Atrioventricular Septal Defect Research Articles

Cardiac magnetic resonance parameters associated with successful conversion from a single ventricular to a one-and-a-half or biventricular circulation in patients with a hypoplastic right ventricle

BackgroundSome patients with pulmonary atresia with an intact ventricular septum (PA/IVS) or a left ventricle dominant atrioventricular canal defect (LDAVC) with a hypoplastic right ventricle (RV) and univentricular (1 V) circulation may be candidates for conversion to either a complete biventricular (2 V) repair or a one-and-a-half ventricle repair (1.5 V). We sought to identify pre-operative cardiovascular magnetic resonance (CMR) findings associated with successful conversion from 1V to 1.5V or 2V circulation. MethodsIn this single center retrospective study, subjects with PA/IVS or LDAVC and no conotruncal abnormalities were included if they had a 1 V circulation at the time of CMR followed by a surgical intervention intended to convert them to a 1.5 V or 2 V circulation. Conversion failure was defined as any of the following: (1) oxygen saturation < 90% at the most recent follow-up, (2) conversion back to a 1.5 V or 1 V circulation, or (3) death. ResultsIn the PA/IVS cohort (n = 15, median age 1.32 years), 10 patients underwent surgical conversion to a 1.5 V circulation and 5 to a 2 V circulation. In the attempted 1.5 V group, there were 2 failures, and these cases had a lower RV mass (p = 0.04). In the attempted 2 V group, there was 1 failure, and no CMR parameters were significantly different compared to the successes. Among the successful 2 V group patients, the minimum RV end-diastolic volume (EDV) was 27 ml/m2. In the LDAVC cohort (n = 15, median age 1.0 years), 1 patient underwent surgical conversion to a 1.5 V circulation and 14 patients to a 2 V circulation. In the attempted 1.5 V group, the 1 conversion was a failure and had an RV EDV of 15 ml/m2. In the attempted 2 V group, there were 2 failures, and these cases had a smaller RV:LV stroke volume ratio (p = 0.05) and a lower RV ejection fraction (p = 0.05) compared to the successes. Among the successful 2 V group patients, the minimum RV EDV was 22 ml/m2. ConclusionsWe identified multiple CMR parameters associated with successful conversion from 1 V circulation to 1.5 V or 2 V circulation in patients with PA/IVS and LDAVC. This information may improve patient selection for conversion procedures and encourage larger studies to better define the role of CMR.

Frequency of Attention-Deficit Hyperactivity Disorder (ADHD) in Patients with Congenital Heart Diseases (CHD) at NICVD Karachi, Pakistan

Background: The study aimed to assess the frequency and impact of attention-deficit hyperactivity disorder (ADHD) on behavior, social-emotional development, intelligence, and overall quality of life among school-aged children with congenital heart diseases (CHD). Methodology: A descriptive cross-sectional study was undertaken at the National Institute of Cardiovascular Diseases (NICVD) between July 2021 to August 2021. A total of 100 CHD patients aged 6 to 18 were assessed using the non-probability purposive sampling technique. The data was collected using a questionnaire comprising SNAP-IV 26 Scale that is a screening tool for ADHD. The collected data was entered and analyzed by using Statistical Package for Social Sciences (SPSS) v 24.0. The statistical analysis was carried out with a confidence interval set at 95% and no p-value was determined as a threshold of statistical significance, owing to the qualitative nature of the data. Result: The study comprises n=100 CHD patients, aged 6 to 18 with the mean age ± standard deviation of 11.18 ± 3.914, and males and females stratifiable into 56% and 44% respectively. The ADHD screening identified n=22 (22%) subjects as highly suspected of having ADHD with no statistically significant gender difference (n=10 females vs. n=12 males). The study revealed Ventricular Septal Defect (VSD) to be the most common (28%) congenital heart defect across the in-patient and out-patient settings. VSD is followed by Atrial Septal Defect (ASD) at 24%, Tetralogy of Fallot (TOF) at 22%, Atrioventricular Septal Defect (AVSD) at 19%, and Aortic Stenosis (AS) in 7% of the patients. Out of the 25% facing comorbidities, hypertension dominated the subset of 23%. Conclusion: This study suggests that children with CHD are at an increased risk of developing ADHD, the hyperactive and the combined subtype in particular, compared to the healthy population. About 22 percent of the subjects manifested a typical clinical picture of an ADHD patient with mostly living a ‘somewhat difficult’ life. Keywords: Children, Congenital Heart Disease, CHD, Attention-Deficit Hyperactivity Disorder, ADHD, Ventricular Septal Defect, VSD, Atrial Septal Defect, ASD

