The Fate of the Left Ventricular Outflow Tract Following Interrupted Aortic Arch Repair.

Objectives: To examine the probability of left ventricular outflow tract (LVOT) reintervention following interrupted aortic arch (IAA) repair in neonates with LVOT obstruction (LVOTO) risk. Methods: This retrospective multicenter study included 150 neonates who underwent IAA repair (2003-2017); 100 of 150 (67%) had isolated IAA repair (with ventricular septal defect closure) and 50 of 150 (33%) had concomitant LVOT intervention: conal muscle resection (n = 16), Ross-Konno (n = 7), and Yasui operation (n = 27: single-stage n = 8, staged n = 19). Demographic and morphologic characteristics were reviewed. Factors associated with LVOT reoperation were explored using multivariable analysis. Results: Concomitant LVOT intervention was more likely in neonates with type B IAA, bicuspid aortic valve, aberrant right subclavian artery, smaller aortic valve annulus, and ascending aorta dimensions. On follow-up, five-year freedom from LVOT reoperation was highest following Ross-Konno (100%), 77% following Yasui (mainly for neo-aortic regurgitation), 77% following isolated IAA repair (mainly for LVOTO), and 47% following IAA repair with concomitant conal resection, P = .033. While all patients had low peak LVOT gradient at time of discharge, those who had conal resection developed higher gradients on follow-up (P = .007). Ross-Konno and Yasui procedures were associated with higher right ventricular outflow tract (RVOT) reoperation. In the cohort following isolated IAA repair, aortic sinus Z score was associated with LVOT reoperation. Conclusions: Both Yasui and Ross-Konno operations effectively mitigate late LVOTO risk. The highest risk of reintervention for LVOTO was associated with conal muscle resection while the lowest risk is associated with Ross-Konno. The RVOT reoperation risk in patients who had Ross-Konno or Yasui does not seem to affect survival.

Early outcomes of moderate-to-high-risk pediatric congenital cardiac surgery and predictors of extracorporeal circulatory life support requirement.

Background: Cardiopulmonary failure refractory to medical management after moderate-to-high-risk congenital cardiac surgery may necessitate mechanical support with veno-arterial extracorporeal membrane oxygenation (ECMO). On the extreme, ECMO can also be initiated in the setting of cardiac arrest (extracorporeal cardiopulmonary resuscitation, ECPR) unresponsive to conventional resuscitative measures. Methods: This was a single-center retrospective cohort study of patients (n = 510) aged <3 years old who underwent cardiac surgery with cardiopulmonary bypass with a RACHS-1 score ≥3 between 2011 and 2014. Perioperative factors were reviewed to identify predictors of ECMO initiation and mortality in the operating room (OR) and the intensive care unit (ICU). Results: A total of 510 patients with a mean surgical age of 10.0 ± 13.4 months were included. Among them, 21 (4%) patients received postoperative ECMO-12 were initiated in the OR and 9 in the ICU. ECMO cannulation was associated with cardiopulmonary bypass duration, aortopulmonary shunt, residual severe mitral regurgitation, vaso-inotropic score, and postprocedural lactate (p < 0.001). Of the 32 (6%) total deaths, 7 (22%) were ECMO patients-4 were elective OR cannulations and 3 were ICU ECPR. Prematurity [hazard ratio (HR): 2.61, p < 0.01), Norwood or Damus-Kaye-Stansel procedure (HR: 4.29, p < 0.001), postoperative left ventricular dysfunction (HR: 5.10, p = 0.01), residual severe tricuspid regurgitation (HR: 6.06, p < 0.001), and postoperative ECMO (ECPR: HR: 15.42, p < 0.001 vs. elective: HR: 5.26, p = 0.01) were associated with mortality. The two patients who were electively cannulated in the ICU survived. Discussion: Although uncommon, postoperative ECMO in children after congenital cardiac surgery is associated with high mortality, especially in cases of ECPR. Patients with long cardiopulmonary bypass time, residual cardiac lesions, or increased vaso-inotropic requirement are at higher risk of receiving ECMO. Pre-emptive or early ECMO initiation before deterioration into cardiac arrest may improve survival.

Coronary artery lesions are associated with adverse cardiac events in children undergoing supravalvular aortic stenosis repair.

OBJECTIVES: The aim of this study was to identify the prevalence and anatomic characteristics of coronary artery lesions and their associated postoperative risk in patients undergoing supravalvular aortic stenosis repair. METHODS: The association between structural risk factors, postoperative ST-segment changes, and major adverse cardiac events was explored using logistic regression and the Fisher's exact test. RESULTS: In 51 consecutive patients with supravalvular aortic stenosis treated between 2000 and 2017, a total of 48 coronary lesions were identified in 27 patients (53%). Prominent ostial ridge (type I) was the most common coronary lesion, followed by small ostium with (IIIb) or without (IIIa) diffuse long-segment coronary narrowing, and adhesion of the coronary cusp (type II). There were 54 concomitant coronary procedures, including 43 primary corrections and 11 revisions. Thirty-three patients underwent supravalvular aortic stenosis repair with a bifurcated patch, of which 13 (39.4%) had right coronary artery distortion/kinking requiring patch plication (n = 8) and reimplantation (n = 5). Postoperative major adverse cardiac events (MACE) occurred in 9 patients (17.6%), including 3 deaths, 4 needing mechanical circulatory support, and 6 experiencing ventricular arrhythmias. Twenty-two patients (43.1%) had postoperative ST-segment changes, including 13 early changes that resolved within 24 h and 9 persistent changes lasting >24 h. Patients with type III lesions were associated with postoperative persistent ST-segment change (P = 0.04) and these lesions independently predicted postoperative MACE (P = 0.02). Patients with pre-existing coronary lesions were at elevated risk of right coronary artery distortion/kinking (P = 0.045). CONCLUSIONS: The prevalence of ST-segment changes and MACE is high in patients undergoing supravalvular aortic stenosis repair. The preoperative presence of complex coronary lesions is the most important predictor for postoperative major adverse cardiac events.

