The mid-term outcomes of aortic root replacement after surgical repair for CHD.

OBJECTIVE: The purpose of this study is to assess the mid-term outcomes of aortic root replacement after repair of CHDs. METHOD: This is a single-institutional retrospective, cohort study with consecutive patients undergoing aortic root replacement after surgical repair of CHDs between 1999 and 2022. Operative indications included aortic root dilatation with/without aortic insufficiency, sinus of Valsalva rupture, or aortic dissection involving the root. RESULTS: Forty-four patients (36 male and 8 female) were enrolled. Mean age at the root replacement was 36.6 ± 11.9 years. The most frequent primary diagnosis was congenital aortic stenosis (n = 10) and ventricular septal defect (n = 10). Mean time from the surgical repair to aortic root replacement was 26.6 ± 13.0 years. Operative indications were aortic root enlargement with or without aortic valve aetiology (n = 40), sinus of Valsalva rupture (n = 2), and aortic dissection (n = 2). Forty-two patients underwent valve-replacing aortic root replacement, and two patients underwent valve-sparing, with 40 concomitant procedures. The median follow-up was 3.5 (1.3-7.6) years. There were one early and five late mortalities and five cardiovascular-related reoperations. Actuarial survival at 5-10 years after root replacement was 81.0 ± 6.6%. The cumulative incidence of cardiovascular-related reoperation and aortic root or valve-related reoperation at 5 years after root replacement was 11.9% and 5.6%, respectively. CONCLUSION: The early and mid-term outcomes of aortic root replacement for patients with a history of repair of CHDs were favourable in terms of survival and aortic root or valve-related reoperation.

Tricuspid valve replacement provides better long-term survival and tricuspid valve function than repair in patients with systemic right ventricle.

Objective: The purpose of this study is to compare the long-term outcomes of 2 different tricuspid surgeries including valvuloplasty and replacement for significant tricuspid regurgitation in patients with systemic right ventricle. Method: This is a retrospective study of 34 patients with dextro-transposition of the great arteries or levo-transposition of the great arteries with biventricular circulation and systemic right ventricle undergoing tricuspid valve surgery between April 1979 and April 2022. Patients were divided into 2 groups based on the procedure: tricuspid valvuloplasty (n = 11) and tricuspid valve replacement (n = 23). These groups were compared in terms of survival, tricuspid valve dysfunction, and tricuspid valve-related reoperation. Results: There was no significant difference between the groups in operative age, body weight, the proportion of dextro-transposition of the great arteries, Ebstein-like tricuspid dysplasia, and preoperative right ventricular volume/function. During the median follow-up of 9.7 years, there was 1 early death (tricuspid valvuloplasty group) and 4 late deaths (3 in tricuspid valvuloplasty group and 1 in tricuspid valve replacement group). There were 7 tricuspid valve dysfunctions, including 6 significant tricuspid regurgitations in the tricuspid valvuloplasty group and 1 prosthetic valve dysfunction in the tricuspid valve replacement group, and 4 tricuspid valve-related reoperations (3 in the tricuspid valvuloplasty group and 1 in the tricuspid valve replacement group) were performed. There were significant differences between the groups in survival (tricuspid valvuloplasty vs tricuspid valve replacement: 72.7 vs 94.7% at 10 years after surgery, P = .0328) and cumulative incidence of tricuspid valve dysfunction at 10 years after tricuspid surgery (tricuspid valvuloplasty vs tricuspid valve replacement: 27.3% vs 0%, P = .0121). Conclusions: Tricuspid valve replacement provided better long-term survival and tricuspid function in patients with systemic right ventricle compared with tricuspid valvuloplasty.

Atrioventricular conduction recovery immediately after the re-operation in a repaired CHD patient.

This is a case of a 2.7-year-old girl with trisomy 21 and double outlet right ventricle who underwent epicardial pacemaker system placement for a surgical atrioventricular block and achieved atrioventricular conduction recovery immediately after residual ventricular septal defect closure. Although ventricular pacing ratio was 100% before re-operation, it declined to approximately 25% on the 6th post-operative day and was <1% 3 years after re-operation.

Favorable long-term outcomes in hospital survivors of a neonatal arterial switch operation.

