Saja Anabusi, Tim Van Mieghem, Greg Ryan, Shiri Shinar
Introduction: Our aim was to investigate the incidence, comorbidities, and outcomes of fetuses with an elevated middle cerebral artery peak systolic velocity (MCA-PSV) >1.5 multiples of median (MoM), despite normal hemoglobin (Hgb) levels on fetal blood sampling (FBS).
Methods: A single-center observational retrospective cohort study of all fetuses undergoing FBS and MCA-PSV >1.5 MoM. Only those fetuses with no or mild anemia were included. Indications for Doppler assessment, associated anomalies, and neonatal outcomes were collected.
Results: Overall, 383 fetuses had an MCA-PSV >1.5 MoM and underwent FBS. Twenty-three (6%) fetuses met our inclusion criteria and had no or only mild anemia. Associations with elevated MCA-PSV were elucidated in 12 of the 23 cases (52.2%) and included mild anemia (n = 2), intracranial hemorrhage (n = 3), genetic disease (n = 1), idiopathic nonimmune hydrops (NIH, n = 1), hypoxic-ischemic encephalopathy (n = 1), maternal and or fetal acidosis (n = 3), and fetal growth restriction (n = 1). Favorable perinatal outcomes were observed in truly unexplained 11 cases with no additional anomalies (47.8%).
Conclusion: Elevated MCA-PSV >1.5 MoM with normal Hgb levels is seen in 6% of pregnancies undergoing FBS and is often associated with other significant maternal or fetal problems. Those with unexplained and isolated MCA-PSV elevation have normal outcomes.
{"title":"Elevated Middle Cerebral Artery Peak Systolic Velocity in Non-Anemic Fetuses: Providing a Better Understanding of Enigmatic Middle Cerebral Artery Peak Systolic Velocity.","authors":"Saja Anabusi, Tim Van Mieghem, Greg Ryan, Shiri Shinar","doi":"10.1159/000540342","DOIUrl":"10.1159/000540342","url":null,"abstract":"<p><strong>Introduction: </strong>Our aim was to investigate the incidence, comorbidities, and outcomes of fetuses with an elevated middle cerebral artery peak systolic velocity (MCA-PSV) >1.5 multiples of median (MoM), despite normal hemoglobin (Hgb) levels on fetal blood sampling (FBS).</p><p><strong>Methods: </strong>A single-center observational retrospective cohort study of all fetuses undergoing FBS and MCA-PSV >1.5 MoM. Only those fetuses with no or mild anemia were included. Indications for Doppler assessment, associated anomalies, and neonatal outcomes were collected.</p><p><strong>Results: </strong>Overall, 383 fetuses had an MCA-PSV >1.5 MoM and underwent FBS. Twenty-three (6%) fetuses met our inclusion criteria and had no or only mild anemia. Associations with elevated MCA-PSV were elucidated in 12 of the 23 cases (52.2%) and included mild anemia (n = 2), intracranial hemorrhage (n = 3), genetic disease (n = 1), idiopathic nonimmune hydrops (NIH, n = 1), hypoxic-ischemic encephalopathy (n = 1), maternal and or fetal acidosis (n = 3), and fetal growth restriction (n = 1). Favorable perinatal outcomes were observed in truly unexplained 11 cases with no additional anomalies (47.8%).</p><p><strong>Conclusion: </strong>Elevated MCA-PSV >1.5 MoM with normal Hgb levels is seen in 6% of pregnancies undergoing FBS and is often associated with other significant maternal or fetal problems. Those with unexplained and isolated MCA-PSV elevation have normal outcomes.</p>","PeriodicalId":12189,"journal":{"name":"Fetal Diagnosis and Therapy","volume":null,"pages":null},"PeriodicalIF":1.6,"publicationDate":"2024-07-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141723325","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Chantelle de Vet, Hossy Zamani, Daisy van der Woude, Sally-Ann Clur, Guid Oei, Judith van Laar, Noortje van Oostrum
Introduction: The aim of this systematic review and meta-analysis was to evaluate fetal cardiac function in fetuses of mothers with diabetes compared to those of mothers without diabetes using 2D-STE.
Methods: Embase, MEDLINE, and CENTRAL were searched for observational studies on 2D-STE fetal left and right ventricular global longitudinal strain and strain rate that included singleton, non-anomalous pregnancies complicated by pregestational or gestational diabetes mellitus compared to uncomplicated pregnancies. The strain values were pooled per 4 weeks of gestation for meta-analysis using random-effects models.
Results: Fifteen studies met the criteria, including 990 fetuses of diabetic mothers and 1,645 control fetuses. The study design was cross-sectional in fourteen studies and longitudinal in one study. Gestational age, type of diabetes, ultrasound device, and 2D-STE software varied between the studies. Glycemic control and type of treatment were often lacking. In fetuses of diabetic mothers versus healthy mothers, left ventricular strain was significantly decreased (7 studies), increased (1 study), or not significantly different (7 studies). Right ventricular strain was decreased (7 studies), increased (1 study), or not different (2 studies). Left ventricular strain rate was decreased (3 studies), increased (1 study), or not different (2 studies). Right ventricular strain rate was increased (1 study) or not different (2 studies).
