Supakul Nucharin

Final Pr. ID: Poster #: EDU-017

To summarize our experience using Computed Tomography Angiography (CTA) of the liver as an imaging tool for pre-living donor liver transplantation (LDLT) evaluation in biliary atresia (BA) patients. This pictorial review will illustrate and discuss several aspects of imaging techniques and the important findings that clinician need to know before LDLT.

Key imaging findings for vascular anatomy/collateral vessels will be made, emphasizing what clinicians need to know. Correlation with intraoperative findings will also be provided.
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Authors:  Supakul Nucharin

Keywords:  Biliary atresia, LDLT, liver transplantation, CTA liver, Pediatric

Gould Sharon,  Harty M.,  Ostrowski John,  Tsuda Takeshi

Final Pr. ID: Poster #: SCI-008

Diseases of the coronary arteries are rare in children, but can present as unexpected catastrophic events without preceding symptoms. It is essential to identify patients at risk to prevent potentially serious cardiovascular events. Coronary CT angiography (CCTA) has been widely studied in adults, but its clinical applications are not well established in children. Read More

Authors:  Gould Sharon , Harty M. , Ostrowski John , Tsuda Takeshi

Keywords:  Coronary, CTA, Pediatric

Stern Joseph,  Ramirez Suarez Karen,  Victoria Teresa,  Otero Hansel

Final Pr. ID: Poster #: CR-033

Hepatopulmonary fusion (HPF) is a very rare type of right-sided congenital diaphragmatic hernia (CDH) with a reported prevalence of 3 in 1000 right-sided CDH cases. By June 2020, less than 50 cases have been reported in the literature. Out of 147 patients with right CDH managed at our institution, we found only one case (0.68%) of HPF, which is presented here.

HPF is typically not identified preoperatively, and the presence of HPF changes the operative management. It has been reported that a large right CDH without leftward cardiomediastinal shift and atelectatic lung adherent to the liver are imaging features of HPF.

This patient was a full-term, newborn girl with a normal prenatal course who developed respiratory distress immediately after birth. Chest radiograph at birth demonstrated a right CDH. On day four of life, the patient underwent surgical repair of right CDH. During the procedure, hepatopulmonary fusion involving the cephalad portion of the liver was discovered. The lung and liver could not be separated safely so the diaphragmatic defect was closed around the portion of the liver that extended into the thorax. Postoperative CT angiography of the chest and abdomen demonstrated no abnormal vascular communication between the lungs and liver; however, anomalies of systemic veins were identified. Follow-up chest radiograph at 9 months of age demonstrated a large right CDH that did not include bowel or right-sided colon and was without leftward mediastinal deviation. Concurrent contrast-enhanced CT images of the thorax at this time confirm the right CDH with herniated liver occupying most of the right hemothorax and expected rightward traction of the liver vasculature. However, no significant leftward mediastinal deviation was noted. A small amount of atelectatic lung overlying the liver was also noted, another imaging sign expected with HPF. Last follow-up at age 4, patient was thriving with normal growth and development. Read More

Authors:  Stern Joseph , Ramirez Suarez Karen , Victoria Teresa , Otero Hansel

Keywords:  Hepatopulmonary Fusion, Congenital diaphragmatic hernia, CTA

Rapp Jordan,  Poletto Erica,  Urbine Jaqueline,  Malik Archana,  Kazmi Faaiza,  Mallon Mea

Final Pr. ID: Poster #: EDU-007

The rare entity of an absent pulmonary artery has appeared in the literature since 1868, with most cases associated with congenital heart disease of various types. It has also long been observed that the absent pulmonary artery is contralateral to the aortic arch in almost every case. Isolated absence of a single pulmonary artery without associated congenital heart disease is less common, and these patients may present at any time from prenatal screening, neonatal period, early childhood, or even adolescence and adulthood. We will discuss the embryologic origins, clinical presentations, expected imaging findings, and treatment options based on patient ages from newborn to adolescence.

In neonates with an isolated absent pulmonary artery, a patent ductus arteriosus will allow for continued systemic blood supply. Even early on, narrowing of the PDA may be seen as involution is inevitable without intervention. The lung parenchyma is typically preserved, without yet evidence of hypoplasia or oligemia. Once the PDA has closed, robust collateral formation will occur. As patients age without repair, the lung parenchyma may become hypoplastic with diminished lung volumes and vascular markings. Findings suggestive of recurrent infection such as bronchiectasis may also be evident.

Early discovery and treatment is ideal as this will allow for prevention of long term sequelae and the greatest restoration of lung function as the options for repair are limited in the older patient. There is no universal standard approach for repairing the underlying mechanism of providing blood flow to the intrapulmonary pulmonary artery. Early intervention in neonates included PDA stenting or anastomosing the main pulmonary artery with the intrapulmonary pulmonary artery using a synthetic graft. Patients that present after the neonatal period are not likely to be eligible for surgical repair. The most common long term effect of an absent pulmonary artery is pulmonary hypertension, seen in 40% of patients.

The entity of isolated unilateral absence of a pulmonary artery is rare, however demonstrates typical cardiothoracic findings depending on age at presentation. Understanding of embryology, specifically the 4th and 6th primitive aortic arches, allows one to understand why this malformation occurred and what findings to expect on imaging. The maintained PDA is vital for early lung blood supply and development and can aid in repair. Read More

Authors:  Rapp Jordan , Poletto Erica , Urbine Jaqueline , Malik Archana , Kazmi Faaiza , Mallon Mea

Keywords:  Pulmonary artery, congenital, CTA

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

Zucker Evan,  Kino Aya,  Vasanawala Shreyas,  Newman Beverley,  Barth Richard,  Chan Frandics

Final Pr. ID: Paper #: 013

To evaluate by CCTA the prevalence and morphology of pediatric LAD MBs (coronary segments that contact or tunnel within myocardium), which are common and usually incidental in adults but only sporadically reported in children. Read More

Authors:  Zucker Evan , Kino Aya , Vasanawala Shreyas , Newman Beverley , Barth Richard , Chan Frandics

Keywords:  Myocardial Bridge, Coronary CTA, Coronary

Smitthimedhin Anilawan,  Otero Hansel,  Cahill Anne Marie,  Durand Rachelle

Final Pr. ID: Paper #: 143

Renal artery stenosis (RAS) is an important cause of hypertension in children. When suspected, imaging options include Doppler US, CTA and MRA. However, conventional angiography remains the gold standard. We investigate the accuracy and inter-reader reliability of CTA in children with suspected renal artery stenosis. Read More

Authors:  Smitthimedhin Anilawan , Otero Hansel , Cahill Anne Marie , Durand Rachelle

Keywords:  Renal Artery Stenosis, CTA, Angiography