Long-term outcomes of heart transplantation in adults with congenital heart disease: The impact of single-ventricle versus biventricular physiology.

Objective: Congenital heart disease is a risk factor for mortality after orthotopic heart transplantation; however, the impact of preoperative circulation type and primary congenital heart disease diagnosis remains poorly delineated. Methods: We retrospectively reviewed patients with adult congenital heart disease aged 16 years or more who underwent orthotopic heart transplantation at our institution between 2008 and 2022. Patients were categorized as having single-ventricle or biventricular circulation. The primary end point was 5-year post-transplant survival. Results: Sixty-one patients with adult congenital heart disease (single-ventricle: n = 26 [42.6%], biventricular: n = 35 [57.4%]) underwent orthotopic heart transplantation at 33.7 [interquartile range, 19.1-48.7] years. The most common congenital heart disease diagnosis was hypoplastic left heart syndrome (n = 11, 42.3%) in the single-ventricle group and congenitally corrected transposition of the great arteries (n = 7, 20.0%) in the biventricular group. Twenty-four patients previously underwent Fontan palliation. At transplant, patients in the single-ventricle group were younger (18.5 [interquartile range, 17.6-32.3] years vs 45.0 [interquartile range, 33.0-52.2] years, P < .001) and more likely to have biopsy-proven cirrhosis (46.2% vs 14.3%, P = .01) and protein-losing enteropathy (42.3% vs 2.9%, P < .001). Patients in the single-ventricle group also had longer bypass times (223.4 ± 65.3 minutes vs 187.4 ± 59.5 minutes, P = .03) and longer durations of mechanical ventilatory support (3.5 [interquartile range, 2.0-6.0] days vs 1.0 [interquartile range, 1.0-2.0] days, P < .001). Operative mortality was comparable (11.5% vs 8.6%, P = 1). Median follow-up was 6.0 [interquartile range, 2.4-10.0] years. Five-year survival was worse in the single-ventricle group (66.0% ± 10.0% vs 91.3% ± 4.8%, P = .03), as was freedom from major rejection (58.3% ± 10.2% vs 84.0% ± 6.6%, P = .02). In univariable analysis, hypoplastic left heart syndrome and Fontan circulation were risk factors for post-transplant mortality (hypoplastic left heart syndrome: hazard ratio, 5.0, P < .001; Fontan: hazard ratio, 3.5, P = .03). Conclusions: Adult patients with congenital heart disease undergoing heart transplant with single-ventricle physiology experienced a more complicated post-transplant course, with worse long-term survival and freedom from rejection. Multicenter studies are required to guide orthotopic heart transplantation decision-making in this complex cohort.

Rare Coronary Artery Variants are Associated with Increased Mortality and Reinterventions Following the Arterial Switch Operation.

OBJECTIVE: To determine the influence of coronary anatomy on long-term outcomes of the arterial switch operation (ASO). METHODS: We retrospectively reviewed patients with transposition of the great arteries or Taussig-Bing anomaly who underwent ASO at our institution between 1992 and 2022. The primary endpoint was freedom from a composite of death, transplant, or coronary reintervention. RESULTS: A total of 632 patients (median age: 5.0 days [IQR, 4.0-7.0]) underwent ASO. Coronary anatomy included: usual (n=411, 65%), circumflex from sinus 2 (n=89, 14%), inverted (n=55, 9%), single sinus (n=46, 7%), and intramural (n=31, 5%). Overall operative mortality was 3% (n=16) and highest among intramurals (n=3, 10%), though dropped to 0% in this group in the most recent decade. Median follow-up was 14.5 years [IQR, 6.0-20.3]. Twenty-year freedom from the primary endpoint was 95%±1% for usual anatomy, 99%±1% for circumflex from sinus 2, 90%±4% for inverted, 91%±4% for single sinus, and 80%±9% for intramural (P<0.001). Intramurals had the highest 20-year incidence of coronary reintervention (11%±8%). Cox modeling identified intraoperative coronary revision (HR 20.1, 95% CI:[9.4-53.9], P<0.001), Taussig-Bing anomaly (HR 4.9, 95% CI:[2.2-10.9], P<0.001), and an intramural coronary artery (HR 2.9, 95% CI: [1.0-8.2], P=0.04) to be risk factors for the composite endpoint. CONCLUSIONS: Rare coronary artery variants-particularly intramural-are associated with increased mortality and coronary reinterventions after ASO. A low threshold for unroofing intramurals is likely associated with declining mortality and improved outcomes. Additional investigations are required to determine the long-term fate of the coronary arteries after ASO.

