Echocardiogram-Guided Balloon Valvuloplasty of the Aortic Valve in Neonates and Infants Reduces Contrast Exposure with Maintained Efficacy and Less Aortic Regurgitation.

Balloon aortic valvuloplasty (BAV) is performed in children with significant aortic stenosis (AS). Traditionally, contrast angiography measures the annulus and assesses aortic regurgitation (AR) after each dilation. Echocardiographic guidance is hypothesized to reduce contrast and radiation exposure, without compromising efficacy or safety. Patients < 10 kg undergoing BAV from 2013 to 2022 were retrospectively investigated. Agreement between echocardiographic and angiographic annulus measurements was assessed. Echocardiogram-guided (eBAV) and traditional angiogram-guided (tBAV) outcomes were compared controlling for weight, critical AS, and other congenital heart disease (CHD). Twelve eBAV and 19 tBAV procedures were performed. The median age was 33 days, median weight was 4.3 kg, 7 patients (23%) had critical AS, and 9 patients (29%) had other CHD. Annulus measurements by intraprocedural echocardiography and angiography displayed excellent correlation (ICC 0.95, p < 0.001). eBAV patients received less contrast (0.5 vs 3.5 ml/kg, p < 0.01). Five recent eBAV procedures were performed contrast free. Radiation exposure was not statistically different between the eBAV and tBAV groups (155 vs 313 µGy·M2, p = 0.12). One eBAV patient (8%) and 3 tBAV patients (16%, p = 0.62) experienced serious adverse events. Technical success (gradient < 35 mmHg and increase in AR by ≤ 1 grade) occurred in 11 eBAV patients (92%) and 16 tBAV patients (84%, p = 0.22). AR increased in 2 eBAV patients (17%) and 8 tBAV patients (44%, p = 0.02). eBAV was associated with similar efficacy and significantly lower contrast exposure and risk of aortic regurgitation. There was strong agreement between aortic valve annulus measurements by intraprocedural echocardiography and angiography, ultimately permitting contrast free BAV.

Prenatally Diagnosed Interventricular Septal Aneurysm with Associated Ventricular Dysfunction.

Congenital interventricular septal aneurysms (IVSA) of the muscular septum are rare and can be associated with other familial abnormalities of the ventricular septum, arrhythmias, additional congenital heart disease, and chromosomal abnormalities. IVSA is also linked to ventricular dysfunction and non-compaction, although there are limited reports of this association presenting in utero. We describe a case of fetal ventricular septal aneurysm associated with ventricular dysfunction and pericardial effusion.

Combined Echo and Fluoroscopy-Guided Pulmonary Valvuloplasty in Neonates and Infants: Efficacy and Safety.

Percutaneous balloon pulmonary valvuloplasty (PBPV) is the treatment of choice for isolated pulmonary valve stenosis. While this procedure is highly efficacious and has an excellent safety profile, as currently practiced, patients are obligatorily exposed to the secondary risks of ionizing radiation and contrast media. To mitigate these risks, we developed a protocol which utilized echo guidance for portions of the procedure which typically require fluoroscopy and/or angiography. Ten cases of echo-guided pulmonary valvuloplasty (EG-PBPV) for isolated pulmonary stenosis in children less than a year of age were compared to a historical cohort of nineteen standard cases using fluoroscopy/angiography alone, which demonstrated equivalent procedural outcomes and safety, while achieving a median reduction in radiation (total dose area product) and contrast load of 80% and 84%, respectively. Our early experience demonstrates that EG-PBPV in neonates and infants has results equivalent to standard valvuloplasty but with less radiation and contrast.

Deployed Dimensions of the GORE® CARDIOFORM ASD Occluder as Function of Defect Size.

