Use of Speckle Tracking Echocardiography to Assess Left Ventricular Systolic Function in Patients with Surgically Repaired Tetralogy of Fallot: Global and Segmental Assessment.

Patients with repaired Tetralogy of Fallot (rToF) can have significant right and left ventricular dysfunction which can impact clinical outcome. Conventional echocardiographic evaluation using ejection fraction can be limited in detecting early stages of deteriorating left ventricular (LV) systolic function. Speckle-tracking echocardiography (STE) provides a promising emerging tool for early detection of LV systolic dysfunction. The aim of this cross-sectional study is to evaluate the left ventricular global and regional function using STE in rToF patients with preserved left ventricular ejection fraction to detect early signs of LV dysfunction. The study is a retrospective review of the medical records and transthoracic echocardiograms of rTOF patients who were seen in the pediatric cardiology clinic at our institution from August 2016 till May 2018. The rTOF group was compared with a matched healthy control group. Offline strain analysis of TTE studies was completed in each subject according to mode set by the American Society of Echocardiography. Twenty-two patients with rTOF and 22 age-matched controls (rTOF 18.7 ± 6 years and control 14.6 ± 9.6 years, P value 0.19) were included during the study period. The rTOF group consisted of 12 males and 10 females, whereas the control group consisted of 15 males and 7 females. There was no significant difference in terms of LV ejection fraction using both 2-chamber as well as 4-chamber views. Global LV longitudinal strain was significantly lower in the rTOF group compared with the control group (rTOF - 15.92 ± 3.16 vs control - 22.79 ± 2.45, P value 0.00). All 17 segments showed significantly lower longitudinal strains in rTOF group compared with the control group. Global circumferential left ventricle strain was significantly lower in the rTOF patients compared with the control group (- 17.24 ± 5.86 vs - 22.74 ± 3.99, P value 0.001). 13 out of 16 segments had lower circumferential strains in the rTOF patients compared with controls. Asymptomatic surgically repaired TOF patients exhibited abnormal segmental and global left ventricle longitudinal and circumferential strains despite having normal left ventricle ejection fraction. Our findings suggest subclinical damage to the left ventricle systolic function post-surgical repair of TOF. 2-D speckle-tracking echocardiography may be used as a valuable method in the early recognition of left myocardial systolic function impairment in patients after TOF repair. The application of this method to guide clinical decision and management of rTOF patients such as earlier pulmonary valve replacement or risk stratification for sudden cardiac death needs further investigation.

Determination of the Frequency of Right and Left Internal Mammary Artery Embolization in Single Ventricle Patients: A Two-Center Study.

Embolization of systemic to pulmonary artery collaterals to regulate pulmonary arterial flow or pressure of the cavopulmonary circulation in patients with single ventricle is a common practice. The relative incidence and impact of this practice on future interventions like coronary artery bypass grafting is poorly understood. This study aims to evaluate the frequency and implications of internal mammary artery (IMA) embolization in the single ventricle (SV) population. A retrospective chart review was performed of SV patients who underwent cardiac catheterization before and after Fontan procedure between February 2007 and 2017. Data were collected from two tertiary care centers in the Midwest. Of the 304 SV patients, 62 (20.4%) underwent embolization of one or more IMAs, whereas 242 (79.6%) did not. The rate of embolization of IMA was 40.5% in one center and 14.5% in the second center. Among patients who received IMA embolization, left internal mammary artery (LIMA) embolization was seen in 6 (9.7%) patients. Majority of patients underwent either right internal mammary artery (RIMA) embolization (n = 25; 40.3%) or RIMA and LIMA embolization (n = 27; 43.5%). IMA embolization in SV patients is common. Embolizing IMAs early in life will likely eliminate a valuable graft option for coronary artery bypass grafting should it be required in the future care of these patients. Multi-center, prospective, nation-wide studies are warranted to examine coronary artery disease in the SV population and true frequency of IMA embolization. Delineation of which IMAs were embolized is a necessary in surgical and cardiac intervention national data, such as Society of Thoracic Surgeons (STS) database. All measures should be taken to preserve IMAs patency, if deemed feasible and safe.