Reconstruction of the Left Atrioventricular Valve with Pericardial Patch Closure of the Ostium Primum Atrial Septal Defect in a Patient with Partial Atrioventricular Septal Defect under Mild Hypothermic Extracorporeal Circulation and Cardioplegic Arrest (UKC’s Modification): A Video Presentation

A 26-year-old male patient diagnosed with partial type of atrioventricular septal defect in sinus rhythm, cleft left atrioventricular valve with mild pulmonary arterial hypertension, and severe left atrioventricular valvular regurgitation successfully underwent reconstruction of the left atrioventricular valve and pericardial patch closure of the atrial septal defect using UKC’s modification. The technical details of the surgical procedure have been elaborated in detail.

The impact of surgical repair on left ventricular outflow tract in atrioventricular septal defect with common atrioventricular valve orifice

ObjectiveAlthough a narrow left ventricular outflow tract in atrioventricular septal defect is related to its intrinsic morphology, the contribution from the repair technique remains to be quantified. MethodsA total of 108 patients with an atrioventricular septal defect with a common atrioventricular valve orifice were divided into 2 groups: 2-patch (N = 67) and modified 1-patch (N = 41) repair. The left ventricular outflow tract morphometric was analyzed by quantifying the degree of disproportion between subaortic and aortic annular dimensions (disproportionate morphometrics ratio was defined as ≤ 0.9). Z-scores (median, interquartile range) were further analyzed in a subset of 80 patients with immediate preoperative and postoperative echocardiography. A total of 44 subjects with ventricular septal defects served as controls. ResultsBefore repair, 13 patients (12%) with an atrioventricular septal defect had disproportionate morphometrics (vs 6 [14%] ventricular septal defect P = .79), but the subaortic Z-score (−0.53, −1.07 to 0.06) was lower than the ventricular septal defect (0.07, −0.57 to 1.17; P < .001). After repair, both 2-patch (8 [12%] preoperatively vs 25 [37%] postoperatively; P = .001) and modified 1-patch (5 [12%] vs 21 [51%], P < .001) procedures showed a greater degree of disproportionate morphometrics. Both 2-patch (postoperatively −0.73, −1.56 to 0.08 vs preoperatively −0.43, −0.98 to 0.28; P = .011) and modified 1-patch (−1.42, −2.63 to –0.78 vs −0.70, −1.18 to −0.25; P = .001) procedures also demonstrated lower subaortic Z-scores postrepair. The postrepair subaortic Z-scores were lower in the modified 1-patch group (–1.42 [−2.63 to −0.78]) compared with the 2-patch group (–0.73 [−1.56 to 0.08]; P = .004). Low postrepair subaortic Z-scores (<−2) were observed in 12 patients (41%) in the modified 1-patch group and 6 patients (12%) in the 2-patch group (P = .004). ConclusionsSurgical correction resulted in greater disproportionate morphometrics seen immediately postrepair. The impact on the left ventricular outflow tract was observed in all repair techniques, with a greater burden seen after modified 1-patch repair. Video AbstractThis morphometric study in AVSD with common atrio-ventricular valve orifice confirmed further derangements of LV outflow tract morphometrics immediately after surgical repair.

