Young infants with symptomatic tetralogy of Fallot: Shunt or primary repair?

Objectives: The optimal treatment strategy for symptomatic young infants with tetralogy of Fallot (TOF) is unclear. We sought to compare the outcomes of staged repair (SR) (shunt palliation followed by second-stage complete repair) versus primary repair (PR) at 2 institutions that have exclusively adopted each strategy. Methods: We performed propensity score-matched comparison of 143 infants under 4 months of age who underwent shunt palliation at one institution between 1993 and 2021 with 122 infants who underwent PR between 2004 and 2018 at another institution. The primary outcome was mortality. Secondary outcomes were postoperative complications, durations of perioperative support and hospital stays, and reinterventions. Median follow-up was 8.3 years (interquartile range, 8.1-13.4 years). Results: After the initial procedure, hospital mortality (shunt, 2.8% vs PR, 2.5%; P = .86) and 10-year survival (shunt, 95%; 95% confidence interval [CI], 90%-98% vs PR, 90%; 95% CI, 81%-95%; P = .65) were similar. The SR group had a greater risk of early reinterventions but similar rates of late reinterventions. Propensity score matching yielded 57 well-balanced pairs. In the matched cohort, the SR group had similar freedom from reintervention (55%; 95% CI, 39%-68% vs 59%; 95% CI, 43%-71%; P = .85) and greater survival (98%; 95% CI, 88%-99.8% vs 85%; 95% CI, 69%-93%; P = .02) at 10 years, as the result of more noncardiac-related mortalities in the PR group. Conclusions: In symptomatic young infants with TOF operated at 2 institutions with exclusive treatment protocols, the SR strategy was associated with similar cardiac-related mortality and reinterventions as the PR strategy at medium-term follow-up.

Outcomes of extracardiac Fontan operation: A single institution experience with 398 patients.

OBJECTIVE: The study objective was to evaluate the outcomes of the extracardiac Fontan operation at a single institution. METHODS: We conducted a retrospective study of 398 patients from a single institution who underwent their initial extracardiac Fontan operation between 1997 and 2020. We determined the incidence of and risk factors for failure of the Fontan circulation, which includes death, Fontan takedown, heart transplantation, protein-losing enteropathy, plastic bronchitis, and functional status at the last follow-up. RESULTS: The median follow-up time was 10.3 years (interquartile range, 6.4-14.6). The overall survival was 96% and 86% at 10 and 20 years after extracardiac Fontan operation, respectively. There were 6 early deaths (6/398, 1.5%) and 15 late deaths (15/398, 3.8%). Forty-nine patients (12.5%) developed failure of the Fontan circulation. Freedom from the failure of Fontan circulation was 88% at 10 years and 76% at 20 years. Risk factors for failure of the Fontan circulation were right ventricular dominance (hazard ratio, 4.7; P < .001; 95% CI, 2.1-10.5), aortic atresia (hazard ratio, 5.5; P < .001; 95% CI, 2.3-12.8), and elevated mean pulmonary artery pressure (hazard ratio, 2.3; P = .002; 95% CI, 1.2-6.7). CONCLUSIONS: Rates of failure of the Fontan circulation are low after the contemporary extracardiac Fontan operation. Risk factors for failure of the extracardiac Fontan circulation include right ventricular dominance, aortic atresia, and elevated pulmonary artery pressures.

Heart Transplantation after Univentricular Palliation: Improved Outcomes and Increased Complexity.

