Concealed Neo-Aortic Incompetence and New Right Ventricular Outflow Tract Obstruction After Arterial Switch Operation in a Late Presenter

Concealed Neo-Aortic Incompetence and New Right Ventricular Outflow Tract Obstruction After Arterial Switch Operation in a Late Presenter

Case Reports 9. Delaney SG, Doyle TCA, Burton RW, et al. Pulmonary artery sarcoma mimicking pulmonary embolism. Chest 1993;103:1631–3. 10. Akomea AC,...

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Case Reports

9. Delaney SG, Doyle TCA, Burton RW, et al. Pulmonary artery sarcoma mimicking pulmonary embolism. Chest 1993;103:1631–3. 10. Akomea AC, Dussek JE, Anderson DR, et al. Pulmonary artery sarcoma mimicking Pulmonary embolism: successful surgical intervention. Ann Thorac Surg 1996;61:1536–8. 11. Altman NH, Shelley WM. Primary intimal sarcoma of the pulmonary artery. Johns Hopkins Med J 1973;133:214–22.

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12. Wackers FJ, Van Der Schoot JB, Hampe JF. Sarcoma of the pulmonary trunk associated with hemorrhagic tendency. Cancer 1969;23:339–51. 13. Hopwood D, McNeil G. Spindle cell sarcoma of the pulmonary trunk: a case report with histochemistry and electron microscopy. J Pathol 1979;128:71–7. 14. Jose SL, Fedullo PF, Franco P, et al. Digital clubbing associated with pulmonary artery sarcoma. Chest 1996;109:1651–3.

Concealed Neo-Aortic Incompetence and New Right Ventricular Outflow Tract Obstruction After Arterial Switch Operation in a Late Presenter Sameh I. Sersar, MD ∗ , Ghassan M. Baslaim, MD, Division of Cardiothoracic Surgery, King Faisal Specialist Hospital & Research Centre, Jeddah 21499, Saudi Arabia.

We report a 9 year old boy who presented late with cyanosis as a case of dextro-transposition of great arteries, intact ventricular septum and left ventricular outflow tract obstruction (D-TGA/IVS/LVOTO). Arterial switch operation (ASO) with resection of sub-neo aortic membrane and repair of mitral valve were done for the naturally trained LV. On the second postoperative day, the newly discovered right ventricular outflow tract obstruction (RVOTO) was relieved and mitral valve replacement (MVR) was done for significant mixed stenotic/regurgitant mitral valve disease, and intraoperative extra-corporeal membrane oxygenation (ECMO) support was instituted for pulmonary dysfunction for 4 days. Failure of extubation warranted further assessment that revealed significant aortic incompetence (AI) during cardiac catheterisation study, which was underestimated by echocardiography. Aortic valve replacement (AVR) was done on the 11th postoperative day and he was then extubated and had uneventful hospital course in spite of two emergency procedures: drainage of sub-dural haematoma and appendectomy. (Heart, Lung and Circulation 2008;17:417–436) © 2007 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved. Keywords. Neo-aortic incompetence; Late presenter

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espite the fact that potential postoperative problems of the arterial switch operation (ASO) still remain mostly unknown for considerably late presenters with dextro-transposition of great arteries, intact ventricular septum and left ventricular outflow tract obstruction (DTGA/IVS/LVOTO), this operation remains the procedure of choice for patients with transposition. This operation can be performed in certain subsets of patients with DTGA/IVS/LVOTO with early and late survival similar to that of matched patients with normal pulmonary valve and LVOT.1 The development of right ventricular outflow tract obstruction (RVOTO) and neo-aortic incompetence (AI) Received 24 February 2007; received in revised form 6 August 2007; accepted 8 August 2007; available online 4 October 2007 ∗ Corresponding author at: Division of Cardiothoracic Surgery, King Faisal Specialist Hospital & Research Centre, MBC-J 16, PO Box 40047, Jeddah 21499, Saudi Arabia. Tel.: +966 26 677 777; fax: +966 26 639 581. E-mail address: [email protected] (S.I. Sersar).

after ASO are known potential problems during intermediate-to-late follow-up.2,3 However, immediate postoperative RVOTO and neo-AI requiring intervention are rare. Hereby, we describe the challenge of diagnosis and treatment of these two complications in a late presenter for ASO.