Long-term incidence of infective endocarditis among patients with congenital heart disease

Patients with congenital heart disease (CHD) are at lifelong high risk of infective endocarditis (IE). The risk of IE presumably differs among different CHD, but little knowledge exists on the area. In this observational cohort study, all CHD-patients born in 1977 to 2018 were identified using Danish nationwide registries and followed from the date of birth until first-time IE, emigration, death, or end of study (December 31, 2018). The comparative risk of IE among CHD-patients vs age- and sex-matched controls from the background population was assessed. The risk of IE was stratified according to the type of CHD and factors associated with IE including sex and relevant time-varying coefficients (ie, cyanosis, cardiac prostheses, diabetes mellitus, chronic kidney disease, and cardiac implantable electronic devices) were examined using Cox-regression analysis. A total of 23,464 CHD-patients (50.0% men) were identified and matched with 93,856 controls. During a median follow-up of 17.7 years, 217(0.9%) CHD-patients and 4(0.0%) controls developed IE, corresponding to incidence rates of 5.2(95%CI 4.6-6.0) and 0.02(95%CI 0.01-0.1) per 10,000 person-years, respectively. The incidence of IE was greatest among patients with tetralogy of fallot, malformations of the heart chambers (including transposition of the great arteries, univentricular heart, and truncus arteriosus), atrioventricular septal defects, and heart valve defects. Factors associated with IE among CHD-patients included male sex, cyanosis, cardiac prostheses, chronic kidney disease, and cardiac implantable electronic devices. CHD-patients have a substantially higher associated incidence of IE than the background population. With the increasing longevity of these patients, relevant guidelines concerning preventive measures are important.

Cytogenetic study of subtypes of Down syndrome and its relation with pattern of congenital cardiac defects.

To determine the frequency of subtypes of Down syndrome by karyotyping, and to establish the frequency of congenital cardiac defects in this population. The cross-sectional study was conducted at the Department of Genetics, Children Hospital, Lahore, Pakistan, from June 2016 to June 2017, and comprised of Down Syndrome patients aged <15 years. They were subjected to karyotypic analysis for determining the subtype of the syndrome, and echocardiography of all cases was done for the assessment of congenital cardiac defects. The two findings was subsequently used to establish a relation between the subtypes and congenital cardiac defects. Data collected, entered and analyzed by the SPSS version 20.0. Among the 160 cases, trisomy 21 was found in 154(96.2%), translocation 5(3.1%) and mosaicism 1(0.6%). Overall, 63(39.4%) children had cardiac defects. Among such patients, patent ductus arteriosus was most common 25(39.7%), followed by ventricular septal defects24(38.1%), atrial septal defects16(25.4%), complete atrioventricular septal defects 8(12.7%), and Tetralogy of Fallot3(4.8%), while 6(9.5%) children had other defects. Atrial septal defects was the most common double defect 9(56.2%) and had the highest coexistence with patent ductus arteriosus in Down syndrome cases with congenital cardiac defects. In Trisomy 21, the most common cardiac defect was patent ductus arteriosus, followed by ventricular septal defects in isolated defects, whereas in mixed defects, atrial septal defects and patent ductus arteriosus were the highest.

A Case of Intermediate Type Atrioventricular Septal Defect Associated with a Membranous Ventricular Septal Aneurysm in a 70-Year-Old Female Patient

A Case of Intermediate Type Atrioventricular Septal Defect Associated with a Membranous Ventricular Septal Aneurysm in a 70-Year-Old Female Patient

Anaesthetic Management of a Down’s Syndrome Patient Acyanotic Congenital Cardiac Anomalies and Hypothyroidism: A Case Report

Down Syndrome (DS) is a common chromosomal disorder that is associated with multiple anomalies in different organ systems. Cardiac anomalies are frequently observed in individuals with DS, and as patients with Congenital Heart Disease (CHD) are at an increased risk of developing complications related to anaesthesia during the perioperative and postoperative periods, it is crucial to pay attention to the anaesthetic management of DS patients undergoing corrective surgery for cardiac anomalies. Complete Atrioventricular Septal Defects (CAVSD) are the most prevalent cardiac defects in DS patients, followed by isolated ventricular septal defects, atrial septal defects, patent ductus arteriosus, and tetralogy of Fallot. This case report focuses on the anaesthetic management of a one-year-old female with DS who was diagnosed with a combination of Atrioventricular Septal Defects (AVSDs) and patent ductus arteriosus. Due to the concurrent diagnosis of hypothyroidism, the case required a thorough preanaesthetic examination and meticulous attention to perioperative anaesthetic management, considering factors such as airway difficulty, cervical spine instability, ligament laxity, and susceptibility to infections. The patient underwent cardiac surgery following a standard anaesthetic protocol, and the procedure was well tolerated. After the surgery, she was transferred to the Intensive Care Unit (ICU). The postoperative period was uneventful, and the patient was discharged on the eighth postoperative day

