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Right Atrial Myxoma and Severe Left Ventricular Dysfunction: Which Explanations? Which Management?
BRIEF COMMUNICATION
Brief Communication
Right Atrial Myxoma and Severe Left Ventricular Dysfunction: Which Explanations? Which Management? Alaae Boutayeb, MD a,∗ , Lahcen Marmade, PhD a , Adil Bensouda, PhD b and Said Moughil, PhD a a b
Department of Cardiovascular Surgery B, Ibn Sina Hospital, Rabat, Morocco Department of Cardiovascular Reanimation, Ibn Sina Hospital, Rabat, Morocco
A 57 year-old woman with a large right atrial myxoma underwent emergency surgical resection in our institute. It is known that surgical management of such tumours is difficult regarding venous cannulation and embolic risk, but in our patient, the surgery was more challenging because of the severe left ventricular dysfunction. Thus, we aim on the one hand, to discuss the association between the left ventricular dysfunction and the right atrial myxoma, and, on the other hand, to describe the on-pump beating heart technique we used to remove this mass. (Heart, Lung and Circulation 2013;22:309–311) © 2012 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier Inc. All rights reserved. Keywords. Right atrial myxoma; Left ventricular dysfunction; Beating heart surgery
Case Report
W
e report the case of 57 year-old woman with a six months history of progressive dyspnoea associated with cough and palpitations. During these months, she was treated symptomatically with no amelioration. At admission, physical exam was normal except a slight tricuspid diastolic murmur. At ECG, the patient was into sinus rhythm with no signs of myocardial ischaemia. TTE revealed a huge right atrial mass measuring 7 cm which prolapsed into the right ventricle during diastole. The mass was pedunculated and the stalk was 6 mm long (Fig. 1). Moreover, severe left ventricular dysfunction (SLVD) (FE: 25%) with regional wall motion abnormalities was observed. Coronary angiography was normal. The intervention was performed through a median sternotomy and on normothermic on-pump beating heart technique. To avoid mass fragmentation, care was taken during venous cannulation and right atrium opening. Thus both the superior and the inferior venae cavae were cannulated as far as possible using a 26 Fr right angled cannula and a 32 Fr straight cannula respectively. Also, a tie was passed around the pulmonary artery in the order to prevent a potential pulmonary embolism.
Received 14 May 2012; received in revised form 1 July 2012; accepted 24 July 2012; available online 24 October 2012 ∗ Corresponding author at: Postal Box 6766, Madinat Al Irfane, Rabat, Morocco. Tel.: +212 661552214; fax: +212 537553002. E-mail address: Alaae [email protected] (A. Boutayeb).
Careful handling allowed a perfect control of the mass and thereafter the side-clamping of both the base as well as the surrounded atrial septum (Fig. 2A). The mass was then completely removed. The clamp was left until closing the atrial septal defect by a double line of running sutures (Fig. 2B). After right atrium closure, the patient was weaned gradually from CPB. No inotropic support was needed. The subsequent course was uneventful. The patient was extubated at the third postoperative hour and discharged at the seventh postoperative day. The pathologic analysis confirmed the diagnosis of myxoma.
Discussion While myxomas are the most common cardiac tumours, the right-sided location accounts for only 20% of cases [1]. Furthermore, the association between right atrial myxoma and left ventricular dysfunction is unusual. Thus, we think that the reported case raises two particular issues. Whereas the former is the association of the right atrial myxoma and the SLVD, the second one involves the optimal surgical management of such condition. It is clear that the association may be purely hazardous if the SLVD is due to an undiagnosed dilated cardiomyopathy. However, a potential relationship is not to be excluded. Chockalingam et al. already reported two cases of left sided myxoma associated with SLVD but with no regional wall motion abnormalities. The authors suggested a cardio-depressant effect of the tumour [2].
© 2012 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier Inc. All rights reserved.
1443-9506/04/$36.00 http://dx.doi.org/10.1016/j.hlc.2012.07.049
BRIEF COMMUNICATION
310
Boutayeb et al. Right Atrial Myxoma and Severe Left Ventricular Dysfunction
Heart, Lung and Circulation 2013;22:309–311
Figure 1. 4-Chamber apical view showing the right atrial myxoma and its prolapse into the right ventricle.