AIM: Heart transplantation (HT) in patients with failing univentricular circulation is often challenging. This is compounded by the ever-increasing number of patients with prior Norwood-type reconstruction of the aorta, large aortic root, and often dense adhesions from multiple prior operations. We aimed to elucidate differences in outcomes of HT in patients with prior univentricular palliations, with and without prior Norwood-type aortic arch reconstruction (ArchRec). METHODS: All patients who underwent HT for failed univentricular palliation during the 1990-2022 period were included in the study. RESULTS: Of 45 patients, 18 had undergone ArchRec. Hospital mortality improved in the recent era (17.4% before 2006 vs 0% after 2006; p=0.11), despite a higher proportion of patients with ArchRec (17.4% before 2006 vs 60.8% after 2006, p=0.002). Patients with ArchRec had a higher number of prior cardiac surgeries (4.1±1.5 vs 3.2±1.3, p=0.04), longer cardiopulmonary bypass time (320±23 vs 242±21 min, p=0.02), more concomitant arch reconstruction (33.3% vs 0%, p=0.02), greater need for post-HT extracorporeal membrane oxygenation (33.3% vs 3.7%; p=0.01) and longer hospital stay (37.1±30.5 days vs 23.6±11.8 days, p=0.04). Freedom from death or retransplantation for all patients was 91%, 73%, 67%, and 53% at 1, 5, 10, and 15-years, respectively. Prior ArchRec, Fontan procedure, and earlier eras were not risk factors for death. CONCLUSIONS: The outcomes of HT after univentricular palliation have improved in recent times and low operative mortality can be achieved. Despite increased complexity, good similar outcomes can be achieved in patients with and without prior arch reconstruction regardless of the palliation stage.

Long-Term Outcomes of Mechanical Aortic Valve Replacement in Children.

When the options of aortic valve repair or the Ross procedure are not feasible or have been exhausted, mechanical aortic valve replacement (AVR) may provide a reliable and structurally durable alternative, but with the limitations of long-term anticoagulation, thrombosis risk and lack of valve growth potential. In this article, we review the longitudinal outcomes of mechanical AVR in children in our institution and compare them to those recently reported by others. From 1978 to 2020, 62 patients underwent mechanical AVR at a median age of 12.4 years (interquartile range (IQR): 8.6-16.8 years). The most common underlying diagnoses were: conotruncal anomalies (40%, 25/62), congenital aortic stenosis (16%, 10/62), rheumatic valve disease (16%, 10/62), connective tissue disease (8.1%, 5/62) and infective endocarditis (6.5%, 4/62). Thirty-two patients (52%, 32/62) had at least 1 prior aortic valve surgery prior to mechanical AVR. Early death was 3.2% (2/62). Median follow-up was 14.4 years (IQR: 8.4-28.2 years). Kaplan-Meier survival was 96.8%, 91.9%, 86.3%, and 81.9% at 1, 5, 10, and 20 years. On competing risk analysis, the proportion of patients alive without aortic valve reoperation at 1, 5, 10, and 20 years was 95.2%, 87.0%, 75.5% and 55.4%, respectively, while the proportion of patients that had aortic valve reoperation (with death as a competing event) at 1, 5, 10, and 20 years was 1.6%, 4.9%, 12.8%, and 28.5%, respectively. In conclusion, when the options of aortic valve repair or the Ross procedure are not feasible in children, mechanical AVR is an alternative, yet the long-term rates of mortality and need for aortic valve reoperation are of concern.

Clinical assessment can be lifesaving: Salvage of catastrophic cerebral ischemia by conversion to central extracorporeal membrane oxygenation.

Peripheral veno-artertial extracorporeal membrane oxygenation (VA-ECMO) is commonly used in the paediatric population for intractable respiratory and cardiac failure. One of the devastating complications of VA-ECMO is severe brain damage due to ischemia or haemorrhage. We describe a case of peripheral cervical VA-ECMO complicated by evolving right cerebral ischemia which was rescued with rapid conversion from peripheral to central VA-ECMO support. Notably, the patient had a complete circle of Willis. Following conversion, we observed complete resolution of neurological symptoms with full functional recovery.

Valved Sano conduit improves immediate outcomes following Norwood operation compared with nonvalved Sano conduit.

