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This document has been developed to provide a guide for basic and advanced reporting in paediatric echocardiography. Furthermore, it aims to help clinicians in the interpretation of echocardiographic measurements and functional data for estimating the severity of disease in different paediatric age groups. The following topics will be reviewed and discussed in the present document: (i) the general principle in constructing a paediatric echocardiographic report, (ii) the basic elements to be included, and (iii) the potential and limitation of currently employed tools used for disease severity quantification during paediatric reporting. A guide for the interpretation of Z-scores will be provided. Use and interpretation of parameters employed for quantification of ventricular systolic function will be discussed. Difficulties in the adoption of adult parameters for the study of diastolic function and valve defects at different ages and pressure and loading conditions will be outlined, with pitfalls for the assessment listed. A guide for careful use of prediction scores for complex congenital heart disease will be provided. Examples of basic and advanced (disease-specific) formats for reporting in paediatric echocardiography will be provided. This document should serve as a comprehensive guide to (i) structure a comprehensive paediatric echocardiographic report; (ii) identify the basic morphological details, measures, and functional parameters to be included during echocardiographic reporting; and (iii) correctly interpret measurements and functional data for estimating disease severity. [ABSTRACT FROM AUTHOR]

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Congenital heart diseases (CHDs) represent a heterogeneous group of congenital defects, with high prevalence worldwide. Non-invasive imaging is essential to guide medical and surgical planning, to follow the patient over time in the evolution of the disease, and to reveal potential complications of the chosen treatment. The application of cardiac magnetic resonance imaging (CMRI) in this population allows for obtaining detailed information on the defects without the necessity of ionizing radiations. This review emphasizes the central role of CMR in the overall assessment of CHDs, considering also the limitations and challenges of this imaging technique. CMR, with the application of two-dimensional (2D) and tri-dimensional (3D) steady-state free precession (SSFP), permits the obtaining of very detailed and accurate images about the cardiac anatomy, global function, and volumes' chambers, giving essential information in the intervention planning and optimal awareness of the postoperative anatomy. Nevertheless, CMR supplies tissue characterization, identifying the presence of fat, fibrosis, or oedema in the myocardial tissue. Using a contrast agent for angiography sequences or 2D/four-dimensional (4D) flows offers information about the vascular, valvular blood flow, and, in general, the cardiovascular system hemodynamics. Furthermore, 3D SSFP CMR acquisitions allow the identification of coronary artery abnormalities as an alternative to invasive angiography and cardiovascular computed tomography (CCT). However, CMR requires expertise in CHDs, and it can be contraindicated in patients with non-conditional devices. Furthermore, its relatively longer acquisition time and the necessity of breath-holding may limit its use, particularly in children under eight years old, sometimes requiring anesthesia. The purpose of this review is to elucidate the application of CMR during the pediatric age. [ABSTRACT FROM AUTHOR]

Objectives: Prenatal detection of congenital heart disease (CHD) by using fetal echocardiography (FE) helps early diagnosis leading to prompt management and treatment. FE provides the high accurate non-invasive modality to improve the survival or life quality of CHD patients. The aim of this study was to evaluate the antenatal detection of CHD by FE and compare with post-delivery echocardiography results. Method: A prospective cohort study of pregnant women referred to tertiary center Imam-Reza hospital, Mashhad, Iran for performing FE in hands of a skilled pediatric cardiologist between 2012 and 2021. Cardiac echocardiography was performed by GE Vivid 7 color Doppler and Mindray Resona 7 color Doppler with a convex probe 5-7 megahertz during late first trimester or early second trimester and after birth till 2-month later. Data was analyzed with SPSS and MedCalc software and agreement evaluated by using kappa. Result: Out of 261 studied fetuses, 101 normal cases detected in total agreement with postnatal-echo diagnosis. Acceptable diagnosis found for Septal defects; VSDs high statically detected (sensitivity= 90%, specificity= 93%). complex CHDs noted to be the mostly precise accurate prenatal diagnosis. Right Arch abnormalities, aortic stenosis, hypoplastic left heart syndrome and cardiac masses were completely acceptable but detecting coarctation of aorta faced with over-diagnosed. Prenatal diagnosed arrhythmias without structural defects mostly premature beats shifted to normal after-birth Echo. Conclusion:FE is a safe and sensitive modality in the prenatal diagnosis of CHDs. The study showed the effectiveness accuracy of early first trimester; also complete detection in both-side of defect spectrum.

This article presents the case of a 27-year-old female patient with idiopathic congenital complete heart block who does not consent to the implantation of a cardiac pacemaker but was referred by her primary care physician for cardiological evaluation. The conduction disturbance was recognized at the age of 6 and was asymptomatic. The professional disqualification from pacemaker implantation included a detailed history of a patient's symptoms, an echocardiographic assessment of the heart, exercise testing and ECG Holter monitoring. The aid of salbutamol administered orally was also useful. [ABSTRACT FROM AUTHOR]

Introduction: Objective: Prenatal detection of congenital heart disease (CHD) using fetal echocardiography (FE) helps in early diagnosis leading to prompt management and treatment. FE provides a highly accurate non-invasive modality to improve the survival or quality of life of CHD patients. The aim of this study was to evaluate the antenatal detection of CHD by FE and compare it with the results of postnatal echocardiography. Methods: A prospective cohort study of pregnant women referred to a tertiary center Imam-Reza Hospital, Mashhad, Iran, for performing FE in the hands of an experienced pediatric cardiologist between 2012 and 2021. Cardiac echocardiography was performed by GE Vivid 7 color Doppler and Mindray Resona 7 color Doppler with convex probe 5-7 megahertz during late first trimester or early second trimester and after birth until 2 months later. Data were analyzed using SPSS and MedCalc software, and agreement was assessed using kappa. Results: Out of 261 studied fetuses, 101 normal cases were detected in full agreement with postnatal echo diagnosis. Acceptable diagnosis was found for septal defects; VSDs were highly statistically detected (sensitivity= 90%, specificity= 93%). Complex CHDs were found to be the most accurate prenatal diagnosis. Right arch anomalies, aortic stenosis, hypoplastic left heart syndrome and cardiac masses were perfectly acceptable, but detection of coarctation of the aorta faced with over-diagnosis. Prenatally diagnosed arrhythmias without structural defects, mostly premature beats, shifted to normal postnatal echo. Conclusion: FE is a safe and sensitive modality in prenatal diagnosis of CHDs. The study showed the effectiveness accuracy of early first trimester; also complete detection in both sides of the defect spectrum. [ABSTRACT FROM AUTHOR]

