Fetal 24-Segment Sphericity Index and Ventricular Fractional Shortening Between 12 and 20 Weeks of Gestation.

BACKGROUND: Although the fetal sphericity index (SI) and fractional shortening (FS) of 24 transverse segments have been previously reported after the 20th gestational week, there have been no reports during the first and early second trimester. OBJECTIVE: This study aimed to clarify the SI and FS of 24 transverse segments in normal fetuses before the 20th gestational week. METHODS: A total of 101 normal fetuses aged between 12 and 20 gestational weeks were examined. The displacement of the ventricular endocardium during the cardiac cycle was computed using speckle-tracking software (GE Healthcare, Milwaukee, WI). We analyzed the length of 24 end-diastolic lateral segments and the end-diastolic basal (seg1-6)- middle (Seg7-15)-apical (Seg16-24) distribution from the base to the apex of each ventricle, according to the method described by DeVore et al. The SI and FS were computed for each of the 24 segments by dividing the mid-basal-apical length by the transverse size. RESULTS: The SI for each segment was independent of the gestational age. The SI of the right ventricle was significantly lower than that of the left ventricle for segments 1-14, suggesting that the right ventricle was more spherical than the left ventricle in the basal segment only. The FS of the right ventricle was significantly lower than that of the left ventricle in segments 1 to 2 and 13 to 24. CONCLUSION: The morphology of the ventricles before 20 weeks of gestation differs from that between 20 and 40 weeks of gestation. This difference may be related to myocardial densification or performance.

Growing Coronary Aneurysm Secondary to Coronary Fistula Despite Decreased Pulmonary Blood Flow/Systemic Blood Flow Ratio in a Child: A Case Report.

Coronary aneurysm secondary to coronary fistula is a rare condition, with no existing report on its pathological examination. We report the case of a patient diagnosed with a right coronary artery fistula with coronary aneurysm during the fetal period. During follow-up after delivery, the aneurysm became larger, even though the shunt size decreased. We were afraid the aneurysm would rupture and therefore, planned elective catheter embolization. At the age of 4 years, the patient underwent surgery, which involved closing the fistula and making the lumen of the aneurysm smaller. However, the surgery was not catheter embolization as planned because segment 3 branched off from the largest aneurysm where we planned to embolize. Pathologically, the structure of the coronary artery differed from that of a healthy one, with thickened intima and media, fewer scattered smooth muscle cells, widely distributed elastic fibers, and mucoid degeneration in the media. The structure of the coronary artery suggested that the vessel wall was weak and that the aneurysm would rupture if not treated. Postoperative coronary angiography showed that segment 2 was obstructed, while the collaterals from the left coronary artery perfused the area. We could have treated the fistula with a catheter as scheduled.

A predictive scoring system for small diaphragmatic defects in infants with congenital diaphragmatic hernia.

PURPOSE: To develop a predictive score for small diaphragmatic defects in infants with congenital diaphragmatic hernia (CDH) for determining thoracoscopic surgery indication. METHODS: The Japanese CDH Study Group cohort was randomly divided into derivation (n = 397) and validation (n = 396) datasets. Using logistic regression, a prediction model and weighted scoring system for small diaphragmatic defects were created from derivation dataset and validated with validation dataset. RESULTS: Six weighted variables were selected: no hydramnios, 1 point; 1 min Apgar score of 5-10, 1 point; apex type of the lung (left lung is detected radiographically in apex area), 1 point; oxygenation index < 8, 1 point; abdominal nasogastric tube (tip of the nasogastric tube is detected radiographically in the abdominal area), 2 points; no right-to-left flow of ductus arteriosus, 1 point. In validation dataset, rates of small diaphragmatic defects for Possible (0-3 points), Probable (4-5 points), and Definite (6-7 points) groups were 36%, 81%, and 94%, respectively (p < 0.001). Additionally, sensitivity, specificity, positive predictive value, and C statistics were 0.78, 0.79, 0.88, 0.76, and 0.45, 0.94, 0.94, 0.70 for Probable and Definite groups, respectively. CONCLUSION: Our scoring system effectively predicted small diaphragmatic defects in infants with CDH.

Prenatal predictors of mortality in fetuses with congenital diaphragmatic hernia: a systematic review and meta-analysis.