Conclusion: Fetuses of mothers with diabetes show evidence of systolic dysfunction, which is more visible in the right ventricle. Contradictory results are probably due to suboptimal study designs and variation in gestational age, diabetes severity, image acquisition, and software. Large prospective longitudinal studies are needed to assess fetal myocardial function with 2D-STE in pregestational diabetes mellitus type 1 and 2 and gestational diabetes mellitus pregnancies. The influence of glycemic control, BMI, and treatment should be evaluated.
{"title":"Fetal Strain and Strain Rate Measured with Speckle Tracking Echocardiography in Maternal Diabetes: Systematic Review.","authors":"Chantelle de Vet, Hossy Zamani, Daisy van der Woude, Sally-Ann Clur, Guid Oei, Judith van Laar, Noortje van Oostrum","doi":"10.1159/000538413","DOIUrl":"10.1159/000538413","url":null,"abstract":"<p><strong>Introduction: </strong>The aim of this systematic review and meta-analysis was to evaluate fetal cardiac function in fetuses of mothers with diabetes compared to those of mothers without diabetes using 2D-STE.</p><p><strong>Methods: </strong>Embase, MEDLINE, and CENTRAL were searched for observational studies on 2D-STE fetal left and right ventricular global longitudinal strain and strain rate that included singleton, non-anomalous pregnancies complicated by pregestational or gestational diabetes mellitus compared to uncomplicated pregnancies. The strain values were pooled per 4 weeks of gestation for meta-analysis using random-effects models.</p><p><strong>Results: </strong>Fifteen studies met the criteria, including 990 fetuses of diabetic mothers and 1,645 control fetuses. The study design was cross-sectional in fourteen studies and longitudinal in one study. Gestational age, type of diabetes, ultrasound device, and 2D-STE software varied between the studies. Glycemic control and type of treatment were often lacking. In fetuses of diabetic mothers versus healthy mothers, left ventricular strain was significantly decreased (7 studies), increased (1 study), or not significantly different (7 studies). Right ventricular strain was decreased (7 studies), increased (1 study), or not different (2 studies). Left ventricular strain rate was decreased (3 studies), increased (1 study), or not different (2 studies). Right ventricular strain rate was increased (1 study) or not different (2 studies).</p><p><strong>Conclusion: </strong>Fetuses of mothers with diabetes show evidence of systolic dysfunction, which is more visible in the right ventricle. Contradictory results are probably due to suboptimal study designs and variation in gestational age, diabetes severity, image acquisition, and software. Large prospective longitudinal studies are needed to assess fetal myocardial function with 2D-STE in pregestational diabetes mellitus type 1 and 2 and gestational diabetes mellitus pregnancies. The influence of glycemic control, BMI, and treatment should be evaluated.</p>","PeriodicalId":12189,"journal":{"name":"Fetal Diagnosis and Therapy","volume":null,"pages":null},"PeriodicalIF":1.6,"publicationDate":"2024-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141456116","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ömer Gökhan Eyisoy, Oya Demirci, Ümit Taşdemir, Mucize Özdemir, Aydın Öcal, Özge Kahramanoğlu
Introduction: The aim of this study was, first, to investigate the difference in fetal atrioventricular conduction in patients with and without intrahepatic cholestasis of pregnancy (ICP) by measuring the fetal PR interval; second, to evaluate the altering effect of ursodeoxycholic acid (UDCA) treatment on the fetal PR interval in ICP patients.
Methods: The study consisted of 42 ICP patients and 48 healthy pregnant women. Fetal echocardiography was performed to measure the mechanical PR interval. The fetal PR interval and the clinical characteristics were compared between the two groups. The effect of UDCA treatment on the fetal PR interval in ICP patients was evaluated.
Results: In ICP patients, significantly longer fetal PR intervals were observed than in the control group (123.21 ± 8.54 vs. 115.13 ± 5.95 ms, p < 0.001). In the ICP group, there was a positive correlation between the fetal PR interval and maternal fasting total bile acid (TBA) levels (r = 0.514, p = 0.001). After 1 week of treatment with UDCA in patients with ICP, the PR interval was shorter than before, although the reduction was not statistically significant (120.98 ± 6.70 vs. 123.21 ± 8.54 ms, p = 0.095). In patients with severe ICP (TBA >40 mmol/L, n = 10), a significant reduction in the fetal PR interval was observed after treatment with UDCA (127.5 ms [IQR, 118.0-134.75] before vs. 122 ms [IQR, 109.5-126.5] after, p = 0.037).
Conclusion: Fetal PR interval increased in ICP patients in correlation with maternal serum TBA concentration. Treatment with UDCA may have limited positive effects on the fetal AV conduction system. The beneficial effects of UDCA on the fetal PR interval may be more pronounced in patients with higher bile acid levels.