Background: Balloon sizing (BS) has been used for device size selection in percutaneous atrial septal defect (ASD) closure. Due to its limitations, alternative imaging techniques like three‐dimensional transesophageal echocardiography (3D‐TEE) are valuable for guiding ASD device size selection during ASD closure procedures. The purpose of this study was to compare ASD sizing using measurements obtained from 3D–TEE to those utilizing the standard balloon sizing method. Methods: We identified 53 patients with single secundum type ASD without PFO who underwent percutaneous closure at the Tehran Heart Center between 2019 and 2022. Balloon sizing was performed in all patients with the stop‐flow technique, and the choice of device size was determined based on the sizing derived from BS. 3D‐TEE imaging was performed before the intervention, and the ASD shape and quality of ASD rims were assessed. Results: Among the 53 patients who underwent single ASD device closure, multiple 3D TEE measurements significantly correlated with balloon sizing results. This included defect area, perimeter, and diameter obtained from 3D‐TEE images multi‐planar reconstruction. ASD perimeter detected by 3D TEE had the best correlation with BS results. When divided by the shape of ASD, there was no significant difference between our 3D‐images data and BS in round or oval‐shaped ASDs. Conclusion: The 3D‐TEE study is reliable for assessing ASD configurational characteristics in percutaneous device closure candidates. 3D‐TEE has the potential to accurately determine the appropriate device size and reduce complications, costs, and procedural duration. Further research is needed to validate these findings and establish the role of 3D‐TEE measurements in guiding the best treatment decisions for ASD closure. [ABSTRACT FROM AUTHOR]

Congenital heart disease is now the most common reason for women to attend a high-risk joint cardiac–obstetric clinic. With advances in medical care and surgical techniques, most children born with congenital heart disease reach childbearing age and have a good prognosis for long-term survival with a good quality of life. Pregnancy remains a major life event for these women, posing additional risks with the potential for severe morbidity and mortality. The hemodynamic changes induced by pregnancy can unmask undiagnosed congenital heart disease or exacerbate pre-existing conditions. Therefore, specialist care is essential for a successful pregnancy outcome. Women with complex congenital heart disease embarking on pregnancy need the support of a multidisciplinary team, including cardiologists, obstetricians, anaesthetists, midwives and adult congenital heart disease clinical nurse specialists. Clinical nurse specialists in particular play a crucial role in coordinating, supporting, educating and advocating for the mother and fetus through preconception, pregnancy, the puerperium and beyond. [ABSTRACT FROM AUTHOR]

Background: Quadricuspid aortic valve (QAV) is a rare congenital disease. The clinical characteristics of this disease remain unclear except for those in relatively young patients reported from tertiary referral hospitals. The aim of this study was to determine the clinical features of QAV in a regional population. Methods and Results: We retrospectively investigated 25 340 consecutive patients over middle age (median age, 73 (IQR 65-80) years; range, 45-102 years) who underwent transthoracic echocardiography (TTE) at our institute during the period from April 2008 to December 2023. Eight (0.032%) of the patients (median age, 65 years; range, 47-91 years) were diagnosed withQAV. Six patients suffered from aortic regurgitation (AR), and one patient had mild aortic stenosis at the time of QAVd iagnosis. Two patients who had severe AR at referral underwent aortic valve surgery. The severity of AR in the other patients was moderate or less. During a median follow-up period of 27 months (range, 1-171 months), none of the patients other than above two patients had cardiac events. One patient died from a non-cardiac cause at 94 years of age. Conclusions: Patients diagnosed with QAV after middle age, who do not exhibit severe valve insufficiency at the time of diagnosis, may not experience worse clinical outcomes. However, further research is required for a better understanding of the long-term outcomes. [ABSTRACT FROM AUTHOR]

Introduction: Arrhythmogenic cardiomyopathy (AC) is an inherited cardiomyopathy characterized by fibro‐fatty replacement of cardiomyocytes, leading to life‐threatening ventricular arrhythmia and heart failure. Pathogenic variants of desmoglein2 gene (DSG2) have been reported as genetic etiologies of AC. In contrast, many reported DSG2 variants are benign or variants of uncertain significance. Correct genetic variant classification is crucial for determining the best medical therapy for the patient and family members. Methods: Pathogenicity of the DSG2 Ser194Leu variant that was identified by whole exome sequencing in a patient, who presented with ventricular tachycardia and was diagnosed with AC, was investigated by electron microscopy and immunohistochemical staining of endomyocardial biopsy sample. Results: Electron microscopy demonstrated a widened gap in the adhering junction and a less well‐organized intercalated disk region in the mutated cardiomyocytes compared to the control. Immunohistochemical staining in the proband diagnosed with AC showed reduced expression of desmoglein 2 and connexin 43 and intercalated disc distortion. Reduced expression of DSG2 and Connexin 43 were observed in cellular cytoplasm and gap junctions. Additionally, we detected perinuclear accumulation of DSG2 and Connexin 43 in the proband sample. Conclusion: Ser194Leu is a missense pathogenic mutation of DSG2 gene associated with arrhythmogenic left ventricular cardiomyopathy. [ABSTRACT FROM AUTHOR]

The article describes the case of a 58-year old female diagnosed with a rare combination of correct transposition of the great arteries and isolated levocardia. It describes the position of the heart chambers and abnormal spatial relationship of the great arteries, including the right and left atrium, right ventricle and pulmonary artery, verified by transthoracic echocardiography, Color Doppler flow imaging, cardiac magnetic resonance imaging, echocardiography and cardiac segmental analysis.

Fetal echocardiography is the gold standard modality to detect suspected congenital heart disease (CHD). Accurate diagnosis and subsequent prognosis is even more challenging in the presence of a raised maternal body mass index (BMI). This retrospective study aimed to gain insight into the prevalence of obesity within the cohort of patients referred for fetal echocardiography. Retrospective analysis of all pregnant patients referred to the Scottish National Fetal Cardiology Service between 2015 and 2021 due to a suspected fetal cardiac abnormality and examining the associated trends in maternal BMI and the Scottish Index of Multiple Deprivation (SIMD). BMI data were available for 962 (96.3%) of the 998 patients referred during the study period. Median BMI during the study period was 31. BMI range in the seven-year period was 16–63. There was no association between BMI group and year (P = 0.889). A median of 58% of patients referred were classified as overweight (BMI > 25 kg/m2), and only 37% were reported to have a BMI within normal limits. Referral BMI was relatively consistent in the seven years with no dramatic increase in the obese categories. Mean BMI in SIMD 5 (lowest level of deprivation), was significantly lower (P = 0.001), than in SIMD 1 (highest deprivation). People of child bearing age should be aware the potential limitations that a raised BMI may have upon diagnostic/screening accuracy impacting subsequent ability to provide accurate fetal cardiac diagnoses and prognostic fetal cardiac imaging. [ABSTRACT FROM AUTHOR]

