A surgical approach of an unusual variant of complete atrioventricular defect; A case report

Background Complete atrioventricular canal is a congenital heart defect that is characterized by an atrial septal defect, ventricular septal defect, and a common atrioventricular valve. Standard surgical techniques for repairing complete atrioventricular canal defect mainly includes repairing the defect with a single patch, a double patch, or with the modified single patch technique. Case presentation This paper presents a novel surgical repair technique of an unusual anatomical presentation for a complete atrioventricular canal defect in a patient with Down syndrome. Conclusions Unusual anatomical variant for congenital heart defects occurs frequently, which gives surgeons real opportunities to innovate surgical approaches. This patient was an example of an unusual anatomical presentation for complete atrioventricular canal, and the surgical technique used for this patient was novel. Follow up for these patients is mandatory for long term results.

Antenatal Diagnosis of a Partial Atrioventricular Canal with Ebstein’s Anomaly

The simultaneous occurrence of an atrioventricular canal defect (AVCD) and Ebstein’s anomaly is extremely rare, occurring in less than 0.5% of all patients with AVCD. Only 22 cases are described in the literature. This patient’s antenatal diagnosis of both Ebstein’s anomaly and partial AVCD was made at 25 weeks of gestation. The delivery was organized in a tertiary center. The initial neonatal course was difficult but with adequate treatment, a rapid improvement allowed for a gap of almost 2 years before a complete surgical repair including a cone tricuspid plasty. To our knowledge, this is the first case of antenatal diagnosis, with carefully tailored delivery, neonatal care and subsequent follow-up before indication for successful surgery.

EFFECTIVE CORRECTION OF THE COMMON ATRIOVENTRICULAR CANAL WITH TETRALOGY OF FALLOT AND HYPOPLASIA OF THE RIGHT VENTRICLE IN A CHILD

The paper presents a description of a case of successful one and a half ventricular correction of a complex congenital cardiac anomalies, including the common atrioventricular canal, tetralogy of Fallot, and hypoplasia of the right ventricle in a child (2 year and 4 months). Ararecongenitalanomalycharacterized by acombinationof intracardiac defects, required an innovative surgical approach, the exclusivity of this clinical case has determined. The surgical strategy of complete atrioventricular canal defect is determined by a number of factors. One of the most important is balance between right and left ventricular outputs. The balanced type with adequate development of the ventricles involves the biventricular repair performance. Unbalancedatrioventricular canal defects includea hypoplastic ventricle. Reconstructive surgeryfor onehypoplasticventricle is oriented towards the degree of hypoplasia. In particular, a mild right ventricular hypoplasia allows perfoming biventricular radical operation while a severe degree suggests univentricular repair. In case of borderline, moderate degree of hypoplastic right ventricle the one and half ventricle repair can be carried out. Another important point is the presence of concomitant pulmonic stenosis required the choice of optimal method and material for reconstruction which is not always obvious and often it is the subject of debates. This report presents a description of the diagnostic stages with an emphasis on determining the type of balance of the common atrioventricular canal, the degree of hypoplasia of the right ventricle, the approach to choosing the optimal method for correcting the defect in general and the material for reconstructing the outflow pathway from the right ventricle in particular, as well as the subsequent successful correction of congenital multicomponent cardiac abnormalities in a young patient.

