Atrioventricular Septal Defect Research Articles

Modified single-patch repair for atrioventricular septal defects results in good functional outcomes in the absence of deep ventricular septal defects

ObjectivesWe compared 2-patch repair (TP) with modified single-patch repair (MSP) for complete atrioventricular septal defects and evaluated their effect on the left atrioventricular valve (LAVV) competence. We also identified risk factors for unfavorable functional outcomes. MethodsThis retrospective study included 118 patients with complete atrioventricular septal defects who underwent intracardiac repair from 1998 to 2020 (MSP: 69; TP: 49). The median follow-up period was 10.4 years. The functional outcome of freedom from moderate or greater LAVV regurgitation (LAVVR) was estimated using the Kaplan–Meier method. ResultsThe hospital mortality was 1.7% (2/118) and late mortality was 0.8% (1/118). Eight patients required LAVV-related reoperation (MSP: 4; TP: 4) and none required left ventricular outflow tract-related reoperation. In the MSP group without LAVV anomaly, the receiver operating characteristic curve analysis revealed that the ventricular septal defect (VSD) depth was strongly associated with moderate or greater postoperative LAVVR, with the best cutoff at 10.9 mm. When stratified according to the combination of intracardiac repair type and VSD depth, the MSP-deep VSD (VSD depth >11 mm) group showed the worst LAVV competence among the 4 groups (P = .002). According to multivariate analysis, weight <4.0 kg, LAVV anomaly, and moderate or greater preoperative LAVVR were independent risk factors for moderate or greater postoperative LAVVR, whereas MSP was not a risk factor. ConclusionsPostoperative LAVVR remains an obstacle to improved functional outcomes. MSP provides LAVV competence similar to TP unless deep VSD is present. The surgical approach should be selected on the basis of anatomical variations, specifically VSD depth.

Impact of Total Anomalous Pulmonary Venous Connection in Staged Single Ventricle Palliation

BackgroundTotal anomalous pulmonary venous connection (TAPVC) with a functional single ventricle is a risk factor for mortality during staged palliation. This study aimed to assess TAPVC’s impact on staged palliation outcomes. MethodsIn a total of 602 patients with a functional single ventricle who underwent stage 1 palliation (S1P) at our center between 2001 and 2020, 39 (6.5%) patients were associated with TAPVC. Median age at S1P was 12.0 (interquartile range, 7-21) days with a body weight of 3.1 (interquartile range, 2.8-3.6) kg. Outcomes during staged palliation were compared with the remaining 563 patients without TAPVC. Risk factors for mortality were identified using a Cox proportional hazards regression model. ResultsPrimary diagnosis in functional single-ventricle patients with TAPVC included hypoplastic left heart syndromes (n = 13), unbalanced atrioventricular septal defects (n = 12) tricuspid atresias (n = 2), double inlet left ventricle (n = 1), and others (n = 11). Types of TAPVC were supracardiac (n = 21), cardiac (n = 10), infracardiac (n = 6), and mixed (n = 2). Pulmonary venous obstruction (PVO) was associated in 19 (49%) patients. S1Ps included Norwood (n = 13), aortopulmonary shunt (n = 21), and pulmonary artery banding (n = 5). Thirty-day mortality after S1P was significantly increased in patients with TAPVC vs without TAPVC (43.6% vs 16.3%; P < .001). After bidirectional cavopulmonary shunt and total cavopulmonary connection procedures, mortality was low in both groups, and no statistically significant differences were found. Correction of TAPVC at the time of S1P was not found to be a significant risk factor in univariable Cox regression analysis. In univariate and multivariate analysis, PVO was identified as an independent risk factor for mortality in patients with TAPVC (P < .001). ConclusionsOverall survival is lower in TAPVC single-ventricle patients than in non-TAPVC patients. Most deaths after S1P were associated with TAPVC, but not after S2P. PVO is a mortality risk factor in TAPVC patients.

