Tetralogy of Fallot in Low- and Middle-Income Countries.

Low- and middle-incomes countries (LMICs) have limited resources for the diagnosis and treatment of congenital heart diseases such as tetralogy of Fallot. This is in part due to lack of infrastructures, financial means, and expertise. As a result, patients undergo surgery much later than in high-income countries. This delay in treatment results in right ventricular dysfunction, cardiac arrhythmias, and poor psychomotor development-complications that are all related to chronic hypoxia. There are limited data and a few small studies of patients treated for tetralogy of Fallot in LMICs, and, therefore, the aim of this review is to analyse and summarize the surgical outcomes of this LMIC population.

Dans les pays à revenu faible ou intermédiaire (PRFI), les ressources sont limitées pour diagnostiquer et prendre en charge les cardiopathies congénitales comme la tétralogie de Fallot. Cette situation est attribuable en partie au manque d'infrastructures, de moyens financiers et d'expertise. Les patients subissent donc une correction chirurgicale beaucoup plus tard que dans les pays à revenu élevé. Les délais de traitement peuvent entraîner une dysfonction ventriculaire droite, une arythmie cardiaque et des problèmes de développement psychomoteur : des complications toutes liées à l'hypoxie chronique. Il existe des données limitées et quelques études de faible envergure sur des patients traités pour une tétralogie de Fallot dans les PRFI. L'objectif du présent article de synthèse est donc d'analyser et de résumer les issues des interventions chirurgicales dans les PRFI pour cette population.

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.

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.

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.

Learning Curve in Left Ventricular Assist Device Implantation: Low Volumes Do Not Equate Bad Outcomes.

INTRODUCTION: Most implantations of left ventricular assist devices (LVAD) are performed in low-volume centers. This study aimed to evaluate the procedural learning curve of HeartMate II (HM2) implantations by comparing outcomes between two time periods in a low-volume center. METHODS: All 51 consecutive patients undergoing HM2 implantation between January 2009 and December 2017 were reviewed and allocated into 2 groups: early-era group (from 2009 to 2014; n=25) and late-era group (from 2015 to 2017; n=26). The primary outcome was the 90-day mortality rate, and the secondary outcome was a composite of mortality, neurological event, reoperation for bleeding, need for temporary right ventricular assist device, and pump thrombosis at 90 days. Median follow-up time was 51 months (0-136). A cumulative sum (CUSUM) control analysis was used to establish a threshold of implantations that optimizes outcomes. RESULTS: Patients in the early era had a higher rate of diabetes, previous stroke, and inotrope support before HM2 implantation. The 90-day mortality rate was not significantly higher in the early era (24% vs. 15%, P=0.43), but the composite endpoint was significantly higher (76% vs. 42%, P=0.01). The CUSUM analysis found a threshold of 23 operations after which the composite endpoint was optimized. CONCLUSION: Patients undergoing HM2 implantation in a low-volume center have improving outcomes with number of cases and optimized results after a threshold of 23 cases. Significant changes in patient selection, surgical techniques, and patient management might lead to improved outcomes after LVAD implantation.

Learning Curve in Left Ventricular Assist Device Implantation: Low Volumes Do Not Equate Bad Outcomes

ABSTRACT Introduction: Most implantations of left ventricular assist devices (LVAD) are performed in low-volume centers. This study aimed to evaluate the procedural learning curve of HeartMate II (HM2) implantations by comparing outcomes between two time periods in a low-volume center. Methods: All 51 consecutive patients undergoing HM2 implantation between January 2009 and December 2017 were reviewed and allocated into 2 groups: early-era group (from 2009 to 2014; n=25) and late-era group (from 2015 to 2017; n=26). The primary outcome was the 90-day mortality rate, and the secondary outcome was a composite of mortality, neurological event, reoperation for bleeding, need for temporary right ventricular assist device, and pump thrombosis at 90 days. Median follow-up time was 51 months (0-136). A cumulative sum (CUSUM) control analysis was used to establish a threshold of implantations that optimizes outcomes. Results: Patients in the early era had a higher rate of diabetes, previous stroke, and inotrope support before HM2 implantation. The 90-day mortality rate was not significantly higher in the early era (24% vs. 15%, P=0.43), but the composite endpoint was significantly higher (76% vs. 42%, P=0.01). The CUSUM analysis found a threshold of 23 operations after which the composite endpoint was optimized. Conclusion: Patients undergoing HM2 implantation in a low-volume center have improving outcomes with number of cases and optimized results after a threshold of 23 cases. Significant changes in patient selection, surgical techniques, and patient management might lead to improved outcomes after LVAD implantation.

