Perforation and right ventricular outflow tract stenting: Alternative palliation for infants with pulmonary atresia/ventricular septal defect.

BACKGROUND: Right ventricular outflow tract (RVOT) stents have been used as palliation in patients with severe tetralogy of Fallot (TOF). Radiofrequency perforation of the RVOT has also been described in patients with pulmonary atresia (PA)/ventricular septal defect (VSD). However, RVOT stenting in conjunction with radiofrequency perforation as a means for establishing reliable pulmonary blood flow in patients with PA/VSD has not previously been reported. OBJECTIVES: Our aim is to report our experience with using perforation of plate-like pulmonary valve atresia combined with stenting of RVOT as an alternative and equally efficacious intervention for infants with PA/VSD, as compared to a surgical pulmonary artery shunt (SPS). METHODS: A retrospective review of patients with PA/VSD at our institution from Jan 2006 to October 2015 was performed. RESULTS: Twenty-seven patients received palliation for PA/VSD. Five (median 2.5 kg, 2.1-4.1 kg) underwent RVOT stenting and 22 (3.5 kg, 2.1-4.6 kg) underwent surgical SPS. Of the patients who received an RVOT stent, 3 underwent guidewire perforation and balloon dilation of the plate-like pulmonary valve, and 2 underwent radiofrequency perforation of the pulmonary plate, followed by balloon dilation. Postprocedure saturations averaged 93% in the stent group and 89% in the shunt group. One of the patients who underwent RVOT stenting required additional stenting of the PDA for isolation of the LPA, but none required re-intervention. Four patients in the SPS cohort required re-intervention (18%). Two required stenting of the shunt, and 2 required surgical revision of the shunt. There was one death in the stent group, however this was secondary to withdrawal of support given an underlying genetic diagnosis (trisomy 13). There was one fatality in the SPS cohort prior to hospital discharge. CONCLUSIONS: Perforation followed by RVOT stenting may be a safe and effective alternative to surgical SPS in infants with PA/VSD.

Stenting of the ascending aorta revisited.

Despite few institutions stenting the ascending aorta, it has been discouraged because of the proximity of the aortic valve, the coronary artery orifices, and the aortic arch branches. We describe a small case series of patients having acquired stenosis of the ascending that was relieved successfully by stenting. © 2017 Wiley Periodicals, Inc.

Normal Range of Left Ventricular Strain, Dimensions and Ejection Fraction Using Three-dimensional Speckle-Tracking Echocardiography in Neonates.

AIMS: Three-dimensional speckle-tracking echocardiography (3D-STE) is a promising new technique to evaluate left ventricular (LV) mechanics. The feasibility and normal values of LV strain using 3D-STE have recently been established in adults and children. Unfortunately, no data are available in neonates. The aims of this study were to evaluate the feasibility and establish normal values of 3D LV volumes, ejection fraction (EF), and the 4 normal strains in healthy neonates. MATERIALS AND METHODS: Of 50 consecutive newborns who were delivered at our hospital or returned to their first newborn follow-up within the first 3 weeks of life, 38 babies underwent full echocardiographic evaluation, including the acquisition of at least 3 full volume data sets from the apical window, while naturally sleeping. Data sets were analyzed offline. Global LV longitudinal, circumferential, and radial strain, as well as 3D LV volumes and EF, were measured using 3D-STE. RESULTS: Of the 50 newborns, 2 patients were excluded because of significant intra-cardiac shunts, and in another 10 subjects, parents did not give consent. At least one data set was adequate for analysis in all the remaining subjects. Mean indexed LV diastolic, systolic volumes, and EF were 24.7 ± 3.6 ml/m2, 9.2 ± 1.3 ml/m2, and 63% ± 3.7%, respectively. Normal global longitudinal, circumferential, radial, and tangential 4D strain were -20.9% ± 2.8%, -32.4% ± 3.1%, 44.3% ± 3.4%, and -39.7% ± 3.4%, respectively. CONCLUSIONS: 3D-STE is feasible in newborns without the needed for sedation. Reference values of normal, regional, and global LV 4D strain and volumes were obtained.

