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1.
Pediatr Cardiol ; 2024 Sep 02.
Article in English | MEDLINE | ID: mdl-39223337

ABSTRACT

Complete atrioventricular septal defect (CAVSD) can lead to the development of pulmonary obstructive vascular disease due to high pulmonary blood flow and pressures. This study aimed to evaluate the changes in pulmonary hemodynamics with aging and with patent ductus arteriosus (PDA) in children with CAVSD. We retrospectively evaluated 137 children (94% with trisomy 21, median age of 195 (25-2963) days, 58.4% female) with CASVD referred to cardiac catheterization from January 2000 to December 2020. Those with associated congenital heart disease, except PDA, had been excluded. They were divided into three age terciles (T1, T2, and T3). Aging was directly associated with higher mean (T1: 34.2 ± 9.1; T2: 37.1 ± 5.8; T3: 42 ± 10.6 mmHg, p < 0.001) and diastolic (T1: 19.4 ± 5.3; T2 21.6 ± 5.0; T3: 26.0 ± 9.5 mmHg, P < 0.001) pulmonary arterial pressures, and with higher pulmonary vascular resistance (T1: 3.24 ± 1.69, T2: 3.47 ± 1.19; T3: 4.49 ± 3.91 Wu.m2, p = 0.023). This resulted in a loss of eligibility for anatomical correction, which became evident only after 300 days of age. PDA was associated with a higher mean (37.2 [35.9; 38.5] vs. 41.3 [37.5; 45.0] mmHg, p = 0.049) and diastolic (21.7 [20.7; 22.6] vs. 26.4 [24.1; 29.0] mmHg, p = 0.001) pulmonary pressure, and resistor-compliance time (0.28 [0.26; 0.29] vs. 0.36 [0.31; 0.40], p = 0.001) after adjusting for age and sex. In children with CAVSD, aging was associated with worsening of pulmonary vascular hemodynamics, particularly when PDA was associated, resulting in loss of eligibility for anatomical correction after 10 months of age as the first surgical option.

2.
Cardiol Young ; 32(1): 132-134, 2022 Jan.
Article in English | MEDLINE | ID: mdl-34134810

ABSTRACT

CHD may, at times, occur in the framework of other rare pathologies. These, having similar clinical manifestations, present a diagnostic dilemma for the clinician.The authors present the case of an infant with non-syndromic complete atrioventricular septal defect, whose post-operative period was surprisingly complicated by progressive pulmonary hypertension. Despite intensive care, the infant ultimately died. The diagnosis of unilateral primary pulmonary lymphangiectasia was only possible post mortem.


Subject(s)
Heart Septal Defects , Hypertension, Pulmonary , Lung Diseases , Lymphangiectasis , Humans , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/etiology , Infant , Lung Diseases/complications , Lung Diseases/diagnosis , Lymphangiectasis/diagnosis
3.
Cardiol Young ; 31(4): 556-561, 2021 Apr.
Article in English | MEDLINE | ID: mdl-33303047

ABSTRACT

OBJECTIVE: Infants with complete atrioventricular septal defect occasionally accompany pulmonary hypertension; however, the pulmonary circulation can be altered by pulmonary vascular conditions as well as the left heart lesions. This study aimed to explore whether the left heart lesions were related to the pulmonary circulation among them. METHODS: We performed echocardiography and cardiac catheterisation in 42 infants with complete atrioventricular septal defect and studied relationships between the pulmonary haemodynamic parameters and the left heart morphology. RESULTS: Age and weight at preoperative evaluation were 65 days (47-114) (the median following interquartile range) and 5.5 kg (4.0-7.1), respectively. There were 27 individuals with Down syndrome. Gestational age was 38 weeks (37-39). Catheterisation showed mean pulmonary arterial pressure: 36 (29-46) mmHg, the ratio of pulmonary to systemic blood flow: 3.45 (2.79-4.98), pulmonary vascular resistance: 2.20 Wood units·m2 (1.53-3.65), and pulmonary arterial compliance: 2.78 (1.86-4.10) ml/Hg/m2. Echocardiography showed the Rastelli classification type A in 28 and type C in 14, moderate or severe left atrioventricular valve regurgitation in 19 patients (45%), atrioventricular valve index of 0.67 (0.56-0.79), left ventricular end-diastolic volume z score of 4.46 (1.96-7.78), and aortic valve diameter z score of -0.70 (-1.91 to 0.20). Multivariable regression analysis revealed that preoperative pulmonary vascular resistance was significantly correlated to gestational age (p = 0.002), and that preoperative pulmonary arterial compliance was significantly correlated to gestational age (p = 0.009) and Down syndrome (p = 0.036). CONCLUSIONS: The pulmonary circulation does not depend upon the presence of left heart lesions but gestational age and Down syndrome in infants with complete atrioventricular septal defect.


