Pulmonary Artery Sarcoma Mimicking Pulmonary Embolism

Primary sarcomas that arise from major blood vessels are exceedingly rare, and some of the published cases have been autopsy reports. Most patients are adults. We report a case of pulmonary artery sarcoma in a 77-year-old man who presented with acute onset of dyspnea. Magnetic resonance imaging of the chest revealed a large mass within the pulmonary trunk and its main branches. Because massive pulmonary embolism was suspected, both anticoagulant and thrombolytic therapies were initiated. The patient responded poorly to these therapies, which then necessitated resection of both the mass and the pulmonary valve. A bioprosthetic porcine valve replaced the native valve, and we reconstructed the right ventricular outflow tract with a Dacron patch. Histopathologic examination revealed a high-grade sarcoma with focal myogenic and chondrogenic differentiation. The patient tolerated the procedure well and was discharged from the hospital on postoperative day 7. He was subsequently treated with chemotherapy and radiation and continued to show no evidence of disease.The diagnosis of pulmonary artery sarcoma should be suspected in patients who present with manifestations of pulmonary embolism, especially when there is no evidence of deep venous thrombosis and poor response to anticoagulant therapy. Multimodal therapy can provide prolonged survival.     

The Diagnosis and Treatment of Primary Pulmonary Artery Sarcoma: Two Case Reports

Pulmonary artery sarcoma (PAS) is considered a very rare tumor with a poor prognosis. We herein report two cases of PAS that were diagnosed by positron emission tomography (PET)/computed tomography (CT). In both cases, PET was an effective option for diagnosing tumors, and surgical resection was a valid treatment for these diseases. If a pulmonary artery tumor is suspected, PET/CT is useful for diagnosing PAS and very helpful for choosing the surgical treatment strategy.     

Intimal Sarcoma in the Aortic Arch Partially Obstructing the Aorta with Metastasis to the Brain

Primary tumors of the aorta are rare entities. We report the unusual manifestation of an aortic intimal sarcoma that presented as a brain metastasis in a 56-year-old, otherwise healthy woman. After the brain mass had been resected, multiple imaging methods revealed pseudocoarctation and the primary tumor in the aortic arch. To our knowledge, this is the first report of the diagnosis of an aortic intimal sarcoma with use of real-time, 3-dimensional transesophageal echocardiography.     

18F-FDG PET/CT纳入肝细胞癌肝移植标准的趋势与价值

肝移植是治疗肝细胞癌(HCC)的有效方法,为降低HCC肝移植术后可能出现较高肿瘤复发率,有学者率先提出著名的Milan标准。但该标准过于严格,部分患者因其肿瘤病变较大或多个结节,虽其生物行为相对“温良”,也被排除在等待肝移植名单之外,随之世界各地出现了众多的“扩大Milan版标准”。HCC组织病理学的微血管侵犯(MVI)、肿瘤组织低分化与HCC肝移植术后较高复发率有显著相关性。复习总结了近年来国内外18氟-脱氧葡萄糖(18F-FDG)PET/CT在HCC肝移植方面的应用文献,发现18F-FDG在HCC病变部位不同的摄取程度,反映了肿瘤组织生物学行为特征即侵袭性的差异;18F-FDG高摄取与HCC病变的MVI、低分化呈正相关;18F-FDG还能敏感、准确地发现HCC肝外转移灶。认为术前18F-FDG PET/CT结果对HCC肝移植预后评估有巨大价值,将其结果纳入HCC肝移植标准是趋势所归,也有望统一“扩大Milan版标准”。建议新的肝移植标准可定义为,原则上遵循Milan标准;对超出Milan标准者,满足HCC病变18F-FDG PET/CT阴性,且排除大血管侵犯和肝外转移。     

18F-FDG PET/CT对显像阳性原发性肝癌125I粒子植入治疗疗效价值的评估

目的:探讨<'18>F-FDG PET/CT对显像阳性的原发性肝癌行<'125>I粒子植入治疗疗效价值的评价.方法:原发性肝癌患者39例,共55个肿瘤病灶,均于放射性<'125>I粒子植入治疗前确定病灶为<'18>F-FDG PET/CT显像阳性;放射性<'125>I粒子植入治疗后2 mo行PET/CT检查评价疗效,之...     

