Interaction between clinicians and artificial intelligence to detect fetal atrioventricular septal defects on ultrasound: how can we optimize collaborative performance?

OBJECTIVES: Artificial intelligence (AI) has shown promise in improving the performance of fetal ultrasound screening in detecting congenital heart disease (CHD). The effect of giving AI advice to human operators has not been studied in this context. Giving additional information about AI model workings, such as confidence scores for AI predictions, may be a way of further improving performance. Our aims were to investigate whether AI advice improved overall diagnostic accuracy (using a single CHD lesion as an exemplar), and to determine what, if any, additional information given to clinicians optimized the overall performance of the clinician-AI team. METHODS: An AI model was trained to classify a single fetal CHD lesion (atrioventricular septal defect (AVSD)), using a retrospective cohort of 121 130 cardiac four-chamber images extracted from 173 ultrasound scan videos (98 with normal hearts, 75 with AVSD); a ResNet50 model architecture was used. Temperature scaling of model prediction probability was performed on a validation set, and gradient-weighted class activation maps (grad-CAMs) produced. Ten clinicians (two consultant fetal cardiologists, three trainees in pediatric cardiology and five fetal cardiac sonographers) were recruited from a center of fetal cardiology to participate. Each participant was shown 2000 fetal four-chamber images in a random order (1000 normal and 1000 AVSD). The dataset comprised 500 images, each shown in four conditions: (1) image alone without AI output; (2) image with binary AI classification; (3) image with AI model confidence; and (4) image with grad-CAM image overlays. The clinicians were asked to classify each image as normal or AVSD. RESULTS: A total of 20 000 image classifications were recorded from 10 clinicians. The AI model alone achieved an accuracy of 0.798 (95% CI, 0.760-0.832), a sensitivity of 0.868 (95% CI, 0.834-0.902) and a specificity of 0.728 (95% CI, 0.702-0.754), and the clinicians without AI achieved an accuracy of 0.844 (95% CI, 0.834-0.854), a sensitivity of 0.827 (95% CI, 0.795-0.858) and a specificity of 0.861 (95% CI, 0.828-0.895). Showing a binary (normal or AVSD) AI model output resulted in significant improvement in accuracy to 0.865 (P < 0.001). This effect was seen in both experienced and less-experienced participants. Giving incorrect AI advice resulted in a significant deterioration in overall accuracy, from 0.761 to 0.693 (P < 0.001), which was driven by an increase in both Type-I and Type-II errors by the clinicians. This effect was worsened by showing model confidence (accuracy, 0.649; P < 0.001) or grad-CAM (accuracy, 0.644; P < 0.001). CONCLUSIONS: AI has the potential to improve performance when used in collaboration with clinicians, even if the model performance does not reach expert level. Giving additional information about model workings such as model confidence and class activation map image overlays did not improve overall performance, and actually worsened performance for images for which the AI model was incorrect. © 2024 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Predictors of post-operative left atrioventricular valve regurgitation in pediatric patients with complete atrioventricular canal defects.

BACKGROUND: In infants with complete atrioventricular canal (CAVC) defects, post-operative left atrioventricular valve regurgitation (LAVVR) is a known major cause of morbidity and mortality and a common indication for re-operation. However, there is scarce data to identify risk factors for poor outcomes. Our study aims to find echocardiographic characteristics that predict post-operative LAVVR at discharge and 1-year follow-up. METHODS: Retrospective cohort study of patients with initial CAVC repair at our hospital who were followed for 1 year between 2013 and 2022. Patients with major co-morbid conditions were excluded. Serial echocardiograms were reviewed. Anatomic details, quantitative and qualitative measure of LAVVR including the number of regurgitant jets, regurgitant jet length and vena contracta width, and ventricular function were collected. The time points measured include pre-operative transthoracic echocardiogram (TTE), post-operative transesophageal echocardiogram (PO-TEE), routine protocol based post-operative day 1 (POD1) TTE, discharge TTE and 1-year post-operative (1yPO) TTE. Paired t-tests, chi-square analysis, and linear regression analysis were performed comparing measured variables to LAVVR outcomes. RESULTS: Fifty-two patients were included; 92% had Trisomy 21. The majority were classified as Rastelli A (71%), others Rastelli C (29%). Only two patients had moderate or greater LAVVR pre-operatively. The mean age at repair was 125 ± 44 days. Pre-operative LAVVR was the only significant predictor of LAVVR severity at 1 year after backward stepwise regression. Of those with < moderate LAVVR on PO-TEE, 20% had worsening to ≥ moderate at discharge, but only 9% remained that way at 1 year. Of those with ≥ moderate LAVVR on PO-TEE, 40% improved to < moderate by 1 year. Two patients who worsened at 1 year, both secondary to likely cleft suture dehiscence. Only one patient required reoperation in the immediate post-operative period secondary to severe LAVVR due to suture dehiscence. Routine protocol-based POD1 echo did not have any association with altered outcomes. CONCLUSION: Pre-operative LAVVR was the only significant predictor of LAVVR severity at 1 year. A significant percentage (40%) of patient with ≥ moderate LAVVR on PO-TEE improved to < moderate by 1 year. Furthermore, routine protocol-based POD1 echo did not have any association with altered outcomes.