PURPOSE: This study aimed to evaluate prenatal predictors of mortality in fetuses with congenital diaphragmatic hernia (CDH). METHODS: A systematic literature search was performed to identify relevant observational studies that evaluated the ability of lung-to-head ratio (LHR), observed-to-expected LHR (o/e-LHR), observed-to-expected total fetal lung volume (o/e-TFLV), lung-to-thorax transverse area ratio (L/T ratio), intrathoracic herniation of the liver and the stomach, and side of diaphragmatic hernia, using a threshold for the prediction of mortality in fetuses with CDH. Study quality was assessed using the QUADAS-2 tool. Hierarchical summary receiver operating characteristic curves were constructed. RESULTS: A total of 50 articles were included in this meta-analysis. The QUADAS-2 tool identified a high risk of bias in more than one domain scored in all parameters. Among those parameters, the diagnostic odds ratio of mortality with o/e-LHR < 25%, o/e-TFLV < 25%, and L/T ratio < 0.08 were 11.98 [95% confidence interval (CI) 4.65-30.89], 11.14 (95% CI 5.19-23.89), and 10.28 (95% CI 3.38-31.31), respectively. The predictive values for mortality were similar between the presence of liver herniation and retrocardiac fetal stomach position. CONCLUSIONS: This systematic review suggests that o/e-LHR, o/e-TFLV, and L/T ratio are equally good predictors of neonatal mortality in fetuses with isolated CDH.

The efficacy of the postnatal nasogastric tube position as a prognostic marker of left-sided isolated congenital diaphragmatic hernia.

PURPOSE: The prenatal diagnosis of the stomach position in congenital diaphragmatic hernia (CDH) has been a reliable prognostic factor, but few studies have focused on the postnatal position. We therefore evaluated the significance of the nasogastric (NG) tube position just after birth. METHODS: The Japanese CDH Study Group database enrolled 1037 CDH neonates over 15 years. In our multicenter retrospective study, 464 cases of left-sided isolated CDH with prenatal diagnoses were divided into two groups: NG tube below the diaphragm (BD; n = 190) or above the diaphragm (AD; n = 274). The primary outcome was the 90-day survival rate, and the secondary outcomes were mechanical ventilation duration, hospitalization duration, and recurrence rate. RESULTS: The BD group had a significantly higher 90-day survival rate (98.4 vs. 89.4%, p < 0.001), shorter mechanical ventilation (11 vs. 19 days, p < 0.001), shorter hospitalization (38 vs. 59 days, p < 0.001), and lower recurrence rate (p = 0.002) than the AD group. A multivariate analysis showed that BD (adjusted odds ratio, 3.68; 95% confidence interval 1.02-13.30) was a favorable prognostic factor for the 90-day survival. CONCLUSION: The assessment of the NG tube position revealed it to be a reliable prognostic factor of left-sided isolated CDH. Therefore, it should be included as a routine assessment.

The prenatal diagnosis of a rare circular shunt with absent pulmonary valve syndrome.

Absent pulmonary valve syndrome (APVS) with tricuspid atresia is very rare. We report a case of APVS in which the fetus died due to severe hydrops fetalis. In addition to weakened cardiac contraction due to left ventricular noncompaction cardiomyopathy, a circular shunt through a ventricular septal defect exacerbated heart failure. Although APVS with tricuspid valve atresia is not supposed to cause a circular shunt, we herein present the echocardiographic demonstration of a case of circular shunt.

The telediagnosis of double aortic arch using spatio-temporal image correlation.

We diagnosed two cases of double aortic arch (DAA) remotely. In both cases, the right aortic arch was suspected, and a remote diagnosis was requested. We performed a remote diagnosis by the spatio-temporal image correlation (STIC) method. DAA was diagnosed by detecting the left aortic arch in an axial transverse section in the three-vessel trachea view. Since the STIC method can make multiple cross sections, making a diagnosis by the STIC method was useful for the remote diagnosis of these patients. In particular, HD flow render mode clearly showed left aortic arch, so it was possible to diagnose DAA.

Thoracoscopic repair of congenital diaphragmatic hernia in neonates: findings of a multicenter study in Japan.

