The relative simplicity of the clinical presentation and management of an atrial septal defect belies the complexity of the developmental pathogenesis. Here, we describe the anatomic development of the atrial septum and the venous return to the atrial chambers. Experimental models suggest how mutations and naturally occurring genetic variation could affect developmental steps to cause a defect within the oval fossa, the so-called secundum defect, or other interatrial communications, such as the sinus venosus defect or ostium primum defect.

A 73-year-old man with a history of hypertension, hyperlipidemia, and obesity presented for cardiovascular evaluation. He was experiencing mild fatigue and dyspnea on exertion. Transthoracic echocardiogram (TTE) showed right ventricular dilation, which was otherwise unremarkable.

We report successful transcatheter correction of a sinus venosus defect in a 72-year-old woman with anomalous pulmonary venous return in a challenging anatomical configuration. The procedure was facilitated by hands-on simulation training on a newly developed, perfused, 3D-printed model.

Superior sinus venosus defect is a communication between the right and left atrium located above the upper margin of the oval fossa, immediately inferior to the junction of the superior vena cava and the right atrium. It is systematically associated with partial anomalous pulmonary venous drainage, especially of the right upper pulmonary vein. Surgical repair has been the gold standard approach to close that defect. Introduced in 2014, percutaneous closure has gradually become a safe and effective alternative to surgery in carefully selected patients, although worldwide experience remains limited. This article provides an appraisal of the patients\' selection process and a step-by-step description of the procedure as well as a comprehensive review of its outcomes.

OBJECTIVE: Long-term results after sinus venosus defect (SVD) closure are sparse and many studies lack a proper control cohort. This nationwide cohort evaluated the long-term outcome after SVD surgery.
METHODS: The study enrolled every surgical SVD correction from the nationwide hospital discharge registry (FHDR) and surgical registries of two tertiary centers. Patients with more complex congenital heart defects were excluded. Surgeries were performed from 1969 to 2019. Five sex and birth-year-matched controls per SVD patient were gathered from the general population.
RESULTS: In total, 182 surgical SVD corrections were performed during the study period. The median age at the time of surgery was 8.3 years (range 0.06-75.7), and the majority (77.5%, n = 141) were under 18 years old. The median follow-up period was 18 years (range 0.1-53). There was no significant difference in mortality during the follow-up (logrank p = 0.62, MRR 0.78, 95% CI: 0.30-2.0). However, SVD patients had elevated risk for new-onset atrial fibrillation (RR 4.9, 95% CI: 2.2-10.9), heart failure (RR 4.0, 95% CI: 1.2-13.2), ischemic heart disease (4.3, 95% CI, 1.5-11.7), migraine (RR 3.6, 95% CI: 1.5-9.1) and sick sinus syndrome, II- or III-degree AV-block or pacemaker implantation (RR 11.3, 95% CI: 2.9-43.8).
CONCLUSIONS: Young patients with SVD have an excellent survival prognosis after the surgery. Risk for sick sinus syndrome or conduction disorders, atrial fibrillation, and heart failure remains elevated in the long-term follow-up.

BACKGROUND: Several surgical techniques have been proposed to repair right partial anomalous pulmonary venous connection (PAPVC) along with sinus venosus defect (SVD). This study aimed to compare the perioperative data and outcomes of double-patch repair using a minimally invasive approach versus conventional sternotomy in pediatric and adult patients.
METHODS: This retrospective study was conducted on 48 minimally invasive cases and 35 sternotomy cases, undergoing surgery by a single surgeon between July 2002 and August 2020. For all patients, repair was performed using the double-patch technique. In the minimally invasive approach, right mini-thoracotomy was performed with central cannulation for children and with peripheral cannulation for adults. The patients were classified into two pediatric and adult groups, and each group was categorized into minimally invasive and sternotomy approaches. They were followed-up by transthoracic echocardiography and electrocardiography before and early after surgery, 3 and 6 months after surgery, and then annually. The relative data were compared between the two approaches in terms of perioperative findings, postoperative pulmonary vein or superior vena cava (SVC) stenosis, and sinus node dysfunction.
RESULTS: This study included 25 minimally invasive cases and 19 sternotomy cases in the pediatric group (mean age, 4.99 ± 4.28 and 6.10 ± 4.39 years, respectively) and 23 minimally invasive cases and 16 sternotomy cases in the adult group (mean age, 35.73 ± 8.06 and 32.62 ± 9.80 years, respectively). The mean and median follow-ups were 6.31 ± 4.92 years and 6 years (range: 6 month-18 year) in the pediatric group and 6.15 ± 4.53 years and 5 years (range: 6 month-18 year) in the adult group, respectively. The mean chest tube drainage was significantly lower in the minimally invasive pediatric group (p = .03), and the mean blood transfusion volume was significantly lower in the minimally invasive adult group compared to the other groups (p = .03). No stenosis occurred in the pulmonary veins. Mild SVC stenosis occurred in one patient in the minimally invasive pediatric group, with no need for reintervention. All patients had a normal sinus rhythm, except for the mentioned case with a transient, first-degree atrioventricular block, which spontaneously reverted to the normal sinus rhythm.
CONCLUSIONS: The minimally invasive approach can be a safe and practical alternative for the double-patch repair of PAPVC and SVD. It ensures a repair with comparable quality to sternotomy, but with better cosmetic and psychological outcomes.