Undiagnosed Case of Ventricular Septal Syndrome with Eisenmenger Syndrome and Epistaxis Presentation

Eisenmenger syndrome is a rare irreversible cardiac condition as a consequence of increased Pulmonary Arterial Hypertension (PAH). This causes a reversal of flow and cyanosis. This pulmonary arterial condition developed because of unrestricted pulmonary flow. It is seen mostly in unrepaired acyanotic congenital cardiac defects with an initial left to right shunt. The common conditions are large Ventricular Septal Defect (VSD), Patent Ductus Arteriosus (PDA), Atrioventricular Septal Defect (AVSD), and atrial septal defect. The patient presented with cyanosis and worsening exertional dyspnoea due to the reversal of the shunt. It can be prevented with early diagnosis and by successfully repairing the defect. The mode of diagnosis is a good physical examination and two-dimensional (2D) echocardiography. This case report is about an 11-year-old female patient who presented with a complaint of exertional dyspnoea for five years. She had very low oxygen saturation on room air when presented in the paediatric Outpatient Department (OPD). She went for 2D echocardiography as her physical examination and laboratory investigation were not supporting any diagnosis. Her echocardiography (echo) showed large sub-aortic VSD with Eisenmenger complex and prescribed tablet Sildenafil. On the next day of admission, her dyspnoea improved, and maintained saturation without oxygen of 96-97%. She was successfully discharged and called for regular follow-up.

Cardiovascular Abnormalities Associated with Heterotaxy Syndrome: A Case Series

Abstract Heterotaxy syndromes result from the failure of normal lateralization, resulting in the abnormal arrangement of cardiothoracic and abdominal viscera. These are associated with a disproportionate morbidity and mortality rate, mandating early diagnosis, and surgical intervention. Accurate determination of situs, as well as congenital abnormalities, is necessary for surgical planning and determination of postoperative outcome and quality of life. In this case series, we present the spectrum of cardiac, pulmonary, and systemic vascular abnormalities associated with heterotaxy syndromes. Cardiac computed tomography angiography (CTA) was performed on 10 patients with heterotaxy syndromes diagnosed on echocardiography. We found that the most common congenital heart disease associated with heterotaxy in our case series was atrioventricular septal defect. Among the extracardiac vascular abnormalities, pulmonary infundibular stenosis, right-sided aortic arch, and double superior vena cava were common. Moreover, not all cases displayed congruence between cardiac, tracheobronchial, and visceral situs. In conclusion, CTA is useful in the diagnosis of intracardiac and extracardiac vascular abnormalities associated with heterotaxy syndromes. In addition, it gives valuable information about the tracheobronchial situs, visceral situs, and other congenital abnormalities.

An insight into genetics of congenital heart defects associated with Down syndrome

Down syndrome is the most frequent live born human aneuploidy. Down syndrome is characterized by a set of phenotypically identified dysmorphism and various congenital malformations of which congenital heart defects is seen in about 50% of all cases. Atrio-ventricular Septal Defect is the most common form of congenital heart defect observed in Down syndrome. However, the exact molecular cause underlying the incidence of congenital heart defects and its differential phenotypic expression in DS are still not perfectly understood. In this review we have brought together findings from different studies and discussed multiple perspectives of the genetics of congenital cardiac defects in trisomy 21 background, viz., the contributions of triplicated dosage of chromosome 21 genes along with the effect of mutations in non- chromosome 21 disomic genes. Further, the roles of copy number variation and microRNAs have been discussed. We have also tried to shed light on the role of folate metabolism gene polymorphisms on congenital cardiac anomalies observed in Down syndrome. Lastly, we summarized the role of genes from different signaling pathways involved in cardiogenesis and also the recent developments in understanding of Down syndrome associated congenital heart defect from the studies on mice models. This review provides an overview regarding current state-of-art of knowledge related to Down syndrome associated congenital heart defects based on what have been discovered so far in this domain and hints at what prospect of research lies in this field in the future.

