Outcomes of Neonates Born with Symptomatic Tetralogy of Fallot and Absent Ductus Arteriosus.

OBJECTIVE: To test the hypothesis that neonates with symptomatic tetralogy of Fallot (TOF) and absent ductus arteriosus (ADA) have worse clinical outcomes compared with those with a ductus arteriosus (DA), and that this difference is driven by those born with ADA and with critically deficient pulmonary blood flow (CDPBF). STUDY DESIGN: We performed a retrospective, multicenter cohort study of neonates who underwent intervention for symptomatic TOF comparing death and reintervention between subjects with and without a DA identified on fetal echocardiogram or on echocardiogram performed in the first postnatal day. Exclusion criteria were as follows: inability to define DA status, collaterals supplying pulmonary blood flow, atrioventricular septal defect, and absent pulmonary valve. We defined CDPBF as undergoing a procedure to augment pulmonary blood flow on the date of birth or extracorporeal membrane oxygenation prior to such a procedure. RESULTS: The study cohort included 519 patients, among whom 11% had ADA. Patients with ADA were more likely to have a genetic syndrome and had smaller branch pulmonary artery size. In analyses adjusting for center, interventional treatment strategy, genetic syndrome, and minimum branch pulmonary artery size, ADA was associated with higher mortality risk (adjusted hazard ratio of 2.37 (95% CI: 1.07,5.27; P = .034). Seven patients had CDPBF (1.3% of the entire cohort and 12% of patients with ADA). CONCLUSIONS: A minority of symptomatic TOF neonates have ADA, which is associated with higher adjusted mortality risk compared with those with a DA. CDPBF appears to be a rare but important entity in this population.

Atrial septal interventions during and after hybrid stage I palliation of hypoplastic left heart syndrome.

BACKGROUND: Hybrid stage I palliation (HS1P) is an alternative approach for initial palliation in hypoplastic left heart syndrome (HLHS) patients. Unlike surgical stage I palliation where atrial septectomy is routinely performed, atrial septal intervention (ASI) during HS1P is variable. In this study, we described our experience with ASI in single ventricle (SV) patients who underwent HS1P and identified factors associated with need for ASI after HS1P. METHODS: Data were retrospectively collected for all HLHS patients who underwent HS1P at our center over the past 12 years. We evaluated ASIs performed during the HS1P (intra-HS1P ASI) and ASIs performed during the period from HS1P to the subsequent surgical stage, either interval Norwood stage I or comprehensive stage II (post-HS1P ASI). Patient factors and procedural data were compared to identify factors associated with undergoing post-HS1P ASI and the impact of ASI on patient outcomes was evaluated. RESULTS: Of 50 SV patients included, 23 (46%) underwent intra-HS1P ASI and 26 (52%) underwent post-HS1P ASI. Need for post-HS1P ASI was lower among patients who had an intra-HS1P ASI as compared to those who did not (30% vs. 70%; p = 0.005). There were no significant differences in short or Midterm outcomes between patients who underwent intra-HS1P ASI or post-HS1P ASI and their counterparts. CONCLUSIONS: ASI is common both during and after HS1P but is generally well tolerated and type of ASI does not significantly impact overall patient outcomes. Our findings suggest that the current approach of individualizing management of ASI in the HS1P population is effective and safe.

Sinus venosus atrial septal defect presenting with platypnea-orthodeoxia syndrome in the setting of COVID-19 infection.

Platypnea-orthodeoxia syndrome is characterised by hypoxemia and dyspnoea while upright that resolves when supine, typically associated with an interatrial communication. We present a case of platypnea-orthodeoxia syndrome associated with a sinus venosus atrial septal defect in a patient with multiple possible aetiologies of hypoxemia, including COVID-19 infection. Cardiac catheterisation with provocative maneuvers confirmed the diagnosis and symptoms resolved following defect closure. We suggest that in patients with platypnea-orthodeoxia syndrome, it is useful to obtain haemodynamic data while supine and upright.

Timing of Balloon Atrial Septostomy in Patients with d-TGA and Association with Birth Location and Patient Outcomes.

