September

Anatomical Survey Versus Fetal Echocardiograms for Diagnosis of Cardiac Defects with a Single Umbilical Artery Cases: A Retrospective Cohort Study and Diagnostic Meta-analysis

Anatomical Survey Versus Fetal Echocardiograms for Diagnosis of Cardiac Defects with a Single Umbilical Artery Cases: A Retrospective Cohort Study and Diagnostic Meta-analysis View Article Tiffany Tonismae 1, Jessica H Kline 1, Jennifer J Choe 1, Frank Schubert 1, Methodius Tuuli 1, Anthony Shanks 1 J Ultrasound Med. 2020 Sep 4.  doi: 10.1002/jum.15483. Online ahead of print. PMID: 32885858.  DOI: 10.1002/jum.15483 Take-Home Points: In a fetus with a Single Umbilical Artery, a standard anatomic survey with recommended cardiac screening highly predicts congenital heart disease. Addition of fetal echocardiogram does not yield more when cardiac views are normal on anatomic survey. Commentary from Dr. Venu Amula (Salt Lake City, USA), section editor of Pediatric & Fetal Cardiology Journal Watch: Single Umbilical Artery ( SUA) in the fetus is associated with cardiac and extracardiac anomalies. The American Institute of Ultrasound in Medicine recommends a fetal anatomic survey for prenatal detection of significant abnormalities. Standard cardiac screening includes a four-chamber view, left ventricular, and right ventricular outflow tract views at a minimum.  A question arises whether those with isolated SUA would benefit from a fetal echocardiogram to rule out congenital heart disease in addition to anatomic ultrasound. To answer this question, Tonismae et al. performed this retrospective cohort study of prenatally diagnosed SUA over ten years at a single institution. The authors hypothesized that in isolated SUA, there is no additional benefit to performing a fetal echocardiogram. The study reviewed patients with documented SUA who had both an anatomical survey and a fetal echocardiogram available.  They compared cardiac anatomy on a detailed anatomy survey with a fetal echocardiogram in the fetuses with SUA.  Additionally, they report a diagnostic meta-analysis of studies with similar comparison during the period 2010 and 2019. Predictive characteristics of anatomical ultrasound were estimated with a fetal echocardiogram to detect congenital heart disease as a “gold standard.” Of the 22,666 anatomical surveys during the study period, the authors identified SUA in 320 cases. The analysis was done on 93 patients that met inclusion criteria.  Forty-four of these had normal anatomic surveys, and all were subsequently reported to have normal fetal echocardiograms.  Of the 49 who had suboptimal or abnormal anatomic surveys, thirteen had a normal fetal echo, while 36 had an abnormal fetal echo. The sensitivity and specificity of the anatomic ultrasound were found to be 100% and 77% (p < 0.01), respectively, while the positive predictive value was 73% (p < 0.01). When an anatomical ultrasound evaluation of the fetal heart was compared with the fetal echocardiogram in this population, the ultrasound had a negative predictive value (NPV) of 100% (p < 0.01). The diagnostic meta-analysis reported a pooled sensitivity of 97% (95% CI 81, 100%) and a pooled specificity of 95% (95% CI 72, 99%). The summary ROC curve showed that the anatomic survey was highly predictive of cardiac defect on fetal echo. The risk of selection bias was assessed, and the authors show significant heterogeneity between studies for both sensitivity and specificity. The study is well-conducted and addresses an essential question of fetal echocardiogram utility in those with isolated SUA and the anatomical survey showing normal cardiac anatomy. The authors highlight that fetal echocardiography is a specialized imaging modality and can impose a high cost on the mother and the health care system. Besides, the personal cost of travel to some patients living remotely and the anxiety provoked by the investigation would make it hard to recommend when a standard anatomic survey is normal. However, the conclusions have to be read in the face of the limitations of this single-center study.  The study is performed at a single prenatal diagnosis center with experience conducting anatomic surveys and standard cardiac views. Any extrapolation to other centers with heterogeneous expertise should be made with caution. Besides, no information exists on postnatal confirmation of congenital heart disease, and the authors acknowledge the limitation of using prenatal echocardiography as the “gold” standard. The current updated guidelines of the American Institute of Ultrasound in Medicine recommend obtaining the 4-chamber heart, left ventricular outflow tract, right ventricular outflow tract views, and the addition of 3-vessel view and 3-vessel trachea view. They recommend a fetal echocardiogram with SUA if the anatomical ultrasound is suspicious for CHD or if cardiac views are suboptimal.  The study reaffirms this statement. It also offers credible evidence to the debate of the lack of utility of prenatal echocardiogram when the cardiac screening, done in conformity with the standard guidelines, is normal. However, the center's experience and technical expertise to meet the guidelines will continue to dictate individual decision-making.    

