An Iron-chelating Agent Improved the Cardiac Function in a Patient with Severe Heart Failure Due to Hereditary Hemochromatosis.

A 24-year-old man was admitted to our hospital because of severe heart failure. Although he was treated with diuretics and positive inotropic agents, his heart failure progressed. An endomyocardial biopsy revealed iron deposition in his myocytes. Finally, he was diagnosed with hereditary hemochromatosis. After starting administration of an iron-chelating agent in addition to conventional treatment for heart failure, his condition improved. We should consider hemochromatosis in heart failure patients with severe right ventricular dysfunction in addition to left ventricular dysfunction.

Prevalence of Left Ventricular Myocardial Crypts in Japanese Patients.

Background Myocardial crypts are congenital abnormalities associated with hypertrophic cardiomyopathy (HCM) and other conditions. This study assessed the prevalence of myocardial crypts in Japanese patients. Methods and Results Myocardial crypts were evaluated in a consecutive series of 300 patients (13-92 years old) who underwent computed tomography angiography (CTA) because of clinical suspicion of ischemic heart disease. We found a myocardial crypt incidence of 9.7% (29 patients) in our study population, with multiple crypts observed in 2.3% (7 patients). Among these, myocardial crypts were found in 2 out of 8 (25%) patients with hypertrophic cardiomyopathy (HCM), 1 of which was apical-type HCM. In patients with a single crypt (22 patients), the most common location of the crypt was at the left ventricular apex (16/22 patients, 72.7%), followed by the inferior wall (5/22 patients, 22.7%) and the interventricular septum (1/22 patients, 4.6%). Conclusion The incidence of myocardial crypts observed in our study aligns with that reported in previous studies, although the most common location among the Japanese population was the left ventricular apex.

The hemodynamics of adrenal veins with four-dimensional computed tomography using quantitative time-density curve: a study based on aldosteronism patients.

Present study quantitatively analyzed adrenal venous flow using four-dimensional computed tomography (4D CT). We reviewed 4D CT images of 55 patients [mean age, 52 years ± 11 (standard deviation); 23 females] who underwent adrenal venous sampling between August 2017 and February 2021. Time-density curves were referred for the adrenal venous enhancement. The clinical factors affecting hemodynamics were assessed using uni- and multivariate linear regression analyses. The right and left adrenal veins (RAV and LAV, respectively) were visualized in all cases. Mean peak enhancement values in RAV and LAV were 247 ± 67 and 292 ± 70 Hounsfield units (P < 0.01), and were reached at 44.43 ± 6.86 and 45.39 ± 7.53 s (P < 0.01), respectively. The body mass index (BMI), plasma renin activity and potassium were significant factors influencing the peak enhancement of RAV blood flow [standardized regression coefficients, - 0.327 (P = 0.017), - 0.346 (P = 0.013), 0.426 (P = 0.016), respectively]. A linear relationship between sex and the time-to-peak was observed for RAV [standardized regression coefficient, 0.348 (P = 0.046)]. RAV had a lower contrast effect than LAV and reached its peak faster. BMI, plasma renin activity, and potassium were associated with flow density in RAV. Sex independently influenced the time-to-peak.

Benefits of adrenal venous sampling with preoperative four-dimensional CT imaging.

BACKGROUND: The effects of adrenal venous sampling (AVS) may be limited by the anatomical variants of adrenal veins. PURPOSE: To investigate the benefits of AVS for patients who underwent four-dimensional computed tomography (4D CT) before AVS. MATERIAL AND METHODS: We reviewed the images of contrast-enhanced four phase three-dimensional (3D) and 4D CT in patients who received AVS between February 2010 and February 2021. A total of 112 patients (59 women; mean age = 55.3 ± 11.8 years) were enrolled. Of the entire population, 49.1% (55/112) underwent 4D CT, whereas 50.9% (57/112) underwent 3D CT. The anatomical features of adrenal veins and procedural data were obtained. Simple linear regression analyses were performed to determine the relationship between imaging protocols and AVS. RESULTS: On comparison of the two groups, the 4D cohort had a higher success rate (98.2% vs. 78.9%; P = 0.001), shorter procedure and fluoroscopy time (73.6 ± 37.3 min vs. 110.5 ± 47.9 min; P < 0.001 and 28.7 ± 31.2 min vs. 97.4 ± 251.7 min; P = 0.047, respectively), lower radiation exposure (243.5 ± 315.5 mGycm2 vs. 613.4 ± 674.6 mGycm2; P < 0.001) and less contrast volume (46.2 ± 42.7 ml vs. 68.3 ± 47.4 ml vs; P = 0.014). In simple linear regression analysis, positive and negative identification of right adrenal vein before AVS significantly influenced the success rate (unstandardized coefficients [UC] = 0.304, standardized coefficients [SC] = 0.304; P = 0.001) and operation duration (UC = -46.124, SC = -0.318; P = 0.001). CONCLUSION: Pre-procedural 4D CT may facilitate successful AVS. Compared with four-phase 3D CT, this protocol is better to shorten the operation and fluoroscopy time, and to reduce the radiation dose and contrast consumption.