Outcomes After Heartmate 3 Left Ventricular Assist Device Implantation Using a 10 mm Outflow Graft.

The presence of adhesions and patent bypass grafts may create challenges for standard 14 mm outflow graft placement during left ventricular assist device implantation. We retrospectively describe our experience using a 10 mm Bioline Fusion graft (Getinge, Goteborg, Sweden) as the outflow graft in patients undergoing primary Heartmate 3 (Abbott, Abbott Park, IL) implantation. One hundred one patients underwent Heartmate 3 left ventricular assist device implantation, 80% via a thoracotomy approach, with the standard 14 mm outflow graft (78) or a 10 mm Bioline Fusion outflow graft (23). Initial postoperative rotor speed-to-flow ratio (the revolutions per minutes (RPMs) required to achieve a given flow) was significantly higher in 10 mm graft patients (1,472 vs. 1,283 RPM/L/min; p = 0.03), suggesting elevated resistance in the smaller graft. Furthermore, the initial postoperative vasoactive-inotrope score was higher in the 10 mm graft patients (24.1 vs. 17.6; p = 0.022). Postoperative outcomes were similar between groups. In conclusion, the use of a 10 mm graft was associated with higher RPMs needed to generate a given flow and a higher vasoactive-inotrope score, but these differences were not associated with increased right ventricular failure or mortality.

Medium-Term Outcomes of Concomitant Aortic Valve Repair in Patients with Continuous-Flow Left Ventricular Assist Device.

OBJECTIVE: Our objective was to analyze the development of aortic insufficiency in patients who received central aortic valve repair when undergoing continuous-flow left ventricular assist device implantation. METHODS: We conducted a retrospective review of patients who underwent HeartMate II or 3 (Abbott Lab) implantation between 2004 and 2022. Ninety-four patients were excluded from analysis for history of aortic valve procedures, a bicuspid aortic valve, baseline trace aortic insufficiency, or other concomitant aortic valve procedure. Patients who had ≥ mild aortic insufficiency had concomitant aortic valve repair. Clinical characteristics, serial echocardiograms, and outcomes were determined. RESULTS: Of the 656 patients who underwent HeartMate II or 3 implantation, 105 patients (59 HeartMate II and 46 HeartMate 3) met study criteria. Median age was 68 years [60-74 years], 91.4% [n=96] were male, 54.4% [n=56] were white, and 68.6% [n=72] received support as destination therapy. Preoperative aortic insufficiency degree was 54.3% (n=57) mild, 23.8% (n=25) mild-to-moderate, 20.0% (n=21) moderate, 1.0% (n=1) moderate-to-severe, 1.0% (n=1) severe. In hospital mortality was 5.7% [n=6]. Freedom from ≥ moderate aortic insufficiency was 96.4% (95%CI: 92.5%-100%), 93.3% (95%CI: 87.6%-99.2%), and 91.0% (95%CI: 84.1%-98.5%) at 1-year, 2-year, and 3-year post-implantation, respectively. One HeartMate II patient experienced severe aortic insufficiency and was treated with a heart transplant. Three-year survival was 63.4% [95%CI: 52.9%-75.9%]. CONCLUSIONS: Central aortic valve repair may be an effective technique to mitigate aortic insufficiency in HeartMate II and 3. A larger cohort study with longer duration of follow up is warranted to further investigate the clinical impact.