Mid-term differences in right ventricular function in patients with congenital diaphragmatic hernia compared with controls.

BACKGROUND: Patients with congenital diaphragmatic hernia (CDH) may have abnormal lung development, which may cause detrimental effects on right ventricular (RV) function. This study aimed to determine if there are persistent echocardiographic differences in RV function in patients with CDH years after repair versus control patients. METHODS: Patients who underwent repair for CDH were recruited. RV function was evaluated by strain analysis and tissue Doppler imaging (TDI). Wilcoxon's rank-sum test was used for analysis. RESULTS: Seven CDH patients and 16 control patients were studied. There was no difference in age between the CDH and control groups (6.2±1.7 years vs. 5.7±1.7 years). TDI demonstrated significantly lower values in the RV early diastolic wave (12.8±1.5 cm/s vs. 16.1±3.1 cm/s) and RV systolic wave (10.2± 0.8cm/s vs. 13.4±1.3 cm/s) when comparing the CDH group and the control group. Interventricular apical septal strain was signifi cantly lower in the CDH group than in the control group (-20.1±4.6% vs. -25.4±4.1%). There was a trend towards lower strain values in the RV mid-lateral segment in the CDH group (-30.8±9.9% versus -39.7±6.0%, P=0.06) and a lower global RV strain (-27.8±3.0% vs. -31.1±3.1%, P=0.06). CONCLUSIONS: Patients who underwent CDH repair continue to have differences in RV function years after repair. Follow-up is needed to determine how these differences impact cardiac function in adult survivors of CDH.

Strain echocardiographic assessment of ventricular function after percutaneous pulmonary valve implantation.

INTRODUCTION: Percutaneous pulmonary valve implantation (PPVI) is an emerging therapy for pulmonary valve dysfunction. Minimal data on the midterm effects of PPVI on ventricular function exist. We describe the effects of PPVI on right and left ventricular (RV, LV) function with speckle tracking echocardiography. METHODS: Patients who met the inclusion criteria of the Food and Drug Administration Phase 1 Feasibility Clinical Trial PPVI were identified. Patients were studied with echocardiograms at baseline, post-PPVI (day of discharge), 3 months, and at 6 months. Patients were studied by cardiac magnetic resonance at baseline and at 6 months. Longitudinal strain was measured at the basal, mid, and apical portions of the RV, interventricular septum (IVS), and LV. Global RV and LV strain and strain rates were recorded. Paired t-tests were used for analysis. RESULTS: Ten patients were analyzed: nine patients were a variant of tetralogy of Fallot and one patient had complex LV outflow obstruction requiring a Ross and RV-pulmonary atresia conduit. Mean age was 24.4 ± 7.6 years. Indication for PPVI was pulmonary regurgitation in six patients, stenosis in two patients, and stenosis/regurgitation in two patients. After PPVI, both RV systolic pressure and RV to pulmonary artery pressure gradient significantly decreased. Cardiac magnetic resonance RV end-diastolic volume significantly decreased. IVS-mid, IVS-apical, and LV-global strain significantly increased and RV-basal decreased immediately after PPVI. Global RV a' strain rate significantly increased immediately after PPVI. However, RV, IVS, and LV strain/strain rate values between baseline and the 6 month echocardiographic study were either similar or significantly decreased. CONCLUSION: Despite improvement in RV hemodynamics, there was a decrease or no improvement in RV and LV function as measured by strain echocardiographic values at midterm follow-up. Larger studies with longer follow-up are needed to determine if these results remain consistent.

Two-dimensional speckle strain and dyssynchrony in single left ventricles vs. normal left ventricles.

