Bilateral Harmony™ valve placement in branch pulmonary arteries.

While newer self-expanding pulmonic valves were primarily designed for larger right ventricular outflow tracks, there are instances where even larger anatomies cannot accommodate these devices. In this report, we describe the successful implantation of two Harmony™ valves in bilateral branch pulmonary arteries after exhausting other options.

Utility of simultaneous triple balloon inflation technique through a single 6-French sheath in treating pulmonary vein stenosis.

Pulmonary vein stenosis continues to pose significant challenges in children, frequently requiring repeated cardiac catheterisation procedures. This report describes a successful application of a "triple kissing balloon" technique to treat complex pulmonary vein stenosis in two patients, all accomplished with the use of a single 6-French sheath.

Bedside repositioning of a migrated avalon ECMO cannula in an infant: Novel technique.

Background: Although the Avalon Elite bi-caval dual lumen catheter for veno-venous extracorporeal membranous oxygenation (ECMO) has many advantages, it requires precise positioning and dislodgement is common.Case presentation: A 2-year-old male was placed on ECMO due to respiratory failure utilizing a 20 Fr Avalon Elite bi-caval dual lumen catheter (AEC). The AEC migrated twice with unsuccessful repositioning using the classic manual manipulations. The AEC was successfully repositioned on the two occasions using a novel method by direct access of the ECMO inflow tube using a combination of catheter and guide wire.Conclusions: A migrated AEC could be successfully repositioned with simple direct access of the inflow tube. This technique was successfully utilized twice at the bedside in an infant without needing additional venous access.

Left atrial appendage occlusion using hydrogel coils in a child.

Left atrial appendage occlusion in young children has not been reported before. Herein, we describe a successful occlusion using hydrogel coils in a toddler. The decision to occlude the appendage was made to mitigate the potential risk of systemic thromboembolism, given the child's unusual anatomy.

Hybrid approach for harmony transcatheter pulmonary valve replacement.

The Harmony™ Transcatheter Pulmonary Valve (Medtronic) was recently approved by the Food and Drug Administration for transcatheter pulmonary valve replacement in native right ventricular outflow tracts. Despite this milestone, some patients have main pulmonary arteries that are severely dilated and continue to require surgical pulmonary valve replacement. The hybrid approach combines surgical creation of a landing zone, transcatheter valve deployment, and suture stabilization of the implanted valve. In this case series, we report the first use of a hybrid approach for Harmony™ transcatheter pulmonary valve replacement. Two cases are reported with varying approaches for surgical creation of a landing zone followed by successful placement of a Harmony™ valve.

Left ventricular function before and after percutaneous patent ductus arteriosus closure in preterm infants.

BACKGROUND: Definitive closure of the patent ductus arteriosus (PDA) is associated with significant changes in the loading conditions of the left ventricle (LV), which may lead to cardiovascular and respiratory instability. The objective of the study was to evaluate targeted neonatal echocardiography (TnECHO) characteristics and the clinical course of preterm infants ≤2 kg undergoing percutaneous PDA closure. METHODS: Retrospective cohort study of prospectively acquired pre- and post-closure TnECHOs to assess hemodynamic changes. Cardiorespiratory parameters in the first 24 h following PDA closure were also evaluated. RESULTS: Fifty patients were included with a mean age of 30.6 ± 9.6 days and weight of 1188 ± 280 g. LV global longitudinal strain decreased from -20.6 ± 2.6 to -14.9 ± 2.9% (p < 0.001) after 1 h. There was a decrease in LV volume loading, left ventricular output, LV systolic and diastolic parameters. Cardiorespiratory instability occurred in 24 (48%) [oxygenation failure in 44%] but systolic hypotension and/or need for cardiovascular medications was only seen in 6 (12%). Patients with instability had worse baseline respiratory severity score and lower post-closure early diastolic strain rates. CONCLUSIONS: Percutaneous PDA closure leads to a reduction in echocardiography markers of LV systolic/diastolic function. Post-closure cardiorespiratory instability is characterized primarily by oxygenation failure and may relate to impaired diastolic performance. IMPACT: Percutaneous patent ductus arteriosus closure leads to a reduction in echocardiography markers of left ventricular volume loading, cardiac output, and left ventricular systolic/diastolic function. Post-procedural cardiorespiratory instability is characterized primarily by oxygenation failure. Post-procedural cardiorespiratory instability may relate to impaired diastolic performance.

Medium-Term Outcomes in Pediatric Patients Undergoing Cardiac Catheterization Early After Congenital Cardiac Surgery.

