Dramatic Improvement of Pulmonary Tumor Thrombotic Microangiopathy in a Breast Cancer Patient Treated With Bevacizumab.

A 47-year-old woman diagnosed with stage IV left-sided breast cancer (T3N3aM1; OSS, HEP, LYM) 6 months back presented with respiratory distress. On admission, she developed respiratory failure, requiring 4 L of oxygen support. Pulmonary embolism was ruled out because computed tomography revealed no obvious pulmonary artery thrombus. Transthoracic echocardiography revealed a significant enlargement of the right ventricle and atrium. Pulmonary hypertension was confirmed via right heart catheterization. Pulmonary artery wedge aspiration cytology revealed adenocarcinoma cells. Based on these findings, we diagnosed the patient with pulmonary tumor thrombotic microangiopathy (PTTM) caused by breast cancer. Immediate chemotherapy (paclitaxel and bevacizumab) for breast cancer and concurrent treatment for pulmonary hypertension and disseminated intravascular coagulation were initiated. We could successfully control her condition with paclitaxel and bevacizumab for a year, and the patient survived for 1 year and 8 months. PTTM is a rare disease characterized by pulmonary hypertension and hypoxemia arising due to tumor embolization of the peripheral pulmonary arteries. PTTM is a rapidly progressing condition with no established treatment guidelines; its pathogenesis involves vascular endothelial growth factor (VEGF). This report highlighted the potential of bevacizumab, known for its anti-VEGF effect, in improving the pathological condition of patients with PTTM caused by breast cancer.

Association between sarcopenia and exercise capacity in patients with pulmonary hypertension without left heart disease.

BACKGROUND: Pulmonary hypertension (PH) has recently been described as a complex clinical syndrome affecting multiple organ systems, including the heart, lungs, and skeletal muscle, each of which plays an important role in exercise capacity. However, the relationship between exercise capacity and skeletal muscle abnormalities in patients with PH has not been fully elucidated. METHODS: We retrospectively analysed the exercise capacity and measures of skeletal muscle of 107 patients with PH without left heart disease (mean age 63 ± 15 years, 32.7% males, n = 30/6/66/5 in the clinical classification Group 1/3/4/5). RESULTS: Sarcopenia, low appendicular skeletal muscle mass index, low grip strength, and slow gait speed, determined by international criteria, were found in 15 (14.0%), 16 (15.0%), 62 (57.9%), and 41 (38.3%) patients, respectively. The mean 6-min walk distance of all patients was 436 ± 134 m and was independently associated with sarcopenia (standardised ß = -0.292, p < 0.001). All patients with sarcopenia showed reduced exercise capacity defined as 6-min walk distance < 440 m. Multivariable logistic regression analysis showed that each of the components of sarcopenia was associated with reduced exercise capacity (adjusted odds ratio and 95% confidence interval of appendicular skeletal muscle mass index: 0.39 [0.24-0.63] per 1 kg/m2, p = 0.006, grip strength: 0.83 [0.74-0.94] per 1 kg, p = 0.003, and gait speed: 0.31 [0.18-0.51] per 0.1 m/s, p < 0.001). CONCLUSIONS: Sarcopenia and its components are associated with reduced exercise capacity in patients with PH. A multifaceted evaluation may be important in the management of reduced exercise capacity in patients with PH.

Impact of grip strength and gait speed on exercise tolerance in patients with pulmonary hypertension without left heart disease.

Patients with pulmonary hypertension (PH) suffer from poor exercise tolerance due to impaired oxygenation and/or reduced cardiac output. However, the relationship between exercise tolerance and physical function remains unclear. The purpose of this study was to investigate the relationship between exercise tolerance and physical function in patients with PH. A total of 94 patients without left heart disease (61.3 ± 14.7 years old, 69.1% females, 22/8/60/4 patients with Group 1/3/4/5 PH) were retrospectively analysed. Physical function was measured using muscle strength (grip strength, knee extension muscle strength), balance function (one-leg standing time), and gait speed within 7 days of cardiac catheterization. Exercise tolerance was measured using the 6-min walking distance (6-MWD). A total of 194 6-MWD measurements and the corresponding physical function were evaluated in 94 patients. Multivariable linear regression analysis using adaptive-LASSO methods indicated that the World Health Organization functional classification, pulmonary vascular resistance, mixed venous oxygen saturation, grip strength, and gait speed were independently associated with the 6-MWD. Low grip strength (< 28 kg for males and < 18 kg for females; adjusted odds ratio and 95% confidence interval: 2.06 [1.30-3.26], p = 0.002), and slow gait speed (< 1.0 m/s for both sexes; 13.33 [3.61-49.19], p < 0.001) were independent predictors of poor exercise tolerance (6-MWD < 440 m) in a logistic regression analysis. Grip strength and gait speed as measures of physical function, pulmonary vascular resistance, and mixed venous oxygen saturation were associated with exercise tolerance in patients with PH without left heart disease.

Importance of identifying the direction of pulmonary venous flow in diagnosing a cavopulmonary window: A case report and review of literature.