Outcomes of bidirectional Glenn surgery done without prior cardiac catheterization

BackgroundCardiac catheterization is usually done routinely in patients with univentricular hearts before palliative Bidirectional Glenn (BDG) surgery. The objective of this study was to compare the outcomes of patients with physiological univentricular hearts and restrictive pulmonary flow that did not undergo routine cardiac catheterization before BDG with the patients that did have cardiac catheterization done. We retrospectively reviewed the data of all patients with single ventricle physiology and restrictive pulmonary blood flow who underwent BDG surgery from January 2016 till December 2020. Patients were divided into two groups: the catheterization and the non-catheterization groups.ResultsOut of 93 patients, 25 (27%) underwent BDG surgery without prior cardiac catheterization. The median age of patients was ten months, interquartile range (IQR) was 5–18 months. Tricuspid atresia represented 36% of the non-catheterization group, while unbalanced atrioventricular septal defect and hypoplastic left heart syndrome represented 19% and 17.6% of the catheterization group. No patients in the catheterization group were excluded from further BDG surgery based on the catheterization data. Moreover, no significant differences were found between the patients' groups regarding the length of hospital stay, length of intensive care unit stay, postoperative oxygen saturation, or survival (P = 0.266, P = 0.763, P = 0.543, P = 0456).ConclusionsAlthough pre-BDG cardiac catheterization is the routine and standard practice, in certain situations, some patients with single ventricle physiology and restrictive pulmonary blood flow may go directly to BDG without cardiac catheterization if noninvasive imaging is satisfactory on a case-by-case basis and according to center experience. Pre-BDG catheterization could be reserved for patients with limited echocardiographic studies, high-risk patients, or those indicated for catheter intervention before BDG surgery.

Pentalogy of Cantrell.

Pentalogy of Cantrell.

Isolated Balanced Complete Atrioventricular Septal Defects: Prenatal Detection and Outcome in Nevada.

We analyzed patients with isolated, balanced complete atrioventricular septal defects. We identified 71 patients born in Nevada, between January 2008 and December 2020. We also analyzed prenatal detection rates. Of the 71, 61 (85%) had trisomy 21, 1 (1%) had CHARGE syndrome and 22q.11 deletion, and 10 (14%) had no chromosomal abnormalities. Of the 71, 67 had prenatal care, and 43/67 (64%) were prenatally diagnosed. Prenatal detection rate for 2008-2012 was 9/20 (45%) and 18/21 (86%) for 2018-2020, P = .03. Of the 71, 67 underwent surgical repair with 1 current postpulmonary artery banding and 0 surgical deaths. Of the 67, 3 (4%) had heart block. Only 1 (1.5%) patient had reoperation for a mitral valve replacement. Of the 71, 67 (94%) are alive during a 6-year average (range = 0-12 years) follow-up. In conclusion, surgical and long-term outcomes were excellent. Also, high state-wide, general population prenatal detection rates were achieved.

Genetic characteristics and prognostic analysis of 162 fetal cardiac abnormalities