{"title":"Effect of Maternal Ursodeoxycholic Acid Treatment on Fetal Atrioventricular Conduction in Patients with Intrahepatic Cholestasis of Pregnancy.","authors":"Ömer Gökhan Eyisoy, Oya Demirci, Ümit Taşdemir, Mucize Özdemir, Aydın Öcal, Özge Kahramanoğlu","doi":"10.1159/000540261","DOIUrl":"10.1159/000540261","url":null,"abstract":"<p><strong>Introduction: </strong>The aim of this study was, first, to investigate the difference in fetal atrioventricular conduction in patients with and without intrahepatic cholestasis of pregnancy (ICP) by measuring the fetal PR interval; second, to evaluate the altering effect of ursodeoxycholic acid (UDCA) treatment on the fetal PR interval in ICP patients.</p><p><strong>Methods: </strong>The study consisted of 42 ICP patients and 48 healthy pregnant women. Fetal echocardiography was performed to measure the mechanical PR interval. The fetal PR interval and the clinical characteristics were compared between the two groups. The effect of UDCA treatment on the fetal PR interval in ICP patients was evaluated.</p><p><strong>Results: </strong>In ICP patients, significantly longer fetal PR intervals were observed than in the control group (123.21 ± 8.54 vs. 115.13 ± 5.95 ms, p < 0.001). In the ICP group, there was a positive correlation between the fetal PR interval and maternal fasting total bile acid (TBA) levels (r = 0.514, p = 0.001). After 1 week of treatment with UDCA in patients with ICP, the PR interval was shorter than before, although the reduction was not statistically significant (120.98 ± 6.70 vs. 123.21 ± 8.54 ms, p = 0.095). In patients with severe ICP (TBA >40 mmol/L, n = 10), a significant reduction in the fetal PR interval was observed after treatment with UDCA (127.5 ms [IQR, 118.0-134.75] before vs. 122 ms [IQR, 109.5-126.5] after, p = 0.037).</p><p><strong>Conclusion: </strong>Fetal PR interval increased in ICP patients in correlation with maternal serum TBA concentration. Treatment with UDCA may have limited positive effects on the fetal AV conduction system. The beneficial effects of UDCA on the fetal PR interval may be more pronounced in patients with higher bile acid levels.</p>","PeriodicalId":12189,"journal":{"name":"Fetal Diagnosis and Therapy","volume":null,"pages":null},"PeriodicalIF":1.6,"publicationDate":"2024-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141619756","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ladina Vonzun, Ladina Ruegg, Julia Zepf, Nele Strübing, Patrice Grehten, Martin Meuli, Luca Mazzone, Ueli Moehrlen, Nicole Ochsenbein-Koelble
Introduction: Reduced middle cerebral artery resistance indices (MCA-RI) in fetuses with spina bifida (fSB) are commonly observed. Compression of neuronal pathways in the brainstem due to hindbrain herniation (HH) and disturbed cerebrospinal fluid circulation likely cause an imbalance of the autonomic nervous system. This may increase systemic vasoconstriction and compensatory increase cerebral vasodilation (like brain sparing). The aim of this study was to systematically analyze all fetal MCA-RI before and after fSB repair and to compare their correlation with the presence and postsurgical resolution of HH.
Methods: 173 patients were included. Standardized ultrasound examinations including MCA and umbilical artery (UA) Doppler as well as assessment of HH presence and regression were performed. Fetuses with MCA-RI <5th percentile (P) before fetal surgery were compared to the group with normal MCA-RI and correlated to the presence of HH before and its regression after fSB repair.
Results: 30% (49/161) fetuses showed RI's <5th P before fSB repair. All fetuses had normal UA-RI. 99.4% of fetuses (160/161) showed normal of MCA-RI before delivery. Normalization occurred within a mean of 1.3 ± 1.2 weeks. HH regression was observed in 97% in the group with normal MCA-RI and in 96% in the group with MCA-RI <5th P before surgery (p = 0.59). Time lapse to HH regression after fSB repair was 1.8 ± 1.7 and 1.9 ± 1.6 weeks, respectively.
Conclusion: In fetuses with MCA-RIs <5 P before fSB repair, a parallel timely course of MCA-RI normalization and HH regression was noted. To suggest common pathogenic factor(s), more studies are needed. However, normalization of the fetal cerebral circulation could be a further benefit of fSB repair.
{"title":"Middle Cerebral Artery Doppler before and after Fetal Spina Bifida Repair: An Indirect Sign of Hindbrain Compression and Decompression?","authors":"Ladina Vonzun, Ladina Ruegg, Julia Zepf, Nele Strübing, Patrice Grehten, Martin Meuli, Luca Mazzone, Ueli Moehrlen, Nicole Ochsenbein-Koelble","doi":"10.1159/000539773","DOIUrl":"10.1159/000539773","url":null,"abstract":"<p><strong>Introduction: </strong>Reduced middle cerebral artery resistance indices (MCA-RI) in fetuses with spina bifida (fSB) are commonly observed. Compression of neuronal pathways in the brainstem due to hindbrain herniation (HH) and disturbed cerebrospinal fluid circulation likely cause an imbalance of the autonomic nervous system. This may increase systemic vasoconstriction and compensatory increase cerebral vasodilation (like brain sparing). The aim of this study was to systematically analyze all fetal MCA-RI before and after fSB repair and to compare their correlation with the presence and postsurgical resolution of HH.</p><p><strong>Methods: </strong>173 patients were included. Standardized ultrasound examinations including MCA and umbilical artery (UA) Doppler as well as assessment of HH presence and regression were performed. Fetuses with MCA-RI <5th percentile (P) before fetal surgery were compared to the group with normal MCA-RI and correlated to the presence of HH before and its regression after fSB repair.</p><p><strong>Results: </strong>30% (49/161) fetuses showed RI's <5th P before fSB repair. All fetuses had normal UA-RI. 99.4% of fetuses (160/161) showed normal of MCA-RI before delivery. Normalization occurred within a mean of 1.3 ± 1.2 weeks. HH regression was observed in 97% in the group with normal MCA-RI and in 96% in the group with MCA-RI <5th P before surgery (p = 0.59). Time lapse to HH regression after fSB repair was 1.8 ± 1.7 and 1.9 ± 1.6 weeks, respectively.</p><p><strong>Conclusion: </strong>In fetuses with MCA-RIs <5 P before fSB repair, a parallel timely course of MCA-RI normalization and HH regression was noted. To suggest common pathogenic factor(s), more studies are needed. However, normalization of the fetal cerebral circulation could be a further benefit of fSB repair.</p>","PeriodicalId":12189,"journal":{"name":"Fetal Diagnosis and Therapy","volume":null,"pages":null},"PeriodicalIF":1.6,"publicationDate":"2024-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141330699","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Joyce M Cheng, Ahmet A Baschat, Meredith A Atkinson, Mara Rosner, Michelle L Kush, Denise Wolfson, Sarah Olson, Kristin Voegtline, Lindsey Goodman, Angie C Jelin, Jena L Miller
Introduction: The optimal protocol for serial amnioinfusions to maintain amniotic fluid in pregnancies with early-onset fetal renal anhydramnios before 22 weeks is not known. We compared the performance of two different approaches.
