Prediction of cardiac allograft vasculopathy using splenic switch-off on myocardial PET.

BACKGROUND: Heart transplantation (HTx) is a definitive therapy for refractory heart failure. Cardiac allograft vasculopathy (CAV), characterized by diffuse arteriopathy involving the epicardial coronary arteries and microvasculature, is the major cause of death for patients with HTx. 13 N-ammonia positron emission tomography (NH3-PET) can offer diagnostic and prognostic utility for CAV. The splenic switch-off (SSO) detected in NH3-PET is a hemodynamic indicator of favorable response to adenosine. We hypothesized that both CAV and SSO reflected a pathology that progresses in parallel with systemic vascular endothelial dysfunction. Therefore, we quantitatively evaluated splenic adenosine reactivity measured using NH3-PET as an index of endothelial function, and examined its predictability for CAV. METHODS: Forty-eight patients who underwent NH3-PET after HTx were analyzed. The spleen ratio was calculated as the mean standardized uptake value, measured by placing an ROI on the spleen, at stress divided by that at rest. SSO was defined by a cutoff determined using receiver operating characteristic (ROC) analysis for the spleen ratio. The endpoint was appearance or progression of CAV. Predictability of SSO was analyzed using Kaplan-Meier analysis. RESULTS: The endpoint occurred in 9 patients during a mean follow-up of 45 ±â€¯17 months. ROC curve analysis demonstrated a cutoff of 0.94 for spleen ratio. Patients without SSO displayed a significantly higher CAV rate than those with SSO (p = 0.022). CONCLUSIONS: SSO reflects the endothelial function of systemic blood vessels and was a predictor of CAV in patients with HTx.

Caregivers of patients with long-term implantable ventricular assist device: a republication of the article published in the Japanese Journal of Artificial Organs.

Implantable ventricular assist device (VAD) has enabled patients with severe heart failure to be discharged from the hospital and to continue their care at home. The presence of caregivers is essential to maintain home care of patients with VAD. The caregivers are mainly the family members who live with the patient with VAD. The caregivers need to be trained (1) VAD device management, (2) disinfection of driveline skin punctures, (3) medication management, and (4) responding to sudden changes. The caregivers' lifestyle is also forced to change. In this article, we discuss the role of caregivers in the VAD era, where long-term support beyond 5 years is now possible. This review was created based on a translation of the Japanese review written in the Japanese Journal of Artificial Organs in 2023 (Vol. 52, No. 1, pp. 81-84), with some modifications.

Association between interstitial lung abnormality and mortality in patients with esophageal cancer.

PURPOSE: To investigate the relationship between interstitial lung abnormalities (ILAs) and mortality in patients with esophageal cancer and the cause of mortality. MATERIALS AND METHODS: This retrospective study investigated patients with esophageal cancer from January 2011 to December 2015. ILAs were visually scored on baseline CT using a 3-point scale (0 = non-ILA, 1 = indeterminate for ILA, and 2 = ILA). ILAs were classified into subcategories of non-subpleural, subpleural non-fibrotic, and subpleural fibrotic. Five-year overall survival (OS) was compared between patients with and without ILAs using the multivariable Cox proportional hazards model. Subgroup analyses were performed based on cancer stage and ILA subcategories. The prevalences of treatment complications and death due to esophageal cancer and pneumonia/respiratory failure were analyzed using Fisher's exact test. RESULTS: A total of 478 patients with esophageal cancer (age, 66.8 years ± 8.6 [standard deviation]; 64 women) were evaluated in this study. Among them, 267 patients showed no ILAs, 125 patients were indeterminate for ILAs, and 86 patients showed ILAs. ILAs were a significant factor for shorter OS (hazard ratio [HR] = 1.68, 95% confidence interval [CI] 1.10-2.55, P = 0.016) in the multivariable Cox proportional hazards model adjusting for age, sex, smoking history, clinical stage, and histology. On subgroup analysis using patients with clinical stage IVB, the presence of ILAs was a significant factor (HR = 3.78, 95% CI 1.67-8.54, P = 0.001). Subpleural fibrotic ILAs were significantly associated with shorter OS (HR = 2.22, 95% CI 1.25-3.93, P = 0.006). There was no significant difference in treatment complications. Patients with ILAs showed a higher prevalence of death due to pneumonia/respiratory failure than those without ILAs (non-ILA, 2/95 [2%]; ILA, 5/39 [13%]; P = 0.022). The prevalence of death due to esophageal cancer was similar in patients with and without ILA (non-ILA, 82/95 [86%]; ILA 32/39 [82%]; P = 0.596). CONCLUSION: ILAs were significantly associated with shorter survival in patients with esophageal cancer.