The GORE® CARDIOFORM ASD occluder (ASDO) is approved for closure of ASDs up to 35 mm diameter. With an adaptable central waist, each device size is suitable over a range of defect diameters. Understanding deployed dimensions across various defect sizes will assist operators. Therefore, this study investigates the deployed dimensions of the ASDO as a function of defect size. A 2-mm-thick ASD model with circular defects ranging from 5 to 35 mm was 3D printed. Diameter, width, and left-right disc diameter were measured by fluoroscopy after ASDO devices were deployed in applicable defects. Linear regression evaluated relationships between device size, defect size, and deployed dimensions. Six ASDOs of each size (27, 32, 37, 44, and 48 mm) were deployed in all applicable defects. There was significant ASDO size-defect size interaction in determining deployed ASDO diameter. Diameter was positively associated with defect size for 48-mm (B = 0.13, p < 0.001) and 44-mm (B = 0.11, p < 0.001) ASDOs, while no association was seen for 27-mm, 32-mm, or 37-mm ASDOs. No such interaction existed for deployed width or left-right disc difference. Controlling for ASDO size, width (B = - 0.12, p < 0.001) and left-right disc difference (B = - 0.06, p < 0.001) were negatively associated with defect size. In smaller defects, the 44-mm and 48-mm ASDOs display progressive diameter foreshortening, and all devices display progressive increase in width and left-right disc asymmetry. Anticipating the degree of diameter foreshortening may be critical when attempting closure of fenestrated lesions and/or in patients with limited total atrial septal length.

Impact of Breast Milk, Respiratory Insufficiency, and Gastroesophageal Reflux Disease on Enteral Feeding in Infants With Omphalocele.

OBJECTIVES: The aim of this study was to document the process of achieving full enteral feeding in infants with omphalocele and to identify factors that affect feeding success. METHODS: After institutional review board approval (study no. 5100169), 123 infants with omphalocele, born between 1993 and 2011 were reviewed. Mortalities were excluded. All survivors had complete follow-up. Variables suspected to impact enteral feeding in infants with non-giant versus giant omphalocele were compared. Independent t test, Mann-Whitney, and χ test were used. Regression evaluated for variable independence. RESULTS: Of 123 infants with omphalocele, 97 (79%) survived, 62/97 (64%) had non-giant, and 35/97 (36%) giant omphalocele. For survivors, the mean gestational age was 37 ±â€Š4 weeks with median follow-up of 4.4 years (range: 1.4-7.4 years). The median time to full feeds was 4 days (range: 0-85 days) for non-giant versus 8 days (range: 1-96 days) for giant, a significant difference (P < 0.01). Breast milk significantly decreased time to full feeds independent of omphalocele size. Giant omphalocele infants had a significantly higher incidence of respiratory insufficiency at birth (P < 0.01) and sac rupture (P = 0.02), but fewer chromosomal anomalies (P = 0.04). Respiratory insufficiency at birth (P < 0.01) and gastroesophageal reflux disease (P < 0.01) independently delayed feeding in omphalocele infants. CONCLUSIONS: Infants with non-giant omphalocele can achieve full enteral feeds within the first week of life, but giant omphalocele infants require significantly more time. Breast milk independently promotes feeding success whereas gastroesophageal reflux disease and respiratory insufficiency at birth independently delay feeding in infants with omphalocele.

Interventricular Septal Hematoma Complicating Ventricular Assist Device Placement in an Infant: Case Report and Review of the Literature.

Interventricular septal hematoma is a rare and life-threatening complication of pediatric cardiac surgery. Commonly seen following ventricular septal defect repair, it has also been associated with ventricular assist device (VAD) placement. Although conservative management is usually successful, operative drainage of interventricular septal hematoma occurring in pediatric patients undergoing VAD implantation should be considered.

Left Atrial Hypertension and Respiratory Failure Requiring Venoarterial ECMO After Transcatheter Pulmonary Valve Replacement.

A 33-year-old woman with aortic valve stenosis status-post Ross at age 6 years developed symptomatic right heart failure from right ventricle to pulmonary artery conduit stenosis. Conduit rehabilitation and transcatheter pulmonary valve replacement resulted in acute left atrial hypertension and respiratory failure requiring venoarterial extracorporeal membrane oxygenation and atrial septal defect creation as a bridge to recovery.

Intervendor Agreement for Right Ventricular Global Longitudinal Strain in Children.