Objectives: To assess long-term survival and reoperation-free survival after the arterial switch operation (ASO) and analyze the outcomes of reoperations after hospital discharge. Methods: This was a single-institution retrospective study of 476 hospital survivors of ASO since August 1982. Preoperative diagnoses included 286 transpositions of the great arteries with intact septum, 143 transpositions with ventricular septal defect, and 47 double outlet right ventricles. There were 236 neonatal ASOs, 30 aortic arch repairs, 22 concomitant left ventricular outflow tract obstruction reliefs, 16 preoperative mild pulmonary regurgitations, and 13 intramural coronary arteries. Results: During a median follow-up of 21.1 years (range, 0.1-39.2 years), 25 late deaths (5.3%) and 91 reoperations in 69 patients (14.5%) were noted. The reoperations included 44 left-sided reoperations in 34 patients (7.1%), 35 right-sided reoperations in 30 patients (6.3%), and 12 other reoperations in 12 patients (2.5%). Among the 69 patients who underwent reoperation, those with left-sided reoperations had lower survival at 15 years after reoperation compared to those with non-left-sided reoperations (91.2% vs 100%; P = .015; log-rank, 5.9). Multivariable analysis identified non-neonatal ASO, preoperative pulmonary regurgitation, intramural coronary artery, aortic arch repair, and concomitant left ventricular outflow tract obstruction relief as risk factors for left-sided reoperations. Reoperation-free survival was significantly higher in neonatal ASO compared with non-neonatal ASO (89.2% vs 75.2% at 20 years; P < .001; log-rank, 13.4). Conclusions: Hospital survivors of neonatal ASO had favorable long-term outcomes.

Septic pulmonary embolism secondary to prosthetic conduit fungal endocarditis in a child.

An 11 year-old boy underwent pulmonary vegetectomy and right ventricle-to-pulmonary artery conduit replacement for septic pulmonary embolism secondary to prosthetic conduit fungal endocarditis. He had previous surgical history of Senning/Rastelli procedure for corrected transposition of the great arteries at 5 years old. He was diagnosed with prosthetic fungal endocarditis caused by Candida parapsilosis, and suffered from growing vegetation and progressive septic pulmonary embolism despite fungal treatment. At emergent operation, pulmonary vegetectomy was performed under intermittent moderate hypothermic circulatory arrest, and infected conduit with vegetation was replaced. Approximately 2 years after the operation, he is well under anti-fungal medication.

Ventricular assist device for end-stage adult congenital heart disease patients: Current status.

As long-term surgical outcome of congenital heart disease has continued to improve, most pediatric patients with congenital heart disease are able to reach adulthood. However, adult congenital heart disease (ACHD) patients have increased risk of arrhythmia, valvular diseases, infectious endocarditis, and heart failure. The end-stage ACHD patients with advanced heart failure may require mechanical circulatory support to improve the heart failure symptoms or to recover from circulatory collapse, and may eventually aim to heart transplant or destination therapy. In general, long-term mechanical support for dilated cardiomyopathy or ischemic cardiomyopathy has been achieved with left ventricular assist device with excellent survival outcomes and improved quality of life. However, the ventricular assist device for end-stage ACHD patients can be challenging due to patient-specific anatomical feature, multiple histories of surgical and catheter-based interventions and possible multiple end-organ dysfunctions, and offered less frequently compared to non-ACHD patients. The Interagency Registry for Mechanically Assisted Circulatory Support data published recently showed that ACHD patients receiving long-term mechanical circulatory support consisted <1 % of all registrants and had higher mortality after mechanical support than non-ACHD patients. However, the ACHD patients supported with left ventricular assist device had similar survival with non-ACHD patients and a large proportion of the mortality difference between ACHD and non-ACHD patients seemed to result from operative and perioperative factors. Therefore, the ventricular assist device therapy can be an excellent treatment for selected ACHD patients. In this paper, we describe the current status of ventricular assist device support for end-stage ACHD patients and consideration to the future.

[Emergent Operation for Total Anomalous Pulmonary Venous Return].

Total anomalous pulmonary venous return with pulmonary venous obstruction is one of the congenital heart diseases requiring an emergent operation just after birth. The patients with this condition have severe lung congestion and pulmonary hypertension, and cannot survive without relief of pulmonary venous obstruction. During the emergent operation, an appropriate confluence between the common pulmonary venous chamber and left atrium needs to be created under cardiopulmonary bypass. The typical approaches for the anastomosis and the anastomosis techniques are summarized in this article. At the weaning from the cardiopulmonary bypass, the necessary treatments such as nitric oxide or open chest need to be applied. The early surgical outcome for total anomalous pulmonary venous return is referenced from the Japanese National Clinical Database.

