Management of Fetal Supraventricular Tachycardia: Case Series from a Tertiary Perinatal Cardiac Center.

BACKGROUND: Fetal supraventricular tachycardia is a relatively uncommon cardiac rhythm abnormality which is often associated with adverse perinatal outcomes if untreated. Although there are several treatment modalities and protocols in use globally, there is no consensus as to the most effective antiarrhythmic to manage this condition. AIM: This study aimed to evaluate perinatal outcomes following prenatal maternal therapy for fetal supraventricular tachycardia. MATERIALS AND METHODS: This was a 20-year retrospective cohort study. Institutional records were reviewed for antenatal therapy choice and maternal and fetal outcomes. RESULTS: Sixty-nine cases met diagnostic criteria for fetal SVT, of which 56 (81%) received maternal antiarrhythmic therapy. Digoxin was the most common, but least effective, first-line therapy in 28 patients, achieving successful rate reversion in 35.7%. Thirty-one patients (55%) required second-line therapy, and this was most successful with digoxin and flecainide polytherapy achieving rate reversion in 17 of 18 cases (94.5%) at a median of 3 days (1.5-7). Hydrops was present in 23 (33%) cases at initial presentation, 16 of which achieved rate reversion. There was minimal difference in treatment efficacy comparing single- or multiple-agent treatment in the setting of hydrops (50% vs. 42.8%). Side effects occurred in 14/56 treated patients (25%) but were severe in only 8 (14.3%) women, most commonly with digoxin and flecainide polytherapy (6 of 8 cases). There were 3 (4%) fetal deaths amongst the study cohort. CONCLUSIONS: Digoxin and flecainide polytherapy were well tolerated and successfully achieved rhythm and rate control in fetuses with prenatally diagnosed supraventricular tachycardia. The presence of hydrops was a poor prognostic feature.

Longitudinal assessment of ventricular strain, tricuspid and mitral annular plane systolic excursion (TAPSE and MAPSE) in fetuses from pregnancies complicated by diabetes mellitus.

OBJECTIVES: To evaluate fetal cardiac function using myocardial deformation analyses, tricuspid annular plane systolic excursion (TAPSE), mitral annular plane systolic excursion (MAPSE) and diastolic function parameters in pregnancies complicated by maternal diabetes mellitus. METHODS: Myocardial deformation using velocity vector imaging (VVI), TAPSE, MAPSE and diastolic function was measured in 126 women with uncomplicated singleton pregnancies and 50 women with diabetes mellitus. Women underwent ultrasound scans every four weeks from recruitment (18-28 weeks gestational age) until delivery. RESULTS: Left ventricle strain and strain rate, right ventricle strain and strain rate, TAPSE, MAPSE and diastolic parameters were not different between the diabetic cohort and controls throughout gestation. We did not find any significant correlation between the fetal cardiac function parameters with parity or smoking status. There was however a significant difference in strain and strain rate values in the left ventricle, but not the right ventricle in women with BMI >30 kg/m2, and reduced TAPSE values in this same group. Fetuses in the diabetes group had thicker interventricular septum (IVS) throughout gestation. CONCLUSION: Myocardial deformation of the fetal left ventricle, as measured by VVI, and TAPSE were reduced in fetuses of mothers in association with maternal obesity but not in women with diabetes mellitus. No significant differences in the fetal cardiac function parameters measured were different between the two groups, except for IVS thickness.

Global longitudinal reference ranges for fetal myocardial deformation in the second half of pregnancy.

PURPOSE: To construct longitudinal fetal reference ranges for global longitudinal myocardial deformation (strain and strain rate) of the left and right ventricles in the second half of pregnancy. METHODS: A prospective, observational, longitudinal study of 120 women with uncomplicated singleton pregnancies. The participants underwent ultrasonographic scans every 4 weeks from enrolment (18-28 weeks) until delivery. Strain and strain rate were measured at each examination using velocity vector imaging. Changes in strain and strain rate as functions of gestational age (GA) were modeled using Bayesian mixed effects models. RESULTS: A total of 406 assessments of global longitudinal strain and strain rate were performed for 120 women. Global longitudinal strain and strain rate decreased with increasing GA in the left ventricle. There was, however, no change in strain measurements of the right ventricle over the same gestational time frame. Posterior predictive distributions were used to derive reference centiles for each week of GA. CONCLUSION: Assessment of myocardial deformation of the fetal heart is easily performed and may be useful for quantitative assessment of heart function, particularly in fetuses at risk of cardiac dysfunction.

Longitudinal Reference Ranges for Tricuspid Annular Plane Systolic Excursion and Mitral Annular Plane Systolic Excursion in Normally Grown Fetuses.

OBJECTIVES: The aim of this study was to construct reference ranges for fetal tricuspid annular plane systolic excursion (TAPSE) and mitral annular plane systolic excursion (MAPSE) using conventional M-mode ultrasound (US) in the second half of pregnancy. METHODS: Participants underwent US scans every 4 weeks from 18 weeks' gestation until delivery. The TAPSE and MAPSE were measured by conventional M-mode US at each examination. The relationships between TAPSE and MAPSE and gestational age and estimated fetal weight were modeled by Bayesian mixed effects linear regression. RESULTS: Positive linear relationships were observed between both MAPSE and TAPSE and gestational age and estimated fetal weight. Reference centiles for TAPSE and MAPSE were developed. CONCLUSIONS: This simple technique is a useful tool for assessing cardiac function and could be used for quantitative assessments of fetal cardiac function, particularly in high-risk pregnancies such as those complicated by maternal diabetes.

Use of ß-blockade and levosimendan for separation from extracorporeal life support in an infant with postoperative diastolic dysfunction.

Diastolic dysfunction is common in infants and neonates with left ventricular (LV) outflow tract obstruction and may lead to low-cardiac output in the postoperative period. We present a management strategy for severe postrepair diastolic dysfunction in an infant with critical congenital aortic stenosis and LV hypertrophy, employing ß-blockade and levosimendan.

Secondary repair of Ebstein's anomaly with atrioventricular septal defect using the cone technique.

A nine-year-old boy with incomplete atrioventricular septal defect (AVSD) and Ebstein's anomaly had undergone a closure of primum atrial septal defect and repair of right and left AV valves at four years of age. He presented with severe regurgitation of the right AV valve. He underwent further surgery, and a cone reconstruction of the right AV valve was performed. The association of Ebstein's anomaly with AVSD has been reported only rarely. We present a case of this association treated with the previously unreported application of the cone reconstruction (da Silva repair) as a secondary strategy.