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1.
Plastic bronchitis (PB) and protein-losing enteropathy (PLE) are rare but potentially devastating complications of the Fontan circulation. PB occurs in ~4% of Fontan patients, typically presents within 2 to 3 years of Fontan completion with chronic cough, wheezing, fever, or acute asphyxiation, and is characterised by proteinaceous airway casts that are expectorated or found on bronchoscopy. PLE develops in 4% to 13% of patients, usually within 5 to 10 years post Fontan, and manifests with edema, ascites, hypoalbuminemia, lymphopenia, hypogammaglobulinemia, and elevated fecal alpha-1 antitrypsin 1. These disorders have similar pathophysiology involving disruption of the lymphatic system resulting from elevated central venous pressure combined with elevated lymphatic production and inflammation, resulting in lymphatic drainage into low-pressure circuits such as the airways (PB) and duodenum (PLE). Our understanding of these disorders has greatly improved over the past decade as a result of advances in imaging of the lymphatic system through magnetic resonance lymphangiography and early success with lymphatic interventions including lymphatic embolisation, thoracic duct embolisation, and percutaneous thoracic duct decompression. Both PB and PLE require a multidisciplinary approach that addresses and optimises residual hemodynamic lesions through catheter-based intervention, lowers central venous pressure through medical therapy, minimises symptoms, and targets abnormal lymphatic perfusion when symptoms persist. This review summarises the pathophysiology of these disorders and the current evidence base regarding management, proposes treatment algorithms, and identifies future research opportunities. Key considerations regarding the development of a lymphatic intervention program are also highlighted.  相似文献   

2.

Background

It was this study's objective to evaluate the echocardiographic characteristics and flow patterns in abdominal arteries of Fontan patients before the onset of protein‐losing enteropathy (PLE) or plastic bronchitis (PB).

Design

In this retrospective cohort investigation, we examined 170 Fontan patients from 32 different centers who had undergone echocardiographic and Doppler ultrasound examinations between June 2006 and May 2013. Follow‐up questionnaires were completed by 105 patients a median of 5.3 (1.5–8.5) years later to evaluate whether one of the complications had occurred since the examinations.

Results

A total of 91 patients never developed PLE or PB (“non‐PLE/PB”); they were compared to 14 affected patients. Eight of the 14 patients had already been diagnosed with “present PLE/PB” when examined. Six “future PLE/PB” patients developed those complications later on and were identified on follow‐up. The “future PLE/PB” patients presented significantly slower diastolic flow velocities in the celiac artery (0.1 (0.1–0.5) m/s vs 0.3 (0.1–1.0) m/s (P = .04) and in the superior mesenteric artery (0.0 (0.0–0.2) m/s vs 0.2 (0.0–0.6) m/s, P = .02) than the “non‐PLE/PB” group. Median resistance indices in the celiac artery were significantly higher (0.9 (0.8–0.9) m/s vs 0.8 (0.6–0.9) m/s, (P = .01)) even before the onset of PLE or PB.

Conclusion

An elevated flow resistance in the celiac artery may prevail in Fontan patients before the clinical manifestation of PLE or PB.  相似文献   

3.
Objective. Protein‐losing enteropathy (PLE) is a known complication of surgical procedures for congenital heart disease. The pathogenesis and pathophysiology of PLE remain poorly understood. However, lymphatic insufficiency appears central to the disease process. We sought to investigate the role of lymphatic obstruction and central venous catheter‐related central venous thrombosis in patients with congenital heart disease and PLE. Design. A case‐control study design was constructed consisting of patients with congenital heart disease and PLE and 2:1 matched controls having undergone the same definitive surgical procedure. Obstruction to lymphatic return was considered present if the thoracic duct was ligated, or if there was complete central venous obstruction at the usual site of thoracic duct drainage. Results. Obstruction to lymphatic return was identified in 4 of 16 cases (25%) and 1 of 32 controls (4%), P = .06. There was no association between PLE and central venous catheter use or duration, and no discriminating characteristics between cases and controls with respect to anatomy, pre‐Fontan hemodynamic variables, operative or perioperative factors, or hemodynamic variables at the time of PLE diagnosis. Mortality for patients with PLE was 25% compared with 9% in controls (P = not significant). Long‐term resolution of PLE was obtained in six patients (38%). Conclusion. There is a high prevalence of apparent lymphatic obstruction in patients with congenital heart disease and PLE, suggesting that physical lymphatic obstruction may play an important, and previously unrecognized role in the development of PLE in patients with complex congenital heart disease.  相似文献   

