Endocardial Fibroelastosis as an Independent Predictor of Atrioventricular Valve Rupture in Maternal Autoimmune Antibody Exposed Fetus: A Systematic Review with Clinicopathologic Analysis
Abstract
:1. Introduction
2. Case Studies
2.1. Case Description
2.1.1. Case A
2.1.2. Case B
2.2. Postmortem Examination
2.2.1. Gross Morphology
2.2.2. Histopathology
3. Review of the Literature
3.1. Method
3.2. Search Strategy
3.3. Inclusion and Exclusion Criteria
3.4. Study Selection
3.5. Data Extraction
3.6. Quality Assessment
3.7. Data Analysis
4. Results
4.1. Study and Patient Demographics
4.2. Comorbidities and Course
4.3. Maternal Therapy
4.4. Age of Valve Rupture and Valves Involved
4.5. Postnatal Management
4.6. Histopathological Findings
5. Discussion
5.1. Causes of Valve Rupture
5.2. Timing of Valve Rupture
5.3. Physiology of Valve Rupture
5.4. Endocardial Fibroelastosis
5.5. Echocardiography Correlation
5.6. Histologic Correlation
5.7. Surgical Repair
5.8. Medical Management and Follow-up
6. Limitations
7. Conclusions
Supplementary Materials
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
Abbreviations
CHB | Complete heart block |
EFE | Endocardial fibroelastosis |
TV | Tricuspid valve |
TR | Tricuspid valve regurgitation |
MR | Mitral valve regurgitation |
MV | Mitral valve |
AV | Atrioventricular |
NL | Neonatal lupus |
RV | Right ventricle |
LV | Left ventricle |
RA | Right atrium |
LA | Left atrium |
IVIG | Intravenous immunoglobulin |
bpm | Beats per minute |
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Case | Reference Year of Publication | Maternal Age (Years) | Gravida | Prior Affected Pregnancy | Known Case of Auto Immune Disease | Anti-Ro/Anti La |
---|---|---|---|---|---|---|
1 | Case A | 35 | 2 | No | No | +/+ |
2 | Cuneo et al. 2011 [9] | 25 | 2 | No | Yes | +/− |
3 | Cuneo et al. 2009 [10] | 2 | Yes | Sjogren’s disease | +/− | |
4 | Weber et al. 1994 [17] | 31 | 2 | Yes | Sjogren’s disease | +/− |
5 | Shiraishi et al. 2014 [15] | - | - | |||
6 | Hamaoka et al. 2009 [13] | - | - | +/+ | ||
7 | Bellon et al. 2022 [8] | 26 | 1 | - | No | +/− |
8 | Tarca et al. 2017 [16] | 31 | 2 | 1 | Antiphospholipid syndrome | +/+ |
9 | Gonzalez-Lopez et al. 2017 [12] | 2 | - | No | +/+ | |
10 | Brooks et al. 2015 [14] | 1 | No | +/+ | ||
11 | Fleming et al. 2008 [11] | No | +/− | |||
12 | Cuneo et al. 2011 [9] | 27 | 2 | 0 | Yes | +/− |
Case | GA (Weeks) for Detection | Valve Involved | Maternal Therapy | Timing of Valve Rupture | GA (Weeks) Delivered | ||||||
---|---|---|---|---|---|---|---|---|---|---|---|
EFE | CHB | Effusions | Medication | Initiation | Effect | Antenatal | Perinatal | Postnatal | |||
1 | 21 | 21 | 21–29 | MV+TV | D+T | 24 | Pleural effusions resolved, reduction in pericardial effusion to mild | - | - | TV and MV—45 days | 32 + 4 |
2 | 19 | - | 19 | MV+TV | D+IVIG | 34 | No change | TV—34–36 weeks | - | MV—23 days | 36 |
3 | 19 | - | - | MV+TV | D+IVIG | 17 | Improvement in patchy echogenicity | - | - | TV and MV—7 weeks | 35 |
4 | 24 | 24 | - | MV+TV | P+T | 22 | Increased echogenicity along the AV valves without insufficiency, no hydrops fetalis | - | - | TV = 2 weeks MV—2.5 months | 35 |
5 | - | MV | - | - | - | - | - | 5 months | |||
6 | - | - | MV | - | - | - | - | - | 21 days | 40 | |
7 | 20 | - | 39 | TV | - | - | - | 39 weeks | - | - | 39 |
8 | 19 | - | - | TV | - | - | - | - | Immediately after delivery | - | 38 + 6 |
9 | 21 | 20 | - | TV | D | 21 | Worsening of TR, moderate RV dysfunction | - | Immediately after delivery | - | 34 |
