Franco-Fuenmayor et al. Int J Clin Cardiol 2019, 6:150 Volume 6 | Issue 3 DOI: 10.23937/2378-2951/1410150 Open Access ISSN: 2378-2951 International Journal of Clinical Cardiology • Page 1 of 3 • Citaton: Franco-Fuenmayor ME, Aleem NA, Salazar JD, Aly AM (2019) Unroofed Coronary Sinus Syn- drome Presentng with Early Pulmonary Hypertension. Int J Clin Cardiol 6:150. doi.org/10.23937/2378- 2951/1410150 Accepted: June 11, 2019; Published: June 13, 2019 Copyright: © 2019 Franco-Fuenmayor ME, et al. This is an open-access artcle distributed under the terms of the Creatve Commons Atributon License, which permits unrestricted use, distributon, and reproducton in any medium, provided the original author and source are credited. Franco-Fuenmayor et al. Int J Clin Cardiol 2019, 6:150 Unroofed Coronary Sinus Syndrome Presentng with Early Pulmonary Hypertension Maria E Franco-Fuenmayor, MD 1* , Numra A Aleem, MD 1 , Jorge D Salazar, MD 2 and Ashraf M Aly, MD 3 1 Department of Pediatrics, University of Texas Medical Branch, USA 2 Division of Pediatric Cardiovascular Surgery, University of Texas McGovern Medical School and Children’s Memorial Hermann Hospital, USA 3 Division of Pediatric Cardiology, Department of Pediatrics, University of Texas Medical Branch, USA *Corresponding author: Maria E Franco-Fuenmayor, Department of Pediatrics, University of Texas Medical Branch, 301 University Blvd. RB6 Room 3.230, Galveston, TX, 77555, USA Abstract Isolated partial or complete absence of the coronary sinus roof hemodynamically behaves like an atrial septal defect and rarely causes pulmonary hypertension. We report a case of unroofed coronary sinus (URCS) presenting with post-operative cyanosis due to pulmonary hypertensive crisis following surgical repair of a ventricular septal defect at 6 months of age. He had progressively decreased stamina with gradual increase in pulmonary pressure requiring surgical closure of the URCS by 6 years of age with resolution of symptoms and normalization of the RV pressure. Keywords Coronary sinus defect, Pulmonary hypertension, Congenital heart disease, Congenital heart surgery CASE REpoRt Case A two-week-old male infant was referred for eval- uaton of a heart murmur. An echocardiogram (echo) showed a large membranous VSD and a dilated CS without persistent LSVC. At 5 weeks, he developed con- gestve heart failure managed initally medically but required surgical repair at 6 months of age. Post-op- eratvely, he developed cyanosis and underwent car- diac catheterizaton that revealed a right to lef shunt across a large defect in the CS roof “Qp/Qs rato of 0.6 and mildly elevated right atrial (RA) and right ventricu- lar (RV) end-diastolic pressures (8 mmHg) and elevated pulmonary artery pressure to 60 mmHg. The pulmonary resistance was also increased to 3.8 Woods units. The pulmonary pressure gradually decreased and the URCS was monitored clinically [3]. The pulmonary hyperten- sion was initally managed with inhaled nitric oxide and supplemental oxygen and later with oral Sildenafl for 3 months. He did relatvely well except for some growth delay, progressive exercise intolerance and snoring with a gradual increase in RV pressure. He underwent ton- sillectomy and adenoidectomy to exclude upper airway obstructon as the cause at 5 years of age. Sequental echocardiograms demonstrated gradual increases in RV pressure. An echo done at 6 years revealed a lef to right shunt across a large size defect (14 mm) between the CS and LA (Figure 1 and Figure 2). A 30 mmHg TR jet ve- Check for updates Introducton URCS is a rare congenital heart defect (CHD) com- monly associated with persistent lef superior vena cava (LSVC), VSD, cortriatriatum, tetralogy of Fallot, total anomalous pulmonary venous connectons and hetero- taxy [1]. Clinical presentaton depends on the associat- ed CHD, though when isolated it behaves like an ASD. This defect increases the risk for paradoxical emboli, brain abscess or atrial arrhythmias [2]. As with other types of ASD, it is unlikely for URCS to present with early pulmonary hypertension.