Scimitar syndrome with anomalous connection of left superior pulmonary vein to left innominate vein
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《血管的通路杂志》
Department of Cardiovascular and Thoracic Surgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram – 695011, Kerala, India
Abstract
Scimitar syndrome is a rare variant of partial anomalous pulmonary venous connection. The combination of scimitar syndrome with anomalous connection of the left superior pulmonary vein to the left innominate vein is extremely rare, and is yet to be documented in literature. We report the successful single-stage correction of such a rare type of anomalous pulmonary venous connection.
Key Words: Congenital heart disease; Scimitar syndrome; Anomalous pulmonary venous connection
1. Introduction
Partial anomalous pulmonary venous connection has numerous variations described both in surgical subjects and on autopsy [1]. Scimitar syndrome is one subtype constituting only 3–5% of all such anomalies [2,3]. Bilateral partial anomalous pulmonary venous connection is very rare with only a few cases being documented, the most common variant being the anomalous connection of the left superior pulmonary vein to the left innominate vein and right superior pulmonary vein to the superior vena cava-right atrium junction [1,4]. The combination of right-sided scimitar syndrome with partial anomalous pulmonary venous connection of left superior pulmonary vein to the innominate vein is extremely rare. We present our experience with successful single-stage correction of such a rare anomaly.
2. Case report
A 13-year-old girl presented in New York Heart Association functional class II with a history of recurrent respiratory tract infections. Salient clinical findings included absence of cyanosis, apical impulse in the right fifth intercostal space at the mid-clavicular line, wide and fixed normal intensity second heart sound and a grade 2/6 ejection systolic murmur at the right sternal border. Chest roentgenography showed dextroversion, elevated right dome of diaphragm, plethoric lung fields and the ‘scimitar’ sign parallel to the right cardiac border (Fig. 1a). Echocardiography revealed ostium secundum atrial septal defect with partial anomalous pulmonary venous connection of the right pulmonary veins to inferior vena cava – right atrium junction. Since pulmonary venous drainage of the left lung was not clear by echocardiography, cardiac catheterization confirmed the echocardiographic findings and, in addition, revealed anomalous drainage of the left upper pulmonary vein through the vertical vein into the left innominate vein (Fig. 1c and d). The left lower pulmonary vein had a normal drainage to the left atrium. Mean pulmonary artery pressure was 16 mm of mercury with a total pulmonary vascular resistance of 1.3 U/m2. Calculated left to right shunt ratio was 1.84. Aortogram did not show any systemic arterial supply to the right lung. Computed tomography of chest revealed right lung hypoplasia (Fig. 1b).
She underwent a total correction wherein the ‘scimitar’ vein was rerouted to the left atrium through the atrial septal defect using a pericardial patch under total circulatory arrest. The vertical vein was divided and its proximal part anastomosed to the left atrial appendage. She had an uneventful recovery. Her three-month follow-up echocardiogram showed normal pulmonary venous drainage to the left atrium, which was documented by cardiac catheterization (Fig. 2).
3. Discussion
Bilateral partial anomalous pulmonary venous connections are very rare [1,4]. Among them commonly described is the anomalous connection of the right superior pulmonary vein to the right superior vena cava with the left superior pulmonary vein to the left innominate vein [1,4].
Scimitar syndrome is characterized by partial or complete anomalous pulmonary venous drainage of the right or left lung to the inferior vena cava [3]. Right-sided scimitar syndrome is commonly associated with ipsilateral lung hypoplasia, dextroversion, elevation of ipsilateral diaphragm and ipsilateral pulmonary artery hypoplasia with systemic arterial supply to the lung [3,4]. Except for a normal pulmonary artery and absence of systemic arterial supply to the hypoplastic lung, our patient had all the classical characteristics of scimitar syndrome.
The surgical procedure was carried out under total circulatory arrest owing to the proximity of the hepatic veins to the scimitar vein–inferior vena cava junction, thereby rendering the isolation of inferior caval return difficult, technically.