OBJECTIVE: Use of a valved Sano during the Norwood procedure has been reported previously, but its impact on clinical outcomes needs to be further elucidated. We assessed the impact of the valved Sano compared with the nonvalved Sano after the Norwood procedure in patients with hypoplastic left heart syndrome. METHODS: We retrospectively reviewed 25 consecutive neonates with hypoplastic left heart syndrome who underwent a Norwood procedure with a valved Sano conduit using a femoral venous homograft and 25 consecutive neonates with hypoplastic left heart syndrome who underwent a Norwood procedure with a nonvalved Sano conduit between 2013 and 2022. Primary outcomes were end-organ function postoperatively and ventricular function over time. Secondary outcomes were cardiac events, all-cause mortality, and Sano and pulmonary artery reinterventions at discharge, interstage, and pre-Glenn time points. RESULTS: Postoperatively, the valved Sano group had significantly lower peak and postoperative day 1 lactate levels (P = .033 and P = .025, respectively), shorter time to diuresis (P = .043), and shorter time to enteral feeds (P = .038). The valved Sano group had significantly fewer pulmonary artery reinterventions until the Glenn operation (n = 1 vs 8; P = .044). The valved Sano group showed significant improvement in ventricular function from the immediate postoperative period to discharge (P < .001). From preoperative to pre-Glenn time points, analysis of ventricular function showed sustained ventricular function within the valved Sano group, but a significant reduction of ventricular function in the nonvalved Sano group (P = .003). Pre-Glenn echocardiograms showed competent conduit valves in two-thirds of the valved Sano group (n = 16; 67%). CONCLUSIONS: The valved Sano is associated with improved multi-organ recovery postoperatively, better ventricular function recovery, and fewer pulmonary artery reinterventions until the Glenn procedure.

Bex-Nikaidoh operation and the impact of double root translocation on outcomes.

OBJECTIVES: The Bex-Nikaidoh operation can effectively relieve left ventricular outflow tract obstruction. However, if a conduit is used for right ventricular outflow tract reconstruction, a late reoperation can be anticipated. We examined the impact of double root translocation on outcomes. METHODS: We performed a retrospective single-centre study of patients who underwent aortic root translocation between 2006 and 2019. RESULTS: Aortic root translocation was performed in 23 patients at a median age of 1.6 years [interquartile range (IQR) 0.9-2.5]. Concomitant repairs were done in 52.2% of patients (12/23) including the Senning atrial switch in 34.8% (8/23). The right ventricular outflow tract was reconstructed with valved conduits in 39.1% (9/23), direct anastomoses in 4.35% (1/23) and pulmonary autografts in 56.5% of patients (13/23). Aortic cross-clamp time was significantly longer in patients with double root translocation [308 min (IQR 270-259) vs 209 min (IQR 179-281), P = 0.02]; 2 patients in this group required temporary mechanical circulatory support. There were no early deaths. Median follow-up time was 7.5 years (IQR 3.3-10.5). The estimated 10-year survival was 90% [95% confidence interval (CI): 47.3%, 98.5%]. There was no recurrent left ventricular outflow tract obstruction. Freedom from any reoperation was 64.2% (95% CI: 40.8%, 80.3%) at 3 years and 44.5% (95% CI: 21.2%, 65.5%) at 6 years. The main indication for late reoperation was conduit degeneration. Freedom from a right ventricular outflow tract reoperation was significantly higher, and the number of reoperations per patient was lower when a double root translocation had been performed (P = 0.03). CONCLUSIONS: The Bex-Nikaidoh operation effectively relieved left ventricular outflow tract obstruction. A double root translocation further increased procedural complexity but was associated with better mid-term freedom from a right ventricular outflow tract reoperation. It should be considered in suitable patients.

Outcomes of neo-aortic valve and root surgery late after arterial switch operation.

