Search Results (7)

Background. Cor triatriatum dexter (CTD) is a rare congenital heart defect where a membrane divides the right atrium into two chambers, resulting from the incomplete regression of the right valve of the sinus venosus. Due to its rarity, only individual case reports and a limited number of case series have been published to date. This study constitutes the most extensive comprehensive review conducted in this area. Eight factors were evaluated: age at diagnosis, sex, clinical presentation, electrocardiographic findings, imaging (ultrasound, CT, or MRI), associated cardiac anomalies, and patient outcomes. Methods. The electronic databases PubMed and Scopus were searched from their inception until 30 October 2025. Only case reports and case series were considered for inclusion. Studies involving foetuses, autopsies, and animals were excluded. The collected data were primarily presented as percentages. Results. One hundred fourteen studies were found encompassing 124 patients. The mean age at diagnosis was 33.3 ± 9.4 years The most common clinical presentations were dyspnoea (44.3%) and cyanosis (29.5%). The most commonly encountered ECG changes were supraventricular tachycardia/atrial flutter/atrial fibrillation (33.3%) and right bundle branch block (22.6%). On chest X-ray, cardiomegaly was noted in 46.5%. CTD was suspected or diagnosed by echocardiography in 95.2% of cases. The diagnosis was confirmed by CT and/or MRI in 34.1% of cases. A concomitant congenital heart defect was found in 67.7%, especially in the form of all kinds of atrial septal defect (38.1%) and of right valvular and right ventricular involvement (20.1%). An outcome was reported in 97/124. Surgical correction was the treatment of choice in 51.6%. Since 1991, a percutaneous approach has been employed in selected cases (5.1%). Conservative management was the treatment of choice in 43.3%. The mortality rate was 8.2%. Discussion. The principal limitation of this systematic review lies in its reliance solely on case reports and small case series, reflecting the absence of large-scale studies on CTD. Nonetheless, it constitutes the most comprehensive analysis available to date. Full article

(1) Background: There has been an increase in the utilization of the minimally invasive vertical right axillary thoracotomy approach for repairing congenital heart defects in children recently. We aim, in the current study, to evaluate the outcomes of this approach in repairing anomalous pulmonary venous connections with or without an associated sinus venosus defect. (2) Methods: A total of 23 consecutive patients underwent surgical repair of anomalous pulmonary venous connections between April 2018 and February 2024. Perioperative and clinical follow-up data were obtained. (3) Results: The median age and weight were 36 months (1–277 months) and 14.4 kg (3.6–79.4 kg), respectively. More than half were females (13; 56.5%). There was no conversion to sternotomy. Partial anomalous pulmonary venous connections were the most frequent primary diagnoses (14; 60.9%), followed by scimitar syndrome (3; 13%), while two patients (8.7%) had total anomalous pulmonary venous connections. Repair techniques included single patch in 10 patients (43.5%), Warden in 6 (26.1%), and two-patch technique in 4 (17.4%). The median cardiopulmonary bypass and aortic cross-clamp times were 91 and 62 min, respectively. All patients were extubated in the operating room. The median length of hospital stay was 2 days. There were no mortalities or reoperations for pulmonary/systemic venous pathway obstruction. (4) Conclusions: Vertical right axillary thoracotomy is a valuable approach for repairing anomalous pulmonary venous connections with or without sinus venosus defects. All repair techniques, including Warden and scimitar, can be performed safely through this approach. The cosmetic superiority and short hospital stay make this approach worth considering. Full article

Sinus venosus atrial septal defects (SVASDs), concurrent with partial anomalous pulmonary venous connections (PAPVCs), are a rare congenital heart disease in dogs. Surgical correction is essential when clinical signs or significant hemodynamic changes are present. We aimed to report on the successful surgical correction of an SVASD with PAPVCs, using a computed tomography (CT)-based customized 3D cardiac model. A 10-month-old male poodle was referred for corrective surgery for an ASD. Echocardiography confirmed a hemodynamically significant left-to-right shunting flow through an interatrial septal defect and severe right-sided heart volume overload. For a comprehensive diagnosis, a CT scan was performed, which confirmed an SVASD with PAPVCs. A customized 3D cardiac model was used for preoperative decision-making and surgical rehearsal. The defect was repaired using an autologous pericardial patch under a cardiopulmonary bypass (CPB). Temporary pacing was applied for sinus bradycardia and third-degree atrioventricular block. The patient recovered from the anesthesia without further complications. The pacemaker was removed during hospitalization and the patient was discharged without complications 2 weeks post-surgery. At the three-month follow-up, there was no shunting flow in the interatrial septum and the right-sided volume overload had been resolved. The cardiac medications were discontinued, and there were no complications. This report indicates the validity of surgical correction under CPB for an SVASD with PAPVCs, and the advantages of utilizing a CT-based 3D cardiac model for preoperative planning to increase the surgical success rate. Full article