4.
We report the case of a 5.5-year-old patient (16 kg/105 cm) who presented with plastic bronchitis (PB) refractory to conservative treatment 3 months after completion of Fontan palliation. Bi-inguinal transnodal fluoroscopy-guided lymphangiogram confirmed the chylous leak originating from the thoracic duct (TD) into the chest and did not opacify any central lymphatic vessel for direct transabdominal puncture. Retrograde transfemoral approach was adopted to catheterize the TD and selectively embolize its caudal portion using microcoils and liquid embolic adhesive. Recurrence of symptoms after 2 months indicated a redo catheterization to occlude the TD entirely using the same technique. The procedure was successful and the patient was discharged after 2 days with sustained clinical improvement at 24 months postoperative. In the context of refractory PB, end-to-end transvenous retrograde embolization of the TD appears to be an interesting alternative to more complex interventions such as transabdominal puncture, decompression, or surgical ligation of the TD.  相似文献   

5.
本文以1984年5月至1991年12月间行机械瓣膜替换的144例风湿性心脏病为基础,分析心房颤动(房颤)的发生及术后的演变与有关因素的关系。144例中二尖瓣病变124例,二尖瓣与主动脉瓣联合病变20例;术前合并房颤者108例(75%)。分析表明:年龄及左房直径大者易发生房颤,术前为窦性心律及术后维持2周以上者,其左房直径均值均<53mm;术后早期存活的102例房颤患者中,部分病例房颤一度消失,但于2周内89例(86.4%)又转为房颤;术中应用电击除颤复跳对术后房颤的消失有一定作用;左房血栓患者电击除颤后房颤的再现时间比无血栓者为早。瓣膜替换术后虽然瓣膜功能的矫正使血液动力学得到改善,但根据本文统计,术后房颤仍不能得到根除,为了提高合并房颤的瓣膜疾病的手术疗效,必须在替换瓣膜的同时寻求一种针对房颤的有效附加方法。  相似文献   

6.
Protein losing enteropathy (PLE), defined as severe loss of serum protein into the intestine, occurs in 4-13% of patients after the Fontan procedure and carries a dismal prognosis with a five year survival between 46% and 59%. Chronically raised systemic venous pressure is thought to be responsible for the development of PLE in these patients, with perhaps superimposed immunological or inflammatory factors. The success rate of contemporary medical, transcatheter, and surgical treatments attempting to reduce systemic venous pressure ranges from 19% to 40%. Prednisone treatment for PLE has been tried, with variable success rates reported in children. The effect of prednisone in adult patients with PLE after the Fontan procedure is largely unknown. Two cases of PLE in adults (a 39 year old woman and a 25 year old man) after modified Fontan procedure who responded dramatically to oral prednisone treatment are reported, suggesting that a trial of this "non-invasive" treatment should be considered as long term palliation or bridge to cardiac transplantation.  相似文献   

7.
Presence of rate-dependent (RD) intraventricular conduction defects (IVCD) was documented by inducing variations in heart rate in 30 acute myocardial infarction (AMI) patients (10 right bundle branch block, six left bundle branch block, 13 left anterior hemiblocks, and two left posterior hemiblocks). Five IVCDs were tachycardia-dependent (TD), 20 were bradycardia-dependent (BD), and six were both TD and BD. In TD blocks shortest cycles showing normal intraventricular conduction ranged from 410 to 1330 msec (697 ± 84 SE); in BD blocks longest cycles with normal intraventricular conduction ranged from 450 to 1450 msec (962 ± 52). In 60% of cases intermittent incomplete RD blocks were also present. In one patient RD-IVCD intermittency remained until discharge; in the others it lasted from 4 minutes to 10 days. Afterwards 19 RD-IVCDs disappeared and four became stable; six patients died during RD-IVCD intermittency period. Disappearance of RD block was preceded by gradual reduction in cycle length showing TD block and lengthening of cycles stopped beats with BD block. Serial observation of RD-IVCDs provides information about sequence of electophysiologic effects on the intraventricular conduction system in clinical AMI.  相似文献   

