Ann Thorac Surg 2008;86:282-283. doi:10.1016/j.athoracsur.2007.11.025
© 2008 The Society of Thoracic Surgeons
Case Reports
Topsy-Turvy Heart With Associated Congenital Tracheobronchial Stenosis and Airway Compression Requiring Surgical Reconstruction
Patricio Herrera, MD,
Christopher A. Caldarone, MD,
Vito Forte, MD,
Helen Holtby, MD,
Peter Cox, MD,
Priscilla Chiu, MD, PhD,
Peter C.W. Kim, MD, PhD*, Airway Reconstruction Team*
Division of Cardiovascular Surgery, Hospital for Sick Children, Toronto, Ontario, Canada
Accepted for publication November 12, 2007.
* Address correspondence to Dr Kim, 555 University Ave, Rm 1286 (Hill Wing), Toronto, Ontario M5G 1X8, Canada (Email: peter.kim{at}sickids.ca).
 |
Abstract
|
|---|
We describe a case of "topsy-turvy heart" that necessitated simultaneous left tracheobronchial repair, aortic arch reconstruction, and bronchial stenting in an 11-month-old girl. This case highlights the significant therapeutic dilemmas clinicians are faced with when dealing with conditions having an unknown natural history and unpredictable surgical outcomes, and reinforces the need for these patients to be managed by a multidisciplinary team approach.
|
Introduction
|
|---|
Congenital rotational abnormalities of the heart, referred to as "topsy-turvy heart," are rare yet are recognized and described in the literature [1]. The associated airway anomalies that may lead to severe airway compromise and a treatment strategy for dealing with this rare combination are presented.
This baby girl was born at 36 weeks gestational age and weighed 2710 g. The mother was a 24-year-old G2P0A1 with a history of a terminated previous pregnancy because of an antenatal diagnosis of hypoplastic left heart syndrome. The parents were first cousins. Fetal ultrasound showed a "structurally normal heart."
Apgar scores were 8 and 9 at 1 and 5 minutes, respectively. The infant required management in the neonatal intensive care unit and pediatric intensive care unit for ongoing respiratory care. An echocardiogram and chest computed tomography (CT) demonstrated a malformation of the great vessels, described as a "topsy-turvy heart" [1]. This term depicts the rotated position of the heart, with the origin of the great vessels being inferior, close to the diaphragm and the apex, and pointing to the left and in a cephalad direction. Bronchoscopy and chest CT showed an extremely long, tortuous, and narrowed left main bronchus (LMB) coursing below the dilated and low aortic arch to the level of the left hemidiaphragm before turning upwards towards the pulmonary hilum (Fig 1). The patient was mechanically ventilated for 10 days and was transferred to the ward for optimization of nutritional status. The patent ductus arteriosus was surgically ligated at 20 days of age.
View larger version (118K):
[in this window]
[in a new window]
|
Fig 1. Noteworthy in this computed tomography coronal view of the chest is the dilated aortic arch in the center and the U-shaped left main bronchus coursing below it.
|
|
Progressive aortic arch dilatation caused compression of the right lower and intermediate bronchi, and the collapse of the malacic LMB resulted in air trapping of the left lung and further distortion of the anatomy by pushing the trachea to the right. As a consequence, she had multiple acute cyanotic episodes of respiratory distress with exertion or excitement. Two attempts to manage the patient at home failed, exhausting the parents after a short 5-day period, only to have the infant return to the hospital in respiratory distress and needing oxygen for resolution.
During the months of hospitalization, progressive hypercarbia (70 mm Hg) developed, and low-flow oxygen was needed to maintain acceptable saturations. A ventilation perfusion (V/Q) scan showed 46% vs 54% right-to-left ventilation and asymmetrical perfusion of 68% to 32% left to right. A better ventilated but poorly perfused left lung made resection nonfeasible.
After lengthy and thoughtful discussion with the parents and wide consultation among relevant specialties, all options were considered, including palliation.
