Search

BackgroundNeonates with complex congenital heart disease (CHD) and pulmonary overcirculation have been historically treated surgically. However, sub-cohorts of patients may benefit from less invasive procedures. Transcatheter palliation with pulmonary flow restrictors (PFRs) may represent a compelling alternative, but data are limited.MethodsWe present our experience of palliation with PFRs in neonates with CHD and pulmonary overcirculation, including procedural feasibility, technical details, and patient-level outcomes. We then compared our sub-cohort of high-risk single ventricle (SV) neonates palliated with PFRs with a historical cohort of high-risk SV neonates palliated with a hybrid Stage 1. Cox regression was used to evaluate the association between palliation strategy and all-cause mortality risk at 6 months.ResultsFrom 2021 to 2023, 17 patients (median age 4 days, interquartile range [IQR] 2-8); median weight 2.51 kilograms [IQR 2.09-3.26]) underwent a PFR procedure; 15 (88%) had SV physiology; 15 (88%) were considered high-risk surgical candidates. All the procedures were technically successful. At a median follow-up of 5.3 months (IQR 1.9-9.6), 13 patients (76%) were either successfully bridged to surgery (n=10, 59%) or are awaiting surgery (n=3, 17%). Patients underwent the target surgery after a median of 2.6 months (IQR 1.2-3.4) from the PFR procedure (median weight 4.6 kilograms [IQR 3.2-5.4]). Their pulmonary arteries were found to have grown adequately for age. All PFR devices were easily removed without the need for arterioplasty. The all-cause mortality rate before target surgery was 24% (n=4). Compared to a historical cohort of high-risk SV neonates palliated with a hybrid Stage 1 (n=23), after adjustment for main confounding (age, weight, presence of intact atrial septum or severely restrictive patent foramen ovale, and presence of left ventricle to coronary fistulae), the PFR procedure was associated with a significantly lower all-cause 6-month mortality risk (adjusted Hazard Ratio=0.30 [95% CI 0.10- 0.93]).ConclusionsTranscatheter PFR palliation in high-risk neonates with CHD is feasible, safe, and may represent an effective alternative strategy to bridge such high-risk neonates to surgical palliation, complete repair, or transplant while allowing for clinical stabilization and somatic growth.Clinical perspectivesWhat is new?Transcatheter PFR palliation in high-risk neonates with congenital heart disease is feasible, safe, and effective in reducing pulmonary blood flow and allow for clinical stabilization and growing.PFR devices can be easily removed both at cardiac catheterization or surgery with no need for pulmonary arterioplasty, and pulmonary artery grow adequately over time.Compared to a historical cohort of high-risk single ventricle neonates palliated with a hybrid Stage-1, after adjustment for main confounding, the PFR palliation was shown to be associated with a significantly lower 6-month all-cause mortality risk.What are the clinical implications?Transcatheter PFR palliation in high-risk neonates may represent an effective alternative strategy to bridge such high-risk neonates to surgical palliation, complete repair, or transplant while avoiding a surgical procedure and allowing for clinical stabilization and somatic growth.

Data regarding the influence of intraoperative residual lesions on extracorporeal membrane oxygenation (ECMO) following the Norwood procedure are limited. Moreover, the significance of postoperative ECMO timing on in-hospital outcomes remains incompletely characterized.This was a single-center, retrospective review of consecutive patients who underwent the Norwood operation from January 1997 to November 2017. Patients with at least minor residual lesions based on the intraoperative postcardiopulmonary bypass echocardiogram were identified. The association between residual lesions and postoperative ECMO was assessed with logistic regression, adjusting for age, weight, prematurity, various preoperative system-specific and procedural risk factors, shunt type, and era. Among patients receiving ECMO, associations between late ECMO (≥3 days post-Norwood) and in-hospital mortality or transplant, postoperative hospital length-of-stay, and cost of hospitalization were evaluated using logistic regression or generalized linear models with a gamma distribution and logarithmic link.Among 500 patients, 78 (15.6%) received ECMO postoperatively. On multivariable analysis, the presence of at least minor residual lesions (odds ratio, 4.4; 95% CI, 2.1-9.3; P .001) was associated with postoperative ECMO. In the ECMO subpopulation, there were 44 (56.4%) deaths or transplants. Late ECMO was associated with increased risk of in-hospital mortality or transplant (adjusted odds ratio, 6.2; 95% CI, 1.5-26.0), longer postoperative hospital length of stay (regression coefficient, 0.7; 95% CI, 0.3-1.1), and greater cost (regression coefficient, 0.6; 95%, CI 0.4-0.7), versus early ECMO (all P values .05).The presence of even minor intraoperative residua significantly increases the risk of ECMO following the Norwood operation. Among patients receiving ECMO postoperatively, early institution of ECMO is associated with lower mortality and resource utilization.