Case Report The patient is a 9-year-old boy referred as a late presenter with chronic cyanosis secondary to D-TGA/IVS/LVOTO. Trans-thoracic echocardiography (TTE) showed a subpulmonic membrane with a gradient of 70 mmHg across the LVOT, large atrial septal defect (ASD), mild pulmonary incompetence, MV regurgitation and well-balanced ventricles with preserved function (Fig. 1a). The patient underwent ASO, Lecompte manoeuvre for antero-posteriorly related great arteries with the main pulmonary artery slightly larger than the aorta, buttonhole transfer technique for the usual coronary pattern, mitral valve repair for a significantly thickened and dys-

© 2007 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved.

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Heart, Lung and Circulation 2008;17:417–436

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Case Reports

CASE REPORTS

Heart, Lung and Circulation 2008;17:417–436

Fig. 1. (a) Initial TTE showing the widely patent right ventricular outflow tract, mitral regurgitation and LVOTO. (b) TTE post-ASO showing the turbulence in the RVOTO. (c) Angiography illustrating the reversal of the contrast from the descending aorta during diastole (note the open position of the mitral prosthesis).

plastic valve and a fenestrated patch repair of the ASD. LA or PA pressure monitoring lines are not routine in our centre. Intraoperative TEE revealed RVOTO with a peak gradient of 30 mmHg, but it was decided unnecessary to deal with within the same setting. On the second postoperative day, just prior to extubation, the patient manifested signs of severe pulmonary oedema with hypotension. Urgent TTE revealed a moderate-tosevere MV stenosis and regurgitation, mild-to-moderate neo-AI and RVOTO with a gradient of 60 mmHg (Fig. 1b). Emergency reoperation was performed for the RVOTO relief by infundibular muscle resection, and mechanical MV replacement was done. Mitral valve pathology was partly related to the repair but mainly to its dysplasia. Although, the intraoperative TEE showed wellfunctioning MV prosthesis, mild AI and reduced gradient oxygenation and ventilation were borderline and required the institution of trans-thoracic veno-arterial extracorporeal membrane oxygenation (VA-ECMO) which was maintained for 4 days. The repeat TTE study was unremarkable except for an underestimated moderate neo-AI, thought to be secondary to the increased after-load exerted by the VA-ECMO. However, the patient was managed to be extubated 3 days after the discontinuation of VA-ECMO and needed to be reintubated 4 h later due to significant pulmonary oedema but with a stable haemodynamics (blood pressure of 90/50 mmHg). A repeat TTE showed a well-functioning MV prosthesis, good biventricular function, significantly moderate neoAI, but with an element of reversal flow in the thoracic aorta. An urgent cardiac catheterisation was done and documented a left ventricular end-diastolic pressure (LVEDP) of 19 mmHg, absence of any aorto-pulmonary collateral and a significant reversal flow of the injected contrast from the descending aorta back into ascending aorta during diastole (Fig. 1c). The patient was taken to the operating room for a third procedure where his aortic valve (AV) was replaced with a mechanical prosthesis. Intraoperatively, no evidence of trauma to the aortic valve was noted and the healthy looking trileaflet dilated and non-coapting

valve had a larger pro-lapsing posterior cusp. Repair of the valve was not considered for the fear of complex revision and the patient already had the mechanical MV replacement. He was extubated 2 days later and had a smooth recovery. During hospitalisation, the patient underwent two uneventful emergency procedures. He developed a symptomatic right-sided sub-dural haematoma that resolved after maintaining a drain for 48 h. Also, he was subjected to an appendectomy for acute appendicitis. He recovered well and his follow-up TTE showed good ventricular function, well-functioning mitral and aortic valve prostheses with no further increase in the RVOTO gradient.

Discussion Arterial switch operation with resection of LVOTO is considered as a better option in technically suitable patients with D-TGA/IVS/LVOTO for the advantage of restoring the concordant ventriculo-arterial connection.1 Resection of LVOTO in D-TGA usually restores a normal subvalvular calibre. The pulmonary valve in D-TGA hearts may have unequal cusps sizes, leading to eccentric closure which may have impact on postoperative valve function, and may contribute to neo-AI when the valve functions at systemic pressure. Immediate postoperative RVOTO has been reported in a patient who underwent ASO and had previously undergone pulmonary artery banding for a D-TGA and sub-pulmonic ventricular septal defect.4 The dynamic obstruction which did not require reoperation, was related to the conal septum malalignment aggravated by the longstanding pulmonary artery banding, and heightened by improvement in the contractility with the use of epinephrine. In our case, there was no conal septum malalignment but the newly developed RVOTO is believed to be due to the decreased right ventricular afterload and the use of inotrope. The first reoperation was mainly for the MV replacement and hence infundibular muscle resection was done.