Outcomes and risk analysis after tricuspid valve surgery for non-Ebstein 2-ventricle congenital tricuspid valve diseases.

Objectives: Tricuspid valve (TV) surgery remains understudied and little data exist describing the surgical indications, outcomes, and prognostic factors for pediatric patients with non-Ebstein 2-ventricle congenital TV lesions. This study aims to describe early and late outcomes of pediatric patients with non-Ebstein congenital TV lesions undergoing isolated TV procedures at a single institution. Methods: All patients who underwent TV surgery for non-Ebstein congenital TV disease between 2006 and 2018 were included. Patients who had missing preoperative data, patients with single-ventricle physiology, congenitally corrected transposition of the great arteries, and patients undergoing TV intervention as part of repair of an atrioventricular canal defect were excluded. The primary end point was the occurrence of TV reintervention or TV regurgitation (TR) ≥ moderate. Results: A total of 85 patients were included. The tricuspid lesion was isolated TR in 80 (94.1%), isolated tricuspid stenosis in 3 (3.5%) and mixed disease in 2 (2.4%) patients. Median age at surgery was 33 years (interquartile range, 12-53 years). TV repair and TV replacement were performed in 66 (77.6%) and 19 (22.4%) patients, respectively. One (1.2%) patient underwent TV reoperation during the same admission. There was no in-hospital mortality. Median follow-up was 3.3 years (interquartile range, 0.1-4.7 years). The overall cumulative incidence of TV reintervention or TR deemed moderate or greater at 1, 3, and 5 years was 3% ± 2%, 11% ± 4%, and 20% ± 8%. In multivariable analysis, age younger than 12 years (P = .04) and mitral valve regurgitation deemed moderate or greater (P = .01) were independent risk factors for TV reintervention or recurrent TR deemed to be moderate or greater at last follow-up. Conclusions: TV surgery in patients with non-Ebstein congenital TV disease can be performed with good outcomes. TV reintervention or TR deemed moderate or greater occurred in 20% of patients on midterm follow-up. Patients younger than age 12 years are at higher risk for recurrent TR or TV reintervention, whereas preoperative MR deemed moderate or greater increases this risk, especially in patients older than age 12 years. There was no difference in outcomes between TV replacement and repair.

Surgical Management of Complex Aortic Valve Disease in Young Adults: Repair, Replacement, and Future Alternatives.

The ideal aortic valve substitute in young adults remains unknown. Prosthetic valves are associated with a suboptimal survival and carry a significant risk of valve-related complications in young patients, mainly reinterventions with tissue valves and, thromboembolic events and major bleeding with mechanical prostheses. The Ross procedure is the only substitute that restores a survival curve similar to that of a matched general population, and permits a normal life without functional limitations. Though the risk of reintervention is the Achilles' heel of this procedure, it is very low in patients with aortic stenosis and can be mitigated in patients with aortic regurgitation by tailored surgical techniques. Finally, the Ozaki procedure and the transcatheter aortic valve implantation are seen by many as future alternatives but lack evidence and long-term follow-up in this specific patient population.

Transcatheter Closure of Atrial and Ventricular Septal Defects: JACC Focus Seminar.

The field of congenital interventional cardiology has experienced tremendous growth in recent years. Beginning with the development of early devices for transcatheter closure of septal defects in the 1970s and 1980s, such technologies have evolved to become a mainstay of treatment for many atrial septal defects (ASDs) and ventricular septal defects (VSDs). Percutaneous device closure is now the preferred approach for the majority of secundum ASDs. It is also a viable treatment option for selected VSDs, though limitations still exist. In this review, the authors describe the current state of transcatheter closure of ASDs and VSDs in children and adults, including patient selection, procedural approach, and outcomes. Potential areas for future evolution and innovation are also discussed.

Interventions for Congenital Atrioventricular Valve Dysfunction: JACC Focus Seminar.

Innovation and creativity have led to tremendous advancements in the care and management of patients with congenital heart disease (CHD) that have resulted in considerably increased survival. Catheter-based interventions have contributed significantly to these advancements. However, catheter-based interventions for congenital lesions of the atrioventricular (AV) valves have been limited in scope and effectiveness mainly because of patient size and anatomical challenges. Thus, surgical repair and replacement for congenital AV valve lesions have remained the preferred therapy. However, the ongoing transcatheter heart valve revolution has led to techniques and technologies that are changing the landscape, particularly for adult CHD patients. Many devices for AV valve repair and replacement are being studied in adult patients without CHD, and translation of select practices to CHD patients has begun, with many more to come. Transcatheter AV valve interventions represent exciting opportunities for the growing numbers of adult CHD patients.