8.
There is still some debate regarding the prognostic significance of left ventricular longitudinal systolic dysfunction as assessed by tissue Doppler (TD) imaging in patients with chronic heart failure (HF), since previous studies have included patients with postischemic wall motion abnormalities. Thus, this study was designed to ascertain whether TD-derived longitudinal systolic dysfunction may influence the outcome of patients with nonischemic chronic HF. In 200 consecutive patients with chronic HF secondary to dilated cardiomyopathy and no history of ischemic heart disease, peak systolic mitral annular velocity (S(m) ) was measured by pulsed TD at the septal and lateral annular sites. The end points were cardiac death or hospitalization for worsening HF. Mean follow-up duration was 30 months. In a time independent analysis, averaged S(m) calculated as the average of septal and lateral S(m) , resulted to be a significant predictor of outcome in the study population (area under receiver-operator characteristic curve: cardiovascular death, 0.69, P < 0.0001; cardiovascular events, 0.64, P = 0.0005). In a time-dependent analysis, average S(m) was associated with both cardiovascular death (hazard ratio 0.832, P = 0.0019) and cardiovascular events (hazard ratio 0.904, P = 0.039), independently of other clinical risk factors and echocardiographic parameters of systolic function. Septal S(m) but not lateral S(m) was independently associated with the outcome measures. In conclusion, the assessment of systolic mitral annular velocity by pulsed TD is a useful indicator for prognostic stratification of patients with nonischemic dilated cardiomyopathy and chronic HF.  相似文献   

9.
目的:探讨单极与双极射频消融改良迷宫术在心脏瓣膜手术中治疗心房颤动(房颤)的疗效及安全性. 方法:连续入选2010年1月至2012年12月在我院行心脏瓣膜手术,同期行射频消融改良迷宫术的患者137例.根据射频消融系统的不同,分为单极射频消融组(n=56)及双极射频消融组(n=81).比较两组患者术中射频消融时间、围术期严重并发症的发生率及死亡率、术后房颤消除率及心功能等临床指标. 结果:两组围术期严重并发症的发生率及死亡率无统计学差异.术中两组消融所需时间亦无统计学差异.两组患者术后1年的心功能分级均较术前改善,左房内径明显减小(P<0.01).与单极消融组相比,双极消融组同期的房颤消除率显著提高(P<0.05). 结论:心脏瓣膜手术同期行射频消融迷宫术是治疗心脏瓣膜疾病合并房颤安全、有效的方法.与单极射频消融相比,双极射频消融的房颤消除率更高,具有较好的临床应用价值.  相似文献   

10.
A previously described preformed No. 7 Oucor catheter for retrograde left atrlal catheterizatlon was modified for coronary anglography and used In 358 consecutive patients, 22 of whom had 26 of 51 previous aortocoronary bypass grafts still patent in 42 patients these catheters were also employed for retrograde left atrial catheterlzation. Sixty percent of right coronary, 96% of left coronary, and 96% of aortocoronary bypass anglography attempts were successful. Complete left heart study and anglography were accomplished with one catheter in ten patients (2.8%), with two catheters in 200 patients (55.9%), with three catheters in 132 patients (36.9%), and with more than three catheters In 16 patients (4.5%). Except in 4 of 12 patients with aortic valve deformities, left atrial catheterlzation was achieved whenever attempted. The complication rate was within acceptable limits. Average left heart and angiography procedure and fluoros-copy times were respectively 29.4 and 6.5 minutes. In addition to reconfirming usefulness for retrograde left atrial catheterlzation, these data demonstrate this family of catheters is useful for coronary–particularly left–and aortocoronary bypass angiography.  相似文献   

