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Article ; Online: Ebstein Anomaly: More Than Just a Tricuspid Valve Issue.

Valente, Anne M / Harrild, David M / Beroukhim, Rebecca S

Journal of the American College of Cardiology

2023 Volume 82, Issue 6, Page(s) 514–516

MeSH term(s)	Humans ; Ebstein Anomaly/diagnostic imaging ; Tricuspid Valve/diagnostic imaging ; Tricuspid Valve/surgery ; Cardiac Surgical Procedures ; Tricuspid Valve Insufficiency/diagnostic imaging ; Tricuspid Valve Insufficiency/surgery
Language	English
Publishing date	2023-04-04
Publishing country	United States
Document type	Editorial ; Comment
ZDB-ID	605507-2
ISSN	1558-3597 ; 0735-1097
ISSN (online)	1558-3597
ISSN	0735-1097
DOI	10.1016/j.jacc.2023.05.041
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Zs.A 1846: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (1.OG) ab Jg. 2022: Lesesaal (EG)
Zs.MO 196: Show issues

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Article ; Online: Morphology, Clinical Associations, and Pathologic Follow-up of Quadricuspid Aortic Valves in Children.

Ajayi, Omotola / Alizadeh, Faraz / Sekhavat, Sepehr / Bonello, Kristin / Beroukhim, Rebecca S / Ghelani, Sunil J

Pediatric cardiology

2024

Abstract: While quadricuspid morphology is commonly observed in truncal valves, quadricuspid aortic valves (QAV) are rare and their natural history is not well described. This retrospective study of 37 patients describes the diagnostic associations and morphologic ...

Abstract	While quadricuspid morphology is commonly observed in truncal valves, quadricuspid aortic valves (QAV) are rare and their natural history is not well described. This retrospective study of 37 patients describes the diagnostic associations and morphologic variability of QAVs in children (median age at diagnosis 4.3 y IQR 0-12 y). Associated congenital heart diseases were present in 54% (most commonly tetralogy of Fallot (TOF) and valvar pulmonary stenosis). Among patients with isolated QAV, 11 had genetic syndrome and 5 had skeletal anomalies. Valve morphology was most commonly type B (41%) and A (35%; Hurvitz and Roberts). Dilated aortic root (Z ≥ 2) was present in 5 and dilated ascending aorta in 9 patients at diagnosis. All patients with type C (n = 3) and G (n = 1) had aortic dilation. At diagnosis, >mild AR was rare (n = 1), mild regurgitation was common (n = 12, 32%), >mild AS was rare (n = 2), and mild AS was uncommon (n = 4). Over a median follow-up of 3.3y (IQR 0.9-11y), progression of AR was seen in 2 patients and progression of aortic root or ascending aorta dilation (increase in Z score by ≥ 2) was seen in 5 patients. In conclusion, QAV is a rare congenital anomaly and about half of the cases are found in hearts that are otherwise structurally normal. A relatively high prevalence is seen in patients with TOF, pulmonary stenosis, skeletal deformities, and genetic syndromes. Meticulous evaluation of aortic valve morphology should be conducted on echocardiograms performed for these indications.
Language	English
Publishing date	2024-03-13
Publishing country	United States
Document type	Journal Article
ZDB-ID	800857-7
ISSN	1432-1971 ; 0172-0643
ISSN (online)	1432-1971
ISSN	0172-0643
DOI	10.1007/s00246-024-03471-3
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Zs.A 1528: Show issues

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Article ; Online: Rare Cardiac Inflammatory Pseudotumor in a Toddler: Complementary Roles of Cardiac Magnetic Resonance and Positron Emission Tomography.

Mejia-Bautista, Melissa / Romanowicz, Jennifer / Hollowell, Monica / Geva, Tal / Carreon, Chrystalle Katte / Beroukhim, Rebecca S

Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology

2024 , Page(s) 107639

Abstract: We present a rare pediatric case of cardiac inflammatory pseudotumor (IPT) with a unique presentation of fever of unknown origin with markedly elevated inflammatory markers. A right atrial mass was discovered incidentally by echocardiography. The cardiac ...