Prediction of solid and micropapillary components in lung invasive adenocarcinoma: radiomics analysis from high-spatial-resolution CT data with 1024 matrix.

PURPOSE: To predict solid and micropapillary components in lung invasive adenocarcinoma using radiomic analyses based on high-spatial-resolution CT (HSR-CT). MATERIALS AND METHODS: For this retrospective study, 64 patients with lung invasive adenocarcinoma were enrolled. All patients were scanned by HSR-CT with 1024 matrix. A pathologist evaluated subtypes (lepidic, acinar, solid, micropapillary, or others). Total 61 radiomic features in the CT images were calculated using our modified texture analysis software, then filtered and minimized by least absolute shrinkage and selection operator (LASSO) regression to select optimal radiomic features for predicting solid and micropapillary components in lung invasive adenocarcinoma. Final data were obtained by repeating tenfold cross-validation 10 times. Two independent radiologists visually predicted solid or micropapillary components on each image of the 64 nodules with and without using the radiomics results. The quantitative values were analyzed with logistic regression models. The receiver operating characteristic curves were generated to predict of solid and micropapillary components. P values < 0.05 were considered significant. RESULTS: Two features (Coefficient Variation and Entropy) were independent indicators associated with solid and micropapillary components (odds ratio, 30.5 and 11.4; 95% confidence interval, 5.1-180.5 and 1.9-66.6; and P = 0.0002 and 0.0071, respectively). The area under the curve for predicting solid and micropapillary components was 0.902 (95% confidence interval, 0.802 to 0.962). The radiomics results significantly improved the accuracy and specificity of the prediction of the two radiologists. CONCLUSION: Two texture features (Coefficient Variation and Entropy) were significant indicators to predict solid and micropapillary components in lung invasive adenocarcinoma.

Increased Left Ventricular End-Diastolic Volume Index Is Associated With Increased Adverse Events After MitraClip Implantation.

This study investigated the association between the left ventricular end-diastolic volume index (LVEDVI) and the incidence of adverse clinical events in patients after MitraClip implantation. In this retrospective, observational study, 123 patients who underwent the MitraClip procedure were enrolled. Participants were divided into 2 groups according to the LVEDVI cut-off level, calculated using receiver operating characteristic curve analysis, to predict the primary end point and the occurrence of cardiovascular events was compared between the groups. The primary end point was all-cause mortality and hospitalization because of heart failure. The receiver operating characteristic curve analysis for the composite primary end point revealed an LVEDVI cut-off point of 118 ml/m2. Based on this threshold, 61 patients (49.6%) were categorized into the LVEDVI <118 ml/m2 group, whereas 62 (50.4%) fell into the LVEDVI ≥118 ml/m2 group. Over a median follow-up period of 336 days (interquartile range 80 to 667), the primary end points occurred in 15 and 26 patients in the LVEDVI <118 and LVEDVI ≥118 ml/m2 groups, corresponding to incidence rates of 24.6% and 41.9%, respectively. Patients in the LVEDVI ≥118 ml/m2 group demonstrated a significantly higher risk of adverse clinical events than those in the LVEDVI <118 ml/m2 group (hazard ratio 2.24, 95% confidence interval 1.17 to 4.28, p = 0.01). This trend persisted even after adjusting for several confounders (p = 0.02). In conclusion, increased LVEDVI values were associated with increased adverse clinical events after MitraClip implantation in patients with severe mitral valve regurgitation.