Quadricuspid aortic valve is a very rare congenital anomaly. Its association with rheumatoid arthritis is exceptional with this being the third case reported in the literature. We report a case of a 52 year old female patient with quadricuspid aortic valve type C accompanied by moderate to severe aortic regurgitation and longstanding, advanced form of rheumatoid arthritis. Having refused surgical aortic valve intervention 4 years ago, the patient is currently under a watchful follow-up strategy. The patient received a diagnosis of rheumatoid arthritis over 15 years before and presently has serious deformities in the hands, legs, feet, and spine. In conclusion, quadricuspid aortic valve and rheumatoid arthritis together are extremely rare. While it is possible that this association is coincidental, considering the genetic background of both disorders, there is a potential for them to be interconnected comorbidities. This report is the first to highlight the association between the 2 disorders. [ABSTRACT FROM AUTHOR]

To evaluate the changes of cardiac morphology and function in fetuses conceived through assisted reproductive technologies (ART) by speckle tracking echocardiography. A retrospective study was conducted in 101 spontaneously conceived (SC) fetuses and 99 ART-conceived ones. Fetal echocardiography was performed, fetal cardiac morphology and function were analyzed using two-dimensional speckle tracking software, including global sphericity index (GSI), global longitudinal strain (GLS), fractional area change (FAC) of the left and right ventricles, as well as segmental sphericity index (SI), end-diastolic diameter (ED), and fractional shortening (FS) in 24 segments. Compared to the SC fetuses, the ART-conceived fetuses exhibited decreased GSI (median [interquartile range], 1.22 [1.16–1.27] vs. 1.18 [1.11–1.24], p=0.007), decreased right ventricular GLS (24.9 [21.5–27.6] vs. 23.2 [20.4–26.8], p=0.026), and decreased right ventricular FAC (mean ± standard deviation, 39.7 ± 6.4 vs. 37.2 ± 7.1, p=0.003). Analysis of the 24 segments showed that ART-conceived fetuses had reduced SI in the apical segments of right ventricle and increased ED in several segments of the right ventricle. Fetuses conceived through ART had a more spherical shape of the global heart and predominantly right-sided cardiac remodeling and systolic function impairment. [ABSTRACT FROM AUTHOR]

Right aortic arch (RAA) is a rare anomaly with an incidence of 0.1 % in the adult population and low-risk fetuses. Our aim in this study was to evaluate associated anomalies and conditions according to subtypes. This was a retrospective study examining consecutive pregnancies diagnosed with RAA in our hospital between 2018 and 2022. Fetuses with RAA were divided into three groups, RAA with right-sided ductus arteriosus (RAA-RDA), RAA with left-sided ductus arteriosus (RAA-LDA), and RAA with a double aortic arch (RAA-DAA). A total of 81 fetuses were diagnosed as having RAA during the study period. The rate of cardiac anomalies (82.8 %) in the RAA-RDA group was higher than in the RAA-LDA (17.6 %) and RAA-DAA (22.2 %) groups (p<0.001). No statistically significant difference was found between the groups in terms of maternal age, diagnosis week, pregnancy outcome, extracardiac anomalies, and genetic anomalies. Three (8 %) of 36 fetuses with isolated RAA who resulted in live birth developed symptoms related to the vascular ring, and one (2.7 %) newborn with RAA-DAA underwent surgery. The incidence of cardiac anomalies is high in fetuses with RAA-RDA. Ultrasound examinations should be performed for cardiac anomalies and additional structural anomalies. Vascular ring formation is a rare but important complication due to compression risk to the trachea and esophagus. [ABSTRACT FROM AUTHOR]

Background: Rheumatic heart disease (RHD) is a potentially life-threatening condition that causes long-standing public health concerns. Echocardiography is a reliable diagnostic and screening technique for many cardiovascular conditions, including RHD. It is commonly used in tertiary care facilities worldwide but less so in the community setting. The primary aim of this study was to introduce and elaborate on the echocardiographic screening for RHD that was implemented by a Malaysian primary care clinic in Penampang district, Sabah. We also set out to present the uptake of the service in its initial years of establishment, as well as the challenges faced. Methods: In the first part of this study, document reviews were conducted to compile relevant information about the conceptualization and implementation of this service. Following that, we also obtained secondary data on the echocardiographic screening service from its first implementation in April 2020 until May 2021 to examine the uptake and the patient profile. Results: From April 2020 to May 2021, a total of 189 echocardiographic screening was conducted by primary care doctors using handheld ultrasound. Of the 189 children screened, 19 (10.1%) were found to have cardiac anomalies and were referred for a formal echocardiogram. Upon follow-up, 8 were detected with mild mitral regurgitation and referred to the nearest tertiary hospital for further management. Conclusion: Based on our review, the echocardiographic screening for RHD among children conducted by the Penampang Health Clinic was deemed successful. Echocardiogram service provided by primary care centers located in suburban and rural areas is highly beneficial for patients with poor access to specialized health care services because they stay far away from tertiary care facilities. Tapping into family medicine physicians located closer to communities to conduct echocardiographic screening and review the results can improve the detection of cardiac anomalies requiring further investigation. With the success of this project, echocardiographic services in the primary healthcare setting can be expanded by garnering the necessary collaborative efforts and consistent support from various stakeholders. [ABSTRACT FROM AUTHOR]

User-centered models for the development of digital health interventions are not consistently applied in healthcare settings. This study used a five-phase, user-centered approach to develop HEARTPrep©, a psychosocial intervention delivered via mobile app and telehealth to mothers expecting a baby with congenital heart disease (CHD) to promote maternal, family, and child well-being. Phases of intervention development were: (I) establishing partnerships; (II) creating content; (III) developing prototype and testable intervention; (IV) conducting think-aloud testing; and (V) completing beta testing. Partnerships with parents, clinicians, and design/technology experts were integral throughout the development of HEARTPrep©. Parents of children with CHD also served as participants in Phases II-V, contributing to the creation of content and providing feedback to inform the iterative refinement of HEARTPrep©. These five phases produced a refined digital health intervention with promising feasibility, usability, and acceptability results. This user-centered approach can be used to develop digital health interventions targeting various health outcomes. [ABSTRACT FROM AUTHOR]