Variable cardiovascular phenotypes associated with SMAD2 pathogenic variants.

SMAD2 is a downstream effector in the TGF-ß signaling pathway, which is important for pattern formation and tissue differentiation. Pathogenic variants in SMAD2 have been reported in association with arterial aneurysms and dissections and in large cohorts of subjects with complex congenital heart disease (CHD). We used whole exome sequencing (WES) to investigate the molecular cause of CHD and other congenital anomalies in three probands and of an arterial aneurysm in an additional patient. Patients 1 and 2 presented with complex CHD, developmental delay, seizures, dysmorphic features, short stature, and poor weight gain. Patient 3 was a fetus with complex CHD and heterotaxy. The fourth patient is an adult female with aortic root aneurysm and physical features suggestive of a connective tissue disorder. WES identified pathogenic truncating variants, a splice variant, and a predicted deleterious missense variant in SMAD2. We compare the phenotypes and genotypes in our patients with previously reported cases. Our data suggest two distinct phenotypes associated with pathogenic variants in SMAD2: complex CHD with or without laterality defects and other congenital anomalies, and a late-onset vascular phenotype characterized by arterial aneurysms with connective tissue abnormalities.

Routine Cardiac Catheterization Prior to Fontan Operation: Is It a Necessity?

Prior to the Fontan procedure, patients with single ventricle physiology with Glenn shunt are typically referred for cardiac catheterization to assess hemodynamics and potentially provide interventional measures. Currently, echocardiography provides detailed information which together with other non-invasive imaging such as CT scan and MRI may obviate the need for routine cardiac catheterization prior to the Fontan procedure. In this study, we examine the findings in cardiac catheterization in this population to determine: (a) the accuracy of echocardiography in providing adequate information prior to the Fontan procedure, particularly in identifying those in need of per-catheter intervention, and (b) the percentage of patients requiring interventional procedures during cardiac catheterization. We performed a retrospective chart review of echocardiographic and cardiac catheterization data for patients who underwent pre-Fontan cardiac catheterization at our center in the period from 02/01/2008 to 02/28/2017. We aimed to re-examine the necessity of routine cardiac catheterization in all single ventricle patients. This was performed through examining pre-catheterization echocardiography reports and comparing them to findings of the subsequent cardiac catheterization reports. Echocardiography reports were evaluated for accuracy in identifying significant anatomical or hemodynamic findings, which may impact success of Fontan procedure as well as the ability of echocardiography to predict findings important to know prior to the Fontan procedure. In this cohort of 40 children, 3 patients were found to have significant hemodynamic findings through cardiac catheterization which were not previously known by echocardiography. In addition, 28 out of 40 patients (70%) required interventional procedures to address significant abnormalities (systemic to pulmonary arterial collaterals, pulmonary artery stenosis, aortic arch stenosis, etc.). All cases of aortic arch stenosis were detected by echocardiography, however, all patients who required systemic to pulmonary arterial or left SVC embolization were not detected by echocardiography. Furthermore, echocardiography did not detect the need for branch pulmonary artery stenosis in 50% of cases. Cardiac catheterization appears to be an essential part of patient assessment prior to Fontan completion in patients with single ventricle physiology. This current practice may change in the future if a non-invasive screening tool is found to have high positive and negative predictive values in identifying the subset of patients who require potential intervention in pre-Fontan cardiac catheterization.

Acute and midterm results following perventricular device closure of muscular ventricular septal defects: A multicenter PICES investigation.