Considerations & challenges of mitral valve repair in females: diagnosis, pathology, and intervention.

PURPOSE OF REVIEW: Disparities in mitral valve (MV) repair outcomes exist between men and women. This review highlights sex-specific differences in MV disease aetiology, diagnosis, as well as timing and type of intervention. RECENT FINDINGS: Females present with more complicated disease: anterior or bileaflet prolapse, leaflet dysplasia/thickening, mitral annular calcification, and mixed mitral lesions. The absence of indexed echocardiographic mitral regurgitation (MR) severity parameters contributes to delayed intervention in women, resulting in more severe symptom burden at time of surgery. The sequelae of chronic MR also necessitate concomitant procedures (e.g. tricuspid repair, arrhythmia surgery) at the time of mitral surgery. Complex MV pathology, greater patient acuity, and more complicated procedures collectively pose challenges to successful MV repair and postoperative recovery. As a consequence, women receive disproportionately more MV replacement than men. In-hospital mortality after MV repair is also greater in women than men. Long-term outcomes of MV repair are comparable after risk-adjustment for preoperative status; however, women experience a greater incidence of postoperative heart failure. SUMMARY: To address the inequity in MV repair outcomes between sexes, indexed diagnostic measurements, diligent surveillance of asymptomatic MR, increased recruitment of women in large clinical trials, and mandatory reporting of sex-based subgroup analyses are recommended.

Branch pulmonary artery stenosis after arterial switch operation: The effect of preoperative anatomic factors on reintervention.

BACKGROUND: We hypothesized that preoperative patient characteristics and branch pulmonary artery (PA) size might influence the rate of postoperative branch PA reintervention in patients with transposition of the great arteries who undergo the arterial switch operation (ASO). METHODS: The retrospective single-center study included 262 consecutive (2008-2017) newborns who underwent the ASO. Demographic characteristics, echocardiography, and clinical outcomes were reviewed. Competing risk analysis modeled incidence of branch PA reintervention and cause-specific hazard regression for predictors analyses. RESULTS: Median age and weight were 7 (range, 5-11) days and 3.4 (range, 3.1-3.8) kg, respectively. Various types of early branch PA reinterventions (concomitant revision or reintervention during the intensive care unit stay) were required in 28 (10.7%) patients. These patients had prolonged ventilation (P < .001), intensive care unit duration (P < .001), worse right ventricular function (P = .043), and high in-hospital mortality (P = .010). Branch PA dimensions significantly decreased immediately after ASO compared with baseline measurements. The median follow-up duration was 20.8 (range, 0.9-44.7) months. Branch PA reintervention was common among survivors without early reinterventions (9.4%), and even more frequent among those with early reinterventions (25%). Subsequent reintervention (all catheter-based) was necessary for more than one-third of patients after initial branch PA reintervention. The multivariable analysis showed preoperative dimension of the left PA (hazard ratio, 0.527 [95% CI, 0.337-0.823]; P = .005), and right PA (hazard ratio, 0.503 [95% CI, 0.318-0.796]; P = .003) were independently associated with late branch PA reinterventions. CONCLUSIONS: Branch PA reintervention was common and often required surgical or catheter-based reinterventions after ASO. PA branch diameters became significantly smaller after ASO. Smaller preoperative branch PA predicted late branch PA reintervention, indicating a smaller margin of geometrical tolerance to this effect.

Low preoperative superior vena cava blood flow predicts bidirectional cavopulmonary shunt failure.

BACKGROUND: In this study we sought to determine whether preoperative superior vena cava (SVC) blood flow measured using cardiac magnetic resonance (CMR) predicts physiology and clinical outcome after bidirectional cavopulmonary shunt (BCPS). METHODS: The retrospective single-center study included 65 (2012-2017) patients who underwent BCPS. Preoperative CMR imaging, echocardiography, catheterization, and clinical outcomes were reviewed. SVC flow was measured using phase contrast CMR. The Kaplan-Meier method and Cox regression was used for BCPS takedown-free survival and predictor analyses. RESULTS: The absolute and indexed SVC flow was 0.5 (interquartile range [IQR], 0.4-0.7) L/min and 1.7 (IQR, 1.4-2.0) L/min/mm2 respectively, which was comparable with the SVC blood flow volume previously measured. The median age and body weight at BCPS was 6.5 (IQR, 5.5-8.5) months and 6.9 (IQR, 6.0-7.7) kg. After follow-up, at a median of 17.1 (IQR, 7.9-41.3) months, 14 patients (21.5%) underwent the Fontan completion and 40 (61.5%) with BCPS physiology were waiting for the Fontan completion. The 11 remaining patients (16.9%), included those who underwent takedown (n = 7; 10.8%) or died with a BCPS (n = 4; 6.2%). Severe hypoxia was the leading cause of mortality, directly accounting for two-thirds of deaths (66.6%; 6/9). The BCPS takedown-free survival was 96.8% at 6 months, and 79.9% at 3 years. Preoperative SVC blood flow was significantly positively correlated with early post-BCPS arterial saturation (P = .00). The multivariable analysis showed SVC flow was the only factor associated with BCPS failure (hazard ratio, 0.186; P = .04) among the predictors related to the pre-BCPS anatomy and physiology. CONCLUSIONS: SVC blood flow might be as critically important as pulmonary artery anatomic and physiologic parameters in the evaluation of BCPS candidacy in the single-ventricle population.