Anatomic considerations in the management of complete atrioventricular canal.

OBJECTIVE: Patients with complete atrioventricular canal have a variable clinical course prior to repair. Many patients balance their circulations well prior to elective repair. Others manifest clinically significant pulmonary over circulation early in life and require either palliative pulmonary artery banding or complete repair. The objective of this study was to assess anatomic features that impact the clinical course of patients. METHODS: In total, 222 patients underwent complete atrioventricular canal repair between 2012 and 2022 at a single institution. Twenty-seven (12%) patients underwent either pulmonary artery banding (n = 15) or complete repair (n = 12) at less than 3 months of age (Group 1). The remaining 195 (88%) underwent repair after 3 months of age (Group 2). Patient records and imaging were reviewed. RESULTS: The median post-operative length of stay following complete repair was 25 [7,46] days for those patients in Group 1 and 7 [5,12] days for those in Group 2 (p < 0.0001). There was relative hypoplasia of left-sided structures in Group 1 versus Group 2. Mean z-score for the ascending aorta was -1.2 (±0.8) versus -0.3 (±0.9) (p < 0.0001), the aortic isthmus was -2.1 (±0.8) versus -1.4 (±0.8) (p = 0.005). The pulmonary valve to aortic valve diameter ratio was median 1.47 [1.38,1.71] versus 1.38 [1.17,1.53] (p 0.008). CONCLUSIONS: Echocardiographic evaluation of the systemic and pulmonary outflow of patients with complete atrioventricular canal may assist in predicting the clinical course and need for early repair vs pulmonary artery banding.

Efficacy of atrioventricular valve regurgitation in the first trimester for the diagnosis of atrioventricular septal defect.

PURPOSE: To investigate the efficacy of atrioventricular valve regurgitation (AVVR) for predicting atrioventricular septal defect (AVSD) in the first trimester. METHODS: We performed a prospective observational study, screening for complicated congenital heart diseases and AVVR in fetuses at 11 to 13+6 weeks of gestation by advanced dynamic flow in four-chamber view and three-vessel-trachea view. RESULTS: 43 549 fetuses at 11 to 13+6 weeks of gestation were screened by echocardiography, of which 37 cases were diagnosed with AVSD, including complete AVSD (31 cases), intermediate AVSD (3 cases) and partial AVSD (1 cases), undiagnosed intermediate AVSD (2 cases), and misdiagnosed case (2 cases). AVVR was observed in 34 cases (34/37) in the first trimester, 59. 46% (22/37) nuchal translucency greater than 95th percentile, 29. 73% (11/37) absence of nasal bone, 32. 43% (12/37) ductus venosus A wave inversion, and 40. 54% (15/37) had tricuspid regurgitation. The sensitivity of common AVVR in predicting AVSD is better than other ultrasonic indexes. CONCLUSIONS: AVVR can be used as an ultrasonic indicator to predict AVSD in the first trimester, which is beneficial to detect AVSD.

A case of a complete atrioventricular canal defect in a ferret.