Methods: A secondary analysis was conducted of serial amnioinfusions performed by a single center during the external pilot and feasibility phases of the Renal Anhydramnios Fetal Therapy (RAFT) trial. During the external pilot, higher amnioinfusion volumes were given less frequently; in the feasibility study, smaller volume amnioinfusions were administered more frequently. Procedural details, complications, and obstetric outcomes were compared between the two groups using Pearson's χ2 or Fisher's exact tests for categorical variables and Student's t tests or Wilcoxon rank-sum tests for continuous variables. The adjusted association between procedural details and chorioamniotic separation was obtained through a multivariate repeated measure logistic regression model.
Results: Eleven participants underwent 159 amnioinfusions (external pilot: 3 patients, 21 amnioinfusions; feasibility: 8 patients, 138 amnioinfusions). External pilot participants had fewer amnioinfusions (7 vs. 19.5 in the feasibility group, p = 0.04), larger amnioinfusion volume (750 vs. 500 mL, p < 0.01), and longer interval between amnioinfusions (6 [4-7] vs. 4 [3-5] days, p < 0.01). In the external pilot, chorioamniotic separation was more common (28.6% vs. 5.8%, p < 0.01), preterm prelabor rupture of membranes (PPROM) occurred sooner after amnioinfusion initiation (28 ± 21.5 vs. 75.6 ± 24.1 days, p = 0.03), and duration of maintained amniotic fluid between first and last amnioinfusion was shorter (38 ± 17.3 vs. 71 ± 19 days, p = 0.03), compared to the feasibility group. While delivery gestational age was similar (35.1 ± 1.7 vs. 33.8 ± 1.5 weeks, p = 0.21), feasibility participants maintained amniotic fluid longer.
Conclusion: Small volume serial amnioinfusions performed more frequently maintain normal amniotic fluid volume longer because of delayed occurrence of PPROM.
{"title":"Comparison of Serial Amnioinfusion Strategies for Isolated Early-Onset Fetal Renal Anhydramnios.","authors":"Joyce M Cheng, Ahmet A Baschat, Meredith A Atkinson, Mara Rosner, Michelle L Kush, Denise Wolfson, Sarah Olson, Kristin Voegtline, Lindsey Goodman, Angie C Jelin, Jena L Miller","doi":"10.1159/000539732","DOIUrl":"10.1159/000539732","url":null,"abstract":"<p><strong>Introduction: </strong>The optimal protocol for serial amnioinfusions to maintain amniotic fluid in pregnancies with early-onset fetal renal anhydramnios before 22 weeks is not known. We compared the performance of two different approaches.</p><p><strong>Methods: </strong>A secondary analysis was conducted of serial amnioinfusions performed by a single center during the external pilot and feasibility phases of the Renal Anhydramnios Fetal Therapy (RAFT) trial. During the external pilot, higher amnioinfusion volumes were given less frequently; in the feasibility study, smaller volume amnioinfusions were administered more frequently. Procedural details, complications, and obstetric outcomes were compared between the two groups using Pearson's χ2 or Fisher's exact tests for categorical variables and Student's t tests or Wilcoxon rank-sum tests for continuous variables. The adjusted association between procedural details and chorioamniotic separation was obtained through a multivariate repeated measure logistic regression model.</p><p><strong>Results: </strong>Eleven participants underwent 159 amnioinfusions (external pilot: 3 patients, 21 amnioinfusions; feasibility: 8 patients, 138 amnioinfusions). External pilot participants had fewer amnioinfusions (7 vs. 19.5 in the feasibility group, p = 0.04), larger amnioinfusion volume (750 vs. 500 mL, p < 0.01), and longer interval between amnioinfusions (6 [4-7] vs. 4 [3-5] days, p < 0.01). In the external pilot, chorioamniotic separation was more common (28.6% vs. 5.8%, p < 0.01), preterm prelabor rupture of membranes (PPROM) occurred sooner after amnioinfusion initiation (28 ± 21.5 vs. 75.6 ± 24.1 days, p = 0.03), and duration of maintained amniotic fluid between first and last amnioinfusion was shorter (38 ± 17.3 vs. 71 ± 19 days, p = 0.03), compared to the feasibility group. While delivery gestational age was similar (35.1 ± 1.7 vs. 33.8 ± 1.5 weeks, p = 0.21), feasibility participants maintained amniotic fluid longer.</p><p><strong>Conclusion: </strong>Small volume serial amnioinfusions performed more frequently maintain normal amniotic fluid volume longer because of delayed occurrence of PPROM.</p>","PeriodicalId":12189,"journal":{"name":"Fetal Diagnosis and Therapy","volume":null,"pages":null},"PeriodicalIF":1.6,"publicationDate":"2024-06-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141300472","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Steven T Papastefan, Daniel R Liesman, Katherine C Ott, Federico Scorletti, Xavier F Pombar, Aimen F Shaaban, Amir M Alhajjat
Introduction: Fetal thoracoamniotic shunts are common lifesaving interventions but frequently require replacement. Needle fetal thoracoscopy is a technique that uses standard thoracoamniotic shunt introducer sheaths to permit direct visualization and even instrument manipulation during shunt deployment to facilitate optimal positioning and primary shunt function in the most challenging cases.
