Raghib Syndrome and Pulmonary Arterial Hypertension in a Pediatric Patient: Case Report and Literature Review.

Background: Raghib syndrome is a rare malformation complex consisting of the drainage of the left superior vena cava (LSVC) into the left atrium, ostial atresia of the coronary sinus and an atrial septal defect (ASD). Case Report: This report aims to present the case of a child newly diagnosed with Raghib syndrome, complicated by pulmonary arterial hypertension, and to review previously published cases with the same diagnosis. A six-year-old female patient presented with signs and symptoms of heart failure (Ross III), reduced exercise tolerance and severe delay in stature and ponderal development. The imagistic work-up included echocardiography, followed by computer tomography (CT) and magnetic resonance imaging (MRI), through which a diagnosis of Raghib syndrome was established, complicated by pulmonary hypertension. As in other cases presented in the literature, MRI allowed for an accurate diagnosis, detecting the absent coronary sinus. The decision regarding the surgical closure of the ASD was made, with the patient having a favorable clinical evolution but with the persistence of elevated pulmonary artery pressure, for which Sildenafil therapy was instituted. Conclusions: The malformation complex consisting of an atrial septal defect, ostium atresia of the coronary sinus, uncovered coronary sinus, and persistent left superior vena cava, as identified through multiple imagistic investigations, was suggestive of the rare diagnosis of Raghib syndrome in this case. Among the limited number of cases of Raghib syndrome available in the literature, the present case is distinguished by the severity of the pulmonary artery hypertension at a very young age and in the absence of other concurrent cardiac malformations.

Comparative assessment of myocardial function between late premature newborns and term neonates using the 2D speckle tracking method.

Introduction: Assessment of myocardial function through speckle tracking echocardiography (STE) can bring benefits to conventional echocardiography in premature newborns, a particular vulnerable group in terms of adaptation to extra-uterine life. Furthermore, it represents a non-invasive imagistic method which can guide therapeutic approach in the hemodynamically unstable newborn. This study aims to highlight the particularities of myocardial function in late premature newborns, by conducting a comparison with a group of healthy neonates, by using STE. Methods: Conducted over a timespan of two years, this prospective study enrolled 64 term neonates and 21 premature newborns, with gestational ages ranging between 28 and 36 weeks, who prior to discharge underwent a cardiac ultrasound, involving two-dimensional image acquisitions of the apical four-chamber view of both ventricles. Afterwards, the images were offline analyzed, by using the autostrain function. Results: After segmental strain analysis, no significant discrepancies between the two groups in terms of interventricular values were found. However, left ventricle and right ventricle strain measurements differed significantly (p < 0.01), for each of the analyzed segments (basal, medial or apical). Moreover, a linear increase in interventricular (IV) basal strain with corrected gestational age progression was noted (p = 0.04). Peak global longitudinal strain (pGLS) and EF were similar between the two study groups. Premature newborns presented significantly more negative mean values of right ventricular free wall longitudinal strain (RVFWSL), (-24.19 ± 4.95 vs. -18.05 ± 5.88, p < 0.01) and of right ventricle global four chamber longitudinal strain (RV4CSL), (-19.71 ± 3.62 vs. -15.46 ± 5.59, p < 0.01), when compared to term neonates. Conclusions: The 2D STE is a reliable method for cardiac assessment of late preterm newborns. The evaluation of two-dimensional global longitudinal LV and RV strains might represent a useful tool in clinical practice. A better response of the right ventricle to the longitudinal deformation within premature neonates was noted. Thus, this study facilitates the identification of accurate reference values for this particular population segment, which will enable the evaluation of ventricular function in premature newborns with concurring disorders. Future longitudinal studies, assessing the fetal heart, could provide more insight into the development of myocardial function.

GATA4 rs61277615, rs73203482, and rs35813172 in Newborns with Transposition of the Great Arteries.

Congenital heart disease is the most common malformative pathology in newborns, with a worldwide incidence at 0.4-5%. We investigated the possible relationship between variations in nucleotide sequences and specific cardiac malformations in the GATA-binding factor 4 (GATA4) exon 1 region by using Sanger sequencing. Forty-four newborns from a third-level neonatal intensive care unit who were diagnosed with nonsyndromic, ductal-dependent congenital heart disease (i.e., transposition of the great arteries or ductal-dependent coarctation of the aorta) were enrolled. Their DNA was extracted using commercial methods and tested using the multiplex ligation-dependent probe amplification (MLPA) technique. The Sanger sequencing for GATA4 exon 1 in the newborns' DNA identified rs61277615, rs73203482, and rs35813172 variants not reported in the ClinVar archive of human variations in newborns previously diagnosed with transposition of the great arteries (n=5) and coarctation of the aorta (n=1). The identification of these novel variants in newborns with transposition of the great arteries or ductal-dependent coarctation of the aorta may be the first step in determining the variants' contribution to the occurrence of congenital heart disease. However, these results may be inconclusive, since the observed variants within GATA4 gene were not previously reported.

