Utility of the Angle between the Right Aortic Arch and First Branch for Detecting Double Aortic Arch via Fetal Echocardiography.

INTRODUCTION: The presence of a double aortic arch (DAA) is manifested by compressive symptoms, requiring surgery. DAA cases are classified as either complete or incomplete type. DAA and a right aortic arch with mirror image branching (mRAA) have a similar configuration to the first branch artery. The first branch of the mRAA is the left brachiocephalic artery, which appears to be the same as that of an incomplete DAA due to blood flow interruption. The present retrospective study aimed to evaluate the differences between DAA and mRAA by fetal echocardiography. METHODS: This single retrospective cohort study included all patients diagnosed with complete DAA, incomplete DAA, or mRAA at our facility between 2010 and 2022. The patients were diagnosed with complete DAA, incomplete DAA, or mRAA after birth and remaining fetal echocardiograms. The patients were divided into the DAA (complete DAA: n = 4, incomplete DAA: n = 3) and mRAA (n = 4) groups. The following three outcomes were compared: (1) angle between the right aortic arch and first branch (RF angle), (2) ratio of height to width of the region bounded by the aortic arch, first branch of the aortic arch, and descending aorta, and (3) maximum tracheal diameter on a three-vessel trachea view. RESULTS: The incomplete DAA cases were difficult to diagnose via fetal echocardiography. On fetal echocardiography, the RF angle was significantly steeper in the DAA group than in the mRAA group (median 57° [36°-69°] vs. 75° [62°-94°]; p < 0.05). The DAA and RAA groups showed no significant differences in the ratio of height to width of the region bounded by the aortic arch, first branch of the aortic arch, and descending aorta (median 0.57 [0.17-0.68] vs. 0.73 [0.56-1.0]) and maximum tracheal diameter (median 2.5 [1.4-3.3] vs. 3.2 [2.8-3.5] mm). The cut-off value for the presence of DAA was an RF angle <71°. CONCLUSION: The DAA group (complete and incomplete DAA) had a significantly steeper RF angle than the mRAA group. Therefore, RF angle measurement could improve the fetal diagnosis and postnatal prognosis of DAA.

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.

Post-Extubation Inhaled Nitric Oxide Therapy via High-Flow Nasal Cannula After Fontan Procedure.

In 2014, our hospital introduced inhaled nitric oxide (iNO) therapy combined with high-flow nasal cannula (HFNC) oxygen therapy after extubation following the Fontan procedure in patients with unstable hemodynamics. We report the benefits of HFNC-iNO therapy in these patients. This was a single-center, retrospective review of 38 patients who underwent the Fontan procedure between January 2010 and June 2016, and required iNO therapy before extubation. The patients were divided into two groups: patients in Epoch 1 (n = 24) were treated between January 2010 and December 2013, receiving only iNO therapy; patients in Epoch 2 (n = 14) were treated between January 2014 and June 2016, receiving iNO therapy and additional HFNC-iNO therapy after extubation. There were no significant differences between Epoch 1 and 2 regarding preoperative cardiac function, age at surgery, body weight, initial diagnosis (hypoplastic left heart syndrome, 4 vs. 2; total anomalous pulmonary venous return, 5 vs. 4; heterotaxy, 7 vs. 8), intraoperative fluid balance, or central venous pressure upon admission to the intensive care unit. Epoch 2 had a significantly shorter duration of postoperative intubation [7.2 (3.7-49) vs. 3.5 (3.0-4.6) hours, p = 0.033], pleural drainage [23 (13-34) vs. 9.5 (8.3-18) days, p = 0.007], and postoperative hospitalization [36 (29-49) vs. 27 (22-36) days, p = 0.017]. Two patients in Epoch 1 (8.3%), but none in Epoch 2, required re-intubation. Our results suggest that HFNC-iNO therapy reduces the duration of postoperative intubation, pleural drainage, and hospitalization.

