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Objective: Acute outcomes after atrioventricular canal defects (AVCD) surgery in the current era are excellent; yet despite surgical advances, ∼15% of patients require future left atrioventricular valve (LAVV) repair. Among patients with AVC who undergo LAVV repair after primary AVC surgery, we sought to characterize the durability of these repairs. Specifically, we aimed to determine predictors for reintervention following an LAVV repair in patients with repaired AVCD, with a focus on postoperative transesophageal echocardiography (TEE)., Methods: We reviewed all patients undergoing LAVV repair (after a primary AVCD surgery) at Boston Children's Hospital between 2010 and 2020. Competing risk analysis was performed to evaluate cumulative incidence of LAVV reinterventions. Predictors of LAVV reintervention were evaluated using multivariable Cox regression., Results: A total of 137 LAVV repairs following primary AVCD surgery were performed in 113 patients. Median age and weight at LAVV repair were 25 months (interquartile range, 12-76 months) and 11.1 kg (interquartile range, 7.8-19.4 kg). Original anatomy was complete AVCD in 87 (63%), transitional AVCD in 27 (20%), and partial AVCD in 23 (17%) cases. Over a median follow-up of 12 months (interquartile range, 1.3 months-4 years), 47 (34%) of the LAVV repairs required LAVV reintervention. Reinterventions included a total of 27 LAVV re-repairs and 20 LAVV replacements. In multivariable analysis, age at LAVV repair younger than 72 months, partial AVCD anatomy, left ventricle dysfunction, mean LAVV stenosis gradient ≥5 mm Hg, and multiple jets of regurgitation on postoperative LAVV repair TEE were associated with LAVV reintervention. Grade of LAVV regurgitation on postoperative TEE was not an independent risk factor, but reintervention rates were high when residual LAVV stenosis gradient was ≥5 mm Hg and residual mild LAVV regurgitation was present on postoperative TEE (47%) and even higher when residual LAVV stenosis gradient was ≥5 mm Hg and LAVV regurgitation was greater than mild (73%)., Conclusions: Reintervention rates remain high for LAVV repairs that occur after primary AVCD surgery, particularly for patients with LAVV stenosis gradient ≥5 mm Hg and mild or greater LAVV regurgitation on postoperative TEE., (Copyright © 2023 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: Midline unifocalization has been developed for the surgical treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. All patients will eventually require reoperation due to the presence of a conduit, and some may also require revision of the distal unifocalized bed. The purpose of this study was to evaluate the surgical results of unifocalization revision., Methods: This was a retrospective review of 254 patients who underwent midline unifocalization for treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Forty-eight of 254 patients (18%) have subsequently undergone unifocalization revision. Thirty-two of these patients had previously undergone a single-stage complete repair, whereas 16 had a unifocalization and placement of a central shunt., Results: For the 32 patients who previously underwent a complete repair, there have been no early or late deaths. The peak systolic pulmonary artery to aortic pressure ratio was 0.44 ± 0.11 after the initial repair and increased to 0.82 ± 0.18 before revision. The pressure ratio decreased to 0.41 ± 0.09 after revision. Three of 32 patients (9%) have subsequently undergone a second unifocalization revision. The 16 patients who previously had a unifocalization/shunt underwent unifocalization revision and complete repair (n = 14) and revision and repeat shunt (n = 2). There was 1 operative mortality and 3 late deaths (25% total) in this cohort. Three (25%) of 12 survivors have subsequently undergone a second unifocalization revision., Conclusions: The data demonstrate that unifocalization revision can be performed with a successful outcome in a majority of patients. Patients who underwent an initial unifocalization/shunt had a higher failure rate than patients who were initially repaired., (Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is a complex and heterogeneous form of congenital heart disease. There is a controversy regarding the optimal treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. The purpose of this study was to summarize our algorithm and surgical results for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries., Methods: This was a retrospective review of 307 patients undergoing primary surgical treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Excluded from this analysis were patients who had undergone prior surgical treatment at another institution and patients with single ventricle and major aortopulmonary collateral arteries. There were 3 surgical pathways, including midline unifocalization (n = 241), creation of an aortopulmonary window (n = 46), and other (n = 20)., Results: For the 241 patients who underwent midline unifocalization, 204 (85.4%) had a single-stage complete repair. There were 37 patients who underwent a midline unifocalization and central shunt, and 24 have subsequently undergone complete repair. Forty-six patients underwent an aortopulmonary window, of whom 36 have subsequently had a complete repair. There were 20 patients who had complex anatomy and underwent procedures other than described, and14 have subsequently undergone complete repair. Thus, for the patients currently eligible, 280 (93.0%) have achieved complete repair. For the 204 patients who had a single-stage complete repair, the mean right ventricle to aortic pressure ratio was 0.36 ± 0.09. Seventy-six patients underwent a staged repair, and the mean right ventricle to aortic pressure ratio was 0.40 ± 0.09 (P < .05 compared with single-stage repair). There were 3 (1.5%) early and 8 (4.0%) late deaths for the single-stage complete repair cohort versus 4 (4.0%) early and 15 (14.9%) late deaths for all other procedures (P < .01)., Conclusions: The data demonstrate that more than 90% of patients with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries achieved complete repair. The overall mortality was significantly lower in the subgroup of patients who underwent single-stage complete repair., (Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Atrioventricular valve regurgitation is a significant cause of morbidity and mortality in patients with unbalanced atrioventricular septal defect. However, knowledge of the outcomes of atrioventricular valve repair in children with unbalanced atrioventricular septal defect and univentricular physiology is limited., Methods: We conducted a retrospective review of all patients with unbalanced atrioventricular septal defect treated with single-ventricle palliation who underwent atrioventricular valve surgery at The Royal Children's Hospital., Results: Between 1976 and 2016, 139 children with unbalanced atrioventricular septal defect underwent single-ventricle palliation, of whom 31.7% (44/139) required atrioventricular valve surgery. Repair of the atrioventricular valve was attempted in 97.7% (43/44) of patients, of whom 4.7% (2/43) were converted to replacement during the initial operation. Replacement of the atrioventricular valve without attempted repair was performed in 2.3% (1/44) of patients. Early mortality was 18.2% (8/44). Freedom from death or transplantation at 10 years was 66.0% (95% confidence interval, 49.1-78.5) and at 20 years was 53.3% (95% confidence interval, 32.1-70.6). In multivariable analysis, significant predischarge atrioventricular valve regurgitation (hazard ratio, 6.4; P = .002), age less than 1 year (hazard ratio, 8.3; P = .01), and repair before stage II palliation (hazard ratio, 3.4; P = .04) were associated with death. Freedom from reoperation at 10 years was 61.9% (95% confidence interval, 41.9-76.8) and at 20 years was 56.3% (95% confidence interval, 35.3-72.8). Moderate or greater atrioventricular valve regurgitation at discharge was associated with an increased risk of reoperation (hazard ratio, 1.8; P = .03). Of transplant-free survivors, atrioventricular valve regurgitation was less than moderate in 60.0% (15/25) at the most recent follow-up., Conclusions: Atrioventricular valve surgery in patients with unbalanced atrioventricular septal defect is associated with substantial mortality and a high rate of reoperation. Successful atrioventricular valve repair is associated with better survival and freedom from reoperation., (Crown Copyright © 2017. Published by Elsevier Inc. All rights reserved.)