Patients with d-looped transposition of the great arteries (d-TGA), especially those without an adequate atrial septal defect, can experience severe hypoxemia and hemodynamic compromise in the neonatal period. This can be mitigated by urgent balloon atrial septostomy (BAS). However, some patients with d-TGA are born at centers without this capability. The aim of this retrospective study of d-TGA patients who had urgent or emergent BAS at our institution between 2010 and 2021 was to evaluate time from birth to BAS for infants born at a tertiary care center as compared to those requiring transport from other institutions and to examine correlation between time to BAS and patient outcomes. Our primary outcome was time from birth to BAS. Secondary outcomes included hospital and ICU length of stay, mortality, and evidence of pulmonary or neurologic abnormalities including pulmonary hypertension, abnormal neuroimaging, or seizures. Of 96 patients, 67 (70%) were born at our institution. The median time to BAS was 4 h for patients born at our institution vs. 14.1 h for those born elsewhere (p < .0001). A longer time from birth to BAS was associated with longer ICU (r = 0.21, p = 0.046) and hospital length of stay (r = 0.24, p = 0.02) and increased likelihood of elevated right ventricular pressure on post-operative discharge echocardiogram (p = 0.01). There were no differences in mortality between the groups. Therefore, prenatal planning for patients with known d-TGA should include a delivery plan with access to urgent BAS.

Augmented Reality Visualization of 3D Rotational Angiography in Congenital Heart Disease: A Comparative Study to Standard Computer Visualization.

Augmented reality (AR) visualization of 3D rotational angiography (3DRA) provides 3D representations of cardiac structures with full visualization of the procedural environment. The purpose of this study was to evaluate the feasibility of converting 3DRAs of congenital heart disease patients to AR models, highlight the workflow for 3DRA optimization for AR visualization, and assess physicians' perceptions of their use. This single-center study prospectively evaluated 30 retrospectively-acquired 3DRAs that were converted to AR, compared to Computer Models (CM). Median patient age 6.5 years (0.24-38.8) and weight 20.6 kg (3.4-107.0). AR and CM quality were graded highly. RV pacing was associated with higher quality of both model types (p = 0.02). Visualization and identification of structures were graded as "very easy" in 81.1% (n = 73) and 67.8% (n = 61) of AR and CM, respectively. Fifty-nine (66%) grades 'Agreed' or 'Strongly Agreed' that AR models provided superior appreciation of 3D relationships; AR was found to be least beneficial in visualization of aortic arch obstruction. AR models were thought to be helpful in identifying pathology and assisting in interventional planning in 85 assessments (94.4%). There was significant potential seen in the opportunity for patient/family counseling and trainee/staff education with AR models. It is feasible to convert 3D models of 3DRAs into AR models, which are of similar image quality as compared to CM. AR models provided additional benefits to visualization of 3D relationships in most anatomies. Future directions include integration of interventional simulation, peri-procedural counseling of patients and families, and education of trainees and staff with AR models.

The Need for Surgery After Vascular or Cardiac Trauma, or Technical Adverse Events in the Congenital Cardiac Catheterization Laboratory.

Data on the frequency and outcome of surgical interventions as a result of adverse events (AE) encountered in the pediatric and congenital cardiac catheterization laboratory are limited. This study analyzes the outcomes of specific types of AE that are most likely to require immediate surgical intervention. Data from the C3PO registry were analyzed to identify specific types of significant vascular/cardiac trauma or technical adverse events (stent/device/coil embolization/migration). The relationship between these AE and an "adverse outcome" (defined as either surgery, ECMO, or death) were analyzed. Between 01/2014 and 12/2017, 25,731 cases were entered into the C3PO registry. Vascular or cardiac trauma were observed in 92 cases (0.36% cases in C3PO), and technical adverse events were observed in 176 cases (0.68% cases in C3PO). The two highest procedure type risk categories (PREDIC3T) accounted for 61% of the cases in the cardiac/vascular trauma cohort, and 34% in the technical AE cohort. For vascular/cardiac trauma, 24 (26%) had an adverse outcome, with ECMO in 8 (9%), surgery in 19 (20%), and death in 9 (10%). For technical AE 25 (14%) had an adverse outcome, with ECMO in 3 (2%), surgery in 23 (13%), and death in 3 (2%). Survival after cardiac surgery secondary to an AE was 68% for cardiac/vascular trauma, and 96% for technical adverse events. RF perforation of the pulmonary valve was the procedure most likely to result in cardiac/vascular trauma (10%), with 57% of those having an adverse outcome. Atrial septal interventions accounted for 29% of all adverse outcomes in the cardiac/vascular trauma cohort. Non-elective or emergent cases were associated with a significantly higher incidence of an adverse outcome for both, cardiac/vascular trauma (OR 7.1) and technical adverse events (OR 2.7). Surgery within the last 30 days was associated with a significantly higher incidence of an adverse outcome for cardiac/vascular trauma only (OR 4.2). Significant cardiac/vascular trauma or stent/device/coil embolization/migration are rare, but high consequence AE. With appropriate surgical and ECMO backup, a high survival can be achieved. The potential need for and impact of immediate surgical backup seems to be higher for cardiac/vascular trauma (in particular after specific case types), than for device/coil migration/embolization, and as such case specific backup arrangements are required.

