Outcomes following surgical repair of absent pulmonary valve syndrome : 30 years of experience from a Swedish tertiary referral centre

Surgical approach with reduction pulmonary artery plasty and valved conduit in patients with respiratory compromise prior to repair is associated with excellent long-term survival at the cost of a higher reintervention rate. OBJECTIVES: Absent pulmonary valve syndrome is a rare congenital heart defect with pulmonary artery dilatation and secondary airway compression. Although preoperative respiratory support and early surgical repair with pulmonary arterioplasty are often required in patients with airway compromise, the need for extensive plasty in these patients and for plasty in general in those with no or mild respiratory issues remains debatable. METHODS: We performed a retrospective survey of patients with this diagnosis and repair from 1988 to 2018. RESULTS: Twenty patients were identified. The median age and weight at repair were 0.8 (0.1-2.4) years and 7.0 (2.5-13.8) kg and included a valved conduit in 17 (85%) patients and a transannular patch in 3 patients. Five (29%) patients were ventilator-dependent prior to repair at the age of 0.3 (0.1-0.4) years. Pulmonary arterioplasty was performed in 7 patients (35%), including all 5 with ventilator dependency and 2 with respiratory symptoms due to recurrent infections. Two patients (10%) with preoperative ventilator dependency underwent extensive intrahilar arterioplasty. Preoperative ventilator dependency was associated with earlier repair and reinterventions (P < 0.05). There were 3 late deaths among cases with repair after 2000 (n = 14), none with preoperative ventilator dependency. CONCLUSIONS: The long-term outcomes of patients with this rare defect are good, comparable to those of other previous studies. Reduction pulmonary arterioplasty, which in this study was used only in patients with respiratory distress and ventilator dependency, is associated with excellent survival. Reinterventions are common in these patients

Preoperative Coronary Anatomy Assessment with Echocardiography and Morbidity After Arterial Switch Operation of Transposition of the Great Arteries

In transposition of the great arteries (TGA), certain coronary patterns have been associated with major adverse events early after the arterial switch operation (ASO). We sought to determine the impact of preoperative echocardiographic (ECHO) diagnosis on the intra­ and postoperative morbidity. All patients with TGA born between June 2001 and June 2017 and who underwent ASO were reviewed. Data on presumed coronary anatomy (CA) preoperatively were obtained from the preoperative ECHO report. Intraoperative CA was categorized according to Yacoub classification. Major postoperative morbidity included at least one of the following: delayed sternal closure (DSC), prolonged (> 72 h) mechanical ventilation, reintubation, peritoneal dialysis (PD), ECMO, reoperation, and readmission within 30 days after surgery. 240 patients with median age of 5 days (range 1–614) and mean weight at surgery was 3.6 kg (1.8–8.4) were included. Preoperative ECHO assessment of CA was available in 228 patients. Intraoperatively, 181 patients (75%) were found to have type A, 25 patients had type B or C or intramural (B–C–IM; 10%), and 34 patients had type D or E (D–E; 14%). Patients with types B, C, and intramural coronary (B–C–IM) had increased risk for delayed sternum closure (9/25 vs. 20/181 in type A and 8/34 in type D–E; p = 0.04), peritoneal dialysis (4/25 vs. 8/181 and 1/34; p = 0.04), and ECMO (2/25 vs. 1/131 and 1/34; p = 0.02). Within the B–C–IM group, preoperative ECHO raised suspicion of type A in 13 patients (i.e., incorrect diagnosis, ID; 52%), whereas non-A CA was suspected in 12 patients (i.e., correct diagnosis, CD; 48%). With the exception of reoperation, which was seen only in the ID subgroup (4/12 vs. 0/10 in the CD subgroup; p = 0.04), the intraoperative (cardiopulmonary bypass time and cross-clamp time) and postoperative morbidity indices were comparable in both ID and CD subgroups (p > 0.1). Although there is a significant risk for early postoperative morbidity in TGA patients with single, interarterial, and intramural CA, there seems to be relatively limited influence of preoperative ECHO assessment of coronary anatomy on this morbidity burden

Surgical Age and Morbidity After Arterial Switch for Transposition of the Great Arteries