Objective: To analyze the type and characteristics of fetal cardiac abnormalities and their relationships with genetic abnormalities and clinical prognosis. Methods: The clinical data of 162 pregnant women with fetal cardiac abnormalities who came to the prenatal diagnosis center of Peking University First Hospital and performed genetic tests from February 2013 to February 2021 were reviewed. Genetic testing methods included chromosome karyotype analysis, array-based comparative genomic hybridization (aCGH) and pathogenic gene detection. Fetuses with isolated cardiac abnormalities and no fatal genetic abnormalities were assessed using the fetal cardiac birth defects clinical outcome score and followed up. Results: (1) Ultrasonography results: among the 162 fetuses, 86 cases (53.1%, 86/162) had isolated cardiac abnormalities, and 76 cases (46.9%, 76/162) had extra-cardiac abnormalities; single cardiac abnormalities were in 84 (51.9%,84/162) cases, and multiple cardiac abnormalities occurred in 78 cases (48.1%,78/162). (2) Genetic examination results: there were 39 cases (24.1%, 39/162) of pathogenic genetic abnormalities, including 35 cases (21.6%, 35/162) of pathogenic chromosome karyotype abnormality, 3 cases (1.9%, 3/162) of pathogenic copy number variant (CNV), and 1 case (0.6%, 1/162) of pathogenic gene variation. The detection rates of pathogenic genetic abnormalities were 16.3% (14/86) in fetuses with isolated cardiac abnormalities and 32.9% (25/76) in fetuses with cardiac abnormalities and extra-cardiac abnormalities, and the difference was statistically significant (χ2=6.094, P=0.014). The detection rate of genetic abnormalities was 28.6% (24/84) in the single cardiac abnormalities, among which ventricular septal defect was 36.7% (11/30), atrioventricular septal defect was 8/13, tetralogy of Fallot was 3/17, persistent trancus arteriosus was 1/1, cardiac tumor was 1/1; no genetic abnormality was detected in the other single cardiac abnormality types (22 cases in total). The main types of pathogenic genetic abnormalities were trisomy 21 (41.7%, 10/24) and trisomy 18 (41.7%, 10/24). (3) Pregnancy outcome and fetal prognosis: among 72 fetuses with isolated heart abnormalities without pathogenic genetic abnormalities, there were 4 cases of grade Ⅰ, all of which continued pregnancy; 39 cases of grade Ⅱ, with 21 cases induced labor, 18 cases continued pregnancy; 26 cases of grade Ⅲ, with 23 cases induced labor, 3 cases continued pregnancy; 3 cases of grade Ⅳ, all of which induced labor. Totally, there were 47 cases induced labor and 25 cases continued pregnancy, 24 cases (96.0%, 24/25) of which were alive. Conclusions: When fetal cardiac abnormalities are detected by prenatal ultrasound, comprehensive cardiac and extra-cardiac ultrasound assessment and further genetic testing are recommended. Fetuses excluded pathogenic genetic abnormalities and extra-cardiac abnormalities should perform clinical prognostic score evaluation through multidisciplinary collaboration, to improve maternal and fetal outcomes.

The influence of age and weight on the outcomes of complete atrioventricular septal defect repair

BackgroundThe appropriate age and weight for surgical repair of atrioventricular septal defect (AVSD) is an area of controversy. We aimed to study the effect of weight and age at the time of surgical repair for complete AVSD in children less than 2 years of age on postoperative outcomes. A retrospective data review was performed for patients who underwent the AVSD repair from 2012 to 2019 at our institutions. Our primary outcome was the postoperative in-hospital length of stay (LOS). Secondary outcomes included total positive pressure ventilation (PPV), ventilation time, maximum vasoactive–inotropic score (max VIS), and other postoperative complications.ResultsThe study included fifty patients. The median age was 191 days, and the median weight was 4.38 kg at the time of surgery. Weight < 4 kg was associated with longer PPV time and postoperative in-hospital LOS (p value of 0.033 and 0.015, respectively). Additionally, they had higher max VIS at 24 h and 48 h than the other groups with bodyweight 4–5.9 kg or ≥ 6 kg (p value of 0.05 and 0.027, respectively). Patients with older age or lower weight at operation had a longer in-hospital LOS and total length of PPV after surgery. There were no postoperative in-hospital deaths.ConclusionsOlder age and lower weight at the time of surgical repair of atrioventricular septal defect could be independent predictors of prolonged postoperative in-hospital length of stay and total length of positive pressure ventilation.

High-risk genes involved in common septal defects of congenital heart disease

The septation defect is one of the main categories of congenital heart disease (CHD). They can affect the septation of the atria leading to atrial septal defect (ASD), septation of ventricles leading to ventricular septal defect (VSD), and formation of the central part of the heart leading to atrioventricular septal defect (AVSD). Disruption of critical genetic factors involved in the proper development of the heart structure leads to CHD manifestation. Because of this, to identify the high-risk genes involved in common septal defects, a comprehensive search of the literature with the help of databases and the WebGestalt analysis tool was performed. The high-risk genes identified in the analysis were checked in 16 Indian whole-exome sequenced samples, including 13 VSD and three Tetralogy of Fallot for in silico validation. This data revealed three variations in GATA4, i.e., c.C1223A at exon 6: c.C602A and c.C1220A at exon 7; and one variation in MYH6, i.e., c.G3883C at exon 28 in two VSD cases. This study supports previously published studies that suggested GATA4 and MYH6 as the high-risk genes responsible for septal defects. Thus, this study contributes to a better understanding of the genes involved in heart development by identifying the high-risk genes and interacting proteins in the pathway.