11.
Plastic bronchitis (PB) is an uncommon, potentially fatal disease, marked by endobronchial cast formation causing variable degrees of respiratory distress. Primary and secondary pulmonary lymphatic abnormalities have been identified among the underlying mechanisms of cast formation. We present a case of PB where lymphoscintigraphy demonstrated the underlying lymphatic defect. A 6‐year‐old Hispanic male with congenital heart disease (CHD; post‐Fontan) presented with recurrent pneumonia, respiratory distress. Bronchoscopy showed inflamed hypervascular mucosa and thick mucus plugs; no casts were seen. Later, PB was diagnosed after the patient expectorated a bronchial cast. Cast analysis showed lymphocytic aggregates with mucin and fibrin. Lymphoscintigraphy revealed abnormal lymphatic collaterals and retrograde trace reflux into the superior mediastinum, a picture consistent with thoracic duct lymph leakage into the tracheobronchial tree. The pathogenesis of PB is not fully understood, especially in patients with CHD. Chyle in bronchial casts suggests abnormal lymphatic flow. Reports of lymph flow abnormalities, especially endobronchial lymph leakage in CHD are limited. Lymphoscintigraphy in our case demonstrated clear evidence of retrograde lymph reflux and leakage into the bronchial tree. The case presented suggests that in some patients following Fontan surgery, high intrathoracic lymphatic pressure and retrograde lymph flow may contribute to recurrent cast formation. Finding the underlying lymphatic abnormality helps in specific case management. Lymphoscintigraphy is a safer and easier method than lymphangiography. Surgical lymphatic–venous shunting may be possible in select cases. Pediatr Pulmonol. 2013; 48:515–518. © 2012 Wiley Periodicals, Inc.  相似文献   

12.
Although tissue Doppler (TD) imaging of the left ventricle is now commonly used in clinical settings, TD imaging of the right ventricle (RV) is not routinely practiced. Yet, there are significant data on clinical uses of RV TD imaging, including established normal values using both color and spectral TD. In acute left ventricular (LV) inferior wall myocardial infarction, depressed RV TD velocities have been shown to correlate with the presence of RV impairment, and with patient outcome. In patients with LV heart failure, TD imaging has been correlated to RV ejection fraction by radionuclide angiography, and is an independent predictor of outcome. In patients with congenital heart disease, RV TD has been especially valuable for assessing RV function, and has been correlated to invasive hemodynamic indices, and RV ejection fraction by magnetic resonance imaging. The RV performance (Tei) index has been calculated and validated using TD-derived, rather than conventional pulsed Doppler time intervals. RV TD indices have been shown to be useful in the detection of subclinical and clinical disease in morbid obesity, chronic pulmonary, and systemic disease. TD-derived RV strain imaging can detect segmental myocardial dysfunction, overcoming limitations to conventional TD imaging resulting from tethering. For both TD velocity and strain imaging, however, appreciation of the limitations of these techniques is necessary for their appropriate use. Given its rapid acquisition times, reproducibility, and ease of addition to standard transthoracic echocardiographic protocols, RV TD and strain imaging are important additional modalities in the comprehensive echo-Doppler assessment of RV function.  相似文献   

13.
Objectives: To evaluate all complications that occurred during or after cardiac catheterizations for Amplatzer PFO device closure of patent foramen ovale (PFO), determine the cause of the complications and recommend techniques to minimize complications in the future. Background: Rare complications were reported to the manufacturer of the Amplatzer PFO occluder since the introduction of the device. Methods: A panel of independent physicians reviewed all complications reported to the manufacturer to determine whether the complication was related to the device or related to the cardiac catheterization procedure. Demographic data, echocardiograms, operative reports, and time to occurrence of complications were reviewed. Results: A total of 11 events were reported. Only two patients had device related complications (erosion), an incidence of 0.018%. Two patients were found to have additional atrial septal defect after PFO closure. Two patients were thought to have an inflammatory reaction without any serious sequelae. Five complications were related to the cardiac catheterization procedure (atrial appendage perforation). Conclusions: Device related complications after Amplatzer PFO occluder placement are extremely rare. Cardiac catheterization related complications appear to be the most common cause of the hemodynamic compromise. Careful manipulation of catheters and wires, recognition of the location of the catheter by fluoroscopy and echocardiography will decrease the risk of such complications. © 2008 Wiley‐Liss, Inc.  相似文献   