OBJECTIVES: To describe acute and mid-term results of hybrid perventricular device closure of muscular ventricular septal defects (mVSDs). BACKGROUND: Perventricular device closure of mVSDs can mitigate technical limitations of percutaneous closure and need for cardiopulmonary bypass or ventriculotomy with a surgical approach. METHODS: This is a multicenter retrospective cohort study of patients undergoing hybrid perventricular mVSD device closure from 1/2004 to 1/2014. Procedural details, adverse events, outcomes, and follow-up data were collected. Patients were divided into two groups: (1) simple (mVSD closure alone) and (2) complex (mVSD closure with concomitant cardiac surgery). RESULTS: Forty-seven patients (60% female) underwent perventricular mVSD device closure at a median age of 5.2 months (IQR 1.8-8.9) and weight of 5.1 kg (IQR 4.0-6.9). Procedural success was 91% [100% (n = 22) simple and 84% (n = 21/25) complex]. Adverse events occurred in 19% (9/47) [9% (2/22) simple and 28% (7/25) complex]. Hospital length of stay (LOS) was shorter in the simple vs. complex group (4 vs. 14 days, P < 0.01). At mid-term follow-up of 19.2 months (IQR 2.3-43) 90% of pts had complete mVSD closure; none developed late heart block, increased atrioventricular (AV) valve insufficiency or ventricular dysfunction. CONCLUSIONS: Perventricular device closure of simple mVSD was associated with a high rate of procedural success, few adverse events, and short hospital LOS. Procedural adverse events were associated with the presence of concomitant complex surgery. Residual mVSD, AV valve insufficiency, or ventricular dysfunction were uncommon at mid-term follow-up. © 2017 Wiley Periodicals, Inc.

Radiation Exposure by Three-Dimensional Rotational Angiography (3DRA) During Trans-catheter Melody Pulmonary Valve Procedures (TMPV) in a Pediatric Cardiac Catheterization Laboratory.

This retrospective study aims to evaluate radiation exposure by three-dimensional rotational angiography (3DRA) during trans-catheter Melody pulmonary valve (TMPV) procedures. 3DRA has been reported to have added value in the management of complex congenital heart disease aiding in the performance of interventional procedures albeit with concerns of higher radiation exposure. We test the hypothesis that 3DRA does not cause additional radiation exposure during TMPV procedures. We analyzed all 81 TMPV procedures performed at St. Louis Children's Hospital, MO, USA, from January 1, 2011 to December 31, 2014. Dose-area product (DAP), DAP indexed to body weight (DAP/BW), fluoroscopy time (FT), and weight-fluoroscopy time product of each procedure were recorded. We reviewed each procedure's images to determine whether additional interventions were performed (e.g., pulmonary artery angioplasty or treatment of conduit pseudo-aneurysm). 3DRA was used in 36 % of the procedures. 3DRA group had a higher number of additional procedures performed. The 3DRA group did not differ from the non-3DRA group in DAP, DAP/BW, and weight-fluoroscopy time product. 3DRA does not cause greater radiation exposure during TMPV procedures.

Improved survival after heart transplant for failed Fontan patients with preserved ventricular function.

BACKGROUND: Patients with a failing Fontan continue to have decreased survival after heart transplant (HT), particularly those with preserved ventricular function (PVF) compared with impaired ventricular function (IVF). In this study we evaluated the effect of institutional changes on post-HT outcomes. METHODS: Data were retrospectively collected for all Fontan patients who underwent HT. Mode of failure was defined by the last echocardiogram before HT, with mild or no dysfunction considered PVF and moderate or severe considered IVF. Outcomes were compared between early era (EE, 1995 to 2008) and current era (CE, 2009 to 2014). Management changes in the CE included volume load reduction with aortopulmonary collateral (APC) embolization, advanced cardiothoracic imaging, higher goal donor/recipient weight ratio and aggressive monitoring for post-HT vasoplegia. RESULTS: A total of 47 patients were included: 27 in the EE (13 PVF, 14 IVF) and 20 in the CE (12 PVF, 8 IVF). Groups were similar pre-HT, except for more PLE in PVF patients. More patients underwent APC embolization in the CE (80% vs 28%, p < 0.01). There was no difference in donor/recipient weight ratio between eras. There was a trend toward higher primary graft failure for PVF in the EE (77% vs 36%, p = 0.05) but not the CE (42% vs 75%, p = 0.20). Overall, 1-year survival improved in the CE (90%) from the EE (63%) (p = 0.05), mainly due to increased survival for PVF (82 vs 38%, p = 0.04). CONCLUSIONS: Post-HT survival for failing Fontan patients has improved, particularly for PVF. In the CE, our Fontan patients had a 1-year post-HT survival similar to other indications.