Anesthetic Management in Parturients With Fontan Physiology

Anesthetic Management in Parturients With Fontan Physiology

Modified atrioventricular valve replacement in children

One major challenge of atrioventricular valve replacement in children is the small size of the native valve annulus. In addition, in cases of atrioventricular septal defect, the atrioventricular node (AVN) shifts to a different location, making this condition difficult to treat. This report describes a technique that uses a trapezoid-shaped patch to separate the implanted valve suture-line from the AVN, which allows surgeons to implant larger valves while simultaneously preventing injury to the AVN.

Common arterial trunk in functionally univentricular hearts: a case series.

The association of a univentricular heart defect with common arterial trunk is extremely rare. There is a lack of population-based outcome studies reported in the literature. The hospital records, echocardiographic and other imaging modality data, outpatients' records, operation notes, and other electronic data were reviewed. Patients were reviewed, and the final outcomes of surgery were observed. Six cases (two males) with common arterial trunk presented over a 30-year period. Five had a complete unbalanced atrioventricular septal defect (83%) and one (17%) had tricuspid atresia associated with common arterial trunk. All had antenatal diagnosis. Two cases (33%) were excluded from initial surgical palliation due to Trisomy 21 in one and severe truncal valve regurgitation in one. Initial surgical palliation was performed in four cases (67%) at median age of 31 days (2-60) and consisted of disconnection and reconstruction of the pulmonary arteries and establishing controlled pulmonary blood flow. There were no early deaths. Conversion to cavopulmonary shunt was not possible in two due to severe airway problems in one and pulmonary arteries anatomy in one. They died at 11 and 16 months, respectively. Two patients (33%) underwent cavopulmonary shunt with 1 (17%) being alive at 18 months - 12 months after cavopulmonary shunt. The second patient proceeded to Fontan completion at 19 months but required catheter takedown 3 months later and died 3.5 years later. Univentricular hearts with common arterial trunk carry extremely poor short- to medium-term outcomes. This should inform antenatal and postnatal counselling and decision-making.

Congenital Heart Malformations in Iraq, a Review

Background: Heart malformations are among the commonest indications for admission to hospital and may causes mortality of children. There is a need to highlight this important medical issue as there is no review article on this subject in Iraq.&#x0D; Aim: This study aims to focus on this diseases in allover Iraq by reviewing the articles that have been published previously among different Iraqi academic scientific journal (IASJ) and concerned with the congenital heart diseases in various Iraqi governorates.&#x0D; Materials and Methods: Reviewing of the publications were conducted in accordance of that already described. Identification of available articles were done via applying search on IASJ (from 1997 to 2022). The keywords of ‘congenital heart disease was used in the search. Comprehensive full text articles in English were enrolled, while those published locally in periodic journals were excluded as there they were difficult to access them. Data extraction from selected publications was done in an independent manner via specialized design including the title of the study, the date of publication, area of research, topic of research, number of cases enrolled, and major results.&#x0D; Results: Most cases are diagnosed at infancy. Male predominance was recognized in Ventricular septal defect (VSD), Tetrolgy of Fallot (TOF), stenosis of Aorta, transposition of the great arteries (D), and aorta's coarctation, Female supremacy was recognized in atrio ventricular canal defect, Patent ductus arteriosus-PDA, atrial septal defects-ASD, and stenosis of pulmonary artery. The acyanotic type was more frequent than those accompanied with cyanosis as reported by many studies. A study from Basra Province has registered the highest incidence of CHD throughout analysis of data of patients with CHD which constitutes 57(40%) out of 1414 examined children. The reviewing of data from several articles in IASJ revealed a concern with correlation of CHD with other congenital disorders. Few researches were conducted on the treatment of CHD. Several works in Iraq tried to discuss the issue from other points of views as the nutritional status and feeding problems, the impact on the growth, and even the oral and dental health.&#x0D; Conclusions: According to the results of all reviewed Iraqi studies, there is a need for establishing a high standard centers with providing equipments for pediatric surgical operations .A well prepared staff is required for such type of operations. In addition, a well cooperation between the pediatricians and cardiac surgeons to achieve accurate diagnosis, managements, and follow up is needed.