BACKGROUND: Atrioventricular canal defect is a rare congenital disorder of the heart and describes the presence of an atrial septal defect, a variable presentation of ventricular septal alterations including ventricular septal defect malformations in the mitral and tricuspid valves. The defect has been described in human beings, dogs, cats, pigs, and horses. CASE PRESENTATION: This paper describes the case of a complete atrioventricular canal defect in a four-year-old intact male pet ferret (Mustela putorius furo), which was presented due to posterior weakness, ataxia, and decreased appetite. A loud systolic murmur, dyspnea, and hind limb paraparesis were detected during the clinical examination. Thoracic radiographs showed generalized cardiomegaly and lung edema. ECG showed sinus rhythm with prolonged P waves and QRS complexes. Echocardiography showed a large atrial septal defect, atrioventricular dysplasia, and a ventricular septal defect. Palliative treatment with oxygen, furosemide, spironolactone, enalapril, diltiazem, and supportive care was chosen as the therapy of choice. The ferret recovered gradually during hospitalization. A follow-up examination at three and six months showed stabilization of cardiac function. CONCLUSIONS: To the authors knowledge, this is the first time an atrioventricular canal defect has been described in a pet ferret.

Long-Term Outcome Up To 40 Years after Single Patch Repair of Complete Atrioventricular Septal Defect in Infancy or Childhood.

OBJECTIVES: Patients with repaired complete atrioventricular septal defect (CAVSD) represent an increasing portion of grown-ups with congenital heart disease. For repair of CAVSD, the single-patch technique has been employed first. This technique requires division of the bridging leaflets, thus, among other issues, long-term function of the atrioventricular valves is of particular concern. METHODS: Between 1978 and 2001, 100 consecutive patients with isolated CAVSD underwent single-patch repair in our institution. Hospital mortality was 11%. Primary endpoints were clinical status, atrioventricular valve function, and freedom from reoperation in long term. Follow-up was obtained contacting the patient and/or caregiver, and the referring cardiologist. RESULTS: Eighty-three patients were eligible for long-term follow-up (21.0 ± 8.7, mean ± standard deviation [21.5; 2.1-40.0, median; min-max] years after surgical repair). Actual long-term mortality was 3.4%. Quality of life (QoL; self- or caregiver-reported in patients with Down syndrome) was excellent or good in 81%, mild congestive heart failure was present in 16%, moderate in 3.6% as estimated by New York Heart Association classification. Echocardiography revealed normal systolic left ventricular function in all cases. Regurgitation of the right atrioventricular valve was mild in 48%, mild-moderate in 3.6%, and moderate in 1.2%. The left atrioventricular valve was mildly stenotic in 15% and mild to moderately stenotic in 2%; regurgitation was mild in 54%, mild to moderate in 13%, and moderate in 15% of patients. Freedom from left atrioventricular-valve-related reoperation was 95.3, 92.7, and 89.3% after 5, 10, and 30 years, respectively. Permanent pacemaker therapy, as an immediate result of CAVSD repair (n = 7) or as a result of late-onset sick sinus syndrome (n = 5), required up to six reoperations in single patients. Freedom from pacemaker-related reoperation was 91.4, 84.4, and 51.5% after 5, 10, and 30 years, respectively. CONCLUSION: Up to 40 years after single-patch repair of CAVSD, clinical status and functional results are promising, particularly, in terms of atrioventricular valve function. Permanent pacemaker therapy results in a life-long need for surgical reinterventions.

Atrioventricular Septal Defects: Pathology, Imaging, and Treatment Options.

PURPOSE OF REVIEW: Atrioventricular septal defects (AVSD) represent a broad spectrum of congenital anomalies from simple to the most complex heart defects including some distinct types. Clinical presentation and timing of intervention differ by morphological subset and functional anatomy. Herein, we review morphological variations and characteristics that determine appropriate intervention and provide insights into functional anatomy based on detailed three-dimensional (3D) assessment of AVSDs. RECENT FINDINGS: The understanding of functional morphology of AVSDs has improved significantly with detailed 3D echocardiographic evaluation of the atrioventricular junction and valve morphology. As prenatal detection of AVSDs has increased significantly, it has become the most common fetal cardiac diagnosis enabling antenatal counseling and delivery planning. Advances in diagnosis and perioperative care have resulted in optimal outcomes. The diagnosis and management of AVSDs have improved over the years with enhanced understanding of anatomy and perioperative care resulting in optimal short and long-term outcomes.