The combination of right-sided scimitar syndrome with anomalous drainage of the left superior pulmonary vein to the left innominate vein is extremely rare, and to the best of our knowledge, is yet to be reported in medical literature. Pre-operative angiography has a central role in the diagnosis of such a variation when the pattern of pulmonary venous drainage is not clearly identified by echocardiography. This anomaly warrants early surgery as any pathology involving the normally drained left lower lobe may result in fatal hypoxia. Awareness of such variants of bilateral partial anomalous pulmonary venous connection is required for recognition and early successful single-stage complete correction. It would also avoid symptoms persisting post-operatively necessitating a second surgery for the missed partial anomalous pulmonary venous connection of the other side.
In conclusion, bilateral partial anomalous pulmonary venous connection of the right pulmonary veins to inferior vena cava with the left superior pulmonary vein to the left innominate vein is a rare anomaly and we report the first case of this type with successful complete single-stage correction.
References
Snellen HA, van Ingen HC, Hoefsmit EC. Patterns of anomalous pulmonary venous drainage. Circulation 1968;38:45–63.
Mathey J, Galey JJ, Logeais Y, Santoro E, Vanetti A, Maurel A, Wuerflein R. Anomalous pulmonary venous return into inferior vena cava and associated bronchovascular anomalies (the scimitar syndrome): report of three cases and review of the literature. Thorax 1968;23:398–407.
Najm HK, Williams WG, Coles JG, Rebeyka IM, Freedom RM. Scimitar syndrome: twenty years' experience and results of repair. J Thorac Cardiovasc Surg 1996;112:1161–1169.
Kouchoukos NT, Blackstone EH, Doty DB, Hanley FL, Karp RB. Atrial Septal Defect and Partial Anomalous Pulmonary Venous Connection: Kirklin/Barratt-Boyes cardiac surgery: morphology, diagnostic criteria, natural history, techniques, results and indications Third edition Philadelphia: Churchill Livingstone, Elsevier Science, 2003:715–747.(Manoranjan Misra, Adil Sa)
Abstract
Scimitar syndrome is a rare variant of partial anomalous pulmonary venous connection. The combination of scimitar syndrome with anomalous connection of the left superior pulmonary vein to the left innominate vein is extremely rare, and is yet to be documented in literature. We report the successful single-stage correction of such a rare type of anomalous pulmonary venous connection.
Key Words: Congenital heart disease; Scimitar syndrome; Anomalous pulmonary venous connection
1. Introduction
Partial anomalous pulmonary venous connection has numerous variations described both in surgical subjects and on autopsy [1]. Scimitar syndrome is one subtype constituting only 3–5% of all such anomalies [2,3]. Bilateral partial anomalous pulmonary venous connection is very rare with only a few cases being documented, the most common variant being the anomalous connection of the left superior pulmonary vein to the left innominate vein and right superior pulmonary vein to the superior vena cava-right atrium junction [1,4]. The combination of right-sided scimitar syndrome with partial anomalous pulmonary venous connection of left superior pulmonary vein to the innominate vein is extremely rare. We present our experience with successful single-stage correction of such a rare anomaly.
2. Case report
A 13-year-old girl presented in New York Heart Association functional class II with a history of recurrent respiratory tract infections. Salient clinical findings included absence of cyanosis, apical impulse in the right fifth intercostal space at the mid-clavicular line, wide and fixed normal intensity second heart sound and a grade 2/6 ejection systolic murmur at the right sternal border. Chest roentgenography showed dextroversion, elevated right dome of diaphragm, plethoric lung fields and the ‘scimitar’ sign parallel to the right cardiac border (Fig. 1a). Echocardiography revealed ostium secundum atrial septal defect with partial anomalous pulmonary venous connection of the right pulmonary veins to inferior vena cava – right atrium junction. Since pulmonary venous drainage of the left lung was not clear by echocardiography, cardiac catheterization confirmed the echocardiographic findings and, in addition, revealed anomalous drainage of the left upper pulmonary vein through the vertical vein into the left innominate vein (Fig. 1c and d). The left lower pulmonary vein had a normal drainage to the left atrium. Mean pulmonary artery pressure was 16 mm of mercury with a total pulmonary vascular resistance of 1.3 U/m2. Calculated left to right shunt ratio was 1.84. Aortogram did not show any systemic arterial supply to the right lung. Computed tomography of chest revealed right lung hypoplasia (Fig. 1b).