The closing process of the ductus arteriosus connecting the left common carotid artery and main pulmonary artery.

An isolated left common carotid artery is very rare, and only 13 cases have been reported thus far. All those cases were accompanied by a right aortic arch and aberrant left subclavian artery, and the connecting vessel between the pulmonary artery and left common carotid artery was thought to be ductal tissue. However, there have been no reports that have followed the natural closure of this vessel. We present a case in whom we could observe the closing process of this vessel at the connection between the left common carotid artery and main pulmonary artery in association with a tetralogy of Fallot.

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.

Prognosis in tetralogy of Fallot with absent pulmonary valve.

BACKGROUND: Tetralogy of Fallot with absent pulmonary valve (TF/APV) is a rare and severe congenital heart disease with high mortality. The aim of this study was to assess whether TF/APV prognosis is related to fetal and postnatal clinical course and pulmonary artery (PA) configuration. METHODS: The fetal and postnatal echocardiograms and clinical outcomes of 13 patients with TF/APV (diagnosed antenatally in 9 patients and postnatally in 4) were reviewed, and divided into two groups: group A (n = 6), alive; and group D (n = 7), dead. RESULTS: Fetal period: group A, polyhydramnios n = 0, hydrops fetalis (HF) n = 0, patent ductus arteriosus (PDA) n = 2; group D, polyhydramnios n = 3, HF n = 2, PDA n = 0. Postnatal period: group A, five patients underwent intracardiac repair, including one requiring artificial ventilation (AV). A further AV patient required three operations before extubation. Postoperative courses were all good. Group D, excluding the two intrauterine fetal deaths, four patients required AV (three of whom died neonatally or in early childhood) and one underwent intracardiac repair. PA configuration: all group A patients had bulbous expansion of left and right PA (clover type). Three patients in group D had bulbous expansion of main PA (balloon type). CONCLUSIONS: PDA was a factor associated with good prognosis. Hydramnion and HF were factors associated with poor prognosis. Given that there was a higher rate of postnatal AV and poorer prognosis in balloon type than in clover type PA (P < 0.05), PA configuration is also considered an important factor to predict postnatal outcome in TF/APV.

Unilateral absence of the pulmonary artery: a report of two cases that presented different clinical courses.

We recently diagnosed two cases of isolated unilateral absence of the pulmonary artery just after birth. Through the therapy, we could not prevent obstruction of the pulmonary artery and lead to complications. There have been no symptoms attributed to isolated unilateral absence of the pulmonary artery so far. We should carefully consider the strategy for therapeutic intervention for asymptomatic cases.

The evaluation of diastolic function using the diastolic wall strain (DWS) before and after radical surgery for congenital diaphragmatic hernia.

OBJECTIVE: The measurement of diastolic wall strain (DWS), a new method of evaluating cardiac diastolic function, was employed to evaluate ventricular diastolic function in patients with congenital diaphragmatic hernia (CDH). MATERIALS AND METHODS: Eighteen neonates with a CDH who were born and treated in our hospital between September 2009 and January 2013 were studied. The left ventricular posterior wall thickness during the systolic phase (PWs) and diastolic (PWd) phase was measured using M-mode imaging, and the DWS was calculated as (PWs-PWd)/PWs. The Tei index, the isovolumic relaxation time (IRT), and the fraction shortening (FS) were measured as indices of cardiac function in 14, 15, and 18 cases, respectively. Cardiac function was measured before and after surgery. Statistical analyses were performed using the paired t test. RESULTS: The pre- and postoperative DWS, Tei index, IRT and FS values were 0.19 ± 0.06 and 0.26 ± 0.11 (P < 0.01), 0.40 ± 0.12 and 0.31 ± 0.11 (P < 0.05), 48 ± 14 and 39 ± 5.0 ms (P < 0.05), 30 ± 7.7 and 34 ± 7.4 % (P < 0.05), respectively. CONCLUSION: The diastolic and systolic functions were not only measured by the Tei index, IRT and FS values, but also by the DWS value, which improved after surgery. The measurement of DWS is an easy and useful method for evaluating the diastolic function of CDH patients.