Mechanical Circulatory Support During Surgical Revascularization for Ischemic Cardiomyopathy.

BACKGROUND: This study aimed to describe the use of perioperative mechanical circulatory support (MCS) and its impact on outcomes in patients with ischemic cardiomyopathy who were undergoing surgical revascularization. METHODS: Patients with an ejection fraction <35% who underwent isolated coronary artery bypass grafting (CABG) from 2015 to 2021 were identified (N = 378). Patients were divided into no MCS, preoperative MCS, and postoperative MCS groups on the basis of timing of MCS initiation, which included intraaortic balloon pump, extracorporeal membrane oxygenation, or Impella device (Abiomed) use. The primary outcome of interest was operative mortality. RESULTS: The median Society of Thoracic Surgeons Predicted Risk of Mortality was 2.4%. Sixty-six percent (n = 246) of patients had a previous myocardial infarction, and 61.8% of these patients were within 21 days of CABG. Twenty-one patients (5.6%) presented in cardiogenic shock. The preoperative MCS cohort consisted of 31 patients (8.2%) who underwent CABG a median of 2 days after MCS initiation. Thirty (7.9%) patients required postoperative MCS. Independent risk factors for requiring postoperative MCS included the preoperative ejection fraction (odds ratio, 0.93; P = .01 and the presence of preoperative MCS (odds ratio, 3.06; P = .02). Overall, operative mortality was 3.4%, and 3-year survival was 87.0%. Operative mortality in patients who did and did not receive preoperative MCS was 7.7% and 2.9% (P = .12) with no difference in long-term survival (P = .80), whereas patients requiring postoperative MCS had significantly increased operative (16.7%) and late mortality (63%; P <.01). CONCLUSIONS: CABG can be performed safely in patients with ischemic cardiomyopathy with selective use of perioperative MCS. Despite advanced disease severity, patients requiring preoperative MCS demonstrate acceptable short- and long-term survival. Patients requiring postoperative MCS have increased postoperative morbidity and mortality.

Left-Ventricular Unloading With Impella During Refractory Cardiac Arrest Treated With Extracorporeal Cardiopulmonary Resuscitation: A Systematic Review and Meta-Analysis.

OBJECTIVES: Extracorporeal cardiopulmonary resuscitation (ECPR) is the implementation of venoarterial extracorporeal membrane oxygenation (VA-ECMO) during refractory cardiac arrest. The role of left-ventricular (LV) unloading with Impella in addition to VA-ECMO ("ECMELLA") remains unclear during ECPR. This is the first systematic review and meta-analysis to characterize patients with ECPR receiving LV unloading and to compare in-hospital mortality between ECMELLA and VA-ECMO during ECPR. DATA SOURCES: Medline, Cochrane Central Register of Controlled Trials, Embase, and abstract websites of the three largest cardiology societies (American Heart Association, American College of Cardiology, and European Society of Cardiology). STUDY SELECTION: Observational studies with adult patients with refractory cardiac arrest receiving ECPR with ECMELLA or VA-ECMO until July 2023 according to the Preferred Reported Items for Systematic Reviews and Meta-Analysis checklist. DATA EXTRACTION: Patient and treatment characteristics and in-hospital mortality from 13 study records at 32 hospitals with a total of 1014 ECPR patients. Odds ratios (ORs) and 95% CI were computed with the Mantel-Haenszel test using a random-effects model. DATA SYNTHESIS: Seven hundred sixty-two patients (75.1%) received VA-ECMO and 252 (24.9%) ECMELLA. Compared with VA-ECMO, the ECMELLA group was comprised of more patients with initial shockable electrocardiogram rhythms (58.6% vs. 49.3%), acute myocardial infarctions (79.7% vs. 51.5%), and percutaneous coronary interventions (79.0% vs. 47.5%). VA-ECMO alone was more frequently used in pulmonary embolism (9.5% vs. 0.7%). Age, rate of out-of-hospital cardiac arrest, and low-flow times were similar between both groups. ECMELLA support was associated with reduced odds of mortality (OR, 0.53 [95% CI, 0.30-0.91]) and higher odds of good neurologic outcome (OR, 2.22 [95% CI, 1.17-4.22]) compared with VA-ECMO support alone. ECMELLA therapy was associated with numerically increased but not significantly higher complication rates. Primary results remained robust in multiple sensitivity analyses. CONCLUSIONS: ECMELLA support was predominantly used in patients with acute myocardial infarction and VA-ECMO for pulmonary embolism. ECMELLA support during ECPR might be associated with improved survival and neurologic outcome despite higher complication rates. However, indications and frequency of ECMELLA support varied strongly between institutions. Further scientific evidence is urgently required to elaborate standardized guidelines for the use of LV unloading during ECPR.