Impact of COVID-19 in adult patients with congenital heart disease

Background COVID-19 is responsible for a worldwide pandemic, causing more than 13 000 deaths to date in Portugal. Data already exists regarding the increased risk of adverse events in patients with cardiovascular diseases, however the impact of SARS-CoV-2 infection in patients (P) with congenital heart disease (CHD) is still under investigation. Aims To evaluate the impact of COVID-19 in adult patients with congenital heart disease in our tertiary centre Methods Adult patients seen at the CHD outpatient's clinic at a tertiary centre, who became infected with SARS-CoV-2 infection up to February 2021 were included. Assessment of patients' symptoms, need for hospitalization and admission in an intensive care unit was assessed based on medical records. Results We identified 36 patients (pts) with COVID-19 infection. Symptoms were present in 31 (86%). The median age was 39 (32–49) years, 58% were females. Seven P (19%) had complex cyanotic disease; three (8%) Tetralogy of Fallot; three (8%) transposition of great arteries (one after Senning procedure and 2 after arterial switch); six (14%) right ventricle obstacle; two (8%) atrioventricular canal defect; four (11%) atrial septal defect; five (14%) ventricular septal defect; five (14%) aortic coarctation; two aortopathies (one submitted do David procedure); one subaortic stenosis; two (6%) had Eisenmenger syndrome. The majority (61%) of P had previous surgery and 58% were at New York Heart Association class of I. Mild symptoms were reported by 24 P (67%). Seven adults experienced moderate symptoms (dyspnea and hypoxia) that led to hospitalization for oxygen therapy, although none required mechanical ventilation. One death was reported. There was a significant association between the gravity of CHD and hospitalizations (p=0.012). Conclusion Our pts had mainly mild to moderate symptoms and did not appear to have a disproportionately negative outcome; the need for hospitalization was more frequent in patients with higher CHD gravity. These findings are in line with the emerging data regarding COVID-19 in CHD P, and may be in part explained by the patient's young age and functional status. FUNDunding Acknowledgement Type of funding sources: None.

The effect of Surgical Technique, Age and Trisomy 21 on early outcome of Surgical Management of Complete Atrioventricular Canal Defect

Background The optimal timing, surgical technique and the influence of Down syndrome on outcome of surgical repair of Complete Atrio-Ventricular Canal Defect (CAVC) remains uncertain. We reviewed our experience in repair of CAVC to identify the influence of these factors on operative outcome. Methods A prospective study included 70 patients who underwent repair of CAVC at Ain Shams university hospitals, academy of cardiothoracic surgery during the period from July 2016 to October 2019. Age at surgery (less than 6 months old versus later), surgical technique used [(modified single patch versus double patch technique), (use of posterior annuloplasty for repair of left AV valve or not)] and association of Down syndrome were evaluated for their impact on the outcome of surgical repair using multivariate analysis. Patients were followed up for 6 months; primary end point was mortality and secondary end point was reappearance of LAVV regurgitation. Results No significant difference between patients operated on, at the first 6 months of age versus later, regarding mortality or LAVV regurgitation. Down patients showed significant difference in the occurrence of postoperative compared to non-Down patients (LAVVR grade 2 + = 8.9% vs 24%, P value =0.005) respectively. Surgical repair by Modified single patch technique showed significant reduction in cross clamp time (mean = 47.6 ± 9.227 min vs 73.55 ± 21.087 min, P value 0.00), shorter bypass time (mean = 71.13 ± 13.507 min vs 99.19 ± 27.092 min, P value =0.00) and shorter duration of ICU stay (mean =3.2 ± 1.657 days vs 5.3 ± 2.761 days, P value=0.01) as compared to double patch technique. Posterior annuloplasty used for repair of LAVV compared to closure of cleft only resulted in significant reduction in the occurrence of post-operative valve regurgitation during the early period (LAVVR 2+ 43% vs 7%, P value=0.03) and at 6 months of follow up. (LAVVR 2+ 35.4% vs 0%, P value=0.01) respectively. Conclusion early intervention, in the first 6 months in patients with CAVC by surgical repair gives comparable acceptable results to later repair, Down syndrome was not found to be a risk factor for early intervention. Modified single patch and double patch techniques for repair, can be used both with comparable results even in large VSD component (8mm and larger), finally, repair of common AV valve by cleft closure with posterior LAV annulplasty showed better results with significant decrease in postoperative LAV regurgitation and early mortality in comparison to closure of cleft only.

The effect of surgical technique, age, and Trisomy 21 on early outcome of surgical management of complete atrioventricular canal defect