Subject(s)
Heart Septal Defects , Heart Valve Diseases , Echocardiography , Heart Septal Defects/complications , Heart Septal Defects/diagnostic imaging , Heart Septal Defects/surgery , Humans , Infant , Pulmonary Circulation , Retrospective Studies , Treatment Outcome
4.
J Card Surg ; 35(1): 75-82, 2020 Jan.
Article in English | MEDLINE | ID: mdl-31692110

ABSTRACT

OBJECTIVES: The "modified single-patch" technique (Nunn's technique) has become more widely adopted, especially in small infants with common atrioventricular septal defect (CAVSD). In this study, we retrospectively reviewed our surgical experiences of the "modified single-patch" technique with CAVSD patients, including newborns and adults, to reveal the early and midterm clinical results. METHODS: There were 233 cases diagnosed with CAVSD undergoing biventricular repair in our hospital from June 2009 to 2016. A total of 197 cases (84.5%) with the "modified single-patch" technique were enrolled in the final analysis, including type A in 106 cases (53.8%), type B in 13 cases (6.6%), type C in 15 cases (7.6%), and intermediate type in 63 cases (32%). Major associated cardiovascular malformations included five cases of unroofed coronary sinus syndrome with LSVC (UCS), five cases of coarctation of the aorta and one case of translocation of the great artery. The median age at operation of the studied 197 cases was 44.3 ± 103.3 months (ranging from 18 days to 58 years old), including two newborns. Detailed information on the "modified single-patch" procedure was described previously, and the other cardiac malformations were repaired simultaneously with surgical repair. RESULTS: There were 14 (7.1%) early deaths and 3 (1.5%) late deaths at 3 months and 1 year after the initial operation, respectively. The total mortality rate was 8.1%, and there was no significant difference in mortality among the different age groups (P = .291). Five patients (2.5%) underwent reoperation because of severe mitral insufficiency (MI), and another patient was reoperated for left ventricular outflow tract obstruction 3 years after the initial surgery. Long-term survival in the <3 months group was significantly lower than that in the group of older children (1-18 years old). Of the 180 survivors who were followed up for at least 1 year, the proportion of severe MI began to drop from 26.2% before the operation to 13.8% just 1-week postoperation and continued to decline to the lowest level, 3.4%, at 3 months postoperation. However, MI began to rebound at 3 months and reached 20.8% at 1-year postoperation. The rebound of MI was evident in the (3 months, 6 months) age group and the >18 years age group. However, compared to the preoperation situation, the proportion of severe tricuspid insufficiencies decreased each month from 24.8% to 0.0% until 3 months after the operation and remained at a relatively low level within 1 year after surgery. CONCLUSIONS: In our experience, age is not a limitation of the modified single-patch technique, and patients even in adults with surgical indications can apply this technique. In older children and adults, we should pay more attention to atrioventricular valve annuloplasty. Infants may suffer from severe residual left atrioventricular valve regurgitation, especially in the <3 months age group, so we should improve postoperative follow-up work during infancy.


Subject(s)
Cardiac Surgical Procedures/methods , Heart Septal Defects/surgery , Adolescent , Child , Child, Preschool , Female , Humans , Male , Time Factors , Treatment Outcome
5.
Heart Lung Circ ; 27(3): e4-e6, 2018 Mar.
Article in English | MEDLINE | ID: mdl-28705664

ABSTRACT

We describe a more physiological technique of complete atrioventricular septal defect repair which restores normal heart anatomy with the offset between the insertions of the mitral and tricuspid valves. This technique overcomes the drawbacks of the previous approaches, and may improve surgical outcomes, particularly in small infants with high ventricular septal defect component or dextroposed aorta.