Blood speckle tracking echocardiography (BSTE) is a new, promising 4D flow ultrafast non-focal plane imaging technique. The aim of the present investigation is to provide a review and update on potentialities and application of BSTE in children with congenital heart disease (CHD) and acquired heart disease. A literature search was performed within the National Library of Medicine using the keywords "echocardiography", "BST", and "children". The search was refined by adding the keywords "ultrafast imaging", "CHD", and "4D flow". Fifteen studies were finally included. Our analysis outlined how BSTE is highly feasible, fast, and easy for visualization of normal/abnormal flow patterns in healthy children and in those with CHD. BSTE allows for visualization and basic 2D measures of normal/abnormal vortices forming the ventricles and in the main vessel. Left ventricular vortex characteristics and aortic flow patterns have been described both in healthy children and in those with CHD. Complex analysis (e.g., energy loss, vorticity, and vector complexity) are also highly feasible with BSTE, but software is currently available only for research. Furthermore, current technology allows for BSTE only in neonates and low-weight children (e.g., <40 kg). In summary, the feasibility and potentialities of BSTE as a complementary diagnostic tool in children have been proved; however, its systemic use is hampered by the lack of (i) accessible tools for complex quantification and for acquisition at all ages/weight, (ii) data on the diagnostic/prognostic significance of BSTE, and (iii) consensus/recommendation papers indicating when and how BSTE should be employed. [ABSTRACT FROM AUTHOR]

Background: Aorto‐left ventricular tunnel (ALVT) is a paravalvular communication between aorta and left ventricle. It is one of the rare congenital heart diseases which could present with heart failure. Case presentation: A case of ALVT was diagnosed in infancy. Preliminary assessment was possible using conventional echocardiography; however, functional assessment and accurate anatomy of ALVT were further verified via variable imaging modalities starting from speckle tracking and three‐dimensional echocardiography to cardiac CT angiogram. The tunnel was successfully repaired with uneventful recovery. Conclusions: Multimodality imaging can accurately assess cardiac function and demonstrate the anatomy of ALVT noninvasively to plan for successful intervention. [ABSTRACT FROM AUTHOR]

Bicuspid aortic valve is the most common congenital heart defect. Transthoracic echocardiogram is the initial tool to assess and diagnose this condition, however, transesophageal echocardiogram with 3D modalities, including transillumination have a better anatomical and functional evaluation of the valve, allowing to classify the bicuspid aortic valve according to the position of the raphe and assess the main vessels for complications or exclude other cardiovascular diseases. [ABSTRACT FROM AUTHOR]

Accessory chordae tendineae is an extremely rare anomaly. In this case report, we described a 61‐year‐old female patient newly diagnosed with the combination of an accessory mitral valve chordae extending from left atrium which is an extremely rare congenital anomaly and a bicuspid aortic valve. In our patient, three‐dimensional echocardiography showed incremental value over two‐dimensional echocardiography in the assessment of the exact localization and the extend of accessory chordea. [ABSTRACT FROM AUTHOR]

A neonate born of third-degree consanguineous marriage presented on day 12 of life with congestive cardiac failure. A male sibling died at 3 months of age, cause of which was not known. He was treated with decongestive measures and multiple inotropes. 2D Echocardiogram revealed severe Left ventricular dysfunction with prominent trabeculations and deep recesses in the left ventricle suggestive of Left ventricular non-compaction. He was also found to have horse-shoe kidney. Considering the presence of cardiac left ventricular non compaction, horse-shoe kidney and family history of neonatal death and pregnancy loss clinical exome sequencing was done. It detected a homozygous missense variant in exon 6 of the AGK gene suggestive of Senger's syndrome. Baby was on regular follow-up and was thriving well on diuretics, sacubitril-valsartan and weekly levosimendan infusions. At 8 months of age, cardiac transplantation was successfully done and baby has been doing well posttransplantation. LVNC in children is rare with an estimated incidence of 0.11 per 100,000, the highest incidence being during infancy. Senger's syndrome is autosomal recessive in inheritance. Senger's syndrome associated with Left ventricular non compaction has been reported only once in literature so far. Renal manifestations in the form of horse shoe kidney like in our index baby has not been reported previously with Senger's syndrome. [ABSTRACT FROM AUTHOR]

Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital heart disease. Many patients remain asymptomatic when no cardiac lesions are present (isolated ccTGA). In ccTGA, the atrioventricular conduction system may be abnormal, resulting in progressive dysfunction and, eventually, a Complete Heart Block (CHB). In ccTGA with situs inversus, the conduction pathway resembles a normal tract, but the atrioventricular node is located posteriorly. Compared with situs solitus, spontaneous CHB is uncommon in ccTGA patients with situs inversus. We report the case of a 40-year-old female without previous medical conditions or having a family or personal history of heart disease presented with loss of consciousness. At admission, electrocardiography revealed bradycardia, CHB, and hypertrophy of the right ventricle. Cardiovascular imaging tests detected an isolated ccTGA with situs inversus and levocardia. The Holter monitor revealed intermittent CHB. Exercise testing demonstrated chronotropic incompetence. An epicardial pacemaker was implanted, and the patient was discharged symptom-free. At a two-years follow-up remains asymptomatic. This case illustrates the importance of cardiovascular imaging in defining cardiac anatomy, ruling out other congenital heart defects, and facilitating pacing therapy in complex congenital heart disease. Congenital heart disease patients should be treated by a multidisciplinary team with expertise in permanent pacing. [ABSTRACT FROM AUTHOR]

Purpose: To develop, implement and assess the results of psychoeducation to improve the QoL of parents with CHD newborns. Methods: Participants were parents of inpatient newborns with the diagnosis of non‐syndromic CHD. We conducted a parallel RCT with an allocation ratio of 1:1 (intervention vs. control), considering the newborns, using mixed methods research. The intervention group received psychoeducation (Parental Psychoeducation in CHD [PPeCHD]) and the usual routines, and the control group received just the regular practices. The allocation concealment was assured. PI was involved in enrolling participants, developing and implementing the intervention, data collection and data analysis. We followed the Consolidated Standards of Reporting Trials (CONSORT) guidelines. Results: Parents of eight newborns were allocated to the intervention group (n = 15 parents) and eight to the control group (n = 13 parents). It was performed as an intention‐to‐treat (ITT) analysis. In M2 (4 weeks), the intervention group presented better QoL levels in the physical, psychological, and environmental domains of World Health Organization Quality of Life instrument (WHOQOL‐Bref). In M3 (16 weeks), scores in physical and psychological domains maintained a statistically significant difference between the groups. Conclusions: The PPeCHD, the psychoeducational intervention we developed, positively impacted parental QoL. These results support the initial hypothesis. This study is a fundamental milestone in this research field, adding new essential information to the literature. [ABSTRACT FROM AUTHOR]