Efficacy of the circulatory management of an antenatally diagnosed congenital diaphragmatic hernia: outcomes of the proposed strategy.

OBJECT: The purpose of this study is to evaluate the outcome of our therapeutic strategy for antenatally diagnosed congenital diaphragmatic hernia (ADCDH). METHODS: We treated 61 cases of ADCDH according to our strategy. Prostaglandin E1 was required to be maintained the patency of the ductus arteriosus (PDA) in 39 cases (Group I) while it was not administered in 22 cases (Group II). Left ventricular end-diastolic dimension (LVDD) and Tei index were measured with echocardiography on days 0, 2, and 7 after birth. Radical surgery was performed on all cases by day 2. RESULTS: On day 0, Group I showed smaller LVDD and Tei index than those in Group II. Between day 0 and day 2, these parameters increased significantly in Group I, but not in Group II. On day 7, no significant difference in these parameters was observed between the two groups. Five patients died of cardiac and respiratory failure, resulting in a survival rate of 92 %. CONCLUSION: Our therapeutic strategy improves the clinical outcome of ADCDH. This can be attributed to two factors: earlier surgery resulting in improved LV function. The latter attenuates pulmonary hypertension and maintains PDA with a consequent decrease in right ventricular afterload to compensate for the low cardiac output resulting from PDA.

Successful bailout stenting for critical aortic coarctation in a premature baby weighing 590 g.

Severe aortic coarctation (CoA) is a critical congenital heart disease that requires surgery as the first-line treatment in neonates. However, in very small premature infants, aortic arch repair has a relatively high mortality and morbidity rate. Bailout stenting is an alternative method that can be performed safely and effectively with low morbidity.We present a case of severe CoA in a premature baby, a monochorionic twin with selective intrauterine growth restriction. The patient was born at 31 weeks of gestation with a birth weight of 570 g. Seven days following her birth, she experienced anuria due to critical neonatal isthmic CoA. She underwent a stent implantation procedure at term neonatal, weighing 590 g. She had good dilatation of the coarcted segment with no complications. Follow-up at infancy showed no CoA recurrence. This is the world's smallest case of stenting for CoA.

Utility of novel echocardiographic measurements to improve prenatal diagnosis of coarctation of the aorta.

Prenatal recognition of coarctation of the aorta (CoA) may improve neonatal survival and reduce morbidity. However, prenatal diagnosis of CoA remains challenging, with relatively high false-positive and false-negative rates. This study aimed to identify a novel formula based on fetal echocardiographic measures to predict prenatal identification of CoA. A retrospective comparison on the echocardiographic evaluation of 30 patients with suspected CoA between May 2016 and April 2021 was performed. The patients were divided into a postnatal surgical intervention group (n = 13) and a non-intervention group (n = 17). The measurements that showed significant differences were aortic isthmus diameter Z-score (p < 0.001), ductus arteriosus diameter/aortic isthmus diameter (p < 0.001), and distal aortic arch (DA) index (p < 0.001). In the receiver operating characteristic curves analysis, the DA index was the largest with an area under the curve of 0.941 and a cutoff value of 1.28, with a sensitivity of 85% and a specificity of 94%. Measurement of the DA index improved the diagnostic rate of fetal CoA and a DA index ≧ 1.28 indicated fetal CoA cases requiring surgical intervention.

Prenatal complex congenital heart disease with Loeys-Dietz syndrome.