Diagnosis of Chronic Thromboembolic Pulmonary Hypertension Using Quantitative Lung Perfusion Parameters Extracted From Dual-energy Computed Tomography Images.

PURPOSE: To evaluate quantified iodine mapping parameters in dual-energy computed tomography in normal patients versus those with chronic thromboembolic pulmonary hypertension (CTEPH) with and without pulmonary thromboembolism. MATERIALS AND METHODS: Using automatically quantified iodine mapping in dual-energy computed tomography, we evaluated lung relative average enhancement, standard deviation (SD), and the SD/lung relative average enhancement ratio. We compared the values for these parameters in normal patients versus those with CTEPH. We also performed a receiver operating characteristic curve analysis to determine the diagnostic cutoffs for the parameters. RESULTS: Patients constituted 41 patients (10 male [24.4%] and 31 female [75.6%]; mean age [SD]: 70.0 y [13.3]) with CTEPH and 237 (92 male [38.8%] and 145 female [61.2%]; mean age [SD]: 65.9 y [15.9]) normal patients. We found significant differences in lung relative average enhancement (34.9±6.3 vs. 26.9±6.3; P <0.0001), SD (11.6±1.9 vs. 14.7±3.3; P <0.001), and the SD/lung relative average enhancement ratio (33.7±5.0 vs. 55.7±10.4; P <0.001) between the normal and CTEPH groups, respectively. The ROC analyses demonstrated high discriminatory power (area under the curve=0.99) for using the SD/lung relative average enhancement ratio to differentiate between patients in the normal group and CTEPH group. At a threshold for the area under the curve of 44.2, diagnostic sensitivity, specificity, positive predictive value, and negative predictive value for the ratio were 92.7%, 97.5%, 86.5%, and 98.7%, respectively. CONCLUSIONS: Patients with CTEPH were well-discriminated from normal patients using the SD/lung relative average enhancement ratio.

CT findings of type A acute aortic dissection that did and did not result in prehospital death.

ABSTRACT: The differences between the pathologies of cases of type A acute aortic dissection (AAD) that did and did not result in prehospital death (PHD) have not been fully elucidated.This study aimed to compare the CT findings and clarify the differences between the pathologies of such cases.Ninety four consecutive type A AAD patients between 2010 and 2020 were enrolled in this study. There were 47 males and 47 females (mean age: 69.0 ±â€Š14.4 years). The patients were divided into those that did (n = 25, 27%) and did not (n = 69, 73%) suffer PHD. We retrospectively evaluated the CT or postmortem CT findings of each case and analyzed the relationships between clinical factors (CT findings and clinical characteristics) and PHD using logistic regression analysis.Bloody pericardial effusion (96% vs 35%, P < .0001), bloody pleural effusion (40% vs 1%, P < .0001), and mediastinal hematomas (88% vs 14%, P < .0001) were significantly more common in the PHD group than in the no PHD group.In the multivariate logistic regression analysis, bloody pericardial effusion and lung consolidation were found to be significant risk factors for PHD (odds ratio: 21.29 [95% confidence intervals {CI}: 1.19-248.29] and 13.72 [95% CI: 1.79-105.06], respectively; P = .014 and P  = .012, respectively). AD affecting the abdominal aorta was identified as a significant negative risk factor for PHD (odds ratio: 0.02 [95% CI: 0.01-0.65]; P = .0042).Most PHD due to type A AAD are associated with hemorrhaging. Bleeding into the pericardium and type A AAD confined to the thoracic aorta are significant risk factors for PHD. Secondary respiratory failure might contribute to PHD in such cases.