Doppler echocardiography inaccurately estimates right ventricular pressure in children with elevated right heart pressure.

BACKGROUND: Doppler echocardiography (DE) is widely used as a surrogate for right heart catheterization (RHC), the gold standard, to assess and monitor elevated right heart pressure in children. However, its accuracy has not been prospectively validated in children. The objectives of this study were to evaluate the accuracy of DE in predicting simultaneously measured right ventricular (RV) pressure by RHC in pediatric patients and to determine if the degree of RV hypertension affects the accuracy of DE in assessing right heart pressure. METHODS: Eighty children (age range, 0-17.9 years; median age, 5.5 years) with two-ventricle physiology and a wide range of right heart pressures underwent simultaneous DE and RHC. The pressure gradient between the right ventricle and the right atrium was directly measured by RHC and simultaneously estimated by DE using tricuspid regurgitation. Patients were then grouped on the basis of RHC-measured RV systolic pressure (RVSP): group 1 (n = 43), with RVSP < 1/2 systemic systolic blood pressure (SBP); group 2 (n = 37), with RVSP ≥ 1/2 SBP; group 3 (n = 56), with RVSP < 2/3 SBP; and group 4 (n = 24), with RVSP ≥ 2/3 SBP. Correlation and Bland-Altman analyses were performed on all groups. Accuracy was predefined as 95% limits of agreement within ±10 mm Hg. RESULTS: Despite a reasonable correlation between DE and RHC in all groups, there was poor agreement between techniques as RVSP/SBP increased. DE was inaccurate in one of 43 patients in group 1 (2%) versus nine of 37 in group 2 (24%) and was inaccurate in one of 56 patients in group 3 (2%) versus eight of 24 in group 4 (33%). Overestimation and underestimation occurred equally in all groups. CONCLUSION: DE inaccurately estimates RV pressure in children with elevated right heart pressure. It should not be relied on as the sole method of assessing right heart hemodynamics in children with RV hypertension.

Safety of percutaneous patent ductus arteriosus closure: an unselected multicenter population experience.

BACKGROUND: The technique and safety of transcatheter patent ductus arteriosus (PDA) closure have evolved during the past 20 years. We sought to report a multicenter experience of PDA closure with a focus on the rate of adverse events (AE) and a review of institutional practice differences. METHODS AND RESULTS: Outcome data on transcatheter PDA closure were collected at 8 centers prospectively using a multicenter registry (Congenital Cardiac Catheterization Project on Outcome Registry). Between February 2007 and June 2010, 496 PDA closures were recorded using a device in 338 (68%) or coils in 158 (32%). Most patients had an isolated PDA (90%). Fifty percent of patients were between 6 months and 3 years old, with only 40 patients (8%) <6 months old. Median minimum PDA diameter was 2.5 mm (range 1 to 12 mm; IQR 2 to 3 mm) for device closure and 1 mm (range 0.5 to 6 mm; IQR 1 to 2 mm) for coil closure (P<0.001). A device rather than coil was used in patients <3 years, weight <11 kg, and with a PDA minimum diameter >2 mm (all P<0.001). Three of 8 centers exclusively used a device for PDAs with a diameter >1.5 mm. In 9% of cases (n=46), an AE occurred; however, only 11 (2%) were classified as high severity. Younger age was associated with a higher AE rate. Coil-related AEs were more common than device-related AEs (10% versus 2%, P<0.001). CONCLUSIONS: PDA closure in the present era has a very low rate of complications, although these are higher in younger children. Technical intervention-related events were more common in coil procedures compared with device procedures. For PDAs ≤2.5 mm in diameter, institutional differences in preference for device versus coil exist.