Subject(s)
Cardiac Surgical Procedures/methods , Heart Septal Defects/surgery , Hemodynamics/physiology , Suture Techniques , Heart Septal Defects/physiopathology , Humans
6.
Pediatr Cardiol ; 38(7): 1456-1464, 2017 Oct.
Article in English | MEDLINE | ID: mdl-28711966

ABSTRACT

Technical selection for surgical repair of complete atrioventricular septal defect (CAVSD) still remains controversial. This meta-analysis aimed to compare the modified single-patch (MP) technique with the two-patch (TP) technique for patients with CAVSD. Relevant studies comparing the MP technique with the TP technique were identified through a literature search using MEDLINE, EMBASE, Google Scholar, Cochrane Library, and the China National Knowledge Infrastructure databases. The variables were ventricular septal defect (VSD) size, cardiopulmonary bypass (CBP) time, aortic cross-clamp (ACC) time, intensive care unit stay, hospital stay, and other outcomes involving mortality, left ventricular outflow tract obstruction, atrioventricular valve regurgitation, residual septal shunt, atrioventricular block, and reoperation. A random-effect/fixed-effect model was used to summarize the estimates of mean difference/odds ratio with 95% confidence interval. Subgroup analysis stratified by region was performed. Fifteen publications involving 1034 patients were included. This meta-analysis demonstrated that (1) VSD size in the MP group was significantly smaller; (2) CBP time, ACC time, and hospital stay in the MP group experienced improvement; (3) Other postoperative outcomes showed no significant differences between two groups; and (4) The trends in China and other countries were close. The MP and TP techniques had comparable outcomes; however, the MP technique was performed with significantly shorter CBP and ACC times in patients with smaller VSDs. Given this limitation of data, the results of comparison of the two techniques in patients with larger VSDs remain unknown. Further studies are needed.


Subject(s)
Cardiac Surgical Procedures/methods , Heart Septal Defects/surgery , Cardiac Surgical Procedures/adverse effects , Cardiopulmonary Bypass/statistics & numerical data , Female , Heart Septal Defects/mortality , Humans , Length of Stay/statistics & numerical data , Male , Postoperative Complications/epidemiology , Reoperation/statistics & numerical data , Survival Rate , Treatment Outcome
8.
J Cardiothorac Surg ; 19(1): 77, 2024 Feb 09.
Article in English | MEDLINE | ID: mdl-38336747

ABSTRACT

BACKGROUND: Complete atrioventricular septal defect is a complicated congenital heart malformations, and surgical correction is the best treatment, the severe tricuspid stenosis is a rare long-term complication after the surgery. CASE PRESENTATION: We report a case with the complication of severe tricuspid stenosis 7 years after the surgical correction of complete atrioventricular septal defect in a child. Then the patient underwent tricuspid mechanical valve replacement, Glenn, atrial septostomy, and circumconstriction of the right pulmonary artery. CONCLUSIONS: The patient recovered successfully with good short-term.


Subject(s)
Cardiac Surgical Procedures , Heart Septal Defects , Child , Humans , Constriction, Pathologic/complications , Follow-Up Studies , Heart Septal Defects/surgery
9.
Semin Thorac Cardiovasc Surg ; 35(3): 530-538, 2023.
Article in English | MEDLINE | ID: mdl-35738495

ABSTRACT

Our aim was to evaluate the total burden of reoperations after previous repair for atrioventricular septal defects, including long-term survival and identify risk factors for reoperation. All patients with surgical correction for atrioventricular septal defect (AVSD) 1993- 2020 underwent a follow-up in October 2020. Clinical data were obtained by retrospective review and evaluated with Kaplan-Meier and competing risk analysis. Of 477 patients who underwent initial repair, 53 patients (11.1%) underwent a total of 82 reoperations. The perioperative mortality at reoperation was 3.8% (2/53). There were no late deaths (0/51) during follow-up. In patients requiring reoperation for left atrioventricular valve regurgitation, a re-repair was performed in 90% (26/29) at first attempt. Estimated overall survival was 96.2 ± 2.6% (95% CI 91.2-100) in the Any reoperation group and 96.7 ± 0.9% (95% CI 94.9-98.5) in the No reoperation group at 20 years (P = 0.80). The cumulative incidence function of Any reoperation (with death as competing risk) was 13.0% (95% CI 9.4-16.5) at 20 years. Independent risk factors for Any reoperation included severe mitral regurgitation after primary repair (HR 40.7; 95% CI 14.9-111; P < 0.001). The risk of perioperative mortality in AVSD patients undergoing reoperation was low in the present study. Long-term survival was very good and not significantly different when compared to patients who did not need reoperation. Re-repair for left atrioventricular valve regurgitation was possible in most cases and showed long-term durability. Our data suggest that reoperations after primary repair of AVSD have very good long-term outcomes when performed at a high-volume pediatric cardiac surgery center.

