Hajime Kasai

Effects of Surgical and Medical Treatment on Quality of Life for Patients With Chronic Thromboembolic Pulmonary Hypertension

BACKGROUND This study aimed to investigate the predictors of quality of life (QOL) in patients with chronic thromboembolic pulmonary hypertension (CTEPH), changes in QOL after surgical and medical treatments, and the relationship between baseline QOL and survival. METHODSANDRESULTS QOL was measured in 128 patients with CTEPH (male/female: 42/86, age: 56±12 years, surgical/medical: 65/63) using the Short-Form 36 (SF-36) questionnaire. Multiple regression analysis showed pulmonary vascular resistance (PVR) and 6-min walking distance (6MWD) were associated with physical functioning (PF) (P<0.01) and physical component summary (PCS) (P<0.01). In the surgical group, 7 subscales and 2 summary scores improved significantly, and in the medical group 6 subscales and the mental component summary, although the change in QOL was greater in the surgical group. The patients in the conventional therapy group with higher PF had significantly better survival than those with lower PF (5-years survival: 89.5% vs. 50.8%, P=0.002). This difference in survival was not observed in the group receiving pulmonary arterial hypertension (PAH)-specific therapy (100% vs. 100%, P=0.746). CONCLUSIONS PVR and 6MWD were associated with PF or PCS in CTEPH patients. QOL improved after surgical or medical therapy, with a greater change in the surgical group. PAH-specific therapy improved survival in patients with lower PF at diagnosis.

Home-based pulmonary rehabilitation in patients with inoperable or residual chronic thromboembolic pulmonary hypertension: A preliminary study

BACKGROUND Management of chronic thromboembolic pulmonary hypertension (CTEPH) has recently improved because of advances in pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA), and disease-targeted medications. However, patients with inoperable CTEPH or persistent pulmonary hypertension (PH) after these interventions continue to exhibit impaired exercise capacity and limited quality of life (QOL). METHODS Eight patients with inoperable or residual CTEPH (mean age, 64±12 years; WHO functional class II/III, 6/2; mean pulmonary artery pressure, 47±13 mmHg) in stable condition and receiving disease-targeted medications participated in a 12-week home-based pulmonary rehabilitation program (muscle strength training, respiratory exercises, and walking) with supervised hospital sessions from March 2012 to January 2014. Efficacy parameters were prospectively evaluated at baseline and at completion of the 12-week program. RESULTS After completion of the pulmonary rehabilitation program, the 6-minute walking distance (6MWD) (33.3±25.1 m), St. George׳s Respiratory Questionnaire activity score, quadriceps force, and 7-day physical activity level were significantly improved compared with baseline. All subjects completed the rehabilitation program. Although one patient experienced presyncope during the in-hospital exercise sessions, no other severe adverse events or complications of pulmonary rehabilitation were observed. CONCLUSIONS These findings suggest that home-based pulmonary rehabilitation with closely supervised sessions may safely improve exercise capacity, leg muscle strength, general activity in daily life and health-related QOL in CTEPH patients.

The effects of emphysema on airway disease: Correlations between multi-detector CT and pulmonary function tests in smokers

BACKGROUND Chronic obstructive pulmonary disease (COPD) is characterized by airflow limitation caused by emphysema and small airway narrowing. Quantitative evaluation of airway dimensions by multi-detector computed tomography (MDCT) has revealed a correlation between airway dimension and airflow limitation. However, the effect of emphysema on this correlation is unclear. OBJECTIVE The goal of this study was to determine whether emphysematous changes alter the relationships between airflow limitation and airway dimensions as measured by inspiratory and expiratory MDCT. METHODS Ninety-one subjects underwent inspiratory and expiratory MDCT. Images were evaluated for mean airway luminal area (Ai), wall area percentage (WA%) from the third to the fifth generation of three bronchi (B1, B5, B8) in the right lung, and low attenuation volume percent (LAV%). Correlations between each airway index and airflow limitation were determined for each patient and compared between patients with and without evidence of emphysema. RESULTS In patients without emphysema, Ai and WA% from both the inspiratory and expiratory scans were significantly correlated with FEV1. No correlation was detected in patients with emphysema. In addition, emphysematous COPD patients with GOLD stage 1 or 2 disease had significantly lower changes in B8 Ai than non-emphysematous patients. CONCLUSIONS A significant correlation exists between airway parameters and FEV1 in patients without emphysema. Emphysema may influence airway dimensions even in patients with mild to moderate COPD.

Electrocardiogram-gated 320-slice multidetector computed tomography for the measurement of pulmonary arterial distensibility in chronic thromboembolic pulmonary hypertension.