Aortic insufficiency following balloon aortic valvuloplasty does not impact long-term autograft durability after the Ross procedure.

OBJECTIVE: Preoperative aortic insufficiency (AI) is associated with inferior autograft durability after the Ross procedure. However, many patients with aortic stenosis (AS) undergo balloon aortic valvuloplasty (BAV) early and present with longstanding AI before Ross. We studied how BAV and subsequent valvular pathology impacts autograft durability. METHODS: Patients undergoing the Ross operation from 1993 to 2020 were identified. Those who underwent BAV before Ross were compared with patients who did not undergo BAV and underwent Ross for predominant AI (AI group) or AS (AS group). Those who underwent previous open surgical aortic valve intervention were excluded. Primary outcome of interest was autograft failure, defined as a composite of autograft reintervention or severe insufficiency. RESULTS: A total of 198 patients were included. Seventy-nine (39.9%) underwent BAV and subsequently underwent the Ross for predominant AI (45.6%) or AS (54.4%). Of patients who did not undergo BAV, 66 (33.3%) presented with predominant AI and 53 (26.8%) with AS. Freedom from autograft failure at 15 years was 90%, 92%, and 62% in BAV, AS, and AI groups, respectively. The AI group was at significantly increased risk of long-term autograft failure (hazard ratio, 5.6; P = .01), whereas the AS and BAV groups had similar, low risk (hazard ratio, 1.1; P = .91). Autograft durability was similar among patients who received BAV and presented with AS or AI before the Ross (P = .84). CONCLUSIONS: BAV before the Ross procedure is common in patients with AS. These patients have excellent long-term autograft durability regardless of preoperative valvular pathology and should strongly be considered for the Ross operation.

Liberal use of veno-arterial extracorporeal membrane oxygenation in combined sequential heart-liver transplantation.

Combined heart-liver transplantation (CHLT) is a rarely though increasingly performed procedure with evolving indications. Despite CHLT being performed at only a handful of centers, the use of intraoperative mechanical circulatory support to optimize hemodynamics and facilitate dual-organ transplantation varies widely. At our center, we liberally utilize veno-arterial extracorporeal membrane oxygenation (V-A ECMO) when a veno-venous shunt is anticipated to be insufficient in mitigating the hemodynamic perturbations associated with liver reperfusion. In this series, we describe our experience with V-A ECMO in sequential (heart-first) CHLT and demonstrate highly favorable outcomes with this strategy.

Long-term autograft dilation and durability after the Ross procedure are similar in infants, children, and adolescents with primary aortic stenosis.

