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

Kooraki Soheil,  Iskander Paul,  Ghahremani Shahnaz,  Prosper Ashley,  Finn John,  Bedayat Arash

Final Pr. ID: Poster #: CR-003

Scimitar syndrome, also called congenital venolobar syndrome, is a rare phenomenon characterized by partial anomalous pulmonary venous return (PAPVR) with variable degrees of right lung and pulmonary artery hypoplasia. In this report, we present an extremely rare case demonstrating co-occurrence of Tetralogy of Fallot (TOF) and scimitar syndrome.

A two-day-old female was presented with dyspnea and acidosis. An echocardiography and subsequent ECG-gated CT angiogram of the chest revealed findings of TOF and complete pulmonary atresia, accompanied by arterial and ventricular septal defects (ASD, VSD) and a large patent ductus arteriosus (PDA) connecting to hypoplastic left pulmonary artery. Additionally, PAPVR was observed with drainage from the hypoplastic right lung to the IVC in the form of scimitar syndrome. Correctional surgery was undertaken, with creation of a right ventricular to pulmonary artery conduit, pulmonary arterio-plasty, ASD closure and creation of a fenestrated VSD patch. Follow-up imaging in two years showed development of multiple aortopulmonary collateral arteries (MAPCAs), persistent PAPVR and development of chronic right lung interstitial edema.

When there is suspicion for the association of TOF and scimitar syndrome, CT angiography, gated cardiac MRI, and 3-D contrast enhanced MR angiography are all preferred diagnostic modalities, providing excellent delineation of the abnormal pulmonary vein, its course and drainage, and quantification of the pulmonary venous flow. The anomalous vein most commonly drains into the IVC and less likely to the hepatic veins, right atrium, or portal vein.

While scimitar syndorme in conjunction with Tetralogy of Fallot is an extremely rare association, meticulous evaluation of the pulmonary venous connections is important in all patients with TOF, as the presence of PAPVR can result in right ventricular dilation and dysfunction. Read More

Authors:  Kooraki Soheil , Iskander Paul , Ghahremani Shahnaz , Prosper Ashley , Finn John , Bedayat Arash

Keywords:  Scimitar, Tetralogy Of Fallot, PAPVR

Ndolo Josephine,  Crane Gabriella,  Hernanz-schulman Marta

Final Pr. ID: Poster #: CR-001

Introduction
Scimitar syndrome and subdiaphragmatic total anomalous pulmonary venous connections (TAPVC) are congenital pulmonary vascular anomalies that are not typically diagnosed on routine abdominal sonography. We present three cases of ultrasound diagnosis of neonates with subdiaphragmatic pulmonary veins diagnosed on abdominal ultrasound; to the best of our knowledge, this is the first reported case of abdominal ultrasound diagnosis of previously unknown scimitar syndrome.

Case reports
Case 1: 16 day old girl born at term with cardiac dextroposition and right lung hypoplasia. On abdominal ultrasound, a large vascular structure was seen draining into the inferior vena cava (IVC) near the hepatic confluence, arising in lung above the diaphragm. Doppler confirms venous flow into the IVC (Figure 1); an arterial aortic branch with pulmonary spectral Doppler signature, extended into the base of the right lung. The diagnosis of scimitar syndrome was made, confirmed several weeks later with CT angiography.

Case 2: One day old girl born at 29 weeks gestation had a prenatal diagnosis of complex congenital heart disease. Abdominal ultrasound demonstrated asplenia. As part of the complex, the examination demonstrated the common pulmonary vein draining into the left portal vein, decompressing into the heart via the ductus venosus, with color and spectral demonstration of flow direction and velocity. (Figure 2) Closure of the ductus venosus contributed to the demise of this child who was inoperable due to her extreme prematurity and her other congenital cardiac lesions.

Case 3: Eight day old term infant with multiple congenital anomalies. An abdominal ultrasound for heterotaxy evaluation demonstrated a large anomalous vessel arising in the chest between the descending aorta and esophagus posteriorly, and the atrium anteriorly, descending into the abdomen draining into the portal vein, with a partially obstructing membrane near the insertion point. (Figure 3). The TAPVC was repaired on day 9 of life with direct anastomosis to the left atrium.

Conclusion:
In conclusion, subdiaphragmatic vascular components of thoracic anomalies should be recognized at abdominal sonography by pediatric radiologists, who may be the first to both recognize and diagnose these conditions. Read More

Authors:  Ndolo Josephine , Crane Gabriella , Hernanz-schulman Marta

Keywords:  total anomalous pulmonary venous connection (TAPVC), Scimitar syndrome, ultrasound