Abstract	We present a rare pediatric case of cardiac inflammatory pseudotumor (IPT) with a unique presentation of fever of unknown origin with markedly elevated inflammatory markers. A right atrial mass was discovered incidentally by echocardiography. The cardiac magnetic resonance (CMR) signal characteristics and mass location were not consistent with any of the common benign cardiac tumors of childhood. The presence of high signal intensity on T2 imaging and late gadolinium enhancement, in conjunction with intense metabolic activity at the mass site on positron emission tomography (PET), raised the possibility of an inflammatory or malignant mass. The diagnosis of IPT was confirmed by biopsy. Our case highlights the utility of PET imaging to confirm the inflammatory nature and extent of an IPT.
Language	English
Publishing date	2024-04-01
Publishing country	United States
Document type	Case Reports
ZDB-ID	1134600-0
ISSN	1879-1336 ; 1054-8807
ISSN (online)	1879-1336
ISSN	1054-8807
DOI	10.1016/j.carpath.2024.107639
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Zs.A 3572: Show issues

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Article ; Online: Children at Risk: Multisystem Inflammatory Syndrome and COVID-19.

Beroukhim, Rebecca S / Friedman, Kevin G

JACC. Case reports

2020 Volume 2, Issue 9, Page(s) 1271–1274

Keywords	covid19
Language	English
Publishing date	2020-06-16
Publishing country	Netherlands
Document type	Editorial ; Comment
ISSN	2666-0849
ISSN (online)	2666-0849
DOI	10.1016/j.jaccas.2020.06.016
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Article: Children at risk: multisystem inflammatory syndrome and COVID-19

Beroukhim, Rebecca S / Friedman, Kevin

Keywords	covid19
Publisher	WHO
Document type	Article
Note	WHO #Covidence: #599458
Database	COVID19

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Article: Prognostic Significance of Computed Tomography Findings in Pulmonary Vein Stenosis.

Sena, Laureen / Callahan, Ryan / Sleeper, Lynn A / Beroukhim, Rebecca S

Children (Basel, Switzerland)

2021 Volume 8, Issue 5

Abstract: 1) Pulmonary vein stenosis (PVS) can be a severe, progressive disease with lung involvement. We aimed to characterize findings by computed tomography (CT) and identify factors associated with death; (2) Veins and lung segments were classified into five ... ...

Abstract	(1) Pulmonary vein stenosis (PVS) can be a severe, progressive disease with lung involvement. We aimed to characterize findings by computed tomography (CT) and identify factors associated with death; (2) Veins and lung segments were classified into five locations: right upper, middle, and lower; and left upper and lower. Severity of vein stenosis (0-4 = no disease-atresia) and lung segments (0-3 = unaffected-severe) were scored. A PVS severity score (sum of all veins + 2 if bilateral disease; maximum = 22) and a total lung severity score (sum of all lung segments; maximum = 15) were reported; (3) Of 43 CT examinations (median age 21 months), 63% had bilateral disease. There was 30% mortality by 4 years after CT. Individual-vein PVS severity was associated with its corresponding lung segment severity (
Language	English
Publishing date	2021-05-17
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2732685-8
ISSN	2227-9067
ISSN	2227-9067
DOI	10.3390/children8050402
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Article ; Online: Mitral valve orifice area predicts outcome after biventricular repair in patients with hypoplastic left ventricles.

Liddle, David / Gearhart, Addison / Sleeper, Lynn A / Lu, Minmin / Feins, Eric / Schidlow, David N / Ghelani, Sunil / Powell, Andrew J / Emani, Sitaram / Beroukhim, Rebecca S

Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance

2024 Volume 26, Issue 1, Page(s) 101029

Abstract: Background: Identification of risk factors for biventricular (BiV) repair in children with hypoplastic left ventricles (HLV) has been challenging. We sought to identify preoperative cardiovascular magnetic resonance (CMR) predictors of outcome in ... ...