BACKGROUND: Patients with single left ventricle (LV) physiology maybe at increased risk for myocardial dysfunction and mechanical dyssynchrony. Newer echocardiographic modalities may be able to better quantitate LV function in this unique population. Our objective was to use 2D-speckle analysis of strain and strain rate to quantify systolic function and dyssynchrony in single LV post-Fontan patients and compare them to control LV patients. METHODS: Patients with single LV status post-Fontan procedure and patients with normal biventricular anatomy were studied. Two-dimensional speckle echocardiography was used to measure strain, strain rate, time to peak, and longitudinal displacement in a six-segment model of the LV. Independent t-tests were used to compare group means. RESULTS: Twelve patients with single LV physiology and thirteen control patients were studied. There was no significant difference in ages for the single LV patients vs. control patients (7.1 + 2.8 years vs. 5.7 + 1.8 years). Single LV strain values were significantly lower in four of the six segments compared with control LV (mid interventricular septal [IVS]: -19.3 + 6.4% vs. -23.8 + 3.5%, apical IVS: -20.8 + 5.4% vs. -27.4 + 4.7%, basal LV: -20.6 + 6.6% vs. -25.8 + 3.4%, and apical LV: -21.0 + 6.2% vs. -26.1 + 4.3%). Longitudinal displacement was lower for all three segments of the IVS for the single LV vs. control LV. Modified Yu index for strain and strain rate time to peak was longer in the single LV patients vs. controls (51.2 + 18.2 ms vs. 27.9 + 8.0 ms and 90.2 + 24.2 ms vs. 52.5 + 23.7 ms, respectively). CONCLUSION: Significant differences in strain analysis between single LV patients vs. control patients with normal biventricular physiology exist at a relatively young age. Future studies are needed to determine the significance of these differences.

Differences in tissue Doppler imaging between single ventricles after the fontan operation and normal controls.

Tissue Doppler imaging measurements could be used to quantitate ventricular function in patients with systemic single ventricles. The objective of the present study was to measure the tissue doppler imaging-derived indexes of function and dyssynchrony in patients with single ventricular physiology and compare these values with those from control patients. Echocardiograms were performed in patients with single left or right ventricular physiology and control patients. Measurements were made of the free walls at the level of the atrioventricular annulus. Differences were assessed with one-way analysis of variance. Post hoc comparisons were performed with a Tukey adjustment. No significant difference was present in age among the control (5.75 +/- 1.73 years, n = 16), single left ventricle (7.07 +/- 2.81 years, n = 12), or single right ventricle (6.25 +/- 2.2 years, n = 14) groups. The single right ventricular free wall peak systolic annular, peak early diastolic annular, and peak late diastolic annular velocities were significantly lower and the myocardial performance index and interval to mechanical activation greater compared to the controls. The single left ventricular free wall peak systolic annular, peak early diastolic annular, and peak late diastolic annular velocities were also significantly lower and the myocardial performance index and interval to mechanical activation greater compared to controls. In conclusion, significant decreases in systolic and diastolic function, as well as differences in dyssynchrony, using tissue Doppler imaging exist in patients with single ventricular physiology compared to control patients.

Two-dimensional speckle strain and dyssynchrony in single right ventricles versus normal right ventricles.

BACKGROUND: Patients with single-right ventricle (RV) physiology are at increased risk for myocardial dysfunction and mechanical dyssynchrony. Newer echocardiographic modalities may be better able to quantitate right ventricular function in this unique population. The aim of this study was to use two-dimensional speckle analysis of strain and strain rate to quantify systolic function and dyssynchrony in single-RV post-Fontan patients and compare them with values for controls. METHODS: Patients with single RV who underwent Fontan palliation and patients with normal biventricular anatomy were studied. Two-dimensional speckle echocardiography was used to measure strain, strain rate, time to peak, and longitudinal displacement in a 6-segment model of the RV. Independent t tests were used to compare group means. P values < .05 were considered significant. RESULTS: Thirteen patients were studied in each group. There was no significant difference in age between single-RV patients and controls (6.60 +/- 2.07 vs 5.75 +/- 1.83 years, respectively). Single-RV strain values were significantly lower in all 6 segments compared with values in controls (basal interventricular septum [IVS], -14.28 +/- 7.78% vs -22.00 +/- 2.36%; mid IVS, -17.70 +/- 4.54% vs -22.99 +/- 2.71%; apical IVS, -19.46 +/- 4.97% vs -25.42 +/- 4.06%; basal RV, -22.40 +/- 5.7% vs -41.42 +/- 5.42%; mid RV, -21.20 +/- 3.21% vs -39.67 +/- 6.04%; apical RV, -20.70 +/- 4.90% vs -33.68 +/- 3.90%). Systolic strain rate and longitudinal displacement were also lower in the free wall and apical IVS in single-RV patients compared with controls. The modified Yu index for strain time to peak was longer in the single-RV patients (43.16 +/- 13.63 vs 21.72 +/- 7.25 ms). CONCLUSION: Significant differences in strain analysis between single-RV patients and patients with biventricular physiology exist at a relatively young age. Future studies are needed to determine the clinical significance of these differences.