Absent pulmonary valve syndrome (APVS) is a rare congenital anomaly characterized by rudimentary PV tissue with variable degrees of PV stenosis and regurgitant pulmonary blood flow. In most cases, it is associated with tetralogy of Fallot. In a minority of APVS cases, with an unknown frequency, intact ventricular septum (IVS), patent ductus arteriosus, and possible tricuspid atresia are present. This condition is known as non-Fallot type APVS. We describe a case of an antenatal diagnosis of APVS with IVS, a large patent ductus arteriosus, and ascending aorta dilatation. The mother was referred to our center at 32 weeks of gestation due to cardiomegaly on sonography. Fetal echocardiography revealed cardiomegaly, right atrial and ventricular enlargement, aneurysmal dilatation of the main pulmonary artery, and mild dilatation of the pulmonary artery branches. Also observed were IVS, rudimentary PV tissue with severe to-and-fro turbulence across the PV, a widely open ductus arteriosus, ascending aorta dilatation, and levorotation of the heart. After childbirth, our diagnosis was confirmed by echocardiography and surgery. The baby experienced severe respiratory distress. At 15 days of life, surgical intervention in the form of pulmonary artery arterioplasty was performed, resulting in good outcomes. The patient underwent follow-up for 6 months and showed reasonable health. [ABSTRACT FROM AUTHOR]

Congenital anomalies of inferior vena cava are increasingly being recognized with the technical advancements and increased utilization of cross-sectional imaging techniques. Duplication of inferior vena cava classically involves duplication of the infrarenal segment, where both inferior vena cava ascend on either side of the abdominal aorta until they form a confluence at the level of the renal veins. It has been extensively described in literature with few reports of more complex variation in the form of duplicated infrarenal inferior vena cava with azygos or hemiazygos continuation. This article describes extremely rare complete duplication of inferior vena cava involving both suprarenal and infrarenal segments. Moreover, the complete duplication of inferior vena cava is seen in association with concomitant double superior vena cava, in a patient with visceroatrial situs solitus and associated congenital heart disease, which to the best of our knowledge, has not been reported so far in literature. This study also highlights the utility of multidetector computed tomography in accurate identification of such anomalies. [ABSTRACT FROM AUTHOR]

Background: Newborns with a critical congenital heart disease left undiagnosed and untreated have a substantial risk for serious complications and subsequent failure to thrive. Prenatal ultrasound screening is not widely available, nor is postnatal echocardiography. Physical examination is the standard for postnatal screening. Pulse oximetry has been proposed in numerous studies as an alternative screening method. This systematic review and meta-analysis aims to determine the diagnostic accuracies of both screening methods separately and combined. Methods: A systematic literature search of the Embase, PubMed, and Global Health databases up to 30 November 2023 was conducted with the following keywords: critical congenital heart disease, physical examination, clinical scores, pulse oximetry, and echocardiography. The search included all studies conducted in the newborn period using both physical examination and pulse oximetry as screening methods and excluded newborns admitted to the intensive care unit. All studies were assessed for risk of bias and applicability concerns using the QUADAS-2 score. The review adhered to the PRISMA 2020 statement guideline. Results: Out of 2711 articles, 20 articles were selected as eligible for meta-analysis. Cumulatively, the sample included 872,549 screened newborns. The pooled sensitivity of the physical examination screening method was found to be 0.69 (0.66–0.73 (95% CI)) and specificity was found to be 0.98 (0.98–0.98). For the pulse oximetry screening method, the pooled sensitivity and specificity yielded 0.78 (0.75–0.82) and 0.99 (0.99–0.99), respectively. The combined method of screening yielded improved diagnostic characteristics at a sensitivity and specificity of 0.93 (0.91–0.95) and 0.98 (0.98–0.98, respectively. Conclusions: The evidence indicates that combining both physical examination and pulse oximetry to screen for critical congenital heart disease exceeds the accuracy of either separate method. The main limitation is that solely newborns with suspected critical congenital heart disease were subjected to the reference standard. We recommend adapting both methods to screen for critical congenital heart diseases, especially in settings lacking standard fetal ultrasound screening. To increase the sensitivity further, we recommend increasing the screening time window and employing the peripheral perfusion index. [ABSTRACT FROM AUTHOR]

Methemoglobinemia is a rare dyshemoglobin disorder which can either be congenital or acquired. Dyshemoglobin disorders can be asymptomatic or symptomatic. We narrate the case of a 12-year-old girl who presented with a fever, cough, and oxygen saturation of 85%. She was diagnosed with COVID-19, along with a large atrial septal defect and pulmonary arterial hypertension. Arterial blood gas analysis revealed normal partial pressure of oxygen and on 100% exposure to oxygen, blood color turned chocolate brown. After the resolution of COVID-19 in 10 days, the patient was treated with oral ascorbic acid and successful atrial septal defect repair. It is important to suspect dyshemoglobin disorder in a patient who presents with hypoxia/hypoxemia. [ABSTRACT FROM AUTHOR]

The article addresses the complex issue of differential diagnosis of disproportion in the four-chamber view of the fetal heart during late pregnancy. Topics discussed include physiological versus pathological causes of ventricular disproportion, diagnostic techniques for distinguishing these causes, and the implications of these findings for prenatal and postnatal care.

Background: In developing countries, fewer women have access to multidisciplinary congenital heart disease and reproductive programs staffed by experts. We report pregnancy outcomes of a multidisciplinary healthcare strategy utilizing an in-hospital teamwork approach in Vietnam. Methods: This retrospective cohort study included pregnant women with unrepaired congenital heart disease managed at a referral cardiovascular center. Results: Undiagnosed congenital heart disease before pregnancy, a lack of pre-pregnancy cardiology counseling, and modified World Health Organization class III/IV were common. Under the multispecialty healthcare strategy, although the rate of maternal death was 8.2% in the modified World Health Organization class IV group, no deaths occurred in any other group. Fetal/neonatal complications occurred in 54% of pregnancies, and 49.4% of neonates survived. Poor pregnancy outcomes were associated with admission during the first/seconde trimester for fetus/neonates, third trimester for mother, modified World Health Organization class III/IV, cyanosis, and heart failure. Conclusion: The outcomes of pregnant women with unrepaired congenital heart disease were poor but seemed to improve with a multidisciplinary in-hospital healthcare teamwork strategy. [ABSTRACT FROM AUTHOR]

