Ordonez Alvaro,  Stecher Priscilla,  Rogers Lindsay,  Bhatti Tricia,  Noor Abass

Final Pr. ID: Poster #: CR-006

Single ventricle heart defects (SVHD) refer to a rare and diverse group of severe congenital cardiac abnormalities in which the systemic and pulmonary venous return enter a functionally single ventricular chamber. This condition is typically caused by underdevelopment of an atrioventricular valve and/or a ventricular chamber, leading to a range of complex congenital heart defects. With an incidence of approximately 3 to 5 per 10,000 live births, newborns with SVHD are critically ill, and without medical intervention, the condition is fatal. Advancements in surgical techniques, namely staged palliation and orthotopic heart transplantation, have enabled more patients with SVHD to survive into adulthood. However, despite these improvements, the perinatal period remains tenuous.

We present a five-week-old male infant born at 38 weeks of gestation with a functional SVHD in the form of severe subaortic stenosis, arcade-like mitral valve, left ventricular dilation and dysfunction, and restrictive atrial septum. He received multiple surgical interventions, including atrial septal stenting with pulmonary artery banding and stage I reconstruction with a right Blalock-Thomas-Taussig shunt. Postoperative course was complicated by delayed sternal closure, cardiac arrest, right hemidiaphragm plication, and progressive heart failure. At five weeks, CT angiography showed a functional single ventricle status post Damus-Kaye-Stansel anastomosis, hypoperfusion of the left ventricle myocardium, and pneumoperitoneum. Exploratory laparotomy with segmental bowel resection revealed perforated acute appendicitis and mucosal ulceration of the cecum. Following the procedure, he developed acute hypoxemia and bradycardia. Life-sustaining support was withdrawn. Postmortem examination of the heart was notable for severe aortic stenosis with subaortic obstruction, stenotic mitral valve with arcade-like fibrous plaques, and biventricular hypertrophy. Histopathology of the left ventricle showed significant subendocardial fibrosis without areas of myocyte injury.

Due to the complexities involved in its management, SVHD demands special attention from radiologists. While advancements in medical and surgical care have improved outcomes, ongoing progress depends on a deeper understanding of the factors contributing to ventricular dysfunction and heart failure in this vulnerable population. Read More

Authors:  Ordonez Alvaro , Stecher Priscilla , Rogers Lindsay , Bhatti Tricia , Noor Abass

Keywords:  Ventricular Dysfunction, Cardiac CTA, Cardiovascular Disease

Scace Candace

Final Pr. ID: Poster #: EDU-009

To provide a working knowledge of normal cardiac anatomy and a systematic guide to findings in common and uncommon congenital heart disease as seen on cardiac CTA. Read More

Authors:  Scace Candace

Keywords:  Congenital Heart Disease, Cardiac CTA, Presurgical CHD

Patel Alisha,  Lefebvre Melissa,  Ahmed Asmaa

Final Pr. ID: Poster #: CR-003

Congenital anomalies of the coronary sinus can present with a variety of morphological features. An unroofed coronary sinus is a rare type of atrial septal defect (ASD), also known as a coronary sinus ASD. This partial or complete absence of the roof of the coronary sinus results in a communication between the coronary sinus and left atrium. We present the first reported case of this rare intra-atrial communication in the setting of a right-sided aortic arch and a Kommerell diverticulum (KD) in an asymptomatic pediatric patient. A 16-year-old male presented for cardiology consultation for an asymptomatic heart murmur. Cardiac auscultation revealed a soft, low-pitched continuous hum audible in the right clavicular region, consistent with a venous hum and a soft grade I/VI systolic murmur heard along the left sternal border. Electrocardiography (ECG) showed a normal sinus rhythm with early repolarization. Given his age and new onset murmur, an echocardiogram was obtained, demonstrating flow acceleration in the coronary sinus as it opened into the right atrium, a right-sided aortic arch, and a dilated coronary sinus. Further evaluation with an ECG gated cardiac computed tomography (CT) angiography illustrated unroofing of the coronary venous sinus with a jet of contrast from the coronary sinus into the right atrium. The cardiac CT also confirmed a right-sided aortic arch with mirror image branching pattern and an isolated diverticulum of Kommerell without the presence of an aberrant left subclavian artery. Given an oxygen saturation of 100% and lack of any symptoms, no surgical intervention was recommended and regular follow-up was scheduled. The presence of an unroofed coronary sinus is most often found as part of a complex congenital heart defect. While an isolated coronary sinus ASD is often difficult to diagnose, patients are considered to have excellent prognosis. However, both the unroofed coronary sinus and KD predispose a patient to long-term complications if not diagnosed and monitored in a timely manner. This case highlights the importance of clinical judgement in the setting of new heart murmurs, the use of sensitive imaging modalities for diagnosis of rare cardiac anomalies, and the need for long-term follow-up. Read More

Authors:  Patel Alisha , Lefebvre Melissa , Ahmed Asmaa

Keywords:  Congenital Heart Disease, Cardiac CTA, Stenosis