Objective: Significant atrioventricular valve regurgitation (AVVR) increases mortality in patients with unbalanced atrioventricular septal defects (uAVSDs) and a single ventricle. We tested the hypothesis that abnormal leaflet tethering is associated with progressive AVVR in patients with a single ventricle with uAVSD., Methods: We retrospectively reviewed the initial presentation and prebidirectional cavopulmonary anastamosis echocardiograms of 46 consecutive patients with uAVSD with single ventricle palliation. AVVR was graded as moderate to severe if the sum of vena contracta width to dominant valve annulus ratio was ≥ 0.33. We measured tenting height, annular to leaflet angle and annular diameter, indexed to patient size where appropriate. Multivariate analysis of variables to predict progressive AVVR was performed., Results: At follow-up of 3.3 ± 2.4 years, 24 patients had mild AVVR (Group A) and 22 had moderate to severe AVVR. Overall mortality was 6%, whereas 10 had valve repair/replacement surgery. Of 22 patients with severe AVVR at follow-up, 9 had severe AVVR at initial presentation (Group B), whereas 13 had mild AVVR at presentation but developed severe AVVR at their prebidirectional cavopulmonary anastamosis echocardiogram (Group C). Group A patients had a smaller tenting height at initial presentation compared with patients in Group B and Group C, and also had early progressive reduction of indexed tenting height (P < .01). An absolute tenting height >6 mm (odds ratio, 6.6; 95% confidence interval, 1.1-39.0; P = .03) at the initial echocardiogram was identified as an independent predictor of subsequent severe AVVR., Conclusions: Early leaflet tethering is predictive of subsequent AVVR in patients with a single ventricle with uAVSD. Patients with competent AVV had progressive reduction in the degree of leaflet tethering, whereas patients with AVVR did not. This may represent an important adaptive process to maintain valve competency in uAVSD., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: Contemporary outcomes data for complete atrioventricular septal defect (CAVSD) repair are limited. We sought to describe early outcomes of CAVSD repair across a large multicenter cohort, and explore potential associations with patient characteristics, including age, weight, and genetic syndromes., Methods: Patients in the Society of Thoracic Surgeons Congenital Heart Surgery Database having repair of CAVSD (2008-2011) were included. Preoperative, operative, and outcomes data were described. Univariate associations between patient factors and outcomes were described., Results: Of 2399 patients (101 centers), 78.4% had Down syndrome. Median age at surgery was 4.6 months (interquartile range, 3.5-6.1 months), with 11.8% (n = 284) aged ≤ 2.5 months. Median weight at surgery was 5.0 kg (interquartile range, 4.3-5.8 kg) with 6.3% (n = 151) < 3.5 kg. Pulmonary artery band removal at CAVSD repair was performed in 122 patients (4.6%). Major complications occurred in 9.8%, including permanent pacemaker implantation in 2.7%. Median postoperative length of stay (PLOS) was 8 days (interquartile range, 5-14 days). Overall hospital mortality was 3.0%. Weight < 3.5 kg and age ≤ 2.5 months were associated with higher mortality, longer PLOS, and increased frequency of major complications. Patients with Down syndrome had lower rates of mortality and morbidities than other patients; PLOS was similar., Conclusions: In a contemporary multicenter cohort, most patients with CAVSD have repair early in the first year of life. Prior pulmonary artery band is rare. Hospital mortality is generally low, although patients at extremes of low weight and younger age have worse outcomes. Mortality and major complication rates are lower in patients with Down syndrome., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: We sought to describe the hospital management and early outcome of critically ill infants presenting with large ventricular septal defects and pneumonia requiring mechanical ventilation at a referral center in a developing country. Infants with large ventricular septal defects who have pneumonia might present with respiratory failure requiring mechanical ventilation. In the developing world this presentation is relatively common, but few data exist describing patient management strategies., Methods: Hospital data of consecutive infants admitted with large ventricular septal defects and pneumonia requiring mechanical ventilation were reviewed and analyzed., Results: We identified 18 infants (mean age, 3.6 +/- 3.0 months). On admission, all the infants were significantly malnourished, and echocardiography showed bidirectional shunting (predominantly right-to-left shunting) in 6 infants. Thirteen (72%) patients improved with intensive medical management that included mechanical ventilation for 1 to 16 days (median, 6.5 days); unequivocal left-to-right shunting was subsequently documented by means of echocardiography in all 13 patients. Twelve patients underwent surgical repair, and 11 (91.6%) were discharged after median mechanical ventilation of 100 hours (range, 42-240 hours) and intensive care unit stay of 8 days (range, 4-15 days). Five of 6 unoperated patients died, 4 of them within a few hours of admission. One child with multiple ventricular septal defects was discharged and subsequently underwent pulmonary artery banding., Conclusion: Corrective cardiac surgery for selected critically ill infants with large ventricular septal defects, severe malnutrition, and pneumonia requiring mechanical ventilation is feasible and should be considered a viable management strategy.