The regional wall motion abnormalities observed in our case, were strongly suggestive of the ischaemic origin, however, coronary angiography was normal. For that reason, other explanations should be hypothesised. Indeed, the myxoma’s prolapse may result in coronary sinus occlusion particularly during the diastole. Since the LV is exclusively perfused during this period, this may explain why only the left ventricular function was impaired. The left ventricular ischaemia can be also related to a coronary emboli. Even if it seems exceptional, paradoxical
embolism through a permeable foramen oval (PFO) is quite possible. This phenomenon can be favoured by tricuspid valve obstruction and the secondary increase in the right atrial pressure. Shunt inversion through PFO was already considered by Guhathakurta and Riordan reporting the case of patient with similar features who presented with cyanosis and hypoxia [3]. The second issue was the surgical management. Due to the SLVD, we preferred to excise the myxoma using on-pump beating heart technique. This strategy allowed better myocardial protection by avoiding cardioplegia and
Figure 2. (A) Careful handling of the right atrial myxoma. (B) Closure of the atrial septum.
Boutayeb et al. Right Atrial Myxoma and Severe Left Ventricular Dysfunction
its ischaemic consequences. Another advantage was the control of the tricuspid valve competence just after the removal of the myxoma. This evaluation was quite impossible preoperatively at TTE. The embolic risks seem to be the major drawbacks of the technique. On the one hand, the removal of both the base of implantation as well as the surrounded atrial septum with unclamped aorta, involves a risk of gaseous embolism. On the other hand, the manipulation of the tumour on beating heart may engender its fragmentation with pulmonary embolism. Nonetheless, our preventive
measures are easily reproducible and seem to heavily minimise these risks.
References [1] Reynen K. Cardiac myxomas. N Engl J Med 1995;333:1610–7. [2] Chockalingam A, Jaganathan V, Gnanavelu G, Dorairajan S, Chockalingam V. Severe left ventricular dysfunction in left atrial myxoma: report of 2 cases. Angiology 2006;57:119–22. [3] Guhathakurta S, Riordan JP. Surgical treatment of right atrial myxoma. Tex Heart Inst J 2000;27:61–3.
BRIEF COMMUNICATION
311
Heart, Lung and Circulation 2013;22:309–311
Brief Communication
Right Atrial Myxoma and Severe Left Ventricular Dysfunction: Which Explanations? Which Management? Alaae Boutayeb, MD a,∗ , Lahcen Marmade, PhD a , Adil Bensouda, PhD b and Said Moughil, PhD a a b
Department of Cardiovascular Surgery B, Ibn Sina Hospital, Rabat, Morocco Department of Cardiovascular Reanimation, Ibn Sina Hospital, Rabat, Morocco
A 57 year-old woman with a large right atrial myxoma underwent emergency surgical resection in our institute. It is known that surgical management of such tumours is difficult regarding venous cannulation and embolic risk, but in our patient, the surgery was more challenging because of the severe left ventricular dysfunction. Thus, we aim on the one hand, to discuss the association between the left ventricular dysfunction and the right atrial myxoma, and, on the other hand, to describe the on-pump beating heart technique we used to remove this mass. (Heart, Lung and Circulation 2013;22:309–311) © 2012 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier Inc. All rights reserved. Keywords. Right atrial myxoma; Left ventricular dysfunction; Beating heart surgery
Case Report
W
e report the case of 57 year-old woman with a six months history of progressive dyspnoea associated with cough and palpitations. During these months, she was treated symptomatically with no amelioration. At admission, physical exam was normal except a slight tricuspid diastolic murmur. At ECG, the patient was into sinus rhythm with no signs of myocardial ischaemia. TTE revealed a huge right atrial mass measuring 7 cm which prolapsed into the right ventricle during diastole. The mass was pedunculated and the stalk was 6 mm long (Fig. 1). Moreover, severe left ventricular dysfunction (SLVD) (FE: 25%) with regional wall motion abnormalities was observed. Coronary angiography was normal. The intervention was performed through a median sternotomy and on normothermic on-pump beating heart technique. To avoid mass fragmentation, care was taken during venous cannulation and right atrium opening. Thus both the superior and the inferior venae cavae were cannulated as far as possible using a 26 Fr right angled cannula and a 32 Fr straight cannula respectively. Also, a tie was passed around the pulmonary artery in the order to prevent a potential pulmonary embolism.