PURPOSE: We compared the efficacy of thoracoscopic repair (TR) with that of open repair (OR) for neonatal congenital diaphragmatic hernia (CDH). METHODS: The subjects of this multicenter retrospective cohort study were 524 infants with left-sided isolated CDH, diagnosed prenatally, and treated at one of 15 participating hospitals in Japan between 2006 and 2018. The outcomes of infants who underwent TR and those who underwent OR were compared, applying propensity score matching. RESULTS: During the study period, 57 infants underwent TR and 467 underwent OR. Ten of the infants who underwent TR required conversion to OR for technical difficulties and these patients were excluded from the analysis. The survival rate at 180 days was similar in both groups (TR 98%; OR 93%). Recurrence developed after TR in 3 patients and after OR in 15 patients (TR 7%, OR 3%, p = 0.40). The propensity score was calculated using the following factors related to relevance of the surgical procedure: prematurity (p = 0.1), liver up (p < 0.01), stomach position (p < 0.01), and RL shunt (p = 0.045). After propensity score matching, the multivariate analysis adjusted for severity classification and age at surgical treatment revealed a significantly shorter hospital stay (odds ratio 0.50) and a lower incidence of chronic lung disease (odds ratio 0.39) in the TR group than in the OR group. CONCLUSIONS: TR can be performed safely for selected CDH neonates with potentially better outcomes than OR.

Optimal timing of surgery in infants with prenatally diagnosed isolated left-sided congenital diaphragmatic hernia: a multicenter, cohort study in Japan.

PURPOSE: To date, there is no compelling evidence of the optimal timing of surgery for congenital diaphragmatic hernia (CDH). This study aimed to establish the optimal timing of surgery in neonates with isolated left-sided CDH. METHODS: This multicenter cohort study enrolled 276 patients with isolated left-sided CDH at 15 institutions. Based on the timing of surgery, the patients were classified into four groups [< 24 h (G1), 24-47 h (G2), 48-71 h (G3), and ≥ 72 h (G4)]. The clinical outcomes were compared among the groups using a logistic regression model, after adjusting for potential confounders, such as disease severity. RESULTS: Multivariate analyses showed that G2 also had a lower mortality rate than the other groups. In mild and severe cases, there were no significant differences in mortality across the four groups. In moderate cases, G2 resulted in significantly increased survival rates, compared with G1. CONCLUSION: The study results suggest that surgery within 24 h of birth is not recommended for patients with moderate severity CDH, that there is no benefit in the delay of surgery for more than 72 h in mild severity CDH patients, and that there is no definite optimal time to perform surgery in severe cases of CDH.

Best pre-ductal PaO2 prior to extracorporeal membrane oxygenation as predictor of mortality in patients with congenital diaphragmatic hernia: a retrospective analysis of a Japanese database.

PURPOSE: Predicting lethal pulmonary hypoplasia in infants with congenital diaphragmatic hernia (CDH) before extracorporeal membrane oxygenation (ECMO) initiation is difficult. This study aimed to predict lethal pulmonary hypoplasia in patients with CDH prior to ECMO. METHODS: This was a multicenter cohort study involving neonates prenatally diagnosed with isolated unilateral CDH (born 2006-2020). Patients who required ECMO due to respiratory insufficiency were included in this study. Patients who underwent ECMO due to transient disorders were excluded from analysis. Blood gas analysis data within 24 h of birth were compared between survivors and non-survivors. Predictive abilities were assessed for factors with significant differences. RESULTS: Overall, 34 patients were included (18 survivors and 16 non-survivors). The best pre-ductal PaO2 was significantly lower in non-survivors than in survivors (50.4 [IQR 30.3-64.5] vs. 67.5 [IQR 52.4-103.2] mmHg, respectively; p = 0.047). A cutoff PaO2 of 42.9 mmHg had a sensitivity, specificity, and positive predictive value of 50.0%, 94.4%, and 88.9%, respectively, to predict mortality. CONCLUSION: The best PaO2 within 24 h after birth predicted mortality following ECMO initiation. This should be shared to families and caregivers to optimize the best interests of the infants with CDH.

The Evaluation of Fetal Cardiac Remote Screening in the Second Trimester of Pregnancy Using the Spatio-Temporal Image Correlation Method.

We prospectively performed remote fetal cardiac screening using the spatio-temporal image correlation (STIC), and examined the usefulness and problems of remote screening. We performed heart screening for all pregnant women at four obstetrics clinics over the three years from 2009 to 2014. The STIC data from 15,404 examinations in normal pregnancies (16-27 weeks, median 25 weeks) were analyzed. Obstetricians and sonographer collected STIC data from four-chamber view images. Eight pediatric cardiologists analyzed the images offline. A normal heart was diagnosed in 14,002 cases (90.9%), an abnormal heart was diagnosed in 457 cases (3.0%), and poor images were obtained in 945 cases (6.1%). 138 cases had congenital heart disease (CHD) after birth, and severe CHD necessitating hospitalization occurred in 36 cases. We were not able to detect CHD by screening in 12 cases. The sensitivity and specificity of STIC in CHD screening was 50% and 99.5%, respectively. The sensitivity and specificity of STIC in screening for severe CHD was 82% and 99.9%, respectively. The STIC method was useful in fetal remote screening for CHD. However, the fact that > 10% of images that could not be analyzed by this method was a problem.

