W Mustapha Wan Irfan

Final Pr. ID: Poster #: CR-026

Horseshoe lung is a rare congenital anomaly and is mostly accompanied by scimitar syndrome. We reported a 3 months old baby girl was initially referred from the district hospital for ventilator support for severe congenital pneumonia and dextrocardia with features suggestive of elevated pulmonary artery pressure. She was born via emergency caesar for fetal distress with moderate meconium-stained liquor. Apgar's score was 5 in 1 minute and 9 in 5 minutes. She initially required non-invasive ventilation support and was able to wean to nasal prong on day 2 of life subsequently was intubated for worsening respiratory distress on day 8 of life and was transferred to our hospital. In our hospital, she was treated for nosocomial pneumonia and ventilated for 2 days and subsequently able to extubate to nasal prong. She completed a total of 2 courses of antibiotics within 2 weeks of life. Clinically patient is not dysmorphic but in respiratory distress with sub and intercostal recession. No chest deformity. Auscultation reduced air entry over the right lung, and no murmur was heard. Echo showed situs solitus with mesocardia, dominant RA/RV, AV/VA concordant, small PFO with intact IVS, tricuspid regurgitation 3 mmHg, dilated pulmonary artery and PDA 1.2 mm bidirectional predominantly right to left. Chest radiograph showed opacified right chest, dextrocardia and enlarged left hemithorax. CT Thorax showed a tiny right pulmonary artery, the right pulmonary vein is not clear, probably draining into the IVC- RA junction and a hypoplastic right lung suggestive of scimitar syndrome with horseshoe lung complicated with pulmonary hypertension. She was kept on nasal prong oxygen since extubated because of elevated pulmonary artery pressure and respiratory distress and was given sildenafil. Horseshoe lung is rare, and most of the reported cases share the same spectrum of cardiovascular anomalies identified in scimitar syndrome, and it can be confirmed by CT and angiography. Read More

Authors:  W Mustapha Wan Irfan

Keywords:  Scimitar syndrome, horsehoe lung, pulmonary hypertension

Vargas M Cecilia,  Crido Silvina,  Quintero Karina,  Alonso Jose,  Rizzi Ana,  Pibernous J,  Lipsich Jose,  Moguillanky Slvia

Final Pr. ID: Poster #: CR-018

Aortic dissection in children is often associated with congenital heart disease (bicuspid aortic valve or aortic coarctation), collagenopathies or serious injuries. It rarely arises as a differential diagnosis in the presence of thoracic or abdominal pain in an apparently healthy child. Early diagnosis and treatment is crucial to their survival.
Our purpose is describe the clinical and images characteristics that allowed to diagnose . Read More

Authors:  Vargas M Cecilia , Crido Silvina , Quintero Karina , Alonso Jose , Rizzi Ana , Pibernous J , Lipsich Jose , Moguillanky Slvia

Keywords:  aortic dissection, stent-grafts, imaging, Chronic hypertension

Hirsig Leslie,  Verma Nupur,  Sharma Priya,  Rajderkar Dhanashree

Final Pr. ID: Poster #: EDU-115

Congenital and childhood findings of pulmonary artery anomalies are uncommon but not infrequently encountered by referral centers. We present by case examples anomalies of pulmonary arterial vasculature, and discuss their clinical presentation, associated cardiothoracic anomalies, and overall prognosis. Read More

Authors:  Hirsig Leslie , Verma Nupur , Sharma Priya , Rajderkar Dhanashree

Keywords:  Pulmonary anamolies, Congenital malformations, Pulmonary Origin, Pulmonary Hypertension, Pulmonary complications

Lemessa Natae,  Martinez-correa Santiago,  Lerebo Wondwossen,  Hwang Misun

Final Pr. ID: Poster #: SCI-009

Pediatric hydrocephalus is a common disease in the United States, accounting for more than 39,000 annual admissions and a healthcare cost of $1.4-2.0 billion. Timely detection and intervention of intracranial hypertension (ICP), a complication of hydrocephalus, is associated with an improved neurologic outcome. This research aims to evaluate the feasibility of assessing the movement of the floor of the third ventricle and intracranial arterial pulsations on mid sagittal view of the grayscale brain ultrasound for predicting ICP in neonates and infants. Read More

Authors:  Lemessa Natae , Martinez-correa Santiago , Lerebo Wondwossen , Hwang Misun

Keywords:  intracranial arterial pulsation, intracranial hypertension, gray scale ultrasound

Dicamillo Paul,  Berlin Sheila,  Vasavada Pauravi

Final Pr. ID: Poster #: CR-010

Generalized arterial calcification of infancy (GACI) is a rare, often fatal disease due to cardiovascular sequellae (cyanosis, respiratory distress, hypertension and cardiomegaly) from widespread arterial calcification and/or narrowing of medium and large diameter vessels. Other findings can include periarticular calcification, pseudoxanthoma elasticum, hearing loss, intestinal ischemia, rickets and hypo/hyperphasphatemia. A database of worldwide cases implicates genes ENPP1 and ABCC6.

Our patient presented late in gestation. Although a 20 week fetal ultrasound was unremarkable, a 36 week ultrasound showed polyhydramnios, moderate pericardial effusion and moderate to severe tricuspid regurgitation; these findings prompted a C-section delivery. Early in his course, the patient developed biventricular dysfunction, systemic and pulmonary hypertension and respiratory failure requiring mechanical ventillation. Splenic calcifications, left pelvicaiectasis and lenticulostriate vasculopathy was documented in first week of life. The thoracic aorta, pulmonary artery and coronary artery were echogenic and thickened. Etidronate therapy, a treatment used for the first months to years of life to block bone mineralization until the arterial calcifications resolve, was started within 24 hours of life. However, this therapy can and did result in the development of rickets. Genetic testing revealed two mutations in the ABCC6 gene as can be seen in early onset GACI, a subtype with risk of pseudoxanthoma elasticum; our patient did exhibit hypermobile lower extremity joints. The patient's hypertension was eventually controlled with Amlodipine. Bulging fontenelles developed, likely due to ricket-impared skull growth. Calcification/narrowing of the bilateral carotids was seen. Additional complications included chronic pulmonary disease shown to be combination of chronic aspiration, nonspecific interstitial pneumonia and mild pulmonary arterial hypertensive changes. Rickets-related rib fractures further complicated the lung disease. Failure to thrive resulted in enteric feeding. Because of the severity of our patient's disease in which 6 month mortality can be as high as 85%, the treatment has aimed to prevent progression. Significant reduction in the arterial calcium burden has not yet been achieved, however the patient survived one year of treatment. Read More

Authors:  Dicamillo Paul , Berlin Sheila , Vasavada Pauravi

Keywords:  genetics, diphosphonate, hypertension

Zafar Faizeen,  Trout Andrew,  Palermo Joseph,  Alsaied Tarek,  Dillman Jonathan

Final Pr. ID: Paper #: 046

Portal hypertension is a manifestation of Fontan-related venous congestion and hepatic fibrosis. We studied the prevalence of radiologic evidence of portal hypertension (RP) and its association with Fontan hemodynamics and adverse outcomes. We also tested the validity of the VAST score, used in a prior study, in our population. Read More

Authors:  Zafar Faizeen , Trout Andrew , Palermo Joseph , Alsaied Tarek , Dillman Jonathan

Keywords:  Fontan, Portal Hypertension, Congenital Heart Disease