Direct Link Between Cardiac Failure and Global Cerebral Atrophy in a Young Adult: A Case Report on Reduced Cerebral Artery Blood Flow.

BACKGROUND Heart failure is associated with structural brain abnormalities, including atrophy of multiple brain regions. Previous studies have reported brain atrophy in middle-aged patients with systolic heart failure. In this report, we present the case of a 21-year-old woman with idiopathic dilated cardiomyopathy, cardiac failure, and global cerebral atrophy due to reduced cerebral artery blood flow. We also discuss the impact of brain atrophy in this young adult patient with severe heart failure and no risk factors for atherosclerosis. CASE REPORT A 21-year-old woman with dyspnea and leg edema was admitted to our hospital. After several examinations, an endomyocardial biopsy led to a diagnosis of idiopathic dilated cardiomyopathy, and transthoracic ultrasound cardiography revealed that her left ventricular ejection fraction was 36%. One year after the first hospitalization, her heart failure was classified as New York Heart Association Class III. Magnetic resonance imaging showed severe global brain atrophy, and single-photon emission computed tomography combined with brain computed tomography showed reduced blood flow to the entire brain. She had no risk factors for atherosclerosis and no atherosclerotic changes to her brain or carotid arteries, but her neuropsychological and neurological findings indicated more pronounced brain and cognitive dysfunction. CONCLUSIONS This young adult patient with idiopathic dilated cardiomyopathy, cardiac failure, and global cerebral atrophy showed reduced cerebral artery blood flow and cognitive impairment. The findings of this report indicate that low cardiac output may directly cause brain atrophy in patients with systolic heart failure.

Usefulness of Three-Dimensional Iodine Mapping Quantified by Dual-Energy CT for Differentiating Thymic Epithelial Tumors.

Background: Dual-energy CT has been reported to be useful for differentiating thymic epithelial tumors. The purpose is to evaluate thymic epithelial tumors by using three-dimensional (3D) iodine density histogram texture analysis on dual-energy CT and to investigate the association of extracellular volume fraction (ECV) with the fibrosis of thymic carcinoma. Methods: 42 patients with low-risk thymoma (n = 20), high-risk thymoma (n = 16), and thymic carcinoma (n = 6) were scanned by dual-energy CT. 3D iodine density histogram texture analysis was performed for each nodule on iodine density mapping: Seven texture features (max, min, median, average, standard deviation [SD], skewness, and kurtosis) were obtained. The iodine effect (average on DECT180s-average on unenhanced DECT) and ECV on DECT180s were measured. Tissue fibrosis was subjectively rated by one pathologist on a three-point grade. These quantitative data obtained by examining associations with thymic carcinoma and high-risk thymoma were analyzed with univariate and multivariate logistic regression models (LRMs). The area under the curve (AUC) was calculated by the receiver operating characteristic curves. p values < 0.05 were significant. Results: The multivariate LRM showed that ECV > 21.47% in DECT180s could predict thymic carcinoma (odds ratio [OR], 11.4; 95% confidence interval [CI], 1.18-109; p = 0.035). Diagnostic performance was as follows: Sensitivity, 83.3%; specificity, 69.4%; AUC, 0.76. In high-risk thymoma vs. low-risk thymoma, the multivariate LRM showed that the iodine effect ≤1.31 mg/cc could predict high-risk thymoma (OR, 7; 95% CI, 1.02-39.1; p = 0.027). Diagnostic performance was as follows: Sensitivity, 87.5%; specificity, 50%; AUC, 0.69. Tissue fibrosis significantly correlated with thymic carcinoma (p = 0.026). Conclusions: ECV on DECT180s related to fibrosis may predict thymic carcinoma from thymic epithelial tumors, and the iodine effect on DECT180s may predict high-risk thymoma from thymoma.

Clinical Validation of a Neointima-Inducing Inflow Cannula in a Continuous Flow Left Ventricular Assist Device.