Ulcer-like projections into the dilated false lumen after stent-graft placement for aortic dissection: A case report.

RATIONALE: Acute type A aortic dissection and chronic type B aortic dissection (TBAD) occurs simultaneously in rare cases. Although the development of ulcer-like projection (ULP) is associated with an increase in adverse aorta-related events, the false-lumen enlargement caused by the ULP progression is uncommon. PATIENT CONCERNS: A 72-year-old female with chronic TBAD was admitted to our unit with back and chest pain. Computed tomography revealed acute type A aortic dissection and a hematoma caused by rupturing of the descending aorta due to chronic TBAD. After endovascular intervention, the false lumen thrombosed and shrunk. DIAGNOSIS: After 9 months, a developing ULP, which projected into a dilating false lumen, was found. An impending ruptured descending aortic aneurysm was confirmed. INTERVENTIONS: Emergency Total arch replacement and thoracic endovascular aortic repair (TEVAR) was performed. OUTCOMES: The procedure was successful. One year later, regular follow-up showed that the false lumen had completely shrunk. LESSONS: ULP can arise and cause progressive dilation of false lumen after TEVAR. Careful and regular computed tomography examinations are required for early diagnosis of false lumen becoming thrombosed after TEVAR. Close follow-up and timely intervention, including TEVAR, should be considered in cases of aortic enlargement due to a newly developed ULP.

Comparison of Outcomes and Complications Among Patients with Different Indications of Acute/Subacute Complicated Stanford Type B Aortic Dissection Treated by TEVAR: Data from the JaPanese REtrospective multicenter stuDy of ThoracIc Endovascular Aortic Repair for Complicated Type B Aortic Dissection (J-Predictive Study).

PURPOSE: To investigate the relationships between indications for thoracic endovascular aortic repair for acute/subacute complicated Stanford type B aortic dissection and clinical outcomes, and complications specific to thoracic endovascular aortic repair. MATERIAL AND METHODS: The J-predictive study retrospectively collected data of patients treated with thoracic endovascular aortic repair for complicated Stanford type B aortic dissection at 20 institutions from January 2012 to March 2017. From the database, those treated for acute/subacute complicated Stanford type B aortic dissection were extracted (n = 118; 96 men; average age, 66.1 years; standard deviation, ± 13) and classified into groups 1, 2, and 3 according to thoracic endovascular aortic repair indications (rupture, superior mesenteric artery malperfusion, and renal or lower extremity malperfusion, respectively). Primary and secondary measures were mortality (overall and aortic-related) and complications related to thoracic endovascular aortic repair, respectively. For each outcome, the risks of being in groups 1 and 2 were statistically compared with that of being in group 3 as a control using Fisher's exact test. RESULTS: Mortality rate (odds ratio, 5.22; 95% confidence interval [CI], 1.33-20.53) and prevalence of paraparesis/paraplegia (odds ratio, 30.46; confidence interval, 1.71-541.77) were higher in group 1 than in group 3. Compared to group 3, group 2 showed no statistically significant differences in mortality or complications related to thoracic endovascular aortic repair. CONCLUSIONS: Rupture as an indication for thoracic endovascular aortic repair for type B aortic dissection was more likely to result in worse mortality and high prevalence of spinal cord ischemia. LEVEL OF EVIDENCE: Level 4, Case series.

Initial experience with intensity distribution analysis of hemodynamic parameters in the thoracic aorta using four-dimensional magnetic resonance imaging: A comparison between groups with different ejection fractions.

