Utility of serial 12-lead electrocardiograms in children with Marfan syndrome.
Cardiology in the young
Arunamata, A. A., Nguyen, C. T., Ceresnak, S. R., Dubin, A. M., Olson, I. L., Murphy, D. J., Selamet Tierney, E. S.
2018: 1–5
Abstract
OBJECTIVES: The goal of this study was to assess the utility of serial electrocardiograms in routine follow-up of paediatric Marfan patients.METHODS: Children ⩽18 years who met the revised Ghent criteria for Marfan syndrome and received a 12-lead electrocardiogram and echocardiogram within a 3-month period were included. Controls were matched by age, body surface area, gender, race, and ethnicity, and consisted of patients assessed in clinic with a normal cardiac evaluation. Demographic, clinical, echocardiographic, and electrocardiographic data were collected.RESULTS: A total of 45 Marfan patients (10.8 [2.4-17.1] years) and 37 controls (12.8 [1.3-17.1] years) were included. Left atrial enlargement and left ventricular hypertrophy were more frequently present on 12-lead electrocardiogram of Marfan patients compared with controls (12 (27%) versus 0 (0%), p<0.001; and 8 (18%) versus 0 (0%), p=0.008, respectively); however, only two patients with left atrial enlargement on 12-lead electrocardiogram were confirmed to have left atrial enlargement by echocardiogram, and one patient had mild left ventricular hypertrophy by echocardiogram, not appreciated on 12-lead electrocardiogram. QTc interval was longer in Marfan patients compared with controls (427±16 versus 417±22 ms, p=0.03), with four Marfan patients demonstrating borderline prolonged QTc intervals for gender.CONCLUSIONS: While Marfan patients exhibited a higher frequency of left atrial enlargement and left ventricular hypertrophy on 12-lead electrocardiograms compared with controls, these findings were not supported by echocardiography. Serial 12-lead electrocardiograms in routine follow-up of asymptomatic paediatric Marfan patients may be more appropriate for a subgroup of Marfan patients only, specifically those with prolonged QTc interval at their baseline visit.
View details for DOI 10.1017/S1047951118000707
View details for PubMedID 29972109
Abstract
Perinatal mortality remains high among fetuses diagnosed with Ebstein's anomaly of the tricuspid valve. The subgroup of patients with pulmonary valve regurgitation is at particularly high risk. In the setting of pulmonary valve regurgitation, early constriction of the ductus arteriosus may be a novel perinatal management strategy to reduce systemic steal resulting from circular shunt physiology. We report the use of chronic antepartum maternal oxygen therapy for constriction of the fetal ductus arteriosus and modulation of fetal pulmonary vascular resistance in a late presentation of Ebstein's anomaly with severe tricuspid valve regurgitation, reversal of flow in the ductus arteriosus, and continuous pulmonary valve regurgitation.
View details for DOI 10.4103/apc.APC_20_17
View details for PubMedID 28928616
View details for PubMedCentralID PMC5594941
Abstract
Previous studies have suggested reduced pulmonary valve annulus (PVA) growth and progression of pulmonary outflow obstruction in fetuses with tetralogy of Fallot (TOF). The goals of this study were to (1) investigate the trajectory of PVA growth in utero, and (2) compare two methods of z-score determination for fetal and postnatal PVA size by echocardiography in order to improve prenatal counseling for patients with TOF. Fetal echocardiograms (FE) at a single institution with a diagnosis of TOF between 8/2008 and 12/2015 were retrospectively reviewed. Patients included had at least 2 FEs and 1 immediate postnatal echocardiogram (TTE). Fetal and postnatal demographic, clinical, and echocardiographic data were collected. Fetal body surface area (BSA) was calculated by estimating fetal weight and height; z-scores were determined based on fetal gestational age (GA) and BSA for both FEs and TTEs. Fetal PVA z-scores by GA or BSA were then compared to postnatal PVA z-scores by BSA. Twenty-two patients with 44 FEs and 22 TTEs were included. GA at the first FE was 23 weeks ± 3.4 and 32 weeks ± 3.1 at the second FE. There was no difference in PVA z-scores (by BSA) between the first and second FE (p = 0.34), but a decrease in PVA z-scores (by BSA) between the second FE and TTE (- 1.6 ± 0.5 vs. - 2.0 ± 0.7; p = 0.01). Repeat comparison with fetal PVA z-scores indexed to GA revealed no difference in z-scores between the first and second FE, but an increase in PVA z-scores between the second FE (by GA) and TTE (by BSA) (- 4.1 ± 1.0 vs. - 2.0 ± 0.7; p < 0.0001). The rate of PVA growth between the two FEs (23 µm/day ± 9.8) and between the second FE and TTE (28 µm/day ± 42) remained comparable (p = 0.57); however, the rate of BSA increase was greater in later gestation (9 cm2/day ± 3 vs. 20 cm2/day ± 11; p = 0.001). In patients with TOF, the rate of PVA growth appears to remain consistent through gestation; however, somatic growth rate increases in late gestation. Fetal PVA z-scores indexed to GA are thus inaccurate in predicting postnatal PVA z-scores typically indexed to BSA. This observation should be considered during prenatal consultation and delivery planning.