Unlabelled: From July 1982 through September 1994, 19 children had operative treatment of subaortic stenosis associated with an atrioventricular septal defect. Specific diagnosis were septum primum defects in 7, Rastelli type A defects in 6, transitional defects in 4, inlet ventricular septal defect with malattached chordae in 1, and tetralogy of Fallot with Rastelli type C defect in 1. Twenty-seven operations for subaortic stenosis were performed. Surgical treatment of the outlet lesion was performed at initial atrioventricular septal defect repair in 3 children and in the remaining 16 from 1.2 to 13.1 years (mean 4.9 years, median 3.9 years) after repair. Eighteen of the 19 children had fibrous resection and myectomy for relief of obstruction. Seven children had an associated left atrioventricular valve procedure. One child received an apicoaortic conduit. Seven children (36.8%) required 8 reoperations for previously treated subaortic stenosis. Time to the second procedure was 2.8 to 7.4 years (mean 4.9 years). Follow-up is 0.4 to 14.0 years (median 5.6 years). Six-year actuarial freedom from reoperation is 66% +/- 15%. The angle between the plane of the outlet septum and the plane of the septal crest was measured in 10 normal hearts (86.4 +/- 13.7) and 10 hearts with atrioventricular septal defects (22.2 +/- 26.0; p < 0.01). The outflow tract can be effectively shortened, widened, and the angle increased toward normal by augmenting the left side of the superior bridging leaflet and performing a fibromyectomy., Conclusion: Standard fibromyectomy for subaortic stenosis in children with atrioventricular septal defects leads to a high rate of reoperation. Leaflet augmentation and fibromyectomy may decrease the likelihood of reoperation.