Background: Transposition of the great arteries (TGA) is a complex congenital heart disease that requires early diagnosis as well as advanced surgical repair and postoperative support. We sought herein to study the impact of surgical timing on early postoperative morbidity.Methods: We reviewed all patients with TGA corrected at our institution via arterial switch operation (ASO) between June 2001 and June 2016. Major postoperative morbidity (MPM) and death within 30 days after ASO were documented. Patients with double outlet right ventricle, chromosome abnormalities and non-cardiac diseases were excluded. MPM was defined as presence of at least 1 of the following: delayed sternum closure, reoperation, prolonged mechanical ventilation, noninvasive ventilation after extubation, peritoneal dialysis, ECMO and readmission. Results: 241 patients were included, with medians for birth weight, gestational week and age at surgery of 3.5 kg, 39 weeks, and 5 days, respectively. MPM was encountered in 32.3% of patients. Prematurity (p=0.001) and need for aortic arch repair at the time of ASO (p=0.04) were associated with significant increase in MPM. Non-A coronary anatomy, associated ventricular septal defect requiring surgical closure and fetal diagnosis of TGA had no significant impact on MPM (p=0.35, 0.08 and 0.21, respectively). There was no significant difference in MPM between the surgical groups (p=0.49).Conclusions: Early complications after ASO do occur and are mostly associated with prematurity and need for aortic arch repair. Timing of surgical repair does not seem to influence the rate of these complications

Long‐Term Survival After Single‐Ventricle Palliation: A Swedish Nationwide Cohort Study

Background Long‐term survival after single‐ventricle palliation and the effect of dominant ventricle morphology in large, unselected series of patients are scarcely reported. Methods and Results This nationwide cohort study included all children undergoing operation with single‐ventricle palliation during their first year of life in Sweden between January 1994 and December 2019. Data were obtained from institutional records and assessment of underlying cardiac anomaly and dominant ventricular morphology was based on complete review of medical records, surgical reports, and echocardiographic examinations. Data on vital status and date of death were retrieved from the Swedish Cause of Death Register, allowing for complete data on survival. Among 766 included patients, 333 patients (43.5%) were classified as having left or biventricular dominance, and 432 patients (56.4%) as having right ventricular (RV) dominance (of whom 231 patients had hypoplastic left heart syndrome). Follow‐up was 98.7% complete (10 patients emigrated). Mean follow‐up was 11.3 years (maximum, 26.7 years). Long‐term survival was significantly higher in patients with left ventricular compared with RV dominance (10‐year survival: 91.0% [95% CI, 87.3%–93.6%] versus 71.1% [95% CI, 66.4%–75.2%]). RV dominance had a significant impact on outcomes after first‐stage palliation but was also associated with impaired survival after completed total cavopulmonary connection. In total, 34 (4.4%) patients underwent heart transplantation. Of these 34 patients, 25 (73.5%) had predominant RV morphology. Conclusions This study provides clinically relevant knowledge about the long‐term prognosis in patients with different underlying cardiac anomalies undergoing single‐ventricle palliation. RV dominance had a significant impact on outcomes after initial surgical treatment but was also associated with impaired survival after completed Fontan circulation. Registration URL: https://www.clinicaltrials.gov; Unique identifier: NCT03356574

Impact of concomitant complex cardiac anatomy in nonsyndromic patients with complete atrioventricular septal defect