Case presentation: In this study, 5 patients who underwent needle fetal thoracoscopy-assisted thoracoamniotic shunt placement were reviewed. Three patients with large, macrocystic congenital pulmonary airway malformations (CPAMs) with evidence of worsening mediastinal shift and/or hydrops and 2 patients with large chylothorax with fetal hydrops were treated. Four cases had previous shunts that failed due to poor sonographic visualization during initial placement, cyst septations, shunt obstruction, or dislodgment. Needle fetal thoracoscopy was used to disrupt cyst walls and septations, clear hematoma, and confirm the optimal initial position of the shunt. In this series, 1 severe CPAM patient with a short cervix developed preterm labor postoperatively resulting in neonatal demise. The remaining 4 patients experienced resolution of hydrops and progressed to successful delivery with excellent neonatal outcomes.
Conclusion: Needle fetal thoracoscopy is a procedure that may be selectively deployed in challenging thoracoamniotic shunt cases impacted by recurrent failure, poor sonographic windows, and challenging fetal positioning.
{"title":"Needle Fetal Thoracoscopy: A Technique to Assist with Ultrasound-Guided Placement of Challenging Thoracoamniotic Shunts.","authors":"Steven T Papastefan, Daniel R Liesman, Katherine C Ott, Federico Scorletti, Xavier F Pombar, Aimen F Shaaban, Amir M Alhajjat","doi":"10.1159/000539274","DOIUrl":"10.1159/000539274","url":null,"abstract":"<p><strong>Introduction: </strong>Fetal thoracoamniotic shunts are common lifesaving interventions but frequently require replacement. Needle fetal thoracoscopy is a technique that uses standard thoracoamniotic shunt introducer sheaths to permit direct visualization and even instrument manipulation during shunt deployment to facilitate optimal positioning and primary shunt function in the most challenging cases.</p><p><strong>Case presentation: </strong>In this study, 5 patients who underwent needle fetal thoracoscopy-assisted thoracoamniotic shunt placement were reviewed. Three patients with large, macrocystic congenital pulmonary airway malformations (CPAMs) with evidence of worsening mediastinal shift and/or hydrops and 2 patients with large chylothorax with fetal hydrops were treated. Four cases had previous shunts that failed due to poor sonographic visualization during initial placement, cyst septations, shunt obstruction, or dislodgment. Needle fetal thoracoscopy was used to disrupt cyst walls and septations, clear hematoma, and confirm the optimal initial position of the shunt. In this series, 1 severe CPAM patient with a short cervix developed preterm labor postoperatively resulting in neonatal demise. The remaining 4 patients experienced resolution of hydrops and progressed to successful delivery with excellent neonatal outcomes.</p><p><strong>Conclusion: </strong>Needle fetal thoracoscopy is a procedure that may be selectively deployed in challenging thoracoamniotic shunt cases impacted by recurrent failure, poor sonographic windows, and challenging fetal positioning.</p>","PeriodicalId":12189,"journal":{"name":"Fetal Diagnosis and Therapy","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-05-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140916026","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Stephanie A Eyerly-Webb, Lisa Howley, Clifton O Brock, Joseph B Lillegard, James Fisher, Benjamin Reynolds, Emily F Barthel, Eric Dion, Saul Snowise
INTRODUCTION No evidence-based protocols exist for fetal cardiac monitoring during fetoscopic myelomeningocele (fMMC) repair and intraprocedural spectral Doppler data is limited. We determined the feasibility of continuous fetal echocardiography during fMMC repair and correlated Doppler changes with qualitative fetal cardiac function during each phase of fMMC repair. METHODS Patients undergoing fMMC repair had continuous fetal echocardiography interpreted in real-time by pediatric cardiology. Fetal data included fetal heart rate (FHR), qualitative cardiac function, mitral and tricuspid valve inflow waveforms, and umbilical artery (UA), umbilical vein (UV), ductus arteriosus (DA) and ductus venosus (DV) Dopplers. RESULTS UA abnormalities were noted in 14/25 patients, UV abnormalities were observed in two patients, and DV and DA abnormalities were each noted in 4 patients. Qualitative cardiac function was normal for all patients with the exception of one with isolated left ventricular dysfunction during myofascial flap creation, concurrent with an abnormal UA flow pattern. All abnormalities resolved by the first postoperative day. CONCLUSIONS Continuous fetal echocardiography was feasible during all fMMC repairs. Spectral Doppler changes in the UA were common during fMMC procedures but qualitative cardiac dysfunction was rare. Abnormalities in the UV, DV and DA Dopplers, FHR, and cardiac function were less common findings.