She underwent a total correction wherein the ‘scimitar’ vein was rerouted to the left atrium through the atrial septal defect using a pericardial patch under total circulatory arrest. The vertical vein was divided and its proximal part anastomosed to the left atrial appendage. She had an uneventful recovery. Her three-month follow-up echocardiogram showed normal pulmonary venous drainage to the left atrium, which was documented by cardiac catheterization (Fig. 2).
3. Discussion
Bilateral partial anomalous pulmonary venous connections are very rare [1,4]. Among them commonly described is the anomalous connection of the right superior pulmonary vein to the right superior vena cava with the left superior pulmonary vein to the left innominate vein [1,4].
Scimitar syndrome is characterized by partial or complete anomalous pulmonary venous drainage of the right or left lung to the inferior vena cava [3]. Right-sided scimitar syndrome is commonly associated with ipsilateral lung hypoplasia, dextroversion, elevation of ipsilateral diaphragm and ipsilateral pulmonary artery hypoplasia with systemic arterial supply to the lung [3,4]. Except for a normal pulmonary artery and absence of systemic arterial supply to the hypoplastic lung, our patient had all the classical characteristics of scimitar syndrome.
The surgical procedure was carried out under total circulatory arrest owing to the proximity of the hepatic veins to the scimitar vein–inferior vena cava junction, thereby rendering the isolation of inferior caval return difficult, technically.
The combination of right-sided scimitar syndrome with anomalous drainage of the left superior pulmonary vein to the left innominate vein is extremely rare, and to the best of our knowledge, is yet to be reported in medical literature. Pre-operative angiography has a central role in the diagnosis of such a variation when the pattern of pulmonary venous drainage is not clearly identified by echocardiography. This anomaly warrants early surgery as any pathology involving the normally drained left lower lobe may result in fatal hypoxia. Awareness of such variants of bilateral partial anomalous pulmonary venous connection is required for recognition and early successful single-stage complete correction. It would also avoid symptoms persisting post-operatively necessitating a second surgery for the missed partial anomalous pulmonary venous connection of the other side.
In conclusion, bilateral partial anomalous pulmonary venous connection of the right pulmonary veins to inferior vena cava with the left superior pulmonary vein to the left innominate vein is a rare anomaly and we report the first case of this type with successful complete single-stage correction.
References
Snellen HA, van Ingen HC, Hoefsmit EC. Patterns of anomalous pulmonary venous drainage. Circulation 1968;38:45–63.
Mathey J, Galey JJ, Logeais Y, Santoro E, Vanetti A, Maurel A, Wuerflein R. Anomalous pulmonary venous return into inferior vena cava and associated bronchovascular anomalies (the scimitar syndrome): report of three cases and review of the literature. Thorax 1968;23:398–407.
Najm HK, Williams WG, Coles JG, Rebeyka IM, Freedom RM. Scimitar syndrome: twenty years' experience and results of repair. J Thorac Cardiovasc Surg 1996;112:1161–1169.
Kouchoukos NT, Blackstone EH, Doty DB, Hanley FL, Karp RB. Atrial Septal Defect and Partial Anomalous Pulmonary Venous Connection: Kirklin/Barratt-Boyes cardiac surgery: morphology, diagnostic criteria, natural history, techniques, results and indications Third edition Philadelphia: Churchill Livingstone, Elsevier Science, 2003:715–747.(Manoranjan Misra, Adil Sa)