The Role of Galectin-3 in Predicting Congenital Heart Disease Outcome: A Review of the Literature.

Galectin-3 (Gal-3) is a novel pro-fibrotic biomarker that can predict both right and left cardiac dysfunction caused by various cardiovascular conditions. Its expression seems to be progressively altered with evolving cardiac remodeling processes, even before the onset of heart failure. Hence, Gal-3 has been found to be an individual predictor of acute and chronic heart failure or to serve as part of an integrated biomarker panel that can foresee adverse cardiac outcomes. In congenital heart disease (CHD), Gal-3 correlates with cardiac mortality and complications in both children and adults and is proposed as a therapeutic target in order to reverse the activation of pro-fibrosis pathways that lead to heart failure. Positive associations between serum Gal-3 levels, post-operatory hospitalization rates, complications and ventricular dysfunction have also been reported within studies conducted on patients with CHD who underwent corrective surgery. Thus, this review tried to address the potential utility of Gal-3 in patients with CHD and particularly in those who undergo corrective surgery. The heterogeneity of the literature data and the lack of validation of the results obtained by the current studies on larger cohorts cannot be neglected, though. Further longitudinal research is required to establish how Gal-3 can relate to long-term outcomes in pediatric CHD.

Left ventricular function evaluation of the fetal heart: reference intervals and inter-observer variability of 2D speckle-tracking echocardiography measurements.

AIMS: Measurement of myocardial strain using 2D speckle-tracking echocardiography can successfully quantify ventricular function, being considered superior to conventional echocardiography. This study aimed to establish reference intervals, interobserver agreements and reliability of two fetal echocardiographic parameters which reflect left ventricular myocardial function, left ventricular apical 4 chamber endo peak strain (AP4pLS) and ejection fraction (EF). MATERIAL AND METHODS: We conducted a prospective study on 103 healthy fetuses. In each case, cardiac ultrasound images obtained were stored and afterwards were subject to offline 2D speckle-tracking echocardiographic analyses. In 15 randomly chosen subjects a second examiner also carried out an offline analysis of the 4-chamber view and the archived images, in order to assess inter-observer reproducibility and agreement level. Our study group was sub-divided into four different gestational age groups. RESULTS: Reference ranges were established for the two measured parameters, AP4pLS and EF, which did not differ significantly between four different gestational age groups (p=0.98 and p=0.64) and neither correlated with gestational age progression (p=0.37 and p=0.08). An excellent level of agreement between the two examiners was found for the echocardiographic measurements, expressed through an intra-class correlation coefficient (ICC) value of 0.85 (0.62-0.94 for 95%CI) for AP4pLS and 0.78 (0.47- 0.92 for 95% CI) for EF. CONCLUSIONS: Speckle tracking AP4pLS and EF parameters are useful for assessment of ventricular myocardial function in healthy fetuses and can be reliably reproduced by two different skilled examiners. Further studies conducted on larger populations are required to standardize reference values of fetal speckle-tracking measurements.

The Impact of Prenatal Diagnosis in the Evolution of Newborns with Congenital Heart Disease.

Congenital heart malformations are cardiac and/or vascular structural abnormalities that appear before birth, the majority of which can be detected prenatally. The latest data from the literature were reviewed, with reference to the degree of prenatal diagnosis regarding congenital heart malformations, as well as its impact on the preoperative evolution and implicitly on mortality. Studies with a significant number of enrolled patients were included in the research. Prenatal congenital heart malformations detection rates were different, depending on the period in which the study took place, the level of the medical center, as well as on the size of enrolled groups. Prenatal diagnosis in critical malformations such as hypoplastic left heart syndrome, transposition of great arteries and totally aberrant pulmonary venous drainage has proven its usefulness, allowing an early surgical intervention, thus ensuring improved neurological development, increasing the survival rate and decreasing the rate of subsequent complications. Sharing the experience and results obtained by each individual therapeutic center will definitely lead to drawing clear conclusions regarding the clinical contribution of congenital heart malformations prenatal detection.