引言 胎儿镜下脊髓空洞症(fMMC)修复术中的胎儿心脏监测尚无循证方案,且术中频谱多普勒数据有限。我们确定了胎儿髓母细胞瘤修补术中连续胎儿超声心动图的可行性,并将多普勒变化与胎儿髓母细胞瘤修补术各阶段的胎儿心功能定性相关联。胎儿数据包括胎心率(FHR)、定性心功能、二尖瓣和三尖瓣血流波形以及脐动脉(UA)、脐静脉(UV)、动脉导管(DA)和静脉导管(DV)多普勒。除一名患者在肌筋膜瓣形成过程中出现孤立性左心室功能障碍,同时出现 UA 血流模式异常外,所有患者的心功能均正常。所有异常均在术后第一天消除。胎儿超声心动图检查在所有胎儿超声心动图修补术中都是可行的。胎儿超声心动图检查过程中,UA 的频谱多普勒变化很常见,但定性心功能异常却很少见。UV、DV和DA多普勒、FHR和心功能异常是较少见的发现。
{"title":"Continuous Fetal Cardiac Monitoring During Fetoscopic Myelomeningocele Repair and Relationship to Spectral Doppler Changes.","authors":"Stephanie A Eyerly-Webb, Lisa Howley, Clifton O Brock, Joseph B Lillegard, James Fisher, Benjamin Reynolds, Emily F Barthel, Eric Dion, Saul Snowise","doi":"10.1159/000538122","DOIUrl":"https://doi.org/10.1159/000538122","url":null,"abstract":"INTRODUCTION\u0000No evidence-based protocols exist for fetal cardiac monitoring during fetoscopic myelomeningocele (fMMC) repair and intraprocedural spectral Doppler data is limited. We determined the feasibility of continuous fetal echocardiography during fMMC repair and correlated Doppler changes with qualitative fetal cardiac function during each phase of fMMC repair.\u0000\u0000\u0000METHODS\u0000Patients undergoing fMMC repair had continuous fetal echocardiography interpreted in real-time by pediatric cardiology. Fetal data included fetal heart rate (FHR), qualitative cardiac function, mitral and tricuspid valve inflow waveforms, and umbilical artery (UA), umbilical vein (UV), ductus arteriosus (DA) and ductus venosus (DV) Dopplers.\u0000\u0000\u0000RESULTS\u0000UA abnormalities were noted in 14/25 patients, UV abnormalities were observed in two patients, and DV and DA abnormalities were each noted in 4 patients. Qualitative cardiac function was normal for all patients with the exception of one with isolated left ventricular dysfunction during myofascial flap creation, concurrent with an abnormal UA flow pattern. All abnormalities resolved by the first postoperative day.\u0000\u0000\u0000CONCLUSIONS\u0000Continuous fetal echocardiography was feasible during all fMMC repairs. Spectral Doppler changes in the UA were common during fMMC procedures but qualitative cardiac dysfunction was rare. Abnormalities in the UV, DV and DA Dopplers, FHR, and cardiac function were less common findings.","PeriodicalId":12189,"journal":{"name":"Fetal Diagnosis and Therapy","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-04-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140681952","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Lorena María Sebastián de Lucas, Polán Ordás Álvarez, Laura de Castro Marzo, Tamara Illescas Molina, Beatriz Herrero, J. L. Bartha, Eugenia Antolín
INTRODUCTION Non-immune hydrops fetalis (NIHF) is the most frequent etiology of hydrops fetalis (HF), accounting for around 95% of cases. It associates high perinatal mortality and morbidity rates. The aim of the study was firstly, to investigate etiology, prenatal management, and perinatal outcome in a large single center series of HF; secondly, to identify prenatal prognostic factors with impact on perinatal outcome. MATERIAL AND METHODS Observational retrospective study of 80 HF diagnosed or referred to a single tertiary center between 2012 and 2021. Clinical characteristics, etiology, prenatal management, and perinatal outcome were recorded. Adverse perinatal outcome was defined as intrauterine fetal death (IUFD), early neonatal death (first 7 days of life) and late neonatal death (between 7 and 28 days). RESULTS Seventy-six of the 80 cases (95%) were NIHF, main etiology being genetic disorders (28/76; 36.8%). A total of 26 women (32.5%) opted for termination of pregnancy, all of them in the NIHF group. Intrauterine fetal death (IUFD) occurred in 24 of 54 patients (44.4%) who decided to continue the pregnancy. Intrauterine treatment was performed in 29 cases (53.7%). There were 30 newborns (55.6%). Adverse perinatal outcome rate was 53.7% (29/54), significantly higher in those diagnosed < 20 weeks of gestation (82.4% <20w vs. 40.5% ≥ 20w; p=0.004). Survival rate was higher when fetal therapy was performed compared to the expectantly managed group (58.6% vs. 32%; p=0.05). Intrauterine blood transfusion and thoraco-amniotic shunt were the procedures that achieved the highest survival rates (88.9% and 100% respectively, p=0.003). CONCLUSION NIHF represented 95% of HF with genetic disorders as the main etiology. Most of them were diagnosed before 20 weeks of gestation, with worse prognosis than cases detected later in gestation. Rates of TOP, IUFD and early neonatal death were higher in NIHF. Intrauterine therapy when indicated, improved the perinatal outcome.