BACKGROUND: Neo-aortic root dilatation and valve regurgitation are emerging problems late after arterial switch operation (ASO). We sought to evaluate the prevalence and outcomes of neo-aortic root or valve reoperation after ASO. METHODS: All patients with biventricular circulation who underwent an ASO between 1983 and 2015 were included at a single institution. RESULTS: In our cohort of 782 late ASO survivors, the median duration of follow-up was 18.1 years (interquartile range [IQR], 11.3-25.6 years). During follow-up, 47 patients (6.0%) underwent 60 reoperations on the neo-aortic valve/root. The first neo-aortic valve/root reoperation occurred at a median of 15.2 years (IQR, 7.8-18.4 years) after ASO. Operations included mechanical Bentall (31.9%; n = 15), aortic valve repair (25.5%; n = 12), mechanical aortic valve replacement (AVR) (21.3%; n = 10), valve-sparing root replacement (19.1%; n = 9), and the Ross procedure (2.1%; n = 1). There was 1 late death (2.1%). Multivariable predictors of neo-aortic valve/root reoperation were bicuspid valve (hazard ratio [HR], 4.8; 95% confidence interval [CI], 2.1-10.7; P < .001), Taussig-Bing anomaly (HR, 3.0; 95% CI, 1.2-7.4; P < .02), previous pulmonary artery band (HR, 2.8; 95% CI, 1.2-6.3; P < .01) and left ventricular outflow tract obstruction before ASO (HR, 2.4; 95% CI, 1.0-5.8; P < .04). Freedom from neo-aortic valve or root reoperation was 98.0% (95% CI, 96.7%-98.8%) at 10 years, 93.3% (95% CI, 90.8%-95.2%) at 20 years, and 88.5% (95% CI, 84.1%-91.8%) at 30 years after ASO. Among the 47 patients who underwent neo-aortic reoperation, freedom from AVR was 82.3% (95% CI, 67.7%-90.7%) at 10 years, 58.0% (95% CI, 41.8%-71.2%) at 20 years, and 43.2% (95% CI, 27.0%-58.3%) at 25 years after ASO. CONCLUSIONS: The need for neo-aortic valve or root reoperation surpasses 10% by 30 years post-ASO. Evolving understanding of the mechanisms of neo-aortic valve insufficiency and techniques of neo-aortic valve repair may decrease the need for AVR.

Long-term outcomes of tetralogy of Fallot repair: A 30-year experience with 960 patients.

OBJECTIVE: This study evaluates the long-term results of tetralogy of Fallot repair and assesses the risk factors for adverse outcomes. METHODS: This retrospective study included 960 patients who underwent transatrial transpulmonary tetralogy of Fallot repair between 1990 and 2020. RESULTS: A transannular patch was placed in 722 patients, and pulmonary valve preservation was achieved in 233 patients. The median age at tetralogy of Fallot repair was 9.4 (interquartile range, 6.2-14.2) months. The median follow-up duration was 10.6 (interquartile range, 5.4-16.3) years. There were 8 early deaths (0.8%) and 20 late deaths (2.1%). Genetic syndrome and pulmonary valve annulus Z score less than -3 were risk factors for mortality. The survival was 97.7% (95% confidence interval, 96.4-98.5) and 94.5% (95% confidence interval, 90.9-96.7) at 10 and 30 years, respectively. Freedom from any reoperation was 86.4% (95% confidence interval, 83.6-88.7) and 65.4% (95% confidence interval, 59.8-70.4) at 10 and 20 years, respectively. Postoperative right ventricular outflow tract peak gradient of 25 mm Hg or greater correlated with reoperation. Propensity score-matched analysis demonstrated that freedom from pulmonary valve replacement at 15 years was higher in the pulmonary valve preservation group compared with the transannular patch group (98.2% vs 78.4%, P = .004). Freedom from reoperation for right ventricular outflow tract obstruction at 15 years was lower in the pulmonary valve preservation group compared with the transannular patch group (P = .006). CONCLUSIONS: The long-term outcomes of tetralogy of Fallot repair are excellent. A postoperative right ventricular outflow tract peak gradient less than 25 mm Hg appears to be optimal to prevent reoperation. If the pulmonary valve size is suitable, pulmonary valve preservation reduces the risk of pulmonary valve replacement, yet increases the reoperation rate for right ventricular outflow tract obstruction.

T-Remodeling of the Pulmonary Artery Bifurcation for Pulmonary Artery Origin Stenosis.

