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Imaging Cardio-vascular Anatomy and Function in D-Transposition of the Great Arteries after Mustard Procedure
CLINICAL SPOTLIGHT
Clinical Spotlight
Imaging Cardio-vascular Anatomy and Function in D-Transposition of the Great Arteries after Mustard Procedure Lucas Joerg, MD, Cameron Bridgman, FRACP and Joseph B. Selvanayagam, FRACP ∗ Department of Cardiovascular Medicine, Flinders Medical Centre, Flinders University, Flinders Drive, Bedford Park, 5042 South Australia, Australia
We used cardiovascular magnetic resonance (CMR) to demonstrate cardio-vascular anatomy and function in a 42 year-old man with a D-Transposition of the great arteries who survived a sudden cardiac arrest. (Heart, Lung and Circulation 2011;20:666–668) © 2010 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved. Keywords. Cardiovascular magnetic resonance (CMR); D-Transposition of the great arteries; Congenital heart diseases; Sudden cardiac arrest
Case Presentation
T
his 42 year-old man presented via paramedic services after an out-of-hospital ventricular fibrillation cardiac arrest and successful resuscitation. Further history revealed that he suffered from a D-Transposition of the great arteries (TGAD) and had undergone surgery with a Mustard procedure in his first week of life as well as surgery for coarctation of the aorta. He had had redo baffle surgery at age 11. He was on treatment with an ACE-inhibitor as well as with clopidogrel for recurrent idiopathic stroke. The cardiac biomarkers were elevated with a troponin peaking at 2.04 g/L and creatine kinase (CK) peaking at 1557 U/L. Echocardiography showed biventricular dilatation and moderately impaired systolic function of both the systemic (anatomic right) ventricle, and the subpulmonic (anatomic left) ventricle. Coronary angiography showed unobstructed coronaries. Cardiac MRI was performed for better delineation of ventricular, great vessel and baffle anatomy prior to the implantation of a defibrillator. Anatomical images showed widely patent baffles without any obvious stenosis or leakage and a minimal residual aortic coarctation. Functional images revealed moderate to severe systemic (right ventricle) and subpulmonic (left ventricle) dysfunction with a calculated ejection fraction of
Received 31 July 2009; received in revised form 24 February 2011; accepted 7 March 2011 ∗
Corresponding author. Tel.: +61 884042195; fax: +61 882045625. E-mail addresses: [email protected], [email protected] (L. Joerg), [email protected] (C. Bridgman), [email protected] (J.B. Selvanayagam).
31% and 38% respectively. Furthermore, mild to moderate tricuspid valve regurgitation was noticed (Fig. 1). Delayed hyperenhancement technique with intravenous gadolinium-DTPA contrast agent (0.1 mmol/kg) showed patchy mid-wall hyperenhancement of the ventricular septum indicating myocardial fibrosis (Fig. 2).
Discussion The incidence of congenital heart disease is difficult to ascertain but varies from 4/1000 to 50/1000 live births. In transposition of the great arteries, which accounts for 5% of the cases of congenital heart disease, there is ventriculoarterial dissociation with the two main arteries being connected to the “wrong” ventricles. The aorta leaves the right ventricle and takes venous blood to the body whilst the pulmonary artery leaves the left ventricle and transfers oxygenated blood to the lungs. This condition is three times more common amongst males [1,2]. Systemic oxygen saturation falls to very low levels shortly after birth and death would ensue without emergent interventions such as reopening of the ductus arteriosus with Prostaglandin E and increasing shunting at the atrial level by balloon atrial septostomy “Rashkind procedure” to improve oxygenation. In 50% of the cases, D-type Transposition of the great arteries is associated with other defects. These include ventricular septal defects, aortic coarctation, coronary artery abnormalities, and left ventricular outflow tract obstruction [1,2]. Patients with this anomaly are not expected to survive without a more definitive surgical procedure. The atrial switch procedures by Senning (1958) and Mustard (1964) created baffles within the heart
© 2010 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved.
1443-9506/04/$36.00 doi:10.1016/j.hlc.2011.03.001
Joerg et al. Cardio-vascular anatomy and function
667
Fig. 1. Panel (A) is a sagittal ‘white blood’ image of the heart illustrating typical D-Transposition anatomy. The aorta (Ao) leaves the subaortic right ventricle (RV) whilst the pulmonary artery (PA) leaves the subpulmonic left ventricle (LV). Panels (B) and (C) show the patent baffles which direct the blood flow from one atrium to the other after the Mustard repair (arrows).