We report an infantile case of Loeys-Dietz syndrome prenatally diagnosed with congenital complex heart disease - double outlet right ventricle and interruption of the aortic arch. The patient also showed prominent dilatation of the main pulmonary artery. Emergency bilateral pulmonary artery banding was performed on the 9th day. However, on the 21st day, the patient died of massive bleeding due to rupture of the right pulmonary artery. Subsequently, a mutation of the TGFBR1 gene was detected. As cardiovascular lesions of Loeys-Dietz syndrome appear early and progress rapidly, the prognosis is generally poor. Patients require periodic examination and early intervention with medical therapy such as Losartan administration and surgical therapy. Early genetic screening is thought to be useful for the prediction of complications as well as vascular disease.

Transconduit approach for pulmonary vein stenting in an extracardiac Fontan and plastic bronchitis case.

Plastic bronchitis (PB) is a rare but severe complication in patients following the Fontan operation, the final palliative procedure for single ventricle heart disease. PB is characterised by the formation of rubbery casts of the tracheobronchial tree that may cause cough, wheezing, dyspnoea and hypoxia. Progressive airway obstruction may lead to asphyxia and death, and the overall mortality rate is 15.2%. Most patients receive a combination therapy from three different treatment strategies, that is, therapy for relief of airway obstruction, anti-inflammatory treatment and treatment to improve haemodynamics of the Fontan physiology. Therapy improving haemodynamics consists of optimising the Fontan circulation or cardiac function, antiarrhythmic therapy, inhibition of lymph leakage/production, decompression of the Fontan circulation and heart transplantation.We present the case of a child with PB after extracardiac Fontan operation, who was refractory to balloon angioplasty and surgical release for pulmonary vein (PV) stenosis. We decided to perform stent implantation for the right PVs through transconduit puncture instead of fenestration because the position of fenestration was too high to access the PVs. After PV stenting via the transconduit approach, she experienced improvement in cough, wheezing and breathlessness, and a significant reduction in airway cast formation.

Fetal echocardiographic assessment of endocardial fibroelastosis in maternal anti-SSA antibody-associated complete heart block.

BACKGROUND: There are few reports describing the features of maternal anti-SSA antibody-associated congenital complete heart block (CCHB) patients developing endocardial fibroelastosis (EFE). The aim of this study was to describe the clinical features and the outcome of patients with CCHB, with or without EFE. METHODS AND RESULTS: Over a 20-year period, 12 consecutive patients diagnosed with maternal anti-SSA antibody-associated CCHB were identified. The maternal anti-SSA antibody levels were measured and fetal echocardiographic findings were reviewed. The ratios of the thickness of the endocardium to that of the whole wall of the left ventricle (LE/W) and right ventricle (RE/W) were measured to investigate the degree of endocardial thickening. A total of 7 patients survived (living group) and were not diagnosed as having EFE. The remaining 5 patients died and were diagnosed with EFE during autopsy (dead group). Fetal echocardiography of the patients showed differences in the thickening and hyperintensity of the endocardium. The RE/W value was significantly higher in the dead group than in the living group. The titers of both maternal anti-52-kDa and anti-60-kDa SSA antibodies were high, but showed no significant differences between the 2 patient groups. CONCLUSIONS: EFE was the major negative prognostic factor for CCHB. Myocardial damage, predominantly in the right ventricle, was related to the outcome of CCHB associated with EFE.

Prenatal diagnosis of aortopulmonary window with interrupted aortic arch by fetal echocardiography.

We report the first case of prenatally diagnosed aortopulmonary window (APW) with interrupted aortic arch. The mother was referred for fetal echocardiography at 29 weeks' gestation because of suspected congenital heart disease. On the short-axis view, we clearly imaged the aortopulmonary septal defect at the proximal pulmonary artery and type A interrupted aortic arch. The window was 4 mm and the shunt flow detected by color flow Doppler was right-to-left in systole and early diastole. All aortic arch branches originated from the aorta proximal to the interruption. The well-developed ascending aorta was not continuous with the descending aorta in the sagittal view of the aortic arch. Visualization of the direction of the shunt flow across the APW and each origin of subclavian arteries was useful for the diagnosis of APW with interrupted aortic arch.