Interobserver Agreement and Reference Intervals for Biventricular Myocardial Deformation in Full-Term, Healthy Newborns: A 2D Speckle-Tracking Echocardiography-Based Strain Analysis.

Data regarding reference intervals for strain parameters derived from 2D speckle-tracking echocardiography in full-term newborns are limited and still under development. Our objectives were to establish the level of reproducibility and reference intervals in assessing myocardial function using 2D speckle-tracking echocardiography for longitudinal and regional strain measurements. A total of 127 full-term newborns were examined to be included in the study, of which 103 were analyzed. We used two-dimensional acquisitions from apical four-chamber view of both ventricles and analyzed the autostrain function offline. We obtained interobserver agreement between the two observers ranging from good to excellent for all speckle-tracking parameters except for the strain of the medial portion of the left ventricle (LV) lateral wall and the strain measured on the basal portion of the inter-ventricular septum, which reflected a fair interobserver reproducibility (ICC = 0.52, 95% IC: 0.22-0.72 and ICC = 0.43, 95% IC: 0.12-0.67, respectively). The reference values obtained for the LV peak longitudinal strain were between -24.65 and -14.62, those for the right ventricle (RV) free wall were from -28.69 to -10.68, and those for the RV global four-chamber were from -22.30 to -11.37. In conclusion, two-dimensional peak longitudinal LV and RV strains are reproducible with good to excellent agreement and may represent a possible alternative for the cardiac assessment of healthy newborns in the clinical practice.

Fetal Tachyarrhythmia Management from Digoxin to Amiodarone-A Review.

Sustained fetal tachycardias are rare but represent a high risk of mortality and morbidity. Consensus has yet to be found regarding their optimal management. The aim of this narrative review is to summarize the data available in the current literature regarding the efficacy and safety of medications used in the management of intrauterine tachyarrhythmias and to provide possible treatment protocols. In this review, we would like to emphasize the importance of a thorough evaluation of both the fetus and the mother, prior to transplacental antiarrhythmic drug initiation. Factors such as the hemodynamic status of the fetus, possible mechanisms of fetal arrhythmia, and concomitant maternal conditions are of primordial importance. As a possible treatment protocol, we would like to recommend the following: due to the risk of sustained supraventricular tachycardia (SVT), fetuses with frequent premature atrial beats should be evaluated more frequently by echocardiography. A careful hemodynamic evaluation of a fetus with tachycardia is primordial in forestalling the appearance of hydrops. In the case of atrial flutter (AFL), sotalol therapy could represent a first choice, whereas when dealing with SVT patients, flecainide should be considered, especially for hydropic patients. These data require consolidation through larger scale, non-randomized studies and should be handled with caution.

Assessment of Biventricular Myocardial Function with 2-Dimensional Strain and Conventional Echocardiographic Parameters: A Comparative Analysis in Healthy Infants and Patients with Severe and Critical Pulmonary Stenosis.

Our aim was to compare the global longitudinal and regional biventricular strain between infants with severe and critical pulmonary stenosis (PS), and controls; to compare pre- and post-procedural strain values in infants with severe and critical PS; and to assess the correlations between echocardiographic strain and conventional parameters. We conducted a retrospective single-center study. The comparisons of echocardiographic variables were performed using separate linear mixed models. The overall mean right ventricle (RV) regional strains measured before intervention in PS patients was significantly different when compared to the control group (p = 0.0324). We found a significant change in the left ventricle, RV, and inter-ventricular septum strain (IVS) values from basal to apical location (p < 0.05). IVS strain values showed a higher decrease in mean strain values from basal to apical in PS patients. There was no significant difference in means of baseline and post-interventional strain values in PS patients (p > 0.05). Following the strain analysis in patients with PS, we obtained statistically significant changes in the RV global-4-chamber longitudinal strain (RV4C). The RV4C, which quantifies the longitudinal strain to the entire RV, can be used in current clinical practice for the evaluation of RV function in infants with severe and critical PS. The longitudinal and segmental strain capture the pathological changes in the IVS, modifications that cannot be highlighted through a classical echocardiographic evaluation.

Severe early-onset manifestations of generalized arterial calcification of infancy (mimicking severe coarctation of the aorta) with ABCC6 gene variant - Case report and literature review.