Background We aimed to study whether pulmonary arterial distensibility (PAD) correlates with hemodynamic parameters in chronic thromboembolic pulmonary hypertension (CTEPH) using electrocardiogram (ECG)-gated 320-slice multidetector computed tomography (MDCT). Methods and Findings ECG-gated 320-slice MDCT and right heart catheterization (RHC) was performed in 53 subjects (60.6±11.4 years old; 37 females) with CTEPH. We retrospectively measured the minimum and maximum values of the cross sectional area (CSA) of the main pulmonary artery (mainPA), right pulmonary artery (rtPA), and left pulmonary artery (ltPA) during one heartbeat. PAD was calculated using the following formula: PAD = [(CSAmaximum−CSAminimum)/CSAmaximum]×100(%). The correlation between hemodynamic parameters and PAD was assessed. Mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR) were 40.8±8.7 mmHg and 8.3±3.0 wood units, respectively. PAD values were as follows: mainPA (14.0±5.0%), rtPA (12.8±5.6%), and ltPA (9.7±4.6%). Good correlations existed between mainPAD, with mPAP (r = −0.594, p<0.001) and PVR (r = −0.659, p<0.001). The correlation coefficients between rtPAD and ltPAD with pulmonary hemodynamics were all lower or equal than for mainPAD. Conclusions PAD measured using ECG-gated 320-slice MDCT correlates with pulmonary hemodynamics in subjects with CTEPH. The mainPA is suitable for PAD measurement.

Mean Pulmonary Artery Pressure Using Echocardiography in Chronic Thromboembolic Pulmonary Hypertension

BACKGROUND Mean pulmonary arterial pressure (MPAP) is an important pulmonary hemodynamic parameter used in the management of patients with chronic thromboembolic pulmonary hypertension (CTEPH). We compared echocardiography-derived estimates of MPAP with right heart catheterization (RHC) to identify reliable noninvasive methods of estimating MPAP-derived RHC (MPAPRHC) in these patients. METHODSANDRESULTS Echocardiography and RHC were performed in 56 patients with CTEPH (60.5±12.0 years; 44 females). We measured the tricuspid regurgitation (TR) pressure gradient (TRPG) using echocardiography. The mean systolic right ventricular (RV)-right atrial (RA) gradient was calculated by tracing the TR time velocity flow. Systolic and mean pulmonary artery pressures (SPAPTRand MPAPTR) estimated from TRPG and mean systolic RV-RA gradient were calculated by adding RA pressure based on the inferior vena cava. MPAPChemlawas calculated using Chemlas formula: 0.61×SPAPTR+2 mmHg. MPAPRHCand pulmonary vascular resistance were 35.9±11.3 mmHg and 6.6±3.6 Wood units, respectively. The mean difference from MPAPRHCand limits of agreement were -1.5 mmHg and -19.6 to 16.5 mmHg for MPAPTR, and -4.6 mmHg and -24.5 to 15.2 mmHg for MPAPChemla. Accuracy within 10 mmHg and 5 mmHg of MPAPRHCwas 80.4% and 46.4% for MPAPTR, and 71.4% and 48.2% for MPAPChemla, respectively. CONCLUSIONS MPAPTRand MPAPChemlaare reliable estimates for MPAPRHCin patients with CTEPH. (Circ J 2016; 80: 1259-1264).

Noninvasive assessment of pulmonary vascular resistance by echocardiography in chronic thromboembolic pulmonary hypertension

BACKGROUND Pulmonary vascular resistance (PVR) is an important parameter in the management of patients with chronic thromboembolic pulmonary hypertension (CTEPH), and numerous noninvasive methods for PVR prediction have been proposed. However, a systematic evaluation of the methods that are specific for CTEPH has not been conducted. We compared a variety of echocardiography-derived prediction indices with direct right heart catheterization (RHC) to identify the most reliable noninvasive indicator of PVR in patients with CTEPH. PATIENTS AND METHODS Echocardiography and RHC were performed sequentially in 40 patients (mean age: 62.4±11.4 years; 30 females) with CTEPH. We measured the peak flow velocity of tricuspid regurgitation (TRV), tricuspid regurgitation pressure gradient (TRPG), right ventricular outflow tract (RVOT) time-velocity integral (TVIRVOT), left ventricular outflow tract (LVOT) time-velocity integral (TVILVOT), cardiac output at RVOT (CORVOT), and the LVOT (COLVOT) using echocardiography. The parameters TRV/TVIRVOT, TRV/TVILVOT, TRV/CORVOT, TRV/COLVOT, TRPG/TVIRVOT, TRPG/TVILVOT, TRPG/CORVOT, and TRPG/COLVOT were then calculated to predict the PVR. Finally, correlations between these echocardiographic predictors of PVR and the PVR data obtained from RHC (PVRRHC) were assessed. RESULTS The mean pulmonary arterial pressure and PVRRHC were 32.1±11.4mmHg and 5.4±2.9 Wood units, respectively. TRV/TVIRVOT, TRV/TVILVOT, TRV/COLVOT, TRPG/TVIRVOT, TRPG/TVILVOT, TRPG/CORVOT, and TRPG/COLVOT were all significantly correlated with the PVRRHC, and TRPG/COLVOT was the most strongly correlated with the PVRRHC (r=0.807, p<0.001). CONCLUSIONS Echocardiographic measurement of TRPG/COLVOT is a reliable noninvasive predictor of PVR in CTEPH patients.