Accuracy of Fetal Echocardiography in Defining Pulmonary Artery Anatomy and Source of Pulmonary Blood flow in Pulmonary Atresia with Ventricular Septal Defect (PA/VSD).

Precise delineation of central and branch pulmonary artery anatomy, patent ductus arteriosus, and major aorto-pulmonary collateral artery anatomy in the fetal diagnosis of pulmonary atresia with ventricular septal defect is challenging but important to prenatal counseling and postnatal management. We aimed to evaluate the accuracy of fetal echocardiography to determine these anatomical nuances in pulmonary atresia with ventricular septal defect. This was a retrospective, single-institution, 10-year chart review of consecutive prenatal diagnosis of pulmonary atresia with ventricular septal defect for assessment of pulmonary artery, patent ductus arteriosus, and major aorto-pulmonary collateral artery anatomy and comparison with postnatal imaging including echocardiography, cardiac catheterization, and computerized tomography angiography. Twenty-six fetuses were diagnosed with pulmonary atresia with ventricular septal defect during the review period and complete postnatal follow-up was available in 18, all confirming the basic prenatal diagnosis. Fetal echocardiography accurately predicted central and branch pulmonary artery anatomy in 16 (89%) [confluent in 14, discontinuous in 2], patent ductus arteriosus status in 15 (83%) [present in 10, absent in 5], and major aorto-pulmonary collateral arteries in 17 (94%) [present in 9, absent in 8]. Accuracy increased to 100% for pulmonary artery anatomy (16/16) and major aorto-pulmonary collateral artery (17/17) when excluding patients whose anatomy was reported as uncertain on fetal echocardiography. Fetal echocardiography can provide accurate anatomical details in the vast majority of fetuses with pulmonary atresia with ventricular septal defect. This allows for more anatomy-specific counseling, prognostication, and improved selection of postnatally available management options.

Can Left Atrioventricular Valve Reduction Index (LAVRI) Predict the Surgical Strategy for Repair of Atrioventricular Septal Defect?

Despite improved survival, surgical treatment of atrioventricular septal defect (AVSD) remains challenging. The optimal technique for primary left atrioventricular valve (LAVV) repair and prediction of suitability for biventricular approach in unbalanced AVSD are still controversial. We evaluated the ability of our recently developed echocardiographic left atrioventricular valve reduction index (LAVRI) in predicting LAVV reoperation rate and surgical strategy for unbalanced AVSD. Retrospective echocardiographic analysis was available in 352 of 790 patients with AVSD treated in our institution and included modified atrioventricular valve index (mAVVI), ventricular cavity ratio (VCR), and right ventricle/left ventricle (RV/LV) inflow angle. LAVRI estimates LAVV area after complete cleft closure and was analyzed with regard to surgical strategy in primary LAVV repair and unbalanced AVSD. Of the entire cohort, 284/352 (80.68%) patients underwent biventricular repair and 68/352 (19.31%) patients underwent univentricular palliation. LAVV reoperation was performed in 25/284 (8.80%) patients after surgical correction of AVSD. LAVRI was significantly lower in patients requiring LAVV reoperation (1.92 cm2/m2 [IQR 1.31] vs. 2.89 cm2/m2 [IQR 1.37], p = 0.002) and significantly differed between patients receiving complete and no/partial cleft closure (2.89 cm2/m2 [IQR 1.35] vs. 2.07 cm2/m2 [IQR 1.69]; p = 0.002). Of 82 patients diagnosed with unbalanced AVSD, 14 were suitable for biventricular repair (17.07%). mAVVI, LAVRI, VCR, and RV/LV inflow angle accurately distinguished between balanced and unbalanced AVSD and predicted surgical strategy (all p < 0.001). LAVRI may predict surgical strategy in primary LAVV repair, LAVV reoperation risk, and suitability for biventricular approach in unbalanced AVSD anatomy.

Characteristics of the pulmonary circulation in infants with complete atrioventricular septal defect.

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.