BACKGROUND: Autograft durability and remodeling are thought to be superior in younger pediatric patients after the Ross operation. We sought to delineate the fate of autografts across the pediatric age spectrum in patients with primary aortic stenosis (AS). METHODS: We retrospectively reviewed patients age ≤18 years with primary AS who underwent the Ross operation between 1993 and 2020. Patients were categorized by age. The primary endpoint was autograft dimensional change, and secondary endpoints were severe neo-aortic insufficiency (AI) and autograft reintervention. RESULTS: A total of 119 patients underwent the Ross operation, including 37 (31.1%) in group I (age <18 months), 24 (20.2%) in group II (age 18 months-8 years), and 58 (48.7%) in group III (age 8-18 years). All groups exhibited similar annular growth rates within the first 5 postoperative years, followed by a collective decrease in annulus growth rates from year 5 to year 10. Group III experienced rapid sinus dilation in the first 5 years, followed by stabilization of the sinus z-score from year 5 to year 10, whereas groups I and II demonstrated stable sinus z-scores over 10 years. There were 4 early deaths (3.4%) and 2 late deaths (1.7%) at a median follow-up of 8.1 years (range, 0.01-26.3 years). At 15 years, the incidences of severe neo-AI (0.0 ± 0.0% vs 0.0 ± 0.0% vs 3.9 ± 3.9%; P = .52) and autograft reintervention (8.4 ± 6.0% vs 0.0 ± 0.0% vs 2.4 ± 2.4%; P = .47) were similar in the 3 groups. CONCLUSIONS: Age at the time of Ross operation for primary AS does not influence long-term autograft remodeling or durability. Other physiologic or technical factors are likely greater determinants of autograft fate.

Influence of preoperative aortic regurgitation on long-term autograft durability and dilatation in children and adolescents undergoing the Ross procedure.

OBJECTIVE: Primary aortic insufficiency (AI) is a risk factor for autograft reintervention in adults undergoing the Ross procedure. We sought to examine the influence of preoperative AI on autograft durability in children and adolescents. METHODS: From 1993 to 2020, 125 consecutive patients between ages 1 and 18 underwent a Ross procedure. The autograft was implanted using a full-root technique in 123 (98.4%) and included in a polyethelene terephthalate graft in 2 (1.6%). Patients with aortic stenosis (aortic stenosis group) (n = 85) were retrospectively compared with those with AI or mixed disease (AI group) (n = 40). Median length of follow-up was 8.2 years (interquartile range, 3.3-15.4 years). The primary end point was the incidence of severe AI or autograft reintervention. Secondary end points included changes in autograft dimensions analyzed using mixed-effect models. RESULTS: The incidence of severe AI or autograft reintervention was 39.0% ± 13.0% in the AI group and 8.8% ± 4.4% in the aortic stenosis group at 15 years (P = .02). Annulus z scores increased in both aortic stenosis and AI groups over time (P < .001). However, the annulus dilated at a faster rate in the AI group (absolute difference, 3.8 ± 2.0 vs 2.5 ± 1.7; P = .03). Sinus of Valsalva z scores increased in both groups as well (P < .001), but at similar rates over time (P = .11). CONCLUSIONS: Children and adolescents with AI undergoing the Ross procedure have higher rates of autograft failure. Patients with preoperative AI have more pronounced dilatation at the annulus. Akin to adults, a surgical aortic annulus stabilization technique that modulates growth is needed in children.

Acute right ventricular geometric change predicts outcomes in HeartMate 3 patients.

BACKGROUND: The physiological response of the right ventricle (RV) following left ventricular assist device (LVAD) implantation is difficult to predict. We aimed to investigate RV geometric and functional changes after LVAD insertion and their effects on clinical outcomes. METHODS: We retrospectively reviewed 188 patients who underwent HeartMate 3 implantation at our center between November 2014 and September 2021. The RV end-diastolic diameter (RVEDD) and RV end-diastolic area (RVEDA) were measured on preoperative and predischarge transthoracic echocardiography. The nonadapted group included patients with increased RVEDD and RVEDA at discharge. The composite outcome was defined as death or readmission due to worsening right heart failure. RESULTS: There were 82 patients (44%) who had a nonadapted and 106 patients (56%) who had an adapted RV. Preoperatively, the nonadapted group had smaller RVEDD (46 vs 49 mm, p < 0.001) and RVEDA (27 vs 31 cm2, p < 0.001). At discharge, the nonadapted group had larger RVEDD (51 vs 43 mm, p < 0.001) and RVEDA (33 vs 27 cm2, p < 0.001). Kaplan-Meier analysis demonstrated worse 3-year survival (77% vs 91%, p = 0.006) and freedom from composite outcome (58% vs 85%, p < 0.001) in the nonadapted group. A multivariable Cox proportional hazards model showed that nonadaption (hazard ratio [HR] 3.09, 95% confidence interval [CI] 1.29-7.40, p = 0.01) and age (HR 3.73, 95% CI 1.42-9.77, p = 0.007) were independent predictors of composite outcome. CONCLUSIONS: Acute RV dimensional changes after LVAD insertion may represent intrinsic RV function and may be a useful prognostic marker.