BACKGROUND: Right ventricular global longitudinal strain (RVGLS) has emerged as an important technique for clinical evaluation of (RV) function. The routine application of RVGLS in pediatrics remains limited by a lack of data on agreement between vendors. The aim of this study was to investigate intervendor agreement for RVGLS between the two commonly used analysis vendors in pediatrics, hypothesizing that RVGLS has good intervendor agreement, although it is likely lower than intravendor agreement (inter- and intraobserver reproducibility). METHODS: Seventy infants and children with normal cardiac anatomy and varying ventricular function were included after prospectively obtaining RV-focused four-chamber apical images on the GE Vivid E95. Images were analyzed for RVGLS at acquired frame rates in EchoPAC (GE) and TomTec (TT) and in the compressed Digital Imaging and Communications in Medicine format in TT. Intraclass correlation coefficients and Bland-Altman plots were used to test intervendor agreement and intravendor reproducibility. RESULTS: RVGLS measurements were equally feasible using TT and EchoPAC analysis (92%). There was good to excellent agreement for RVGLS between TT and EchoPAC analysis, with a relatively higher intraclass correlation coefficient between GE and TT at the acquired frame rate (0.85) than between GE and TT at the compressed frame rate (0.75) and significantly higher agreement in patients with abnormal RV function (0.7-0.9) than those with normal function (0.4-0.6). Intra- andinterobserver reproducibility for RVGLS was excellent (intraclass correlation coefficient = 0.74-0.96). Heart rate ≥ 100 beats/min and acquisition frame rate/heart rate ≤ 0.7 were associated with diminished agreement, especially when compressed data were involved. CONCLUSIONS: RVGLS analyzed using EchoPAC and TT show good agreement, especially when analyzed at acquisition frame rates and in the setting of abnormal RV function. Otherwise, RVGLS should ideally be analyzed using the same vendor, and intervendor comparisons should be undertaken with caution, particularly if data are in a compressed format.

Reproducibility and Intervendor Agreement of Left Ventricular Global Systolic Strain in Children Using a Layer-Specific Analysis.

BACKGROUND: Speckle-tracking strain analysis provides additive data to the assessment of pediatric and congenital heart disease; however, the variety of strain analysis software platforms by different vendors and the lack of data on intervendor strain agreement in children have limited its utility. The purpose of this study is to evaluate the intervendor agreement of strain on two commonly used analysis platforms in pediatrics by layer of myocardium and data compression. METHODS: This prospective study analyzed two-dimensional speckle-tracking strain on two software platforms in 53 children with normal cardiac segmental anatomy and varying function. Three standard apical views and one parasternal short-axis view were exported at their acquired frame rates to workstations with GE EchoPAC and TomTec software and then also to TomTec at compressed frame rates. Both software platforms had been updated with European Association of Cardiovascular Imaging/American Society of Echocardiography Task Force recommendations for left ventricular (LV) global strain. Intravendor and intervendor agreement between layer-specific comparisons were assessed using Bland-Altman analysis (limits of agreement and bias) and intraclass correlation coefficients. RESULTS: This study included subjects with normal LV function (n = 38) and cardiomyopathy (n = 15) with an age range of 1 month to 18 years. Intertechnique agreement by default vendor myocardial layer (GE mid-TomTec endocardial layer) was robust for both global longitudinal (GLS) and circumferential strain (GCS; higher for GLS than GCS). Intravendor (inter- and interreader) agreement was slightly higher than intervendor. Only small differences in intraclass correlation coefficients were present between various myocardial layers and acquired versus compressed TomTec data with narrow limits of agreement and small bias except in certain subgroup comparisons. CONCLUSIONS: Comparison of LV GLS and GCS between two commonly used software platforms after European Association of Cardiovascular Imaging/American Society of Echocardiography Industry Task Force recommendations demonstrated good to excellent agreement in pediatrics, regardless of the layer of analysis or the image format, although some degree of variability remains between vendor platforms. Overall, GLS agreement was more robust than GCS, and this difference is exaggerated in specific subanalyses. These data suggest that comparisons of strain values obtained on these two vendors will be reasonable, but caution should be used when the indication is the detection of small differences between serial echocardiograms.

Neonatal acute myocardial infarction of unknown etiology treated with surgical thrombectomy.

Coronary artery thrombosis and resultant myocardial infarction in neonates are exceedingly rare. Seldom does a neonate survive this myocardial insult. Often there is an underlying predisposition to coronary artery thrombosis, such as thrombophilia, central line placement, or myocarditis. Treatment attempts have consisted of thrombolysis and supportive care. We present a case of acute left main coronary artery (LMCA) thrombosis of unknown etiology in a neonate, without ventricular dilatation. Flow into the LMCA was suggested echocardiographically, but a high clinical suspicion led to urgent cardiac catheterization. Once complete occlusion of the LMCA was demonstrated angiographically, emergent surgical thrombectomy was performed, which has never previously been reported.