14.
Transcatheter mitral valve implantation (TMVI) is an emerging field in structural cardiology. A particularly difficult group to treat is high‐risk patients requiring valve in mitral annular calcification (ViMAC) intervention, with overall poor procedural success and outcomes in recent registries. This case highlights an unusual complication of paravalvular regurgitation (PVL) through the uncovered stent frame of a balloon expandable transcatheter heart valve (THV) on the left ventricular side of the prosthesis, leading to mechanical hemolysis and subsequent anuric renal failure post a ViMAC procedure. Attempts to treat the PVL with an occlusion plug device were unsuccessful and led to left circumflex coronary occlusion secondary to mechanical compression of the vessel in the posterior mitral valve annulus, a previously unreported phenomenon. A repeat valve‐in‐valve procedure was performed to treat the PVL, and immediate angioplasty resolved the left circumflex occlusion. High‐risk patients requiring TMVI pose multiple challenges to Heart Teams in the treatment of valve pathology. Optimal procedural planning, multimodality imaging, improved THVs, and the awareness of potential complications are fundamental in overcoming the learning curve of TMVI and improved outcome for patients requiring ViMAC.  相似文献   

15.
目的 总结冠状动脉严重病变的高龄患者行非体外循环冠状动脉旁路移植术 (OPCAB)的治疗经验。方法 回顾性分析 5 3例 ,年龄在 75~ 82 (77± 2 )岁 ,冠状动脉严重病变的患者行OPCAB的临床资料。 3支病变 4 4例 ,左主干病变 2 1例 ,前降支近端 90 %~ 10 0 %狭窄 2 5例 ,右冠状动脉近端 90 %~ 10 0 %狭窄 17例 ,急诊冠状动脉旁路移植术 13例。 5 3例OPCAB手术全部成功。平均远端吻合口数目 (3.0± 0 .8)个 ,移植物取乳内动脉 2 9例 ,大隐静脉 5 1例。结果 术后并发症 4例 :其中新发脑卒中 1例 ,2次开胸止血 1例 ,恶性心律失常 1例 ,多脏器功能衰竭 1例 ;主动脉内球囊反搏 7例 ;住院死亡 2例。随访时间 1~ 2 4 (10± 7)个月 ,均无急性心血管事件发生 ,非心脏性死亡 1例。结论 OPCAB对冠状动脉严重病变的高龄患者是安全可行而且有效的血运重建方式  相似文献   

16.
Background : Intracoronary optical coherence tomography (OCT) is a high‐resolution imaging modality used for evaluation of coronary lesion morphology. However, current time‐domain OCT (TD‐OCT) have a number of limitations with regard to both procedural usage and safety in the clinical setting. The next‐generation frequency‐domain OCT (FD‐OCT), which has a much faster frame rate and pullback speed than TD‐OCT, is expected to overcome these limitations. The aim of this study was to evaluate the feasibility and usability of next generation FD‐OCT in the assessment of coronary lesions. Methods : A comparison study was performed between FD‐OCT and TD‐OCT from the aspect of usability (set‐up time), qualitatively (rate of clear image segment), and safety (adverse event) in 14 ischemic heart disease patients with 20 previously implanted coronary stents. Results : The mean time of the OCT procedure in this study from setup to completion of image acquisition was 3.2 ± 0.8 min for FD‐OCT and 11.2 ± 2.5 min for TD‐OCT (P < 0.01). In qualitative image assessment, FD‐OCT has the potential to yield a higher rate of clear image segments (CIS) than TD‐OCT (99.4% vs. 80.8%, respectively; P < 0.01). In addition to these improved characteristics, there were no ischemic ECG changes or arrhythmia associated with FD‐OCT. Conclusions : The next‐generation intracoronary FD‐OCT has better performance in the clinical setting and the potential to overcome several limitations of conventional TD‐OCT systems. © 2009 Wiley‐Liss, Inc.  相似文献   