Feasibility of intraprocedural augmented reality visualisation of 3D rotational angiography in congenital cardiac catheterisation.

Three-dimensional rotational angiography has become a mainstay of congenital cardiac catheterisation. Augmented reality is an exciting and emerging technology that allows for interactive visualisation of 3D holographic images in the user's environment. This case series reports on the feasibility of intraprocedural augmented reality visualisation of 3D rotational angiography in five patients with CHD.

Neurocognitive function and health-related quality of life in adolescents and young adults with CHD with pulmonary valve dysfunction.

BACKGROUND: Neurocognitive impairment and quality of life are two important long-term challenges for patients with complex CHD. The impact of re-interventions during adolescence and young adulthood on neurocognition and quality of life is not well understood. METHODS: In this prospective longitudinal multi-institutional study, patients 13-30 years old with severe CHD referred for surgical or transcatheter pulmonary valve replacement were enrolled. Clinical characteristics were collected, and executive function and quality of life were assessed prior to the planned pulmonary re-intervention. These results were compared to normative data and were compared between treatment strategies. RESULTS: Among 68 patients enrolled from 2016 to 2020, a nearly equal proportion were referred for surgical and transcatheter pulmonary valve replacement (53% versus 47%). Tetralogy of Fallot was the most common diagnosis (59%) and pulmonary re-intervention indications included stenosis (25%), insufficiency (40%), and mixed disease (35%). There were no substantial differences between patients referred for surgical and transcatheter therapy. Executive functioning deficits were evident in 19-31% of patients and quality of life was universally lower compared to normative sample data. However, measures of executive function and quality of life did not differ between the surgical and transcatheter patients. CONCLUSION: In this patient group, impairments in neurocognitive function and quality of life are common and can be significant. Given similar baseline characteristics, comparing changes in neurocognitive outcomes and quality of life after surgical versus transcatheter pulmonary valve replacement will offer unique insights into how treatment approaches impact these important long-term patient outcomes.

Impact of Management Strategy on Feeding and Somatic Growth in Neonates with Symptomatic Tetralogy of Fallot: Results from the Congenital Cardiac Research Collaborative.

OBJECTIVE: To evaluate early growth following primary or staged repair of neonatal symptomatic tetralogy of Fallot (sTOF). STUDY DESIGN: We performed a retrospective, multicenter cohort study of consecutive infants with sTOF who underwent initial intervention at age ≤30 days, from 2005 to 2017. Management strategies were either primary repair or staged repair (ie, initial palliation followed by complete repair). The primary outcome was change in weight-for-age z-score (ΔWAZ) from the initial intervention to age 6 ± 2 months. Secondary outcomes included method and mode of feeding, feeding-related medications, and feeding-related readmissions. Propensity score adjustment was used to account for baseline differences between groups. A secondary analysis was performed comparing patients stratified by the presence of adequate growth (6-month ΔWAZ > -0.5) or inadequate growth (6-month ΔWAZ ≤ -0.5), independent of treatment strategy. RESULTS: The study cohort included 143 primary repair subjects and 240 staged repair subjects. Prematurity was more common in the staged repair group. After adjustment, median ΔWAZ did not differ between treatment groups over the first 6 months of life (primary: -0.43 [IQR, -1.17 to 0.50]; staged: -0.31 [IQR, -1.31 to 0.71]; P = .55). For the entire cohort, ΔWAZ was negative (-0.36; IQR, -1.21 to 0.63). There were no between-group differences in the secondary outcomes. Secondary analysis revealed that the subjects with adequate growth were more likely to be orally fed at initial hospital discharge (P = .04). CONCLUSIONS: In neonates with sTOF, growth trajectory over the first 6 months of life was substandard, irrespective of treatment strategy. Those patients with adequate growth were more likely to be discharged from the index procedure on oral feeds.