Outcomes and risk analysis after tricuspid valve surgery for non-Ebstein 2-ventricle congenital tricuspid valve diseases

ObjectivesTricuspid valve (TV) surgery remains understudied and little data exist describing the surgical indications, outcomes, and prognostic factors for pediatric patients with non-Ebstein 2-ventricle congenital TV lesions. This study aims to describe early and late outcomes of pediatric patients with non-Ebstein congenital TV lesions undergoing isolated TV procedures at a single institution. MethodsAll patients who underwent TV surgery for non-Ebstein congenital TV disease between 2006 and 2018 were included. Patients who had missing preoperative data, patients with single-ventricle physiology, congenitally corrected transposition of the great arteries, and patients undergoing TV intervention as part of repair of an atrioventricular canal defect were excluded. The primary end point was the occurrence of TV reintervention or TV regurgitation (TR) ≥ moderate. ResultsA total of 85 patients were included. The tricuspid lesion was isolated TR in 80 (94.1%), isolated tricuspid stenosis in 3 (3.5%) and mixed disease in 2 (2.4%) patients. Median age at surgery was 33 years (interquartile range, 12-53 years). TV repair and TV replacement were performed in 66 (77.6%) and 19 (22.4%) patients, respectively. One (1.2%) patient underwent TV reoperation during the same admission. There was no in-hospital mortality. Median follow-up was 3.3 years (interquartile range, 0.1-4.7 years). The overall cumulative incidence of TV reintervention or TR deemed moderate or greater at 1, 3, and 5 years was 3% ± 2%, 11% ± 4%, and 20% ± 8%. In multivariable analysis, age younger than 12 years (P = .04) and mitral valve regurgitation deemed moderate or greater (P = .01) were independent risk factors for TV reintervention or recurrent TR deemed to be moderate or greater at last follow-up. ConclusionsTV surgery in patients with non-Ebstein congenital TV disease can be performed with good outcomes. TV reintervention or TR deemed moderate or greater occurred in 20% of patients on midterm follow-up. Patients younger than age 12 years are at higher risk for recurrent TR or TV reintervention, whereas preoperative MR deemed moderate or greater increases this risk, especially in patients older than age 12 years. There was no difference in outcomes between TV replacement and repair.

Multiple Brain Abscesses in A Newborn with Down Syndrome: Heart Disease or Meningeal Infection?

Introduction: Cerebral abscess is a rare pathology in the newborn, most often secondary to meningeal involvement, but congenital heart disease is to be feared. Objectives: to review a case of multiple cerebral abscesses in the newborn with a doubtful diagnosis through a clinical case. Observation: Newborn at D6 of life, from a full-term pregnancy, with a negative infectious anamnesis, admitted for fever at 38. The first one appeared at D4 of life, in whom the clinical examination found an icteric newborn, with a trisomic face, tachycardia at 180bpm, axial hypotonia, FANT and RS+, systolic cardiac murmur and light sub-costal draught. In front of this picture, a lumbar puncture was carried out objectifying a lymphocytic meningitis, At J8 of life, the newborn presented a convulsion, An ETF was carried out objectifying a hypoechogenic image with hyperchogenic border in cocarde evoking an abces confirmed by CT. The echocardium performed as part of the etiological workup, objective a single ventricle, CIA ostium primum large, IVC admission, single atrio ventricular valve, complete atrio ventricular canal, persistence of the ductus arteriosus and Coarctation of the aorta. The newborn was put on parenteral antibiotic therapy based on C3G meningeal dose + vancomycin + metronidazole by intravenous route then oral relay, with a scannographic control and neurological clinical evaluation. In front of this case, the question that arises: are his abscesses secondary to meningitis or to congenital heart disease?. Conclusion: Brain abscess is a rare pathology, with heavy after-effects, and the prognosis of patients has improved thanks to the progress of imaging techniques, the use of ATB treatments, and minimally invasive neurosurgery. It is to be feared in front of congenital cardiopathies with right-left shunt.