ABSTRACT: The purpose of this study was to investigate whether there were significant differences in the intensity distributions of thoracic aorta hemodynamic parameters between groups with different ejection fractions (EF) using four-dimensional flow magnetic resonance imaging and to investigate the relationships between each parameter.A total of 26 patients, 13 each with EF of >60% and <30%, underwent cardiac four-dimensional flow magnetic resonance imaging (EF >60%: mean age: 54 ±â€Š11.6 years, EF <30%: mean age: 49.2 ±â€Š17.2 years). The thoracic aorta was divided into the proximal and distal ascending aorta (AAo), aortic arch, and the proximal and distal descending aorta, and each section was further divided into the anterior wall, posterior wall, lesser curvature, and greater curvature. The intensity distributions of wall shear stress (WSS), energy loss (EL), and vorticity (Vort) (hemodynamic parameters) and the concordance rates between these distributions were analyzed.The concordance rate between the intensity distributions of EL and Vort was high. Only the intensity distributions of EL and Vort in the distal AAo differed significantly between the groups (P < .001). In the EF >60% group, these intensity distributions showed higher values in the greater curvature of the AAo, whereas in the EF <30% group higher values were seen in the lesser curvature of the AAo.Although there was no significant intergroup difference in the WSS intensity distribution, in the EF <30% group the WSS intensity distribution tended to exhibit higher values in the lesser curvature of the distal AAo, and the WSS intensity distribution values for the greater curvature tended to gradually increase from the arch to the proximal descending aorta.The only significant differences between the EF groups were found in the intensity distributions of EL and Vort in the distal AAo. This suggests that the distributions of atherosclerosis may be EF-dependent.

Effect of Balloon Pulmonary Angioplasty on Homogenization of Lung Perfusion Blood Volume by Dual-Energy Computed Tomography in Patients with Chronic Thromboembolic Pulmonary Hypertension.

OBJECTIVE: Balloon pulmonary angioplasty (BPA) is used to treat patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH); the goal is to improve pulmonary perfusion. We aimed to evaluate lung perfusion blood volume (PBV) with haemodynamic and exercise-capacity parameters to assess the efficacy of BPA in the treatment of CTEPH. METHODS: We retrospectively studied 33 patients over a 6-year period. DECT pulmonary angiography was performed before and after BPA. DECT provided iodine distribution maps; whole-lung and regional PBV images and quantification were generated using post-processing software. A mosaic pattern suggesting perfusion inhomogeneity is typical in CTEPH. Hypothetically, BPA treatment would promote homogenization that would be reflected in the calculated standard deviation. RESULTS: Lung perfusion images showed decreased heterogeneity after BPA. There was a significant difference before and after BPA in the whole-lung PBV and in the regional standard deviation for pulmonary arterial pressure (R = 0.37, p = 0.032 and R = 0.57, p = 0.006), pulmonary vascular resistance (R = 0.51, p = 0.023 and R = 0.60, p = 0.002), transtricuspid pressure gradient (R = 0.50, p = 0.0028 and R = 0.61, p = 0.0001), brain natriuretic peptide (R = 0.54, p = 0.0012 and R = 0.46, p = 0.0078), and 6-min walking distance (R = 0.59, p = 0.003 and R = 0.26, p = 0.14). The effects were especially pronounced after the first BPA procedure. CONCLUSION: Decreased lung heterogeneity may suggest BPA efficacy in treating CTEPH. After BPA treatment, improved lung PBV and improved regional standard deviation showed a strong positive correlation with haemodynamic parameters and exercise capacity, which also suggests that BPA is effective in treating CTEPH.

Embolization for massive bleeding due to spontaneous left external iliac vein rupture: report of a successful case.

BACKGROUND: Spontaneous rupture of the iliac vein has rarely been reported. Its associated hypovolemic shock-related symptoms and signs, including syncope and hypotension, have been observed in most of these cases. Successful transcatheter venous embolization for massive bleeding due to spontaneous rupture of the external iliac vein was herein reported. CASE PRESENTATION: An 82-year-old female patient developed sudden left lower abdominal and back pain. Immediately, she lost consciousness and went into shock. CT images of her abdomen revealed a huge retroperitoneal hematoma, with leakage of contrast medium in the hematoma in the left flank. These findings suggested left external iliac vein rupture. Open surgery was considered; however, since the patient's condition may have deteriorated further due to the time needed to prepare for surgery, including general anesthesia, transcatheter venous embolization of the left iliac vein was selected. A 5.2-Fr compliant balloon catheter (nominal diameter of 10 mm) was inflated at the distal site of the external iliac vein to reduce extravasation. N-butyl-2-cyanoacrylate (NBCA) was mixed with Lipiodol at a ratio of 1:2. The left Iliac vein was filled and completely embolized with the NBCA/Lipiodol mixture (total injected volume, 5 mL) using a 1.8-Fr microcatheter. After embolization, the patient quickly. An inferior vena cava filter was placed 1 day after embolization. CONCLUSION: Spontaneous rupture of the iliac vein is a very rare and lethal condition. Transcatheter venous embolization may control potentially life-threatening bleeding. Rapid bleeding control in a critical condition is facilitated by this minimally invasive approach.