Native Aortic Root Thrombosis in Single-Ventricle Patients with Native-to-Neoaortic Anastomoses.

In single-ventricle patients with native-to-neoaortic anastomoses, the native aortic root serves as a conduit to the coronary arteries. Thrombosis of the native aortic root has been described only in small, limited reports. We described our center's experience with this rare adverse event. All single-ventricle patients who underwent native-to-neoaortic anastomosis from 2002 to 2017 were compiled from institutional databases. Chart review identified cases of native aortic root thrombosis. Of 467 patients, there were 9 (2%) cases of native aortic root thrombosis; all had hypoplastic left heart syndrome. Timing of thrombosis varied and occurred following each stage of single-ventricle palliation. For treatment, 8 patients received systemic anticoagulation and one patient did not receive any treatment. One patient also underwent percutaneous thrombectomy. Three patients (33.3%) died during the follow-up period. Among survivors, 4/6 had right ventricular systolic function that was mildly depressed or worse at a median follow-up of 8.3 years (interquartile range 1.3-10.8). Native aortic root thrombosis is a rare complication occurring at various stages of single-ventricle palliation. In this series, 6 of 9 patients (66.6%) survived; however, ventricular function was often compromised. No risk factors were identified to focus preventative therapies.

Palliation Strategy to Achieve Complete Repair in Symptomatic Neonates with Tetralogy of Fallot.

Neonates with symptomatic tetralogy of Fallot (sTOF) may undergo palliations with varying physiology, namely systemic to pulmonary artery connections (SPC) or right ventricular outflow tract interventions (RVOTI). A comparison of palliative strategies based on the physiology created is lacking. Consecutive sTOF neonates undergoing SPC or RVOTI from 2005-2017 were reviewed from the Congenital Cardiac Research Collaborative. The primary outcome was survival with successful complete repair (CR) by 18 months. A variety of secondary outcomes were assessed including overall survival, hospitalization-related comorbidities, and interstage reinterventions. Propensity score adjustment was utilized to compare treatment strategies. The cohort included 252 SPC (surgical shunt = 226, ductus arteriosus stent = 26) and 68 RVOTI (balloon pulmonary valvuloplasty = 48, RVOT stent = 11, RVOT patch = 9) patients. Genetic syndrome (29 [42.6%] v 75 [29.8%], p = 0.04), weight < 2.5 kg (28 [41.2%] v 68 [27.0%], p = 0.023), bilateral pulmonary artery Z-score < - 2 (19 [28.0%] v 36 [14.3%], p = 0.008), and pre-intervention antegrade flow (48 [70.6%] v 104 [41.3%], p < 0.001) were more common in RVOTI. Significant center differences were noted (p < 0.001). Adjusted survival to CR by 18 months (HR = 0.87, 95% CI = 0.63-1.21, p = 0.41) and overall survival (HR = 2.08, 95% CI = 0.93-4.65, p = 0.074) were similar. RVOTI had increased interstage reintervention (HR = 2.15, 95% CI = 1.36-3.99, p = 0.001). Total anesthesia (243 [213, 277] v 328 [308, 351] minutes, p < 0.001) and cardiopulmonary bypass times (117 [103, 132] v 151 [143, 160] minutes, p < 0.001) favored RVOTI. In this multicenter comparison of physiologic palliation strategies for sTOF, survival to successful CR and overall survival were similar; however, reintervention burden was significantly higher in RVOTI.

Novel use of a mechanical suction thrombectomy system for interposition graft occlusion in complex congenital heart disease.

Complete thrombosis of a pulmonary artery interposition graft in an adolescent with complex repaired CHD was treated successfully with a combination of a novel mechanical thrombectomy system, stent implantation, and thrombolysis. This thrombectomy system used a flexible catheter with a built-in mechanism to attenuate blood loss, while providing effective recanalisation of a foreign graft.