Atrioventricular septal defects were repaired in 87 patients from 1981 to 1988. Interventricular communications were present in 73 patients, and major associated anomalies were present in 17 (tetralogy of Fallot in four, double-outlet right ventricle in two, multiple ventricular septal defects in 11). Five deaths occurred in the hospital (5.7%; 70% confidence limits, 3.2% to 9.7%). A preoperative New York Heart Association class V functional condition was the only incremental risk factor (p = 0.02) for death in the hospital. No patient (0%; 70% confidence limits, 0% to 2.15%) had complete heart block. Actuarial survival rate at 80 months was 81.4%. The only incremental risk factor (p = 0.005) leading to reoperation was a preoperative valve incompetence. Actuarial rate at 80 months for freedom from reoperation was 84.2% in the overall group of patients after repair of atrioventricular septal defect. We conclude that an improvement in survival and success rates should be found when a policy of earlier repair is followed.

We reviewed our experience over a 10-year period to determine whether children with Down's syndrome and complete atrioventricular canal develop pulmonary vascular obstructive disease earlier than children with normal chromosomes and this defect. Comparisons were made between Down's syndrome and normal chromosome children regarding (1) pulmonary blood flow and pulmonary vascular resistance at initial catheterization, (2) operability as related to elevation in pulmonary vascular resistance, and (3) age at diagnosis of fixed pulmonary vascular obstructive disease. The 45 patients with Down's syndrome catheterized under 1 year of age had a lower mean pulmonary blood flow (3.2 versus 5.7; p = 0.0001) and higher mean pulmonary vascular resistance (8.3 versus 4.6 Wood units.m2; p = 0.0003) than their 34 normal chromosome counterparts. When all ages were included, 38 of 81 (47%) of the children with Down's syndrome and 32 of 40 (80%) of the normal children were considered operable. Non-Down's syndrome patients who had operations had a higher pulmonary blood flow (5.8 versus 3.3; p = 0.004) and lower pulmonary vascular resistance (3.6 versus 6.0 Wood units.m2; p = 0.005) than Down's syndrome patients. Of the 34 patients who did not have operations because of pulmonary vascular obstructive disease, 31 had Down's syndrome. In 10 of 81 children with Down's syndrome, fixed pulmonary vascular obstructive disease was diagnosed before the age of 1 year, while this was found in none of 40 normal children. Our data demonstrate that Down's syndrome patients with complete atrioventricular canal have a greater degree of elevation of pulmonary vascular resistance in the first year of life and more rapid progression to fixed pulmonary vascular obstructive disease than children with normal chromosomes.

Calcium-entry blockers prevent ventricular fibrillation during acute myocardial ischemia in laboratory animals. They may be useful as an adjunct to cold cardioplegia by preserving the myocardium during cardiopulmonary bypass. Their use may limit myocardial infarct size. However, the clinical application of calcium-entry blockers for ventricular dysrhythmias associated with myocardial ischemia has been little explored, as yet. We describe four patients, all of whom had significant cardiac hypertrophy (two had idiopathic hypertrophic subaortic stenosis; one had transposition of great vessels; and one had aortic stenosis) and in whom ventricular fibrillation developed after rewarming or shortly after the termination of bypass. The dysrhythmias were refractory to multiple attempts at defibrillation and conventional pharmacologic interventions. However, in each case, defibrillation was successful after administration of verapamil.