A 75-year-old male suffered from dyspnea on exertion. In a referring hospital, cardiac catheterization demonstrated a 25% increase in oxygen saturation between the high superior vena cava (SVC) and the right atrium, suggesting a pre-tricuspid left-to-right shunt. However, neither an intracardiac shunt nor a partial anomalous pulmonary venous connection was detected. Therefore, he was referred to our hospital for further evaluation. A transesophageal echocardiogram revealed a retrograde-dominant bidirectional flow in the right upper pulmonary vein (RUPV). A contrast agent injected via the left upper limb appeared in the SVC and thereafter some contrast entered into the RUPV. A three-dimensional reconstructed computed tomography showed a side-to-side communication between the RUPV and the SVC. A cavopulmonary window was definitively diagnosed, in which the RUPV not only drained into the left atrium but also connected to the SVC side-to-side. .

Prognostic role of bronchial asthma in patients with heart failure.

There are few reports investigating the relationship between bronchial asthma (BA) and heart failure (HF). We hypothesized BA may have impact on prognosis in patients with HF. Among 323 consecutive outpatients with HF, 191 patients without chronic obstructive pulmonary disease were analyzed. Twenty patients had BA, most of whom (80.0%) had preserved left ventricular ejection fraction (LVEF ≥ 50%). The use of ß-blockers was less frequent (55.0% vs 83.0%. p = 0.01), systolic blood pressure (133 ± 22 vs 120 ± 17 mmHg, p = 0.003), and heart rate (83 ± 14 vs 74 ± 15 bpm, p = 0.02) were higher in patients with BA than those without BA. During median follow up of 24 months, 45 (23.6%) experienced primary outcome defined as a composite of all-cause death, nonfatal myocardial infarction, nonfatal ischemic stroke, and unexpected hospitalization due to HF. Multivariate Cox regression analysis revealed that the presence of BA was independently associated with the occurrence of primary outcome (hazard ratio 3.08, 95% CI 1.42-6.71, p = 0.004). In the subgroup analysis of patients with preserved LVEF, patients with BA exhibited worse outcomes (p = 0.03 by log-rank). Patients with HF complicated by BA, most of whom had preserved LVEF, exhibited worse outcomes than those without BA.

Is crossability of the classic crown with the glide assist superior to the micro crown in the Diamondback 360® coronary orbital atherectomy system?

The Diamondback 360® coronary orbital atherectomy system (OAS) can safely debulk calcified lesions by pullback of the crown, if the crown is advanced to the distal of the lesion. The aim of this study was to compare crossability with two types of crown in OAS. Thirty-six consecutive severely calcified lesions in 33 patients who underwent percutaneous coronary intervention with the coronary OAS were included. The micro crown was used in 18 consecutive lesions from April 2018 to February 2019, and the classic crown with the glide assist was used in 18 consecutive lesions from March 2019 to August 2019. Good crossability was defined as the ability to cross the lesion before orbital atherectomy or to cross the lesion with a first session of orbital atherectomy. We also tried to elucidate whether the crown could cross the lesion without using the glide assist in 13 consecutive lesions at the end of the classic crown cases. Good crossability was more often observed in cases with the classic crown (17 of 18 lesions, 94%) than the micro crown (6 of 18 lesions, 33%) (P < 0.001). In 13 consecutive lesions at the end of the classic crown cases, the crown could cross the lesion in 4 lesions (31%) without use of the glide assist or orbital atherectomy, and in 11 lesions (85%) with only use of the glide assist (P = 0.005). The classic crown with the glide assist is superior to the micro crown in terms of crossability for severely calcified lesions.

Time course of restenosis with "black hole" on intravascular ultrasound after implantation of platinum-chromium everolimus-eluting stent: Assessment using optical frequency-domain imaging.

Management of in-stent restenosis (ISR) remains challenging even in the drug-eluting stent era. We report the case of a Japanese female with repeated ISR after primary percutaneous coronary intervention (PCI) for acute coronary syndrome. We observed ISR tissue with "black hole" on intravascular ultrasound, which appeared to be heterogeneous tissue on optical frequency-domain imaging (OFDI). Paclitaxel-coated balloon dilatation of the ISR lesion with "black hole" was ineffective. The morphological assessment of ISR tissue using OFDI might be important to treat ISR lesions by PCI. OFDI is a novel tool to observe the difference in the in-stent tissue characteristics. .

Late gadolinium enhancement on cardiac magnetic resonance imaging is associated with coronary endothelial dysfunction in patients with dilated cardiomyopathy.

