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Warsi T, Ramphul K, Dulay MS, Jeelani S, Verma R, Kumar N, Dhaliwal JS, Carver C, Sakthivel H, Gardezi SKM, Deshpande S, Sherif AA, Liu A, Ahmed R. Risk Factors of Acute Ischemic Stroke and Mortality Among Adults With Endocardial Fibroelastosis. Neurologist 2024; 29:317-322. [PMID: 39034931 DOI: 10.1097/nrl.0000000000000576] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 07/23/2024]
Abstract
OBJECTIVES Endocardial fibroelastosis (EFE) is a rare form of restrictive cardiomyopathy associated with high morbidity and mortality. The literature is sparse on information pertaining to risk stratification. Thus, we sought to highlight the risk factors of acute ischemic stroke (AIS) and mortality in adults with EFE. METHODS The National Inpatient Sample (NIS) database was queried from 2001 to 2020 using the International Classification of Diseases 9th Revision (ICD-9) and 10th Revision (ICD-10) codes for adult patients with EFE. Factors associated with AIS and mortality were identified. RESULTS In all, 18495 cases of EFE fit the inclusion criteria, of which 2370 (12.82%) had AIS. The mean ages for patients with and without AIS were 62.37 and 54.24, respectively. Multivariate regression suggested greater odds of AIS in patients with hypertension (aOR 2.329, P <0.01), dyslipidemia (aOR: 1.566, P <0.01), peripheral vascular disease (PVD) (aOR: 1.736, P <0.01), alcohol abuse (aOR: 1.817, P <0.01), age >60 y (aOR: 1.646, P <0.01), females (vs. males, aOR: 1.238, P <0.01), and smokers (aOR: 1.697, P <0.01). Patients with cirrhosis (aOR: 0.174, P <0.01), CKD (aOR: 0.369, P <0.01), COPD (aOR: 0.402, P <0.01), atrial fibrillation (aOR: 0.542, P <0.01) had lower odds of AIS. 3.1% of EFE patients with AIS died. Diabetes (aOR: 11.665, P <0.01) and COPD (aOR: 3.201, P =0.017) were associated with the greatest odds of all-cause mortality. Dyslipidemia (aOR: 0.387, P =0.010) and females (vs. males, aOR: 0.432, P =0.012) had reduced odds of all-cause mortality. CONCLUSION Several risk factors are associated with AIS in EFE, while diabetes, COPD, and being male are associated with mortality in EFE.
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Affiliation(s)
| | | | | | | | | | | | | | - Caleb Carver
- Gold Coast University Hospital, Gold Coast, Australia
| | | | | | - Saurabh Deshpande
- Sri Jayadeva Institute of Cardiovascular Science and Research, Bangalore, India
| | - Akil A Sherif
- Division of Cardiovascular Diseases, St Vincent Hospital, Worcester, MA
| | | | - Raheel Ahmed
- Royal Brompton Hospital, London, United Kingdom
- National Heart and Lung Institute, Imperial College London, UK
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Zampieri M, Di Filippo C, Zocchi C, Fico V, Golinelli C, Spaziani G, Calabri G, Bennati E, Girolami F, Marchi A, Passantino S, Porcedda G, Capponi G, Gozzini A, Olivotto I, Ragni L, Favilli S. Focus on Paediatric Restrictive Cardiomyopathy: Frequently Asked Questions. Diagnostics (Basel) 2023; 13:3666. [PMID: 38132249 PMCID: PMC10742619 DOI: 10.3390/diagnostics13243666] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/30/2023] [Revised: 11/21/2023] [Accepted: 11/28/2023] [Indexed: 12/23/2023] Open
Abstract
Restrictive cardiomyopathy (RCM) is characterized by restrictive ventricular pathophysiology determined by increased myocardial stiffness. While suspicion of RCM is initially raised by clinical evaluation and supported by electrocardiographic and echocardiographic findings, invasive hemodynamic evaluation is often required for diagnosis and management of patients during follow-up. RCM is commonly associated with a poor prognosis and a high incidence of heart failure, and PH is reported in paediatric patients with RCM. Currently, only a few therapies are available for specific RCM aetiologies. Early referral to centres for advanced heart failure treatment is often necessary. The aim of this review is to address questions frequently asked when facing paediatric patients with RCM, including issues related to aetiologies, clinical presentation, diagnostic process and prognosis.