Massive Pulmonary Artery Aneurysm Causing Left Main Coronary Artery Compression in the Absence of Pulmonary Hypertension

We report the case of a 62-year-old woman who presented with classic symptoms of stable angina. Cardiac images and catheterization results revealed absent pulmonary valve syndrome and compression of the left main coronary artery by a massively dilated pulmonary artery aneurysm. The patient''s anginal symptoms were relieved after pulmonary arterioplasty.Others have described proximal left main coronary artery compression in the presence of a dilated and hypertensive pulmonary artery. To our knowledge, this is the first case in which a pulmonary artery aneurysm caused left main coronary insufficiency in the absence of pulmonary hypertension—a clinically important complication of congenital pulmonary valve-related pulmonary arteriopathy.     

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery: Diagnoses and Surgical Results in 12 Pediatric Patients

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital condition. It responds well to early diagnosis and treatment, but otherwise the prognosis is poor. We present our case series of 12 patients (mean age, 2 ± 2.58 yr; age range, 2 mo–8 yr), emphasizing the diagnostic process and discussing our surgical results. The diagnosis of ALCAPA should be suspected in infants who have dilated cardiomyopathy with electrocardiographic changes that suggest ischemia, and in older children who have isolated mitral regurgitation. When clinical suspicion is high, the results of 2-dimensional echocardiography combined with color-flow Doppler studies in expert hands can establish the diagnosis, thus avoiding angiography in critically ill infants. The treatment of choice in our patients was transfer and reimplantation of the left coronary artery onto the ascending aorta. There were 2 deaths: both were infants in extremis who underwent emergency surgery. An older child with severe ventricular dysfunction was given mechanical ventricular assistance and then heart transplantation. As of this report, all 10 survivors remained well and asymptomatic.     

Trousseau Syndrome in a 25-Year-Old Woman with Occult Colon Malignancy,Lynch Syndrome,and Chronic Thromboembolic Pulmonary Hypertension

We present a rare case of thrombosis associated with an occult colon malignancy (Trousseau syndrome) in a 25-year-old woman who also presented with previously unidentified Lynch syndrome and acute-on-chronic thromboembolic pulmonary hypertension. Staged treatment included bilateral pulmonary endarterectomy under deep hypothermic circulatory arrest, followed 11 days later by laparoscopic subtotal colectomy and creation of a primary anastomosis. The patient tolerated both procedures well and recovered normal functional status. Final pathologic analysis of the resected colon mass revealed a pT3N0, stage IIA adenocarcinoma; no adjuvant therapy was administered. At her one-year follow-up visit, the patient was cancer-free, remained on lifelong apixaban anticoagulation, and was undergoing routine monitoring and genetic counseling. This case highlights the need for multidisciplinary management of a patient with severe chronic thromboembolic pulmonary hypertension and a concomitant malignancy.     

Diagnostic value for extrahepatic metastases of hepatocellular carcinoma in positron emission tomography/computed tomography scan

AIM: To evaluated the value of ¹⁸F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT) scan in diagnosis of hepatocellular carcinoma (HCC) and extrahepatic metastases.METHODS: A total of 138 patients with HCC who had both conventional imaging modalities and ¹⁸F-FDG PET/CT scan done between November 2006 and March 2011 were enrolled. Diagnostic value of each imaging modality for detection of extrahepatic metastases was evaluated. Clinical factors and tumor characteristics including PET imaging were analyzed as indicative factors for metastases by univariate and multivariate methods.RESULTS: The accuracy of chest CT was significantly superior compared with the accuracy of PET imaging for detecting lung metastases. The detection rate of metastatic pulmonary nodule ≥ 1 cm was 12/13 (92.3%), when < 1 cm was 2/10 (20%) in PET imaging. The accuracy of PET imaging was significantly superior compared with the accuracy of bone scan for detecting bone metastases. In multivariate analysis, increased tumor size (≥ 5 cm) (P = 0.042) and increased average standardized uptake value (SUV) uptake (P = 0.028) were predictive factors for extrahepatic metastases. Isometabolic HCC in PET imaging was inversely correlated in multivariate analysis (P = 0.035). According to the receiver operating characteristic curve, the optimal cutoff of average SUV to predict extrahepatic metastases was 3.4.CONCLUSION: ¹⁸F-FDG PET/CT scan is invaluable for detection of lung metastases larger than 1 cm and bone metastases. Primary HCC having larger than 5 cm and increased average SUV uptake more than 3.4 should be considered for extrahepatic metastases.     