At age 11 months the patient underwent airway reconstruction. A median sternotomy was performed. The apex of the heart was anterior and cephalad, with the great vessels oriented in a nearly anterior–posterior direction behind the heart. The child was placed on cardiopulmonary bypass with cannulation of the aorta and right atrium to decompress the heart and allow inspection and dissection of the posterior mediastinum to facilitate assessment of the surgical anatomy and options for correction. Exposure of the LMB was impossible without division of the transverse aortic arch.
After cardioplegic arrest, the aortic arch was divided and the LMB inspected. The bronchus appeared slightly small to normal in diameter but was flattened and very elongated. A slide bronchoplasty and complete left pulmonary hilum mobilization were performed, resulting in the shortening and widening of the LMB. The acute angle between the trachea and the LMB, along with a restrictive cartilage pattern, kept the bronchus closed despite the plasty. To attempt correction, an augmentation rib cartilage graft was performed at this site. Rigid bronchoscopy was performed to determine airway patency, but the carinal opening remained distorted. Despite several maneuvers, including aggressive toilet of bloody pulmonary secretions, there was a lack of ventilation to the left lung.
The arch was reconstructed by tailoring a banked cadaveric homograft to lengthen and reposition the arch to the left side to avoid direct compression of LMB [2]. An omental flap was used to protect the tracheobronchial and vascular suture lines.
The procedure was well tolerated, including the 7-hour duration on pump. She was mechanically ventilated for the next week, with excellent gasometry, despite a nonaerated left lung. The LMB could not be entered by flexible bronchoscopy, but patency was confirmed by segmental bronchography.
On postoperative day 11, a flexible Genesis biliary stent (PG2510B; Cordis, Johnson & Johnson, Miami Lakes, FL) was inserted into the LMB (Fig 2), achieving complete aeration of the left lung by the third day after stenting. Extubation occurred on postoperative day 15, and supplementary oxygen was discontinued by week 4 after the procedure.
View larger version (15K):
[in this window]
[in a new window]
|
Fig 2. This diagram shows the preoperative and postoperative anatomy of the airway and aortic arch. Note the stent position over the area of slide plasty and the change in the angle between the trachea and the left main bronchus. (AA = aortic arch; LMB = left main bronchus; RLL = right lower lobe; RML = right middle lobe; RUL = right upper lobe.)
|
|
The patient has been home for nearly 2 years and is asymptomatic and thriving. She is remarkably normal developmentally. A chest roentgenogram follow-up has been satisfactory (Fig 3).
|
Comment
|
|---|
We report a case of topsy-turvy heart in association with severe airway malformations and the treatment strategy that led to a favorable outcome (albeit short-term follow-up).
A rare ("one-of-a-kind") congenital malformation with an unknown natural history and a lack of clinical outcome data poses a number of ethical, medical, and surgical challenges to health care providers and parents alike. This case highlights the importance of a multidisciplinary approach and inclusion of the family in the key decision-making processes that involve the application and consumption of technologic as well as human resource.
|
Appendix
|
|---|
Airway Reconstruction Team
Priscilla Chiu, MD, PhD, Peter C. W. Kim, MD, PhD, and Patricio Herrera (fellow), MD, general surgery; Peter Chait, MBBCh, FFRAD, and Yoo Shi-Joon, MD, PhD, radiology; Felix Ratjen, MD, FRCP(C), pulmonology; Sanjay Mahant, MD, FRCPC, general pediatrics; Christopher Caldarone, MD, cardiovascular surgery; Vito Forte, MD, FRCP(C), and Paolo Campisi, MSc, MD, otolaryngology; Helen Holtby, MD, anesthesia; and Peter Cox, MB, ChB, intensive care.
|
Acknowledgments
|
|---|
We would like to thank Dr Richard Azizkhan for his confident support and valuable case discussion-consultation and all the members of the Airway Reconstruction Team (see Appendix).
|
Footnotes
|
|---|
* See Appendix for the members of the Airway Reconstruction Team. 
|
References
|
|---|
- Freedom RM, Culham JG, Moes CF. Angiocardiography of congenital heart diseaseNew York, NY: Macmillan Ed; 1984. pp. 629-642.
- Pretre R, Turina MI. Relief of bronchial compression caused by congenital heart defect by remodeling of the aortic arch J Thorac Cardiovasc Surg 2000;119:172-173.
Top
Abstract
Introduction
