Scimitar Syndrome with Tetralogy of Fallot and Pulmonary Atresia

The Egyptian Heart Journal (2014) xxx, xxx–xxx

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SHORT COMMUNICATION Scimitar syndrome with tetralogy of fallot and pulmonary atresia

Sameh R. Ismail *, Mustafa A. Al-Muhaya, Mohamed S. Kabbani

King Abdulaziz Medical City, King Saud University for Health Sciences, Department of Cardiac Sciences, National Guard hospital Health Affairs, Riyadh, Saudi Arabia

Received 2 June 2014; accepted 27 August 2014

KEYWORDS Abstract Scimitar syndrome is a rare variant of partial anomalous pulmonary venous connection. Scimitar syndrome; The association of Scimitar syndrome with another cardiac congenital anomaly such as tetralogy of Tetralogy of fallot; Fallot with pulmonary atresia is extremely rare; we are reporting a successful combined treatment Pulmonary atresia using transcatheter closure of major aorto-pulmonary collateral and a single-stage surgical correction in eighteen month old boy diagnosed as Scimitar syndrome with tetralogy of Fallot and pulmonary atresia. ª 2014 Production and hosting by Elsevier B.V. on behalf of Egyptian Society of Cardiology.

1. Case report arteriosus, bilateral superior vena cava and partial anomalous pulmonary venous drainage (PAPVD). The right pulmonary An eighteen month old boy was referred to our center due to veins are draining to the inferior vena cava–right atrium cyanosis and shortness of breath. He was a product of full term (IVC–RA) junction (Fig. 1). In order to confirm the diagnosis normal vaginal delivery and the first child in his family. His Cardiac Computed Tomography and Angiography (CT angio- birth weight was 3.5 kg. On admission his weight was 14 kg gram) was performed, it confirmed that the right pulmonary (95% centile) and height 83 cm (95% centile), oxygen satura- veins are draining to the IVC–RA junction above the dia- tion on room air 75–80%. The chest radiography showed mes- phragm, with two major aorto-pulmonary collaterals toward ocardia and curvilinear shadow in the right chest (Scimitar the lower lobe of the right lung (Fig. 2). Trans-catheter coil sign). Based on the clinical and chest radiography findings embolization of these major systemic collaterals was done suc- Scimitar syndrome was suspected. 2D echocardiogram cessfully, under general anesthesia; the patient was prepped revealed large sub aortic ventricular septal defect with outlet and draped as per protocol. The left femoral vein was accessed extension, overriding aorta, atretic pulmonary valve with con- percutaneously using a Vygon 20 sheath. The right femoral fluent pulmonary artery branches, moderate size patent ductus artery was accessed using the modified Seldinger technique and a 4 French sheath was inserted over a guide wire. Angiog- raphy of the descending Aorta was performed which showed a * Corresponding author. Address: Department of Cardiac Sciences (MC: 1420), King Abdulaziz Medical City, P.O. Box 22490, Riyadh patent left sided Aortic arch, confluent pulmonary arteries, 11426, Saudi Arabia. Fax: +966 118011111x16855. and two major Aorto-pulmonary collaterals supplying the E-mail address: [email protected] (S.R. Ismail). lower lobe of the right lung. A 6 mm Amplatzer vascular plug Peer review under responsibility of Egyptian Society of Cardiology. II was deployed in the lower collateral, and a 5 · 5 coil http://dx.doi.org/10.1016/j.ehj.2014.08.003 1110-2608 ª 2014 Production and hosting by Elsevier B.V. on behalf of Egyptian Society of Cardiology.

LA AO LV VSD

Figure 1 Echo picture – sub-costal view showing sub-aortic ventricular septal defect (VSD) with aortic overriding. LA: Left atrium; LV: Left ventricle; RV: Right ventricle. Figure 3 Coil occlusion of the 2 abnormal collaterals supplying the right lung.

resected and right ventricle to pulmonary artery contegra con- duit size 16 mm was inserted. The lowest temperature was 30 C. The patient was weaned uneventfully from cardiopul- Scimitar monary bypass. Routine trans-esophageal Echocardiography Vein (TEE) showed free IVC inﬂow and no obstruction at the level of the pulmonary veins. The child recovered well after surgery with normal saturation and was discharged home with satis- factory condition.

2. Discussion

Scimitar syndrome or congenital pulmonary venolobar syndrome is a rare entity but a well described constellation of cardiopulmonary anomalies. The term ‘‘Scimitar’’ was first used by Neill2,9 to describe the syndrome. This anomaly consists of partial or total anomalous venous connection of the right lung draining into the inferior vena cava or the azygos system, Figure 2 Angio CT scan showing anomalous pulmonary venous concomitant with lung hypoplasia and anomalous systemic drainage of the right lung to the IVC–RA junction (red arrow). arterial blood supply to the right lung rising from the abdom- inal aorta or its branches.1 These anomalous vessels are often visible on chest X-ray as a curvilinear shadow just above the right diaphragm resembling a Turkish sword or scimitar.10 embolized also in the lower collateral. A 3 · 3 coil was embol- The incidence of Scimitar syndrome was reported as 0.5–1% ized in the upper collateral (Fig. 3). Then PA/VSD and PAP- of CHD. The incidence of associated congenital cardiovascular VD were repaired surgically. abnormalities is about 36% in the pediatric age group and After longitudinal sternotomy; systemic heparinization and 75% among neonates. These abnormalities include atrial cardiopulmonary bypass were established. The cardioplegia septal defect, ventricular septal defect, and coarctation of the solution was infused through the needle inserted at the aortic aorta. Other congenital anomalies involving the aortic arch root and the heart was arrested. Wide right atriotomy was per- and abnormal relationship of the pulmonary arteries and lung formed downward till the lateral wall of the IVC, autologous abnormalities such as hypoplasia and horseshoe lung should pericardial patch was inserted rerouting the pulmonary vein be considered in the work out of Scimitar syndrome flow toward the atrial septal defect (ASD), keeping the IVC patients.3,7–9,11 Several literatures describe other rare associa- inflow free from any residual stenosis. Through the same atri- tions of Scimitar syndrome with other cardiac abnormalities: otomy the ventricular septal defect (VSD) was closed by a Hakim et al. described two cases of Scimitar syndrome, one Gortex patch. Longitudinal ventriculotomy was performed, case was scimitar syndrome with Sub-Aortic membrane, right ventricular outflow tract (RVOT) muscle bands were and another where the scimitar vein showed tortuous and