Strategies for the Surgical Management of Highly Aggressive Williams Syndrome Aortopathy: A Three Case Report.

Severe aortopathy in Williams syndrome can sometimes present with an initial ascending aortic pathology, followed in short order by more distal multilevel obstruction and recurrence requiring reintervention. In this series, an early, comprehensive surgical approach using a combination of various access and perfusion strategies yielded excellent long-term results.

Does lateral approach preserve the right ventricular function after HeartMate 3 insertion?

OBJECTIVES: Lateral thoracotomy (LT) approach may preserve the right ventricular (RV) function after left ventricular assist device (LVAD) implantation. This study evaluated the short- and long-term RV function using echocardiography after LVAD implantation via LT or median sternotomy (sternotomy). METHODS: The patients who underwent HeartMate 3 implantation were retrospectively reviewed. The RV function was assessed before and 1 month and 1 year after LVAD implantation. The primary and secondary outcomes were all-cause mortality and a composite of death or readmission due to RV failure, respectively. RESULTS: Of the 195 patients, 55 (28%) underwent LT and 140 (72%) underwent sternotomy. There were no significant differences in the preoperative RV geometry or function. One month after the LVAD implantation, the LT group had a smaller RV end-diastolic dimension [42 (29-48) vs 47 (42-52) mm; P = 0.003] and RV end-diastolic area [25 (21-28) vs 29 (24-36) cm2; P < 0.001] and a greater RV fractional area change [30 (25-34)% vs 28 (23-31)%; P = 0.04] and peak systolic tissue velocity [8 (7-9) vs 7 (6-8) cm/s; P = 0.01]. Twenty-four patients died and 46 met the composite end point. Kaplan-Meier curve analysis did not reveal significant differences between LT and sternotomy in the 2-year survival (93% vs 83%; log-rank test, P = 0.28) and adverse event rate (76% vs 71%; log-rank test, P = 0.65). CONCLUSIONS: LT approach yielded a better-preserved RV function at 1 month; however, there were no significant differences in the 2-year survival and adverse event rates.

Long-term outcomes of patients bridged to recovery with venoarterial extracorporeal life support.

OBJECTIVE: Our study examines the long-term outcomes of patients discharged from the hospital without heart replacement therapy (HRT) after recovery from cardiogenic shock using venoarterial extracorporeal life support (VA-ECLS). METHODS: We retrospectively reviewed 615 cardiogenic shock patients who recovered from VA-ECLS at our institution between January 2015 and July 2021. Of those, 166 patients (27.0%) who recovered from VA-ECLS without HRT were included in this study. Baseline characteristics, discharge labs, vitals, electrocardiograms and echocardiograms were assessed. Patients were contacted to determine vital status. The primary outcome was post-discharge mortality. RESULTS: Of 166 patients, 158 patients (95.2%) had post-discharge follow-up, with a median time of follow-up of 2 years (IQR: [1 year, 4 years]). At discharge, the median ejection fraction (EF) was 52.5% (IQR: [32.5, 57.5]). At discharge, 92 patients (56%) were prescribed ß-blockers, 28 (17%) were prescribed an ACE inhibitor, ARB or ARNI, and 50 (30%) were prescribed loop diuretics. Kaplan-Meier analysis showed a 1-year survival rate of 85.6% (95% CI: [80.1%, 91.2%]) and a 5-year survival rate of 60.6% (95% CI: [49.9%, 71.3%]). A Cox regression model demonstrated that a history of congestive heart failure (CHF) was strongly predictive of increased mortality hazard (HR = 1.929; p = 0.036), while neither discharge EF nor etiology of VA-ECLS were associated with increased post-discharge mortality. CONCLUSIONS: Patients discharged from the hospital after full myocardial recovery from VA-ECLS support without HRT should have close outpatient follow-up due to the risk of recurrent heart failure and increased mortality in these patients.

