Fetal Echocardiographic Predictors of Postnatal Surgical Strategies in Critical Pulmonary Stenosis or Atresia with Intact Ventricular Septum: A Meta-Analysis.

BACKGROUND: Critical pulmonary stenosis or atresia with intact ventricular septum (PSAIVS) may be managed either by biventricular repair or univentricular palliation. This systematic review and meta-analysis aimed to synthesize the evidence for the role of fetal echocardiography in predicting the postnatal treatment pathway. METHODS: PubMed/MEDLINE, CINHAL, Cochrane Library, Academic Search Complete, Web of Science, and Trip Pro were searched for observational studies published before July 2021. Random-effects meta-analysis was performed to identify factors associated with biventricular repair. RESULTS: Eleven individual studies published between 2006 and 2021, including a total of 285 participants (159 biventricular repair; 126 univentricular palliation), met our eligibility criteria. The pooled estimated prevalence of biventricular repair among patients with PSAIVS was 55.6% (95% confidence interval 48.5-62.5%). Those who underwent biventricular repair had greater right to left ventricle and tricuspid to mitral valve dimension ratios, greater TV z score, and longer TV inflow duration/cardiac cycle length by fetal echocardiography. They were also more likely to have significant tricuspid regurgitation and less likely to have ventriculo-coronary connections (VCCs). CONCLUSIONS: Commonly obtained fetal echocardiographic measurements have strong associations with treatment pathway choice for patients with PSAIVS. Greater RV growth appears to favor biventricular repair, whereas patients with VCC almost invariably undergo univentricular palliation. Future studies should aim to establish how these fetal echocardiographic parameters might predict outcomes for the two treatment pathways.

End-Diastolic Forward Flow and Restrictive Physiology in Repaired Tetralogy of Fallot: A Systematic Review and Meta-Analysis.

Background Pulmonary arterial end-diastolic forward flow (EDFF) following repaired tetralogy of Fallot has been thought to represent right ventricular (RV) restrictive physiology, but is not fully understood. This systematic review and meta-analysis sought to clarify its physiological and clinical correlates, and to define a framework for understanding EDFF and RV restrictive physiology. Methods and Results PubMed/MEDLINE, Embase, Scopus, and reference lists of relevant articles were searched for observational studies published before March 2021. Random-effects meta-analysis was performed to identify factors associated with EDFF. Forty-two individual studies published between 1995 and 2021, including a total of 2651 participants (1132 with EDFF; 1519 with no EDFF), met eligibility criteria. The pooled estimated prevalence of EDFF among patients with repaired tetralogy of Fallot was 46.5% (95% CI, 41.6%-51.3%). Among patients with EDFF, the use of a transannular patch was significantly more common, and their stay in the intensive care unit was longer. EDFF was associated with greater RV indexed volumes and mass, as well as smaller E-wave velocity at the tricuspid valve. Finally, pulmonary regurgitation fraction was greater in patients with EDFF, and moderate to severe pulmonary regurgitation was more common in this population. Conclusions EDFF is associated with dilated, hypertrophied RVs and longstanding pulmonary regurgitation. Although several studies have defined RV restrictive physiology as the presence of EDFF, our study found no clear indicators of poor RV compliance in patients with EDFF, suggesting that EDFF may have multiple causes and might not be the precise equivalent of RV restrictive physiology.

How to interpret the paediatric 12-lead ECG.

ECG interpretation is a core skill for any healthcare practitioner that looks after children. The article aims to educate the reader in basic interpretation of paediatric ECG in a succinct, interactive, organised manner in a way that it can be easily referenced and applied in everyday clinical practice. We include clinical examples as well as age and sex-related reference ranges for QT intervals, P-wave duration, Q-wave amplitude, QRS complex duration, R-wave and S-wave amplitude, R/S ratio and PR intervals.

Ultrasound Theranostics in Adult and Pediatric Cardiovascular Research.

