A rare constellation of eventration of right diaphragm, severe right lung hypoplasia, and LPA sling
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CASE REPORT
A rare constellation of eventration of right diaphragm, severe right lung hypoplasia, and LPA sling Ananthanarayanan Chandrasekaran 1 & Azamal Ibrahim Mulla 2 & Ramesh Patel 3 & Yashpal Rana 4 & Amit Mishra 5 Received: 29 February 2020 / Revised: 2 May 2020 / Accepted: 13 May 2020 # Indian Association of Cardiovascular-Thoracic Surgeons 2020
Abstract Left pulmonary artery sling (LPAS) is a rare congenital cardiac anomaly and is commonly associated with complete tracheal rings and various degrees of tracheobronchial compression. We present a very rare association of LPAS and congenital eventration of right hemidiaphragm in this case report. Keywords Left pulmonary artery . Congenital eventration of diaphragm . Atrial septal defect
Introduction Left pulmonary artery sling (LPAS) is a rare vascular malformation with reports claiming a hospital-based prevalence of 0.14% among patients with congenital heart defects [1]. It has varied presentations from an incidental finding in adults to severe stridor and respiratory distress in infants. Congenital eventration of diaphragm (CED) is another rare condition caused by a developmental defect of the muscular portion of the diaphragm [2]. We present a very rare association of LPAS and CED in an infant.
Case report A 2-month-old girl presented to us with stridor and difficulty in breathing since birth and was diagnosed to
have LPAS and CED of the right hemi-diaphragm. Echocardiography and computed tomographic (CT) angiography was performed to know further details. The left pulmonary artery (LPA) was arising from the proximal right pulmonary artery (RPA), looping around the trachea and proximal right main bronchus and crossing the midline to enter into the left hilum. There was an associated small atrial septal defect (ASD) with left to right shunt, mild pulmonary arterial hypertension, with preserved right ventricular function (Fig. 1a–c). The liver was found occupying most of the right hemithorax and the right lung was severely hypoplastic. The right main bronchus and RPA were hypoplastic at hilum with a Z score of − 3 (Fig. 2a–c). There was no evidence of complete tracheal rings or other tracheal anomalies. As the child had stridor due to tracheal compression by the LPA along with respiratory distress due to CED, we planned
* Amit Mishra [email protected]
3
Department of Cardiac Anesthesia, U. N. Mehta Institute of Cardiology and Research Center (affiliated to BJ Medical College, Ahmedabad), Gujarat, India
1
Department of Cardiovascular and Thoracic Surgery, U. N. Mehta Institute of Cardiology and Research Center (affiliated to BJ Medical College, Ahmedabad), Gujarat, India
4
Department of Radiology, U. N. Mehta Institute of Cardiology and Research Center (affiliated to BJ Medical College, Ahmedabad), Gujarat, India
5 2
Department of Pediatric Cardiovascular and Thoracic Surgery, U. N. Mehta Institute of Cardiology and Research Center (affiliated to BJ Medical College, Ahmedabad), Gujarat, India
Department of Pediatric Cardiovascular and Thorac
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