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Affiliation(s)
- Mattia Zampieri
- Pediatric Cardiology, Meyer Children’s University Hospital IRCCS, 50134 Florence, Italy (S.F.)
- Cardiomyopathy Unit, Careggi University Hospital, 50134 Florence, Italy
| | - Chiara Di Filippo
- Local Health Unit, Outpatient Cardiology Clinic, 84131 Salerno, Italy
| | - Chiara Zocchi
- Cardiovascular Department, San Donato Hospital, 52100 Arezzo, Italy
| | - Vera Fico
- Pediatric Cardiology, Meyer Children’s University Hospital IRCCS, 50134 Florence, Italy (S.F.)
- Cardiomyopathy Unit, Careggi University Hospital, 50134 Florence, Italy
| | - Cristina Golinelli
- Pediatric Cardiology and Adult Congenital Heart Disease Program, Department of Cardio—Thoracic and Vascular Medicine, IRCCS Azienda Ospedaliero—Universitaria di Bologna, 40138 Bologna, Italy
| | - Gaia Spaziani
- Pediatric Cardiology, Meyer Children’s University Hospital IRCCS, 50134 Florence, Italy (S.F.)
| | - Giovanni Calabri
- Pediatric Cardiology, Meyer Children’s University Hospital IRCCS, 50134 Florence, Italy (S.F.)
| | - Elena Bennati
- Pediatric Cardiology, Meyer Children’s University Hospital IRCCS, 50134 Florence, Italy (S.F.)
| | - Francesca Girolami
- Pediatric Cardiology, Meyer Children’s University Hospital IRCCS, 50134 Florence, Italy (S.F.)
| | - Alberto Marchi
- Pediatric Cardiology, Meyer Children’s University Hospital IRCCS, 50134 Florence, Italy (S.F.)
- Cardiomyopathy Unit, Careggi University Hospital, 50134 Florence, Italy
| | - Silvia Passantino
- Pediatric Cardiology, Meyer Children’s University Hospital IRCCS, 50134 Florence, Italy (S.F.)
| | - Giulio Porcedda
- Pediatric Cardiology, Meyer Children’s University Hospital IRCCS, 50134 Florence, Italy (S.F.)
| | - Guglielmo Capponi
- Pediatric Cardiology, Meyer Children’s University Hospital IRCCS, 50134 Florence, Italy (S.F.)
| | - Alessia Gozzini
- Pediatric Cardiology, Meyer Children’s University Hospital IRCCS, 50134 Florence, Italy (S.F.)
| | - Iacopo Olivotto
- Pediatric Cardiology, Meyer Children’s University Hospital IRCCS, 50134 Florence, Italy (S.F.)
- Cardiomyopathy Unit, Careggi University Hospital, 50134 Florence, Italy
| | - Luca Ragni
- Pediatric Cardiology and Adult Congenital Heart Disease Program, Department of Cardio—Thoracic and Vascular Medicine, IRCCS Azienda Ospedaliero—Universitaria di Bologna, 40138 Bologna, Italy
| | - Silvia Favilli
- Pediatric Cardiology, Meyer Children’s University Hospital IRCCS, 50134 Florence, Italy (S.F.)
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Aldawsari KA, Alhuzaimi AN, Alotaibi MT, Albert-Brotons DC. Endocardial fibroelastosis in infants and young children: a state-of-the-art review. Heart Fail Rev 2023:10.1007/s10741-023-10319-0. [PMID: 37222928 DOI: 10.1007/s10741-023-10319-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 04/26/2023] [Indexed: 05/25/2023]
Abstract
Endocardial fibroelastosis (EFE) is a rare cardiac condition characterized by excessive endocardial thickening secondary to fibroelastic tissues that commonly present in infants and young children. Most of endocardial fibroelastosis cases are secondary forms, which occur in conjunction with other cardiac diseases. Endocardial fibroelastosis has been associated with poor prognosis and outcomes. In light of recent advancements in understanding pathophysiology, several new data have revealed compelling evidence that abnormal endothelial-to-mesenchymal transition is the root cause of endocardial fibroelastosis. This article aims to review the recent development in pathophysiology, diagnostic workup, and management, and to discuss possible differential diagnoses.