Relation Between Contractile Reserve and Positron Emission Tomographic Patterns of Perfusion and Glucose Utilization in Chronic Ischemic Left Ventricular Dysfunction: Implications for Identification of Myocardial Viability

Objectives. This study sought to determine the incidence and extent of dobutamine-induced contractile reserve in myocardial regions characterized by classical and new positron emission tomographic (PET) patterns in patients with chronic ischemic left ventricular dysfunction.

Background. PET is considered the most accurate method for assessment of myocardial viability, which is traditionally identified by perfusion–metabolism mismatch.

Methods. In 23 patients, segmental wall thickening expressed by four echocardiographic scores at rest and during low dose (5 and 10 μg/kg body weight per min) dobutamine infusion and regional myocardial uptake of potassium-38 and fluorine-18 fluorodeoxyglucose (F-18 FDG) during glucose clamp were compared in 16 corresponding segments.

Results. Of a total of 368 segments, data analysis focused on 214 (58%) dyssynergic segments at baseline. Contractile reserve was identified with increasing incidence according to the six following PET patterns: 1) diminished perfusion and moderate reduction of F-18 FDG uptake (3 [11%] of 28 segments); 2) proportional reduction of perfusion and F-18 FDG uptake (10 [23%] of 43 segments); 3) perfusion–metabolism mismatch (19 [46%] of 41 segments); 4) preserved perfusion but moderate reduction of F-18 FDG uptake (13 [46%] of 27 segments); 5) preserved perfusion and F-18 FDG uptake (37 [59%] of 63 segments) compared with our normal database; and 6) normal perfusion but absolute increased F-18 FDG uptake (8 [73%] of 11 segments). In the latter category, only 7 of 24 segments had normal rest function. In dyssynergic segments with F-18 FDG uptake ≥50% supplied by vessels with ≥75% stenosis, improvement in contractility during dobutamine correlated with the presence of collateral channels.

Conclusions. Myocardial regions with the traditional mismatch pattern of viability show contractile reserve in slightly <50%. In segments with moderate reduction of F-18 FDG uptake, the contractile response to dobutamine is linked to the level of rest perfusion. Most segments with preserved perfusion and increased F-18 FDG uptake have impaired rest function, but contractile reserve is still present. These data suggest that in chronic ischemic left ventricular dysfunction, myocardial hibernation is a heterogeneous condition.     

Using 18F-FDG PET/CT to rule out Richter transformation as cause of deterioration in a patient with chronic lymphatic leukemia and severe COVID-19: A case report

Rationale:This case report demonstrates the use of flourine-18 fluorodeoxyglucose (¹⁸F-FDG) positron emission tomography (PET)/computed tomography (CT) to rule out Richter transformation (RT) as the cause of clinical deterioration in a patient with chronic lymphatic leukemia (CLL) and severe COVID-19. ¹⁸F-FDG PET/CT can be used to establish the diagnosis of RT in patients with CLL, but the use of ¹⁸F-FDG PET/CT to exclude RT as the cause of clinical deterioration in patients with CLL and severe COVID-19 has not previously been described.Patient concerns:A 61-year-old male with CLL and COVID-19 developed increased dyspnea, malaise and fever during hospitalization for treatment of severe and prolonged COVID-19.Diagnoses:¹⁸F-FDG PET/CT ruled out RT and revealed progression of opacities in both lungs consistent with exacerbation of severe acute respiratory syndrome coronavirus 2 pneumonia.Interventions:¹⁸F-FDG PET/CT imaging.Outcomes:The patient was discharged at day 52 without the need of supplemental oxygen, with normalized infection marks and continued care for CLL with venetoclax.Lessons:¹⁸F-FDG PET/CT ruled out RT as the cause of deteriorations in a patient with CLL and severe COVID-19, enabling directed care of exacerbation of severe acute respiratory syndrome coronavirus 2 pneumonia.     

