Clinical Course of Residual Ventricular Septal Defects After Congenital Heart Disease Repair.

The clinical course of residual ventricular septal defects after congenital heart disease repair is not completely elucidated in the medical literature. This study assessed the incidence, size, and clinical course of residual defects.This single-center retrospective study included 132 patients who survived after ventricular septal defect patch closure (n = 107) and intracardiac repair of double-outlet right ventricle (n = 16) and tetralogy of Fallot (n = 9). Residual defect was evaluated on transthoracic echocardiogram upon hospital discharge and at outpatient clinic visits.The median age at surgery was 1.2 (0.3-13.9) years. In total, 45 (34.1%) patients presented with residual defects upon hospital discharge. The residual defects were within 2 mm (n = 27), 2-3 mm (n = 15), and > 3 mm (n = 3), and the median size was 1.5 (0.5-3.8) mm. There was no late mortality during a median follow-up of 5.4 years. Among 42 residual defects measuring < 3 mm upon hospital discharge, 37 (82.2%) spontaneously closed. Further, five defects decreased in size (1.8 ± 0.6 mm upon hospital discharge vs1.2 ± 0.8 mm at the latest visits, p = 0.15). However, the size of three residual defects measuring > 3 mm upon hospital discharge increased, and two patients required re-surgery for residual defect.Significant residual defect requiring reoperation was rare. In most cases, residual defects measuring < 3 mm upon hospital discharge spontaneously closed within 5 years, and the size of the other defects decreased.

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.

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.

[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.

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.

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.

Subarachnoid haemorrhage from undiagnosed mycotic aortic aneurysm in a child.

We report a case of subarachnoid haemorrhage resulting from a mycotic aortic aneurysm in a child with CHD. The patient previously underwent operations for CHD and had a subarachnoid haemorrhage of unknown cause before the scheduled re-operation. During the re-operation, a sealed rupture of an undiagnosed mycotic ascending aortic aneurysm was identified, and the causative organism was later identified as Streptococcus. A postoperative MRI indicated a partially thrombosed cerebral aneurysm. This case demonstrates that a mycotic aortic aneurysm can be a cause of intracranial haemorrhage in children.

Single center experience of aortic bypass graft for aortic arch obstruction in children.

The purpose of this study is to access the outcomes of aortic bypass graft placement in children. This is a retrospective review of all children having aortic bypass graft placement for aortic arch obstruction for the first time between 1982 and 2013 at a single institution. The actuarial survival and the freedom from aortic arch reoperation were calculated and compared between the groups. Seventy consecutive children underwent aortic bypass graft placements. The median age and body weight at the operation were 14 days and 3.6 kg. There were 7 early deaths, 6 late deaths, and 7 heart transplants during the median follow-up of 10.8 years (0.0-31.5 years). The actuarial transplant free survival was 64.7 % at 20 years and the freedom from aortic arch reoperation was 50.5 % at 10 years. Between the children younger than 1 year old and older than 1 year old, there were significant differences in actuarial transplant free survival (56.4 vs. 100 % at 15 years, p = 0.0042) and in the freedom from aortic arch reoperation (18.7 vs. 100 % at 10 years, p < 0.001). The children who received aortic bypass graft larger than 16 mm in size had no aortic arch reoperation at 15 years. The aortic bypass graft placement for aortic arch obstruction can be done with low mortality and morbidity for children who can receive bypass graft larger than 16 mm in size. However, it should be avoided for the neonates and infants except selected situations.

Long-term Outcomes of Surgical Repair for Corrected Transposition of the Great Arteries.

BACKGROUND: This study investigated the long-term outcomes of physiological and anatomical repair for corrected transposition of the great arteries and double-outlet right ventricle with discordant atrioventricular connection. METHODS: This single-center retrospective study included 146 patients who underwent biventricular repair of corrected transposition of the great arteries or double-outlet right ventricle with discordant atrioventricular connections from 1972 to 2023. Survival rate, freedom from reoperation, New York Heart Association (NYHA) Functional Classification, and incidence of systemic ventricular dysfunction in the long-term were compared between physiological repair (PR) and anatomical repair (AR) groups. RESULTS: The PR group consisted of 55 patients, with median age at repair of 10.3 years. Thirty-one patients underwent conventional Rastelli procedure, and 24 patients underwent atrial and/or ventricular septal defect closure. The AR group consisted of 91 patients, with median age at repair of 5.8 years. Seventy-two patients underwent atrial switch plus Rastelli procedure and 19 patients underwent atrial plus arterial switch operation. The 30-year survival was 63.5% in the PR group and 72.3% in the AR group (P = .448). The 30-year freedom from reoperation was 71.9% in the PR group and 62.2% in the AR group (P = .220). There was a significant difference in the incidence of systemic ventricular dysfunction between the groups (87.5% in the PR group and 35.3% in the AR group, P < .001) and in the NYHA classification of long-term survivors (mean NYHA class of 1.9 in the PR group and 1.5 in the AR group, P = .009). CONCLUSIONS: The systemic ventricular function and general status in the long-term were significantly better in AR patients, suggesting the potential advantage of AR.

Impact of 22q11.2 deletion on the postoperative course of children after cardiac surgery.

The primary objective of this study was to describe the impact of 22q11.2 deletion (del22q11) on the clinical characteristics, postoperative course, and short-term outcomes of children undergoing surgery for congenital heart disease. The charts of all children ages 1 day-18 years who received cardiac surgery for interrupted aortic arch (IAA), tetralogy of Fallot (TOF), or truncus arteriosus (TA) repair from 1 January 2001 to 31 December 2011 were retrospectively reviewed. The patients were divided into two groups: the 22q11 group including children with del22q11 undergoing surgery for TOF, IAA, or TA and the non-22q11 or control group including children with no chromosomal or genetic abnormality undergoing surgery for TOF, IAA, or TA. Demographic information, cardiac diagnoses, noncardiac abnormalities, preoperative factors, intraoperative details, surgical procedures performed, postoperative complications, and in-hospital deaths were collected. The outcome data collected included days of inotrope use, need for dialysis, length of mechanical ventilation, intensive care unit (ICU) length of stay (LOS), hospital LOS, and mortality. The study enrolled 173 patients: 65 patients in the 22q11 group and 108 patients in the control group. Of the 65 patients in the 22q11 group, 36 (55 %) underwent repair for TOF, 13 (20 %) for IAA, and 16 (25 %) for TA. The two groups did not differ in terms of age or weight. The preexisting conditions were similar in the two groups. Unplanned noncardiac operations were more common in the children with del22q11, but delayed chest closure was similar in the two groups. The incidence of postoperative noncardiac complications such as reintubation, vocal cord paralysis, and diaphragmatic paralysis was similar in the two groups. However, increasing numbers of patients in del22q11 group needed dialysis in one form or the other during the immediate postoperative stay. The incidence of fungal infection and wound infection was higher in the del22q11 group than in the control group. Duration of mechanical ventilation, ICU LOS, and hospital LOS were similar in the two groups, except in certain subgroups. Mortality did not differ significantly between the two groups. In conclusion, children with del22q11 have a higher risk of postoperative complications after cardiac surgery, with no difference in length of mechanical ventilation, ICU LOS, hospital LOS, or mortality. However, short-term outcomes may differ in certain subgroups.

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.

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.

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.

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.

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.

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.

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.

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.