Abstract	Background: Identification of risk factors for biventricular (BiV) repair in children with hypoplastic left ventricles (HLV) has been challenging. We sought to identify preoperative cardiovascular magnetic resonance (CMR) predictors of outcome in patients with HLVs who underwent BiV repair, with a focus on the mitral valve (MV). Methods: Single-center retrospective analysis of preoperative CMRs on patients with HLV (≤50 mL/m Results: Median follow-up was 0.7 (interquartile range 0.1, 2.2) years. Of 122 patients [59 atrioventricular canal (AVC) and 63 non-AVC] age 3 ± 2.8 years at the time of BiV repair, freedom from the primary outcome at 2 years was 53% for AVC and 69% for non-AVC (log rank p = 0.12), and freedom from the secondary outcome at 2 years was 49% for AVC and 79% for non-AVC (log rank p < 0.01). Independent predictors of primary outcome for AVC patients included MV orifice area z-score <-2 and transitional AVC; for non-AVC patients, predictors included MV orifice area z-score <-2, abnormal MV anatomy, and conal-septal ventricular septal defect. Independent predictors of secondary outcome for AVC patients included older age at surgery, transitional AVC, and transposition of the great arteries. Conclusion: In children with HLV, low MV orifice area and pre-existing MV pathology are risk factors for adverse outcome after BiV repair.
Language	English
Publishing date	2024-02-23
Publishing country	England
Document type	Journal Article
ZDB-ID	1458034-2
ISSN	1532-429X ; 1097-6647
ISSN (online)	1532-429X
ISSN	1097-6647
DOI	10.1016/j.jocmr.2024.101029
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Zs.A 5045: Show issues

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Je nach Verfügbarkeit (siehe Angabe bei Bestand)
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Jg. 1995 - 2021: Lesesall (2.OG)
ab Jg. 2022: Lesesaal (EG)

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Article ; Online: Computed tomography angiography (CTA) of anomalous aortic origin of a coronary artery (AAOCA): Which measurements are accurate and reliable?

Ferraro, Alessandra M / Uslenghi, Alessandro / Lu, Minmin / Newburger, Jane W / Nathan, Meena / Quinonez, Luis G / Beroukhim, Rebecca S

Journal of cardiovascular computed tomography

2023 Volume 17, Issue 2, Page(s) 130–137

Abstract: Background: The variation and accuracy of computed tomography angiography (CTA) features of anomalous aortic origin of a coronary artery (AAOCA) have not been completely characterized. We evaluated anomalous right (AAORCA) and left (AAOLCA) coronary ... ...

Abstract	Background: The variation and accuracy of computed tomography angiography (CTA) features of anomalous aortic origin of a coronary artery (AAOCA) have not been completely characterized. We evaluated anomalous right (AAORCA) and left (AAOLCA) coronary arteries by CTA, with an emphasis on reproducibility, comparison with surgical measurements, and effect of nitroglycerin. Methods: CTAs were interpreted for location of coronary origin & exit from the aorta, course, and dominance; minor & major diameters of the proximal & distal coronaries; shape of orifice; and intramural length. Relationships between vessel measurements, body surface area (BSA), and nitroglycerin use were evaluated. Comparisons between CTA and surgical measurements included intramural length, surgical probe size pre-intervention vs. CTA proximal AAOCA diameter, and surgical probe size post-intervention vs. CTA distal AAOCA diameter. Results: Of 104 patients [81 (78%) AAORCA], all but 1 were intramural. Compared to AAOLCA, AAORCA patients were more likely to have a high origin (91% vs. 31%, p < 0.01), and slit-like orifice (58% vs. 26%, p < 0.01). When CTAs with nitroglycerin were compared to those without, no difference in proximal AAOCA dimensions was identified; however distal AAOCA and normal-origin coronary dimensions were larger in scans with nitroglycerin. Aside from slit-like orifice, reliability of coronary CTA measurements, including proximal AAOCA minor diameter and intramural length, was moderate to good between readers. In a subgroup of 54 patients, proximal AAOCA minor diameter and intramural length had good agreement and correlation with surgery. Conclusion: Proximal AAOCA dimensions and intramural length are reproducible variables. The lack of difference in proximal AAOCA dimensions with nitroglycerin may reflect abnormal vessel mechanics. Multicenter studies are an important next step in understanding the generalizability of our findings.
MeSH term(s)	Humans ; Coronary Vessels/diagnostic imaging ; Coronary Vessels/surgery ; Computed Tomography Angiography/methods ; Reproducibility of Results ; Nitroglycerin ; Retrospective Studies ; Predictive Value of Tests ; Aorta ; Coronary Vessel Anomalies/diagnostic imaging ; Coronary Vessel Anomalies/surgery ; Coronary Angiography/methods
Chemical Substances	Nitroglycerin (G59M7S0WS3)
Language	English
Publishing date	2023-02-16
Publishing country	United States
Document type	Journal Article
ZDB-ID	2394360-9
ISSN	1876-861X ; 1934-5925
ISSN (online)	1876-861X
ISSN	1934-5925
DOI	10.1016/j.jcct.2023.02.003
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Zs.A 6761: Show issues

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Article ; Online: 2 Cases of Spontaneous Coronary Artery Dissection in Neonates.