Surgical Outcomes in Children With Partial and Transitional Atrioventricular Septal Defect.

BACKGROUND: This study aimed to evaluate the long-term outcomes of partial and transitional atrioventricular septal defect repair, focusing on left atrioventricular valve reoperation. METHODS: We conducted a retrospective review of 104 children who underwent partial or transitional atrioventricular septal defect repair between August 1983 and January 2021. The major outcomes included patient survival and left atrioventricular valve reoperation. RESULTS: Median age at repair was 4.0 (0.1-17.0) years, with 17 patients being infants (age ≤ 1 year; 16.3%). All but eight patients (92.3%) underwent left atrioventricular valve cleft closure. After initial repair, there were 18 cases of moderate-to-severe left atrioventricular valve regurgitation (17.3%). Three in-hospital deaths (2.9%) and four late deaths (3.8%) occurred. At follow-up (median 14.3 years), actuarial survival was 95.1% and 93.0% at 1 and 20 years, respectively, and 16 patients (15.4%) had undergone a total of 19 left atrioventricular valve reoperations. Initial repair performed during infancy was associated with significantly higher mortality than a repair performed after infancy (35.3% vs 1.5%, P < .01, hazard ratio = 26.4). On multivariable analysis, repair during infancy was associated with mortality (P < .01, hazard ratio = 27.4, 95% confidence interval = 2.7-283). Partial or no cleft closure of left atrioventricular valve (P = .03, hazard ratio = 4.7, 95% confidence interval = 1.2-18.8) and moderate-to-severe left atrioventricular valve regurgitation after repair (P < .01, hazard ratio = 9.9, 95% confidence interval = 3.0-32.2) were associated with left atrioventricular valve reoperation. CONCLUSIONS: Partial and transitional atrioventricular septal defect repair outcomes were generally satisfactory. However, repair during infancy had worse survival outcomes, and moderate-to-severe left atrioventricular valve regurgitation after a repair was associated with future left atrioventricular valve reoperation.

Long-term surgical results of transposition of the great arteries with left ventricular outflow tract obstruction.

OBJECTIVE: The objective of this study was to evaluate the long-term surgical results of transposition of the great arteries with left ventricular outflow tract obstruction. METHODS: We conducted a retrospective study of patients with transposition of the great arteries or double outlet right ventricle with left ventricular outflow tract obstruction undergoing biventricular repair between 1980 and 2017. RESULTS: One hundred and eleven patients were enrolled and classified into five groups: atrial switch (n = 20), arterial switch (n = 12), Nikaidoh (n = 7), Rastelli (n = 48), and REV operation groups (n = 24). Early mortality was highest in Nikaidoh group (29%). Median follow-up was 18.2 years. Long-term survival was by far lowest in Nikaidoh group and comparable among the other 4 groups. Freedom from reoperation at 20 years was lowest in Rastelli group (32.1%) due to right ventricular outflow tract-related reoperations. While having no recurrence of left ventricular outflow tract obstruction, the arterial switch operation group had a high proportion of substantial neo-aortic regurgitation (29%). CONCLUSIONS: The long-term survival was satisfactory regardless of the surgical technique except Nikaidoh group. The surgical option for transposition of the great arteries with left ventricular outflow tract obstruction should be selected based on the features of the respective procedures.

Prominent atrial fibrosis and its correlation with atrial tachyarrhythmia and dilated atrium long after classic Fontan surgery.