Comparison of the investigational device exemption and post-approval trials of the Melody transcatheter pulmonary valve.

OBJECTIVE: We compared 5-year outcomes of transcatheter pulmonary valve (TPV) replacement with the Melody TPV in the post-approval study (PAS) and the investigational device exemption (IDE) trial. BACKGROUND: As a condition of approval of the Melody TPV after the IDE trial, the Food and Drug Administration required that a PAS be conducted to evaluate outcomes of TPV replacement in a "real-world" environment. The 5-year outcomes of the PAS have not been published, and the IDE and PAS trials have not been compared. METHODS: The cohorts comprised all patients catheterized and implanted at 5 IDE sites and 10 PAS sites. Differences in trial protocols were detailed. Time-related outcomes and valve-related adverse events were compared between the two trials with Kaplan-Meier curves and log-rank testing. RESULTS: 167 patients (median age, 19 years) were catheterized and 150 underwent TPV replacement in the IDE trial; 121 were catheterized (median age, 17 years) and 100 implanted in the PAS. Freedom from hemodynamic dysfunction (p = .61) or any reintervention (p = .74) over time did not differ between trials. Freedom from stent fracture (p = .003) and transcatheter reintervention (p = .010) were longer in PAS, whereas freedom from explant (p = .020) and TPV endocarditis (p = .007) were shorter. Clinically important adverse events (AEs) were reported in 14% of PAS and 7.2% of IDE patients (p = .056); the incidence of any particular event was low in both. CONCLUSIONS: Hemodynamic and time-related outcomes in the PAS and IDE trials were generally similar, confirming the effectiveness of the Melody TPV with real-world providers. There were few significant complications and limited power to identify important differences in AEs. The lack of major differences in outcomes between the two studies questions the usefulness of mandated costly post-approval studies as part of the regulatory process for Class III medical devices.

Acute and short-term haemodynamic impact of transcatheter pulmonary valve implantation on left ventricular systolic and diastolic function.

OBJECTIVES: Assess the acute and short-term haemodynamic impact of transcatheter pulmonary valve implantation on left ventricular systolic and diastolic function stratified by pre-transcatheter pulmonary valve implantation physiology. BACKGROUND: Transcatheter pulmonary valve implantation is a widely available option to treat residual or recurrent pulmonary stenosis and pulmonary insufficiency. Transcatheter pulmonary valve implantation acutely increases pulmonary artery size and diastolic pressure in patients with pulmonary insufficiency and acute pulmonary edema has been reported after transcatheter pulmonary valve implantation, possibly related to acute left ventricular volume loading. However, the impact of transcatheter pulmonary valve implantation on left ventricular diastolic function has not been established. METHODS: Patients who underwent transcatheter pulmonary valve implantation from 2010 to 2017 at our centre were grouped by indication for transcatheter pulmonary valve implantation as pulmonary stenosis, pulmonary insufficiency, or mixed disease. Separate analysis was performed on those who underwent transcatheter pulmonary valve implantation for pulmonary stenosis versus pulmonary insufficiency or mixed disease. Intracardiac haemodynamics immediately before and after transcatheter pulmonary valve implantation and echocardiographic assessment of left ventricular systolic and diastolic function at baseline, 1-day post transcatheter pulmonary valve implantation, and 1-year post transcatheter pulmonary valve implantation were compared between groups. RESULTS: In 102 patients who underwent transcatheter pulmonary valve implantation, the indication was pulmonary stenosis in 29 (28%), pulmonary insufficiency in 28 (29%), and mixed disease in 44 (43%). There were no significant differences in left ventricular systolic or diastolic function between groups at baseline, immediately after transcatheter pulmonary valve implantation, or 1-year post implantation. The mean pulmonary artery wedge pressure increased equally across groups. CONCLUSIONS: While patients with pulmonary insufficiency likely have acute left ventricular volume loading following transcatheter pulmonary valve implantation, this does not appear to be haemodynamically significant as transcatheter pulmonary valve implantation was not associated with measurable changes in left ventricular systolic or diastolic function acutely or 1-year post implantation.

Data quality methods through remote source data verification auditing: results from the Congenital Cardiac Research Collaborative.