View details for DOI 10.1007/s00246-018-1878-8
View details for PubMedID 29632959
Abstract
In mothers with pre-gestational or gestational diabetes, abnormal arterial stiffness (stiffer arteries) has been reported. The impact of abnormal maternal arterial stiffness on fetal placental and cardiovascular physiology is unknown. The purpose of the study was to determine the impact of maternal diabetes on maternal arterial stiffness and the association with fetal cardiovascular physiology as measured by fetal echocardiography.Between December 2013 and January 2017 we conducted a prospective study on diabetic (otherwise healthy) and non-diabetic, healthy pregnant mothers (at 20-28 gestational weeks and 18-40 years of age) who had a normal fetal cardiac echocardiogram and obstetric ultrasound. Clinical data were collected by means of a patient questionnaire, measurement of blood pressure, height and weight, arterial augmentation index and fetal placental and cardiovascular parameters by fetal echocardiography. Descriptive statistics were calculated. Comparisons were made using parametric and non-parametric tests between controls and diabetic mothers.Twenty-three healthy pregnant controls and 43 diabetic pregnant women (n = 22 pre-gestational, n = 21 gestational) were included in the study. Maternal arterial augmentation index was higher in those with diabetes compared to healthy controls (12.4 ± 10.6% versus 4.6 ± 7.9%; p = 0.003). Fetal aortic valve velocity time integral was higher in fetuses whose mothers had diabetes compared to non-diabetic mothers (7.7 ± 1.9 cm versus 6.3 ± 2.9 cm; p = 0.022). Left ventricular myocardial performance index was lower in diabetic pregnancies compared to controls (0.40 ± 0.09 versus; 0.46 ± 0.11 p = 0.02). Umbilical artery resistance index was lower in diabetic pregnancies with hemoglobin A1C levels ≥6.5% compared to those with HbA1c levels <6.5 (HbA1c ≥6.5%: 0.69 ± 0.06, n = 15 versus HbA1c <6.5%: 0.76 ± 0.08, n = 21; p = 0.009) but not at higher HbA1C cut-offs. A correlation between arterial augmentation index and velocity time integral, myocardial performance index or umbilical artery resistance index was not found.Arterial stiffness is higher in pregnant women with diabetes compared to controls. Fetuses of diabetic mothers show altered cardiovascular parameters with higher velocity time integral and lower left ventricular myocardial performance index, markers of myocardial function. Placental function assessed by umbilical artery resistance was normal despite differences between groups. Arterial stiffness did not correlate with placental or fetal cardiovascular variables. Instead, the findings likely represent a shared response to the environment of abnormal glucose metabolism. The clinical significance of these findings is yet to be determined.