BACKGROUND: The progression of atrial fibrosis long after Fontan surgery is unclear. This study aimed to evaluate the degree of atrial fibrosis long after the classic Fontan procedure and to investigate the factors associated with atrial fibrosis. METHODS: We obtained atrial free wall specimens resected at Fontan conversion from 43 patients (Fontan group) and studied the degree of atrial fibrosis, along with its association with atrial tachycardia/fibrillation (AT/AF) and other clinical parameters, compared with those of the control group without heart disease (n=6). RESULTS: The time after the initial Fontan procedure was 19.9 (15.9-25.3) years. Atrial fibrosis (%) was more common in the Fontan group than in the control group [24.3 (20.9-35.0)% vs. 6.2 (5.6-7.5)%, p<0.001]. The severity of atrial fibrosis was mild in 16% (n=7), moderate in 54% (n=23), and severe in 30% (n=13) of cases. Atrial fibrosis (%) was more common in the persistent/permanent AT/AF group than in the no AT/AF (p<0.001) and paroxysmal AT/AF (p<0.001) groups. The maximum atrial diameter on computed tomography (CT) (mm) significantly correlated with atrial fibrosis (%) (r=0.52, p<0.001). The maximum diameter of the right atrium (≥75 mm) on CT was a significant risk factor for severe atrial fibrosis on multivariate logistic analysis (hazard ratio=10.22, 95% confidence interval=1.04-254.8, p=0.04). CONCLUSIONS: Atrial fibrosis was prominent long after classic Fontan surgery, especially in patients with non-paroxysmal AT/AF and dilated right atrium.

Surgical outcomes of reoperation after Fontan completion.

OBJECTIVES: Patients who have achieved Fontan circulation may require reoperation. We reviewed the outcomes of reoperation after Fontan completion and assessed the risk factors for poor outcomes. METHODS: This was a retrospective study of 106 patients undergoing open-heart reoperations after Fontan completion in 2003 at a single institution. RESULTS: The mean age at reoperation was 24.6 ± 8.3 years. A history of Fontan failure or end-organ dysfunction was noted in 30 patients. The reoperations included 73 total cavopulmonary connection conversions, 29 atrioventricular or semilunar valve operations (17 with total cavopulmonary connection conversions) and 4 other operations. Eight early deaths occurred. During a median follow-up of 5.5 (0.01-16.2) years, there were 3 late deaths and 9 second cardiac operations. The 10-year survival rate after reoperation was 89.8%, and the 5-year second cardiac operation-free survival was 84.3%. The 10-year survival rates were significantly lower in patients who underwent surgery before 2011 (75.8% vs 100%), had a history of Fontan failure or end-organ dysfunction (71.7% vs 97.3%), had preoperative central venous pressure >15 mmHg (64.9% vs 96.5%) and were operated on with deep hypothermic circulatory arrest (DHCA) (60.0% vs 91.3%). A history of Fontan failure or end-organ dysfunction, preoperative central venous pressure >15 mmHg and requirement of DHCA were identified as risk factors for mortality. CONCLUSIONS: Reoperation after Fontan completion resulted in excellent mid-term outcomes. A history of failed Fontan circulation and the requirement of DHCA negatively affected survival outcomes.

Outcome of Pulmonary Autograft After the Ross Procedure.

BACKGROUND: The purpose of this study was to assess autograft function after the Ross procedure and to review surgical outcomes associated with autograft reoperations. METHODS: This is a retrospective study of patients undergoing the Ross procedure since 1993. Autograft function and autograft reoperation were studied. Autograft failure was defined as more than moderate autograft regurgitation or autograft dilatation to more than 50 mm diameter or z-score of more than +4 in children. One hospital death was excluded from analysis as were patients with unknown late autograft status. RESULTS: Among 75 patients analyzed, preoperative diagnosis before the Ross procedure included aortic regurgitation in 26, aortic stenosis in 19, combined lesions in 28, and 2 mechanical valve malfunctions. Median age at the Ross procedure was 12.1 (0.4-43.6) years with 44 children less than 15 years old. Six patients had greater than mild autograft regurgitation at post-Ross hospital discharge. During median follow-up of 14.9 years, there were 23 autograft failures. Eighteen autograft reoperations were performed on 17 patients (13 children), including 12 aortic valve replacements, 5 aortic root replacements (including 1 valve-sparing root replacement), and 1 Konno procedure. Freedom from autograft failure and autograft reoperation at 20 years after the Ross procedure was 52.0% and 66.3%, respectively. Multivariate analysis identified greater than mild autograft regurgitation at hospital discharge from Ross procedure as a risk factor for autograft failure (P < .01). All patients who underwent autograft reoperation survived and had good health status at a median of 6.9 years after the reoperation. CONCLUSIONS: The Ross procedure is effective in delaying prosthetic aortic valve replacement, although the time-related risk of autograft failure is a real consideration.

