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Article ; Online: Fetal Cardiac Imaging for Congenital Heart Disease-Is Cardiac Magnetic Resonance Imaging the Future?

Arya, Bhawna

JAMA network open

2021 Volume 4, Issue 3, Page(s) e214617

MeSH term(s)	Female ; Heart Defects, Congenital/diagnostic imaging ; Humans ; Magnetic Resonance Imaging ; Magnetic Resonance Spectroscopy ; Pregnancy ; Prenatal Diagnosis
Language	English
Publishing date	2021-03-01
Publishing country	United States
Document type	Journal Article ; Comment
ISSN	2574-3805
ISSN (online)	2574-3805
DOI	10.1001/jamanetworkopen.2021.4617
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Article ; Online: Response to comment on "Utility of novel fetal echocardiographic morphometric measures of the aortic arch in the diagnosis of neonatal coarctation of the aorta".

Arya, Bhawna

Prenatal diagnosis

2018 Volume 38, Issue 10, Page(s) 797

MeSH term(s)	Aorta ; Aorta, Thoracic ; Aortic Coarctation ; Echocardiography ; Humans
Language	English
Publishing date	2018-10-15
Publishing country	England
Document type	Letter ; Comment
ZDB-ID	82031-3
ISSN	1097-0223 ; 0197-3851
ISSN (online)	1097-0223
ISSN	0197-3851
DOI	10.1002/pd.5293
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Zs.A 1625: Show issues

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Article ; Online: Coarctation of the aorta: Prenatal assessment, postnatal management and neonatal outcomes.

Arya, Bhawna / Maskatia, Shiraz A

Seminars in perinatology

2022 Volume 46, Issue 4, Page(s) 151584

Abstract: Coarctation of the aorta (Coa) is a potentially life threatening diagnosis. It occurs in 0.3 per 1000 live births and accounts for 6-8% of all infants with congenital heart defects. Neonates with severe Coa may be completely asymptomatic at birth, as the ...

Abstract	Coarctation of the aorta (Coa) is a potentially life threatening diagnosis. It occurs in 0.3 per 1000 live births and accounts for 6-8% of all infants with congenital heart defects. Neonates with severe Coa may be completely asymptomatic at birth, as the ductus arteriosus can provide flow to the lower body. Those who are not diagnosed prenatally may be diagnosed only after constriction of the ductus arteriosus, when they present in cardiogenic shock. This group has a higher risk for mortality and morbidity relative to those diagnosed prenatally. Despite the increasing practice of universal pulse oximetry screening, many cases with significant coarctation of the aorta still go undiagnosed in the newborn period. In this article, we present the pathophysiology, diagnosis, presentation, treatment and outcomes of Coa.
MeSH term(s)	Female ; Humans ; Infant ; Infant, Newborn ; Pregnancy ; Aorta ; Aortic Coarctation/diagnostic imaging ; Aortic Coarctation/physiopathology ; Aortic Coarctation/therapy ; Ductus Arteriosus/physiology ; Ductus Arteriosus, Patent/physiopathology ; Prenatal Diagnosis
Language	English
Publishing date	2022-03-12
Publishing country	United States
Document type	Journal Article
ZDB-ID	752403-1
ISSN	1558-075X ; 0146-0005
ISSN (online)	1558-075X
ISSN	0146-0005
DOI	10.1016/j.semperi.2022.151584
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Zs.A 1339: Show issues

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Article ; Online: Fetal Echocardiographic Evaluation of Tricuspid Valve and Right Ventricular Function Including Global Longitudinal Strain in Hypoplastic Left Heart Syndrome and Association with Postnatal Outcomes.

Ittleman, Benjamin / Lowenstein, Sarah / Edwards, Lindsay A / Caris, Elizabeth / Bhat, Aarti / Conwell, Jeffrey / Lewin, Mark / Arya, Bhawna

Pediatric cardiology

2024

Abstract: Despite significant advancements in the care of patients with hypoplastic left heart syndrome (HLHS) morbidity and mortality remain high. Postnatal right ventricular dysfunction and tricuspid regurgitation (TR) are associated with worse outcomes in HLHS. ...