With the advent of computed tomographic interrogation, it is increasingly frequent to find venous channels that provide direct connections between the pulmonary and systemic veins. These channels, before the introduction of three-dimensional techniques for clinical imaging, were usually found providing an \"overflow\" for the obstructed left atrium in settings such as hypoplastic left heart syndrome, or divided left atrium. Similar channels, however, had been described almost 100 years ago, with one accurately described as a jugulo-pulmonary vein. Nowadays, however, it is much more usual to find the channels described as levoatrial cardinal veins, even though it is recognized that they are not \"levo,\" often not \"atrial,\" and for sure not \"cardinal.\" In this review, we assemble the evidence supporting the notion that they are better considered as pulmonary-to-systemic collateral channels. We emphasize their similarity, in terms of development, to the sinus venosus and coronary sinus defects.

UNASSIGNED: Congenital thoracic venous anomalies (CTVAs) with right-to-left shunt constitute an uncommon source of paradoxical embolization in adults. We present a case of a healthy and physically fit individual with a rare asymptomatic anomaly first presenting with brain abscesses after a visit to the dental office; persistent left superior vena cavae (PLSVC) without bridging vein, over-riding right-sided superior vena cavae (RSVC) connected to the left atrium (LA), and an extracardiac sinus venosus defect.
UNASSIGNED: A 29-year-old male presented to the neurosurgical unit due to intracranial abscesses requiring intervention following a visit to his dentist. The abscess cultures isolated bacteria commonly found in the normal oral flora. Transthoracic echocardiography revealed an enlarged coronary sinus consistent with PLSVC. An agitated saline study was performed and raised suspicion of simultaneous extra- and intracardiac shunting. Magnetic resonance angiography confirmed the presence of a PLSVC and revealed an RSVC connected to the LA; however, no intracardiac shunt was evident. Electrocardiogram-gated computed tomography was therefore conducted and discovered the rudimentary remains of the physiologic RSVC forming a connection to the right atrium, explaining the bilateral contrast loading seen on the agitated saline study and diagnosing an extracardiac sinus venosus defect (SVD). The patient recovered and has been referred for surgery.
UNASSIGNED: This case illustrates a CTVA and a forme fruste type SVD resulting in a severe complication in a healthy adult. We highlight the diagnostic challenges posed, suggest early usage of agitated saline studies, and discuss the rationale for surgical correction of this patient.

Atrial septal defects are one of the most common forms of congenital heart disease, however sinus venosus communications, particularly pulmonary vein-type defects, are rare and are easily misdiagnosed. Patients with pulmonary vein-type sinus venosus defects often present earlier than those with ostium secundum defects with significant right heart dilation. Correct diagnosis has important implications for management. We discuss the clinical courses and review multimodality imaging of three patients correctly diagnosed with pulmonary vein-type defects after an initial diagnosis of an ostium secundum atrial septal defect, in order to promote understanding of the unique anatomy of this entity.

Atrial septal defects (ASDs) represent the most common congenital heart defect diagnosed in adulthood. Although considered a simple defect, challenges in optimal diagnostic and treatment options still exist due to great heterogeneity in terms of anatomy and time-related complications primarily arrhythmias, thromboembolism, right heart failure and, in a subset of patients, pulmonary arterial hypertension (PAH). Atrial septal defects call for tertiary expertise where all options may be considered, namely catheter vs. surgical closure, consideration of pre-closure ablation for patients with atrial tachycardia and suitability for closure or/and targeted therapy for patients with PAH. This review serves to update the clinician on the latest evidence, the nuances of optimal diagnostics, treatment options, and long-term follow-up care for patients with an ASD.