Quick Fire Cases
Emese Kanyo, MD
Resident Physician
Children's National Medical Center
Emese Kanyo, MD
Resident Physician
Children's National Medical Center
Seiji Ito, MD
Children's National Medical Center
Sarah E. Kollar, DO
Advanced Imaging Cardiologist
Children's National Hospital
Michelle Udine, MD
Advanced Imaging Cardiologist
Children's National Medical Center
Ravi Vamsee Vegulla, MD
Assistant Professor of Pediatrics
Children's National Medical Center
Yue-Hin Loke, MD
Associate Professor
Children's National Medical Center
.jpg "Uyen Truong, MD photo")
Uyen Truong, MD
Associate Professor
Children's National Hospital
Ryo Sakuma, MD
Resident Physician
U.S. Naval Hospital Yokosuka, Japan
Case 1:
An eighteen-year-old female with a history of Trisomy 21 and seizure disorder presented with syncope and severe pulmonary arterial hypertension (PAH) demonstrated by transthoracic echocardiogram. A physical exam revealed a right arm blood pressure of 102/63mmHg and pulse oximetry 95%, with blood pressure and pulse oximetry in her left arm in a similar range.
Case 2:
A 2-year-old was referred to the cardiology clinic for newly diagnosed 22q11.2 deletion syndrome. An echo at this time was significant for suspected discontinuous left PA with flow via aortopulmonary collaterals versus ductus. Continuous flow signal was seen posterior to superior vena cava, thought to be an additional aortopulmonary collateral vessel.
Diagnostic Techniques and Their Most Important Findings:
Case 1:
A computed tomography and magnetic resonance imaging (MRI) of the chest revealed a right aortic arch with isolated left innominate artery from the main pulmonary artery (PA). Cardiac MRI corroborated these findings, noting aberrant left innominate artery originating from the main PA with retrograde flow during diastole, right aortic arch, and signs of right ventricular pressure and volume overload. Qp/QS 1:1 by stroke volume analysis. A brain MRI and angiography showed an intact circle of Willis without an acute intracranial process. After careful consideration of therapeutic options, the patient was initiated on advanced PAH therapy preoperatively before she underwent uncomplicated reimplantation of the innominate artery to ascending aorta. Postoperatively, the echocardiogram continued to show severe PAH, but she was able to be discharged home without clinical deterioration. Five years after surgical repair, the patient remains well and asymptomatic on triple PAH therapy with decreased incidence of syncope.
Case 2:
A cardiac MRI with 4-dimensional flow (4DF) using ferumoxytol as contrast was obtained which demonstrated a right aortic arch with an aberrant left subclavian artery and an isolated left common carotid artery (LCCA) origin from the proximal left PA with cerebral to pulmonary systemic artery steal. It also showed left PA coarctation with differential pulmonary blood flow of left PA 42% and right PA 58%, anomalous pulmonary venous return of right upper pulmonary vein, and Qp/Qs 1.3;. The patient remains asymptomatic from a neurologic standpoint, aside from global developmental delay. Further brain imaging is being pursued.
Learning Points from this Case:
Case 1 illustrates a unique late presentation of a rare aortic arch anomaly with severe PAH and likely symptomatic hypoxia from cerebral steal. Therapeutic options required careful consideration of imaging findings and physiology. Case 2 demonstrates the clinical use of cardiac MRI in understanding the anatomy of the aortic arch and its branches in individuals with 22q11.2 deletion syndrome. The 4DF sequence contributed to the physiological understanding of the case.
