与IgG4 gammdisease相关的严重肺静脉狭窄: 一个不寻常的病例。
- 作者列表："Harky A","El-Saegh M","Earl U","Fergusson J","Theologou T
:IgG4-related disease (IgG4-RD)is a multifactorial and systemic immune-mediated disease. Pulmonary vein stenosis related to IgG4 is a rare entity. We report a case of a 57-year-old male who presented with vague symptoms of weakness, easy fatigue, shortness of breathing, chest tightness, and occasional palpitations. The patient was admitted, and investigations were arranged in the form of anterior mediastinotomy and pleural biopsies were taken to look for a possible diagnosis. The histopathology was reported as IgG4-related immunopathy. A transoesophageal echocardiogram was performed and this showed severe stenosis of left superior pulmonary vein. In patients with rare pathologies such as IgG4-RD, a transesophageal can be a useful diagnostic aid especially in patients with severely impaired renal functions which does not allow utilization of contrast computed tomography.
: IgG4-related疾病 (IgG4-RD) 是一种多因素和全身性免疫介导的疾病。与IgG4相关的肺静脉狭窄是一种罕见的实体。我们报告一例57岁的男性患者，表现为虚弱、乏力、呼吸急促、胸闷和偶尔心慌的模糊症状。患者入院，以前纵隔切开术的形式安排检查，并进行胸膜活检以寻找可能的诊断。组织病理学报告为IgG4-related免疫病。经食管超声心动图显示左上肺静脉严重狭窄。在具有诸如IgG4-RD的罕见病理的患者中，经食管可以是有用的诊断辅助，特别是在具有严重受损的肾功能的患者中，其不允许利用对比计算机断层扫描。
METHODS::We present the case of a 61-year-old woman with a large tumoral infiltration extending from the pelvis throughout the inferior vena cava inferior to the right atrium, protruding into the right ventricle and right ventricular outflow tract. She had been treated 10 years before for low-grade endometrial stromal sarcoma by hysterectomy and adnexectomy followed by hormone- and radio-therapy. Due to cancer recurrence, she underwent peritonectomy, appendectomy, and resection of terminal ileum.
METHODS:AIMS:Significant platelet activation after long stented coronary segments has been associated with periprocedural microvascular impairment and myonecrosis. In long lesions treated either with an everolimus-eluting bioresorbable vascular scaffold (BVS) or an everolimus-eluting stent (EES), we aimed to investigate (a) procedure-related microvascular impairment, and (b) the relationship of platelet activation with microvascular function and related myonecrosis. METHODS AND RESULTS:Patients (n=66) undergoing elective percutaneous coronary intervention (PCI) in long lesions were randomised 1:1 to either BVS or EES. The primary endpoint was the difference between groups in changes of pressure-derived corrected index of microvascular resistance (cIMR) after PCI. Periprocedural myonecrosis was assessed by high-sensitivity cardiac troponin T (hs-cTnT), platelet reactivity by high-sensitivity adenosine diphosphate (hs-ADP)-induced platelet reactivity with the Multiplate Analyzer. Post-dilatation was more frequent in the BVS group, with consequent longer procedure time. A significant difference was observed between the two groups in the primary endpoint of ΔcIMR (p=0.04). hs-ADP was not different between the groups at different time points. hs-cTnT significantly increased after PCI, without difference between the groups. CONCLUSIONS:In long lesions, BVS implantation is associated with significant acute reduction in IMR as compared with EES, with no significant interaction with platelet reactivity or periprocedural myonecrosis.
METHODS:BACKGROUND:Aortopulmonary window is an uncommon congenital heart disease, with untreated cases not surviving beyond childhood. However, very rarely it can present in adult patients with features of pulmonary hypertension. Clinically these patients cannot be differentiated from other more common conditions with left to right shunt. Transthoracic echocardiography if performed meticulously, can depict the defect in aortopulmonary septum. RESULTS:We report a case of large unrepaired aortopulmonary window in a 23 years old patient, diagnosed on transthoracic echocardiography.