10.
Gen Thorac Cardiovasc Surg ; 71(5): 321-322, 2023 May.
Article in English | MEDLINE | ID: mdl-36572755

ABSTRACT

The primum atrial septal defect suture line adjacent to the crux is performed carefully with various techniques to avoid atrioventricular block in repair of complete atrioventricular septal defect. We describe our technical modification to simplify the shallow suture line only into the endocardium above the atrioventricular node without conduction disturbance.


Subject(s)
Heart Septal Defects, Atrial , Heart Septal Defects, Ventricular , Heart Septal Defects , Humans , Atrioventricular Node , Heart Septal Defects, Ventricular/surgery , Wound Closure Techniques , Sutures , Heart Septal Defects, Atrial/surgery
11.
J Thorac Cardiovasc Surg ; 165(3): 1237-1243, 2023 03.
Article in English | MEDLINE | ID: mdl-35933186

ABSTRACT

OBJECTIVE: Several surgical techniques for repair of a complete atrioventricular septal defect have been developed. However, the postoperative complications with these methods may lead to reoperation during follow-up. The aim of this report is to share our experience with a modified surgical technique for complete atrioventricular septal defect that has anatomic advantages postoperatively and could reduce the reoperation rate. METHODS: Twenty-nine patients who underwent repair of complete atrioventricular septal defect using a V-shaped double-layer patch between April 2011 and September 2019 were retrospectively investigated. RESULTS: There were no deaths (0%) and only 1 reoperation (3.4%) in the series. The aortic crossclamp and cardiopulmonary bypass times were 62.7 ± 16.0 minutes and 113.9 ± 25.9 minutes, respectively. The median follow-up duration was 5.1 years. To date, no significant residual ventricular septal defects have been detected and no left ventricular outflow tract obstruction has been seen on echocardiography in any patient. During follow-up, the left atrioventricular valve status was assessed as no incompetence in 9 patients (31.0%), trivial in 18 patients (62.1%), and mild in 2 patients (6.9%). CONCLUSIONS: The V-shaped double-layer patch technique is a valuable surgical option for patients with complete atrioventricular septal defects. The midterm results in our series document excellent performance of this technique, which augments the area of the anterior valve of the left atrioventricular valve to make it closer to a normal mitral valve and may also reduce the need for reoperation.


Subject(s)
Cardiac Surgical Procedures , Heart Septal Defects, Ventricular , Heart Septal Defects , Humans , Retrospective Studies , Cardiac Surgical Procedures/adverse effects , Cardiac Surgical Procedures/methods , Heart Septal Defects/diagnostic imaging , Heart Septal Defects/surgery , Heart Septal Defects, Ventricular/diagnostic imaging , Heart Septal Defects, Ventricular/surgery
12.
J Thorac Cardiovasc Surg ; 163(4): 1437-1444, 2022 04.
Article in English | MEDLINE | ID: mdl-34503843

ABSTRACT

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.


Subject(s)
Abnormalities, Multiple/surgery , Heart Defects, Congenital/surgery , Heart Septal Defects/surgery , Reoperation/statistics & numerical data , Abnormalities, Multiple/mortality , Child , Female , Follow-Up Studies , Heart Defects, Congenital/mortality , Heart Septal Defects/mortality , Humans , Male , Retrospective Studies
13.
Interact Cardiovasc Thorac Surg ; 34(3): 431-437, 2022 02 21.
Article in English | MEDLINE | ID: mdl-34633029