View details for DOI 10.1002/uog.17528
View details for PubMedID 28508434
Abstract
Management of right-dominant atrioventricular septal defect (AVSD) remains a challenge given the spectrum of ventricular hypoplasia. The purpose of this study was to assess whether reported echocardiographic indices and additional measurements were associated with operative strategy in right-dominant AVSD.A blinded observer retrospectively reviewed preoperative echocardiograms of patients who underwent surgery for right-dominant AVSD (January 2000 to July 2013). Ventricular dimensions, atrioventricular valve index (AVVI; left valve area/right valve area), and right ventricular (RV)/left ventricular (RV/LV) inflow angle were measured. A second observer measured a subset of studies to assess agreement. Pearson correlation analysis was performed to examine the relationship between ventricular septal defect size (indexed to body surface area) and RV/LV inflow angle in systole. A separate validation cohort was identified using the same methodology (August 2013 to July 2016).Of 46 patients with right-dominant AVSD (median age, 1 day; range, 0-11 months), overall survival was 76% at 7 years. Twenty-eight patients (61%) underwent single-ventricle palliation and had smaller LV dimensions and volumes, AVVIs (P = .005), and RV/LV inflow angles in systole (P = .007) compared with those who underwent biventricular operations. Three patients undergoing biventricular operations underwent transplantation or died and had lower indexed LV end-diastolic volumes compared with the remaining patients (P = .005). Interobserver agreement for the measured echocardiographic indices was good (intraclass correlation coefficient = 0.70-0.95). Ventricular septal defect size and RV/LV inflow angle in systole had a strong negative correlation (r = -0.7, P < .001). In the validation cohort (n = 12), RV/LV inflow angle in systole ≤ 114° yielded sensitivity of 100% and AVVI ≤ 0.70 yielded sensitivity of 88% for single-ventricle palliation.Mortality remains high among patients with right-dominant AVSD. RV/LV inflow angle in systole and AVVI are reproducible measurements that may be used in conjunction with several echocardiographic parameters to support suitability for a biventricular operation in right-dominant AVSD.
View details for DOI 10.1016/j.echo.2016.10.017
View details for PubMedID 27939051
Abstract
To review current institutional practice and describe factors contributing to variation in inpatient postoperative imaging surveillance after congenital heart surgery.We reviewed records of all children who underwent congenital heart surgery from June to December 2014. Number and primary indications for postoperative transthoracic echocardiograms (TTEs), providers involved, cardiovascular intensive care unit (CVICU) and total hospital length of stay, and Risk-Adjustment for Congenital Heart Surgery-1 scores were recorded.A total of 253 children (age at surgery: 8 months [2 days-19 years]) received 556 postoperative TTEs (median 1 TTE/patient [1-14]), and 23% had ≥3 TTEs. Fifteen of 556 TTEs (2.7%) revealed a new abnormal finding. The majority of TTEs (59%) were performed in the CVICU (1.5 ± 1.1 TTEs/week/patient), with evaluation of function as the most common indication (44%). Attending physician practice >10 years was not associated with fewer TTEs (P = .12). Patients with ≥3 TTEs had higher Risk-Adjustment for Congenital Heart Surgery-1 scores (P = .001), longer CVICU lengths of stay (22 vs 3 days; P < .0001), longer overall hospitalizations (28 vs 7 days; P < .0001), and a higher incidence of mechanical circulatory support (10% vs 0%; P < .0001) than those with <3 TTEs. Eight patients with ≥3 TTEs did not survive, compared with 3 with <3 TTEs (P = .0004).There was wide intra-institutional variation in echocardiographic use among similar complexity surgeries. Frequency of postoperative echocardiographic surveillance was associated with degree of surgical complexity and severity of postoperative clinical condition. Few studies revealed new abnormal findings. These results may help establish evidence-based guidelines for inpatient echocardiographic surveillance after congenital heart surgery.
View details for DOI 10.1016/j.jpeds.2016.09.061
View details for Web of Science ID 000390028100018
Abstract
The levoatrial cardinal vein (LACV), first described in 1926, acts as a decompressing vessel for pulmonary venous return in cases of severe left-sided obstruction with an intact or significantly restrictive atrial septum. The LACV and the persistent left superior vena cava (LSVC) are thought to share similar embryologic origins. To challenge this notion, we present a unique case of a neonate with hypoplastic left heart syndrome, cor triatriatum, and a decompressing LACV in the presence of bilateral superior venae cavae.
View details for DOI 10.1111/echo.13292
View details for PubMedID 27641733
Abstract
Anomalous aortic origin of the left coronary artery is rare and confers increased risk of sudden cardiac death. Accurate diagnosis is crucial and often requires many diagnostic modalities. This case report highlights the echocardiographic characteristics and pitfalls in diagnosing the anomaly in addition to the advantages of using computed tomography and magnetic resonance imaging in a pediatric patient.
View details for DOI 10.1177/2150135114558689
View details for PubMedID 26180168
Abstract
Isolation of the right subclavian artery (RSCA) is rare, and this finding in association with d-transposition of the great arteries (d-TGA) is extremely unusual. We present a case of an isolated RSCA in a newborn with d-TGA in whom the clinical presentation was diagnostic. We discuss the imaging modalities used to confirm the diagnosis, the embryological basis of the finding, and the surgical repair.
