Unbalanced atrioventricular septal defect with dominant right ventricle: diagnostic criteria, indications for biventricular correction, and results. A clinical observation series

Relevance: Surgical treatment for congenital heart disease (CHD) with borderline left ventricle (LV) dimensions is one of the most challenging issues in current pediatric cardiac surgery. Despite the prevailing general trend to the expansion of indications for biventricular repair of an unbalanced atrioventricular septal defect (AVSD) with a dominant right ventricle (RV), the diagnostic criteria for imbalance, feasibility of radical repair and its functional results are intensively discussed in the literature.Aim: To present quantitative criteria for the assessment of the LV size which could serve as an indication for biventricular repair of the right dominant unbalanced AVSD.Materials and methods: We present a series of 4 patients with AVSD and dominant RV, who underwent radical repair of the defect. The mean age of the patients was 2.4 years, and 3 of 4 had concomitant CHD: tetralogy of Fallot, double outlet right ventricle with pulmonary artery stenosis, and moderate hypoplasia of the aortic arch with subaortic stenosis. Three of 4 patients had previously undergone palliative interventions: two of them – pulmonary bending, one, the first stage of univentricular repair (atrioseptostomy, cavopulmonary anastomosis). Two children had been diagnosed with Down syndrome. The mean left ventricular end diastolic diameter (LVEDD) at baseline was 17.9 mm, corresponding to Z score of -5.4.Results: All four patients underwent biventricular repair of the defect with a two-patch technique. In one case, it was supplemented with placement of the pulmonary trunk homograft prosthesis, in the other with pulmonary valve commissurotomy and cavopulmonary anastomosis due to moderate tricuspid valve stenosis and in the third case the mitral valve was replaced. In 3 patients pacemaker implantation was performed. The criterion for feasibility of complete septation was the ratio of the longitudinal dimensions of the left and right ventricles (LAR > 0.7), confirmed by computed tomography performed before the surgery. The mean LVEDD after surgery was 28.3 mm, corresponding to Z score of -0.8. At the time of the hospital discharge, the left and right atrioventricular valves insufficiency did not exceed 2 and 1 degrees, respectively.Conclusion: Computed tomography allows for accurate measurement and comparison of the longitudinal dimensions of the heart ventricles with LAR index as a tool for assessment of the LV size before the biventricular repair. An additional prospective study is required to objectify the data obtained and identify predictors of an unfavorable outcome of radical repair in patients with right dominant unbalanced AVSD.

Mid Term Results of Fenestrated Intra-Extracardiac Fontan Procedure: Insights, Experiences and Expectations

Background: Intraextracardiac Fontan procedure aimed to combine the advantages of lateral tunnel and extracardiac conduit modifications of the original technique. Herein, we present our experience in our patients with intraextracardiac fenestrated Fontan Procedure. Methods: A retrospective analysis was performed in order to evaluate intraextracardiac fenestrated Fontan patients between 2014 and 2021. Seventeen patients were operated on with a mean age and body weight of 9.1 ± 5.5 years and 28.6 ± 14.6 kg. Results: Sixteen patients (94%) were palliated as univentricular physiology with hypoplasia of one of the ventricles. One patient (6%) with well-developed two ventricles with double outlet right ventricle and complete atrioventricular septal defect had straddling of the chordae prohibiting a biventricular repair. All of the patients had cavopulmonary anastomosis prior to Fontan completion, except one case. Fenestration was performed in all cases. Postoperative mean pulmonary artery pressures and arterial oxygen saturation levels at follow up were 10 ± 2.4 mmHg and 91.3 ± 2.7 %, respectively. Mean duration of pleural drainage was 5.4 ± 2.3 days. All of the fenestrations are patent at a mean follow up period of 4.8 ± 7.7 years, except one case. Any morbidity and mortality were not encountered. Conclusions: The mid-term results of intraextracardiac fenestrated Fontan procedure are encouraging. This procedure may improve the results in a patient population who should be palliated as univentricular physiology, especially in cases with complex cardiac anatomy.

