Congenital right coronary artery aneurysm with fistula to right ventricle associated with isolated pulmonary valvular stenosis

Congenital aneurysmal dilatation of coronary artery with coronary cameral fistula is rare in childhood. We report an even rarer association of congenital right coronary artery aneurysm and right coronary artery to right ventricle fistula with bicuspid pulmonary valve stenosis and an intact ventricular septum in a 3-year-old child.     

Surgical repair of candida mycotic pseudoaneurysm with extensive vegetations of the ascending aorta and pulmonary artery after heart and kidney transplant

We report a case of invasive candidiasis causing a mycotic pseudoaneurysm involving the ascending aorta and pulmonary artery. The patient presented two years after heart-kidney transplant with acute limb ischemia resulting from embolization of fungal vegetations. Operative findings included a pseudoaneurysm resulting from near-complete dehiscence of the aortic suture line, and large vegetations within the ascending aorta extended to the aortic arch and pulmonary artery, with localized dehiscence of the pulmonic suture line. The ascending aorta was replaced, and the pulmonary artery was repaired with bovine pericardium. The patient did well, and blood cultures were negative at 6 months follow-up.     

Pipeline血流导向装置治疗颅内复杂动脉瘤的效果

目的观察Pipeline血流导向装置(PED)治疗颅内复杂动脉瘤的有效性及安全性。方法回顾性分析接受PED治疗的11例颅内复杂动脉瘤患者的临床资料。结果11例患者共12个动脉瘤,植入12枚PED,其中10例植入1枚PED、1例2枚,3个动脉瘤单纯植入PED、9个植入PED联合弹簧圈栓塞;术后立即造影示动脉瘤内对比剂滞留,PED贴壁良好,完全覆盖瘤颈,载瘤动脉通畅。术后1例发生局部急性脑梗死,经治疗后康复。术后随访6~9个月,中位随访时间7.5个月,患者症状均明显缓解或消失,未发现严重并发症,动脉瘤闭塞率75.00%(6/9)。根据O'Kelly-Marotta(OKM)分级:A级0个动脉瘤,B级1个,C级2个,D级9个。改良Rankin评分(mRS):0分10例,1分1例。结论PED治疗颅内复杂动脉瘤的效果及安全性均较好。     

腔内治疗逆行撕裂Stanford A型主动脉夹层:Meta分析

目的 采用Meta分析评价腔内治疗逆行撕裂Stanford A型主动脉夹层（RAAD）的效果。方法 检索万方数据库、中国知网及PubMed、Embase、Cochrane library数据库,获得有关腔内治疗RAAD的文献。依据纳入标准筛选文献,采用R软件对文献进行Meta分析,综合评价腔内治疗RAAD围手术期并发症、死亡率及预后。结果 最终纳入11篇文献、共224例RAAD患者。Meta分析结果显示,腔内治疗RAAD围手术期内漏发生率10.22%[95% CI（0.02,0.18）],神经系统并发症发生率0.75%[95% CI（0.00,0.03）],围手术期死亡率1.22%[95% CI（0.00,0.03）]。随访期间6例死亡[0.60%,95% CI（0.00,0.03）],其中5例死因为非主动脉相关性;8例再次接受手术干预[0.89%,95% CI（0.00,0.03）]。结论 腔内治疗RAAD安全、有效。     

近肾腹主动脉瘤的腔内治疗技术进展

腹主动脉瘤腔内修复术目前已成为大部分肾下型腹主动脉瘤患者的首选治疗,但对于近端瘤颈较短的近肾腹主动脉瘤,需要涉及内脏分支重建,常规的腹主动脉瘤腔内修复术疗效并不满意。近年来,烟囱支架技术、开窗支架技术、分支支架技术、八爪鱼技术等创新性技术的使用为近肾腹主动脉瘤患者的腔内治疗提供了多种解决方案,本文回顾并总结当前腔内治疗技术的进展,以期对目前各种技术的应用难点加以分析思考。     

腹主动脉瘤性别差异性的生物力学研究进展

腹主动脉瘤具有性别差异性,男性发病率较高,而女性的破裂发生率与术后死亡率均较高,这一性别差异性现象会影响到腹主动脉瘤患者的诊断、治疗及术后康复。本文综述了腹主动脉瘤性别差异性的研究现状,从阈值判定及生物力学、力学生物学等方面探究了腹主动脉瘤性别差异性这一现象,并指出男女腹主动脉尺寸差异、解剖结构及血管结构成分不同而导致的生物力学以及力生物学的差异,可能是导致两性腹主动脉瘤呈现出差异性的重要原因。针对腹主动脉瘤性别差异性的生物力学研究,将会为腹主动脉瘤诊断、治疗及器械设计、患者护理和康复提出基于性别差异的改进建议。     