Theranostics, the practice of systematically integrating diagnostics with treatment, has evolved as a field of medicine. In the context of ultrasound based theranostics, both traditional microbubbles and inorganic nanoparticles have emerged as technologies of clinical interest. Ultrasound induced microbubble cavitation has demonstrated efficacy in a variety of applications, including thrombolysis, tumor ablation, targeted microvascular flow enhancement, and targeted drug and gene delivery. This commentary summarizes the mechanisms and applications of ultrasound-based theranostics in cardiovascular medicine, including its impact in pediatric cardiology. It also provides an overview of ongoing clinical trials for theranostics in cardiovascular medicine.

Late transcatheter recanalization of a closed ductus arteriosus in a 2-month-old infant with tetralogy of Fallot and isolated left pulmonary artery.

Ductal stenting is increasing as an alternative to surgical shunts in cyanotic newborns. However, most reports include newborns with patent ducts who are often on prostaglandin before the procedure. We report a successful late transcatheter recanalization of the closed ductus arteriosus in a 2-month-old infant with tetralogy of Fallot, right aortic arch, and an isolated left pulmonary artery (LPA). We achieved adequate LPA growth before complete repair. There were no procedural complications. This procedure is rare in Western countries due to universal pulse oximetry screening and increased fetal diagnoses. The procedure is technically challenging in the absence of any angiographic flow into the ductus. Evidence of a ductal ampulla increases the chances of success.

Pseudoaneurysm of the mitral-aortic intervalvular fibrosa following endocarditis and aortic valve surgery in an infant-Case report and exhaustive systematic review of pediatric cases.

Pseudoaneurysm of the mitral-aortic intervalvular fibrosa (P-MAIVF) continues to be a rare diagnosis in children. Etiology, presentation, and management strategies are considerably different from adults. We report a fatal case of P-MAIVF with classical transthoracic and transesophageal echocardiographic findings complemented by CT imaging. The natural course of uncomplicated/asymptomatic P-MAIVF is largely unknown since most patients are offered surgery. We present an extensive literature review of pediatric P-MAIVF and highlight important differences from the adult form of this disease.

Double whammy: A case of bilateral bicuspid arterial valves in transposition, with a review of the literature.

Bicuspidity of both the semilunar valves is rarely reported. We report the first ever case of bilateral bicuspid semilunar valves in a case of transposition.

Variable Treatment Approaches for Extracorporeal Membrane Oxygenation Complications in Neonates: A Case Series.

Extracorporeal membrane oxygenation complications involving vascular injury or intracardiac thrombosis in neonates are rare. We present a series of unique complications and describe their variable treatments.

Age-Related Changes in Inferior Vena Cava Dimensions among Children and Adolescents with Syncope.

OBJECTIVE: To test the hypothesis that increased venous compliance manifested as inferior vena cava (IVC) dilation is an important substrate for syncope in children. STUDY DESIGN: IVC diameters were measured in 191 children and adolescents with syncope and in 95 controls. Subjects were divided based on age <12 years (younger group) and ≥12 years (older group). IVC measurements at the right atrial junction (IVC-RA), 10 mm below the IVC-RA junction (IVC-RA10), and at the point of maximal diameter (IVCmax) were made. The linear relation to body surface area (BSA) was confirmed, as were dimensions indexed to BSA (iIVC). Relationships between iIVC and the time of day were evaluated. RESULTS: In the syncope group, the mean age was 12.9 ± 3.6 years, mean weight was 54.7 ± 23 kg, and mean BSA was 1.5 ± 0.4 m2. Among controls, all IVC dimensions varied linearly with BSA (P < .001). In the older group (140 patients with syncope and 60 controls), all iIVC dimensions were larger in the syncope cohort: iIVC-RA, 9 vs 7.7 mm/m2 (P < .0001); iIVC-RA10, 9.4 vs 8.1 mm/m2 (P < .0001); iIVCmax, 11.7 vs 10.6 mm/m2 (P = .002). In the younger group (51 patients with syncope and 35 controls), there were no differences in iIVC measurements between the syncope cohort and controls: iIVC-RA, 10.2 vs 11.3 mm/m2; iIVC-RA10, 11.7 vs 12.0 mm/m2; iIVCmax, 14.2 vs 14.7 mm/m2 (P > .05 for all). CONCLUSIONS: The IVC is enlarged in teenagers with syncope compared with controls, suggesting that venous capacitance and resultant pooling play roles in the pathogenesis of syncope. In contrast, younger children with syncope do not demonstrate IVC dilation, suggesting that their syncope arises from a different mechanism.