Clinical progress note: Noncardiac complications in adults with congenital heart disease.

Clinical progress note: Noncardiac complications in adults with congenital heart disease.

Technique for Neo-Pulmonary Valve Creation With Living Tissue for Repair of Atrioventricular Septal Defect and Tetralogy of Fallot.

Long-standing effects of pulmonary regurgitation after transannular patch repair in Tetralogy of Fallot (ToF) can be especially deleterious in the setting of combined ToF and complete atrioventricular septal defect (CAVSD). We present a technique for a complete repair of combined ToF/CAVSD using right atrial appendage tissue to create a competent neo-pulmonary valve. This technique provides advantages of right heart protection via pulmonary valve competence and the use of living tissue capable of growth with the patient, potentially obviating the need for repeat interventions.

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

This study aimed to evaluate the long-term outcomes of partial and transitional atrioventricular septal defect repair, focusing on left atrioventricular valve reoperation. 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. 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. 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.

Reoperations After Repair for Atrioventricular Septal Defects: >25 Years Experience at a Single Center

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.

Systematic Mapping of Conduction Tissue Regions During Congenital Heart Surgery

PurposeDamage to the cardiac conduction system is a major risk of congenital cardiac surgery. Localization of the conduction system is commonly based on anatomic landmarks, which are variable in congenital heart diseases. We introduce a novel technique for identification of conduction tissue regions based on real-time fiberoptic confocal microscopy. DescriptionWe developed a fiberoptic confocal microscopy–based technique to document conduction tissue regions and deployed it in pediatric patients undergoing repair of common congenital heart defects. The technique applies clockface schematics for intraoperative documentation of the location of conduction tissue regions. EvaluationWe created clockface schematics for 11 patients with ventricular septal defects, 6 with tetralogy of Fallot, and 10 with atrioventricular canal defects. The approach revealed substantial variability in the location of the conduction system in hearts with congenital defects. The clockface schematics were used to create plans for subsequent surgical repair. ConclusionsThe clockface schematic provides a reliable fiducial system to document and communicate variability of conduction tissue regions in the heart and applies this information for decision-making during congenital cardiac surgery.

Conduction System Pacing in Pediatrics and Congenital Heart Disease, a Single Center Series of 24 Patients.

His-bundle pacing has demonstrated feasibility in numerous adult studies to reverse and prevent pacing-induced cardiomyopathy, however, is met with higher capture thresholds with deployment sheaths designed for adults with his-bundles in the typical location. To describe 24 pediatric and adult congenital patients post-physiologic pacing. Patients at the University of Minnesota Masonic Children's Hospital with congenital complete heart block or congenital heart disease and atrioventricular block presented for pacemaker placement between November 2019 and January 2021. Twenty-four patients had attempted his-bundle placement using either Medtronic's C315 or C308 sheaths and 3830 leads except for 3 patients who had Boston Scientific's His system with the Shape 3 sheath and 7842 leads. Twenty-four total patients underwent physiologic pacing (23 his-bundle, 13 female, 11 male) with median age of 14years (range 8-39years) with median weight of 51kg (range 21.2-81kg) with five right-sided implants performed. Twelve patients had congenital heart disease including atrioventricular canal defects, tetralogy of Fallot, and ventricular septal defect repairs (nine patients with ventricular septal defect repairs). Twelve patients had selective His-bundle pacing (six with congenital heart disease). Median threshold to capture was 0.5V at 0.4ms (range 0.4 to 1.1V at 0.4ms), impedance 570ohms (range 456-1140ohms), and sensing median of 9.7mV (range 1.5-13.8mV if present). The median follow-up time was 610days (range 240-760days). No complications occurred peri-procedurally or during follow-up. His-bundle pacing is feasible in pediatric and congenital heart disease patients.