A mutation update on the LDS‐associated genes TGFB2/3 and SMAD2/3

The Loeys–Dietz syndrome (LDS) is a connective tissue disorder affecting the cardiovascular, skeletal, and ocular system. Most typically, LDS patients present with aortic aneurysms and arterial tortuosity, hypertelorism, and bifid/broad uvula or cleft palate. Initially, mutations in transforming growth factor‐β (TGF‐β) receptors (TGFBR1 and TGFBR2) were described to cause LDS, hereby leading to impaired TGF‐β signaling. More recently, TGF‐β ligands, TGFB2 and TGFB3, as well as intracellular downstream effectors of the TGF‐β pathway, SMAD2 and SMAD3, were shown to be involved in LDS. This emphasizes the role of disturbed TGF‐β signaling in LDS pathogenesis. Since most literature so far has focused on TGFBR1/2, we provide a comprehensive review on the known and some novel TGFB2/3 and SMAD2/3 mutations. For TGFB2 and SMAD3, the clinical manifestations, both of the patients previously described in the literature and our newly reported patients, are summarized in detail. This clearly indicates that LDS concerns a disorder with a broad phenotypical spectrum that is still emerging as more patients will be identified. All mutations described here are present in the corresponding Leiden Open Variant Database.     

A giant inferoposterior true aneurysm of the left ventricle mimicking a pseudoaneurysm

A left ventricular aneurysm (LVA) is most commonly the result of myocardial infarction, usually involving the anterior wall. A left ventricular pseudoaneurysm (LVPSA) or false aneurysm forms when cardiac rupture is contained by adherent pericardium or scar tissue. The accurate diagnosis, although difficult to establish, is an important one to make because these aneurysms are prone to rupture. In this article, we report a challenging case of a cardiac aneurysm a year after a coronary bypass operation which could not be definitively diagnosed despite of imaging with different techniques including echocardiography, coronary angiography, left ventriculography and magnetic resonance imaging (MRI). The patient underwent a second cardiac surgery, the aneurysm was resected, the mitral valve was replaced and the defect in the ventricular wall was repaired. Because of the combined diagnostic capabilities like detailed and functional pathoanatomy and aneurysmal wall characterization, MRI seems to have multiple advantages in differential diagnosis.     

Left ventricular aneurysm in the scope of gated perfusion SPECT: accuracy of detection and ejection fraction calculation

PURPOSE: The objective of this trial was to investigate the capacity of gated perfusion SPECT (GPS) to detect left ventricular aneurysm (ANV) by comparing QGS and 4D-MSPECT (4DM) algorithms with radionuclide ventriculography (RVG). Secondarily, the comparison of GPS ejection fraction (EF) measurements with those of contrast left ventriculography (LVG) and RVG was aimed. METHODS: Twenty-five patients with ANV confirmed by LVG were studied. The patients underwent RVG and rest Tc-99m-tetrofosmin GPS 1 week after LVG. A 9-segment model was used both in RVG and GPS evaluation. Aneurysm was defined by scoring the wall motion (WM) and phase analysis in RVG; perfusion, wall thickening and WM in GPS. RESULTS: The detection rate of ANV was 96%, 84% and 52% for RVG, QGS and 4DM, respectively. The LVG mean EF (43.52% +/- 16.93%) was significantly higher (P < 0.01) than those of RVG (29.40% +/- 10.90), QGS (30.04% +/- 13.25%) and 4DM (34.92% +/- 13.01%). Moderate to high EF correlation values were obtained between LVG and GPS (r = 0.71-0.79) and GPS-RVG (r = 0.69). There was no significant EF difference between the radionuclide methods except between 4DM-EF and RVG-EF (5.52%, P < 0.05). Wide Bland-Altman limits were observed between the radionuclide methods in EF comparisons (range: 30.5-38.5%). CONCLUSION: GPS seems to have a role in the non-invasive investigation of ANV. QGS-GPS proved to be more reliable (84%) than 4DM-GPS (52%) in the ANV detection. The localization and the extent of the aneurysm itself as well as perfusion and function of adjacent segments may affect aneurysm diagnosis by means of GPS. RVG, QGS-GPS and 4DM-GPS seem not to be interchangeable for routine EF calculation in ANV patients.