Fig. 2. Panels (A) (horizontal long axis) and (B) (basal short axis) demonstrate DE-CMR images illustrating patchy mid-wall hyperenhancement of the ventricular septum indicating fibrosis (arrows).
which directed blood flow from one atrium to the opposite semilunar valve and ventricle effectively correcting the abnormal flow of blood. This technique improved longterm survival up to 80% at 28 years [2,3], but also resulted in important sequelea such as reduced exercise capacity, increased risk of brady/tachy-arrythmias, and sudden cardiac death as well as right heart failure due to overload of the systemic ventricle [2–4]. Other complications include scarring and obstruction of the baffles, baffle leaks or multifactorial pulmonary hypertension [2,3]. Today the surgical repair of choice of TGAD, is the Jatene arterial switch operation (1976), usually performed in the first few weeks of life. This involves the transection and reattaching of the great arteries above the semilunar valves, as well as the reimplantation of the coronary arteries into the new aortic root. In most of the cases normal cardiac function and physiology can be achieved as cardiac anatomy is restored. Therefore this approach is superior to the atrial switch operations and the prognosis for arterial switch patients is very good. A follow-up study of 1095 patients showed freedom from intervention in 82% with no further deaths between 10 and 15 years after surgery (88% survival). Arrhythmias and heart block are rare. However,
it should be borne in mind that there is limited long-term follow up data. Some degree of dysfunction in the coronary arteries, left ventricle, pulmonary valve, and/or the aortic valve is somewhat more common, occurring in 5–25% of patients [5].
Treatment and Management Sudden death is the most common cause of late mortality in this population. Our patient who survived sudden cardiac arrest clearly qualified for implantation of an implantable defibrillator (ICD). The data provided by CMR imaging especially the exact delineation of the vessel- and baffle anatomy was of major assistance in the planning of the ICD implantation. The patient recovered well and was subsequently discharged on standard heart failure therapy (ACEI, betablocker) with follow up in the adult congenital heart disease clinic.
Conclusion Echocardiography cannot always provide the necessary information for the evaluation and treatment of patients
CLINICAL SPOTLIGHT
Heart, Lung and Circulation 2011;20:666–668
CLINICAL SPOTLIGHT
668
Joerg et al. Cardio-vascular anatomy and function
with a congenital heart disease. Cardiac MRI with its excellent delineation of cardiac and baffle anatomy was crucial in facilitating management and enabling safer cardioverter defibrillator insertion. Delayed enhancement technique with gadolinium showed patchy mid-wall hyperenhancement of the ventricular septum indicating fibrosis. This area may well have been the location where this patient’s ventricular arrhythmia originated. In addition, functional images allowed an accurate assessment of the myocardial systolic function of both the subpulmonic (left ventricle) and especially the systemic right ventricle. Follow-up of the RV systolic function is of major importance in this patient population because many may ultimately require transplantation due to eventual cardiac failure. CMR is an outstanding imaging tool to evaluate patients with a complex cardiac anatomy and will be increasingly used in the future as the numbers of adults survivors with congenital heart diseases increase due advances in surgical techniques and medical care.
Competing Interests The author(s) declare that they have no competing interests.
Heart, Lung and Circulation 2011;20:666–668
Acknowledgements There was no source of funding used for this study.
Appendix A. Supplementary data Supplementary data associated with this article can be found, in the online version, at doi:10.1016/ j.hlc.2011.03.001.
References [1] Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002;39(June (12)):1890–900. Review. [2] Sommer RJ, Hijazi ZM, Rhodes Jr JF. Pathophysiology of congenital heart disease in the adult. Part I Shunt lesions. Circulation 2008;117(February (8)):1090–9. [3] Warnes CA. Transposition of the great arteries. Circulation 2006;114(December (24)):2699–709. Review. [4] Michael KA, Veldtman GR, Paisey JR, Yue AM, Robinson S, Allen S, et al. Cardiac defibrillation therapy for at risk patients with systemic RV dysfunction secondary to atrial redirection surgery for dextro-transposition of the great arteries. Europace 2007;9(May (5)):281–4. [5] Losay J, Touchot A, Serraf A, Litvinova A, Lambert V, Piot JD, et al. Late outcome after arterial switch operation for transposition of the great arteries. Circulation 2001;104(September (12 Suppl. 1)):I121–6.