Wedge thrombus formation around the inflow cannula of a continuous left ventricular assist device (LVAD) is a source of systemic thromboemboli. We previously reported the potential advantages of a new inflow cannula wrapped with titanium mesh (GU30) over the standard smooth surface oblique cut cannula (GU10). The objective of the present study was to clinically validate this new cannula. A retrospective cohort analysis of patients with implanted LVAD (EVAHEART) comparing the GU10 to the GU30 was conducted. Clinical outcomes, including survival, the incidence of thromboembolism, and bleeding events, were compared. Gross and histopathological analyses of explanted GU30 cannula were conducted following transplant or patient death. No significant differences in the survival rate, severe emboli, or cerebral bleeding were observed during the LVAD implantation. However, severe emboli occurred earlier after LVAD implantation when using the GU30 cannula compared with the GU10. In cases of long LVAD support, the neointima fully covered the inflow of the GU30 cannulae without wedge thrombus formation. The titanium mesh-wrapped inflow cannulae did not reduce the overall incidence of neurological events significantly. However, the titanium mesh-wrapped inflow cannula induced autologous neointimal growth over the cannula and prevented wedge thrombus formation in late-phase LVAD implantation.

Impact of the timing of first appropriate shock on outcomes in patients with an implantable cardioverter-defibrillator: Early versus late.

BACKGROUND: Appropriate implantable cardioverter-defibrillator (ICD) shocks are associated with an increased risk of mortality and heart failure (HF) events. The first appropriate shock may occur late after implantation. However, whether the timing of the first appropriate shock influences prognosis is unknown. This study aimed to evaluate the clinical significance of the timing of the first appropriate shock in patients with ICD. METHODS: This retrospective and observational study enrolled 565 consecutive ICD patients. Patients who received an appropriate shock were divided into the early group (first appropriate shock <1 year after ICD implantation) and late group (first appropriate shock ≥1 year after ICD implantation). All-cause mortality was compared between the two groups. RESULTS: Over a median follow-up of 5.6 years, 112 (19.8%) patients received an appropriate shock, including 32 patients (28.6%) in the early group and 80 patients (71.4%) in the late group. Comparisons of baseline characteristics at ICD implantation revealed that the late group was more likely to receive cardiac resynchronization therapy (66.3% vs. 31.3%, p < 0.001), ICD for primary prevention (60.0% vs. 31.3%, p = 0.001), and angiotensin-converting enzyme inhibitor or angiotensin-receptor blocker treatment (88.8% vs. 71.9%, p = 0.028). Survival after shock was significantly worse in the late group than in the early group (p = 0.027). In multivariable Cox proportional hazards analysis, the late group had an increased risk of all-cause mortality compared with the early group (HR: 2.22; 95% CI 1.01-4.53; p = 0.029). In both groups, the most common cause of death was HF. CONCLUSIONS: Late occurrence of the first appropriate ICD shock was associated with a worse prognosis compared with early occurrence of the first appropriate shock. Cardiac death was the most common cause of death in patients who experienced late occurrence of the first appropriate ICD shock, resulting from HF in most cases.

Implication of modified MELD scores for postdischarge prognosis in hospitalized patients with heart failure.

We evaluated whether modified Model for End-Stage Liver Disease (MELD) scores are useful for predicting the postdischarge prognosis in hospitalized patients with heart failure (HF) who are discharged alive. The MELD-XI and MELD-Na scores were calculated at discharge for a total of 1156 patients in the HIJ-HF II study. We also studied 3 groups on the basis of the left ventricular ejection fraction (LVEF): the HFrEF (LVEF < 40%), HFmrEF (LVEF 40-49%) and HFpEF (LVEF ≥ 50%) groups. The primary outcome was all-cause mortality, and the secondary outcome was rehospitalization due to worsening HF. The median MELD-XI and MELD-Na scores were 12 and 14, respectively. After a median follow-up of 19 months, there were significantly higher rates of all-cause mortality in patients with MELD-XI scores ≥ 12 than in those with MELD-XI scores < 12; there were also higher rates of all-cause mortality in patients with MELD-Na scores ≥ 14 than in those with MELD-Na scores < 14 (both log-rank p < 0.001). The cumulative incidence function based on a competing risks model showed a higher rate of rehospitalization due to worsening HF in patients with MELD-XI scores ≥ 12 than in those with MELD-XI scores < 12 and a higher rate of rehospitalization due to worsening HF in those with MELD-Na scores ≥ 14 than in those with MELD-Na scores < 14 (both Gray's test p < 0.001). The adjusted hazard ratios (HRs) of all-cause mortality for patients with MELD-XI scores ≥ 12 and those with MELD-Na scores ≥ 14 were 2.07 [95% confidence interval (CI) 1.25-3.44] and 2.79 [95% CI 1.63-4.79], respectively, in the HFrEF group; however, the HRs were not significant in the HFmrEF or HFpEF groups. Thus, MELD-XI and MELD-Na scores may be useful for predicting prognosis in hospitalized HF patients who are discharged alive, especially for those in the HFrEF group.