Preoperative left ventricular diastolic dimension index is associated with outcomes after HeartMate 3 implantation.

OBJECTIVES: We investigated the association between indexed left ventricular diastolic dimension and clinical outcomes after HeartMate 3 implantation. METHODS: We retrospectively reviewed patients implanted with the HeartMate 3 at our centre between November 2014 and September 2021. Left ventricular diastolic dimension was assessed via preoperative transthoracic echocardiography and left ventricular diastolic dimension index was calculated as left ventricular diastolic dimension/body surface area. The primary outcome was a composite of death or readmission due to right heart failure or stroke. The cut-off left ventricular diastolic dimension index value most closely associated with outcomes was determined by receiver-operating characteristic curve and restricted cubic spline analyses. RESULTS: Left ventricular diastolic dimension index measurements were available for 252 of 253 (99.6%) patients. Using a left ventricular diastolic dimension index cut-off value of 33.5 mm/m2, the cohort was divided: left ventricular diastolic dimension index ≤ (n = 131) or > (n = 121) 33.5 mm/m2. While there were no significant differences in age, INTERMACS level and preoperative haemodynamics between groups, patients with smaller left ventricular diastolic dimension index were more likely to have a larger body surface area (2.1 vs 1.9 m2, P < 0.001), ischaemic cardiomyopathy [64 (49%) vs 40 (33%), P = 0.01] and smaller left atrium volume index [40.5 (32.3-54.0) ml/m2 vs 54.0 (43.0-66.8) ml/m2, P < 0.001]. Smaller left ventricular diastolic dimension index patients had significantly worse survival (74% vs 88%, log-rank P = 0.009) and freedom from adverse events (55% vs 73%, log-rank P = 0.005) at 3-year follow-up. Smaller left ventricular diastolic dimension index was independently associated with the composite outcome (Hazard ratio 2.24, P = 0.002). CONCLUSIONS: Smaller preoperative left ventricular diastolic dimension index is associated with worse outcomes in patients undergoing HeartMate 3 implantation.

Use of the Inspiris valve in the native right ventricular outflow tract is associated with early prosthetic regurgitation.

OBJECTIVE: The Inspiris Resilia prosthesis (Edwards Lifesciences) has been increasingly used in the pulmonic position with limited performance data. We sought to investigate its durability as a surgical pulmonary valve replacement (PVR). METHODS: We retrospectively reviewed patients who underwent PVR or conduit replacement with an Inspiris or non-Inspiris valve/conduit from 2018 to 2022. The primary end point was freedom from a composite of at least moderate pulmonary regurgitation, pulmonary stenosis, or valve/conduit reintervention. Secondary end points were individual components of the composite outcome. To account for baseline differences, propensity matching identified 70 patient pairs. RESULTS: A total of 227 patients (median age: 19.3 years [interquartile range, 11.8-34.4]) underwent PVR or conduit replacement (Inspiris: n = 120 [52.9%], non-Inspiris: n = 107 [47.1%]). Median follow-up was 26.6 months [interquartile range, 12.4-41.1]. Among matched patients, 2-year freedom from valve failure was lower in the Inspiris group (53.5 ± 9.3% vs 78.5 ± 5.9%, P = .03), as was freedom from at least moderate pulmonary regurgitation (54.2 ± 9.6% vs 86.4 ± 4.9%, P < .01). There was no difference in 2-year freedom from at least moderate pulmonary stenosis (P = .61) or reintervention (P = .92). Inspiris durability was poorer when implanted in the native right ventricular outflow tract compared with as a conduit, with 18-month freedom from valve failure of 59.0 ± 9.5% versus 85.9 ± 9.5% (P = .03). CONCLUSIONS: Early durability of the Inspiris valve is poor when implanted in the native right ventricular outflow tract; its unique design may be incompatible with the compliant pulmonary root. Modified implantation techniques or alternative prostheses should be considered.