Myocardial fibrosis and coronary endothelial dysfunction are important determinants of outcome in patients with heart failure. However, the relationship of these factors in patients with dilated cardiomyopathy (DCM) is not fully understood. This study aimed to investigate the relationship between endothelium-dependent coronary vasomotor abnormality and late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (CMR) in patients with DCM. We examined 38 consecutive patients with DCM. All patients underwent CMR and the acetylcholine (ACh) provocation test using cardiac catheterization. During the ACh provocation test, we sampled blood simultaneously from the coronary sinus and aortic root to compare lactate concentrations, and quantified coronary blood flow volume using an intracoronary Doppler-tipped guidewire. LGE was detected in 17 (44.7%) patients. The lactate extraction ratio (LER) in the ACh provocation test was significantly decreased in the LGE-positive group (before vs after ACh, 18.6 ± 13.6 vs - 13.3 ± 24.8%; p < 0.001) and in the LGE-negative group (before vs after ACh, 14.2 ± 19.5 vs 3.3 ± 16.2%; p = 0.02). The rate of patients with an LER < 0% (indicating myocardial lactate production due to myocardial ischemia) was significantly higher in the LGE-positive group than in the LGE-negative group [12 (70.6%) vs 7 (33.3%); p = 0.02]. Multivariable logistic regression analysis showed that a post-ACh LER < 0% was a significant predictor of LGE positivity (odds ratio 7.75; 95% confidence interval 1.37-43.68; p = 0.02). In conclusion, ACh-provoked coronary vasomotor abnormality is associated with myocardial fibrosis in patients with DCM. These results suggest that coronary endothelial dysfunction is involved in myocardial fibrosis and worsening heart failure concomitant with DCM.

Correlation between microvascular dysfunction and B-type natriuretic peptide levels in non-ischemic heart failure patients with cardiac fibrosis.

BACKGROUNDS: The relationship between microvascular dysfunction and plasma B-type natriuretic peptide (BNP) levels remains unclear in heart failure (HF) patients with cardiac fibrosis. METHODS: This study evaluated 55 consecutive non-ischemic HF patients in an effort to determine the relationship between endothelial independent coronary microvascular dysfunction and plasma BNP levels, as well as whether each measure is correlated with myocardial fibrosis. We evaluated plasma BNP levels in patients with stable HF. We used cardiac catheterization to measure trans-cardiac BNP release levels, measuring from the coronary sinus and the aortic root, and coronary flow reserve (CFR). Patients also underwent cardiac magnetic resonance imaging to evaluate for the presence of late gadolinium enhancement (LGE), as an indicator of cardiac fibrosis. RESULTS: CFR in cardiac catheterization was significantly and inversely correlated with plasma BNP levels (r=0.336, p=0.012) and trans-cardiac BNP release levels (r=0.347, p=0.041). Thirty-three patients were LGE-positive. CFR was significantly correlated with plasma BNP levels in the LGE-positive group (r=0.349, p=0.046), but this correlation was not significant in the LGE-negative group. (r=0.338, p=0.125). Multivariate logistic regression analysis revealed that a plasma BNP levels >180pg/ml at stable HF condition was significant and independent predictor of CFR<2.5 in all patients (p=0.035, odds ratio: 5.2, 95% confidence interval: 1.1-29.0), and in the LGE-positive group (p=0.040, odds ratio: 5.4, 95% confidence interval: 1.1-27.2). CONCLUSIONS: In non-ischemic HF patients especially those with cardiac fibrosis, endothelial independent microvascular dysfunction is closely correlated with plasma BNP levels, and ventricular wall tension.

Late gadolinium enhancement on cardiac magnetic resonance predicts coronary vasomotor abnormality and myocardial lactate production in patients with chronic heart failure.

Myocardial fibrosis and microvascular dysfunction are key determinants of outcome in heart failure (HF); we examined their relationship in patients with HF. Our study included 61 consecutive patients with HF but without coronary stenosis. All underwent gadolinium-enhanced cardiac magnetic resonance to evaluate late gadolinium enhancement (LGE) and an acetylcholine (ACh) provocation test to evaluate microvascular dysfunction. During the ACh provocation test, we sampled blood simultaneously from the coronary sinus and aortic root to compare lactate concentrations. We quantified coronary blood flow volume using an intracoronary Doppler-tipped guidewire. We detected LGE in 34 patients (LGE-positive); 27 were LGE-negative. Coronary blood flow volume increased significantly after the ACh provocation test only in LGE-negative patients (before vs. after ACh, 47.5 ± 36.8 vs. 69.2 ± 48.0 ml/min, respectively; p = 0.004). The myocardial lactate extraction ratio (LER) significantly decreased after the ACh test in both groups (LGE-negative, p = 0.001; LGE-positive, p < 0.001), significantly more so in the LGE-positive group (p = 0.017). Multivariate logistic regression analysis showed that a post-ACh LER < 0 (indicating myocardial lactate production) was a significant predictor of LGE-positivity (odds ratio 4.54; 95 % confidence interval 1.38-14.93; p = 0.013). In the LGE-positive group, an LGE volume greater than the median significantly predicted a post-ACh LER of <0 (p = 0.042; odds ratio 6.02; 95 % confidence interval 1.07-33.86). ACh-provoked coronary vasomotor abnormality is closely correlated with myocardial fibrosis in patients with HF but without organic coronary stenosis. Coronary vasomotor abnormalities in fibrotic myocardium may worsen HF.