BACKGROUND: Various surgical techniques are utilized for reconstructing hypoplastic pulmonary arteries (PAs) in patients with conotruncal anomalies and at times, may be susceptible to restenosis and reoperation. We reviewed our experience with a simple technique of T-shaped remodeling of the PA bifurcation. METHODS: Between 2005 and 2019, 31 patients underwent T-remodeling of central PAs by a single cardiac surgeon. The PA bifurcation was opened cranially, and the opening was augmented with an oval-shaped patch effectively transforming the V-shaped bifurcation into a T-shaped bifurcation. Both origins of the PAs were enlarged, even in the instance of single PA origin stenosis. RESULTS: Median age at time of T-remodeling was 17 months (range: 7 weeks to 14 years). The following cardiac morphologies were observed: tetralogy of Fallot (n = 12, 39%), pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collateral arteries (n = 8, 26%), truncus arteriosus (n = 6, 19%), pulmonary atresia with VSD (n = 3, 9.7%), and transposition of the great arteries (n = 2, 6.5%). Thirteen patients (42%) had previous central shunt, and eight patients (26%) had previous modified Blalock-Taussig shunt. There were no operative mortalities. Immediately after T-remodeling, echocardiographic estimates of right ventricle to PA gradient decreased from 42 [interquartile range 28-58] mm Hg to 20 [12-36] mm Hg (P = .03). Freedom from reoperation on the PA bifurcation for the entire cohort was 100% at one year, 88% (95% CI 68%-96%) at five years and 82% (57%-93%) at ten years. CONCLUSIONS: T-remodeling for PA origin stenosis is a safe procedure with excellent freedom from reoperation that is easily reproducible and applicable to patients with all cardiac morphologies.

Aortic valve repair versus the Ross procedure in children.

BACKGROUND: Aortic valve repair and the Ross procedure are widely used in children; however, it is unclear which provides the best outcomes. METHODS: Patients who underwent primary aortic valve surgery from 1980 to 2018 were included. Propensity score matching was performed to adjust for baseline differences. RESULTS: Of 415 children, 82.7% (343/415) underwent repair and 17.3% (72/415) underwent the Ross procedure. At 15 years, survival was higher for aortic valve repair (93.9% ± 1.8% vs 80.9% ± 6.4%, P = .04); freedom from reoperation (45.7% ± 4.9% vs 48.5% ± 9.0%, P = .29) did not differ, and freedom from aortic valve reoperation was higher in the Ross procedure group (45.7% ± 4.9% vs 70.7% ± 8.0%, P < .001). When analyzed by quality of repair, acceptable repair provided the highest survival (P = .01). Acceptable repair and the Ross procedure had similar freedom from reoperation at 15 years, whereas suboptimal repair performed worse (acceptable: 54.9% ± 6.7%; Ross procedure: 48.5% ± 9.0%; suboptimal: 27.0% ± 7.7%, P < .001). Acceptable repair and the Ross procedure had similar freedom from aortic valve reoperation at 15 years, whereas suboptimal repair showed worse results (acceptable: 54.9 ± 6.7; Ross procedure: 70.7% ± 8.0%; suboptimal: 27.0% ± 7.7%, P < .001). Propensity score matching paired 66 patients who underwent the Ross procedure with 198 patients who underwent repair. At 15 years, repair was associated with better survival (98.0% ± 1.2% vs 78.5% ± 7.2%, P = .03), whereas freedom from reoperation was similar (42.6% ± 7.6% vs 50.7% ± 9.8%, P = .50). However, the Ross procedure was associated with higher freedom from aortic valve reoperation (42.6% ± 7.6% vs 72.3% ± 8.5%, P = .002). CONCLUSIONS: Primary aortic valve repair was associated with better survival than the Ross procedure, whereas overall freedom from reoperation was similar. When an acceptable intraoperative result was achieved, outcomes of repair were favorable. However, when the intraoperative result of repair was suboptimal, the Ross procedure showed better results.

The Ross Procedure in Children: Defining the Optimal Age.