Clinical Management and Outcomes of Patients With Portopulmonary Hypertension Enrolled in the Japanese Multicenter Registry.

Background: Portopulmonary hypertension (PoPH) is one of the major underlying causes of pulmonary arterial hypertension (PAH). However, PoPH, especially treatment strategies, has been poorly studied. Therefore, this study evaluated current treatments for PoPH, their efficacy, and clinical outcomes of patients with PoPH. Methods and Results: Clinical data were collected for patients with PoPH who were enrolled in the Japan Pulmonary Hypertension Registry between 2008 and 2021. Hemodynamic changes, functional class, and clinical outcomes were compared between patients with PoPH treated with monotherapy and those treated with combination therapies. Clinical data were analyzed for 62 patients with PoPH, including 25 treatment-naïve patients, from 21 centers in Japan. In more than half the patients, PAH-specific therapy improved the New York Heart Association functional class by at least one class. The 3- and 5-year survival rates of these patients were 88.5% (95% confidence interval [CI] 76.0-94.7) and 80.2% (95% CI 64.8-89.3), respectively. Forty-one (66.1%) patients received combination therapy. Compared with patients who had received monotherapy, the mean pulmonary arterial pressure, pulmonary vascular resistance, and cardiac index were significantly improved in patients who had undergone combination therapies. Conclusions: Combination therapy was commonly used in patients with PoPH with a favorable prognosis. Combination therapies resulted in significant hemodynamic improvement without an increased risk of side effects.

Stratification of Destination Therapy Candidates by J-HeartMate Risk Score Among Elderly Non-Responders to Cardiac Resynchronization Therapy.

Background: For elderly patients with refractory heart failure (HF), destination therapy (DT) with a continuous-flow left ventricular assist device (LVAD) is a possible treatment. The aim of DT is for long-term, satisfying quality of life on LVAD support. Previously, elderly non-responders to cardiac resynchronization therapy (CRT) were primarily destined for palliative care, but DT has been available in Japan since April 30, 2021. This study investigated the prognosis of elderly CRT non-responders and assessed the feasibility of DT in these patients based on the J-HeartMate Risk Score (J-HMRS). Methods and Results: Of the 559 patients who underwent CRT at Tokyo Women's Medical University between 2000 and 2018, 198 were aged 65-75 years. Among these, 76 were identified as non-responders based on echocardiographic data, and were included in this study. We calculated patients' J-HMRS and investigated associations between the J-HMRS and cardiac events after CRT. Patients were divided into 3 groups according to the J-HMRS: low (n=23), medium (n=29), and high (n=24) risk. Patients in the low-risk group experienced as many HF rehospitalizations and ventricular arrhythmia events as those in the other groups. However, survival analysis revealed that, after CRT, survival was higher for patients in the low- compared with high-risk group (P=0.04). Conclusions: The J-HMRS classified 30% of elderly CRT non-responders as low risk and as suitable candidates for DT in Japan.

The evaluation of the reduction of radiation dose via deep learning-based reconstruction for cadaveric human lung CT images.