First Harvest of Pediatric and Congenital Heart Surgery Multicenter Database in Turkey: Novel Application of Real-Time Online Reporting

Objectives: A new congenital heart surgery database (CKCV) with real-time online reporting function was recently developed in Turkey. All standard international parameters were used, but Aristotle Comprehensive Complexity score was modified. In this study, the first analysis of the CKCV Database is reported. Methods: The CKCV Database included 2307 procedures from 12 centers between January 2018 and March 2020. All parameters, including 10 real-time online reports, which represent the number of centers, number and mortality rates of all procedures, number of extracorporeal membrane oxygenation (ECMO) and results, details of postoperative complications, age-group statistics, analysis for priority status, mean intensive care and hospital stay durations of the procedures, results of Aristotle Basic, Modified Aristotle Comprehensive (MACC) and Society of Thoracic Surgeons-European Association (STAT) Score Categories, comparison of centers were analyzed. Results: Most common 10 procedures were ventricular septal defect (VSD) repair (n = 273), tetralogy of Fallot (TOF) repair (n = 243), atrial septal defect (ASD) repair (n = 181), complete AVSD repair (n = 95), cavopulmonary anastomosis (n = 81), systemic to pulmonary shunt (n = 79), modified Fontan (n = 71), subaortic resection, (n = 66) PA banding (n = 66), and arterial switch operation (n = 66). Cardiopulmonary bypass was used in 84.6% of the procedures. Overall mortality rate was 6.0%. A total of 618 major and 570 minor complications were observed in 333 and 412 patients, respectively. According to six MACC categories, number of the patients and mortality rates were I (293; 0.3%); II (713; 1.4%); III (601; 3.3%); IV (607; 12%); V (84; 35.7%); and VI (9; 55.6%), respectively. Analysis of five STAT Categories showed 0.7, 3.8, 5.4, 14.9, and 54.7% mortality rates. Conclusions: CKCV Database has a great potential for nationwide quality improvement studies. Users could instantly analyze and compare their results to national and international aggregate data using a real-time online reporting function. This is the first multicenter congenital database study in Turkey.

Risk factors associated with post-transplant superior caval vein stenosis in paediatric heart transplantation

Superior caval vein stenosis is a known complication following paediatric heart transplantation. Herein, we sought to assess the incidence of superior caval vein stenosis and need for intervention in a single centre paediatric heart transplantation programme. A retrospective review was performed to identify variables associated with superior caval vein stenosis and need for intervention. Patients were identified based on angiographic and echocardiographic signs of superior caval vein stenosis. Of 204 paediatric heart transplantation recipients, 49 (24.0%) had evidence of superior caval vein stenosis with no need for catheter intervention and 12 (5.9%) had superior caval vein stenosis requiring catheter intervention. Overall, patients with superior caval vein stenosis with and without intervention had more cavopulmonary anastomosis (41.7%; 20.4%), pre-transplant superior caval vein procedures (41.7%; 28.6%), and bicaval approach (100.0%; 98.0%), compared to the group with no stenosis (11.9% and p = 0.015, 12.6% and p = 0.004, 73.4% and p < 0.001, respectively). Smaller recipients and donors were more likely to need intervention. Intervention was also seen more frequently in recipients who were younger at diagnosis (4.7 years) compared to non-intervention (13.3 years; p = 0.040). Re-intervention was required in 16.7% patients (n = 2) and was not associated with any complications.

Trans catheter device closure of a large azygos vein in adult patient with systemic venous collateral development after the bidirectional Glenn shunt

Superior cavopulmonary anastomosis is a type of palliative cardiac surgeries that usually done in children with cyanotic and complex congenital heart disease who have single ventricle profile. BDG shunt is staged palliation procedure for single ventricle patients who are candidates for total cavopulmonary connection (TCPC). Sometimes the surgeon misses ligating or intentionally leaves the azygos vein as a fenestration or emergency exit. This allows an abnormal flow from the superior vena cava (SVC into azygos vein). These patients can present progressive desaturation, chest tightness, progressive dyspnea, edema and shortness of breath. Therapeutic options include observation, surgical ligation and trans catheter closure. Because of high risks and extra traumas of surgery and greater chance for difficulties and the feasibility of trans catheter therapy, it is done in some centers as a method of choice.