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1.
Organ Transplantation ; (6): 192-2019.
Article in Chinese | WPRIM | ID: wpr-780514

ABSTRACT

Objective To explore the treatment strategies of pleuroparenchymal fibroelastosis (PPFE). Methods A 22-year-old male patient was complicated with PPFE after receiving chemotherapy in combination with stem cell transplantation for lymphoma. He underwent thoracoscopic left lung tongue wedge resection, bilateral pleurodesis followed by allogeneic left lung transplantation. Literature review was performed to analyze the etiology, pathogenesis, imaging features, pathological features and treatment of PPFE. Results The PPFE patient required the non-invasive ventilator for 24 h before lung transplantation. After lung transplantation, the shortness of breath and respiratory failure were cured and the quality of life was significantly improved. No eligible studies was found in the domestic database, and 26 literatures published in English were found in the international databases. Among them, 9 literatures (case reports) were finally included after screening. PPFE could be divided into the primary and secondary categories according to the etiology. The clinical manifestations of PPFE mainly included dry cough, dyspnea on exertion, chest pain, repeated pneumothorax and body weight loss. Chest CT scan demonstrated irregular thickening of the pleura in bilateral upper lungs. Pathological manifestations consisted of evident thickening of the visceral pleura, fibroelastosis and arrangement disorder in the pleura and the underlying pulmonary interstitium. PPFE could progress rapidly. Adrenocortical hormone and other immunosuppressive agents yielded low clinical efficacy and poor clinical prognosis. Lung transplantation was a necessary treatment for PPFE. Conclusions PPFE cannot be effectively treated by conservative therapy. It is recommended to deliver lung transplantation as early as possible.

2.
Article in Chinese | WPRIM | ID: wpr-694124

ABSTRACT

Objective To study the clinical efficacy of glucocorticoid in treatment of primary endocardial fibroelastosis (EFE) in children.Methods Fifty-eight primary EFE cases admitted in the Children's Hospital of Chongqing Medical University from Jan.2006 to Dec.2013 were treated and followed-up for more than 3 years.Based on whether the application of glucocorticoid and the length of time of glucocorticoid therapy,cases were divided into three groups:no glucocorticoid group (n=15),glucocorticoid 0.5-1 year group (n=17) and glucocorticoid 1-2 year group (n=26).The following indicators were followed up and observed:the cardiac function indexes before and after treatment [Modified ROSS score,left ventricular ejection fraction (LVEF),left ventricular fractional shortening (LVFS)],cardiac size [cardiothoracic ratio (C/T)] and the proportion of death.The efficacy and safety of glucocorticoid were studied.Results No statistically significant difference of modified ROSS scores,LVEF,LVFS and C/T existed among the 3 groups before treatment (P>0.05).After treatment,the modified ROSS score,LVEF,LVFS and C/T in glucocorticoid 0.5-1 year group were 2.06 ± 1.78,59.29 ± 8.34,31.24 ± 6.0 and 0.580 ± 0.055,respectively,which were significantly different from those before treatment (5.06 ± 1.92,38.76 ± 6.31,18.47 ± 3.14 and 0.67 ± 0.05,respectively,P<0.05);the modified ROSS score,LVEF LVFS and C/T in glucocorticoid 1-2 years group were 1.28 ± 1.54,64.64 ± 9.08,35.44 ± 5.44 and 0.57 ± 0.06,which were significantly different from those before treatment (5.28 ± 2.26,37.88 ± 8.48,18.12 ± 4.99 and 0.67 ± 0.04,P<0.05).After treatment,the modified ROSS score,LVEF,LVFS and C/T in no glucocorticoid group were 6.00 ± 1.48,42.91 ± 14.36,21.55 ± 8.63 and 0.63 ± 0.05,which were significantly different compared with those in glucocorticoid 0.5-1 year group and glucocorticoid 1-2 years group (P<0.05).However,no significant difference existed between glucocorticoid 0.5-1 year group and 1-2 years group on the modified ROSS score,LVEF and C/T after treatment (P>0.05).Conclusion The glucocorticoid has good clinical effect on endocardial fibroelastosis,and no significant difference is found on the efficacy between the total course of glucocorticoid therapy 0.5-1 year group and 1-2 years group.

3.
Article in Chinese | WPRIM | ID: wpr-692599

ABSTRACT

Endocardial fibroelastosis(EFE),associated with diffuse endocardial thickening due to proliferation of fibrous and elastic tissue,is a rare cardiac disorder that mainly occurs in infants. Its etiology is unknown,viral infection,immunology and genetics have been reported having effects on the pathogenesis of EFE.However, its pathogenesis is not yet fully understood. Recently,with the improvement of genetic testing techniques,many studies have shown that a variety of genetic factors involved in the occurrence of EFE. The TAZ gene on the X chromosome,the Nexn gene on the autosome,the Nebulette gene,the CSRP3 gene,the Sarcomere protein gene,and endothelial to mesenchymal transition(EndMT)are confirmed to be involved in the pathogenesis.This provides a new basis for the early diagnosis and accurate treatment of primary endocardial fibroelastosis and helps to improve the prognosis of the disease. This article discussess the etiology of EFE,including viral infection,immunology and genetics,aiming at raising awareness of the pathogenesis of this disease.