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Prenatal features, associated co-morbidities and clinical course of agenesis of the ductus venosus in the current era

Prenatal features, associated co-morbidities and clinical course of agenesis of the ductus venosus in the current era View Article McBrien A, Caluseriu O, Niederhoffer KY, Hornberger LK. Prenat Diagn. 2020 Sep 13. doi: 10.1002/pd.5827. Online ahead of print. PMID: 32920862 Take Home Points: Agenesis of the ductus venosus (ADV) is rare and nearly always associated with genetic or other associated anomalies. While increased cardiac output, especially in late gestation, is common, the risk of hydrops and mortality is lower than previously suspected. Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch:  Evaluation of the ductus venosus (DV) is a routine part of the pediatric echocardiogram and risk scoring. The DV is necessary for placental blood regulation, allowing the higher oxygen concentrated blood flow to stream towards the PFO to allow for more oxygen delivery to the coronary arteries and cerebral circulation. Abnormal flow patterns of the DV have been associated with chromosomal abnormalities and absence has been associated with a wide variety of genetic, cardiac, and non-cardiac associated abnormalities, with about ⅓ having a genetic diagnosis and up to about 40% with cardiac or non-cardiac anomalies. Due to the common association with an extrahepatic connection of the umbilical vein, there is an increased risk of high output cardiac failure, with resulting hydrops or fetal demise. With a significant improvement and availability of genetic testing and imaging, the authors hypothesized that there may be a higher incidence of genetic and structural abnormalities than previously reported, and possibly better outcomes during pregnancy and postnatally. A retrospective review of patients with ADV was done between 2007-2018. Clinical records, including fetal echocardiograms, prenatal ultrasounds, and postnatal echocardiograms were reviewed. Fetal echo included any structural or functional cardiac abnormalities, combined cardiac output (CCO) measurement, and presence of hydrops. Results of genetic testing (pre and postnatal) and pregnancy outcomes were recorded. 14 cases of ADV were found, with a mean GA of 23.9 weeks. None of the cases were isolated (ADV only). 9/14 (64%) had cardiac abnormalities with 2 patients having bilateral SVC as the only cardiac diagnosis (considered to be a non-pathologic variant). 13/14 (93%) had a non-cardiac abnormality with cystic hygroma being the most frequent diagnosis (29%). 7/14 (50%) had prenatal genetic testing and an additional patient had postnatal testing. Only 14% had aneuploidy, suggesting that more robust genetic testing is necessary to obtain a genetic diagnosis. There were two postnatal deaths. See table 1. There were no terminations of pregnancy and only 1 fetal demise (hydrops at presentation).  Mean GA at delivery was 36.4 weeks. Cardiac output was measured in 10 patients and 6 had CCO above a z-score of +2 with only 2 patients developing hydrops (Figure 1). 12/14 had extrahepatic connection of the umbilical vein; the 2 with intrahepatic connection did not have hydrops. The authors discuss the specific genetic diagnoses (previously reported in Turner and Noonan Syndrome) and speculate the association with PHACE and RASA1 mutations. Additionally, they discuss the CCO elevations that were more common in the 3rd trimester/later gestation as compared to other high output conditions such as TTTS or sacrococcygeal teratoma that are more common mid-gestation, which could possibly explain why hydrops was less likely. They also speculate that longer term neurodevelopmental data may be useful given the pathophysiology of decreased oxygenated blood to the fetal cerebral circulation in those with ADV.    

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Effect of Clopidogrel and Aspirin vs Aspirin Alone on Migraine Headaches After Transcatheter Atrial Septal Defect Closure: One-Year Results of the CANOA Randomized Clinical Trial

Effect of Clopidogrel and Aspirin vs Aspirin Alone on Migraine Headaches After Transcatheter Atrial Septal Defect Closure: One-Year Results of the CANOA Randomized Clinical Trial View Article Wintzer-Wehekind J, Horlick E, Ibrahim R, Cheema AN, Labinaz M, Nadeem N,...

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Acute and medium term results of balloon expandable stent placement in the transverse arch-a multicenter pediatric interventional cardiology early career society study

Acute and medium term results of balloon expandable stent placement in the transverse arch-a multicenter pediatric interventional cardiology early career society study View Article Shabana Shahanavaz 1, Osamah Aldoss 2, Kaitlin Carr 2, Brent Gordon 3, Michael D...

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