Background: Parents who have a newborn with a congenital heart defect experience negative emotions, which may determine the emotional state of their children. Methods: The study group included 154 parents of newborns and infants with cyanotic congenital heart disease, before cardiac surgery and after the procedure. HADS m and PSS-10 questionnaires were used to assess parental anxiety, depression, aggression, and the level of stress. Results: High levels of depression, anxiety, total HADS and stress were diagnosed in a large group of parents, regardless of the stage of cardiac surgery treatment. A high level of stress was associated with a higher prevalence of emotional disturbance both in the total HADS (overall) and in all its individual domains. Anxiety and depression were more common in mothers. A high level of stress was a significant predictor of anxiety and depression in parents. Conclusions: A high level of stress was a significant predictor of anxiety and depression in parents of infants with congenital heart disease. The parents' psychological condition is one of many potential determinants over the course of their child's treatment and recovery. [ABSTRACT FROM AUTHOR]

Congenital heart defects (CHDs) are the most common neonatal malformations and are a leading cause of infant death in developed countries. Finding safe and effective diagnostic methods to screen for CHDs is important. The aim of this study was to evaluate the effectiveness of pulse oximetry (PO) and perfusion index (PI) in screening CHD. We conducted a systematic review of studies in PubMed, Embase, and the Cochrane Library published on or before October 1, 2021. Studies based on PICOS were included in this systematic review. The flow chart is made by PRISMA software. The quality of included studies was assessed by RevMan5 software (QUADAS-2: Quality Assessment of Diagnostic Accuracy Studies-2). The sensitivity, specificity, and other measurements of accuracy were pooled using Stata/SE 12.0 software. Five studies containing 46,965 neonates were included in this study. A randomized-effects model was used for the meta-analysis because of significant heterogeneity. The combined sensitivity and specificity were 0.82 (95% confidence interval [CI], 0.53–0.95) and 0.97 (95% CI, 0.57–1.00), respectively. The area under the curve was 0.92 (95% CI, 0.89–0.94). The combination PO and PI was significant in CHD screening. Once diagnosed by the combined method, it means that the neonate is most likely to have a CHD. Key Points Pulse oximetry and PI screening. Congenital heart defects. A systematic review and meta-analysis. [ABSTRACT FROM AUTHOR]

Congenital heart disease (CHD) is the leading cause of death from malformations in the first year of life and carries a significant burden to the family when the diagnosis is made in the prenatal period. We recognize the significance of family counseling following a fetal CHD diagnosis. However, we have observed that most research focuses on assessing the emotional state of family members rather than examining the counseling process itself. The objective of this study was to identify and summarize the findings in the literature on family counseling in cases of diagnosis of CHD during pregnancy, demonstrating gaps and suggesting future research on this topic. Eight databases were searched to review the literature on family counseling in cases of CHD diagnosis during pregnancy. A systematic search was conducted from September to October 2022. The descriptors were "congenital heart disease", "fetal heart", and "family counseling". The inclusion criteria were studies on counseling family members who received a diagnosis of CHD in the fetus (family counseling was defined as any health professional who advises mothers and fathers on the diagnosis of CHD during the gestational period), how the news is expressed to family members (including an explanation of CHD and questions about management and prognosis), empirical and qualitative studies, quantitative studies, no publication deadline, and any language. Out of the initial search of 3719 reports, 21 articles were included. Most were cross-sectional (11) and qualitative (9) studies, and all were from developed countries. The findings in the literature address the difficulties in effectively conducting family counseling, the strengths of family counseling to be effective, opportunities to generate effective counseling, and the main challenges in family counseling. [ABSTRACT FROM AUTHOR]

Pediatric interventional cardiology procedures are essential in diagnosing and treating congenital heart disease in children; however, they raise concerns about potential radiation exposure. Managing radiation doses and assessing image quality in angiographs becomes imperative for safe and effective interventions. This systematic review aims to comprehensively analyze the current understanding of physical image quality metrics relevant for characterizing X-ray systems used in fluoroscopy-guided pediatric cardiac interventional procedures, considering the main factors reported in the literature that influence this outcome. A search in Scopus and Web of Science, using relevant keywords and inclusion/exclusion criteria, yielded 14 relevant articles published between 2000 and 2022. The physical image quality metrics reported were noise, signal-to-noise ratio, contrast, contrast-to-noise ratio, and high-contrast spatial resolution. Various factors influencing image quality were investigated, such as polymethyl methacrylate thickness (often used to simulate water equivalent tissue thickness), operation mode, anti-scatter grid presence, and tube voltage. Objective evaluations using these metrics ensured impartial assessments for main factors affecting image quality, improving the characterization of fluoroscopic X-ray systems, and aiding informed decision making to safeguard pediatric patients during procedures. [ABSTRACT FROM AUTHOR]

Controversy exists over the use of electrocardiograms (ECGs) in sports pre-participation screening. We performed a meta-analysis comparing the effectiveness of history and physical examination (H&P) with ECG at detecting both cardiac disease and sudden cardiac death–associated conditions (SCD-AC). Pre-participation studies published from 2015 to 2020 with athletes 10 to 35 years old were included. This yielded 28 011 athletes screened and 124 cardiac diagnoses, 103 of which were SCD-AC. A meta-analysis of log odds ratios (ORs) was conducted using a random-effects model. The ORs for the association between H&P and detecting both cardiac disease and SCD-AC were not statistically significant (OR = 3.4, P =.076; OR = 2.9, P =.078). The ORs for the association between ECG and detecting both cardiac disease and SCD-AC were statistically significant (60, P <.001; 148, P <.0001). In conclusion, the odds of detecting both cardiac disease and conditions related to SCD with ECG are greater than with H&P during sports pre-participation screening. [ABSTRACT FROM AUTHOR]

Congenital heart defects (CHDs) are the most common birth defects. Recently, artificial intelligence (AI) was used to assist in CHD diagnosis. No comparison has been made among the various types of algorithms that can assist in the prenatal diagnosis. Normal and abnormal fetal ultrasound heart images, including five standard views, were collected according to the International Society of Ultrasound in Obstetrics and Gynecology (ISUOG) Practice guidelines. You Only Look Once version 5 (YOLOv5) models were trained and tested. An excellent model was screened out after comparing YOLOv5 with other classic detection methods. On the training set, YOLOv5n performed slightly better than the others. On the validation set, YOLOv5n attained the highest overall accuracy (90.67 %). On the CHD test set, YOLOv5n, which only needed 0.007 s to recognize each image, had the highest overall accuracy (82.93 %), and YOLOv5l achieved the best accuracy on the abnormal dataset (71.93 %). On the VSD test set, YOLOv5l had the best performance, with a 92.79 % overall accuracy rate and 92.59 % accuracy on the abnormal dataset. The YOLOv5 models achieved better performance than the Fast region-based convolutional neural network (RCNN) & ResNet50 model and the Fast RCNN & MobileNetv2 model on the CHD test set (p<0.05) and VSD test set (p<0.01). YOLOv5 models are able to accurately distinguish normal and abnormal fetal heart ultrasound images, especially with respect to the identification of VSD, which have the potential to assist ultrasound in prenatal diagnosis. [ABSTRACT FROM AUTHOR]