Transcatheter Cardiac Interventions in the Newborn: JACC Focus Seminar.

For neonates with critical congenital heart disease requiring intervention, transcatheter approaches for many conditions have been established over the past decades. These interventions may serve to stabilize or palliate to surgical next steps or effectively primarily treat the condition. Many transcatheter interventions have evidence-based records of effectiveness and safety, which have led to widespread acceptance as first-line therapies. Other techniques continue to innovatively push the envelope and challenge the optimal strategies for high-risk neonates with right ventricular outflow tract obstruction or ductal-dependent pulmonary blood flow. In this review, the most commonly performed neonatal transcatheter interventions will be described to illustrate the current state of the field and highlight areas of future opportunity.

Pulmonary homograft dysfunction after the Ross procedure using decellularized homografts-a multicenter study.

OBJECTIVES: Pulmonary homograft dysfunction is a limitation after the Ross procedure. Decellularized pulmonary homografts can potentially mitigate this complication. The aim of this study was to examine the incidence, predictors, progression, and morphology of pulmonary homograft dysfunction using data from the Canadian Ross Registry. METHODS: From 2011 to 2019, 466 consecutive patients (mean age: 47 ± 12 years, 73% male) underwent a Ross procedure using a decellularized cryopreserved pulmonary homograft (SynerGraft SG; CryoKife, Kennesaw, Ga). Pulmonary homograft dysfunction was defined as any of the following: peak pulmonary gradient ≥30 mm Hg, pulmonary regurgitation >2, or pulmonary homograft reintervention. Patients meeting ≥1 of these criteria (n = 30) were compared with the rest of the cohort (n = 436). Median follow-up is 2.2 years (maximum = 8.5 years) and 99% complete (1176 patient-years). RESULTS: The cumulative incidence of pulmonary homograft dysfunction was 11 ± 2% at 6 years. Pulmonary homograft stenosis was the most frequent presentation (n = 28 patients, 93%). Morphologically, stenosis occurred most often along the conduit (59%). Overall, 4 patients required homograft reintervention. At 6 years, the cumulative incidence of homograft reintervention was 3 ± 1%. The instantaneous risk was greatest in the first year after surgery (3.5%/year) and decreased to <1%/year thereafter. Patient age <45 years was the only independent risk factor associated with pulmonary homograft dysfunction (hazard ratio, 3.1, 95% confidence interval, 1.1-8.6, P = .03). CONCLUSIONS: The use of decellularized cryopreserved pulmonary homografts results in a low incidence of dysfunction and reintervention after the Ross procedure. The risk is greater in the first postoperative year. Younger age is the only independent risk factor for pulmonary homograft dysfunction.

Autograft remodeling after the Ross procedure by cardiovascular magnetic resonance imaging: Aortic stenosis versus insufficiency.

BACKGROUND: Studies suggest that patients undergoing the Ross procedure for aortic insufficiency are at greater risk of autograft dilatation than those with aortic stenosis. By using a tailored Ross technique to mitigate autograft dilatation in patients with aortic insufficiency, we aimed to compare the biomechanical and morphologic remodeling of the autograft at 1 year between patients with aortic insufficiency and patients with aortic stenosis. METHODS: A total of 210 patients underwent a Ross procedure (2011-2016). Of those, 86 patients (mean age 43 ± 13 years; 32% were female) completed preoperative and postoperative cardiovascular magnetic resonance imaging. A total of 71 studies were suitable for analysis: 41 patients with aortic stenosis and 30 patients with aortic insufficiency. Nine healthy adults were used as controls. Autograft root dimensions, individual sinus volumes, and distensibility were measured using cardiovascular magnetic resonance. RESULTS: At 1 year, there was no difference in autograft root dimensions between patients with aortic stenosis (mean annulus 25.1 ± 3.1 mm and sinus diameters 35 ± 4.1 mm) and patients with aortic insufficiency (26.6 ± 3 mm and 37.1 ± 3.5 mm; P = .12 and .06, respectively). Relative sinus of Valsalva volumes were symmetrical in the aortic stenosis (right 34.8% ± 4%, left 33.7% ± 3.5%, noncoronary 31.4% ± 3.2%) and aortic insufficiency groups (34.8% ± 3.9%, 33.8% ± 2.8%, 31.3% ± 3.7%, P = .85, .92, and .82), and similar to those of healthy adults. Aortic root distensibility was reduced in both groups compared with healthy adults (P = .003), but was similar between aortic stenosis (3.12 ± 1.58 × 10-3 mm Hg-1) and aortic insufficiency (3.04 ± 1.15 × 10-3 mm Hg-1; P = .9). CONCLUSIONS: Using a tailored technique, there were no differences in the morphologic or biomechanical remodeling of the autograft root 1 year after the Ross procedure between patients with aortic stenosis and patients with aortic insufficiency. However, autograft roots are stiffer than native aortic roots.

