Chiari Network Associated with Hypoxemia in a Neonate: Case Report and Review of the Literature.

Chiari network is an embryonic remnant of the right sinus venosus. It appears as a thin, fenestrated membrane attached to two or more regions within the right atrium. Usually, a Chiari network has a benign course; however, rare complications associated with a Chiari network have been reported. We present the case of a neonate with hypoxemia and a Chiari network protruding into the right ventricular inflow tract associated with right-to-left shunting across the patent foramen ovale throughout the cardiac cycle. We noted spontaneous improvement after 3 weeks of life.

A Rare Report of Hypoplastic Coronary Arteries and Pulmonary Veins: A Case Report and Review of the Literature.

Congenital coronary artery anomalies are estimated to affect about 1% of the general population. Hypoplastic coronary artery disease is an uncommon subset associated with significant mortality regardless whether another major cardiac anomaly is present. In this case report, we present an extremely rare case of an infant with hypoplastic coronary artery disease and hypoplastic pulmonary veins. A literature review pertinent to the clinical findings is also contained herein.

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.

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.

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.

Can the Pulmonary Artery Wedge Pressure be Used Reliably as a Surrogate for the Left Atrial Mean Pressure in Pre-Fontan Evaluation?

The correlation between mean pulmonary artery wedge pressure (PAWP) and left atrial mean pressure (LAMP) has been poorly studied in patients with single ventricle (SV) physiology (Bernstein et al. in Pediatr Cardiol 33: 15-20 2012). The aim of this study is to determine if the PAWP can be used safely as a surrogate to the LAMP to calculate the pulmonary vascular resistance (PVR) during the pre-Fontan evaluation. Also, we aimed to understand if the presence of significant systemic-to-pulmonary collaterals (SPCs) is a confounding factor for accurate estimation of the LAMP. From February 2007 to February 2017, forty-one patients were eligible for inclusion in the study. These patients were varied in terms of underlying cardiac malformation. Sex distribution was equal with 20 males and 21 females. Median weight was 11.8 kg, median body surface area was 0.51 m2, median age at catheterization was 2 years, and the median age at Glenn surgery was 5 months. We found the left and right PAWP and LAMP correlated strongly and the differences in the measurements were negligible. Similar findings were observed when calculating PVRs using PAWP and LAMP. These findings were more pronounced in the absence of significant SPCs. PAWP and LAMP correlation was still valid in the presence of significant SPCs; however, the correlation among the calculated PVRs was more attenuated.

Chronic Low Dose Prostaglandin and Neonatal Heart Block.

Long-term prostaglandin use is commonly associated with side effects such as cortical proliferation of the bones, hypertrophic pyloric stenosis, and soft tissue swelling of the extremities. We report a neonate with critical coarctation of the aorta, who developed second and third degree atrioventricular blocks associated with prolonged prostaglandin E1 (PGE1) infusion. Interestingly, these conduction blocks only occurred at low PGE1 dose. The rhythm disturbances resolved promptly with the discontinuation of PGE1 following surgical repair.

Coronary Sinus Defect Following Transcatheter Closure of ASD Using Amplatzer Septal Occluder: Potential Erosion by the Device.

We present a case of small coronary sinus defect detected after transcatheter device closure of a large secundum atrial septal defect. Although device erosion of the dilated coronary sinus is suspected, the defect in the coronary sinus may have been present prior to ASD device closure. Dilated coronary sinus may be a risk factor when closing a secundum ASD with a device. To the best of our knowledge, coronary sinus erosion by an ASD device has not yet been reported in the medical literature.

Extended Application of the Hybrid Procedure in Neonates with Left-Sided Obstructive Lesions in an Evolving Cardiac Program.

The hybrid approach to management of hypoplastic left heart syndrome (HLHS) was developed as an alternative to neonatal Norwood surgery, providing a less invasive initial palliation for HLHS. We describe our experience in extending the concept of the hybrid procedure to palliate neonates with anatomically compromised systemic arterial blood flow in a variety of congenital cardiac anomalies and supporting its application as first-line palliation in centers developing their HLHS programs. Retrospective review of patients undergoing therapy for HLHS at a single institution from June 2008 to December 2014 was performed. Subject demographics, clinical and procedural data, along with follow-up, were collected. Thirteen patients had initial hybrid palliation for HLHS during the time frame indicated at a median age of 8 days (range 1-29 days) and median weight of 3.4 kg (range 2.4-4.6 kg). Diagnoses included typical HLHS (n = 6), right-dominant unbalanced atrioventricular septal defect with arch hypoplasia (n = 4), double outlet right ventricle [subpulmonic VSD (n = 1) and intact ventricular septum (n = 1)] with hypoplastic transverse aortic arch and borderline left ventricular dimensions. Standard approach with bilateral pulmonary artery banding and ductal stenting was carried out in all thirteen patients. Two patients required two ductal stents at the time of index procedure. There were no intraprocedural complications. Median intubation length post-procedure was 4 days (range 1-74 days). Median hospital stay post-procedure was 47 days (range 15-270 days). The overall mortality rate on follow-up through comprehensive stage 2 over the 6-year experience was 38 % (5 out of 13). Of note, the mortality rate was significantly lower in the latter 3 years of the study period when the procedure was adopted as a primary palliation for HLHS (14 % or 1 out of 7) compared to the initial 3-year period when it was reserved for higher risk cohorts (67 % or 4 out of 6). Median time to subsequent surgery was 3 months (range 1-4 months). One patient required further ductal stenting on follow-up and developed subsequently airway compression. On median follow-up of 24 months, two patients required pulmonary artery arterioplasty. The hybrid procedure may be used for palliation for a variety of cardiac lesions to avoid high-risk surgery in the neonatal period. This approach may be also an alternative in centers performing lower number of Norwood surgery, which has been associated with higher mortality.