Double inlet left ventricle (DILV) is a rare complex congenital heart defect characterized by the presence of two atrioventricular valve orifices leading into a single left ventricle. Patients rarely survive beyond teenage years without surgical correction. Supraventricular tachycardia (SVT) in DILV is a rare occurrence that adds an additional layer of complexity to diagnosis and management. Here, we report an exceptional case of DILV who survived well into her fourth decade of life without any surgical correction. The patient presented to us with SVT which lead to the discovery of her unique cardiac anomaly. [ABSTRACT FROM AUTHOR]

BNP is the unique cardiac marker that reflects not as much as the degree of heart muscle damage, but mostly the severity of hemodynamic disorder, which is important in congenital heart disease. The only disadvantage of this marker is the barely studied reference values for children. It is known that the younger the child is, the higher the BNP value will be. By shifting from interpreting the absolute values towards the application of zlog-transformed data in clinical practice, we can overcome the above problems. We performed an age-adjusted zlog transformation of BNP concentration. The age dependence was accounted for by a piecewise linear interpolation of the logarithms of BNP concentration among healthy children in different age groups from the logarithms of age. The concentration of BNP was measured in 351 patients (under 1 year old) with various heart diseases. The median age at the time of testing was 52 days [10; 166]; the median weight was 4.1 kg [3.2; 6.2]. The conditions we investigated included almost all known congenital heart diseases, as well as primary cardiac tumors. After the zlog transformation, we eliminated age-dependence, which was proved by comparing BNP concentrations in two groups of patients with univentricular and biventricular hemodynamics. BNP in patients with congenital heart disease reflects the severity of hemodynamic disorders, and zlogBNP is an objective, age-independent and clear mechanism that can be used to interpret this cardiac marker. [ABSTRACT FROM AUTHOR]

Anatomical configurations where the viscero-atrial structures do not follow the usual arrangement or mirror-imaged arrangement is described conventionally as heterotaxy. Isomerism in the context of the congenitally malformed heart is a situation where some paired structures on opposite sides of the left-right axis of the body are, in morphologic terms, symmetrical mirror images of each other. It encompasses two separate entities, right and left isomerism, the former being usually associated with asplenia and the latter with polysplenia. We report herein a rare case of left isomerism that is associated with asplenia in a 4-year-old girl. [ABSTRACT FROM AUTHOR]

Background: Congenital heart disease (CHD) is one of the commonly seen malformations, with incidence varying from 7 to 8/1000 live birth. Causes are multifactorial. Routine examination of neonates may miss more than 50% of cases. Pulse oximetry is a simple, noninvasive, bedside test which estimates the percentage of oxygen bound to hemoglobin (oxygen saturation [SpO2]). Detection of critical CHD (CCHD) has been possible with SpO2 screening. Many countries included SpO2 as part of newborn screening due to this. As a primary approach, both pre- and post-ductal extremity SpO2 is measured after 24 h of life. Echocardiography (ECHO) will be done on neonates with SpO2 readings <95%. Neonates born in high-altitude regions, studies have suggest to use adjusted threshold values. In India, there are limited studies. Aims: The aim of this study was to determine the usefulness of pulse oximetry as a screening tool for early detection of CHD in otherwise asymptomatic newborns. To determine, the accuracy of SpO2 for detecting clinically unrecognized CCHD in newborns. Materials and Methods: This is a prospective observational study done in the department of pediatrics in a tertiary hospital. The study was conducted over 12 months. During the study period, all neonates born who fulfilled the inclusion criteria were included in the study. After 24 h of life, neonates were examined clinically and the pre- and post-ductal SpO2 was measured. Neonates with SpO2 <90% in room air were excluded from the study. If the SpO2 was between 90% and 94%, clinical examination was repeated, if suspicious of CHD, they were referred for ECHO. If there was no suspicion of CHD, SpO2 was repeated after 6 h and ECHO was done if SpO2 ≤95. The difference of SpO2 >3% between the right upper limb and right lower limb was considered positive. Positive neonates were evaluated with two-dimensional echocardiograph. Inclusion criteria - All hemodynamically stable neonates were born during the study period. Exclusion criteria (1) Antenatally diagnosed cardiac anomalies, (2) Outborn neonates, (3) Parents/guardians are not willing to participate in the study and/or further investigation, (4) Sick neonates and those with SpO2 <90% at birth. Results: 1117 (83.7%) were eligible for the study out of 1333 neonates born. 669 (59.9%) were born by cesarean section and 448 (40.1%) by vaginal delivery. 996 (89.2%) were born at term (≥37 weeks of gestation) and 121 (10.8%) were preterm (<37 weeks of gestation). The male-to-female ratio was 1.03:1. The mean birth weight of the neonates was 2.91 ± 0.46 kg (mean ± standard deviation). The mean SPO2 in the right upper limb was 96.62 ± 1.73, in the right lower limb was 96.87 ± 1.76, in the left upper limb was 96.59 ± 1.90, and in the left lower limb was 97.06 ± 1.74. The average SPO2 difference between the right upper limb and right lower limb was 1.04 ± 1.07. Based on SpO2, 858 (76.8%) cases were not suspected of having CHD and 259 (23.3%) were suspected of having CHD and were evaluated with ECHO. Six (0.5%) neonates had CHD in whom echo was done. In our study, for detecting CCHD, SPO2 cutoff value of ≤90% showed 90% sensitivity, 99.94% specificity, 75% positive predictive value, and 99.98% negative predictive value. Conclusions: This study emphasizes noninvasive SPO2 as reliable and feasible, with good negative predictive value screening for CHD in neonates. [ABSTRACT FROM AUTHOR]