Outcomes after total anomalous pulmonary venous connection (TAPVC) repair remain suboptimal due to recurrent pulmonary vein (PV) obstruction requiring reinterventions. We sought to develop a clinical prediction rule for PV reintervention after TAPVC repair.Data from consecutive patients who underwent TAPVC repair at a single institution from January 1980 to January 2020 were retrospectively reviewed after Institutional Review Board approval. The primary outcome was postdischarge (late) unplanned PV surgical or transcatheter reintervention. Echocardiographic criteria were used to assess PV residual lesion severity at discharge (class 1: no residua; class 2: minor residua; class 3: major residua). Competing risk models were used to develop a weighted risk score for late reintervention.Of 437 patients who met entry criteria, there were 81 (18.5%) reinterventions at a median follow-up of 15.6 (interquartile range, 5.5-22.2) years. On univariable analysis, minor and major PV residua, age, single-ventricle physiology, infracardiac and mixed TAPVC, and preoperative obstruction were associated with late reintervention (all P.05). The final risk prediction model included PV residua (class 2: subdistribution hazard ratio [SHR], 4.8; 95% CI, 2.8-8.1; P.001; class 3: SHR, 6.4; 95% CI, 3.5-11.7; P.001), age1 year (SHR, 3.3; 95% CI, 1.3-8.5; P = .014), and preoperative obstruction (SHR, 1.8; 95% CI, 1.1-2.8; P = .015). A risk score comprising PV residua (class 2 or 3: 3 points), age (neonate or infant: 2 points), and obstruction (1 point) was formulated. Higher risk scores were significantly associated with worse freedom from reintervention (P.001).A risk prediction model of late reintervention may guide prognostication of high-risk patients after TAPVC repair.

Outcomes after first-stage palliation of single-ventricle heart disease are influenced by many factors, including the presence of residual lesions requiring reintervention. However, there is a dearth of information regarding the optimal timing of reintervention. We assessed if earlier reintervention would be favorably associated with in-hospital outcomes among patients requiring unplanned reinterventions after the Norwood operation.This was a single-center, retrospective review of all patients who underwent the Norwood procedure from January 1997 to November 2017 and required a predischarge unplanned surgical or transcatheter reintervention on 1 or more subcomponent areas repaired at the index operation. Outcomes of interest included in-hospital mortality or transplant, postoperative hospital length of stay, and inpatient cost. Associations between timing of reintervention and outcomes were assessed using logistic regression (mortality or transplant) or generalized linear models (postoperative hospital length of stay and cost), adjusting for baseline patient-related and procedural factors.Of 500 patients who underwent the Norwood operation, 92 (18.4%) required an unplanned reintervention. Median time to reintervention was 12 days (interquartile range, 5-35 days). There were 31 (33.7%) deaths or transplants, median postoperative hospital length of stay was 49 days (interquartile range, 32-87 days), and median cost was $328,000 (interquartile range, $204,000-$464,000). On multivariable analysis, each 5-day increase in time to reintervention increased the odds of mortality or transplant by 20% (odds ratio, 1.2; 95% confidence interval, 1.1-1.3; P = .004). Longer time to reintervention was also significantly associated with greater postoperative hospital length of stay (P .001) and higher cost (P .001).For patients requiring predischarge unplanned reinterventions after the Norwood operation, earlier reintervention is associated with improved in-hospital transplant-free survival and resource use.

OBJECTIVES Among patients with hypoplastic left heart syndrome (HLHS), tricuspid valve regurgitation (TR) portends a poor prognosis. Our goal was to describe the outcomes of tricuspid valve reconstruction (TVR) concomitant with the Norwood operation and using two-dimensional echocardiography and evaluate the structural factors associated with successful functional correction. METHODS We performed a retrospective, single-centre study of patients with HLHS undergoing TVR at the time of the Norwood operation. Structural echocardiographic parameters were compared between patients with successful correction (≤ mild TR) and those with ≥ moderate regurgitation at discharge. Preoperative dimensions of matched HLHS controls with ≤ trivial TR were used as a reference. RESULTS Of 205 patients with HLHS undergoing the Norwood operation, 18 patients had a concomitant TVR. Ten (56%) patients had an improved TR grade postoperatively, 8 (44%) of whom had ≤ mild TR at discharge. Improvement in TR grade (P = 0.001) and having ≤ mild TR at discharge (P = 0.011) were associated with an improved reintervention and TR-free survival. Patients with successful functional correction had smaller preoperative tricuspid annulus lateral dimensions (P = 0.023), tricuspid valve area (P = 0.007) and right ventricle mid-width (P = 0.064). Preoperatively, the successful TVR cases tended to have had higher anterior leaflet excursion (80 ± 20 vs 55 ± 11, P = 0.010), and a higher proportion of anterior leaflet prolapse (63% vs 10%, P = 0.043) compared to cases where TVR was not successful. CONCLUSIONS Patients with HLHS with significant tricuspid regurgitation undergoing the stage 1 operation were more likely to have successful concomitant tricuspid valve repair if they had less tricuspid annular dilation, less-severe RV enlargement and predominantly anterior leaflet prolapse. Successful tricuspid valve repair was associated with improved mid- and long-term outcomes.