View details for DOI 10.4103/0974-2069.154154
View details for PubMedID 26085773
View details for PubMedCentralID PMC4453190
Abstract
The assessment of left ventricular (LV) systolic function using conventional echocardiographic measures is problematic in the setting of mitral regurgitation (MR) given that altered loading conditions can mask underlying ventricular dysfunction. The purpose of this study was to characterize LV function and deformation before and after effective mitral valve repair or replacement to determine echocardiographic measures associated with early postoperative myocardial dysfunction.Baseline LV function was assessed retrospectively by conventional echocardiography and speckle-tracking strain analysis pre- and postoperatively in patients diagnosed with MR between January 2000 and March 2013, excluding patients with less than mild to moderate MR preoperatively, left-sided obstructive lesions, large septal defects, or more than mild MR postoperatively.Forty-six pediatric patients were evaluated (average age, 8.2 ± 6.4 years). Thirteen patients had normal preoperative ejection fractions but significant postoperative dysfunction (defined as an ejection fraction < 50%). Compared with the 33 patients with normal postoperative function, age (11.5 ± 7.1 vs 7.3 ± 5.7 years, P = .04), global circumferential strain (-13.2 ± 5.6% vs -17.1 ± 4.6%, P = .02), and global circumferential strain rate (-0.94 ± 0.40 vs -1.36 ± 0.42 sec(-1), P = .004) were found to be statistically different. Using receiver operating characteristic curves, an older preoperative age (area under the curve, 0.67; P = .03), lower global circumferential strain magnitude (area under the curve, 0.74; P = .007), and lower global circumferential strain rate magnitude (area under the curve, 0.80; P = .0004) were determined to be factors associated with early postoperative LV dysfunction after surgical repair of MR.Strain measurements may be useful as part of the echocardiographic assessment of patients with MR and can guide timing for surgical repair in the pediatric population.
View details for DOI 10.1016/j.echo.2014.11.010
View details for Web of Science ID 000352144400004
View details for PubMedID 25555521
Abstract
Left ventricular noncompaction (LVNC) has rarely been described in the fetus.The presence of associated congenital heart disease and rhythm disturbance was identified and the presence of heart failure was assessed using the cardiovascular profile score in all fetuses with LVNC presenting from January 1999 to July 2010. The left ventricle was divided into 12 segments-four segments each at the base, midpapillary, and apical regions-in the short-axis view to calculate the noncompaction/compaction ratio for each segment.Of 24 fetuses with LVNC included in the study, 22 had significant congenital heart disease, and 15 had complete heart block. Of the 16 patients with adequate follow-up and not electively terminated, 12 (81%) died or progressed to heart transplantation. The average noncompaction/compaction ratios were 2.02 in patients who died or underwent heart transplantation and 1.67 in survivors (P = .2034). Fifty-seven of 93 measured segments (61%) of the left ventricle in the patients who died or underwent heart transplantation had noncompaction/compaction ratios ≥ 2 compared with five of 17 measured segments (29%) in survivors (P = .0837). The average cardiovascular profile score was 6. The apical region had greater involvement of noncompaction than the midpapillary and basal regions, with ratios of 2.27, 2.14, and 1.10, respectively (P = .00035).Fetuses with LVNC have a poor prognosis that may be related to associated congenital heart disease, increased segmental involvement of noncompaction, and complete heart block and can be predicted by the cardiovascular profile score.
View details for DOI 10.1016/j.echo.2011.09.019
View details for Web of Science ID 000298276500013
View details for PubMedID 22014428
Abstract
Osseous metaplasia of the endometrium is a rare disorder and can be associated with infertility. Although successful diagnosis and treatment have been widely reported, correct diagnosis in many cases still represents a challenge.A 40-year-old woman complaining of infertility presented with a diagnosis of retained intrauterine device (IUD) on ultrasound. Hysteroscopy revealed a normal endometrial cavity, but no IUD was visualized. Curettage pathology specimens showed chronic endometritis and calcification. Repeat hysteroscopy was performed because of persistent echogenic foci in the endometrium on follow-up ultrasound. Several irregular and calcified plaques were successfully removed.Osseous metaplasia can be misdiagnosed because of its rare incidence. Physicians should be aware of osseous metaplasia in the differential diagnosis of patients with uncertain history who present with a sonographic image resembling an IUD.
View details for Web of Science ID 000261222000012
View details for PubMedID 19097523