Association of bilateral inferior vena cava with azygos and hemiazygos continuation and aortic coarctation in a child

Introduction. Developmental variations of the inferior cava vein (IVC) rarely cause symptoms, and they were detected during routine examinations performed for other reasons. The prevalence in the general population is between 0.07% and 8.7%. Various anomalies of the IVC can be seen depending on abnormal regression or abnormal persistence of embryonic veins. They are usually associated with more complex intracardiac and atrioventricular septal defects, partial anomalous pulmonary venous connection, and pulmonary atresia. Case Outline. We presented the 18th-month-old patient with double IVC, IVC interruption, azygos, and hemiazygos continuation associated with aortic coarctation. The vein malformation was discovered during percutaneous balloon angioplasty of the aortic re-coarctation and confirmed by using cross-sectional imaging modalities. Our patient had no symptoms of IVC malformation. Conclusion. In clinical practice, double IVC should be suspected in patients with recurrent pulmonary emboli. Another important point in practice is the identification of those anomalies to avoid potential complications of retroperitoneal surgery and cannulation during cardiac surgery

Pericardial Patch Aneurysm following Repair of Atrioventricular Septal Defect and Mitral Valve Replacement with 10-Year Follow-Up

A 26-year-old female patient who had surgical closure of the ventricular septal defect with an autologous pericardial patch and mitral valve repair for the cleft mitral valve in early childhood at age 4 and then subsequently mitral valve replacement with a bioprosthetic valve in 2011 at the age of 16 years, was found to have a large cystic mass protruding into the right ventricular inflow on echocardiography during a late routine examination in 2012. The cystic mass identified on echocardiography was shown to be an aneurysmal dilatation of the autologous pericardial patch used for the repair of the ventricular septal defect. As the third corrective surgery would have been inherently complicated with risk to the conduction system, she has been followed conservatively for the last 10 years without any significant complaints except mild dyspnea. This case illustrates that the pericardial patch aneurysm is relatively stable and surgery is rarely indicated unless the aneurysm causes mechanical compression or obstruction of the right ventricular inflow or outflow.

A case of Ellis-van Creveld syndrome in Palestine

Background: Ellis-van Creveld syndrome (EVC) causes chondral and ectodermal abnormalities. Although the precise prevalence is still unknown, the Amish group in the United States most frequently reports this uncommon sickness. Case presentation: The reported case was of a 2-year-old female patient presented with dysmorphic facial and digital features, polydactyly, dwarfism, inability to walk normally, and multiple cardiac abnormalities. On examination, the patient\&amp;apos;s growth parameters were below the 5th percentile, with a weight of 10 kg, height of 72 cm, and head circumference of 45 cm (10th percentile). The patient had sparse, thin hair with bi-temporal narrowing and frontal bossing. The patient was advised to undergo surgery, which included AV canal repair, atrial septal defect closure, VSD closure, Mitral and tricuspid valve cleft closure, and left SVC tunneling to the right atrium. One week after the operation, the patient developed sudden bilateral visual impairment, with no hemorrhage or space-occupying lesions. The patient was discharged on day 15 after surgery, and although stable, the visual impairment remained. Conclusion: This case is believed to be one of the first cases in Palestine, as this disease is very rare worldwide. The outcomes of the condition are thought to be well predicted by prenatal discoveries.

Clinical Presentation and Outcome of Management of Congenital Heart Diseases Associated with Down Syndrome in a Tertiary Care Hospital