10 | - | - | 36 | TV | - | - | - | 36 weeks | - | - | 36 |
11 | 20 | 20 | - | TV | D+T | 20 | - | - | 3 months | 34 | |
12 | 21 | 21 | - | TV | D+IVIG | 23 | No change | - | - | 6 months | 38 |
Case | Postnatal Therapy | Age of Rupture | Age of Surgery | Surgery | Outcome |
---|---|---|---|---|---|
1 | - | 45 days | - | Not performed | Died at 55 days of life |
2 | - | TV—34–36 weeks MV—23 days | - | Not performed | Died at 32 days |
3 | Tapering prednisolone over 4 weeks | 7 weeks | 7 weeks | Repair of mitral and TV chordae with gortex and autologous pericardium | 6 months old—diuretics, ACE inhibitors, first degree AV block, and incomplete RBBB |
4 | - | 2 weeks—TV after placing endocardial lead, MV—2.5 months | 2 weeks VVI, 24 h later TV repair and epicardial pacemaker; 2.5 months—MV replacement | 1. Anastomosis of the TV chordae to the papillary muscle and atrial septum was closed 2. MV replacement | Doing well |
5 | - | 5 months | - | - | |
6 | - | 21 days | 21 days | Annuloplasty and anastomosis of chordae with autologous pericardium | 2 year—1st degree heat block |
7 | - | Antenatal | Neonate | Direct attachment of the chordae to neo-papillary muscle and TV annuloplasty | Well at 8 years |
8 | - | Delivery | 11 months | Neochordae with gortex attached to septum and annuloplasty | Well at 15 months |
9 | Hydrocortisone, IVIG, captopril, sildenafil | Delivery | 2 months | Polytetrafluoroethylene neochordae, No annuloplasty | 20 months |
10 | - | 36 weeks | 6 days | TV repair | Normal at 2 years |
11 | - | 3 months | Permanent pacemaker: 3 days, BPV: 3 months, TV repair at 3 months | Repair of anterior tricuspid leaflet with PTFE cords, closure of ASD, dilatation of pulmonary valve | Well |
12 | Prednisolone for 2 months, IVIG 2 g/kg | 6 months | 6 months | Repair with Gore-Tex chordae | Well at 3 years |
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Kotecha, M.K.; Merchant, K.; Chan, C.J.; Choo, J.T.L.; Gopagondanahalli, K.R.; Zhang, D.Z.; Tan, T.H.; Sundararaghavan, S. Endocardial Fibroelastosis as an Independent Predictor of Atrioventricular Valve Rupture in Maternal Autoimmune Antibody Exposed Fetus: A Systematic Review with Clinicopathologic Analysis. Diagnostics 2023, 13, 1481. https://doi.org/10.3390/diagnostics13081481
Kotecha MK, Merchant K, Chan CJ, Choo JTL, Gopagondanahalli KR, Zhang DZ, Tan TH, Sundararaghavan S. Endocardial Fibroelastosis as an Independent Predictor of Atrioventricular Valve Rupture in Maternal Autoimmune Antibody Exposed Fetus: A Systematic Review with Clinicopathologic Analysis. Diagnostics. 2023; 13(8):1481. https://doi.org/10.3390/diagnostics13081481
Chicago/Turabian StyleKotecha, Monika Kantilal, Khurshid Merchant, Charmaine Jiahui Chan, Jonathan Tze Liang Choo, Krishna Revanna Gopagondanahalli, Dyan Zhewei Zhang, Teng Hong Tan, and Sreekanthan Sundararaghavan. 2023. "Endocardial Fibroelastosis as an Independent Predictor of Atrioventricular Valve Rupture in Maternal Autoimmune Antibody Exposed Fetus: A Systematic Review with Clinicopathologic Analysis" Diagnostics 13, no. 8: 1481. https://doi.org/10.3390/diagnostics13081481
APA StyleKotecha, M. K., Merchant, K., Chan, C. J., Choo, J. T. L., Gopagondanahalli, K. R., Zhang, D. Z., Tan, T. H., & Sundararaghavan, S. (2023). Endocardial Fibroelastosis as an Independent Predictor of Atrioventricular Valve Rupture in Maternal Autoimmune Antibody Exposed Fetus: A Systematic Review with Clinicopathologic Analysis. Diagnostics, 13(8), 1481. https://doi.org/10.3390/diagnostics13081481