BACKGROUND: Multicentre research databases can provide insights into healthcare processes to improve outcomes and make practice recommendations for novel approaches. Effective audits can establish a framework for reporting research efforts, ensuring accurate reporting, and spearheading quality improvement. Although a variety of data auditing models and standards exist, barriers to effective auditing including costs, regulatory requirements, travel, and design complexity must be considered. MATERIALS AND METHODS: The Congenital Cardiac Research Collaborative conducted a virtual data training initiative and remote source data verification audit on a retrospective multicentre dataset. CCRC investigators across nine institutions were trained to extract and enter data into a robust dataset on patients with tetralogy of Fallot who required neonatal intervention. Centres provided de-identified source files for a randomised 10% patient sample audit. Key auditing variables, discrepancy types, and severity levels were analysed across two study groups, primary repair and staged repair. RESULTS: Of the total 572 study patients, data from 58 patients (31 staged repairs and 27 primary repairs) were source data verified. Amongst the 1790 variables audited, 45 discrepancies were discovered, resulting in an overall accuracy rate of 97.5%. High accuracy rates were consistent across all CCRC institutions ranging from 94.6% to 99.4% and were reported for both minor (1.5%) and major discrepancies type classifications (1.1%). CONCLUSION: Findings indicate that implementing a virtual multicentre training initiative and remote source data verification audit can identify data quality concerns and produce a reliable, high-quality dataset. Remote auditing capacity is especially important during the current COVID-19 pandemic.

Use of 3D rotational angiography to perform computational fluid dynamics and virtual interventions in aortic coarctation.

Computational fluid dynamics (CFD) can be used to analyze blood flow and to predict hemodynamic outcomes after interventions for coarctation of the aorta and other cardiovascular diseases. We report the first use of cardiac 3-dimensional rotational angiography for CFD and show not only feasibility but also validation of its hemodynamic computations with catheter-based measurements in three patients.

Relationship Between Time to Left Atrial Decompression and Outcomes in Patients Receiving Venoarterial Extracorporeal Membrane Oxygenation Support: A Multicenter Pediatric Interventional Cardiology Early-Career Society Study.

OBJECTIVES: To assess the variation in timing of left atrial decompression and its association with clinical outcomes in pediatric patients supported with venoarterial extracorporeal membrane oxygenation across a multicenter cohort. DESIGN: Multicenter retrospective study. SETTING: Eleven pediatric hospitals within the United States. PATIENTS: Patients less than 18 years on venoarterial extracorporeal membrane oxygenation who underwent left atrial decompression from 2004 to 2016. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 137 patients (median age, 4.7 yr) were included. Cardiomyopathy was the most common diagnosis (47%). Cardiac arrest (39%) and low cardiac output (50%) were the most common extracorporeal membrane oxygenation indications. Median time to left atrial decompression was 6.2 hours (interquartile range, 3.8-17.2 hr) with the optimal cut-point of greater than or equal to 18 hours for late decompression determined by receiver operating characteristic curve. In univariate analysis, late decompression was associated with longer extracorporeal membrane oxygenation duration (median 8.5 vs 5 d; p = 0.02). In multivariable analysis taking into account clinical confounder and center effects, late decompression remained significantly associated with prolonged extracorporeal membrane oxygenation duration (adjusted odds ratio, 4.4; p = 0.002). Late decompression was also associated with longer duration of mechanical ventilation (adjusted odds ratio, 4.8; p = 0.002). Timing of decompression was not associated with in-hospital survival (p = 0.36) or overall survival (p = 0.42) with median follow-up of 3.2 years. CONCLUSIONS: In this multicenter study of pediatric patients receiving venoarterial extracorporeal membrane oxygenation, late left atrial decompression (≥ 18 hr) was associated with longer duration of extracorporeal membrane oxygenation support and mechanical ventilation. Although no survival benefit was demonstrated, the known morbidities associated with prolonged extracorporeal membrane oxygenation use may justify a recommendation for early left atrial decompression.

Impact of Left Atrial Decompression on Patient Outcomes During Pediatric Venoarterial Extracorporeal Membrane Oxygenation: A Case-Control Study.