Endovascular Repair of Diffuse Pulmonary Arteriovenous Malformations with Systemic Arterial Shunts.

Diffuse pulmonary arteriovenous malformations (PAVMs) are defined as arteriovenous malformations (AVMs) involving subsegmental, segmental, or both types of arteries in at least 1 lung lobe and are more extensive than multiple PAVMs. Diffuse PAVMs involving systemic arterial shunts are very rare. We describe a rare case, in which diffuse PAVMs involving systemic arterial shunts were successfully treated with coil embolization.

The Successful Treatment of an Ilio-Iliac Fistula and Aneurysms Affecting the Abdominal Aortic and Iliac Arteries via Endovascular Stent Graft Repair.

Spontaneous ilio-iliac arteriovenous fistula (AVF) associated with aneurysms affecting the abdominal aortic and iliac arteries is a rare condition. The classical clinical symptoms of ilio-iliac AVF include high-output heart failure, abdominal pain, abdominal bruits and thrills, a pulsatile abdominal mass, and venous congestion symptoms (leg edema and hematuria). The prompt repair of AVF is necessary to restore the patient's hemodynamics. We report a case in which a patient with aneurysms affecting the abdominal aortic and iliac arteries and an ilio-iliac AVF presented with high-output heart failure and leg ischemia and was successfully treated via endovascular stent graft repair.

Asymptomatic floating thrombus in the ascending aorta depicted on four-dimensional computed tomography.

Aortic mural thrombi of the ascending aorta are rare. If an aortic mural thrombus is dislodged, it can cause various embolic complications, which can sometimes be fatal. Although contrast-enhanced computed tomography (CT) and transesophageal echography are useful for diagnosing aortic mural thrombi, four-dimensional CT (4D-CT) is one of the most useful modalities for both diagnosis and treatment selection in such cases. 4D-CT can be used to evaluate the morphology and mobility of thrombi. Furthermore, it is minimally invasive. To the best of our knowledge, there have not been any reports about 4D-CT being used to depict an asymptomatic ascending aortic thrombus. We report a very unusual case, involving an aortic mural thrombus of the ascending aorta.

Endovascular repair of an internal mammary artery aneurysm in a patient with Takayasu arteritis.

Internal mammary artery aneurysms are rare, but serious, clinical entities. We describe a rare case in which an internal mammary artery aneurysm in a patient with Takayasu arteritis was successfully treated with coil embolization. To the best of our knowledge, this is the first report of an internal mammary artery aneurysm associated with Takayasu arteritis.

The outcomes of carbon dioxide digital subtraction angiography for percutaneous transluminal balloon angioplasty of access circuits and venous routes in hemodialysis patients.

The outcomes of carbon dioxide digital subtraction angiography (CO2-DSA) for performing percutaneous transluminal balloon angioplasty (balloon PTA) in hemodialysis patients has not been fully clarified. The purpose was to compare the outcomes of balloon PTA of hemodialysis shunts in terms of vessel patency between patients treated using CO2-DSA and conventional digital subtraction angiography using iodine contrast medium (C-DSA).We retrospectively evaluated 76 patients (38 males and 38 females, mean age: 65.0 ±â€Š14.0 years). They were under hemodialysis and treated with balloon PTA using CO2-DSA or C-DSA at our institution between 2009 and 2016. Mean duration of the follow-up period was 25.59 ±â€Š21.45 months. We compared the patency rates obtained after CO2-DSA-based balloon PTA with those after C-DSA-based balloon PTA. Secondary patency, which was defined as the duration of patency after all further endovascular interventions until surgical repair, was considered as the endpoint in this study.Overall, 19 and 57 patients underwent CO2-DSA- and C-DSA-based balloon PTA, respectively. CO2-DSA- and C-DSA-based balloon PTA produced clinical success rates of 100% and 96.5%, respectively. Blood vessel injury occurred in one patient who underwent C-DSA-based balloon PTA. No major complications occurred in CO2 group. At 24 months, the post-PTA secondary patency rates of CO2-DSA- and C-DSA-based balloon PTA were 94.1% and 93.9%, respectively (P = .9594).CO2-DSA is safe for hemodialysis patients. Compared with C-DSA, CO2-DSA-based balloon PTA produces have a similar secondary patency rate.