ABSTRACT

OBJECTIVES: There are limited data available on the height of the ventricular component of the septal deficiency (VSD) in patients undergoing complete atrioventricular septal defect (CAVSD) repair. VSD height may influence optimal choice of repair strategy with potential consequences for long-term outcomes. We aimed to measure VSD height using 2-dimensional echocardiography and review its association with postoperative outcomes. METHODS: We retrospectively reviewed the preoperative echocardiograms of 45 consecutive patients who underwent CAVSD repair between May 2010 and December 2015 at a single centre. VSD height and left ventricular length on the four-chamber view were measured. Demographic details and early and late outcomes including reoperation and long-term survival were studied. RESULTS: Twenty patients underwent modified single-patch repair and 25 patients underwent double-patch repair of CAVSD. VSD height in the modified single-patch group ranged from 4.2 to 11.7 mm and in the double-patch group ranged from 5.1 to 14.9 mm. Nine patients had a deep 'scoop' with a VSD height of >10 mm, (7 double patch, 2 modified single patch). VSD height did not correlate with a specific Rastelli classification. There was no significant difference in the VSD height (P = 0.51) or the VSD height-to-left ventricular length ratio (P = 0.43) between the 2 repair groups. There was no 30-day mortality. Eight patients required reoperation; however, VSD height was not a significant predictor of reoperation (hazard ratio 0.95, 95% confidence interval 0.69-1.33; P = 0.08). CONCLUSIONS: There was no correlation between VSD height and risk of reoperation after CAVSD repair. A deep ventricular scoop is uncommon in CAVSD patients.


Subject(s)
Heart Septal Defects, Ventricular , Heart Septal Defects , Heart Septal Defects/diagnostic imaging , Heart Septal Defects/surgery , Heart Septal Defects, Ventricular/diagnostic imaging , Heart Septal Defects, Ventricular/surgery , Humans , Infant , Reoperation , Retrospective Studies , Treatment Outcome
14.
Gen Thorac Cardiovasc Surg ; 70(10): 835-841, 2022 Oct.
Article in English | MEDLINE | ID: mdl-35332445

ABSTRACT

OBJECTIVES: Complete atrioventricular septal defect with tetralogy of Fallot is a rare and complex heart disease. This study aimed to describe contemporary management approaches for this heart disease and the outcomes. METHODS: Data were obtained from 46 domestic institutions in the Japan Cardiovascular Database (2011-2018). Patients with a fundamental diagnosis of complete atrioventricular septal defect with tetralogy of Fallot, without other complex heart diseases, were included. The primary outcome was operative mortality (30-day or in-hospital mortality). RESULTS: A total of 119 patients underwent initial surgery for a complete atrioventricular septal defect with tetralogy of Fallot during this study period. Primary repair was performed in 40 (34%) patients (primary repair group), and palliative procedure was performed in 79 (66%) patients as part of a planned staged approach (staged group). Forty institutions (87%) experienced at least one case of staged repair. No institution experienced more than or equal to two cases/year on average during the study period. Overall, 11 operative mortalities occurred (9.2%). Operative mortality rates in the primary and staged groups were comparable (p = 0.5). Preoperative catecholamine use, repeat palliative surgeries, and emergency admission were significant risk factors for operative mortality in multivariate analysis (odds ratio, 95% confidence interval: 8.58, [0-0.11]; 12.65, [1.28-125.15]; 8.64, [1.87-39.32, respectively]). CONCLUSIONS: Staged approach for complete atrioventricular septal defect with tetralogy of Fallot was the preferred option. The outcomes of this complex disease were favorable for patients in centers with low cases of complete atrioventricular septal defect with tetralogy of Fallot.


Subject(s)
Cardiac Surgical Procedures , Tetralogy of Fallot , Cardiac Surgical Procedures/methods , Catecholamines , Heart Septal Defects , Humans , Infant , Japan , Tetralogy of Fallot/surgery , Treatment Outcome
15.
Clin Med Insights Pediatr ; 16: 11795565221139118, 2022.
Article in English | MEDLINE | ID: mdl-36518819

ABSTRACT

Background: We sought to determine the difference in geometric parameters in the left atrioventricular valve (LAVV) postoperative complete atrioventricular septal defect (CAVSD) compared to the normal heart, and the correlation between geometric and functional parameters for detecting the mechanism of LAVV regurgitation (LAVVR) in CAVSD. Methods: LAVV geometric parameters based on complete and acceptable quality echocardiograms of 18 patients with repaired CAVSD compared with 17 normal controls. LAVVR severity was also quantified by indexed vena contracta (I-VC) (mm) and % jet area/left atrium area (% Jet/LA), and the correlation with LAVV parameters in the CAVSD group was investigated. Results: In the CAVSD group, the posterior closing angle (Pc) was nearly the same as the anterior closing angle (Ac), yet in the normal heart, the Pc angle was double the Ac angle. The anterior opening angle (Ao) and posterior-to-anterior leaflet diameter ratio (a/p) in the CAVSD group was also significantly smaller. The CAVSD group also had a shorter indexed coaptation length (I-CL) and indexed tenting height (I-TH). Displacement length (ΔD) differed completely between the CAVSD and Normal groups, and also showed a strong positive correlation to the functional parameters of LAVVR (% Jet/LA: r = .70, P = .02; I-VC: r = .60, P = .02). Conclusions: The parameters in this study were applicable to CAVSD AV valve coaptation characteristics. We introduced 2 novel measures that may provide important insights into the differences in geometry and performance of the LAVV in repaired CAVSD as compared to normal hearts.