Abstract	Despite significant advancements in the care of patients with hypoplastic left heart syndrome (HLHS) morbidity and mortality remain high. Postnatal right ventricular dysfunction and tricuspid regurgitation (TR) are associated with worse outcomes in HLHS. We aim to determine if right ventricle functional parameters and TR on fetal echocardiogram are associated with postnatal outcomes in HLHS patients. Retrospective review was performed on all fetuses with HLHS from 2014 to 2022 at our institution. Initial and follow up fetal echocardiogram measurements of right ventricular myocardial performance index (MPI), fractional area change (FAC) and global longitudinal strain (GLS) were retrospectively measured. The presence and severity of TR was recorded from the fetal echocardiogram reports. Postnatal outcomes including transplant-free survival, hospital length of stay > 30 days after initial palliation and need for bidirectional Glenn at < 4 months were reviewed. Forty-three subjects met inclusion criteria. Mean gestational age at presentation was 26.1 ± 5.9 weeks. Nine subjects died and 3 required heart transplantation. Initial fetal echocardiogram MPI was significantly lower (better) (0.36 ± 0.06 vs 0.44 ± 0.11; p = < 0.001) and FAC was significantly higher (better) (45 ± 6% vs 40 ± 8%; p = 0.035) in transplant-free survivors. Fetal right ventricular GLS and presence of mild TR were not associated with postnatal outcome. In fetuses with HLHS, abnormal MPI and right ventricular FAC are associated with decreased transplant-free survival. There was no observed association between GLS and postnatal outcomes. To our knowledge this is the first study examining fetal right ventricular function and GLS in HLHS patients and its link to postnatal outcomes.
Language	English
Publishing date	2024-04-22
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/s00246-024-03453-5
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Zs.A 1528: Show issues

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Article ; Online: Pathophysiology, adaptation, and imaging of the right ventricle in Fontan circulation.

Files, Matthew D / Arya, Bhawna

American journal of physiology. Heart and circulatory physiology

2018 Volume 315, Issue 6, Page(s) H1779–H1788

Abstract: The Fontan procedure, which creates a total cavopulmonary anastomosis and represents the final stage of palliation for hypoplastic left heart syndrome, generates a unique circulation relying on a functionally single right ventricle (RV). The RV pumps ... ...

Abstract	The Fontan procedure, which creates a total cavopulmonary anastomosis and represents the final stage of palliation for hypoplastic left heart syndrome, generates a unique circulation relying on a functionally single right ventricle (RV). The RV pumps blood in series around the systemic and pulmonary circulation, which requires adaptations to the abnormal volume and pressure loads. Here, we provide a complete review of RV adaptations as the RV assumes the role of the systemic ventricle, the progression of RV dysfunction to a distinct pattern of heart failure unique to this disease process, and the assessment and management strategies used to protect and rehabilitate the failing RV of Fontan circulation.
MeSH term(s)	Adaptation, Physiological ; Fontan Procedure/adverse effects ; Heart Ventricles/diagnostic imaging ; Heart Ventricles/physiopathology ; Heart Ventricles/surgery ; Hemodynamics ; Humans ; Postoperative Complications/diagnostic imaging ; Postoperative Complications/etiology ; Postoperative Complications/physiopathology
Language	English
Publishing date	2018-09-21
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	603838-4
ISSN	1522-1539 ; 0363-6135
ISSN (online)	1522-1539
ISSN	0363-6135
DOI	10.1152/ajpheart.00336.2018
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Article ; Online: Accuracy of Fetal Echocardiography in Defining Pulmonary Artery Anatomy and Source of Pulmonary Blood flow in Pulmonary Atresia with Ventricular Septal Defect (PA/VSD).

Naimi, Iman / Clouse, Michele / Arya, Bhawna / Conwell, Jeffrey A / Lewin, Mark B / Bhat, Aarti H

Pediatric cardiology

2021 Volume 42, Issue 5, Page(s) 1049–1057

Abstract: Precise delineation of central and branch pulmonary artery anatomy, patent ductus arteriosus, and major aorto-pulmonary collateral artery anatomy in the fetal diagnosis of pulmonary atresia with ventricular septal defect is challenging but important to ... ...

Abstract	Precise delineation of central and branch pulmonary artery anatomy, patent ductus arteriosus, and major aorto-pulmonary collateral artery anatomy in the fetal diagnosis of pulmonary atresia with ventricular septal defect is challenging but important to prenatal counseling and postnatal management. We aimed to evaluate the accuracy of fetal echocardiography to determine these anatomical nuances in pulmonary atresia with ventricular septal defect. This was a retrospective, single-institution, 10-year chart review of consecutive prenatal diagnosis of pulmonary atresia with ventricular septal defect for assessment of pulmonary artery, patent ductus arteriosus, and major aorto-pulmonary collateral artery anatomy and comparison with postnatal imaging including echocardiography, cardiac catheterization, and computerized tomography angiography. Twenty-six fetuses were diagnosed with pulmonary atresia with ventricular septal defect during the review period and complete postnatal follow-up was available in 18, all confirming the basic prenatal diagnosis. Fetal echocardiography accurately predicted central and branch pulmonary artery anatomy in 16 (89%) [confluent in 14, discontinuous in 2], patent ductus arteriosus status in 15 (83%) [present in 10, absent in 5], and major aorto-pulmonary collateral arteries in 17 (94%) [present in 9, absent in 8]. Accuracy increased to 100% for pulmonary artery anatomy (16/16) and major aorto-pulmonary collateral artery (17/17) when excluding patients whose anatomy was reported as uncertain on fetal echocardiography. Fetal echocardiography can provide accurate anatomical details in the vast majority of fetuses with pulmonary atresia with ventricular septal defect. This allows for more anatomy-specific counseling, prognostication, and improved selection of postnatally available management options.
MeSH term(s)	Echocardiography/standards ; Female ; Heart Septal Defects/diagnostic imaging ; Heart Septal Defects/embryology ; Heart Septal Defects/pathology ; Humans ; Male ; Pregnancy ; Prenatal Diagnosis/standards ; Pulmonary Artery/diagnostic imaging ; Pulmonary Artery/pathology ; Pulmonary Atresia/diagnostic imaging ; Pulmonary Atresia/embryology ; Pulmonary Atresia/pathology ; Pulmonary Circulation ; Retrospective Studies
Language	English
Publishing date	2021-03-08
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/s00246-021-02579-0
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Zs.A 1528: Show issues