Objectives: To investigate primary and secondary surgical outcomes following transcaval repair (TCR), modified Warden repair, and transatrial repair techniques for partial anomalous pulmonary venous connections (PAPVCs) and sinus venosus atrial septal defects (ASDs). Methods: This is an observational cohort clinical study. Patients who underwent TCR, modified Warden repair, and transatrial surgical repair for PAPVC and ASD between January 2003 and October 2019 at our institution were included in the study. Patients had one of the surgical procedures based on the anatomy of the defect. Results: Ten patients, seven (70%) males and three (30%) females, were included in the analysis. Seven patients underwent TCR, two patients the modified Warden technique, and one patient underwent transatrial surgical repair. Mean age was 57 years ± 14.7. Mean EuroScore II was 3.4 ± 3.5. The baseline left ventricle ejection fraction was 45 ± 6.5%. No patient had previous stroke, pacemaker (PM) implantation, or myocardial infarction. Total cardiopulmonary bypass and cross-clamping time were 123 ± 72.5 and 100 ± 48.5 min, respectively. Mean mechanical ventilation, mean intensive care unit, and mean hospital length of stay for the transcaval, modified Warden, and transatrial groups were 4.6 ± 10.7, 5.7 ± 8.8, and 10.5 ± 9.2 days, respectively. Superior caval or pulmonary venous obstruction, sinus node dysfunction, and PM implantation were not present at follow-up. The patient who underwent transatrial repair had died at 5.5-year follow-up due to myocardial infarction. Total survival rate at 6 years was 90%. Conclusions: The findings from this study elicit that all three techniques have low postoperative morbidity and are feasible and reliable procedures. Full article

This review centers on the usefulness of echo-Doppler studies in the diagnosis of ostium secundum atrial septal defects (ASDs) and in their management, both in children and adults. Transthoracic echocardiography can easily identify the secundum ASDs and also differentiate secundum ASDs from other kinds of ASDs, such as ostium primum ASD, sinus venosus ASD and coronary sinus ASD, as well as patent foramen ovale. Preliminary selection of patients for device occlusion can be made by transthoracic echocardiograms while final selection is based on transesophageal (TEE) or intracardiac (ICE) echocardiographic studies with optional balloon sizing of ASDs. TEE and ICE are extremely valuable in guiding device implantation and in evaluating the position of the device following its implantation. Echo-Doppler evaluation during follow-up is also useful in documenting improvements in ventricular size and function, in assessing the device position, in detecting residual shunts, and in identifying rare device-related complications. Examples of echo images under each section are presented. The reasons why echo-Doppler is very valuable in diagnosing and managing ASDs are extensively discussed. Full article

The atrial septal defect is, after bicuspid aortic valve disease, the most common congenital cardiac disease present in the adult population. The most common atrial septal defects are the ostium secundum type (75–80%), followed by the ostium primum type (15%). The sinus venosus atrial septal defects (SV-ASD), defined as a communication in the posterior part of the interatrial septum, account for about 5 to 10% of atrial septal defects. Approximately 90% of SV-ASDs are associated with partial anomalous pulmonary venous drainage (PAPVD). The minimally invasive approach has gained ground in the treatment of ASDs, especially those of the ostium secundum type. The sinus venosus type is a relatively uncommon form of ASD, which, when associated with a PAPVD, is considered a complex cardiac malformation, and is usually treated in a classical manner, through median sternotomy. We describe the case of a 45-year-old woman diagnosed in adolescence with SV-ASD with PAPVD, who successfully underwent minimally invasive repair with fresh autologous pericardial patch reconstruction through an anterolateral mini-thoracotomy incision. The patient presented with shortness of breath and fatigue after heavy exertions, episodes of paroxysmal nocturnal dyspnea, palpitations during effort and at rest, and had a history of syncope dating from 17 years previously. Echocardiography revealed an SV-ASD with PAPVD in the right atrium and the intraoperative examination discovered that both right pulmonary veins were draining into the superior vena cava. Full article