Indications, Limitations, and Development of Tricuspid Valve Interventions in Adults.

Tricuspid regurgitation is associated with excessive mortality and poor outcomes regardless of the cause and associated comorbidities. Despite this clear association with mortality, tricuspid valve diseases remain undertreated. Tricuspid valve surgery, either repair or replacement, has shown little detectable survival benefit. Transcatheter tricuspid valve interventions have emerged as a less invasive approach to tricuspid valve diseases. They can be categorised into coaptation devices, annuloplasty devices, transcatheter tricuspid valve replacement, heterotopic caval valve implantation, and tricuspid valve-in-valve. Despite the late referral and the patient's profile, results remain fairly acceptable at least in the short term, with good procedural device success, excellent safety profile, and sustained reduction of tricuspid regurgitation for up to 1 year. Because results are limited to the mid-term, transcatheter tricuspid valve intervention durability will need to be established before broader adoption of these technologies.

The Ross procedure in patients older than 50: A sensible proposition?

BACKGROUND: The Ross procedure offers several advantages in nonelderly adults; however, the optimal age cutoff remains undetermined. The aim of this study was to compare the safety and mid-term outcomes after the Ross procedure in adult patients age ≤50 years and those age >50 years. METHODS: Between 2011 and 2019, 497 consecutive patients (mean age, 47 ± 12 years; 73% male) underwent a Ross procedure in 5 Canadian centers and were followed prospectively. Of these patients, 232 (47%) were age >50 years (mean, 57 ± 4 years) and 265 (53%) were age ≤50 years (mean, 38 ± 10 years). Early and mid-term outcomes were compared between the 2 groups. RESULTS: Patients age >50 years had more comorbidities: diabetes (14% vs 4%; P < .01), chronic obstructive pulmonary disease (8% vs 2%; P < .01), and coronary artery disease (17% vs 3%; P < .01). In contrast, patients age ≤50 years had more redo surgeries (24% vs 8%; P < .01), pure aortic regurgitation (21% vs 6%; P < .01) and unicuspid valves (42% vs 9%; P < .01). In-hospital mortality was similar in the 2 groups (0.4% vs 0.4%; P = .99). There were no between-group differences in perioperative complications. The cumulative incidence of reintervention was similar at 6 years (>50 years: 0.7 ± 0.7%; ≤50 years: 4 ± 2%; P = .12). Survival at 6 years was 98 ± 2% in patient age >50 years versus 96 ± 2% in those age ≤50 years (P = .43), similar to the age- and sex-matched general population. CONCLUSIONS: The Ross procedure is a safe operation in patients age >50 years and provides excellent hemodynamics, stable valve function, and restored survival at mid-term follow-up. In expert centers, it should be considered as an alternative in selected patients age >50 years.

Early and Long-Term Outcomes after Direct Bridge-to-Transplantation with Extracorporeal Membrane Oxygenation.

BACKGROUND: Veno-arterial extracorporeal membrane oxygenation (VA-ECMO) as direct bridge-to-transplantation (dBTT) remains controversial. We compared the short- and long-term outcomes of adult patients undergoing urgent heart transplantation (HT) with (dBTT) and without (non-BTT) VA-ECMO support at the time of HT. METHODS: Adults who underwent urgent HT in two institutions were assessed (N = 133; dBTT: N = 34 and non-BTT: N = 99). Patient characteristics, donor characteristics, in-hospital outcomes, and overall survival were compared. Mean follow up was 77±38 months and was 100% complete. Mortality predictors were identified using univariate and multivariate analyses. RESULTS: Before HT, patients with dBTT had higher rates of ischemic cardiomyopathy, acute kidney injury, liver failure, respiratory failure, and longer graft ischemia times. More patients in the dBTT group had complications, such as requiring VA-ECMO postoperatively (dBTT=50% vs. non-BTT=20%, P < 0.01). Hospital deaths (dBTT=23% vs. non-BTT=19%, P = 0.58), one-year (74% vs. 80%) and five-year survival (62% vs. 75%, P = 0.74 for overall survival) were not significantly different. The MELD-XI score and previous cardiac surgery were independent predictors of hospital mortality. CONCLUSION: Direct bridge-to-transplantation in patients on VA-ECMO support was not associated with worse long-term outcomes compared with non-VA-ECMO urgent HT, especially in recipients without any associated organ failure and a low MELD-XI score before HT.