Effect of transcatheter pulmonary valve implantation on short-term right ventricular function as determined by two-dimensional speckle tracking strain and strain rate imaging.

Transcatheter pulmonary valve implantation (PVI) is an emerging therapy for right ventricular (RV) outflow dysfunction in congenital heart disease. We investigated, for the first time in children after surgery for congenital heart disease, the short-term effects of PVI on RV and left ventricular (LV) function using 2-dimensional speckle tracking echocardiography and tissue Doppler imaging. We hypothesized that the short-term RV and LV function would improve. Two-dimensional speckle tracking echocardiograms and pulsed tissue Doppler images were obtained before and 1 to 2 days after PVI (18-mm Melody valve). The catheter right heart hemodynamics were recorded. The strain and strain rate of the basal lateral left ventricle, lateral right ventricle, and interventricular septum were measured by 2-dimensional speckle tracking echo, and the pre- and postprocedure values were compared. Of the 16 eligible patients (age 16 +/- 2 years), the scans of 10 had correct image format and adequate quality. PVI was performed for volume (n = 4) or combined pressure-volume (n = 6) loading. After PVI, the RV to pulmonary artery pressure gradient (33 +/- 22 to 12 +/- 4 mm Hg, p = 0.02), pulmonary regurgitation, and RV end-diastolic volume (3.2 +/- 0.8 to 2.8 +/- 0.6 cm, p = 0.02) decreased, and the septal systolic velocities (3.5 +/- 1.1 to 4.7 +/- 1.1 cm/s, p = 0.04), strain (-7.6 +/- 9.3% to -15.6% +/- 6.7%, p = 0.01) and strain rate (-0.3 +/- 1.1 to -1.1 +/- 0.5 1/s, p = 0.04) and RV free wall strain increased (-17.4 +/- 8.6% to -23.4% +/- 6.2%, p = 0.03). The LV tissue velocities, strain, and strain rate were unchanged. In conclusion, PVI leads to RV unloading and acutely improves RV and septal function.

Amplatzer septal occluder associated with late pulmonary venous obstruction requiring surgical removal with acquired aorta to left atrial fistula.

The Amplatzer septal occluder is currently the preferred device for the transcatheter closure of secundum atrial septal defects. Multiple studies have shown that device complications with the Amplatzer occluder are rare and often acute in presentation. The investigators describe the first reported case of late obstruction of the right pulmonary veins with an Amplatzer septal occluder and, in the same patient, an unusual intraoperative finding of a noncoronary aortic sinus to left atrium fistula after device removal.

Putative neutralization epitopes and broad cross-genotype neutralization of Hepatitis E virus confirmed by a quantitative cell-culture assay.

Monolayers of Hep G2/C3A cells were inoculated with genotype 1 Hepatitis E virus (HEV) mixed with either anti-HEV or an appropriate control. After 5 or 6 days, cell monolayers were stained with anti-HEV and infected cells were identified by immunofluorescence microscopy and counted. Anti-HEV from vaccinated or infected rhesus monkeys neutralized the virus, as did mAbs that recognized epitopes on the C terminus of a recombinant vaccine protein. Antibodies were broadly cross-reactive, since convalescent serum from animals infected with any one of the four mammalian genotypes all neutralized the genotype 1 virus.