Utility of Follow-Up Annual Echocardiograms in Patients With Complete Transposition of the Great Arteries After Arterial Switch Operations.

The arterial switch operation (ASO) in complete transposition of the great arteries (TGA) has increased long-term survival. Annual follow-up echocardiograms are recommended, but evidence-based guidelines do not exist. We sought to assess how often a patient with TGA after ASO who had no symptoms or change in physical exam underwent an intervention based solely on echocardiographic changes. We retrospectively reviewed all records from patients with TGA and a history of ASO followed at our institution between November 1983 and January 2015. Changes in echocardiograms resulting in hospital admission, significant medication change, interventional catheterization, or surgical procedure were identified through the surgical and cardiac catheterization laboratory databases and patient charts. These changes were referred to as an actionable change (AC). Interventions were defined as being driven by either clinical (change in physical exam, patient and/or parental concerns) or echocardiographic findings. A total of 1,792 echocardiograms from 149 patients were reviewed. Median number of echocardiograms per patient was 12 (1 to 34). Of the 1,792 echocardiograms, 20 (1.12%) were associated with AC. The most common intervention for an AC was cardiac catheterization (13 of 20, 65%). Most AC (15 of 20, 75%) occurred in the first decade after ASO. AC occurred in 83% (5 of 6) of those with a history of both ASO and arch repair. Annual echocardiograms in patients with TGA after ASO are rarely useful and are unnecessary. In conclusion, decreasing surveillance of asymptomatic patients to biennial follow-up echocardiograms in asymptomatic patients without physical examination changes is safe and would decrease medical expenses.

Characteristics and Fate of Pediatric Heart Disease Abstracts Presented at National Meetings.

BACKGROUND: This study evaluated the characteristics and publication outcomes of pediatric heart disease abstracts presented in national meetings. METHODS: Three years of abstracts (2012-2014) were evaluated from five national meetings: Society of Thoracic Surgeons (STS), American Association of Thoracic Surgeons (AATS), American College of Cardiology (ACC), American Academy of Pediatrics (AAP), and American Heart Association (AHA). Each abstract was assessed for publication in a peer-reviewed scientific journal, time to publication, impact factor of the journal, and number of citations associated with the publication. RESULTS: A total of 1145 abstracts qualified for inclusion. The majority of the abstracts originated from North America (80.8%) and were single institutional (85.7%), clinical (92.5%), and retrospective (77.9%) studies. A total of 767 abstracts (66.9%) were published in peer-reviewed journals as full-length articles. More than 90% of the abstracts presented at the surgical meetings were published as manuscripts in peer-reviewed journals. In contrast, 68.8% of ACC abstracts 53.7% AAP abstracts and 61.4% of AHA abstracts were published as manuscripts in a peer-reviewed journal. The median time to publication was shortest for STS abstracts (10 months) and longest for AHA abstracts (29 months). The median impact factor for published manuscripts varied between 3.0 and 3.5 for AATS abstracts, STS abstracts, ACC abstracts, and AHA abstracts. Manuscripts from AATS abstracts had the highest number of citations. CONCLUSIONS: Our data indicate that the national cardiology and cardiac surgery meetings have become a forum for presenting high-quality research, with >90% of the abstracts presented in these meetings culminating in publication.

Feasibility of Transthoracic Echocardiography Evaluation of Pulmonary Arteries Following Arterial Switch Operation.

Pulmonary artery (PA) stenosis is the most common late sequela following arterial switch for d-transposition of the great arteries. The purpose of this study was to assess the effectiveness of transthoracic echocardiography in evaluating the pulmonary arteries following repair. This was a retrospective, cross-sectional analysis of all echocardiograms performed on patients following arterial switch operation. A numerical scoring system was devised and used to quantify PA visualization based on 2D images, color mapping, and spectral Doppler. The study cohort included 150 patients. The ability to visualize at least one PA was poorer in patients who were older [> 10 years (47%) vs ≤ 10 years (89%) (p < 0.001)], and who had larger body surface area (BSA) (> 1.25 m2 (40%) vs ≤ 1.25 m2 (90%) (p < 0.001)]. Regardless of age, 2D visualization of the pulmonary arteries was poor for the entire cohort. Of those with at least one non-visualized PA, only 54% had alternative imaging performed or ordered within the 5 years at or prior to their last echocardiogram. In conclusion, PA visualization following arterial switch is worse in patients who are older and in those with larger BSA. In such patients, alternative forms of imaging are more likely to be necessary.