Radiation Doses in Patients Undergoing Computed Tomographic Coronary Artery Calcium Evaluation With a 64-Slice Scanner Versus a 256-Slice Scanner

Computed tomographic coronary artery calcium scanning enables cardiovascular risk stratification; however, exposing patients to high radiation levels is an ongoing concern. New-generation computed tomographic systems use lower radiation doses than older systems do. To quantify comparative doses of radiation exposure, we prospectively acquired images from 220 patients with use of a 64-slice GE LightSpeed VCT scanner (control group, n=110) and a 256-slice GE Revolution scanner (study group, n=110). The groups were matched for age, sex, and body mass index; statistical analysis included t tests and linear regression.The mean dose-length product was 21% lower in the study group than in the control group (60.2 ± 27 vs 75.9 ± 22.6 mGy·cm; P <0.001) and also in each body mass index subgroup. Similarly, the mean effective radiation dose was 21% lower in the study group (0.84 ± 0.38 vs 1.06 ± 0.32 mSv) and lower in each weight subgroup. After adjustment for sex, women in the study group had a lower dose-length product (50.4 ± 23.4 vs 64.7 ± 27.6 mGy·cm) than men did and received a lower effective dose (0.7 ± 0.32 vs 0.9 ± 0.38 mSv) (P=0.009). As body mass index and waist circumference increased, so did doses for both scanners.Our study group was exposed to radiation doses lower than the previously determined standard of 1 mSv, even after adjustment for body mass index and waist circumference. In 256-slice scanning for coronary artery calcium, radiation doses are now similar to those in lung cancer screening and mammography.     

Intravenous Leiomyomatosis with Intracardiac Extension: Echocardiographic Study and Literature Review

Uterine leiomyomatosis is a common disease in women; however, intravenous leiomyomatosis with intracaval and intracardiac tumor extension is rare. We sought to analyze the clinical and echocardiographic features of intracardiac leiomyomatosis.From January 2003 through July 2012, 7 women (age range, 24–59 yr) underwent surgical resection of histopathologically diagnosed intracardiac leiomyomas at our hospital. Most of the patients had histories of hysterectomy or uterine leiomyoma. We retrospectively analyzed their preoperative echocardiograms. We found that the tumors had no stalks, did not adhere to the wall of the right side of the heart, were highly mobile, and moved back and forth in the right atrium near the tricuspid orifice. All tumors originated from the inferior vena cava and had borders well demarcated from that structure''s wall. Most of the masses extended into the inferior vena cava and right atrium through the right internal and common iliac veins. Computed tomograms revealed pelvic tumors and contiguous filling defects in 6 patients.When echocardiograms reveal a right-sided cardiac mass that originates from the inferior vena cava, particularly in women who have a history of hysterectomy or uterine leiomyoma, intracardiac leiomyomatosis should be suspected. If the mass has no stalk and freely moves within the inferior vena cava and right-sided cardiac chambers without attachment to the endothelial surface or endocardium, intracardiac leiomyomatosis should be diagnosed. We discuss our findings and briefly review the relevant medical literature.     

Natural History of Thoracic Aortic Aneurysms: Size Matters,Plus Moving Beyond Size

Thoracic aortic aneurysms (TAAs) are a prevalent and deadly disease that, without diagnosis and treatment, eventuates in life-threatening aortic dissection or rupture. While TAAs normally grow in an indolent manner, once a certain size (a “hinge point”) is reached, the risk of dissection, rupture, and death increases dramatically. By virtue of their common clinical “silence,” many TAAs are not diagnosed until such complications occur. While size is a helpful criterion for intervention, there is a need for parameters and markers besides aortic aneurysm size for use in diagnosing and monitoring TAAs so as to prevent natural complications of this disease.     

Pulmonary Artery 18F-Fluorodeoxyglucose Uptake by PET/CMR as a Marker of Pulmonary Hypertension in Sarcoidosis