Common atrium/atrioventricular canal defect and postaxial polydactyly: A mild clinical subtype of Ellis-van Creveld syndrome caused by hypomorphic mutations in the EVC gene.

Clinical expression of Ellis-van Creveld syndrome (EvC) is variable and mild phenotypes have been described, including patients with mostly cardiac and limb involvement. Whether these cases are part of the EvC phenotypic spectrum or separate conditions is disputed. Herein, we describe a family with vertical transmission of atrioventricular canal defect (AVCD), common atrium, and postaxial polydactyly. Targeted sequencing of EVC, EVC2, WDR35, DYNC2LI1, and DYNC2H1 identified different compound heterozygosity in EVC genotypes in the two affected members, consisting of a nonsense (p.Arg622Ter) and a missense (p.Arg663Pro) variant in the father, and the same nonsense variant and a noncanonical splice-site in-frame change (c.1316-7A>G) in the daughter. Complementary DNA sequencing, immunoblot, and immunofluorescence experiments using patient-derived fibroblasts and Evc-/- mouse embryonic fibroblasts showed that p.Arg622Ter is a loss-of-function mutation, whereas p.Arg663Pro and the splice-site change c.1316-7A>G are hypomorphic variants resulting in proteins that retain, in part, the ability to complex with EVC2. Our molecular and functional data demonstrate that at least in some cases the condition characterized as "common atrium/AVCD with postaxial polydactyly" is a mild form of EvC due to hypomorphic EVC mutations, further supporting the occurrence of genotype-phenotype correlations in this syndrome.

Short to medium term outcomes of right ventricular outflow tract stenting as initial palliation for symptomatic infants with complete atrioventricular septal defect with associated tetralogy of Fallot.

OBJECTIVES: To assess the impact of right ventricular outflow tract (RVOT) stenting as the primary palliation in infants with complete atrioventricular septal defect with associated tetralogy of Fallot (cAVSD/TOF). BACKGROUND: Historically, palliation of symptomatic patients with cAVSD/TOF has been achieved through surgical systemic to pulmonary artery shunting. More recently RVOT stenting has evolved as an acceptable alternative in patients with tetralogy of Fallot. METHODS: Retrospective review of all patients with cAVSD/TOF who underwent RVOT stenting as palliation over a 13-year period from two large tertiary referral centers. RESULTS: Twenty-six patients underwent RVOT stenting at a median age of 57 days (interquartile range [IQR] 25.5-106.5). Median weight for stent deployment was 3.7 kg (IQR 2.91-5.5 kg). RVOT stenting improved oxygen saturations from a median of 72% (IQR 70-76%) to 90% (IQR 84-92%), p < .001. There was a significant increase in the median Z-score for both branch pulmonary arteries at median follow-up of 255 days (IQR 60-455). Eight patients required RVOT stent balloon dilatations and 8 patients required re-stenting for progressive desaturation. The median duration between reinterventions was 122 days (IQR 53-294 days). Four patients died during the follow-up period. No deaths resulted from the initial intervention. To date, definitive surgical intervention was achieved in 19 patients (biventricular repair n = 15) at a median age of 369 days (IQR 223-546 days). CONCLUSION: RVOT stenting in cAVSD/TOF is a safe and effective palliative procedure in symptomatic infants, promoting pulmonary artery growth and improving oxygen saturations.

Prediction of pulmonary pressure after Glenn shunts by computed tomography-based machine learning models.

