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.

Left Ventricular Pseudoaneurysm Following Surgical Repair of Ventricular Septal Defect in an Infant.

Left ventricular pseudoaneurysm (LV-PSA) is a rare complication following cardiac surgery, let alone in the pediatric population. Other known causes of LV-PSA are trauma, percutaneous cardiac intervention, and infections. This report describes the development of LV-PSA following surgical repair of ventricular septal defect (VSD) and coarctation of aorta (CoA) in an infant.

Determination of the Frequency of Right and Left Internal Mammary Artery Embolization in Single Ventricle Patients: A Two-Center Study.

Embolization of systemic to pulmonary artery collaterals to regulate pulmonary arterial flow or pressure of the cavopulmonary circulation in patients with single ventricle is a common practice. The relative incidence and impact of this practice on future interventions like coronary artery bypass grafting is poorly understood. This study aims to evaluate the frequency and implications of internal mammary artery (IMA) embolization in the single ventricle (SV) population. A retrospective chart review was performed of SV patients who underwent cardiac catheterization before and after Fontan procedure between February 2007 and 2017. Data were collected from two tertiary care centers in the Midwest. Of the 304 SV patients, 62 (20.4%) underwent embolization of one or more IMAs, whereas 242 (79.6%) did not. The rate of embolization of IMA was 40.5% in one center and 14.5% in the second center. Among patients who received IMA embolization, left internal mammary artery (LIMA) embolization was seen in 6 (9.7%) patients. Majority of patients underwent either right internal mammary artery (RIMA) embolization (n = 25; 40.3%) or RIMA and LIMA embolization (n = 27; 43.5%). IMA embolization in SV patients is common. Embolizing IMAs early in life will likely eliminate a valuable graft option for coronary artery bypass grafting should it be required in the future care of these patients. Multi-center, prospective, nation-wide studies are warranted to examine coronary artery disease in the SV population and true frequency of IMA embolization. Delineation of which IMAs were embolized is a necessary in surgical and cardiac intervention national data, such as Society of Thoracic Surgeons (STS) database. All measures should be taken to preserve IMAs patency, if deemed feasible and safe.

Surgical repair of left ventricular pseudoaneurysm following perventricular device closure of muscular ventricular septal defect.

Left ventricular pseudoaneurysm formation following perventricular device closure of a muscular ventricular septal defect is a rare complication. We describe a case of left ventricular pseudoaneurysm in an infant with Swiss-cheese ventricular septal defects who initially underwent closure with an Amplatzer device using a hybrid approach. The pseudoaneurysm was successfully resected surgically.

Metastatic osteosarcoma to the interventricular septum: an unusual presentation in a child.

Whereas primary cardiac tumors are reported in only 0.17 % of pediatric patients, malignant cardiac tumors are even less common in children. This report presents a 10-year-old child with relapsed osteosarcoma manifesting as metastatic lesions to the interventricular septum.

Congenital anomalous chordae tendinae of the mitral valve: an unusual presentation of mitral insufficiency in children.

Anomalous chordae tendinae (CT) originating from mitral valve leaflet is a rare congenital mitral valve anomaly. Our case report is unique as this anomaly is extremely rare in this pediatric age group. The anomalous CT extended from anterior mitral leaflet to the atrial septum (AS). Surgical repair in the form of anomalous CT excision, anterior leaflet chordoplasty, and posterior mitral annuloplasty was successfully performed. Congenital mitral valve (MV) leaflet or chordae anomalies are rare. In anomalous CT from MV leaflet to the AS, the surgical experience is extremely limited and only reported in adults and adolescents. We describe an unusual presentation of severe mitral insufficiency (MR) associated with anomalous CT from the anterior mitral valve leaflet (AMVL) to the AS that prompted successful repair during childhood.