Bassi, Sunakshi / Gearhart, Addison / Sanders, Stephen P / Carreon, Chrystalle Katte / Quinn, Brian / VanderPluym, Christina / Beroukhim, Rebecca S

JACC. Case reports

2022 Volume 6, Page(s) 101704

Abstract: Spontaneous coronary artery dissection in infants is a rare phenomenon. We present 2 neonates with severe ventricular dysfunction due to coronary artery dissection. Neither patient had evidence of extracardiac fibromuscular dysplasia or other ... ...

Abstract	Spontaneous coronary artery dissection in infants is a rare phenomenon. We present 2 neonates with severe ventricular dysfunction due to coronary artery dissection. Neither patient had evidence of extracardiac fibromuscular dysplasia or other comorbidities that would explain the presentation. (
Language	English
Publishing date	2022-12-08
Publishing country	Netherlands
Document type	Case Reports
ISSN	2666-0849
ISSN (online)	2666-0849
DOI	10.1016/j.jaccas.2022.101704
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Article ; Online: One and One-Half Ventricle Repair: Role for Restricting Antegrade Pulmonary Blood Flow.

Prasanna, Anagha / Tan, Corinne W / Anastasopulos, Alexandra / Beroukhim, Rebecca S / Emani, Sitaram M

The Annals of thoracic surgery

2021 Volume 114, Issue 1, Page(s) 176–183

Abstract: Background: In patients with hypoplastic subpulmonary ventricles, the one and one-half ventricle (1.5V) repair is an alternative to the Fontan procedure. However, in 1.5V-treated patients with pulsatile pulmonary blood flow, superior vena cava (SVC) ... ...

Abstract	Background: In patients with hypoplastic subpulmonary ventricles, the one and one-half ventricle (1.5V) repair is an alternative to the Fontan procedure. However, in 1.5V-treated patients with pulsatile pulmonary blood flow, superior vena cava (SVC) hypertension or right atrial hypertension may develop. This study aimed to (1) describe patient outcomes after 1.5V repair and (2) determine whether pulmonary artery septation at 1.5V repair confers a lower risk of SVC or right atrial hypertension. Methods: This study retrospectively reviewed patients who underwent a 1.5V repair between 1989 and 2020. The primary outcome was transplant-free survival. Secondary outcomes were postoperative SVC hypertension (defined by mean Glenn pressures greater than 17 mm Hg, SVC flow reversal or pulsatility, venovenous collateral vessels, or SVC syndrome) and right atrial hypertension (defined as mean right atrial pressures greater than 10 mm Hg with inferior vena cava and hepatic vein dilation or flow reversal). Results: A total of 74 patients underwent 1.5V repair at a median age of 29.6 months (interquartile range, 8.9 to 45.5 months). Median follow-up time was 39.9 months (interquartile range, 11.4 to 178.1 months). Transplant-free survival at 10 years was 92.4%. Among survivors, 12% (8 of 69) had right atrial hypertension and 39% (27 of 69) had SVC hypertension on follow-up. Survivors with unseptated pulmonary arteries had a greater risk of SVC hypertension compared with patients with septated pulmonary arteries (44% vs 10%; P = .04). No difference was found in right atrial hypertension between the 2 groups. Conclusions: Patients with 1.5V repair avoid Fontan-associated complications with favorable transplant-free survival. However, SVC hypertension remains a significant long-term complication. Pulmonary artery septation at 1.5V repair may reduce the risk of SVC hypertension.
MeSH term(s)	Child, Preschool ; Fontan Procedure/methods ; Humans ; Hypertension/complications ; Infant ; Postoperative Complications/epidemiology ; Postoperative Complications/etiology ; Pulmonary Circulation ; Pulmonary Veins/surgery ; Retrospective Studies ; Vena Cava, Superior/surgery
Language	English
Publishing date	2021-05-05
Publishing country	Netherlands
Document type	Journal Article
ZDB-ID	211007-6
ISSN	1552-6259 ; 0003-4975
ISSN (online)	1552-6259
ISSN	0003-4975
DOI	10.1016/j.athoracsur.2021.04.058
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Zs.A 252: Show issues

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