Introduction: Treatment of children with Down syndrome having congenital heart defects has not been reported well in Bangladesh. This study has been carried out to inform practitioners about findings from cases in Combined Military Hospital, Dhaka. Methods: Three years retrospective observational study (clinical data, hospital course and follow-up) was carried out on 114 patients (49 males and 65 females, age range 1 month to 12 years) who underwent diagnostic and therapeutic work up in the pediatric cardiology department CMH Dhaka between January 2018 and December 2020. Results: Among 114 patients ,64 were (57%) were infants and 50 were older children (43%) , twenty-five had congestive cardiac failure and eighteen had hypothyroidism. The study showed that 33.3% were atrioventricular septal defect (AVSD), 28.1% were patent ductus arteriosus (PDA),8.75% were tetralogy of Fallot (TOF) ,5.25% were coartation of aorta (COA) ,3.5% were patent ductus arteriosus with coarctation of aorta 3.5%, double outlet right ventricle with pulmonary stenosis were 3.5%, ventricular septal defect were 7% whereas atrial septal defect along with ventricular septal defect were 5.3% ,atrial septal defect were 3.5%, transposition of great arteries with multiple ventricular septal defect were 1.8%.Forty (35%) patients using per cutaneous intervention,had 100% procedural success and no mortality .45 patients underwent surgery,few of them were not operated due to severe pulmonary hypertension. Conclusion: Congenital heart disease was more frequent in DS children,having worse prognosis.Children with DS required special attention due to increased risk of pulmonary hypertension. Delayed treatment was an important risk factor for poor prognosis.So early diagnosis is needed in order to provide optimal intervention and surgery ,before occurrence of irreversible PAH. J Bangladesh Coll Phys Surg 2023; 41: 7-14

A randomized controlled comparison of three modes of ventilation during cardiopulmonary bypass on oxygenation in pediatric patients with pulmonary hypertension undergoing congenital heart surgeries

ABSTRACT Background Several studies have attempted to improve post bypass oxygenation, decrease extravascular total lung water volume, utilizing continuous positive airway pressure (CPAP) and high-frequency ventilation (HFV) during cardiopulmonary bypass (CPB). Aims To assess the influence of various ventilation modes during CPB on direct pulmonary artery systolic pressure and post bypass oxygenation in pediatric patients with moderate to severe pulmonary hypertension undergoing corrective cardiac surgeries. Methods Included in the study were 24 patients aged 4 months to 6 years, suffering from moderate to severe pulmonary hypertension, undergoing elective corrective cardiac surgeries for atrial septal defect (ASD) or ventricular septal defect (VSD) or atrioventricular canal defects (AVC) (ASA II and III). Group A patients (n = 8) received high-frequency positive pressure ventilation during cardiopulmonary bypass, Group B patients (n = 8) received continuous positive, while group C patients (n = 8) disconnected from the ventilation (passive deflation) (control group). Results There was no statistically significant difference regarding the pulmonary artery systolic pressure (PASP) and pulmonary artery systolic pressure to systemic systolic blood pressure (PASP/SSBP Ratio) at t1, t2, and t3 between the three groups. Conclusion After cadiopulmonary bypass, no significant changes in pulmonary artery pressure was observed in pediatric patients, regardless of the ventilation mode utilized during cardiopulmonary bypass. Conversely, the pulmonary outcomes; delivered Oxygen (DA-aO2), arterial oxygen tension (paO2) and paO2/FiO2 (p/f ratio) and lung ultrasound (LUS) were significantly improved when comparing continuous positive airway pressure (CPAP) with high frequency positive pressure ventilation (HFPPV) and passive deflation during cardiopulmonary bypass.

The Effect of Analgesia in Fast Track Pediatric Cardiac Patient

Background: Rapid tracheal extubation after cardiac surgery is not novel. "Fast-track management" has gained popularity in recent years, and the provision of cost-effective treatment is now included in with other factors as a means of assessing the success of various surgical procedures. Objective: In this study our main goal is to evaluate the effect of Analgesia in fast track pediatric cardiac patient. Method: This retrospective study was done at Bangladesh Shishu Hospital and Institute, Bangladesh, from January 2021 to January 2022. A total of 100 patients considered suitable for fast-track care were selected prior to surgery. The criteria for the selection of fast-track patients included low- risk cardiac surgery and the absence of other associated complex defects, either a weight over 10 kg or at least 6 months of age, the absence of complex non-cardiac issues and no significant history of repeat chest infections or obstructive airway disease. Results: during the study, mean age group was 5.7 years, followed by 55 cases were male, mean weight was 15.5 kg. 50% had a trial septal defect followed by 15% Ventricular septal defect, 11% Partial anomalous pulmonary, 9% congenital sabaortic stenosis, 10% had Atrioventricular septal defect. While reviewing post-operative faces pain score in day-1, 15% had no hurts followed by 60% had hurts a little bit, 10% had hurts more, 5% had hurts even more, 5% had hurts whole lot, 5% had hurts worst In day-2 25% had no hurts, 50% had hurts a bit, 12% had hurts a little more, 6% had hurts even more. In day-3 40% had no hurts, 40% had hurts a little bit, 20% had hurts little more. However, in day-4 15% had hurts little bit and 10% had hurts little more. In addition, 90% of the patients had between excellent and satisfactory analgesia. Moreover, 48% had vomiting and 2% had itching. Conclusion: The treatment of congenital heart disease after surgery is projected to become more reliant on "fast-track" care as the healthcare system ....