Introduction: Generalized arterial calcification of infancy (GACI) is a rare cause of infantile heart failure and systemic hypertension with a poor prognosis, characterized by extensive calcification and proliferation of the intimal layer of large and medium sized arteries. Case report: We present the first case report of successful surgical treatment of severe aortic arch obstruction by calcified plaques mimicking severe coarctation of the aorta and the outcome (of bisphosphonate therapy) in a newborn with GACI. Furthermore, we report the identification of a variant in ATP Binding Cassette Subfamily C, Member 6 (ABCC6) gene, possibly associated with severe early-onset manifestations of GACI. Conclusion: This case report highlights the importance of considering GACI in an infant with heart failure, systemic hypertension, and evidence of increased echogenicity of the arterial vessels. We noted the favorable outcome in improving the aortic calcification in our patient after surgical treatment and bisphosphonates therapy. Early diagnosis and treatment improve the long-term prognosis. A better understanding of this rare genetic disease could lead to new therapeutic strategies.

Impaired Speckle-Tracking-Derived Left Ventricular Longitudinal Strain Is Associated with Transposition of Great Arteries in Neonates: A Single-Center Study.

The transposition of great arteries (TGA) is one of the most frequent and severe congenital heart diseases. After newborn stabilization and while pending surgical correction, echocardiographic monitoring with a careful evaluation of left ventricle (LV) performance is warranted. In this study, our objectives were (i) to compare myocardial function, assessed via speckle-tracking echocardiography, between neonates with TGA and neonates without TGA and (ii) to identify a strain parameter with a good discriminatory ability for TGA. We conducted a retrospective, single-center study. A total of 90 neonates were examined, of whom 66 were included (16 comprised the TGA group and 50 comprised the control group). The results of a bivariate analysis showed that classic echocardiography parameters displayed no significant differences between the two studied groups (p = 0.785 for EF, p = 0.286 for MAPSE and p = 0.315 for TAPSE). We found a statistically significant difference between the two groups for the mean values of the LVpGLS parameter (adjusted p = 0.0047), with impaired LV myocardium function being observed in the TGA group after adjusting for other covariates. Regarding segmental strain, the mean medial and apical inter-ventricular septum strain values were found to be significantly lower in the neonates with TGA than in the controls (95% CI for difference in means: [-6.45, -0.65], [-8.56, -1.97]). The results of an ROC analysis showed that LVpGLS had a significant ability to differentiate between neonates with TGA and controls (AUC = 0.712, 95% CI: [0.52, 0.903], p = 0.011). In conclusion, LVpGLS is a parameter with a significant discriminatory ability for LV dysfunction, and it is useful in the evaluation of ventricular myocardial function in newborns with TGA.

Diagnostic Difficulties in a Case of Fetal Ventricular Tachycardia Associated with Neonatal COVID Infection: Case Report.

The clinical course of COVID in the pediatric population is considered to be much milder when compared to adults; however, the occurrence of severe and fatal forms of the disease in children is non-negligible, especially in patients with comorbidities such as prematurity or cardiac disease. We report a case of a newborn with sotalol-controlled fetal ventricular tachycardia, who was postnatally diagnosed with COVID infection. The myocardial injury was sustained on the basis of pericardial effusion, left ventricular dysfunction, rapid progression to coronary artery dilation, and an arrhythmic storm. We believe that, in our case, there is a significant overlap between fetal ventricular tachycardia, associated with impaired left ventricular function, and COVID infection, diagnosed after birth; both factors contribute to the myocardial dysfunction with a fulminant clinical evolution. To our knowledge, this is the first case describing neonatal myocardial dysfunction associated with SARS-CoV infection complicating the clinical course of rare fetal tachyarrhythmia.

Speckle-Tracking Global Longitudinal and Regional Strain Analysis in Neonates with Coarctation of Aorta: A Case-Control Study.

Our objectives are to compare speckle-tracking peak global longitudinal (pGLS) and regional strain values in neonates with coarctation of aorta (CoA) and control groups. Echocardiographic parameters measured by speckle-tracking were studied in a retrospective single-center study. A comparison of pGLS and segmental deformation between neonates with CoA and control group was performed using a three-way mixed ANOVA model. There was a significant difference in the means of segmental strain values between CoA and control group at the apical (p = 0.018) and basal segments (p = 0.031) of the interventricular septum and at the apical segment (p = 0.026) of the left ventricle (LV). After correcting for multiple comparisons, the results had a tendency toward statistical significance (adjusted-p < 0.10). There was significant difference in the mean values of pGLS [F(1, 39) = 7.61, p = 0.009, adjusted p = 0.018] between the studied groups. The results of ROC analysis showed that a cut-off value of -16.60% for pGLS provided an estimated sensitivity of 92.31% (95% CI: [63.97, 99.81]) and 71.43% specificity (95% CI: [51.33, 86.78]) for the diagnosis of CoA in neonates (AUC = 0.794, 95% CI: [0.66, 0.93]). pGLS can be regarded as a feasible and reproducible parameter reflecting LV dysfunction in newborns with CoA when compared to newborns with a false-positive diagnosis.