4.
Medisan ; 17(9): 6003-6008, set. 2013.
Article in Spanish | LILACS-Express | LILACS | ID: lil-687243

ABSTRACT

Se presenta el caso clínico de una gestante de 39 años de edad, quien fuera ingresada a las 22 semanas de gravidez en el Hospital Ginecoobstétrico Docente "Tamara Bunke Bider" de Santiago de Cuba, luego de ser remitida del Centro Provincial de Genética por el diagnóstico ecográfico prenatal de fibroelastosis endocárdica, confirmado en el Cardiocentro de esta provincia. Después de la interrupción del embarazo, sugerida en la consulta de Genética, se realizó la autopsia al feto y con el estudio hístico del ventrículo izquierdo se corroboró la miocardiopatía.


The case report of a 39-year-old pregnant woman is presented, who was admitted at 22 weeks of pregnancy to "Tamara Bunke Bider" Teaching Gynecoobstetric Hospital of Santiago de Cuba, after being referred from the Provincial Center of Genetics due to prenatal ultrasound diagnosis of endocardial fibroelastosis, confirmed at the Heart Center of this province. After abortion, suggested at the Department of Genetics, autopsy was performed and the fetal left ventricle tissue study confirmed cardiomyopathy.

5.
Article in Chinese | WPRIM | ID: wpr-439424

ABSTRACT

Objective To investigate surgical treatment and effect of secondary endocardial fibroelastosis,based on respective analysis of clinical data and follow-up data of patients with secondary endocardial fibroelastosis (SEF) between 2010 and 2012.Methods A retrospective analysis was performed including 10 patients with secondary endocardial fibroelastosis from January 2010 to December 2012 in Wuhan Union Hospital.All patients were diagnosed by Untrasonic Cardiogram and/or CT angiography of heart and great vessel,and had cardiac insufficiency in different degree [EF 0.37 ± 0.08 (0.26 ~ 0.48)].All patients except 2 patients with anomalous origin of the coronary artery received treatment of digitaloid drugs before operation,which promoted preoperative cardiac function.5 patients with SEF complicated with Congenital Coarctation of the Aorta (CoA),2 patients underwent correction of CoA,2 patients underwent correction of CoA and partial resection of endocardium,1 patient underwent correction of CoA,partial resection of endocardium and mitral vavuloplasty.2 patients with SEF complicated with anomalous origin of the left coronary artery from the pulmonary artery,who were underwent correction of anomalous origin of coronary artery.2 patients with SEF complicated with aortic stenosis,who were underwent aortic commissurotomy and partial resection of endocardium.1 patient with SEF complicated with mitral stenosis and insufficiency,who underwent mitral valve replacement.The intraopertive gross appearance of endocardium was opaque greyish-white not transparent pink.The postoperative pathological examination showed obviously positive dyeing of elastic fibers.In 3,6,12 and 24 months after operation,Untrasonic Cardiogram evaluated cardiac function and endocardium.Results one 6 months patients with origin of left coronary from pulmonary artery died of severe post-operative low cardiac output syndrome,while another 1 months patients with origin of left coronary from pulmonary artery obtained post-operative good recovery,and the Untrasonic Cardiogram show disappearance of endocardial fibroelastosis.The post-operative mean time of using respirator(4.0 ± 1.5) days (2-7 days).Compared with the preoperative data,the cardiac function index (EF) was not significantly better at 2 weeks and 3-6 months[0.38 ± 0.07 (0.28 ~ 0.48),P > 0.05 ; 0.39 ± 0.08 (0.30 ~ 0.50),P > 0.05],and the non-resected fibroelatic endocardium still existed and were not attenuated.But the cardiac function index (EF) significantly increased [0.44 ± 0.08 (0.38 ~ 0.55),P < 0.05] than the pre-operative EF,and the 3 of 5 cases the fibroelatic endocardium were attenuated or disappeared,while 2 of 5 cases the fibroelatic endocardium still existed.Conclusion SEF is the important causes of the infant intractable heart failure,which has the characteristic of high mortality and limited therapy.For SEF patients with anomalous origin of the coronary artery,the SEF is completely reversed by early diagnosis and early correction of the malformation.For SEF patients with CoA or aortic stenosis,the surgical treatment could promote recovery of cardiac function,but whether the SEF were reversed is still subject to further follow-up.The heart transplantation is the best therapy for SEF with severse heart failure.

6.
Article in Chinese | WPRIM | ID: wpr-427116

ABSTRACT

Endocardial fibroelastosis(EFE) is a rare heart disease with a thickening of the endocardium by layers of collagenous and elastic fibres.Patients with EFE have diastolic and systolic heart failure.With the improvement of medicine,some progress has been achieved in EFE research.This article gave a comprehensive review to deepen the understanding of EFE.