Effect of threshold on the correlation between airflow obstruction and low attenuation volume in smokers assessed by inspiratory and expiratory MDCT.

Background The estimation of emphysematous changes is very sensitive to computed tomography (CT) threshold level. In clinical practice, the predetermined threshold is usually set at −950 Hounsfield units (HU) for the detection of low attenuation volume (LAV). However, threshold levels that are tightly connected to pulmonary function abnormalities have not been determined. Purpose To determine the threshold level for calculating an LAV that closely reflects airflow limitation in patients with chronic obstructive pulmonary disease (COPD). Material and Methods Seventy-six consecutive non-COPD smokers and COPD patients underwent paired inspiratory and expiratory multidetector CT (MDCT). LAV% was segmented every 10 HU between −1000 and −750 HU to examine the correlation between LAV% and indexes of obstructive impairment. Results LAV% gradually increased as the threshold level increased on both inspiratory and expiratory images. LAV% on inspiratory images was higher than that on expiratory images at all threshold levels between −1000 and −750 HU. The threshold level that correlated with obstructive impairment differed between the two images: −930 HU on inspiratory and −870 or −880 HU on expiratory images. Conclusion LAV% dramatically changed according to the threshold level on both inspiratory and expiratory images, indicating that LAV% is dependent on the attenuation threshold level in patients with COPD. The threshold linking LAV% to airflow limitation was higher on expiratory than on inspiratory images.

Riociguat for patients with chronic thromboembolic pulmonary hypertension: Usefulness of transitioning from phosphodiesterase type 5 inhibitor

BACKGROUND Riociguat, the first approved drug for patients with chronic thromboembolic pulmonary hypertension (CTEPH), is a soluble guanylate cyclase (sGC) Stimulator. It directly stimulates sGC independently of nitric oxide (NO) and increases sGC sensitivity for NO. The safety and efficacy of transitioning from a phosphodiesterase 5 inhibitor (PDE5i) to riociguat is unknown. METHODS AND RESULTS Twenty-three patients were prospectively enrolled: 8 symptomatic patients with inadequate clinical responses to PDE5i were changed to riociguat (transitioned group); 15 started riociguat anew (new or add-on group). We analyzed the change from baseline to 6-12 months of riociguat treatment for the 6-minute walk distance (6MWD), mean pulmonary arterial pressure (mPAP), pulmonary vascular resistance (PVR), cardiac index (CI), partial pressure of oxygen in arterial blood (PaO2), brain natriuretic peptide (BNP), World Health Organization (WHO) functional class, safety and adverse events. The mPAP, BNP and WHO functional class significantly improved in total. In the transitioned group, BNP significantly decreased by -116.5±188.6pg/ml (P=0.0156). The 6MWD, mPAP, PVR, CI, and PaO2 improved but not significantly. The baseline condition was significantly more severe in the transitioned than in the new or add-on group. No patients discontinued riociguat. Relatively rapid transitioning from PDE5i to riociguat was safe under careful observation. CONCLUSIONS Transitioning to riociguat may be safe and effective in CTEPH patients with inadequate clinical responses to PDE5i.

The Development of Marked Collateral Circulation due to Inferior Vena Cava Filter Occlusion in a Patient with Chronic Thromboembolic Pulmonary Hypertension Complicated with Anti-phospholipid Syndrome

A 30-year-old Japanese man was diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH) with lupus anticoagulants (LAs) in 2003. He underwent pulmonary endarterectomy after the placement of an inferior vena cava filter (IVCF) in 2004, and treatment with warfarin was continued. In 2014, IVCF occlusion and marked collateral circulation were noted during an examination for transient dyspnea; however, his warfarin level was within the therapeutic range for 88.9% of the time from 2003 to 2014. We herein report a rare case of CTEPH and LAs with IVCF occlusion; in such cases, intense treatment may be required.

Lung Mycobacterium avium developed after removing an acupuncture needle from the lung

Acupuncture needles can cause non‐tuberculosis mycobacteria (NTM) infection on the skin, but there are no reports that acupuncture needles inserted into the lung have caused lung NTM infection. A 63‐year‐old woman, who underwent removal of a broken acupuncture needle inserted into the lung nine years ago, was admitted with nodules in the right lung. The shadow was positioned where the needle had existed. Partial lung resection of the right lower lobe was performed, and the resected area showed caseous necrosis histopathologically. Furthermore, Mycobacterium avium was cultured from the specimen. When abnormal lung shadows are located where a resected foreign body appeared, NTM infection should be considered.