Safety and acute outcomes for patients who need catheterization shortly after congenital cardiac surgery has been established; literature on mid-term outcomes is lacking. We sought to evaluate the mid-term outcomes of patients who undergo early postoperative cardiac catheterization. This is a retrospective cohort study of patients who underwent cardiac catheterization within 6 weeks of congenital cardiac surgery with longitudinal follow-up and assessment of mid-term outcomes. Multivariable analysis was utilized to relate variables of interest to outcomes. 99 patients underwent cardiac catheterizations within 6 weeks of cardiac surgery between January 2008 and September 2016. Forty-six (45.5%) interventional procedures were performed at a median age of 41 days (IQR 21-192) and a median weight of 3.9 kg (3.3-6.6). During a median follow-up duration of 4.24 years (1.6-5.6) in study survivors, 61% of patients remained free from the primary endpoint (death and/or transplant). Sixty-nine patients (69.7%) underwent an unplanned surgical or catheter procedure. Renal failure at catheterization (OR 280.5, p 0.0199), inotropic medication at catheterization (OR 14.8, p 0.002), and younger age were all significantly associated with meeting the primary endpoint. Patients requiring surgical intervention as an initial additional intervention underwent more unplanned re-interventions, while patients who survived to hospital discharge demonstrated favorable mortality, though with frequent need for re-intervention. In patients requiring early postoperative cardiac catheterization, renal failure, younger age, and need for inotropic support at catheterization are significantly associated with meeting the primary endpoint.

A novel approach to place an adult-size stent to treat coarctation of the aorta using small introducers: "Nijres technique".

Aortic arch stent placement in young children is a medical dilemma. This is attributed to the critical lack of commercially available stents that can be delivered through small sheaths and yet have the potential to be dilated to the adult-size aorta. Here in, we describe an innovative first-in-human technique that allows for overcoming the aforementioned challenges. A Palmaz Genesis XD stent was placed to treat coarctation of the aorta in two young children through small-bore sheaths.

Partially unroofed coronary sinus in an infant with pulmonary vein stenosis: mixed blessing.

Transcatheter intervention on the pulmonary vein often requires performing transseptal puncture, which is a risky procedure. We describe a rare association of a partially unroofed coronary sinus with pulmonary vein stenosis causing desaturation in a young infant where the presence of the unroofed coronary sinus allowed for a novel interventional approach to the stenotic left pulmonary vein, avoiding the high risk of transseptal puncture.

Gelatin sponge to close a tract after large-bore transhepatic access: tips for success.

Percutaneous transhepatic venous access has been utilised for numerous transcatheter cardiac procedures. Traditionally, a large transhepatic tract requires the placement of permanent occlusion devices or coils. We describe a successful closure using a simple technique (Surgifoam) without the need for metal hardware placement. Immediate hemostasis was achieved. No complications were encountered.

Transcatheter Fontan completion using novel balloon and stent system.

Despite advances in percutaneous interventions, transcatheter Fontan completion remains experimental and performed only in select cases. Non-surgical Fontan completion requires surgical preconditioning at an earlier stage of palliation. We describe transcatheter Fontan completion in a 15-year-old male with previously failed surgical Fontan palliation without surgical preconditioning.

The road less travelled: percutaneous closure of a secundum atrial septal defect through the left internal jugular vein.

In cases where femoral access is untenable for secundum atrial septal defect closure, the internal jugular vein can be used instead. We report a case of atrial septal defect closure in a patient with significant vascular thrombosis, requiring the use of the left internal jugular vein for access. To the best of our knowledge, this is the first report that documents the use of the left internal jugular vein for atrial septal defect closure in a patient with situs solitus anatomy.

Hybrid Approach to Right Ventricle Decompression in Muscular Pulmonary Atresia with Intact Ventricular Septum.

Muscular pulmonary atresia with intact ventricular septum (PA/IVS) in neonates is traditionally managed by surgery. We describe hybrid approach to decompress the right ventricle (RV) and establish RV to pulmonary artery connection in a neonate avoiding cardiopulmonary bypass. A 21-gauge access needle was used to perforate the atretic pulmonary valve via periventricular approach followed by stent placement. This case is an example of how patients with congenital heart disease can be palliated in creative ways through thoughtful collaboration between surgical and interventional cardiology teams.

Utility of Pulmonary Artery Acceleration Time to Estimate Systolic Pulmonary Artery Pressure in Neonates and Young Infants.