Objective: We studied a cohort of patients with nonsyndromic complete atrioventricular septal defect with and without concomitant complex cardiac anatomy and compared the outcomes after surgical repair. Methods: Between 1993 and 2018, 62 nonsyndromic patients underwent complete atrioventricular septal defect repair. Sixteen patients (26%) had complex complete atrioventricular septal defect with variables representing concomitant cardiac anatomic complexity: tetralogy of Fallot, double outlet right ventricle, total anomalous pulmonary venous return, concomitant aortic arch reconstruction, multiple ventricular septal defects, staged repair of coarctation of the aorta, and a persisting left superior vena cava. The mean follow-up was 12.7 ± 7.9 years. Baseline variables were retrospectively evaluated and analyzed using univariable logistic regression. Survival was studied using Kaplan–Meier estimates, and group comparisons were performed using the log-rank test. A competing-risk analysis estimated the risk of reoperation with death as the competing event. A Gray's test was used to test equality of the cumulative incidence curves between groups. Results: The perioperative mortality was 3.2% (2/62). Actuarial survival was 100% versus 66.7% ± 14.9% at 10 years in the noncomplex and complex groups, respectively (P < .01). There was no significant difference in the overall reoperation rate between the noncomplex group (7/46; 15%) and the complex group (4/16; 25%) (odds ratio, 1.86; 95% confidence interval, 0.46-7.45; P = .30). The competing-risk analysis demonstrated no significant difference in reoperation between the groups (P = .28). Conclusions: Our data show that nonsyndromic patients without complex cardiac anatomy have a good long-term survival and an acceptable risk of reoperation similar to contemporary outcomes for patients with complete atrioventricular septal defect with trisomy 21. However, the corresponding group of nonsyndromic patients with concomitant complex cardiac lesions are still a high-risk population, especially regarding mortality

Comprehensive echocardiographic imaging of atrioventricular valves in children with atrioventricular septal defect : Accuracy of 2D and 3D imaging and reasons for disagreement

OBJECTIVE: To compare the accuracy and reasons for disagreement of two-dimensional (2D) and three-dimensional (3D) echocardiography findings in the assessment of the atrioventricular valve complex in patients with atrioventricular septal defect. METHODS: A total of 20 children (mean age 8 months) with atrioventricular septal defect were enrolled prospectively into this study. The accuracy of and the reasons for disagreement in the assessment of the atrioventricular valve features were analyzed between 2D and 3D echocardiography and surgical findings. RESULTS: We found that in assessing the Rastelli type and the extension of the inferior leaflet into the right ventricle, 3D echocardiography was more accurate compared to 2D echocardiography. In all other features, 2D and 3D echocardiography showed similar accuracy. A significant reason for inaccuracy by both echo modalities was that the technique itself could not visualize the feature, although the image quality was considered to be adequate. In most cases, where it was not possible to visualize the atrioventricular feature by 2D, it was possible by 3D, and vice versa. CONCLUSION: The accuracy of 2D and 3D echocardiography and understanding the potential reasons for disagreements in assessing the atrioventricular valve complex with 2D and 3D can guide the use of those two techniques when combining them in the clinical practice

Long-term outcome after early repair of complete atrioventricular septal defect in young infants

Objective: The long-term outcome after repair of complete atrioventricular septal defect in young infants is still not fully understood. The objective of this study was to evaluate data after repair for complete atrioventricular septal defect over a 25-year period to assess survival and identify risk factors for left atrioventricular valve–related reoperations. Methods: A total of 304 consecutive patients underwent surgical correction for complete atrioventricular septal defect between April 1993 and October 2018. The results for young infants (aged 3 months; n = 249; mean age, 5.1 ± 5.2 months). Mean follow-up was 13.2 ± 7.8 years (median, 14.0 years; interquartile range, 7.0-20.0). The Kaplan–Meier method was used to assess overall survival and freedom from left atrioventricular valve–related reoperation. Results: Overall, 30-day mortality was 1.0% (3/304) with no difference between young and older infants (P = 1.0). Overall survival in the total population at 20-year follow-up was 95.1% (±1.3%). Independent risk factors for poor survival were the presence of an additional ventricular septal defect (P = .042), previous coarctation of the aorta (P < .001), persistent left superior vena cava (P = .026), and genetic syndromes other than Trisomy 21 (P = .017). Freedom from left atrioventricular valve–related reoperation was 92.6% (±1.7%) at 20 years. There was no significant difference in left atrioventricular valve–related reoperation in young infants compared with older infants (P = .084). Conclusions: Our data demonstrated that excellent long-term survival could be achieved with early repair for complete atrioventricular septal defect, and the need for reoperations due to left atrioventricular valve regurgitation was low. Primary correction in patients aged less than 3 months is, when clinically necessary, well tolerated. Palliative procedures can be avoided in the majority of patients