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Article ; Online: Preoperative Physiology, Imaging, and Management of Transposition of the Great Arteries.

Files, Matthew D / Arya, Bhawna

Seminars in cardiothoracic and vascular anesthesia

2015 Volume 19, Issue 3, Page(s) 210–222

Abstract: Transposition of the great arteries (TGA) refers to hearts with concordant atrioventricular connections but discordant ventriculoarterial connections. In this lesion, the aorta arises from the right ventricle and the pulmonary artery arises from the left ...

Abstract	Transposition of the great arteries (TGA) refers to hearts with concordant atrioventricular connections but discordant ventriculoarterial connections. In this lesion, the aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle. As such, the pulmonary and aortic circulations run in parallel as opposed to in series, and this lesion is not compatible with survival without adequate mixing of these circulations. The management and outcomes of TGA parallels the field of pediatric cardiac surgery itself. Uniformly fatal in childhood, palliative procedures from the 1950s to 1970s offered survival, albeit at a high early and late cost. In the 1970s, the arterial switch operation (ASO) provided an anatomical "cure," with survival to adulthood in the current era of around 90%. Detailed perioperative imaging, attention to associated lesions, and comprehension of the physiology are critical to medical and surgical management.
MeSH term(s)	Cardiac Surgical Procedures/methods ; Child ; Humans ; Monitoring, Intraoperative/methods ; Preoperative Care/methods ; Survival ; Transposition of Great Vessels/physiopathology ; Transposition of Great Vessels/surgery
Language	English
Publishing date	2015-09
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	2012371-1
ISSN	1940-5596 ; 1089-2532
ISSN (online)	1940-5596
ISSN	1089-2532
DOI	10.1177/1089253215581851
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Zs.A 5308: Show issues

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Article ; Online: Utility of Screening Fetal Echocardiogram Following Normal Anatomy Ultrasound for In Vitro Fertilization Pregnancies.

Spurr, Rebecca R / Conwell, Jeffrey A / Young, Luciana T / Lewin, Mark B / Edwards, Lindsay A / Arya, Bhawna

Pediatric cardiology

2022 Volume 43, Issue 6, Page(s) 1349–1353

Abstract: In vitro fertilization (IVF) is associated with a higher incidence of congenital heart disease, resulting in universal screening fetal echocardiograms (F-echo) even when cardiac structures on obstetric scan (OB-scan) are normal. Recent studies suggest ... ...