Double orifice mitral valve is a rare congenital anomaly that is often associated with other congenital cardiac abnormalities. Patients may present with valvular dysfunction or may be asymptomatic and have an incidental diagnosis of double outlet mitral valve. Whether symptoms due to this pathology are present or not it is important to accurately characterize this lesion. Three‐dimensional imaging is a powerful diagnostic modality for diagnosing and characterizing lesions such as this as highlighted in this case image series where we describe how three‐dimensional transesophageal echocardiography was utilized to characterize both complete and incomplete bridge type double orifice mitral valve. [ABSTRACT FROM AUTHOR]

The aorto-left ventricular tunnel is an extracardiac communication that has a specific morphological feature. It is important to differentiate this entity from other diagnoses because the treatment options differ significantly and better outcomes are obtained with this entity. [ABSTRACT FROM AUTHOR]

Berry syndrome is an extremely rare constellation of several congenital cardiac anomalies consisting of aortopulmonary window, aortic origin of the right pulmonary artery (AORPA), interrupted aortic arch or hypoplastic aortic arch or coarctation of the aorta, and an intact ventricular septum with high neonatal mortality rates. The disease is fatal with high mortality (90%) in the neonatal period with surviving patients mostly developing pulmonary hypertension. We describe the clinical presentation and diagnostic clues in two patients with Berry syndrome. [ABSTRACT FROM AUTHOR]

ALCAPA is a rare congenital heart disease. Presentation varies from asymptomatic to progressive heart failure and death. Surgical repair is indicated in all patients with a goal of restoring two coronary systems. Data was analysed in regard to presenting features, echocardiographic findings, various surgical approaches used and immediate, early and midterm post-operative results. Most common presentation was growth failure and seen in 6 patients. One patient was taken for elective PDA ligation and diagnosis of ALCAPA was made on table after PDA ligation as patient crashed subsequently. Aortocoronary button transfer was most commonly used surgical technique while 2 patients needed interposition grafting. LV function improved in 5 out of 8 patients with regression of MR. A median improvement of 5+-2% was observed in ejection fraction of 5 patients. Early surgery with aortocoronary transfer offers good results with gradual improvement in LV dysfunction and mitral regurgitation. [ABSTRACT FROM AUTHOR]

Introduction: Undiagnosed congenital heart disease and management of pediatric cardiogenic shock presents a diagnostic challenge for the emergency clinician. These diagnoses are rare and require a high index of suspicion given the overlap with more common pediatric pathology. Point-of-care ultrasound can assist in differentiating these presentations. We present a case of neonatal cardiogenic shock secondary to a previously undiagnosed congenital heart disease, specifically Shone complex, detected using point-of-care ultrasound. Case Report: A six-week-old female presented with severe respiratory distress and was found to be in cardiogenic shock secondary to a previously undiagnosed congenital heart defect. Conclusion: Initial diagnosis of congenital heart disease is uncommon in the emergency department, but it should be recognized by clinicians given the high associated morbidity and mortality. Point-of-care ultrasound is a powerful tool to assist in evaluating for cardiac abnormalities as an etiology for undifferentiated shock in the pediatric population. [ABSTRACT FROM AUTHOR]

Background: The occurrence of Isolated left subclavian artery (ILSA) is relatively rare, ILSA is caused by the persistence of the dorsal segment of the sixth left arch, with regression of the fourth arch artery and interruption of the left dorsal aorta at the distal end of the seventh intersegmental artery on the left side during embryonic development. The left subclavian artery is connected to the pulmonary artery through an arterial duct, which can be closed or unobstructed. This abnormality can lead to congenital subclavian steal syndrome and vertebrobasilar artery insufficiency. Case presentation: We reported three fetuses with ILSA and intracardiac malformation. Among them, one case was suspected to be diagnosed with ILSA by echocardiography, while the other two cases were not diagnosed, but were accidentally discovered during autopsy. We have also conducted a literature review of its prenatal screening, diagnosis, management, and outcomes. Our three cases were tested by WES‐Trio (whole exome sequencing). Worldwide, the ILSA cases reported in English literature have not been detected by WES. And likely pathogenic results were found in our two cases. Although it could not explain the intracardiac malformation we found, it will help to explore the etiology in the future. Conclusions: Prenatal echocardiography detection and diagnosis of ILSA is a new challenge, which has different effects on the prognosis of the fetus. When finding intracardiac malformation with right aortic arch, we need to perform an unconventional view of ultrasound scanning and combine with CDFI to find the origin of the left subclavian artery. Although we cannot find the cause of the disease temporarily, but our genetic results can help prenatal genetic counseling. [ABSTRACT FROM AUTHOR]

We present a case of a 13‐day‐old neonate with shock who was diagnosed with congenital variation of aortic arch and critical coarctation of aorta by cardiac point‐of‐care ultrasound. Finally, surgery confirmed the anomalies. This case demonstrates the value of cardiac point‐of‐care ultrasound in detecting congenital anomalies of great vessels in neonates and may be beneficial in clinical management. [ABSTRACT FROM AUTHOR]

Background: The value of prenatal identification of morphology of ductus arteriosus in fetuses with congenital heart defects (CHD) with pulmonary atresia and duct-dependent pulmonary circulation (DDPC) in planning neonatal ductal stenting procedure is untested. The aim of the study is to analyze the utility of three-dimensional/four-dimensional (3D/4D) spatiotemporal image correlation (STIC) fetal echocardiography in delineating the morphology of ductus arteriosus in fetuses with DDPC undergoing neonatal ductal stenting. Methods: In this retrospective study (2017-22), prenatal imaging of pulmonary artery (PA) anatomy, aortic arch sidedness, and morphology of ductus arteriosus (ductal origin was classified as vertical/horizontal and ductal course as tortuous/straight) was done using 3D/4D STIC imaging and volume datasets. Prenatal findings were correlated with angiographic findings during stenting and the degree of agreement was calculated. Results: We included 27 fetuses with a prenatal diagnosis of CHD with DDPC who underwent neonatal ductal stenting. The accuracy of prenatal assessment of PA anatomy, branch PA stenosis, and arch sidedness was 100%, 92.6%, and 88.9%, respectively. The accuracy of prenatal assessment of ductal origin and course, compared with angiography, was 85.2% and 88.9%, respectively. Prenatal imaging had a diagnostic accuracy of 100% for vertical straight and horizontal tortuous ducts, 84.6% for vertical tortuous, and 67% for horizontal straight ducts. Duct stenting was successful in 25 (92.6%) babies; two died after the procedure from stent occlusion. Conclusion: Fetal echocardiography using 3D/4D STIC imaging enables accurate delineation of the morphology of ductus arteriosus in fetuses with DDPC, thereby aiding parental counseling and planning neonatal ductal stenting. [ABSTRACT FROM AUTHOR]