December

Fetal Echocardiographic Dimension Indices: Important Predictors of Postnatal Coarctation

Fetal Echocardiographic Dimension Indices: Important Predictors of Postnatal Coarctation. Fricke K, Liuba P, Weismann CG. Pediatr Cardiol. 2020 Dec 23. doi: 10.1007/s00246-020-02509-6. PMID: 33355680   Take Home Points: Fetuses that developed CoA postnatally (34%) exhibited significantly smaller Z-scores of left cardiac structures from the mitral valve to the aortic isthmus. The most sensitive and specific predictors were: A carotid-subclavian artery index (CSAI) of < 0.78 (92.3% sensitivity, 96.8% specificity) A product of isthmus-to-duct ratio in the three-vessel trachea view (3VT) and the mitral-to-tricuspid valve ratio (I/D3VTxMV/TV) of < 0.37 (100% sensitivity, 94.6% specificity).   Commentary from Dr. Manoj Gupta (New York, USA), section editor of Pediatric & Fetal Cardiology Journal Watch: In this retrospective study, the authors are trying to reliably identify quantitative fetal echocardiographic predictors of postnatal CoA.   The authors measured the following ratios between left and right cardiac structures: left-to-right ventricular (LV/RV) width and length ratios and mitral-to-tricuspid valve (MV/TV) dimension ratio examined in the four-chamber view; aortic-to-pulmonary valve (AoV/PV) ratio; ascending-to-descending aorta (Ao asc/DAo) ratios measured in the outflow tract and sagittal views; and isthmus aortae-to-arterial duct (I/D) diameter ratios examined in the three-vessel trachea (3VT) and sagittal views. In addition, the carotid-subclavian artery index (CSAI) was also calculated, defined as the ratio of the aortic arch diameter at the left subclavian artery, to the distance between the left carotid artery and the left subclavian artery.   A bidirectional or retrograde flow in the aortic arch (p < 0.001) or bidirectional or left–right shunt across the interatrial communication (p = 0.002) was linked to postnatal CoA. Fetuses with a postnatally confirmed CoA exhibited significantly smaller left cardiac structures from the mitral valve to the aortic isthmus when adjusted for gestational age. Borderline left ventricular hypoplasia, a hypoplastic aortic arch, or posterior shelf are known predictors for postnatal CoA.   The authors validated the CSAI and I/D3VTxMV/TV indices prospectively in 16 fetuses with prenatal suspicion of CoA born in 2019, four of which developed CoA postnatally. A CSAI < 0.78 detected postnatal development of CoA with a sensitivity of 100% and a specificity of 91.7%. The I/D3VTxMV/TV < 0.37 exhibited both a 100% sensitivity and specificity.   Cut-off points, sensitivity, and specificity for highly significant continuous parameters:   

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Effect of In Utero Non-Steroidal Anti-Inflammatory Drug Therapy for Severe Ebstein Anomaly or Tricuspid Valve Dysplasia (NSAID Therapy for Fetal Ebstein anomaly)

Effect of In Utero Non-Steroidal Anti-Inflammatory Drug Therapy for Severe Ebstein Anomaly or Tricuspid Valve Dysplasia (NSAID Therapy for Fetal Ebstein anomaly) Freud LR, Wilkins-Haug LE, Beroukhim RS, LaFranchi T, Phoon CK, Glickstein JS, Cumbermack KM, Makhoul M, Morris SA, Sun HY, Ferrer Q, Pedra SR, Tworetzky W.Am J Cardiol. 2021 Feb 15;141:106-112. doi: 10.1016/j.amjcard.2020.11.013. Epub 2020 Nov 18.PMID: 33217351   Take Home Points: Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD) often produce severe tricuspid regurgitation (TR) in the fetus and in some cases; pulmonary regurgitation (PR) and circular shunting ensue. Since the ductus arteriosus (DA) plays a critical role in the circular shunt and may be constricted by transplacental nonsteroidal anti-inflammatory drugs (NSAIDs) 12 of 15 (80%) achieved DA constriction after a median of 2.0 days. (All fetuses with DA constriction had improved PR; 92% had improved Doppler patterns. Eleven of 12 patients (92%) with DA constriction survived to live-birth, whereas 4 of 9 patients (44%) who did not receive NSAIDs or achieve DA constriction survived (p = 0.046)   Commentary from Dr. Manoj Gupta (New York, USA), section editor of Pediatric & Fetal Cardiology Journal Watch: This is a retrospective case series, which included mothers of second- and third-trimester fetuses with severe EA/TVD and circular shunting who were offered NSAID therapy at multiple international centers from 2010 to 2018. All fetuses had severe EA/TVD with severe TR and circular shunting with at least moderate PR.    High-dose indomethacin (100 mg 2 to 4 times daily) was typically administered as initial therapy to achieve DA constriction. Fetal echocardiograms were performed approximately every 24 to 48 hours to assess whether DA constriction was achieved. If DA constriction was achieved, then therapy was commonly transitioned to maintenance ibuprofen (200 to 600 mg 3 to 4 times daily), throughout the remainder of gestation.     All 12 fetuses with DA constriction had improved PR, with 1 having complete resolution, and half achieved antegrade pulmonary blood flow. With regard to safety, 1 fetus developed complete DA constriction after 6 days of indomethacin therapy; this fetus had resolution of hydrops and normalization of the extracardiac Dopplers and was delivered at 38.0 weeks. Ten patients (67%) developed oligohydramnios, ranging from 3 to 42 days after initiation of indomethacin and/or ibuprofen therapy. In 5 cases, therapy was transitioned from indomethacin to ibuprofen or briefly stopped with resumption of normal amniotic fluid volume. In 2 cases that developed oligohydramnios at 33.6 and 35.7 weeks, the decision was made to proceed with delivery at 34.1 and 35.9 weeks, respectively. The remainder was monitored without intervention until delivery. Postnatally, 2 neonates had renal failure requiring peritoneal dialysis.   

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Pregnancy Outcomes in Women After Arterial Switch Operation for Transposition of the Great Arteries: Results From ROPAC (Registry of Pregnancy and Cardiac Disease) of the European Society of Cardiology EURObservational Research Programme

Pregnancy Outcomes in Women After Arterial Switch Operation for Transposition of the Great Arteries: Results From ROPAC (Registry of Pregnancy and Cardiac Disease) of the European Society of Cardiology EURObservational Research Programme Tutarel O, Ramlakhan KP, Baris...

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Method comparison of HPLC-ninhydrin-photometry and UHPLC-PITC-tandem mass spectrometry for serum amino acid analyses in patients with complex congenital heart disease and controls

Method comparison of HPLC-ninhydrin-photometry and UHPLC-PITC-tandem mass spectrometry for serum amino acid analyses in patients with complex congenital heart disease and controls Michel M, Salvador C, Wiedemair V, Adam MG, Laser KT, Dubowy KO, Entenmann A, Karall D,...

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