16.
J Thorac Cardiovasc Surg ; 161(6): 2145-2153, 2021 Jun.
Article in English | MEDLINE | ID: mdl-32919770

ABSTRACT

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 = 55; mean age 1.6 ± 0.6 months) were compared with older 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.


Subject(s)
Cardiac Surgical Procedures , Heart Septal Defects , Adolescent , Adult , Cardiac Surgical Procedures/adverse effects , Cardiac Surgical Procedures/methods , Cardiac Surgical Procedures/mortality , Child , Female , Heart Septal Defects/mortality , Heart Septal Defects/surgery , Heart Valve Diseases , Humans , Infant , Infant, Newborn , Male , Postoperative Complications , Reoperation/statistics & numerical data , Retrospective Studies , Treatment Outcome , Young Adult
17.
Eur J Cardiothorac Surg ; 61(1): 45-53, 2021 Dec 27.
Article in English | MEDLINE | ID: mdl-34002204

ABSTRACT

OBJECTIVES: Previous studies investigating risk factors associated with reoperation or mortality after repair of complete atrioventricular septal defect (CAVSD) often have not included sizeable cohorts undergoing modified single-patch repair. Both double patch and modified single-patch techniques have been widely used in Australia since the 1990s. Using a large multi-institutional cohort, we aimed to identify risk factors associated with reoperation or mortality following CAVSD repair. METHODS: Between January 1990 and December 2015, a total of 829 patients underwent biventricular surgical repair of CAVSD in Australia at 4 centres. Patients with associated tetralogy of Fallot and other conotruncal abnormalities were excluded. Demographic details, postoperative outcomes including reoperation and survival, and associated risk factors were analysed. RESULTS: Fifty-six patients (6.8%) required early reoperation (≤30 days) for significant left atrioventricular valve regurgitation or residual septal defects. Freedom from reoperation at 10, 15 and 20 years was 82.7%, 81.1% and 77%, respectively. Patients without Down syndrome and moderate left atrioventricular valve regurgitation on postoperative echocardiogram were found to be independent risk factors for reoperation. Operative mortality was 3.3%. Overall survival at 10, 15 and 20 years was 91.7%, 90.7% and 88.7%, respectively. Prior pulmonary artery banding was a predictor for mortality, while later surgical era (2010-2015) was associated with a reduction in mortality risk. CONCLUSIONS: Improved survival in the contemporary era is in keeping with improvements in surgical management and higher rates of primary CAVSD repair over time. The presence of residual moderate left atrioventricular valve regurgitation on postoperative echocardiography is an important factor associated with reoperation and close surveillance is essential to allow timely reintervention. Primary CAVSD repair at age <3 months should be preferenced to palliation with pulmonary artery banding due to the association of pulmonary artery banding with mortality in the long-term.


Subject(s)
Heart Septal Defects , Heart Septal Defects/surgery , Humans , Infant , Reoperation , Retrospective Studies , Treatment Outcome
18.
Gen Thorac Cardiovasc Surg ; 69(2): 360-363, 2021 Feb.
Article in English | MEDLINE | ID: mdl-32814999

ABSTRACT

The patient was a 28-year-old woman who was misdiagnosed with tetralogy of Fallot and straddling mitral valve after birth. She underwent a left modified Blalock-Taussig shunt at the age of 1 year. At age 28, she presented with fatigue and progressive cyanosis. Finally, she was diagnosed with tetralogy of Fallot and complete atrioventricular septal defect. To measure the exact biventricular volumes, we performed cardiac magnetic resonance imaging in addition to cardiac angiography and ensured adequate volume capacity. We eventually decided to perform biventricular repair. Her postoperative course was uneventful, and she returned to full-time work.