OBJECTIVES: This study aimed to develop non-invasive machine learning classifiers for predicting post-Glenn shunt patients with low and high risks of a mean pulmonary arterial pressure (mPAP) > 15 mmHg based on preoperative cardiac computed tomography (CT). METHODS: This retrospective study included 96 patients with functional single ventricle who underwent a bidirectional Glenn procedure between November 1, 2009, and July, 31, 2017. All patients underwent post-procedure CT, followed by cardiac catheterization. Overall, 23 morphologic parameters were manually extracted from cardiac CT images for each patient. The Mann-Whitney U or chi-square test was applied to select the most significant predictors. Six machine learning algorithms including logistic regression, Naive Bayes, random forest (RF), linear discriminant analysis, support vector machine, and K-nearest neighbor were used for modeling. These algorithms were independently trained on 100 train-validation random splits with a 3:1 ratio. Their average performance was evaluated by area under the curve (AUC), accuracy, sensitivity, and specificity. RESULTS: Seven CT morphologic parameters were selected for modeling. RF obtained the best performance, with mean AUC of 0.840 (confidence interval [CI] 0.832-0.850) and 0.787 (95% CI 0.780-0.794); sensitivity of 0.815 (95% CI 0.797-0.833) and 0.778 (95% CI 0.767-0.788), specificity of 0.766 (95% CI 0.748-0.785) and 0.746 (95% CI 0.735-0.757); and accuracy of 0.782 (95% CI 0.771-0.793) and 0.756 (95% CI 0.748-0.764) in the training and validation cohorts, respectively. CONCLUSIONS: The CT-based RF model demonstrates a good performance in the prediction of mPAP, which may reduce the need for right heart catheterization in post-Glenn shunt patients with suspected mPAP > 15 mmHg. KEY POINTS: • Twenty-three candidate descriptors were manually extracted from cardiac computed tomography images, and seven of them were selected for subsequent modeling. • The random forest model presents the best predictive performance for pulmonary pressure among all methods. • The computed tomography-based machine learning model could predict post-Glenn shunt pulmonary pressure non-invasively.

Initial fetal to initial postnatal echocardiogram in uncomplicated atrioventricular septal defects: Do significant changes occur?

BACKGROUND: Yield of serial echocardiography in fetuses with atrioventricular septal defects (fAVSD) has not been well defined. The goal of this study was to document if any major changes occurred from initial fetal to initial postnatal echocardiogram in uncomplicated fAVSD. METHODS: fAVSD were excluded if initial fetal scan documented complex CHD or any concerns. Changes in ventricular function, valvular regurgitation, or diagnosis between first fetal and first postnatal echocardiogram were recorded. RESULTS: Fifty-seven fAVSD met criteria. Ninety-six fetal echocardiograms were done in 57 patients. Initial fetal scan was performed at 24.3 ± 3.7 weeks of estimated gestational age. All fAVSD had normal function, 38 had no atrioventricular valve regurgitation (AVVR), and 19 had mild AVVR. First postnatal echocardiogram was performed at 6.3 ± 15.3 days. Fifty-six patients had normal function, 1 patient had mild dysfunction, 16 patients had no AVVR, 36 had mild AVVR, and 5 had moderate AVVR. Three patients (5%) had an improvement in AVVR by one degree, 27 patients (47%) had no change in AVVR, 24 patients (42%) had an increase in AVVR by one degree, and 3 patients (5%) had an increase in AVVR by two degrees. There was no major missed anatomical diagnosis from first prenatal to first postnatal echocardiogram. CONCLUSION: In fAVSD that had no concerns on their initial fetal echocardiogram, the majority of patients had no major changes noted between their initial fetal echocardiogram and their first postnatal echocardiogram. Repeat fetal echocardiograms may not necessarily be needed in this cohort of patients.

Echocardiographic assessment of atrioventricular canal defects.

AV canal defects (AVCD) are caused by maldevelopment of the endocardial cushions and typically include a primum atrial septal defect (ASD), an inlet ventricular septal defect (VSD), and a common atrioventricular valve. The variations in deformities provide the basis for the many terms used in the anatomical classifications: partial, transitional, intermediate, and complete common AVCD (balanced or unbalanced). The balanced complete common AVCDs are classified as Rastelli A, B, C depending on the anomaly of the anterior bridging leaflet division and attachments. Unbalanced complete AVCDs occur when the common AV valve leads primarily into the RV or LV. Echocardiographic apical, subcostal, and parasternal views are the best views to image AV canal defects. These views can help determine the type of repair required for the various AV canal defects.

Imaging characteristics and associations in twisted atrioventricular connections on multidetector computed tomography angiography.