Stillbirth: the heart of the matter.

We evaluated 2,083 cases within the Wisconsin Stillbirth Service Program (WiSSP) that had autopsy reports or ultrasound data relevant to the heart. Of these, 167/1,782 (9.4%) stillbirths after 20 weeks and 11/301 (3.7%) miscarriages <20 weeks had congenital heart disease (CHD). Cases were classified by type of heart defect and whether it related to cause of death. Among cardiac anomalies that contributed significantly to fetal death, 125/151 (83%) were associated with underlying conditions or syndromes, nearly half of which were chromosomal. The most common forms of CHD in stillborns were severe cyanotic lesions (3%), then ventricular (2.6%) and atrial (1.9%) septal defects. Compared to livebirths, this represents a shift toward more severe cardiac lesions, although all comparable categories, including non-lethal conditions such as atrial septal defect, are more common in stillbirths. Clinical cardiomyopathy was identified as cause of death in 1.2% of stillborns. Cardiomegaly, occurring in 26.7% of all cases and 76.7% of infants born to diabetic mothers, may represent undiagnosed cardiomyopathy and/or may decrease fetal tolerance of hypoxia. In contrast, 78.5% of Turner syndrome infants, all <32 weeks, had small hearts. More attention to cardiac findings can lead to increased understanding of stillbirth causes. Based on our findings, we recommend chromosome studies on all stillbirths and close attention to the heart during second trimester ultrasounds, with chromosome studies offered if CHD is found. Consideration of heart size can result in prenatal identification of infants at risk for stillbirth, particularly large hearts in fetuses of diabetic mothers in the third trimester, which may identify fetal cardiomyopathy before it becomes life-threatening.

Doppler echocardiography imaging in detecting multi-valvular lesions: a clinical evaluation in children with acute rheumatic fever.

RATIONALE: Doppler echocardiography has been demonstrated to be accurate in diagnosing valvular lesions in rheumatic heart disease (RHD) when compared to clinical evaluation alone. OBJECTIVE: To perform Doppler echocardiography in children clinically diagnosed by the Jones criteria to have acute rheumatic fever (ARF), and to then compare the effectiveness of echo in detecting single/multi-valvular lesions with that of the initial clinical evaluation. METHODS AND RESULTS: We enrolled 93 children who were previously diagnosed with ARF by clinical examination. Presence of valvular lesions were enlisted, first by clinical auscultation, and then by performing Doppler echocardiography. We found that Doppler echocardiography was a sensitive technique, capable of detecting valvular lesions that were missed by clinical auscultation alone. Echocardiography of patients with carditis revealed mitral regurgitation to be the most common lesion present (53 patients, 56.98%), followed by aortic regurgitation in 21 patients (22.6%). The difference between clinical and echocardiographic diagnosis in ARF children with carditis was statistically significant for mitral regurgitation, aortic regurgitation and tricuspid regurgitation. Clinical auscultation alone revealed 4 cases of mitral stenosis, 39 mitral regurgitation, 14 aortic regurgitation, 9 tricuspid regurgitation; in contrast, echo revealed 5 cases of mitral stenosis, 53 mitral regurgitation, 21 aortic regurgitation, 18 tricuspid regurgitation. CONCLUSION: Doppler echocardiography is a more sensitive technique for detecting valvular lesions. In the setting of ARF, echo enables a 46.9% higher detection level of carditis, as compared to the clinical examination alone. Echo was very significant in detecting regurgitation lesions, especially for cases of tricuspid regurgitation in the setting of multivalvular involvement. The results of our study are in accordance with previous clinical studies, all of which clearly demonstrate the advantages of Doppler echocardiography, paving the way for its probable inclusion as one of the Jones major criteria for diagnosing ARF.