To compare the quality of CT images of the lung reconstructed using deep learning-based reconstruction (True Fidelity Image: TFI ™; GE Healthcare) to filtered back projection (FBP), and to determine the minimum tube current-time product in TFI without compromising image quality. Four cadaveric human lungs were scanned on CT at 120 kVp and different tube current-time products (10, 25, 50, 75, 100, and 175 mAs) and reconstructed with TFI and FBP. Two image evaluations were performed by three independent radiologists. In the first experiment, using the same tube current-time product, a side-by-side TFI and FBP comparison was performed. Images were evaluated with regard to noise, streak artifacts, and overall image quality. Overall image quality was evaluated in view of whole image quality. In the second experiment, CT images reconstructed using TFI and FBP with five different tube current-time products were displayed in random order, which were evaluated with reference to the 175 mAs-FBP image. Images were scored with regard to normal structure, abnormal findings, noise, streak artifacts, and overall image quality. Median scores from three radiologists were statistically analyzed. Quantitative evaluation of noise was performed by setting regions of interest (ROIs) in air. In first experiment, overall image quality was improved, and noise was decreased in images of TFI compared to that of FBP for all tube current-time products. In second experiment, scores of all evaluation items except for small vessels in images of 25 mAs-TFI were almost the same as that of 175 mAs-FBP (all p > 0.31). Using TFI instead of FBP, at least 85% radiation dose reduction could be possible without any degradation in the image quality.

CT-based radiomics analysis for differentiation between thymoma and thymic carcinoma.

Background: The purpose of our study was to differentiate between thymoma and thymic carcinoma using a radiomics analysis based on the computed tomography (CT) image features. Methods: The CT images of 61 patients with thymic epithelial tumors (TETs) who underwent contrast-enhanced CT with slice thickness <1 mm were analyzed. Pathological examination of the surgical specimens revealed thymoma in 45 and thymic carcinoma in 16. Tumor volume and the ratio of major axis to minor axis were calculated using a computer-aided diagnostic software. Sixty-one different radiomics features in the segmented CT images were extracted, then filtered and minimized by least absolute shrinkage and selection operator (LASSO) regression to select the optimal radiomics features for predicting thymic carcinoma. The association between the quantitative values and a diagnosis of thymic carcinoma were analyzed with logistic regression models. Parameters identified as significant in univariate analysis were included in multiple analyses. Receiver-operating characteristic (ROC) curves were assessed to evaluate the diagnostic performance. Results: Thymic carcinoma was significantly predominant in men (P=0.001). Optimal radiomics features for predicting thymic carcinoma were as follows: gray-level co-occurrence matrix (GLCM)-homogeneity, GLCM-energy, compactness, large zone high gray-level emphasis (LZHGE), solidity, size of minor axis, and kurtosis. Multiple logistic regression analysis of these features revealed solidity and GLCM-energy as independent indicators associated with thymic carcinoma [odds ratio, 14.7 and 14.3; 95% confidence interval (CI): 1.6-139.0 and 3.0-68.7; and P=0.045 and 0.002, respectively]. Area under the curve (AUC) for diagnosing thymic carcinoma was 0.882 (sensitivity, 81.2%; specificity, 91.1%). Multivariate analysis adjusted for sex similarly revealed two features (solidity and GLCM-energy) as independent indicators associated with thymic carcinoma (odds ratio, 14.6 and 23.9; 95% CI: 2.4-89.2 and 1.9-302.8; P=0.004 and 0.014, respectively). Adjusted AUC for diagnosing thymic carcinoma was 0.921 (95% CI: 0.82-0.97): sensitivity, 62.5% and specificity, 100%. Conclusions: Two texture features (GLCM-energy and solidity) were significant predictors of thymic carcinoma.

Acute myocardial infarction and sudden cardiac arrest caused by anomalous left coronary artery arising from the noncoronary cusp.

Anomalous left coronary artery arising from the noncoronary cusp (LCANCC) is a rare congenital disorder. We herein describe a 17-year-old female patient with sudden cardiac arrest followed by refractory cardiogenic shock. LCANCC-induced acute myocardial infarction with left main coronary artery involvement was subsequently diagnosed, and the patient required a durable left ventricular assist device. .