Mortality and Reoperation Risk After Bioprosthetic Aortic Valve Replacement in Young Adults With Congenital Heart Disease.

Bioprosthetic aortic valve replacement (bAVR) in patients with congenital heart disease is challenging due to age, size and complexity. Our objective was to assess survival and identify predictors of re-operation. Data were retrospectively collected for 314 patients undergoing bAVR at 8 centers from 2000-2014. Kaplan-Meier estimation of time to re-operation and Cox regression were utilized. Average age was 45.2 years (IQR 17.8-71.1) and 30% were <21. Indications were stenosis (48%), regurgitation (28%) and mixed (18%). Twenty-eight (9%) underwent prior AVR. Median valve size was 23mm (IQR 21, 25). Implanted valves included CE (Carpentier-Edwards) Perimount (47%), CE Magna/Magna Ease (29%), Sorin Mitroflow (9%), St Jude (2%) and other (13%). Median follow-up was 2.9 (IQR 1.2, 5.7) years. Overall, 11% required re-operation, 35% of whom had a Mitroflow and 65% were <21 years old. Time to re-operation varied among valve type (p=0.020). Crude 3-year rate was 20% in patients ≤21. Smaller valve size indexed to BSA was associated with re-operation (21.7 vs. 23.5 mm/m2). Predictors of reintervention by multivariable analysis were younger age (29% increase in hazard per 5-year decrease, p<0.001), Mitroflow (HR=4 to 8 versus other valves), and smaller valve size (20% increase in hazard per 1 mm decrease, p=0.002). The overall 1, 3 and 5-year survival rates were 94%, 90% and 85% without differences by valve (p=0.19). A concerning reduction in 5-year survival after bAVR is shown. Re-operation is common and varies by age and valve type. Further research is needed to guide valve choice and improve survival.

Right arch with prostaglandin-dependent coarctation and aberrant left carotid artery in a 22q11.2 deletion infant.

Right aortic arch in association with coarctation of the aorta and vascular ring is a rare anatomy. We present an infant with chromosome 22q11.2 deletion, who had the right aortic arch with retroesophageal aberrant left subclavian artery and left internal carotid artery. The left external carotid artery and right common carotid artery originated together from the ascending aorta as a bovine branch. The infant also had severe coarctation, which was prostaglandin dependent.

Long-term surgical results of anomalous origin of the left coronary artery from the pulmonary artery repair in infants and older patients.

OBJECTIVES: The objective of this study was to compare the long-term surgical results of anomalous origin of the left coronary artery (LCA) from the pulmonary artery repair between infants and older patients. METHODS: This is a retrospective study of patients undergoing anomalous origin of the LCA from the pulmonary artery repair between 1982 and 2018. RESULTS: Thirty-five patients were enrolled and divided into two groups based on their age: infants (<1 year of age: n = 11) and older patients (>1 year of age: n = 24). Median age was 5.2 months in infants and 6.3 years in older patients. The procedure included 24 LCA implantations and 11 intrapulmonary baffle repairs. Early mortality was recorded only in two infants (18%). Early complications included two low output syndrome (18%). Survival and freedom from reoperation at median follow-up of 17.2 years were not significantly different between groups (survival, 81.8 ± 11.6% vs. 94.1 ± 5.7% [p = .3846]; freedom from reoperation, 77.8 ± 13.9% vs. 90.0 ± 6.8% [p = .1175]). Mitral regurgitation (MR) developed substantially in two older patients in the long-term. In infants, significant improvements of fractional shortening (p = .0252) and left ventricular (LV) end-systolic (p = .0142) and end-diastolic diameter (p = .0157) occurred at 1 month. CONCLUSIONS: Although there was no significant difference in survival and reoperation rate, the postoperative LV remodeling occurred more evident and rapid in infants. Worsening of MR in the long-term was observed only in older patients.

Bentall and redo-Konno Operation for an Adult With Aortic Paraprosthetic Leak and Root Aneurysm.

We report a case of Bentall and redo-Konno operation. A 39-year-old male with surgical history of Konno operation presented with hemolytic anemia and heart failure. Further evaluations revealed aortic paraprosthetic leak with moderate regurgitation, moderate biventricular outflow tract obstruction, and aortic root aneurysm. During the operation, the old Konno septoplasty patch with organized thrombus and the severely calcified right ventricular patch were replaced, and the aortic root was replaced with a larger mechanical valve in a Valsalva graft. Postoperative images showed appropriately reconstructed biventricular outflow tracts. The Bentall operation with redo-Konno was an excellent option in this situation.