{"title":"Prenatal management and perinatal outcome in a large series of hydrops fetalis.","authors":"Lorena María Sebastián de Lucas, Polán Ordás Álvarez, Laura de Castro Marzo, Tamara Illescas Molina, Beatriz Herrero, J. L. Bartha, Eugenia Antolín","doi":"10.1159/000538857","DOIUrl":"https://doi.org/10.1159/000538857","url":null,"abstract":"INTRODUCTION\u0000Non-immune hydrops fetalis (NIHF) is the most frequent etiology of hydrops fetalis (HF), accounting for around 95% of cases. It associates high perinatal mortality and morbidity rates. The aim of the study was firstly, to investigate etiology, prenatal management, and perinatal outcome in a large single center series of HF; secondly, to identify prenatal prognostic factors with impact on perinatal outcome.\u0000\u0000\u0000MATERIAL AND METHODS\u0000Observational retrospective study of 80 HF diagnosed or referred to a single tertiary center between 2012 and 2021. Clinical characteristics, etiology, prenatal management, and perinatal outcome were recorded. Adverse perinatal outcome was defined as intrauterine fetal death (IUFD), early neonatal death (first 7 days of life) and late neonatal death (between 7 and 28 days).\u0000\u0000\u0000RESULTS\u0000Seventy-six of the 80 cases (95%) were NIHF, main etiology being genetic disorders (28/76; 36.8%). A total of 26 women (32.5%) opted for termination of pregnancy, all of them in the NIHF group. Intrauterine fetal death (IUFD) occurred in 24 of 54 patients (44.4%) who decided to continue the pregnancy. Intrauterine treatment was performed in 29 cases (53.7%). There were 30 newborns (55.6%). Adverse perinatal outcome rate was 53.7% (29/54), significantly higher in those diagnosed < 20 weeks of gestation (82.4% <20w vs. 40.5% ≥ 20w; p=0.004). Survival rate was higher when fetal therapy was performed compared to the expectantly managed group (58.6% vs. 32%; p=0.05). Intrauterine blood transfusion and thoraco-amniotic shunt were the procedures that achieved the highest survival rates (88.9% and 100% respectively, p=0.003).\u0000\u0000\u0000CONCLUSION\u0000NIHF represented 95% of HF with genetic disorders as the main etiology. Most of them were diagnosed before 20 weeks of gestation, with worse prognosis than cases detected later in gestation. Rates of TOP, IUFD and early neonatal death were higher in NIHF. Intrauterine therapy when indicated, improved the perinatal outcome.","PeriodicalId":12189,"journal":{"name":"Fetal Diagnosis and Therapy","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-04-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140680472","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Introduction CHARGE syndrome is an autosomal dominant genetic disorder with known pattern of features. The aim of the study was to present the fetal features of CHARGE syndrome to gain awareness that the antenatal characteristics can be very nonspecific. Case Presentation This was a retrospective study of 13 cases with CHARGE syndrome diagnosed by prenatal or postnatal genetic testing and physical examination. Two (15.4%; 2/13) had normal ultrasound scans during pregnancy. One (7.7%; 1/13) with first-trimester cystic hygroma presented intrauterine fetal demise (IUFD) at 16 weeks gestation. The remaining 10 (76.9%; 10/13) cases had abnormal ultrasound features in utero; among these, 1 had an increased nuchal translucency in the first trimester, 5 had second-trimester abnormal ultrasounds including micrognathia, cardiac defects and facial defects, and 4 third-trimester abnormal ultrasounds including micrognathia, isolated fetal growth restriction and polyhydramnios. Among the 11 cases with abnormal prenatal ultrasound scans, no fetus could reach the diagnostic criteria of CHARGE syndrome if only based on the results of ultrasound. However, the diagnosis was made in all cases when CHD7 defects were detected. Discussion/Conclusion The CHARGE syndrome presents non-specific abnormal ultrasound markers in utero. Exome sequencing in the genetic work up will aid in prenatal diagnosis of this syndrome.
