A 33-year-old presented for fetal MRI at 22 weeks 2 days gestation following pre-natal ultrasound that demonstrated findings suspicious for congenital high airway obstruction syndrome (CHAOS). Findings were characteristic of CHAOS and included focal narrowing of the cervical trachea, pulmonary hyperexpansion, tracheobronchomegaly, everted hemi-diaphragms, and abdominal free fluid. However, follow-up MRI at 27 weeks 3 days gestation showed decreased pulmonary hyper-expansion and abdominal free fluid. Diagnosis of atypical congenital high airway obstruction (aCHAOS) was suggested. Further improvement was noted on fetal MR at 31 weeks 2 days gestation and the diagnosis of aCHAOS was reinforced. CHAOS represents a rare, life-threatening fetal airway anomaly characterized by complete or incomplete obstruction of the upper airway. Prenatal imaging findings of classic CHAOS have been well described and include hyperexpanded lungs with everted diaphragms, tracheobronchomegaly, and hydrops. Our experience at quaternary referral centers is that this applies to a particular subset of patients with complete laryngotracheal obstruction without a pathway of fluid egress, and that there is a spectrum of more subtle but equally critical large airway obstructions. This can be in the form of either incomplete large airway obstruction or complete large airway obstruction with a decompression route such as coexisting tracheoesophageal fistula. Accurate characterization by fetal imaging has increased in importance as treatment options have expanded, including fetal airway management and ex utero intrapartum treatment (EXIT). There is a paucity of literature regarding the characterization of CHAOS variants and their natural history. However, improved understanding is important as it is unknown whether outcomes between classic CHAOS and aCHAOS may differ. Findings on prenatal imaging of aCHAOS are more subtle, and the diagnosis requires nuance and a high index of suspicion. Close follow up is required in fetal life, as a lack of proper preparation risks near-certain neonatal death. Diagnoses that mimic aCHAOS, such as bilateral congenital lung malformation, should be considered and excluded. We present the current case along with companion cases to expand the spectrum of prenatal imaging appearances in congenital large airway obstruction. Read More

Meeting name: SPR 2024 Annual Meeting & Postgraduate Course , 2024

Authors: Epps Caleb, Cassady Christopher, Ketwaroo Pamela

Keywords: Congenital High Airway Obstruction Syndrome, Atypical CHAOS, Fetal MR

Papillary fibroelastomas are benign primary cardiac tumors that usually arise from the valve apparatus and are rare in the pediatric population. Involvement of the tricuspid valve is even rarer, with only a few cases reported in the literature. We present the case of an 11-month-old female referred by her pediatrician after a non-cardiovascular acute visit to investigate a murmur noted since birth. The patient was asymptomatic. Family history was negative for congenital heart disease, seizures or genetic disorders. Her physical exam revealed a soft holosystolic murmur at the left lower sternal border but was otherwise normal. Initial work up with ECG and echocardiogram revealed limb lead reversal and a large mass in the right ventricle. Subsequent MRI showed a mobile 15mm oval mass along the septal surface of the right ventricle, in close proximity to the tricuspid valve, without valve inflow or right ventricular outflow tract obstruction. The mass was faintly visualized on TFE, isointense on T1, centrally-hyperintense and peripherally-hypointense on T2, and peripherally enhancing on post-contrast images. After a multidisciplinary review of the case, the decision was made to proceed with surgical resection. A preoperative CT of the chest did not reveal pulmonary embolism. During surgery, the mass was attached to the septal leaflet and the chordae of the tricuspid valve but not to the septum. Visually, the mass was compatible with a fibroelastoma. Off-bypass intraoperative transesophageal echocardiogram showed moderate tricuspid regurgitation that required on-bypass annuloplasty. Pathology confirmed the diagnosis of papillary fibroelastoma. There were no surgical complications. She was discharged on postoperative day 6 and had been doing well until her most recent follow-up four months after surgery. Papillary fibroelastomas are typically asymptomatic, although they carry potential risks for complications such as pulmonary or systemic emboli, depending on their location. MRI is a valuable examination that can characterize intracardiac tumors and thrombi. To our knowledge, this is the first report of a pathology-proven papillary fibroelastoma of the tricuspid valve characterized by MRI in such a young child Read More

Meeting name: SPR 2017 Annual Meeting & Categorical Course , 2017

Authors: Artunduaga Maddy, Jadhav Siddharth, Ketwaroo Pamela, Masand Prakash

Keywords: Papillary fibroelastoma, MRI, intracardiac mass

Norwood procedure is a 3-stage surgical palliation for patients with functionally single ventricle anatomy, most commonly hypoplastic left heart syndrome. Complications after stage I operation are not uncommon and include Blalock Taussig Thomas (BTT) /Sano shunt abnormalities, Damus-Kaye-Stansel (DKS) anastomotic narrowing, and neo-aortic arch /branch vessel stenosis. Transthoracic echocardiography (TTE) is traditionally the mainstay for evaluation. The purpose of our study is to compare Gated Cardiac Computed Tomographic Angiography (CCTA) with TTE when evaluating post-operative complications after stage I Norwood procedure and implications of CCTA findings on management. Read More

Meeting name: SPR 2020 Annual Meeting & Postgraduate Course , 2020

Authors: Salman Rida, More Snehal, Ferreira Botelho Marcos, Ketwaroo Pamela, Masand Prakash, Jadhav Siddharth

Keywords: Congenital heart disease, CT Angiography, Echocardiography

Anomalous Aortic Origin of Coronary Artery (AAOCA) is the 2nd most common cause of sudden cardiac death in the young. Coronary artery origins are evaluated as part of transthoracic echocardiography (TTE) and diagnostic precision may be limited by technical limitations, operator dependence, and patient cooperation. MRI and gated Cardiac Computed Tomographic Angiography (CCTA) are often utilized in patients where an anomaly is suspected on TTE. There is limited literature comparing TTE and CCTA in children with suspected AAOCA. The purpose of this study is to determine the distribution of various coronary anomalies comparing TTE and CCTA data, and define the added value advanced imaging brings in clinical decision-making. Read More

Meeting name: SPR 2020 Annual Meeting & Postgraduate Course , 2020

Authors: Salman Rida, More Snehal, Ferreira Botelho Marcos, Ketwaroo Pamela, Masand Prakash, Molossi Silvana, Jadhav Siddharth

Keywords: Anomalous coronaries, CT angiography, Echocardiography

Amniotic fluid volume (AFV) measured by maximum vertical pocket (MVP) or amniotic fluid index (AFI), is a key indicator of fetoplacental well-being and is assessed by ultrasound (US) after 20 weeks’ gestational age (GA). Fetal magnetic resonance imaging (MRI) is sometimes performed in isolation, and there is no standard assessment of AFV by MRI. Our purpose is to assess the robustness of US AFV indices and interrater variability by MRI in patients with known normal indices at same day US. Read More

Meeting name: SPR 2020 Annual Meeting & Postgraduate Course , 2020

Authors: Ketwaroo Pamela, Cassady Christopher, Nguyen Haithuy, Kralik Stephen, Espinoza Jimmy, Sangi Haleh, Mehollin-ray Amy

Keywords: Amniotic Fluid, Fetal, Polyhydramnios Oligohydramnios