Congenital complete atrioventricular block from literature to clinical approach - a case series and literature review.

AIM: Congenital atrioventricular block (CAVB) is an immunological condition, secondary to the transfer of maternal Ig G antibodies from seropositive mothers. Although the presence of these antibodies is high among pregnant women, the preva-lence of this fetal pathology is low. The aim of this paper is to analyze a series of cases with intrauterine diagnosis of CAVB and to present their follow-up protocol. MATERIAL AND METHOD: In the period between 2013-2020, five fetuses were diagnosed and followed up in the Pediatric Cardiology Clinic. In each of the cases, assessment of the hemodynamic status was done by calculation of the fetal cardiovascular profile score (CVPS). In the last cases the follow-up protocol was supplemented with longitudinal speckle tracking evaluation of the ventricular function. RESULTS: In the present series, intrauterine death occurred in one case; in another case resumption of atrioventricular conduction was observed. Epicardial pacemaker implantation was required in three of the patients. CONCLUSION: Completing the evaluation of ventricular function with the longitudinal speckle tracking method in fetuses and newborn patients with congenital atrioventricular block may play an important role in establish-ing therapeutic behavior.

Significant Associations between AXIN1 rs1805105, rs12921862, rs370681 Haplotypes and Variant Genotypes of AXIN2 rs2240308 with Risk of Congenital Heart Defects.

This study aimed to investigate possible associations of the susceptibility to congenital heart defects (CHDs) with AXIN1 rs1805105, rs12921862 and rs370681 gene variants and haplotypes, and AXIN2 rs2240308 gene variant. Significant associations were identified for AXIN1 rs370681 and AXIN2 rs2240308 variants. AXIN1 rs370681 variant was significantly associated with decreased odds of CHDs (adjusted OR varying from 0.13 to 0.28 in codominant, dominant and recessive gene models), while the AXIN2 rs2240308 variant was associated with increased odds of CHD in the dominant model. The haplotype-based generalized linear model regression of AXIN1 rs1805105, rs12921862 and rs370681 variants revealed that C-C-C and C-C-T haplotypes significantly increased the risk of CHDs (p < 0.05). No significant second order epistatic interactions were found between investigated variants (AXIN1 rs1805105, rs12921862, rs370681, and AXIN2 rs2240308). Our conclusion is that AXIN1 rs1805105, rs12921862, and rs370681 (C-C-C and C-C-T) haplotypes and AXIN2 rs2240308 contribute to CHDs susceptibility.

Cardiovascular Imaging for Guiding Interventional Therapy in Structural Heart Diseases.

Development of interventional methods has revolutionized the treatment of structural cardiac diseases. Given the complexity of structural interventions and the anatomical variability of various structural defects, novel imaging techniques have been implemented in the current clinical practice for guiding the interventional procedure and for selection of the device to be used. Three- dimensional echocardiography is the most used imaging method that has improved the threedimensional assessment of cardiac structures, and it has considerably reduced the cost of complications derived from malalignment of interventional devices. Assessment of cardiac structures with the use of angiography holds the advantage of providing images in real time, but it does not allow an anatomical description. Transesophageal Echocardiography (TEE) and intracardiac ultrasonography play major roles in guiding Atrial Septal Defect (ASD) or Patent Foramen Ovale (PFO) closure and device follow-up, while TEE is the procedure of choice to assess the flow in the Left Atrial Appendage (LAA) and the embolic risk associated with a decreased flow. On the other hand, contrast CT and MRI have high specificity for providing a detailed description of structure, but cannot assess the flow through the shunt or the valvular mobility. This review aims to present the role of modern imaging techniques in pre-procedural assessment and intraprocedural guiding of structural percutaneous interventions performed to close an ASD, a PFO, an LAA or a patent ductus arteriosus.

Incomplete Refractory Kawasaki Disease in an Infant-A Case Report and a Review of the Literature.