AIM: To evaluate the imaging characteristics and associations in patients with twisted atrioventricular connections on multidetector computed tomography (CT) angiography. MATERIAL AND METHODS: We retrospectively reviewed 2605 CT angiography studies performed for suspected congenital heart diseases in our institution from January 2014 to December 2018. Twisted atrioventricular connections were diagnosed in 12 patients. Segmental sequential approach developed by Anderson et al was employed to characterize the complex congenital heart disease. Ventricular topology was also determined. CTA was also assessed to look for any associated intra- and extracardiac anomalies. RESULTS: Out of 12 patients with twisted atrioventricular connections, usual viscero-atrial arrangement was seen in nine patients, two patients had mirror-imaged viscero-atrial arrangement and one patient had mirror-imaged atrial arrangement. Right-sided heart was seen in four patients. Two patients had discordant atrioventricular connections, seven had concordant atrioventricular connections while three showed double-inlet right ventricle. Ventriculoarterial connections were concordant in two patients, discordant in one patient while nine patients had double-outlet right ventricle. Superior-inferior ventricular morphology with near-horizontal interventricular septum was seen in four patients. Left-sided aortic arch with normal branching pattern was observed in nine patients. No coronary anomaly was seen in any patient. Other associations included ventricular and atrial septal defects, pulmonic stenosis, hypoplasia/atresia of tricuspid valve, straddling of mitral valve, and duplicated superior caval veins. CONCLUSION: CT angiography is useful in diagnosis of twisted atrioventricular connections with accurate identification of viscero-atrial arrangement, atrioventricular and ventriculoarterial connections, and orientation and presence or absence of associated anomalies.

Absent right superior vena cava with left superior vena cava draining into unroofed coronary sinus with common atrium in a child with an atrioventricular septal defect.

The association of absent right superior vena cava and persistent left superior vena cava draining into unroofed coronary sinus with common atrium and the atrioventricular septal defect is an extremely rare form of the congenital cardiac disorder with only one case reported so far, hence, can be missed preoperatively if not carefully looked for. Failure to detect absent right superior vena cava beforehand may otherwise pose difficulties in carrying out invasive surgical or medical interventions.

Edwards Sapien 3 transcatheter aortic valve implantation for management of severe aortic regurgitation in a teenage patient with corrected atrioventricular septal defect and progressive left ventricular dysfunction.

Transcatheter aortic valve replacement is mostly performed in elderly patients with severely calcified aortic valves. There are few reports about its use for pure aortic regurgitation, few reports about its use in adolescent patients and to the best of our knowledge no report about the use of an Edwards Sapien valve in the aortic position in an underage patient after surgically corrected congenital heart disease (CHD). Decompensation of a complex CHD can be difficult to manage and may require unusual solutions. We report a case of a teenage patient presenting with progressive aortic regurgitation and deterioration of left ventricular function after multiple surgical procedures for an atrioventricular septal defect (AVSD). As "bridge-to-transplant," we performed a transcatheter aortic valve implantation using a balloon-expandable Sapien 3 prosthesis. At 6 month follow-up, the patient remained clinically stable with no rehospitalization due to heart failure.

Imaging of Pulmonary Atresia With Ventricular Septal Defect.

The aim of this article was to review computed tomography angiography and magnetic resonance angiography of pulmonary atresia with ventricular septal defect. This disorder is a rare complex congenital heart disease. Preoperative imaging of pulmonary atresia with ventricular septal defect with computed tomography angiography and magnetic resonance angiography is important for complete anatomical delineation and planning for treatment. Preoperative imaging used for assessment of the main pulmonary artery (its size, valve, and confluence), aortopulmonary collaterals (its origin, insertion, course, and size), presence of patent ductus arteriosus, other sources of collaterals as bronchial and coronary arteries, and pattern of pulmonary arborization. Imaging can detect associated aortic, pulmonary venous and coronary anomalies, and other congenital heart disease. Postoperative imaging after unifocalization and stent is for assessment of patency, stenosis, and occlusion of stent or perivascular lesions as seroma.

Electro-vectorcardiographic demonstration of bifascicular block associated with ventricular preexcitation.

Down syndrome occurs more frequently in the offsprings of older pregnant women and may be associated with atrioventricular septal defect. This refers to a broad spectrum of malformations characterized by a deficiency of the atrioventricular septum and abnormalities of the atrioventricular valves caused by an abnormal fusion of the superior and inferior endocardial cushions with the midportion of the atrial septum and the muscular portion of the ventricular septum.