{"title":"Fetal phenotype of CHARGE syndrome with a molecular confirmation: a series of 13 cases.","authors":"Qiuxia Yu, L. Zhen, Dong-Zhi Li","doi":"10.1159/000538949","DOIUrl":"https://doi.org/10.1159/000538949","url":null,"abstract":"Introduction CHARGE syndrome is an autosomal dominant genetic disorder with known pattern of features. The aim of the study was to present the fetal features of CHARGE syndrome to gain awareness that the antenatal characteristics can be very nonspecific. Case Presentation This was a retrospective study of 13 cases with CHARGE syndrome diagnosed by prenatal or postnatal genetic testing and physical examination. Two (15.4%; 2/13) had normal ultrasound scans during pregnancy. One (7.7%; 1/13) with first-trimester cystic hygroma presented intrauterine fetal demise (IUFD) at 16 weeks gestation. The remaining 10 (76.9%; 10/13) cases had abnormal ultrasound features in utero; among these, 1 had an increased nuchal translucency in the first trimester, 5 had second-trimester abnormal ultrasounds including micrognathia, cardiac defects and facial defects, and 4 third-trimester abnormal ultrasounds including micrognathia, isolated fetal growth restriction and polyhydramnios. Among the 11 cases with abnormal prenatal ultrasound scans, no fetus could reach the diagnostic criteria of CHARGE syndrome if only based on the results of ultrasound. However, the diagnosis was made in all cases when CHD7 defects were detected. Discussion/Conclusion The CHARGE syndrome presents non-specific abnormal ultrasound markers in utero. Exome sequencing in the genetic work up will aid in prenatal diagnosis of this syndrome.","PeriodicalId":12189,"journal":{"name":"Fetal Diagnosis and Therapy","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-04-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140693853","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
A. Phillips, R. Punn, Claudia Algaze, Y. Blumenfeld, Valerie Y. Chock, David M Kwiatokowski, Amy Quirin, Theresa A Tacy, Kelly Thorson, S. Maskatia
INTRODUCTION Neonatal presentation of coarctation of the aorta (CoA) is a potentially life-threatening condition that is difficult to diagnose in fetal life. We therefore sought to validate and compare novel metrics that may add diagnostic value for fetal coarctation of the aorta, including the diastolic to systolic aortic isthmus VTI ratio (VTId:VTIs), ascending aorta to descending aorta angle (AAo-DAo), transverse aorta to descending aorta angle (TAo-DAo), and LV longitudinal strain (LVS). Then to evaluate whether these novel metrics improve specificity to identify fetuses at the highest risk for postnatal coarctation of the aorta (CoA) without compromising sensitivity. METHODS Retrospective cohort study of fetuses followed a prospective clinical pathway and previously classified as mild, moderate, or high-risk for CoA based on standard fetal echo metrics. Novel metrics were retrospectively measured in a blinded manner. RESULTS Among fetuses with prenatal concern for coarctation of the aorta, VTId:VTIs, AAo-DAo angle, TAo-DAo angle, and LVS were significantly different between surgical and non-surgical cases (p <0.01 for all variables). In the subgroup of moderate- and high-risk fetuses, the standard high-risk criteria (flow reversal at the foramen ovale or aortic arch) did not discriminate effectively between surgical and non-surgical cases. VTId:VTIs, AAo-Dao angle, Tao-DAo angle, and LVS all demonstrated greater discrimination than standard high-risk criteria, with specificity of 100% and PPV (positive predictive value) of 78-100%. CONCLUSIONS The incorporation of novel metrics added diagnostic value to our clinical pathway for fetal CoA with higher specificity than the previous high-risk criteria. The incorporation of these metrics into the evaluation of fetuses at moderate- or high-risk for surgical CoA may improve prenatal counseling, allow for more consistent surgical planning, and ultimately optimize hospital resource allocation.
{"title":"Left ventricular strain, arch angulation, and velocity-time integral ratio improve performance of a clinical pathway for fetal diagnosis of neonatal coarctation of the aorta.","authors":"A. Phillips, R. Punn, Claudia Algaze, Y. Blumenfeld, Valerie Y. Chock, David M Kwiatokowski, Amy Quirin, Theresa A Tacy, Kelly Thorson, S. Maskatia","doi":"10.1159/000538550","DOIUrl":"https://doi.org/10.1159/000538550","url":null,"abstract":"INTRODUCTION\u0000Neonatal presentation of coarctation of the aorta (CoA) is a potentially life-threatening condition that is difficult to diagnose in fetal life. We therefore sought to validate and compare novel metrics that may add diagnostic value for fetal coarctation of the aorta, including the diastolic to systolic aortic isthmus VTI ratio (VTId:VTIs), ascending aorta to descending aorta angle (AAo-DAo), transverse aorta to descending aorta angle (TAo-DAo), and LV longitudinal strain (LVS). Then to evaluate whether these novel metrics improve specificity to identify fetuses at the highest risk for postnatal coarctation of the aorta (CoA) without compromising sensitivity.\u0000\u0000\u0000METHODS\u0000Retrospective cohort study of fetuses followed a prospective clinical pathway and previously classified as mild, moderate, or high-risk for CoA based on standard fetal echo metrics. Novel metrics were retrospectively measured in a blinded manner.\u0000\u0000\u0000RESULTS\u0000Among fetuses with prenatal concern for coarctation of the aorta, VTId:VTIs, AAo-DAo angle, TAo-DAo angle, and LVS were significantly different between surgical and non-surgical cases (p <0.01 for all variables). In the subgroup of moderate- and high-risk fetuses, the standard high-risk criteria (flow reversal at the foramen ovale or aortic arch) did not discriminate effectively between surgical and non-surgical cases. VTId:VTIs, AAo-Dao angle, Tao-DAo angle, and LVS all demonstrated greater discrimination than standard high-risk criteria, with specificity of 100% and PPV (positive predictive value) of 78-100%.\u0000\u0000\u0000CONCLUSIONS\u0000The incorporation of novel metrics added diagnostic value to our clinical pathway for fetal CoA with higher specificity than the previous high-risk criteria. The incorporation of these metrics into the evaluation of fetuses at moderate- or high-risk for surgical CoA may improve prenatal counseling, allow for more consistent surgical planning, and ultimately optimize hospital resource allocation.","PeriodicalId":12189,"journal":{"name":"Fetal Diagnosis and Therapy","volume":null,"pages":null},"PeriodicalIF":2.2,"publicationDate":"2024-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140699297","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}