arterial pressure due to pulmonary microvascular obstruction, which can result in heart failure and death. PAH can be associated with exposure to certain drugs or toxins. We herein report a case in which PAH developed in a patient with refractory ulcerative colitis during treatment with “Qing-Dai,” a Chinese herbal medicine. The patients PAH improved after the discontinuation of Qing-Dai.

Beneficial Effect of Endovascular Treatment on Villalta Score in Japanese Patients With Chronic Iliofemoral Venous Thrombosis and Post-Thrombotic Syndrome

BACKGROUND Post-thrombotic syndrome (PTS), the most common complication of deep venous thrombosis (DVT), develops in ≥50% of patients with iliofemoral DVT. However, the benefit of endovascular treatment in Japanese patients with chronic DVT and PTS remains unclear. Methods and Results: Between June 2014 and May 2016, endovascular treatment was performed in 11 consecutive Japanese patients with chronic iliofemoral DVT and PTS refractory to anticoagulant therapy and elastic compression stockings. We evaluated the technical success rate, complications, patency, Villalta score, calf circumference, and popliteal vein reflux in both the acute stage (the day following endovascular treatment) and chronic stage (after 6 months). Imaging follow-up included venous duplex scanning and/or magnetic resonance venography. The technical success rate was 81.8%, without complications. In patients with successful intervention, the Villalta score improved significantly, from 9.0±3.7 preoperatively to 3.6±2.5 in the acute phase (P<0.01) and 2.9±2.1 in the chronic phase (P<0.001). The bilateral difference in lower thigh circumference also improved significantly, from 2.6±1.0 cm preoperatively to 1.4±1.0 cm in the chronic phase (P<0.001). However, popliteal vein reflux did not improve. In patients with successful intervention, venous patency rate was 100% at 6 months post-intervention. CONCLUSIONS Endovascular treatment is safe and effective in Japanese patients with chronic iliofemoral DVT and PTS.

Successful catheter intervention for deep vein thrombosis due to inferior vena cava stenosis after retrieval of a temporary inferior vena cava filter

Inferior vena cava (IVC) stenosis is a well-known complication of the IVC filter. However, there are no previous reports of IVC stenosis caused by a temporary IVC filter. In this case report, we describe the case of a 35-year-old man who was referred to our center for the treatment of recurrent proximal deep vein thrombosis (DVT) and severe IVC stenosis that occurred after retrieval of a temporary IVC filter. We performed a catheter-directed thrombolysis and balloon angioplasty. DVT resolved effectively, and his leg symptoms resolved. <Learning objective: Although IVC filter-related stenosis is not common, it should be managed, even when a temporary IVC filter is used. The combination of catheter-directed thrombolysis and balloon angioplasty may be considered for a proximal deep vein thrombosis complicated with IVC stenosis.>.

Multi-step therapy with riociguat, balloon pulmonary angioplasty, and cardiac rehabilitation for inoperable chronic thromboembolic pulmonary hypertension

Background: We have recently shown that balloon pulmonary angioplasty (BPA) safely improves pulmonary haemodynamics, exercise capacity, and right ventricular (RV) function in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). However, exercise intolerance and symptoms on effort persist even after a series of BPA, despite remarkably restored resting haemodynamics, that would be hard to restore with BPA alone. Methods: We tested multi-step therapy of riociguat as a pre-treatment, a series of BPA, and cardiac rehabilitation (CR) as a post-treatment, especially on pulmonary haemodynamics, exercise capacity, and RV function evaluated with brain natriuretic peptide (BNP) and cardiovascular magnetic resonance. Results: First, a pre-BPA treatment with riociguat significantly ameliorated mean pulmonary arterial pressure (mPAP) (40.4±5.9 to 32.6±5.6 mmHg) and BNP (153±203 to 60±60 pg/mL) before the first BPA (n=18, all P Conclusions: Multi-step therapy consisting of riociguat, BPA, and CR is useful to effectively improve haemodynamics, exercise capacity, and RV function in patients with inoperable CTEPH.

BALLOON PULMONARY ANGIOPLASTY IMPROVES RIGHT VENTRICULAR THE PARAMETER OF DIASTOLIC FUNCTION IN PATIENTS WITH INOPERABLE CHRONIC THROMBOEMBOLIC PULMONARY HYPERTENSION

Right ventricular (RV) function is an important predictor in the course of chronic thromboembolic pulmonary hypertension (CTEPH). Recently, balloon pulmonary angioplasty (BPA) has been reported to improve hemodynamics and functional capacity in patients with CTEPH. However, there are no reports

EFFECT OF BALLOON PULMONARY ANGIOPLASTY FOR INOPERABLE PATIENTS WITH CHRONIC THROMBOEMBOLIC PULMONARY HYPERTENSION IN WHOM MEAN PULMONARY ARTERIAL PRESSURE WAS LESS THAN 30MMHG

It had been reported that BPA significantly improved pulmonary artery hypertension, New York Heart Association (NYHA) class and 6-minute walk distances (6MWD), in patients whose mean pulmonary arterial pressure (mPAP) >30mmHg. However, CTEPH patients with mPAP ≤30mmHg still have the symptom such