Background: The optimal timing, surgical technique, and the influence of Trisomy 21 on the outcome of surgical repair of Complete Atrioventricular Canal Defect remains uncertain. We reviewed our experience in the repair of CAVC to identify the influence of these factors on operative outcomes. Methods: A prospective study included 70 patients, who underwent repair of CAVC at our institute between July, 2016 and October, 2019. Primary endpoint was mortality and the secondary endpoint was a degree of left atrioventricular valve regurgitation. Results: No significant difference was noted between patients operated on, at the first 6 months of age versus later, regarding mortality or LAVV regurgitation. Surgical repair by modified single-patch technique showed a significant reduction in bypass time (71.13 ± 13.507 min versus 99.19 ± 27.092 min, p-value = 0.001). Compared to closure of cleft only, posterior annuloplasty used for repair of LAVV resulted in significant reduction in the occurrence of post-operative valve regurgitation during the early period (LAVV 2 + 43 versus 7 %, p-value = 0.03) and at 6 months of follow-up (LAVV 2 + 35.4 versus 0 %, p-value = 0.01), respectively. Conclusions: Early intervention, in the first 6 months in patients with CAVC by surgical repair gives comparable acceptable results to later repair; Trisomy 21 was not found to be a risk factor for early intervention. Repair of common AV valve by cleft closure with posterior LAVV annuloplasty showed better results with a significant decrease in post-operative LAVV regurgitation and early mortality in comparison to the closure of cleft only.

Can Fetal Echocardiographic Measurements of the Left Ventricular Outflow Tract Angle Detect Fetuses with Conotruncal Cardiac Anomalies?

Objectives: The angle between the inter-ventricular septum and the ascending aorta can be measured during a sonographic fetal survey while viewing the left ventricular outflow tract (LVOT angle). Our aim was to compare the LVOT angle between fetuses with and without conotruncal cardiac anomaliesrmations. Methods: In this prospective observational study, we compared the LVOT angle between normal fetuses, at different gestational age, and fetuses with cardiac malformations. Results: The study included 302 fetuses screened at gestational age of 12–39 weeks. The LVOT angle ranged from 127 to 163 degrees (mean 148.2), in 293 fetuses with normal hearts, and was not correlated with gestational age. The LVOT angle was significantly wider in fetuses with D-transposition of the great arteries (D-TGA, eight fetuses) and valvar aortic stenosis (AS, three fetuses), than in fetuses with normal hearts (164.8 ± 5.0 vs. 148.2 ± 5.4, respectively, p < 0.001). Conversely, the LVOT angle was significantly narrower in fetuses with complete atrioventricular canal defect (AVC, eight fetuses), than in fetuses with normal hearts (124.8 ± 2.4 vs. 148.2 ± 5.4, respectively, p < 0.001). On ROC analysis, an angle of 159.6 degrees or higher had a sensitivity of 100% and a specificity of 97.3% for the detection of TGA or AS, whereas an angle of 128.8 degrees or lower had a sensitivity of 100% and a specificity of 99.7% for the detection of AVC defect. Conclusions: The LVOT angle is constant during pregnancy, and differs significantly in fetuses with TGA/AS, and AVC, compared to fetuses with normal hearts (wider and narrower, respectively).

Post-operative course of pulmonary artery pressure after complete atrioventricular canal defect repair

Complete atrioventricular canal defect is a CHD associated with intra-cardiac shunting of blood, which can lead to irreversible changes in pulmonary vascular resistance and pulmonary artery hypertension if unrepaired. Patients with Trisomy 21 are at risk for early development of pulmonary artery hypertension if left uncorrected.1,2 Objectives: The purpose of this study is to describe the evolution of pulmonary artery hypertension after repair of complete atrioventricular canal defect and to determine the time to normalisation of pulmonary artery pressure in both patients with and without Trisomy 21. Methods: This is a single centre, retrospective analysis of patients with complete atrioventricular canal defect admitted for surgical repair at the University of Maryland Medical Center from 2005 to 2015. T-test or Mann–Whitney test and Chi-Square or Fisher’s exact tests were used to compare the two groups (patients with Trisomy 21 and those without). Repeated measures of analysis of variance and serial measurement analysis were performed. Results: Twenty-nine patients meeting the inclusion criteria underwent repair of complete atrioventricular defects during the study period. The right ventricular pressure estimate remained elevated over time and did not show a significant difference between the two groups. Right ventricular to systolic blood pressure ratios for all patients remained > 0.5 over the time periods assessed. Conclusions: Our study suggests that in patients with complete atrioventricular canal defects, the right ventricular pressure remains elevated and does not normalise on echocardiograms performed up to one year after surgery, suggesting a sustained elevation in pulmonary vascular resistance.