Pediatric acute heart failure caused by endocardial fibroelastosis mimicking dilated cardiomyopathy:A case report

BACKGROUND Endocardial fibroelastosis(EFE)is a diffuse endocardial collagen and elastin hyperplasia disease of unknown etiology,which may be accompanied by myocardial degenerative changes leading to acute or chronic heart failure.However,acute heart failure(AHF)without obvious associated triggers is rare.Prior to the report of endomyocardial biopsy,the diagnosis and treatment of EFE are highly susceptible to being confounded with other primary cardiomyopathies.Here,we report a case of pediatric AHF caused by EFE mimicking dilated cardiomyopathy(DCM),with the aim of providing a valuable reference for clinicians to early identify and diagnose EFE-induced AHF.CASE SUMMARY A 13-mo-old female child was admitted to hospital with retching.Chest X-ray demonstrated enhanced texture in both lungs and an enlarged heart shadow.Color doppler echocardiography showed an enlarged left heart with ventricular wall hypokinesis and decreased left heart function.Abdominal color ultrasonography revealed a markedly enlarged liver.Pending the result of the endomyocardial biopsy report,the child was treated with a variety of resuscitative measures including nasal cannula for oxygen,intramuscular sedation with chlorpromazine and promethazine,cedilanid for cardiac contractility enhancement,and diuretic treatment with furosemide.Subsequently,the child’s endomyocardial biopsy report result was confirmed as EFE.After the above early interventions,the child’s condition gradually stabilized and improved.One week later,the child was discharged.During a 9-mo follow-up period,the child took intermittent low-dose oral digoxin with no signs of recurrence or exacerbation of the heart failure.CONCLUSION Our report suggests that EFE-induced pediatric AHF may present in children over 1 year of age without any apparent precipitants,and that the associated clinical presentations are grossly similar to that of pediatric DCM.Nonetheless,it is still possible to be diagnosed effectively on the basis of the comprehensive analysis of auxiliary inspection findings before the result of the endomyocardial biopsy is reported.

Caution in the use of sedation and endomyocardial biopsy for the management of pediatric acute heart failure caused by endocardial fibroelastosis

Endocardial fibroelastosis(EFE)is commonly considered to be an inflammatory reactive lesion of hyperplasia and deposition of tissue fibers and collagen in the endocardium and/or subendocardium,which is strongly associated with endocardial sclerosis,ventricular remodeling and acute and chronic heart failure,and is one of the important causes for pediatric heart transplantation.Early diagnosis and treatment are the key factors in determining the prognosis of the children.In this paper,we would like to highlight the potential unintended consequences of the use of sedation and biopsy for pediatric acute heart failure caused by EFE and the comprehensive considerations prior to clinical diagnosis.

Incremental value of three-dimensional and contrast echocardiography in the evaluation of endocardial fibroelastosis and multiple cardiovascular thrombi: A case report

BACKGROUND Endocardial fibroelastosis(EFE)is a rare heart disease characterized by thickening of the endocardium caused by massive proliferation of collagenous and elastic tissue,usually leading to impaired cardiac function.Multimodality cardiovascular imaging for the evaluation of EFE with thrombi is even rarer.CASE SUMMARY We report a rare case of EFE associated with multiple cardiovascular thrombi.Three-dimensional(3D)and contrast echocardiography(CE)were used to assess ventricular thrombi.Anticoagulant therapy was administered to eliminate the thrombi.The peripheral contrast-enhanced thrombi with the highest risk were dissolved with anticoagulant therapy at the time of reexamination,which was consistent with the presumption of fresh loose thrombi.CONCLUSION This new echocardiography technique has a great advantage in the diagnosis and treatment of EFE.On the basis of conventional echocardiography,3D echocardiography is used to display the position,shape,and narrow base of the thrombus.CE does not only help to confirm the diagnosis of thrombus,but also determines its risk.

Unilateral pleuroparenchymal fibroelastosis as a rare form of idiopathic interstitial pneumonia:A case report

BACKGROUND Pleuroparenchymal fibroelastosis(PPFE)is a rare idiopathic interstitial pneumonia characterized by predominantly upper lobe involvement with pleural fibrosis and subjacent parenchymal fibrosis.Recently,there have been increasing reports of PPFE,and PPFE might coexist with other interstitial lung diseases in the lower lobe and upper lobe.However,cases of unilateral PPFE are scarce.CASE SUMMARY A 75-year-old Korean male presented to our hospital with chronic dry cough and exertional dyspnea.The patient’s symptoms started 6 mo previously and had been gradually worsening.At the time of presentation,he felt dyspnea when walking at his own pace.Radiologic findings suggested PPFE,but the lesion was localized in the upper lobe of the right lung.After multidisciplinary discussion,a transbronchial lung biopsy in the right upper lobe revealed collapsed alveoli with parenchymal fibroelastosis,and elastic van Gieson staining demonstrated septal elastosis with intra-alveolar collagenosis,which met the histopathologic criteria of definite PPFE.After multidisciplinary discussion in an experienced interstitial lung disease center,we confirmed the diagnosis of unilateral PPFE.Furthermore,we confirmed the progression of PPFE on radiologic findings during the followup period.CONCLUSION Clinicians should consider PPFE,even in cases with unilateral,predominantly upper lung involvement in interstitial lung disease patients through multidisciplinary discussion.

Fetal Endocardial Fibroelastosis with Coarctation of Aortic Arch: A Case Report

This study describes a 34-year-old pregnant woman who underwent prenatal echocardiography at 24 weeks of gestation and found fetal heart abnormalities. She underwent fetal echocardiography in our hospital. Echocardiography showed complex malformation of fetal heart, thickening and calcification of left ventricular myocardium and tendon, weakening or even flattening of left ventricular wall movement, very severe mitral stenosis, severe mitral regurgitation, aortic stenosis, narrowing of the aortic arch, countercurrent of the ductal arch to aortic arch, small diameter of the oval foramen and significantly increased flow velocity. The patient decided to induce labor after expert consultation, but no autopsy was performed due to his family’s refusal. Color Doppler ultrasound can find the positive signs of endocardial elastofibroplasia earlier, and accurately diagnose patients with neonatal elastofibroplasia. It provides accurate diagnostic information for the clinic and can be used as the first choice. This report has obtained the informed consent of its parents.