Clinical Spotlight
Imaging Cardio-vascular Anatomy and Function in D-Transposition of the Great Arteries after Mustard Procedure Lucas Joerg, MD, Cameron Bridgman, FRACP and Joseph B. Selvanayagam, FRACP ∗ Department of Cardiovascular Medicine, Flinders Medical Centre, Flinders University, Flinders Drive, Bedford Park, 5042 South Australia, Australia
We used cardiovascular magnetic resonance (CMR) to demonstrate cardio-vascular anatomy and function in a 42 year-old man with a D-Transposition of the great arteries who survived a sudden cardiac arrest. (Heart, Lung and Circulation 2011;20:666–668) © 2010 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved. Keywords. Cardiovascular magnetic resonance (CMR); D-Transposition of the great arteries; Congenital heart diseases; Sudden cardiac arrest
Case Presentation
T
his 42 year-old man presented via paramedic services after an out-of-hospital ventricular fibrillation cardiac arrest and successful resuscitation. Further history revealed that he suffered from a D-Transposition of the great arteries (TGAD) and had undergone surgery with a Mustard procedure in his first week of life as well as surgery for coarctation of the aorta. He had had redo baffle surgery at age 11. He was on treatment with an ACE-inhibitor as well as with clopidogrel for recurrent idiopathic stroke. The cardiac biomarkers were elevated with a troponin peaking at 2.04 g/L and creatine kinase (CK) peaking at 1557 U/L. Echocardiography showed biventricular dilatation and moderately impaired systolic function of both the systemic (anatomic right) ventricle, and the subpulmonic (anatomic left) ventricle. Coronary angiography showed unobstructed coronaries. Cardiac MRI was performed for better delineation of ventricular, great vessel and baffle anatomy prior to the implantation of a defibrillator. Anatomical images showed widely patent baffles without any obvious stenosis or leakage and a minimal residual aortic coarctation. Functional images revealed moderate to severe systemic (right ventricle) and subpulmonic (left ventricle) dysfunction with a calculated ejection fraction of
Received 31 July 2009; received in revised form 24 February 2011; accepted 7 March 2011 ∗
Corresponding author. Tel.: +61 884042195; fax: +61 882045625. E-mail addresses: [email protected], [email protected] (L. Joerg), [email protected] (C. Bridgman), [email protected] (J.B. Selvanayagam).
31% and 38% respectively. Furthermore, mild to moderate tricuspid valve regurgitation was noticed (Fig. 1). Delayed hyperenhancement technique with intravenous gadolinium-DTPA contrast agent (0.1 mmol/kg) showed patchy mid-wall hyperenhancement of the ventricular septum indicating myocardial fibrosis (Fig. 2).
Discussion The incidence of congenital heart disease is difficult to ascertain but varies from 4/1000 to 50/1000 live births. In transposition of the great arteries, which accounts for 5% of the cases of congenital heart disease, there is ventriculoarterial dissociation with the two main arteries being connected to the “wrong” ventricles. The aorta leaves the right ventricle and takes venous blood to the body whilst the pulmonary artery leaves the left ventricle and transfers oxygenated blood to the lungs. This condition is three times more common amongst males [1,2]. Systemic oxygen saturation falls to very low levels shortly after birth and death would ensue without emergent interventions such as reopening of the ductus arteriosus with Prostaglandin E and increasing shunting at the atrial level by balloon atrial septostomy “Rashkind procedure” to improve oxygenation. In 50% of the cases, D-type Transposition of the great arteries is associated with other defects. These include ventricular septal defects, aortic coarctation, coronary artery abnormalities, and left ventricular outflow tract obstruction [1,2]. Patients with this anomaly are not expected to survive without a more definitive surgical procedure. The atrial switch procedures by Senning (1958) and Mustard (1964) created baffles within the heart
© 2010 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved.
1443-9506/04/$36.00 doi:10.1016/j.hlc.2011.03.001
Joerg et al. Cardio-vascular anatomy and function
667
Fig. 1. Panel (A) is a sagittal ‘white blood’ image of the heart illustrating typical D-Transposition anatomy. The aorta (Ao) leaves the subaortic right ventricle (RV) whilst the pulmonary artery (PA) leaves the subpulmonic left ventricle (LV). Panels (B) and (C) show the patent baffles which direct the blood flow from one atrium to the other after the Mustard repair (arrows).