Abstract	In vitro fertilization (IVF) is associated with a higher incidence of congenital heart disease, resulting in universal screening fetal echocardiograms (F-echo) even when cardiac structures on obstetric scan (OB-scan) are normal. Recent studies suggest that when OB-scan is normal, F-echo may add little benefit and increases cost and anxiety. We aim to determine the utility of screening F-echo in IVF pregnancies with normal cardiac anatomy on prior OB-scan. We conducted a retrospective chart review of IVF pregnancies referred for F-echo at the Seattle Children's Hospital between 2014 and 2020. OB-scan results and subspecialty of interpreting physician (Obstetrics = OB; Maternal Fetal Medicine = MFM; Radiology = Rads), F-echoes, and postnatal outcomes were reviewed. Cardiac anatomy on OB-scans was classified as complete if 4-chamber and outflow-tract views were obtained. Supplemental views (three-vessel and sagittal aortic arch views) on OB-scan were also documented. Of 525 IVF referrals, OB-scan reports were available for review in 411. Normal anatomy was demonstrated in 304 (74%) interpreted by OB (128; 42%), MFM (80; 26%), and Rads (96; 32%). F-echo was normal in 278 (91%). Of the 26 abnormal F-echo, none required intervention (17 muscular and 5 perimembranous ventricular septal defects, and 4 minor valve abnormalities). There was no difference in OB-scan accuracy for identifying normal cardiac anatomy when comparing 4-chamber and outflow-tract views vs. addition of supplemental views (91% vs 92% normal F-echo; p > 0.1). Evaluation of OB-scan accuracy by interpreting physician subspecialty demonstrated normal F-echo in 95%, 85%, and 92% (p = 0.95) as read by OB, MFM, and Rads, respectively. A majority of IVF referrals with normal cardiac anatomy visualized on OB-scan using 4-chamber and outflow-tract views resulted in normal F-echo, regardless of interpreting physician subspecialty or addition of supplemental views. Of the minority with abnormal F-echo, none required intervention. Consideration should be given to the cost/benefit of screening F-echo for the indication of IVF if normal cardiac anatomy is demonstrated on OB-scan.
MeSH term(s)	Child ; Echocardiography/methods ; Female ; Fertilization in Vitro ; Fetal Heart/diagnostic imaging ; Heart Defects, Congenital/diagnosis ; Humans ; Pregnancy ; Retrospective Studies ; Ultrasonography, Prenatal/methods
Language	English
Publishing date	2022-02-26
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/s00246-022-02857-5
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Zs.A 1528: Show issues

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Article: Rare Association of Absent Pulmonary Valve Syndrome with Double Outlet Right Ventricle and Hypoplastic Left Heart Complex.

Frandsen, Erik L / Doshi, Arpan R / Buddhe, Sujatha / Arya, Bhawna / Chikkabyrappa, Sathish M

Kansas journal of medicine

2020 Volume 13, Page(s) 21–22

Language	English
Publishing date	2020-01-31
Publishing country	United States
Document type	Journal Article
ISSN	1948-2035
ISSN	1948-2035
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Article ; Online: The role of regional prenatal cardiac screening for congenital heart disease: A single center experience.

Pasierb, Michele M / Peñalver, Josiah M / Vernon, Margaret M / Arya, Bhawna

Congenital heart disease

2018 Volume 13, Issue 4, Page(s) 571–577

Abstract: Background: Accurate prenatal diagnosis of congenital heart disease (CHD) allows for appropriate delivery and postnatal management. Geographic constraints limit access to fetal cardiology subspecialists. In our approach, general pediatric cardiologists ... ...

Abstract	Background: Accurate prenatal diagnosis of congenital heart disease (CHD) allows for appropriate delivery and postnatal management. Geographic constraints limit access to fetal cardiology subspecialists. In our approach, general pediatric cardiologists are first line in regional prenatal cardiac screening. We aim to demonstrate the utility of this approach in diagnosing CHD requiring cardiac interventions within 30 days of life. Methods: This is a retrospective review of fetal echocardiograms performed at Seattle Children's Hospital regional cardiology sites (SCH-RC) from December 2008 to December 2015. Referrals to Seattle Children's Hospital Prenatal Program (SCH-PNP) were evaluated for referral timing, indication, diagnostic accuracy, and postnatal care. Diagnostic accuracy was determined using the initial postnatal echocardiogram as the gold standard. Major discrepancy was defined as one resulting in change in surgical management. Results: Of 699 fetuses evaluated at regional sites throughout Washington and Alaska, a small subset (n = 48; 6.9%) required referral to SCH-PNP. Need for relocation was confirmed in 31 subjects, of which 27 required cardiac intervention within 30 days of life. Of those not referred to SCH-PNP (n = 643, 91.9%), none required neonatal cardiac intervention. There were 22 regional diagnostic discrepancies (31% major, 7% minor). Referral to SCH-PNP improved diagnostic accuracy (2% major, 0% minor). Conclusions: Regional prenatal cardiac screening demonstrated 100% sensitivity and 98.9% specificity for identifying critical CHD. Utilizing regional pediatric cardiologists as first line in prenatal screening in geographically remote regions may improve access to care and outcomes in neonates with critical CHD while improving resource utilization.
MeSH term(s)	Echocardiography/methods ; Female ; Gestational Age ; Heart Defects, Congenital/diagnosis ; Heart Defects, Congenital/embryology ; Heart Defects, Congenital/epidemiology ; Humans ; Incidence ; Infant, Newborn ; Male ; Pregnancy ; Prenatal Diagnosis/methods ; Retrospective Studies ; Ultrasonography, Prenatal/methods ; United States/epidemiology
Language	English
Publishing date	2018-06-25
Publishing country	United States
Document type	Journal Article
ZDB-ID	2274321-2
ISSN	1747-0803 ; 1747-079X
ISSN (online)	1747-0803
ISSN	1747-079X
DOI	10.1111/chd.12611
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