17.
合并大左室瓣膜置换术的外科治疗体会   总被引:4,自引:0,他引:4  
目的总结合并大左室瓣膜病的治疗经验。方法1998年4月~2004月年对28例合并大左室瓣膜病人行瓣膜置换术。其中二尖瓣置换术13例,主动脉瓣置换术13例,主动脉瓣和二尖瓣双瓣置换术1例,Bentall手术1例;同期处理并存畸形及病变7例。结果治愈出院25例,术后早期死亡3例。合并症22例,其中心律失常20例、低心排出量综合征2例。结论充分的术前准备,恰当的手术处理,术后积极防治恶性心律失常和低心排出量综合征是提高手术效果的关键。  相似文献   

18.

Background

A persistent anastomosis between the pulmonary veins that connect with the left atrium and the systemic vein that drains into the right atrium has occasionally been reported. We report characteristics and transcatheter therapy in partially abnormal pulmonary venous return with additional drainage to the left atrium.

Methods

We retrospectively studied such patients in 5 institutions.

Results

Ten patients (6 girls) presented at a median age of 8 (0.1 to 54) years with 2 anatomic types: 8 vertical vein types with drainage of the left upper lobe to the innominate vein via a large vertical vein (left superior cardinal vein) and to the left atrium via the left upper pulmonary vein; and 2 scimitar vein (SV) types with drainage of the right middle and lower pulmonary veins into the inferior vena cava and to the left atrium via an anomalous connecting vein. Associated malformations were aortic coarctation (n = 2) and secundum atrial septal defects (n = 3). Two patients of the vertical vein type were operated. Transcatheter occlusion of the abnormal pulmonary venous return was performed in 7 cases, associated with occlusion of systemic arterial supply (n = 2), secundum atrial septal closure (n = 2), left upper pulmonary vein stenosis stenting (n = 1), and coarctation stenting (n = 1). Including previously published cases, 18 patients (13 vertical veins and 5 scimitar veins) underwent transcatheter repair. Patients over 40 years of age tend to be symptomatic at presentation (p = 0.056).

Conclusion

In partially abnormal pulmonary venous return with dual drainage, transcatheter therapy can be offered in the majority of patients.  相似文献   

19.
The technics of drainage of the right lymphatic duct (RD) and thoracic duct (TD) used in our laboratory have been described and illustrated. In two series of experiments the components of RD and of TD lymph were compared to blood plasma collected concurrently. RD and TD lymph, collected concurrently, were also compared in a third series. RD and TD lymph differ in a number of ways including rate of flow, enzyme activity, cell count and lipid-electrophoretic patterns. The limitations of RD lymph for the study of fluid and protein dynamics of the lungs and the cell population of lung lymph are outlined. Higher levels of enzyme activity in RD compared to TD lymph have been noted. This phenomena appears to depend on a number of complex factors.  相似文献   

20.
Elderly patients with aortic stenosis are often deemed too high risk, and consequently turned down for conventional surgery. Transcatheter aortic valve implantation (TAVI) is a safe and an increasingly attractive option in this group of patients. Although TAVI has been shown to be successful and safe, the cardiovascular assessment of other co‐morbidities in this susceptible group of patients is critical to ensuring good clinical outcomes. The presence of a saccular abdominal aortic aneurysm (AAA) in our patient was an example of an important co‐morbidity which could have a significant impact on the outcome of TAVI, if not managed appropriately. The increased systolic pressure post successful TAVI will result in an increased strain within the wall of the saccular AAA with an increased risk of rupture. Therefore, a timely management strategy for the AAA was necessary. We believe that we report the first case of simultaneous TAVI and endovascular aneurysm repair (EVAR). The patient underwent uncomplicated transfemoral TAVI immediately followed by successful drive‐by percutaneous EVAR delivered over the same superstiff guidewire via the transfemoral route. Our case highlights the importance of a detailed assessment in all patients before consideration for TAVI, and the multi‐disciplinary team and a management strategy for both pathologies tailored to the patient. The case demonstrates the versatility of trans‐catheter techniques which has enabled the treatment of aortic stenosis and abdominal AAA in a single procedure. © 2012 Wiley Periodicals, Inc.  相似文献   

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