A 9-year-old girl was successfully operated upon for transposition of the great arteries (TGA), complete atrioventricular (AV) canal, severe left ventricular outflow obstruction, absence of the coronary sinus, and a large left superior vena cava draining into the left upper corner of a common atrium. The interventricular communication was closed and the common AV orifice was partitioned, a Mustard operation was performed, and a valved conduit was inserted between the left ventricle and the pulmonary artery. This operation, which leads to a four-chamber heart, may be preferable for this condition to the previously reported Fontan type of repair. The same approach may be also used to correct those cases of double-outlet right ventricle with complete AV canal in which the interventricular communication does not extend into the perimembranous area.

An essentially single focus of left ventricular chordal insertion was found in 23 of 164 autopsied cases of common atrioventricular canal (CAVC) with a normally formed spleen (14%). Suture closure of the cleft of the mitral valve in such cases results in the surgical creation of parachute mitral valve, often with fatal iatrogenic mitral stenosis. In such patients, the cleft of the mitral valve is its main orifice and must not be sutured closed. There are four anatomic types of potentially parachute mitral valve in CAVC: (1) with one papillary muscle group and one mitral orifice (type 1A), in 10 cases (43%); (2) with one papillary muscle group and two mitral orifices (type 1B), in one case (4%); (3) with two papillary muscle groups and one mitral orifice (type 2A), in five cases (22%); and (4) with two papillary muscle groups and two mitral orifices (type 2B), in seven cases (30%). Parachute mitral valve exists only when the AVC is divided, either naturally or surgically. The essence of parachute mitral valve is an essentially single focus of chordal insertion. One or both left ventricular papillary muscle groups may be present. The presence of only one focus of left ventricular chordal insertion contraindicates cleft closure.

A severely incompetent mitral valve of partial atrioventricular canal type was repaired by reverse implantation of a Carpentier ring. The flat segment of the ring was adapted to the lateral leaflet of the valve and the curved segment to the combined left superior and left inferior leaflets. The so-called cleft was sutured. The procedure resulted in a fully competent mitral valve that continues to be competent 2 years after the operation.

Data on 8 children with the combined anomalies of complete atrioventricular canal and tetralogy of Fallot are presented. Six of the children had only a palliative procedure, and only 1 of these patients is doing relatively well at the present time from a clinical standpoint. Two of the remaining 5 children undergoing palliative procedures have not been helped significantly, and 3 of the children in this group subsequently died. Two children have had an attempted total correction of this complicated intracardiac problem. One of these children, in whom the diagnosis of atrioventricular canal was not made preoperatively, died in the early postoperative period. In the other child, the correct diagnosis was made preoperatively, and a satisfactory, totally corrective procedure was achieved. The patient is asymptomatic at this time.

Management of symptomatic atrioventricular canal (AVC) in infancy may be difficult. Between July, 1969, and September, 1977, 31 infants with complete AVC presented in congestive heart failure (CHF) to the University of Minnesota Hospitals. Fifteen of these patients have responded to medical management and have been followed as outpatients. The other 16 patients remained in CHF. Six of them died of persistent heart failure within 4 months. The other 10 infants, aged 3 weeks to 1 year (mean 4 months), underwent pulmonary artery banding and seven survived operation. One of the survivors died with apparent pneumonia 1 month postoperatively. Each of the remaining six patients, who have been followed for 9 months to 9 years, had minimal mitral insufficiency and a large ventricular shunt. The three patients dying after banding had significant mitral insufficiency. We believe that pulmonary artery banding is an effective palliative procedure for infants with complete AVC and CHF who have large ventricular shunts and minimal mitral insufficiency.

Ninety consecutive patients with atrioventricular canal were studied to identify the association between left-sided obstructive lesions and Down's syndrome. Three groups were recognized: Group I, 76 patients with atrioventricular canal and no left-sided obstructions; Group II, nine patients with atrioventricular canal and left-sided inlet obstructions; Group III, five patients with atrioventricular canal and left-sided outlet obstructions. Of the 90 patients studied, 47 had Down's syndrome and only one of these had a left-sided obstructive lesion. Of 43 without Down's syndrome, 13 or 30% had left-sided obstructive lesions with predominating lesions at the inlet level caused by right ventricular dominance.