Transthoracic echocardiogram (TTE) is commonly used to screen for pulmonary hypertension (PHTN) in neonates and young infants. However, in the absence of sufficient tricuspid regurgitation (TR), a ventricular septal defect (VSD), or a patent ductus arteriosus (PDA), the estimation of systolic pulmonary artery pressure (SPAP) becomes challenging. Pulmonary artery acceleration time (PAAT) is an alternate parameter that is easy to obtain in almost all patients and does not require the presence of tricuspid valvar regurgitation or an anatomical cardiac defect. We sought to examine the correlation of PAAT with estimated SPAP by TTE and create an equation to estimate the SPAP using PAAT. We performed a retrospective review of TTEs performed on neonates and young infants (4 months of age or younger) at our institution between April 2017 and December 2018, along with the corresponding medical records. We included TTEs that provided estimation for SPAP and at least one PAAT measurement. During the study period, 138 TTEs performed on 82 patients met the inclusion criteria. Strong correlation was delineated between PAAT and SPAP estimated by the maximum velocity of tricuspid valve regurgitation Doppler, correlation coefficient (r) = - 0.83. Moderate correlation was detected between PAAT and SPAP estimated by PDA Doppler, r = - 0.66. Utilizing the following equation "SPAP = 82.6 - 0.58 × PAAT + RA mean pressure", PAAT can be used to estimate SPAP in neonates and young infants. PAAT can be used as an alternative to TR jet to assess SPAP when the latter is absent or insufficient. Further studies are needed to verify the accuracy of this equation.

Aortopulmonary Collaterals in Single Ventricle Physiology: Variation in Understanding Occlusion Practice Among Interventional Cardiologists.

Although aortopulmonary collaterals (APCs) frequently develop in patients with single ventricle palliation, there is a lack of understanding of pathophysiology, natural history, and outcomes with no universal guidelines for management and interventional practice. We conducted a study to assess the views held by interventional congenital cardiologists regarding the hemodynamic impact of APCs in patients with single ventricle palliation, and their embolization practice. An electronic survey using the Pediatric Interventional Cardiology Symposium (PICS) mailing list was conducted between February and March 2019 with one reminder sent 2 weeks after initial invitation for participation. Of the 142 interventional cardiologist respondents, 95 (66.9%) reside in North America and 47 (33.1%) worldwide. We elected to exclude the data from interventionalists outside North America in this analysis as it was not representative of worldwide practice. Hypoxemia was considered to be the most common trigger for development of APCs by 56 (58.9%) respondents. After completion of total cavopulmonary connection, 30 (31.6%) respondents reported the APC burden stays the same while 31 (32.6%) feel it decreases. In evaluating the burden of APC flow, only 4 (4.2%) reported measuring oxygen saturation at different pulmonary artery segments, 21 (22.1%) perform segmental aortic angiograms, and 18 (19%) perform selective bilateral subclavian artery angiograms. A majority of respondents, 71 (74.7%), occlude the feeder vessel at different locations, while 10 (10.5%) occlude only the origin of the vessel. Our study demonstrates significant variation in the understanding of the cause and prognosis of APCs in patients with single ventricle palliation. Furthermore, there is variation in the approach for diagnosis and management among interventional cardiologists. Further studies are required to improve understanding of APCs and develop universal management guidelines.

Catheter-induced acute Blalock-Taussig shunt thrombosis and bailout.

Modified Blalock-Taussig shunt thrombosis is a life-threatening event. We describe an extremely rare catheter-induced shunt thrombosis in an infant with complex CHD and its successful treatment utilising a single low dose of local recombinant tissue plasminogen activator in conjunction with balloon angioplasty.

Intracoronary recombinant tissue plasminogen activator in an infant with hypoplastic left heart syndrome and complete left main coronary artery thrombosis.

An infant with hypoplastic left heart syndrome (HLHS) presented with complete heart block and severe myocardial dysfunction requiring ECMO support due to complete left main coronary artery (LMCA) thrombosis. Current guidelines for managing coronary artery thrombosis in infants with single ventricle physiology are inadequate. We describe successful LMCA and branch recanalization via intra coronary infusion of recombinant tissue plasminogen activator and discuss management of acute coronary thrombosis in children with single ventricle physiology.

Three-dimensional printing for surgical planning in complex congenital heart disease.

Surgical management of complex congenital heart disease (CHD) is challenging. Three-dimensional (3D) printing can improve multidisciplinary team decision-making, patient and family understanding, and education of medical professionals. We describe 3D printing for surgical management of five patients with complex CHD. The anatomical details of the 3D printed models were instrumental in planning surgical techniques especially in determining between single ventricle, 1.5 ventricle, and biventricular repair.

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.

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.