BACKGROUND: It has been proposed that delaying the Ross procedure to later in childhood, allowing autograft stabilisation and placement of a larger pulmonary conduit, may improve outcomes. However, the effect of age at the time of Ross procedure on outcomes remains unclear. METHODS: All patients who underwent the Ross procedure between 1995 and 2018 were included in the study. Patients were divided into four groups: infants, age 1 to 5 years, age 5 to 10 years and age 10 to 18 years. RESULTS: A total of 140 patients underwent the Ross procedure in the study period. Early mortality was 23.3% (7/30) for infants compared to 0% for older children (p<0.001). Survival at 15 years was significantly lower in infants (76.3%±9.9%), compared to children aged 1 to 5 years (90.9%±20.1%), 5 to 10 years (94%±13.3%), and 10 to 18 years (86.7%±10.0%), p=0.01. Freedom from autograft reoperation at 15 years was significantly lower in infants (58.4%±16.2%), compared to children aged 1 to 5 years (77.1%±14.9%), 5 to 10 years (84.2%±6.0%) and 10 to 18 years (87.8%±9.0%), p=0.01. Overall freedom from reoperation at 15 years was 13.0%±6.0% for infants, 24.2%±9.0% for children aged 1 to 5 years, 46.7%±15.8% for children aged 5 to 10 years, and 78.4%±10.4%, p<0.001. CONCLUSIONS: The Ross procedure performed after 10 years of age appears to be associated with improved freedom from reoperation, primarily due to a reduction in reoperation on the pulmonary conduit.

Cardio-cutaneous fistulae-beware the late sternal wound lesion.

Cardio-cutaneous fistula is a very rare complication of cardiac surgery, and the optimal management strategy is unclear. We present a case of a right ventricle-to-pulmonary artery conduit (RV-PA) forming a cutaneous fistulate that was successfully surgically repaired. A 43-year-old male presented for an elective RV-PA conduit replacement with a cutaneous skin lesion and associated sub-sternal collection. The patient underwent redo-sternotomy for the previous surgical replacement RV-PA conduit, of pulmonary atresia, ventricular septal defect, and ligation of main aorto-pulmonary collateral arteries in childhood, with the subsequent upgrade of the RV-PA conduit using pulmonary homograft. Upon entry into the thoracic cavity, it was clear that there was a direct fistula formed from the RV-PA conduit that was responsible for the skin lesion and hence a direct communication to the PA. We discuss the surgical method and surrounding discussions regarding Cardio-cutaneous fistula in a successful surgical repair when the pathology is difficult to truly identify preoperatively.

Aortic Valve Surgery in Children With Infective Endocarditis.

There is limited data on the outcomes of children who undergo surgery for aortic valve infective endocarditis (IE), and the optimal surgical approach remains controversial. We investigated the long-term outcomes of surgery for aortic valve IE in children, with a particular focus on the Ross procedure. A retrospective review of all children who underwent surgery for aortic valve IE was performed at a single institution. Between 1989 and 2020, 41 children underwent surgery for aortic valve IE, of whom 16 (39.0%) underwent valve repair, 13 (31.7%) underwent the Ross procedure, 9 (21.9%) underwent a homograft root replacement, and 3 (7.3%) underwent a mechanical valve replacement. Median age was 10.1 years (interquartile range, 5.4-14.1). The majority of children (82.9%, 34/41) had underlying congenital heart disease, while 39.0% (16/41) had previous heart surgery. Operative mortality was 0.0% (0/16) for repair, 15.4% (2/13) for the Ross procedure, 33.3% (3/9) for homograft root replacement, and 33.3% (1/3) for mechanical replacement. Survival at 10 years was 87.5% for repair, 74.1% for Ross, and 66.7% for homograft (P > 0.05). Freedom from reoperation at 10 years was 30.8% for repair, 63.0% for Ross, and 26.3% for homograft (P = 0.15 for Ross vs repair, P = 0.002 for Ross vs homograft). Children undergoing surgery for aortic valve IE have acceptable long-term survival, although the need for long-term reintervention is significant. The Ross procedure appears to be the optimal choice when repair is not feasible.

Congenital Aortic Valve Repair When the Options aren't Good: Truncus Arteriosus and Transposition of the Great Arteries.

Patients with truncus arteriosus and transposition of great arteries are prone to neo-aortic valve insufficiency. Although presenting at opposite ends of the age spectrum, both conditions tend to be commonly associated with neo aortic root dilatation. In patients with truncus arteriosus there is an additional complexity of quadricuspid valve morphology, which make up the majority of valves requiring repair. A unified approach to all these patients would include reduction and stabilization of the annulus and sinotubular junction, as well as achieving equal and symmetrical coaptation of the valve leaflets. By systematically employing these techniques, valve replacement should be avoidable in most children.