Fig. 2. Panels (A) (horizontal long axis) and (B) (basal short axis) demonstrate DE-CMR images illustrating patchy mid-wall hyperenhancement of the ventricular septum indicating fibrosis (arrows).
which directed blood flow from one atrium to the opposite semilunar valve and ventricle effectively correcting the abnormal flow of blood. This technique improved longterm survival up to 80% at 28 years [2,3], but also resulted in important sequelea such as reduced exercise capacity, increased risk of brady/tachy-arrythmias, and sudden cardiac death as well as right heart failure due to overload of the systemic ventricle [2–4]. Other complications include scarring and obstruction of the baffles, baffle leaks or multifactorial pulmonary hypertension [2,3]. Today the surgical repair of choice of TGAD, is the Jatene arterial switch operation (1976), usually performed in the first few weeks of life. This involves the transection and reattaching of the great arteries above the semilunar valves, as well as the reimplantation of the coronary arteries into the new aortic root. In most of the cases normal cardiac function and physiology can be achieved as cardiac anatomy is restored. Therefore this approach is superior to the atrial switch operations and the prognosis for arterial switch patients is very good. A follow-up study of 1095 patients showed freedom from intervention in 82% with no further deaths between 10 and 15 years after surgery (88% survival). Arrhythmias and heart block are rare. However,
it should be borne in mind that there is limited long-term follow up data. Some degree of dysfunction in the coronary arteries, left ventricle, pulmonary valve, and/or the aortic valve is somewhat more common, occurring in 5–25% of patients [5].
Treatment and Management Sudden death is the most common cause of late mortality in this population. Our patient who survived sudden cardiac arrest clearly qualified for implantation of an implantable defibrillator (ICD). The data provided by CMR imaging especially the exact delineation of the vessel- and baffle anatomy was of major assistance in the planning of the ICD implantation. The patient recovered well and was subsequently discharged on standard heart failure therapy (ACEI, betablocker) with follow up in the adult congenital heart disease clinic.
Conclusion Echocardiography cannot always provide the necessary information for the evaluation and treatment of patients
CLINICAL SPOTLIGHT
Heart, Lung and Circulation 2011;20:666–668
CLINICAL SPOTLIGHT
668
Joerg et al. Cardio-vascular anatomy and function
with a congenital heart disease. Cardiac MRI with its excellent delineation of cardiac and baffle anatomy was crucial in facilitating management and enabling safer cardioverter defibrillator insertion. Delayed enhancement technique with gadolinium showed patchy mid-wall hyperenhancement of the ventricular septum indicating fibrosis. This area may well have been the location where this patient’s ventricular arrhythmia originated. In addition, functional images allowed an accurate assessment of the myocardial systolic function of both the subpulmonic (left ventricle) and especially the systemic right ventricle. Follow-up of the RV systolic function is of major importance in this patient population because many may ultimately require transplantation due to eventual cardiac failure. CMR is an outstanding imaging tool to evaluate patients with a complex cardiac anatomy and will be increasingly used in the future as the numbers of adults survivors with congenital heart diseases increase due advances in surgical techniques and medical care.
Competing Interests The author(s) declare that they have no competing interests.
Heart, Lung and Circulation 2011;20:666–668
Acknowledgements There was no source of funding used for this study.
Appendix A. Supplementary data Supplementary data associated with this article can be found, in the online version, at doi:10.1016/ j.hlc.2011.03.001.
References [1] Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002;39(June (12)):1890–900. Review. [2] Sommer RJ, Hijazi ZM, Rhodes Jr JF. Pathophysiology of congenital heart disease in the adult. Part I Shunt lesions. Circulation 2008;117(February (8)):1090–9. [3] Warnes CA. Transposition of the great arteries. Circulation 2006;114(December (24)):2699–709. Review. [4] Michael KA, Veldtman GR, Paisey JR, Yue AM, Robinson S, Allen S, et al. Cardiac defibrillation therapy for at risk patients with systemic RV dysfunction secondary to atrial redirection surgery for dextro-transposition of the great arteries. Europace 2007;9(May (5)):281–4. [5] Losay J, Touchot A, Serraf A, Litvinova A, Lambert V, Piot JD, et al. Late outcome after arterial switch operation for transposition of the great arteries. Circulation 2001;104(September (12 Suppl. 1)):I121–6.