Risk factors for pneumothorax associated with isolated congenital diaphragmatic hernia: results of a Japanese multicenter study.

PURPOSE: This study aimed to elucidate the clinical characteristics of neonates with congenital diaphragmatic hernia (CDH) associated with pneumothorax and evaluate the risk factors for the development of pneumothorax. METHODS: A retrospective cohort study was conducted in the 15 institutions participating in the Japanese CDH Study Group. A total of 495 neonates with isolated CDH who were born between 2011 and 2018 were analyzed in this study. RESULTS: Among the 495 neonates with isolated CDH, 52 (10.5%) developed pneumothorax. Eighteen (34.6%) patients developed pneumothorax before surgery, while 34 (65.4%) developed pneumothorax after surgery. The log-rank test showed that the cumulative survival rate was significantly lower in patients with pneumothorax than in those without pneumothorax. Univariate analysis revealed significant differences between patients with pneumothorax and those without pneumothorax with regard to the best oxygenation index within 24 h after birth, mean airway pressure (MAP) higher than 16 cmH2O, diaphragmatic defect size, and need for patch closure. Multiple logistic regression analysis indicated that only the MAP was associated with an increased risk of pneumothorax. CONCLUSIONS: The cumulative survival rate was significantly lower in isolated CDH patients with pneumothorax than in those without pneumothorax. A higher MAP was a risk factor for pneumothorax in CDH patients.

Evaluation of the post-LA space index in the normal fetus.

OBJECTIVE: We previously reported the post-LA space index (PLAS index), which is calculated as left atrial-descending aorta distance (LD) divided by the diameter of descending aorta (DA), could be useful for prenatal diagnosis of total anomalous pulmonary venous connection (TAPVC). In this study, we evaluated PLAS index in normal fetuses to assess its usefulness. METHODS: In 304 normal fetuses, LD and DA were retrospectively measured, and the PLAS index was calculated. In 206 fetuses with data on the biparietal diameter (BPD) and femoral length (FL), the relationship between the PLAS index and them was investigated. We also calculated the PLAS index in 13 TAPVC fetuses. RESULTS: Mean LD was 3.0+/-0.94 mm, mean DA was 4.8+/-0.87 mm, and the mean PLAS index was 0.62+/-0.19. DA and LD were correlated with gestational age (R = 0.52, 0.25), while the PLAS index showed little variation with gestational age (R < 0.2). BPD and FL were correlated with DA (R = 0.4, 0.42) but not with LD or the PLAS index (both R < 0.2). In the TAPVC fetuses, both LD and the PLAS index were significantly higher than in normal fetuses. CONCLUSION: In normal fetuses, the PLAS index was independent of gestational age and fetal physique and may be useful for raising the suspicion of congenital heart disease, including TAPVC.

Prognostic factors of premature closure of the ductus arteriosus in utero: a systematic literature review.

BACKGROUND: A number of case reports show various outcomes of premature closure of the ductus arteriosus in utero, including persistent pulmonary hypertension of the newborn and fetal or neonatal death; however, no study clarifies the clinical observations that are related to their prognoses. We aimed to clarify the prognostic factors of intrauterine ductal closure by a systematic literature review. Data sources We searched PubMed database (1975-2014) to identify case reports and studies on intrauterine closure of the ductus arteriosus, including maternal, fetal, and neonatal clinical information and their prognoses. RESULTS: We analysed the data of 116 patients from 39 articles. Of these, 12 (10.3%) died after birth or in utero. Fetal or neonatal death was significantly correlated with fetal hydrops (odds ratio=39.6, 95% confidence interval=4.6-47.8) and complete closure of the ductus arteriosus (odds ratio=5.5, 95% confidence interval=1.2-15.1). Persistent pulmonary hypertension was observed in 33 cases (28.4%), and was also correlated with fetal hydrops (odds ratio=4.2, 95% confidence interval=1.3-4.6) and complete closure of the ductus arteriosus (odds ratio=5.5, 95% confidence interval=1.6-6.0). Interestingly, maternal drug administration was not correlated with the risk of death and persistent pulmonary hypertension. CONCLUSIONS: Fetal hydrops and complete ductal closure are significant risk factors for both death and persistent pulmonary hypertension. Cardiac or neurological prognoses could be favourable if the patients overcome right heart failure during the perinatal period.