Neo-AI continues to develop over time after ASO and may progress to significant valvular dysfunction later in life. Risk factors for developing neo-AI include prior pulmonary artery banding, the presence of ventricular septal defect, Taussig–Bing anomaly and the technique of coronary artery reimplantation.3,5 However, in this case, none of these risk factors were present. Moreover, there was no evidence of operative damage to the valve due to LVOTO resection or MV replacement. Our explanation for the underlying mechanism for this neo-AI is multi-factorial; the presence of the membrane in the LVOT preoperatively may have led to underestimate the valvular incompetence by echocardiography, the high LVEDP may have masked the severity of neoAI in the echocardiographic studies and precluded the appreciation of a wide pulse pressure, possible loss of sub-valvular support due to membrane resection and exposure of the neo-aortic valve to systemic pressure. The severity of the neo-AI was largely appreciated by the reversal flow of the contrast in the thoracic aorta during diastole.

Conclusion ASO can be performed in selected considerably late presenters with D-TGA/IVS/LVOTO but with a greater risk of reoperation. In order to avoid reoperation, infundibu-

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lar muscle resection for the relief of potential RVOTO should be entertained at the time of ASO. Furthermore, the presence of high LVEDP and resection of the membrane from the LVOT warrant a close serial surveillance of the neo-aortic valve to monitor those with the potential of developing significant neo-AI during the immediate postoperative period.

References 1. Sohn YS, Brizard CPR, Cochrane AD, Wilkinson JL, Mas C, Karl TR. Arterial switch in hearts with left ventricular outflow and pulmonary valve abnormalities. Ann Thorac Surg 1998;66:842–8. 2. Gandhi SK, Pigula FA, Siewers RD. Successful late reintervention after the arterial switch procedure. Ann Thorac Surg 2002;73:88–95. 3. Formigari R, Toscano A, Giardini A, Gargiulo G, Di Donato R, Picchio F, Luciano L. Prevalence and predictors of neo-aortic regurgitation after arterial switch operation for transposition of the great arteries. J Thorac Cardiovasc Surg 2003;126:1753–9. 4. Alexi-Meskishvili V, Uhlemann F, Berger F, Lange PE, Hetzer R. Development of subneopulmonary obstruction early after arterial switch operation in an adult. Ann Thorac Surg 1996;61:1518–20. 5. McMahon CJ, Ravekes WJ, O’Brian Smith E, Denfield SW, Pignatelli RH, Altman CA, Ayres NA. Risk factors for neo-aortic root enlargement and aortic regurgitation following arterial switch operation. Pediatr Cardiol 2004;25:329–35.

Acute Coronary Syndrome in a Patient with a Single Coronary Artery Arising from the Right Sinus of Valsalva: A Case Report Alberto Tripodi, MD, Andrea Dell’Amore, MD ∗ , Tommaso Aquino, MD and Marco Pagliaro, MD Corrado Fedeli, MD Diego Magnano, MD Simone Calvi, MD Claudio Zussa, MD Mauro Lamarra, MD Department of Cardiovascular Surgery, Villa Maria Cecila Hospital, Via Corriera 1, Cotignola, Lugo (RA) 48100, Italy

Coronary anomalies can involve origin or distribution of the artery. Most of these anomalies are not clinically important. A single coronary artery arising from the right coronary sinus of Valsalva is an extremely rare anatomic anomaly. Usually coronary artery malformation is associated with other cardiac malformations and the diagnosis is made after birth. More rarely, coronary artery anomalies are an isolated and asymptomatic cardiac malformation. (Heart, Lung and Circulation 2008;17:417–436) © 2007 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved. Keywords. Atherosclerosis; Anatomy; Coronary artery; Coronary artery bypass grafting; Acute coronary syndrome

Received 16 May 2007; received in revised form 18 July 2007; accepted 25 July 2007; available online 1 October 2007 ∗

Corresponding author. Tel.: +39 0335 6223 366. E-mail address: [email protected] (A. Dell’Amore).

Introduction

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oronary anomalies can involve origin or distribution of the artery. Most of these anomalies are not clinically important. A single coronary artery arising from

© 2007 Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Published by Elsevier Inc. All rights reserved.

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Heart, Lung and Circulation 2008;17:417–436