Please cite this article in press as: Ismail SR et al. Scimitar syndrome with tetralogy of fallot and pulmonary atresia, The Egypt Heart J (2014), http://dx.doi.org/ 10.1016/j.ehj.2014.08.003 Scimitar syndrome 3 abnormal course of the pulmonary veins with normal drainage Conflict of interest into the left atrium.1 Takeda reported two cases: in one case the scimitar vein entered both the inferior vena cava and the I disclose that there were no conflict of interest and no funding left atrium without any intracardiac shunt; in the other case for this study and it was approved by the hospital ethics the Scimitar vein showed a meandering course and then committee. drained into the left atrium, so surgical intervention was not required.6 Bilateral scimitar syndrome with abnormal venous drainage to inferior vena cava has also been described.11 References Isolated partial anomalous pulmonary venous return to the inferior caval vein is also called incomplete scimitar syn- 1. Hakim F, Madani A, Abu Haweleh A. Two cases report of drome.4 The typical scimitar sign was present in 57 of the 67 scimitar syndrome: the classical one with subaortic membrane and the scimitar variant. Bahrain Med Bull 2000;22(1):1–5. cases reviewed by Kiely et al.1,5 Association of pulmonary 2. Neill CA, Ferencz C, Sabiston DC, Sheldon H. The familial atresia, ventricular septal defect and Scimitar syndrome is a occurrence of hypoplastic right lung with systemic arterial supply very rare combination. Our case had the classical characteris- and venous drainage ‘‘scimitar syndrome’’. Bull Johns Hopkins tics of Scimitar syndrome with pulmonary atresia and Hosp 1960;107:1–21. ventricular septal defect. Pre-operative diagnostic cardiac 3. Moss, Adams. Heart disease in infant children andadolescent: catheterization and CT angiography have central role in the including the fetus and young adult. In: Emmanouilides GC, Allen diagnosis of such variation especially when the pattern of A, Thomas A, et al., editors. Baltimore: Williams and Wilkins; pulmonary venous drainage is not clearly identified by 2008. p. 761–92, 7th ed. echocardiography.4 After successful combined trans-catheter 4. Shegal Arvind, Loughran-Fowlds Alison. Scimitar syndrome. and surgical repair, the patient had smooth outcome and Indian J Pediatr 2005;72(3):249–51. 5. Kiely B, Flier J, Stenes, et al. A syndrome of anomalous venous was discharged home with full recovery. One month later an drainage of the right lung to inferior vena cava. Am J Cardiol echocardiography follow-up was performed showing no 1967;20:102. residual cardiac lesions and pulmonary vein flow pattern was 6. Takeda SI, Imachi T, Arunitsu K, et al. Two cases of scimitar normal. As the risk of baffle obstruction at follow-up is well variant. Chest 1994;105(1):292–3. 12 reported we keep our patients on low dose aspirin therapy 7. Morgan JR, Forker AD. Syndrome of hypoplasia of the right for prophylaxis. lung. Circulation 1971;43(1):27–30. We can conclude from this case study that CT angiogram 8. Gikonyo DK, Tandon R, Lucas Jr RV, Edwards JE. Scimitar and cardiac angio catheterization are the most appropriate syndrome in neonates: report of four cases and review of the diagnostic modalities to confirm the anatomy of this unusual literature. Pediatr Cardiol 1986;6:193–7. variant of Scimitar syndrome. Combination of therapeutic 9. zadeh Soheila Khalil, Hassanzad Maryam, Khodayari Amir-Ali. Scimitar syndrome. Arch Iran Med 2009;12(1):79–81. angiography and surgery was feasible, and contributed to the 10. Mulligan ME. History of scimitar syndrome. Radiology success of this case management. Elimination of collateral 1999;210(1). flow, correcting the venous drainage and correcting intra- 11. Kabbani M, Haider N, Abu-Sulaiman R. Bilateral scimitar cardiac abnormality help in preventing the development of syndrome. Cardiol Young 2004;14(4):447–9. pulmonary hypertension and normalize the blood flow to the 12. Najm HK, Williams WG, Coles JG, Rebeyka IM, Freedom RM. lung which make the hybrid approach ideal for short and long Scimitar syndrome: twenty years experience and results of repair. J term results. Thorac Cardiovasc Surg 1996;112:1161–9.

Please cite this article in press as: Ismail SR et al. Scimitar syndrome with tetralogy of fallot and pulmonary atresia, The Egypt Heart J (2014), http://dx.doi.org/ 10.1016/j.ehj.2014.08.003