Simplified primum ASD closure technique in complete VSD repair: shallow suture crossing on the AV node.

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 shallowsuture line only into the endocardium above the atrioventricular node without conduction disturbance.

Staged ventricular recruitment and biventricular conversion following single-ventricle palliation in unbalanced atrioventricular canal defects

ObjectiveRestoration of biventricular circulation is an alternative management strategy in unbalanced atrioventricular canal defects (uAVCDs), especially in patients with risk factors for single-ventricle palliation (SVP) failure. When ventricular volume is inadequate for biventricular circulation, recruitment procedures may accommodate its growth. In this study, we review our uAVCD experience with biventricular conversion (BIVC) after prior SVP. MethodsThis is a single-institution, retrospective cohort study of uAVCD patients who underwent BIVC after SVP, with staged recruitment (staged) or primary BIVC (direct) between 2003 to 2018. Mortality, unplanned reinterventions, imaging, and catheterization data were analyzed. ResultsSixty-five patients underwent BIVC from SVP (17 stage 1, 42 bidirectional Glenn, and 6 Fontan). Decision for conversion was based on poor SVP candidacy (n = 43) or 2 adequately sized ventricles (n = 22). Of the 65 patients, 20 patients underwent recruitment before conversion. The staged group had more severe ventricular hypoplasia than the direct group, reflected in prestaging end-diastolic volume z scores (–4.0 vs –2.6; P < .01), which significantly improved after recruitment (–4.0 to –1.8; P < .01). Median follow-up time was 1.0 years. Survival and recatheterizations were similar between both groups (hazard ratio, 0.9; 95% CI, 0.2-3.7; P = .95 and hazard ratio, 1.9; 95% CI, 0.9-4.1; P = .09), but more reoperations occurred with staged approach (hazard ratio, 3.1; 95% CI, 1.3-7.1; P = .01). ConclusionsBiventricular conversion from SVP is an alternative strategy to manage uAVCD, particularly when risk factors for SVP failure are present. Severe forms of uAVCDs can be converted with staged BIVC with acceptable mortality, albeit increased reinterventions, when primary BIVC is not possible.

Conduction mapping during complex congenital heart surgery: Creating a predictive model of conduction anatomy

ObjectivesThe study objectives were to report on a growing experience of conduction system mapping during complex congenital heart surgery and create a predictive model of conduction anatomy. MethodsPatients undergoing complex cardiac repair with conduction mapping were studied. Intraoperative mapping used a multielectrode catheter to collect His bundle electrograms in the open, decompressed, beating heart. Patient anatomy, operative details, His bundle location, and postoperative conduction status were analyzed. By using classification and regression tree analysis, a predictive model of conduction location was created. ResultsA total of 109 patients underwent mapping. Median age and weight were 1.8 years (range, 0.2-14.9) and 10.8 kg (range, 3.5-50.4), respectively. Conduction was identified in 96% (105/109). Median mapping time was 6 minutes (range, 2-33). Anatomy included atrioventricular canal defect, double outlet right ventricle, complex transposition of the great arteries, and multiple ventricular septal defects. By classification and regression tree analysis, ventricular looping and visceroatrial situs were the greatest discriminators of conduction location. A total of 94 of 105 patients (89.5%) were free of complete heart block. Only 1 patient (2.9%) with heterotaxy syndrome developed complete heart block. ConclusionsThe precise anatomic location of the conduction system in patients with complex congenital heart defects can be difficult for the surgeon to accurately predict. Intraoperative conduction mapping enables localization of the His bundle and adds to our understanding of the anatomic factors associated with conduction location. Predictive modeling of conduction anatomy may build on what is already known about the conduction system and help surgeons to better anticipate conduction location preoperatively and intraoperatively.