Right Ventricle to Pulmonary Artery Conduit With Tricuspid Expanded Polytetrafluoroethylene Valves.

BACKGROUND: Excellent outcomes of right ventricle to pulmonary artery conduits with expanded polytetrafluoroethylene (ePTFE) valves have been reported. The purpose of this study was to evaluate the outcomes of the different material conduits with tricuspid ePTFE valves. METHODS: Forty-one consecutive patients who received right ventricle to pulmonary artery conduit with tricuspid ePTFE valves for biventricular repair between April 2004 and December 2016 were studied. The conduits made of autologous pericardial roll or xenograft roll were used in 22 patients (group P) and the conduits made of ePTFE tube were used in 19 patients (group G). The conduit reoperation and the conduit dysfunction were analyzed. RESULTS: During the median follow-up of 5.8 years, no death related to the conduit was observed. There were four reoperations (three in group P and one in group G). Freedom from conduit reoperation at 5 years was 100% in both groups (P = .30). Freedom from more than moderate conduit stenosis at 5 years after operation was not significantly different between groups (46.9% in group P vs 76.3% in group G, P = .23) even though the group G conduits were significantly smaller and freedom from more than moderate conduit regurgitation at 5 years was significantly better in group G (63.3% in group P vs 94.1% in group G, P = .04). CONCLUSIONS: The conduit with ePTFE valves in the ePTFE tubes had better conduit function compared with the conduit with autologous pericardial or xenograft roll, especially in terms of conduit regurgitation.

Reintervention rates after bioprosthetic pulmonary valve replacement in patients younger than 30 years of age: A multicenter analysis.

OBJECTIVES: To assess the difference in time to and predictors of reintervention according to valve type in surgical bioprosthetic pulmonary valve replacement (PVR) in patients younger than 30 years of age from multiple centers. METHODS: Data were retrospectively collected for 1278 patients <30 years of age undergoing PVR at 8 centers between 1996 and 2015. RESULTS: Mean age at PVR was 19.3 ± 12.8 years, with 719 (56.3%) patients ≤18 years of age. Diagnosis was tetralogy of Fallot in 626 patients (50.5%) and 165 (12.9%) had previous PVR. Median follow-up was 3.9 years (interquartile range, 1.2, 6.4). Multiple valve types were used, most commonly CE PERIMOUNT, 488 (38.2%), CE Magna/Magna Ease, 361 (28.2%), and Sorin Mitroflow 322 (25.2). Reintervention occurred in 12.7% and was most commonly due to pulmonary stenosis (68.8%), with most reinterventions occurring in children (85.2%) and with smaller valve sizes (P < .001) Among adults aged 18 to 30 years, younger age was not a significant risk factor for reintervention. Surgical indication of isolated pulmonary regurgitation was associated with a lower risk of reintervention (P < .001). Overall, 1-, 3-, 5-, and 10-year freedom from reintervention rates were 99%, 97%, 92%, and 65%. The only independent risk factors for reintervention after controlling for age and valve size were lack of a concomitant tricuspid valve procedure (P = .02) and valve type (P < .001); Sorin and St Jude valves were associated with similar time to reintervention, and deteriorated more rapidly than other valve types. CONCLUSIONS: In this large multicenter study, 8% of patients have undergone reintervention by 5 years. Importantly, independent of age and valve size, reintervention rates vary by valve type.

Myocardial ischemia from previously placed autologous pericardial roll conduits.

We report two cases of symptomatic cardiac ischemia caused by left coronary artery compression. One was a 16-year-old boy with history of the Ross procedure, aortic root aneurysm, and right ventricle-to-pulmonary artery conduit stenosis. The other was a 32-year-old woman with history of pulmonary atresia with ventricular septal defect repair and a giant right ventricle-to-pulmonary artery conduit aneurysm. In both cases, the left coronary artery was compressed by the previously placed valved autologous pericardial roll right ventricle-to-pulmonary artery conduit. Conduit replacement without direct coronary intervention relieved the coronary ischemia. Early diagnosis and relief of external compression avoided potentially fatal outcomes.