Kawasaki disease (KD) is a febrile vasculitis, which is commonly defined by fever and at least four specific clinical symptoms. Incomplete KD is defined by suggestive echocardiographic findings with an incomplete clinical picture. Refractory KD is diagnosed in patients resistant to intravenous immunoglobulin (IVIG). We report the case of a 6-month-old male infant admitted to our clinic for persistent fever and onset of a generalized polymorphous rash, accompanied by high fever, rhinorrhea, and cough for the past 7 days. The laboratory tests, on the day of admission, revealed leukocytosis with neutrophilia, anemia, thrombocytosis, hypernatremia, hypoalbuminemia, elevated C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR). Echocardiography showed dilation of the left anterior descending coronary artery (LAD). Based on all these findings, we established the diagnosis of KD, and we initiated IVIG and intravenous pulsed methylprednisolone, with an initial favorable outcome. However, the symptoms reappeared, and we administered a second higher single dose of IVIG, but without any clinical improvement. Moreover, the laboratory parameters and echocardiographic findings worsened. We reinitiated a longer course of intravenous methylprednisolone in a smaller dose, which had a favorable impact on the clinical, laboratory, and echocardiographic parameters. Multiple uncertainties exist related to the management of refractory KD despite the wide spectrum of therapeutic options that have been proposed. Our case demonstrates that in patients refractory to aggressive initial therapy, low or moderate doses of steroid given daily may be helpful.

Cantrell Syndrome-A Rare Complex Congenital Anomaly: A Case Report and Literature Review.

Cantrell syndrome (CS) or pentalogy of Cantrell is defined as a rare condition involving a midline anterior abdominal wall defect, a distal sternal cleft, a defect of the anterior diaphragm, and a defect of the apical pericardium with pericardio-peritoneal communication, as well as intracardiac anomalies. We report the case of a male newborn with type 2 CS diagnosed during intrauterine life based on ultrasonographic evaluation. Clinical examination at birth revealed an abdominal wall defect with extrathoracic displacement of the heart and a diastasis of the sagittal suture. Postnatal echocardiography revealed tricuspid atresia, partial extrathoracic and extra-abdominal displacement of the heart and liver, a large ventricular septal defect, severe subpulmonary stenosis, hypoplasia of the pulmonary artery, and a large hourglass-shaped left ventricle secondary to narrowing of the heart at the level of its extrathoracic displacement. Computed tomography showed additional abnormalities including increased left ventricular volume with extrathoracic apical aneurysmal dilatation below the xiphoid process at the level of anterior abdominal wall, a hypoplastic right ventricle, partial transparietal herniation of the left hepatic lobe adjacent to a left ventricular diverticulum, and an adrenal hematoma. The newborn received intensive medical management during his first week of life; however, surgical management had to be postponed owing to his unstable condition. Eventually, it was performed on the 14th day of life, but unfortunately, the newborn died shortly after the procedure.

Prenatal diagnosis of the fetal common arterial trunk. A case series.

Fetal common arterial trunk is an anomaly represented by a unique arterial trunk that arouses from the base of the heart, and gives birth to systemic branches, both pulmonary and coronary, frequently associated with a ventricular septal defect (VSD) and has a poor prognosis. We present a series of 17 cases diagnosed in our tertiary center with different types of fetal common arterial trunk, its associated disorders, the evolution of the pregnancies, and of the neonates. We concluded that our cases support the fact that a complete intrauterine evaluation of each case of the common arterial trunk is impossible. The postnatal prognosis of the cases from our center was fatal, similar to most reports of the literature.

The role of echocardiography in fetal tachyarrhythmia diagnosis. A burden for the pediatric cardiologist and a review of the literature.

Supraventricular tachyarrhythmia represents the most frequent fetal dysrhythmia. In the lack of diagnosis and treatment these fetuses may develop hydrops and even death. For the therapeutic approach it is important to establish the diagnosis of the type of supraventricular tachycardia. In this paper we report 29 cases with different types of supraventricular tachycardia in which the diagnosis was established using our own protocol, which allowed us to make the difference between the types of tachycardia (atrioventricular reentry tachycardia due to the accessory pathway, atrial ectopic tachycardia and permanent junctional reciprocal tachycardia). We acquired the data by a series of recordings in M mode and pulsed Doppler by simultaneous recording of an artery and a vein flow. First of all, we diagnosed the supraventricular tachycardia type, with short or long ventriculoarterial interval, and afterwards, we made the difference between atrial ectopic tachycardia and permanent junctional reciprocal tachycardia using methods to decrease the atrioventricular conduction.