Received 14 May 2012; received in revised form 1 July 2012; accepted 24 July 2012; available online 24 October 2012 ∗ Corresponding author at: Postal Box 6766, Madinat Al Irfane, Rabat, Morocco. Tel.: +212 661552214; fax: +212 537553002. E-mail address: Alaae [email protected] (A. Boutayeb).
Careful handling allowed a perfect control of the mass and thereafter the side-clamping of both the base as well as the surrounded atrial septum (Fig. 2A). The mass was then completely removed. The clamp was left until closing the atrial septal defect by a double line of running sutures (Fig. 2B). After right atrium closure, the patient was weaned gradually from CPB. No inotropic support was needed. The subsequent course was uneventful. The patient was extubated at the third postoperative hour and discharged at the seventh postoperative day. The pathologic analysis confirmed the diagnosis of myxoma.
Discussion While myxomas are the most common cardiac tumours, the right-sided location accounts for only 20% of cases [1]. Furthermore, the association between right atrial myxoma and left ventricular dysfunction is unusual. Thus, we think that the reported case raises two particular issues. Whereas the former is the association of the right atrial myxoma and the SLVD, the second one involves the optimal surgical management of such condition. It is clear that the association may be purely hazardous if the SLVD is due to an undiagnosed dilated cardiomyopathy. However, a potential relationship is not to be excluded. Chockalingam et al. already reported two cases of left sided myxoma associated with SLVD but with no regional wall motion abnormalities. The authors suggested a cardio-depressant effect of the tumour [2].
© 2012 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier Inc. All rights reserved.
1443-9506/04/$36.00 http://dx.doi.org/10.1016/j.hlc.2012.07.049
BRIEF COMMUNICATION
310
Boutayeb et al. Right Atrial Myxoma and Severe Left Ventricular Dysfunction
Heart, Lung and Circulation 2013;22:309–311
Figure 1. 4-Chamber apical view showing the right atrial myxoma and its prolapse into the right ventricle.
The regional wall motion abnormalities observed in our case, were strongly suggestive of the ischaemic origin, however, coronary angiography was normal. For that reason, other explanations should be hypothesised. Indeed, the myxoma’s prolapse may result in coronary sinus occlusion particularly during the diastole. Since the LV is exclusively perfused during this period, this may explain why only the left ventricular function was impaired. The left ventricular ischaemia can be also related to a coronary emboli. Even if it seems exceptional, paradoxical
embolism through a permeable foramen oval (PFO) is quite possible. This phenomenon can be favoured by tricuspid valve obstruction and the secondary increase in the right atrial pressure. Shunt inversion through PFO was already considered by Guhathakurta and Riordan reporting the case of patient with similar features who presented with cyanosis and hypoxia [3]. The second issue was the surgical management. Due to the SLVD, we preferred to excise the myxoma using on-pump beating heart technique. This strategy allowed better myocardial protection by avoiding cardioplegia and
Figure 2. (A) Careful handling of the right atrial myxoma. (B) Closure of the atrial septum.
Boutayeb et al. Right Atrial Myxoma and Severe Left Ventricular Dysfunction
its ischaemic consequences. Another advantage was the control of the tricuspid valve competence just after the removal of the myxoma. This evaluation was quite impossible preoperatively at TTE. The embolic risks seem to be the major drawbacks of the technique. On the one hand, the removal of both the base of implantation as well as the surrounded atrial septum with unclamped aorta, involves a risk of gaseous embolism. On the other hand, the manipulation of the tumour on beating heart may engender its fragmentation with pulmonary embolism. Nonetheless, our preventive
measures are easily reproducible and seem to heavily minimise these risks.
References [1] Reynen K. Cardiac myxomas. N Engl J Med 1995;333:1610–7. [2] Chockalingam A, Jaganathan V, Gnanavelu G, Dorairajan S, Chockalingam V. Severe left ventricular dysfunction in left atrial myxoma: report of 2 cases. Angiology 2006;57:119–22. [3] Guhathakurta S, Riordan JP. Surgical treatment of right atrial myxoma. Tex Heart Inst J 2000;27:61–3.
BRIEF COMMUNICATION
311
Heart, Lung and Circulation 2013;22:309–311