7.
Medisan ; 15(9)sept. 2011. ilus
Article in Spanish | LILACS | ID: lil-616372

ABSTRACT

Se detalla el caso clínico de una gestante de 30 años (segundo trimestre del embarazo), residente en el poblado Siboney, quien asistió a la consulta de ecocardiografía fetal del Hospital General Docente Dr Juan Bruno Zayas Alfonso de Santiago de Cuba, donde se le diagnosticó una fibroelastosis cardíaca fetal a las 23 semanas y malformación renal. Fue remitida al Cardiocentro de esta ciudad y a la consulta de genética provincial, de manera que al confirmarse la miocardiopatía congénita en el feto, la pareja optó por interrumpir la gravidez. La necropsia reveló fibroelastosis endocárdica, estenosis aórtica moderada, hipoplasia ligera de la aorta ascendente y displasia renal bilateral.


The case report of a 30 year-old pregnant woman (second trimester) is detailed, who lives in Siboney neighborhood and attended the fetal ultrasonography department from Dr Juan Bruno Zayas Alfonso Teaching General Hospital of Santiago de Cuba, where she was diagnosed with fetal cardiac fibroelastosis at the 23 weeks and renal malformation. She was referred to the Cardiology Center of this city and to the provincial genetic department, and when congenital fetal myocardiopathy was confirmed the couple decided to terminate the pregnancy. The autopsy revealed endocardial fibroelastosis, moderate aortic stenosis, mild hypoplasia of the ascending aorta and bilateral renal dysplasia.


Subject(s)
Humans , Female , Pregnancy , Abortion, Induced , Cardiomyopathies , Congenital Abnormalities , Endocardial Fibroelastosis , Ultrasonography
8.
Article in Chinese | WPRIM | ID: wpr-384362

ABSTRACT

The clinical data of 76 children with EFE admitted from June, 1998 to December, 2009 was retrospectively analyzed. Tachypnea、 cough and cyanosis were common and all presented with heart failure. Electrocardiogram revealed sinus tachycardia 、left ventricular hyperthrophy and ST-T alterations. On chest radiograph 54 cases (54/76) had a cardiothoracic ratio > 0. 65 and 33 with pneumonia.Echocardiopraphy revealed lowered left ventricular systolic function in all cases and decreased left ventricular diastolic function in 21 (21/76). 63 cases (63/76) presented with thickened and enhanced left ventricular endocardium and 11 (11/76) with thickened interventricular septals. All were treated with digitalis. 58children were improved and discharged, 10 gave up therapy and 8 died during hospitalization. 41 out of 58 improved cases were followed for 1 - 4 years, with 2. 6 years on average. Among them 28 were clinically cured, 7 deteriorated and 6 died of heart failure. Echocardiography is of diagnostic value for ERE. Systolic and diastolic function of the heart can be used to predict the outcome. Pulmonary infection is a major risk factor associated with deterioration. Long-term treatment is imperative to improve the prognosis.

9.
Korean Circulation Journal ; : 204-207, 2010.
Article in English | WPRIM | ID: wpr-170713

ABSTRACT

Endocardial fibroelastosis (EFE) is characterized by deposition of collagen and elastin leading to ventricular hypertrophy and diffuse endocardial thickening. Here we report (for the first time in Korea) the case of a EFE presenting with heart failure. The patient was a 57-year-old woman who had complained of dyspnea on exertion {New York Heart Association (NYHA) functional class 3} and abdominal distension at the time of hospital admission. Echocardiography showed severe diastolic dysfunction with normal systolic function. On MRI, the contrast-enhanced delayed myocardial image demonstrated hyperenhancement in the endocardium. Owing to progressive heart failure, the patient was transplanted. Histological examination of the explanted heart showed irregularly thickened endocardium with fibrosis and elastosis in the both ventricles, compatible with the diagnosis of EFE.


Subject(s)
Cardiomyopathy, Restrictive , Collagen , Dyspnea , Echocardiography , Elastin , Endocardial Fibroelastosis , Endocardium , Female , Fibrosis , Heart , Heart Failure , Heart Transplantation , Humans , Hypertrophy , Middle Aged , Transplants
10.
Article in Chinese | WPRIM | ID: wpr-639279

ABSTRACT

Objective To observe the clinical effect and security of carvedilol on children with endocardial fibroelastosis(EFE).Methods Eighteen children with EFE treatment with carvedilol.The improvement of clinical symptom,heart rate,heart function,side effect and maximal tolerance dose after treatment with carvedilol were observed.Results The clinical symptom was obviously improved;eiection fraction(EF),fractional shortening(FS),mean velocity of circumferential fiber(Mvcf)were significantly increased,left ventricular end-systolic dimension(LVDS),left ventricular mass(LVmass),interventricular septal thickness(IVSs)were notably decreased after treatment with carvedilol.Conclusions The data indicates that carvedilol can significantly reduce left ventricular diastolic dimension(LVDD),IVSs,and LVmass,inhibit the remodeling of ventricle,significantly elevate the heart function of EFE.So carvedilol is benefit and security on children with EFE.

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