Successful catheter ablation of premature ventricular contractions triggering torsade de pointes in a small infant with histiocytoid cardiomyopathy: a case report.

BACKGROUND: A short-coupled variant of torsade de pointes (ScTdP) is rare and resistant to medical treatment. There has not been a reported catheter ablation (CA) of a short-coupled premature ventricular contraction (PVC) triggering ScTdP in an infant. CASE SUMMARY: A neonate was referred to our hospital on the day of birth for Wolff-Parkinson-White syndrome, repeated episodes of supraventricular tachycardia, and a left ventricular non-compaction. She underwent CA of an accessory pathway at 72 days of age. On the 5th day after ablation, she had recurrent TdP episodes resistant to various antiarrhythmic drugs and received extracorporeal membrane oxygenation at 86 days of age. She underwent CA of PVCs triggering TdP at 122 days of age and a weight of 3.4 kg. Two types of PVCs triggering TdP were successfully ablated, which originated from the right ventricle (RV). Pre-potentials were recorded at the earliest ventricular activation sites of the targeted PVCs. After the ablation, she had no TdP episodes and the cardiac assist device was removed. However, she died of uncontrolled heart failure at 6 months of age. The histological findings were compatible with histiocytoid cardiomyopathy and abnormal cells were distributed throughout both ventricles. At the ablation site, fibrotic transmural lesions were noted in the RV wall. DISCUSSION: The PVCs triggering TdP were successfully ablated in a 4-month-old girl with histiocytoid cardiomyopathy. The PVCs were likely caused by triggered activity and associated with abnormal Purkinje cells.

Bilateral Pulmonary Artery Banding in Ebstein's Anomaly With Circular Shunting.

We present a case of critical Ebstein's anomaly with circular shunting, diagnosed in utero. The fetal cardiothoracic area ratio was elevated; tricuspid regurgitation and pulmonary regurgitation worsened with fetal hydrops. At 35 weeks 6 days of gestation, elective caesarean section delivery was performed. Planned bilateral pulmonary artery banding and pulmonary trunk ligation were performed as a palliative operation 4 hours after birth, with the infant in a stable condition. At age 5 days, we performed the Starnes operation. The postoperative course was uneventful and a bidirectional Glenn operation was performed at age 4 months.

Left ventricular function after a Fontan operation in patients with pulmonary atresia with an intact ventricular septum.

OBJECTIVES: In patients with pulmonary atresia with an intact ventricular septum (PA/IVS), there are no reports about the impact of sinusoidal communication (SC) on left ventricular (LV) performance after a Fontan operation; therefore, the purpose of this study was to fill this gap in the literature. METHODS: We performed a single-centre, retrospective study of 46 patients with PA/IVS. Nineteen patients who underwent the Fontan procedure were enrolled and divided into 2 groups: those with SC (SC group) and those without SC (N group). The 2 groups were compared in terms of postoperative cardiac function. RESULTS: Thirteen patients were in the SC group (4 patients with right ventricle-dependent coronary circulation) and 6, in the N group. Although 2 of the patients with right ventricular (RV)-dependent coronary circulation showed focal asynergy of the LV wall, others showed no findings of myocardial infarction. The patients' preoperative age and age during the observation period after the Fontan operation did not differ. There was no difference in LV ejection fraction, cardiac index and RV and pulmonary artery pressure before and after the Fontan operation. Preoperative RV volume in the N group was greater than that in the SC group. Brain natriuretic peptide levels were higher in the SC group after the Fontan operation. On ventricular efficacy analysis, contractility was lower (1.8 ± 0.32 vs 2.5 ± 0.40 mmHg/ml/m2, P = 0.001) and ventricular efficacy was worse (1.0 ± 0.15 vs 0.86 ± 0.11, P = 0.046) in the SC group during the postoperative period. CONCLUSIONS: Compared to patients with PA/IVS without SC, patients with PA/IVS with SC had a low cardiac contractility and decreased cardiac output efficiency after the Fontan procedure.