Alisa Arunamata

Echocardiographic Diagnosis and Prognosis of Fetal Left Ventricular Noncompaction

BACKGROUND Left ventricular noncompaction (LVNC) has rarely been described in the fetus. METHODS 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. RESULTS 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). CONCLUSIONS 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.

Echocardiographic Measures Associated With Early Postsurgical Myocardial Dysfunction in Pediatric Patients With Mitral Valve Regurgitation

BACKGROUND 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. METHODS 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. RESULTS 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. CONCLUSIONS 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.

Right-Dominant Unbalanced Atrioventricular Septal Defect: Echocardiography in Surgical Decision Making

Background: 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. Methods: 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). Results: 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. Conclusions: 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.

Isolation of the right subclavian artery in a patient with d-transposition of the great arteries.

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.

Maternal arterial stiffness and fetal cardiovascular physiology in diabetic pregnancy

In mothers with pregestational or gestational diabetes, abnormal arterial stiffness (stiffer arteries) has been reported. The impact of abnormal maternal arterial stiffness on placental and fetal cardiovascular physiology is unknown. The purpose of this 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.

Practice Patterns in Postoperative Echocardiographic Surveillance after Congenital Heart Surgery in Children: A Single Center Experience

Objectives To review current institutional practice and describe factors contributing to variation in inpatient postoperative imaging surveillance after congenital heart surgery. Study design 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. Results 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). Conclusions 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.

Chronic antepartum maternal hyperoxygenation in a case of severe fetal Ebstein's anomaly with circular shunt physiology

Perinatal mortality remains high among fetuses diagnosed with Ebsteins 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 Ebsteins anomaly with severe tricuspid valve regurgitation, reversal of flow in the ductus arteriosus, and continuous pulmonary valve regurgitation.

Decompressing vein and bilateral superior venae cavae in a patient with hypoplastic left heart syndrome

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.

Diagnosis of Anomalous Aortic Origin of the Left Coronary Artery in a Pediatric Patient

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.