Combined heart and liver transplantation in a patient supported by left ventricular assist device (LVAD) with propionic acidemia.

Propionic acidemia (PA) is a rare inherited metabolic disease due to inborn errors of metabolism. PA results in the accumulation of abnormal organic acid metabolites in multiple systems, mainly the central nervous system and the heart. Cardiac complications include dilated cardiomyopathy (DCM) and carry a 40-50% increased mortality risk. Liver transplantation (LT) is required in PA patients when medical treatment fails and may prevent or slow down the cardiomyopathy progression. However, severe heart disease may be a serious contraindication to LT. We present a complicated case of a PA patient, supported with a Left Ventricular Assist Device, who underwent a heart and Liver transplant. PA patients are at increased risk for metabolic acidosis during surgery, with increased anion gap and hyperammonemia. A strict multi-disciplinary approach is needed to prevent and treat metabolic decompensation. The patient had a successful heart and liver transplant after a strict treatment protocol in the pre, intra, and post-operative periods. His case highlights the complexity of PA patients and the increased risk for metabolic decompensation during surgery and provides an insight into how to manage such complicated patients.

Continuous near-infrared reflectance spectroscopy monitoring to guide distal perfusion can minimize limb ischemia surgery for patients requiring femoral venoarterial extracorporeal life support.

BACKGROUND: Patients requiring femoral venoarterial (VA) extracorporeal life support (ECLS) are at risk of distal lower limb hypoperfusion and ischemia of the cannulated leg. In the present study, we evaluated the effect of using continuous noninvasive lower limb oximetry with near-infrared reflectance spectroscopy (NIRS) to detect tissue hypoxia and guide distal perfusion catheter (DPC) placement on the rates of leg ischemia requiring surgical intervention. METHODS: We performed a retrospective analysis of patients who had undergone femoral VA-ECLS at our institution from 2010 to 2014 (pre-NIRS era) and 2017 to 2021 (NIRS era). Patients who had undergone cannulation during the 2015 to 2016 transition era were excluded. The baseline characteristics, short-term outcomes, and ischemic complications requiring surgical intervention (eg, fasciotomy, thrombectomy, amputation, exploration) were compared across the two cohorts. RESULTS: Of the 490 patients included in the present study, 141 (28.8%) and 349 (71.2%) had undergone cannulation before and after the routine use of NIRS to direct DPC placement, respectively. The patients in the NIRS cohort had had a greater incidence of hyperlipidemia (53.7% vs 41.1%; P = .015) and hypertension (71.4% vs 60%; P = .020) at baseline, although they were less likely to have been supported with an intra-aortic balloon pump before ECLS cannulation (26.9% vs 37.6%; P = .026). These patients were also more likely to have experienced cardiac arrest (22.9% vs 7.8%; P ≤ .001) and a pulmonary cause (5.2% vs 0.7%; P = .04) as an indication for ECLS, with ECLS initiated less often for acute myocardial infarction (15.8% vs 34%; P ≤ .001). The patients in the NIRS cohort had had a smaller arterial cannula size (P ≤ .001) and a longer duration of ECLS support (5 vs 3.25 days; P ≤ .001) but significantly lower rates of surgical intervention for limb ischemia (2.6% vs 8.5%; P = .007) despite comparable rates of DPC placement (49.1% vs 44.7%; P = .427), with only two patients (1.1%) not identified by NIRS ultimately requiring surgical intervention. CONCLUSIONS: The use of a smaller arterial cannula (≤15F) and continuous NIRS monitoring to guide selective insertion of DPCs could be a valid and effective strategy associated with a reduced incidence of ischemic events requiring surgical intervention.