Portopulmonary hypertension and the risk of high right ventricular systolic pressure in liver transplant candidates.

AIM: Portopulmonary hypertension (PoPH) is a rare and serious complication of liver cirrhosis and portal hypertension that can interfere with liver transplantation (LT). We evaluated the prevalence of PoPH and the clinical features of right ventricular systolic pressure (RVSP), which is equivalent to pulmonary artery systolic pressure, in LT candidates. METHODS: This was a single-center retrospective study. A total of 157 Japanese patients with decompensated liver cirrhosis or portal hypertension (76 men, median age = 52 years [range: 18-68 years]) were enrolled. The relationships between RVSP and clinical parameters, and the prevalence of PoPH in LT candidates, were evaluated. RESULTS: The cardiological parameters were as follows: brain natriuretic peptide (BNP), 39.1 (4.0-780.5) pg/mL; RVSP, 31.2 (16.0-122.4) mmHg; ejection fraction, 58% (28-72%); and mean peak tricuspid regurgitation velocity, 2.3 (1.5-5.3) m/s. The RVSP was significantly higher in females (p = 0.02) and primary biliary cholangitis (PBC) patients (p = 0.01), and was weakly correlated with the BNP level (r = 0.40, p = 0.01). For RVSPs of < 36 and ≥ 36 mmHg, the 5-year survival rates were 36.1% versus 34.1%, and 85.4% versus 85.3%, in non-LT and LT cases, respectively (p = 0.47 and 0.69, respectively). Among six patients with an RVSP ≥ 50 mmHg, three (1.9%) were diagnosed with PoPH and treated with vasodilators. CONCLUSIONS: PoPH was observed in 3 cases (1.9%) in 157 LT candidates. In patients with suspected mild pulmonary hypertension (RVSP, 36 - 50 mmHg), LT was successfully performed.

Acute decompensated heart failure in a patient with primary aldosteronism successfully treated with an adrenalectomy: A case report.

Primary aldosteronism is often associated with heart failure (HF), and is reportedly difficult to treat in some cases. We report a case of severe HF associated with primary aldosteronism. A patient with HF, who was suspected of having primary aldosteronism, was referred to and examined at our hospital. After detailed examination, the patient was diagnosed with exacerbation of HF, and was treated at our department. Catheterization after admission revealed Forrester class IV HF. The patient was treated with catecholamine infusion in combination with medical treatment including mineralocorticoid receptor antagonists. The patient was diagnosed with hypertension due to primary aldosteronism and intractable secondary HF with increased peripheral vascular resistance. An open adrenalectomy was successfully performed under intra-aortic balloon pumping. Right heart catheterization, performed soon thereafter, demonstrated improvement in the patient's blood pressure and hemodynamics. We speculate that the improved cardiac function resulted from a reduction in the vascular resistance, as a consequence of the adrenalectomy. .

Systemic sclerosis complicated by chronic thromboembolic pulmonary hypertension treated with balloon pulmonary angioplasty: a case report.

BACKGROUND: Systemic sclerosis (SSc) is known to induce pulmonary hypertension (PH), resulting in poor prognosis. Pulmonary hypertension secondary to connective tissue disease is usually classified as Group 1. In patients with SSc, PH owing to left heart failure (Group 2) and interstitial pneumonia (Group 3) are also known; however, there have been limited reports on chronic thromboembolic PH (CTEPH), which corresponds to Group 4. CASE SUMMARY: A 39-year-old female with SSc was admitted with shortness of breath on exertion for the past 4 months. Right heart catheterization revealed severe PH. Group 1 PH secondary to collagen tissue disease was suspected; however, thoracic computed tomography and lung perfusion scan led to the diagnosis of CTEPH of Group 4. We treated the PH with several medications and balloon pulmonary angioplasty (BPA), which improved the PH and right heart failure. Consequently, her overall condition also improved. DISCUSSION: This is a rare case report of SSc complicated by CTEPH, which was treated with BPA. Patients with SSc are prone to complications of various type of PH. Thus, it is important to distinguish CTEPH in terms of treatment choice and prognosis.