Left heart distension during venoarterial extracorporeal membrane oxygenation (VA ECMO) often necessitates decompression to facilitate myocardial recovery and prevent life-threatening complications. The objectives of this study were to compare clinical outcomes between patients who did and did not undergo left atrial (LA) decompression, quantify decompression efficacy, and identify risk factors for development of left heart distension. This was a single-center retrospective case-control study. Pediatric VA ECMO patients who underwent LA decompression from June 2004 to March 2016 were identified, and a control cohort of VA ECMO patients who did not undergo LA decompression were matched based on diagnosis, extracorporeal cardiopulmonary resuscitation, and age. Among 194 VA ECMO cases, 21 (11%) underwent LA decompression. Compared to the control cohort, patients with decompression had longer hospital length of stay (60 ± 55 vs. 27 ± 23 days, p = 0.012), but similar in-hospital mortality (29% vs. 38%, p = 0.513). Decompression successfully decreased mean LA pressure (24 ± 11 to 14 ± 4 mmHg, p = 0.022) and LA:RA pressure gradient (10 ± 7 to 0 ± 1 mmHg, p = 0.011). No significant differences in early quantitative measures of cardiac function were observed between cases and controls to identify risk factors for left heart distension. Despite higher qualitative risk for impaired cardiac recovery, patients who underwent LA decompression had comparable outcomes to those who did not. Given that traditional quantitative measures of cardiac function are insufficient to predict development of eventual left heart distension, a combination of clinical history, radiographic findings, hemodynamic monitoring, and laboratory markers should be used during the evaluation and management of these patients.

Acute Hemodynamic Effects of Negative Extrathoracic Pressure in Fontan Physiology.

We sought to assess acute hemodynamic changes after implementation of negative extrathoracic pressure (NEP) in spontaneously breathing ambulatory Fontan patients with symptomatic heart failure. We hypothesized that application of NEP would result in an acute decrease in pulmonary artery pressure. Ten patients with clinical evidence of Fontan failure underwent baseline hemodynamic catheterization while breathing spontaneously. Hemodynamic measurements were then repeated after 30 min of continuous NEP. After 30 min of continuous NEP, 4/10 patients had a decrease in their Fontan pressure by 2 mmHg and one patient had a decrease by 1 mmHg. There were three patients that had an increase in Fontan pressure by 2 mmHg. In 7/10 patients, indexed pulmonary vascular resistance decreased by an average of 31%. In symptomatic Fontan patients with a favorable hemodynamic response to NEP during catheterization, potential benefit of longer-term NEP to improve clinical status should be explored.

Echocardiographic predictors of elevated left ventricular end diastolic pressure in adolescent and adult patients with repaired tetralogy of Fallot.

Elevated left ventricular end diastolic pressure is a risk factor for ventricular arrhythmias in patients with tetralogy of Fallot. The objective of this retrospective study was to identify echocardiographic measures associated with left ventricular end diastolic pressure >12 mmHg in this population. Repaired tetralogy of Fallot patients age ≥13 years, who underwent a left heart catheterisation within 7 days of having an echocardiogram were evaluated. Univariate comparison was made in echocardiographic and clinical variables between patients with left ventricular end diastolic pressure >12 versus ≤12 mmHg. Ninety-four patients (54% male) with a median age of 24.6 years were included. Thirty-four (36%) had left ventricular end diastolic pressure >12 mmHg. Patients with left ventricular end diastolic pressure >12mmHg were older (median 32.9 versus 24.0 years, p = 0.02), more likely to have a history of an aortopulmonary shunt (62% versus 38%, p = 0.03), and have a diagnosis of hypertension (24% versus 7%, p = 0.03) compared to those with left ventricular end diastolic pressure ≤12 mmHg. There were no significant differences in mitral valve E/A ratio, annular e' velocity, or E/e' ratio between patients with left ventricular end diastolic pressure >12 versus ≤12 mmHg. Patients with left ventricular end diastolic pressure >12mmHg had larger left atrial area (mean 17.7 versus 14.0 cm2, p = 0.03) and larger left atrium anterior-posterior diameter (mean 36.0 versus 30.6 mm, p = 0.004). In conclusion, typical echocardiographic measures of left ventricular diastolic dysfunction may not be reliable in tetralogy of Fallot patients. Prospective studies with the use of novel echocardiographic measures are needed.