Tetralogy of Fallot with left superior vena cava and coronary sinus atrial septal defect: a rare association.

This report describes a rare case of Tetralogy of Fallot with associated left superior vena cava and coronary sinus atrial septal defect. The initial diagnosis was made by echocardiography. The patient underwent complete repair at 2 months of age. Her postoperative course was complicated by low cardiac output requiring ECMO. She was subsequently weaned off of ECMO and discharged home. She continues to do well on serial follow-up.

The use of intracardiac echocardiography during percutaneous pulmonary valve replacement.

High-quality live imaging assessment of cardiac valves and cardiac anatomy is crucial for the success of catheter-based procedures. We present our experience using Intracardiac echocardiography (ICE) during transcatheter Percutaneous Pulmonary Valve replacement (tPVR).This is a retrospective study that included 35 patients who underwent tPVR between April 2008 and June 2012. Thirty-one of these patients had the procedure performed under continuous ICE guidance. Pre-procedure transthoracic echocardiography (TTE) was obtained in all patients. ICE was performed at baseline, during the procedure, and at the conclusion of the procedure. Comparisons between the pre-procedure TTE and baseline ICE data and between post-procedure ICE data and the following day TTE were performed. Total of 35 patients had tPVR during the above-mentioned time period. Twenty-one patients received the Edwards Sapien valve and 14 patients had the Melody valve. Thirty-one patients had the procedure performed under continuous ICE guidance. The mean Pre-TTE peak gradient (PG) and Pre-ICE-PG were 45.5 ± 20 vs 33 ± 13 mmHg (p < 0.001) and the mean Pre-TTE mean gradient (MG) and Pre-ICE-MG were 27.7 ± 13 vs 21 ± 18 mmHg (p < 0.001). The mean Post-TTE- PG and Post-ICE-PG were 24.3 ± 11 vs 15.3 ± 7 mmHg (p < 0.001) and the mean of the Post-TTE-MG and Post-ICE-MG were 14.2 ± 7 vs 8.4 ± 4 mmHg (p < 0.001). There was a good correlation between peak ICE and TTE gradient at baseline and after valve placement. For the degree of pulmonary regurgitation, there was no significant difference between TTE and ICE. ICE is an important modality to guide tPVR in patients with dysfunctional homograft valve between the right ventricle and pulmonary artery and should be used to assess valve function before, during and immediately after the procedure.

Atrial flutter demonstrated by M-mode echocardiography.

This report and images describe a case of atrial flutter with 2:1 conduction pattern in a 3 year-old male with d-TGA, ASD, and VSD status-post repair, which was demonstrated by M-mode echocardiography.

The pediatric cardiology pharmacopeia: 2013 update.

The use of medications plays a pivotal role in the management of children with heart diseases. Most children with increased pulmonary blood flow require chronic use of anticongestive heart failure medications until more definitive interventional or surgical procedures are performed. The use of such medications, particularly inotropic agents and diuretics, is even more amplified during the postoperative period. Currently, children are undergoing surgical intervention at an ever younger age with excellent results aided by advanced anesthetic and postoperative care. The most significant of these advanced measures includes invasive and noninvasive monitoring as well as a wide array of pharmacologic agents. This review update provides a medication guide for medical practitioners involved in care of children with heart diseases.

Benign outcome of pulmonary hypertension in neonates with a restrictive patent foramen ovale versus result for neonates with an unrestrictive patent foramen ovale.

Premature closure or restriction of foramen ovale (FO) is a rare but known entity. FO diameter <2 mm and Doppler velocity >120 cm/s, diameter <3 mm with Doppler velocity measured gradient >5 mmHg have all being used by various authors to describe this entity. Some neonates with restrictive FO have been noted to have severe pulmonary hypertension with no clinical signs or symptoms and with spontaneous resolution without any intervention. Seven consecutive neonates were indentified in the database between 01/01/2003 and 06/30/2010 with diagnosis of restrictive PFO (diameter <2 mm) with structurally normal heart and their initial and follow-up echocardiogram as well as hospital medical records were reviewed. As a control, seven neonates with diagnosis of pulmonary hypertension and respiratory distress syndrome or meconium aspiration syndrome were randomly selected. Eighty-six percent of the patients in the control group were symptomatic and required treatment as compared to 14% in the restrictive FO group (p = 0.03). Further, the fall in the peak instantaneous pulmonary artery pressure on follow-up echocardiogram was greater in the restrictive FO group compared with the non-restrictive group (p = 0.03). Patients with pulmonary hypertension and a restrictive FO with no other associated congenital heart disease and/or lung pathology behave differently when compared to neonates with non-restrictive FO and pulmonary hypertension with associated lung disease. They seldom manifest symptoms requiring intervention and tend to show a faster drop in their pulmonary artery pressure toward the normal.