ObjectivesThis study investigated whether pulmonary artery (PA) ¹⁸F-FDG uptake is associated with hypertension, and if it correlates to elevated pulmonary pressures.Background¹⁸F-fluorodeoxyglucose (FDG) positron emission tomography (PET) combined with computed tomography or cardiac magnetic resonance (CMR) has been used to assess inflammation mostly in large arteries of the systemic circulation. Much less is known about inflammation of the vasculature of the pulmonary system and its relationship to pulmonary hypertension (PH).MethodsIn a single-center cohort of 175 patients with suspected cardiac sarcoidosis, who underwent hybrid thoracic PET/CMR, ¹⁸F-FDG uptake in the PA was quantified according to maximum standardized uptake value (SUVmax) and target-to-background ratio (TBR) and compared with available results from right heart catheterization (RHC) or transthoracic echocardiography (TTE).ResultsThirty-three subjects demonstrated clear ¹⁸F-FDG uptake in the PA wall. In the subgroup of patients who underwent RHC (n = 10), the mean PA pressure was significantly higher in the group with PA ¹⁸F-FDG uptake compared with the group without uptake (34.4 ± 7.2 mm Hg vs 25.6 ± 9.3 mm Hg; P = 0.003), and 9 (90%) patients with PA ¹⁸F-FDG uptake had PH when a mean PA pressure cutoff of 25 mm Hg was used compared with 18 (45%) in the nonuptake group (P < 0.05). In the subgroup that underwent TTE, signs of PH were present in a significantly higher number of patients with PA ¹⁸F-FDG uptake (14 [51.9%] vs 37 [29.8%]; P < 0.05). Qualitative assessment of ¹⁸F-FDG uptake in the PA wall showed a sensitivity of 33% and specificity of 96% for separating patients with PH based on RHC-derived PA pressures. SUVmax and TBR in the PA wall correlated with PA pressure derived from RHC and/or TTE.ConclusionsWe demonstrate that ¹⁸F-FDG uptake by PET/CMR in the PA is associated with PH and that its intensity correlates with PA pressure.     

Targeting the Immune System in Atherosclerosis: JACC State-of-the-Art Review

Atherosclerosis has long been known as an inflammatory disease. However, whether targeting inflammation improves outcomes was unproven until the recent results of CANTOS (Canakinumab Anti-Inflammatory Thrombosis Outcomes Study). In this review, we reflect on why it has taken a long time to prove the inflammatory hypothesis of atherosclerosis and derive important lessons for the future. In particular, we discuss the off-target immune-modulatory effects of approved cardiovascular therapies, review the attempted anti-inflammatory therapies including the recently published CIRT (Cardiovascular Inflammation Reduction Trial), and discuss the likely reasons for their failures. We further build on CANTOS to review the immune-modulatory therapies for atherosclerosis currently in trials, and discuss the likelihood of their added value as well as the potential hazard associated with their use. We finally argue for a critical approach to the use of animal models, coupled with the use of humans as model organisms to accelerate the identification of the most appropriate targets.     

Adrenergic nervous activity in patients after surgical correction of tetralogy of Fallot

OBJECTIVES

The study was done to define the role of the autonomic nervous system in postoperative tetralogy of Fallot.

BACKGROUND

Subsequent to surgical correction of tetralogy of Fallot, patients are at long-term risk of sudden death owing to ventricular electrical instability. The status of the sympathetic nervous system in these patients, known to play an important role in other patients at risk, remains unknown.

METHODS

We used ¹²³I metaiodobenzylguanidine (MIBG) with tomographic imaging, combined with assessment of heart rate variability (HRV), to evaluate the activity of the sympathetic nervous system. We analyzed 22 patients who had undergone total correction of tetralogy of Fallot: 13 with either no or minor ventricular arrhythmias, and 9 with sustained ventricular tachycardia or ventricular fibrillation.

RESULTS

Analysis of HRV revealed a reduction in vagal control and sympathetic dominance in all patients compared with a healthy control group of 20 subjects. A significant difference was found in the standard deviation of all the adjacent intervals between normal beats (SDNN) in patients with or without severe ventricular arrhythmias. A significant reduction in uptake of ¹²³I MIBG was demonstrated 30 min after IV injection, and a trend toward reduction after 5 h, associated with reduced washout indices. These data reflect a decrease in the number of nerve endings in the right and left ventricular walls, and an inhomogeneous distribution of the adrenergic nervous system. The uptake of MIBG was significantly reduced in the patients at risk of ventricular tachycardia or fibrillation.

CONCLUSIONS

Subsequent to surgical correction of tetralogy of Fallot, the positive correlation between myocardial uptake of MIBG, SDNN and the QRS dispersion confirmed the usefulness of analysis of the adrenergic nervous system to stratify patients at risk of life-threatening arrhythmias.