Subject(s)
Cardiac Surgical Procedures , Heart Septal Defects , Tetralogy of Fallot , Adult , Female , Heart Septal Defects/diagnostic imaging , Heart Septal Defects/surgery , Humans , Tetralogy of Fallot/diagnostic imaging , Tetralogy of Fallot/surgery , Treatment Outcome
19.
Ann Pediatr Cardiol ; 14(1): 35-41, 2021.
Article in English | MEDLINE | ID: mdl-33679059

ABSTRACT

OBJECTIVE: Infants requiring mitral valve replacement have few viable options. Recently, stented bovine jugular vein graft (Melody) has been surgically implanted in such cases. Herein, we report our experience, elaborating on evolution of implantation technique, pitfalls, as well as long-term outcome (including late dilatability). METHODS: Seven Melody valves were implanted (2013-2019). The median patient age and weight were 6.7 (1.8-30.5) months and 5.8 (4.6-9.5) kg, respectively. The indications for implantation were mitral stenosis and/or regurgitation postatrioventricular septal defect (AVSD) repair (5), congenital mitral valve dysplasia (1), and Shone's complex (1). Operative technique involved shortening the valve and creating a neo-sewing ring at 2/3 (atrial)-1/3 (ventricular) junction. Implantation was followed by intraoperative balloon dilatation. RESULTS: Five out of seven patients survived the perioperative period (one death due to technical failure and the other due to acute respiratory distress syndrome postcardiopulmonary bypass). Two out of five medium-term survivors got transplanted (1) or died due to acute myeloid leukemia (1). No valves were replaced. The mean echo gradient at discharge was a median 4 (2-6) mmHg. None of the patients showed left ventricular outflow tract or pulmonary venous obstruction. Two Melody valves were dilated late (5 months and 3 years postoperatively), resulting in decreasing mean gradients from 6 to 1 and from 17 to 4 mmHg. At last follow-up, surviving Melody had a mean gradient of 4 (1-9) mmHg. CONCLUSIONS: Mitral valve replacement with a Melody valve is feasible in infants, is reproducible, shows good immediate results, and offers the possibility of later dilatation. This technique offers a better solution compared to the existing alternatives for infants requiring a prosthetic mitral valve.

20.
J Thorac Cardiovasc Surg ; 159(4): 1493-1503.e3, 2020 04.
Article in English | MEDLINE | ID: mdl-31669019

ABSTRACT

OBJECTIVES: To analyze outcomes after pulmonary artery banding (PAB) in complete atrioventricular septal defect (AVSD), with a focus on surgical pathway outcome and timing, survival, and atrioventricular valve function. METHODS: PAB was performed in 50 of 474 infants (11%) from 28 institutions between 2012 and 2018 at a median age of 1.1 months. The median duration of follow-up was 2.1 years. Atrioventricular valve function was assessed by review of pre-PAB and predischarge echocardiograms (median, 9 days postoperatively). Competing-risks methodology was used to analyze the risks for biventricular repair, univentricular repair, and death. RESULTS: At 2 years, the proportions of patients who underwent biventricular repair, univentricular repair, and death were 68%, 13%, and 12%, respectively, with 8% awaiting definitive repair. After PAB, atrioventricular valve regurgitation decreased in 14 infants and increased in 10, but the distribution of regurgitation severity did not change significantly in the total cohort or subgroups. The intended management plan at PAB was deferred biventricular/univentricular decision (23 infants), 2-stage biventricular repair (24 infants), and univentricular repair (3 infants). Among the 24 infants intended for biventricular repair, 23 achieved biventricular repair and 1 died before repair. Survival at 4 years after biventricular repair among patients with previous PAB (93%) was similar to the 4-year survival of the patients who underwent primary biventricular repair (91%; n = 333). CONCLUSIONS: PAB is a successful strategy in complete AVSD to bridge to biventricular repair and has similar post-biventricular repair survival to primary biventricular repair. Changes in atrioventricular valve regurgitation after PAB were variable.


Subject(s)
Heart Septal Defects/surgery , Pulmonary Artery/surgery , Cohort Studies , Echocardiography , Female , Heart Septal Defects/diagnostic imaging , Heart Septal Defects/mortality , Humans , Infant , Male , Reoperation , Survival Rate , Treatment Outcome
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