Abnormal cardiac axis as a prenatal marker of left pulmonary artery sling.

Left pulmonary artery sling (LPAS) is a rare vascular anomaly. The left pulmonary artery arises distally from the right pulmonary artery on the right side of the trachea and passes between the trachea and esophagus towards the left lung, compressing the lower trachea. LPAS is associated with congenital tracheal stenosis, which frequently requires early surgical intervention and has a poor prognosis due to severe airway obstruction after birth. Therefore, LPAS should be prenatally diagnosed to prepare for surgical intervention for tracheal stenosis. To the best of our knowledge, there are few reports on prenatal echocardiographic findings in LPAS. We report three prenatal cases of LPAS, which resulted in respiratory symptoms. We discuss fetal ultrasound findings and highlight the abnormal rotation of the fetal cardiac axis to the right as a useful sign in the prenatal screening of LPAS.

A new vascular access route for balloon angioplasty in extremely low birth weight infants.

In this study, we present the case of an extremely low birth weight infant with severe coarctation of the aorta after ductus ligation. We treated the patient with balloon angioplasty using the descending aorta as a new access route. This method reduced many complications typically observed during the intervention for extremely low birth weight infants, which may expand the available treatment options for extremely low birth weight infants.

Fetal bradyarrhythmia associated with congenital heart defects - nationwide survey in Japan.

BACKGROUND: Because there is limited information on fetal bradyarrhythmia associated with congenital heart defects (CHD), we investigated its prognosis and risk factors. METHODS AND RESULTS: In our previous nationwide survey of fetal bradyarrhythmia from 2002 to 2008, 38 fetuses had associated CHD. Detailed clinical data were collected from secondary questionnaires on 29 fetuses from 18 institutions, and were analyzed. The 29 fetuses included 22 with isomerism, 4 with corrected transposition of the great arteries (TGA) and 3 with critical pulmonary stenosis; 14 had complete atrioventricular block (AVB), 8 had second-degree AVB, and 16 had sick sinus syndrome; 5 died before birth, and 10 died after birth (5 in the neonatal period). Neonatal and overall survival rates for fetal bradyarrhythmia with CHD were 66% and 48%, respectively. Pacemaker implantation was needed in 17 cases (89%). Beta-sympathomimetics were administered in utero in 13 cases and were effective in 6, but were not associated with prognosis. All cases of corrected TGA or ventricular rate ≥70 beats/min survived. A ventricular rate <55 beats/min had significant effects on fetal myocardial dysfunction (P=0.02) and fetal hydrops (P=0.04), resulting in high mortality. CONCLUSIONS: The prognosis of fetal bradyarrhythmia with CHD is still poor. The type of CHD, fetal myocardial dysfunction, and fetal hydrops were associated with a poor prognosis, depending on the ventricular rate.

Extracorporeal membrane oxygenation for congenital diaphragmatic hernia in Japan.

BACKGROUND: The aim of this study was to clarify how extracorporeal membrane oxygenation (ECMO) is used to treat congenital diaphragmatic hernia (CDH) in Japan. METHODS: We completed a nationwide survey of CDH involving 614 infants. The subjects included 43 patients who underwent ECMO. We compared the clinical data of the patients who did and did not survive ≥ 90 days, and analyzed the 24 h blood gas data in isolated CDH cases in both groups. RESULTS: Of the 43 CDH patients, non-isolated CDH associated with other life-threatening or chromosomal anomalies was diagnosed in six patients. Only one of these six patients was able to discontinue ECMO and survived, and the other five died shortly after birth. The other 37 patients all had isolated CDH. The reason for initiating ECMO in 31 of these patients was persistent pulmonary hypertension of the newborn (PPHN). In the 37 patients with isolated CDH, ECMO was initiated within 24 h after birth. Sixteen patients (37%) survived ≥ 90 days, and intact discharge was possible in eight cases. Among the isolated CDH patients, on ROC analysis of the lowest oxygenation index (OI) to predict 90 day survival, the cut-off was 15. CONCLUSIONS: ECMO is used to treat PPHN starting from an early period after birth, but the mortality and morbidity are not favorable. For lowest OI, the index used to predict survival following ECMO, the cut-off was 15.