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Affiliation(s)
- Khalifah A Aldawsari
- Department of Pediatrics, Nicklaus Children's Hospital, Miami, FL, USA.
- Heart Center, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia.
| | - Abdullah N Alhuzaimi
- Heart Center, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia
- Department of Cardiac Sciences, King Fahad Cardiac Centre, College of Medicine, King Saud University, Riyadh, Saudi Arabia
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Xie YY, Li QL, Li XL, Yang F. Pediatric acute heart failure caused by endocardial fibroelastosis mimicking dilated cardiomyopathy: A case report. World J Clin Cases 2023; 11:1771-1781. [PMID: 36970005 PMCID: PMC10037286 DOI: 10.12998/wjcc.v11.i8.1771] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/10/2022] [Revised: 10/26/2022] [Accepted: 01/09/2023] [Indexed: 03/07/2023] Open
Abstract
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.
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Affiliation(s)
- Yao-Ying Xie
- College of Clinical Medicine, Inner Mongolia Minzu University, Tongliao 028000, Inner Mongolia Autonomous Region, China
- Department of Pediatric Medical Center, Soochow University, Affiliated Hulunbuir Hospital, Hulunbuir 021000, Inner Mongolia Autonomous Region, China
| | - Qiu-Li Li
- College of Medicine, Shantou University, Shantou 515063, Guangdong Province, China
| | - Xin-Le Li
- College of Clinical Medicine, Inner Mongolia Minzu University, Tongliao 028000, Inner Mongolia Autonomous Region, China
| | - Fan Yang
- Department of Pediatric Medical Center, Soochow University, Affiliated Hulunbuir Hospital, Hulunbuir 021000, Inner Mongolia Autonomous Region, China
- School of Medicine, Shihezi University, Shihezi 832000, Xinjiang Uygur Autonomous Region, China
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Xie YY, Li QL, Li XL, Yang F. Pediatric acute heart failure caused by endocardial fibroelastosis mimicking dilated cardiomyopathy: A case report. World J Clin Cases 2023; 11:1771-1781. [DOI: 10.12998/wjcc.v11.i8.1771 xie yy] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 09/30/2023] Open
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Sun LJ, Li Y, Qiao W, Yu JH, Ren WD. Incremental value of three-dimensional and contrast echocardiography in the evaluation of endocardial fibroelastosis and multiple cardiovascular thrombi: A case report. World J Clin Cases 2021; 9:3365-3371. [PMID: 34002146 PMCID: PMC8107911 DOI: 10.12998/wjcc.v9.i14.3365] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/06/2020] [Revised: 02/03/2021] [Accepted: 03/10/2021] [Indexed: 02/06/2023] Open
Abstract
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.
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Affiliation(s)
- Li-Juan Sun
- Department of Ultrasound, Shengjing Hospital of China Medical University, Shenyang 110004, Liaoning Province, China
- Department of Ultrasound, The First Hospital of Qinhuangdao, Qinhuangdao 066000, Hebei Province, China
| | - Ying Li
- Department of Ultrasound, Shengjing Hospital of China Medical University, Shenyang 110004, Liaoning Province, China
| | - Wei Qiao
- Department of Ultrasound, Shengjing Hospital of China Medical University, Shenyang 110004, Liaoning Province, China
| | - Jia-Hui Yu
- Department of Ultrasound, Shengjing Hospital of China Medical University, Shenyang 110004, Liaoning Province, China
| | - Wei-Dong Ren
- Department of Ultrasound, Shengjing Hospital of China Medical University, Shenyang 110004, Liaoning Province, China
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