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Giant Left Atrial Myxoma: An Unusual Cause of Acute Pulmonary Edema
Giant Left Atrial Myxoma: An Unusual Cause of Acute Pulmonary Edema Rodolfo Citro, MD, FANMCO, FESC, Paolo Masiello, MD, Eduardo Bossone, MD, PhD, FESC, FASC, FCCP, Gennaro Provenza, MD, Generoso Mastrogiovanni, MD, Carlo Baldi, MD, Giovanni Gregorio, MD, and Giuseppe Di Benedetto, MD, Vallo della Lucania and Salerno, Italy
We report a case of a huge left atrial myxoma with an unusual clinical presentation characterized by acute pulmonary edema. The possible pathophysiologic mechanism has been discussed. Keywords: Echocardiography, Cardiac tumor
CASE REPORT A 54-year-old woman with long-standing history of systemic arterial hypertension was admitted to our emergency department with a clinical picture of acute pulmonary edema. Arterial systemic blood pressure and heart rate were, respectively, 150/80 mm Hg and 120/min. On auscultation an accentuated first heart sound with a diastolic apical rumble were heard and rales were present on the midbasal pulmonary fields bilaterally. Transcutaneous oxygen saturation was 84%. Electrocardiography demonstrated sinus tachycardia with signs of left ventricular (LV) hypertrophy. After an initial treatment with digitalis, diuretics, morphine, and oxygen, the patient was transferred to the coronary care department, where a 2-dimensional transthoracic echocardiographic (TTE) color Doppler examination revealed a huge, ovoid, homogenously hyperechoic, pedunculated mass adherent to the caudal portion of the left side of interatrial septum, compatible with the diagnosis of myxoma (Figures 1 and 2; Movie). The mass was found prolapsing into the LV. During diastole it occluded the mitral valve orifice almost completely. Mild LV hypertrophy with normal global and regional systolic function was documented. LV filling flow was characterized by only a small diastolic turbulent jet. Pulmonary veins were not obstructed. Pulmonary artery systolic pressure was elevated. Mild posterior pericardial effusion was detected. It is worthy of mention that a TTE performed 20 months before showed no remarkable findings except for mild LV hypertrophy and mild dilatation of the left atrium (LA). The patient underwent preoperative coronary angiography, which revealed normal coronary artery, and was immediately transferred to the surgical department for excision of the LA mass. The operation was performed median opening of the sternum under cardiopulmonary bypass, using mild hypothermia (33°C) and hyperkalemic warm blood cardioplegia. Transeptal approach to the LA allowed resection of a 7- ⫻ 3-cm mass. Histologic analysis of the lesion
From the U.O. UTIC-Cardiologia, San Luca Hospital, Vallo della Lucania, Italy. Reprint requests: Rodolfo Citro, MD, U.O. UTIC-Cardiologia, San Luca Hospital, Via F., Cammarota Vallo della Lucania (SA), Italy (E-mail: [email protected]). 0894-7317/$34.00 Copyright by the American Society of Echocardiography. doi:10.1016/j.echo.2007.10.016
demonstrated typical microscopic features of an atrial myxoma (Figure 3). The postoperative recovery was uncomplicated and the patient was discharged home on the seventh postoperative day. Predischarge TTE showed no residual mass in the LA, complete resolution of the pulmonary hypertension (pulmonary artery systolic pressure ⫽ 25 mm Hg), and only a mild pericardial effusion. At 6-month follow-up the patient remained asymptomatic and TTE result was unchanged. DISCUSSION Primary cardiac tumors may either be benign (approximately 75%) or malignant.1 Myxomas are the most common type of benign cardiac primary tumors occurring predominantly in women between the third and sixth decades of life.2 They are usually located in the LA (80%-90%) and frequently attached to the interatrial septum.2 Although benign, they may cause cardiac obstruction, systemic emboli, and life-threatening complications such as heart failure, syncope, or sudden death.2 Furthermore, they may also trigger, depending on the size and site, secondary pulmonary hypertension. In our case the size of the myxoma was higher than the range (2-6 mm) generally reported in the literature.3 The giant mass obstructed the LV filling, mimicking the pathophysiologic mechanism of the “mitral stenosis.” On these grounds, the unusual clinical presentation might be explained with postcapillary pulmonary hypertension.4 Furthermore, the decreased LV diastolic filling time, because of tachycardia, may have contributed to the onset of acute pulmonary edema. Remarkably, an echocardiogram performed 2 years earlier did not reveal any major abnormalities; this observation implies, in accordance with previous examinations, a growth rate of 0.38 cm/month.5 Thus, the rapid growth rate and the possible recurrence should be kept in mind to plan elective surgery and serial postoperative clinical and imaging follow-up. REFERENCES 1. 2. 3. 4.
Reynen K. Frequency of primary tumors of the heart. Am J Cardiol 1996;77:107. Reynen K. Cardiac myxomas. N Engl J Med 1995;333:1610-7. Hwang JJ, Lien WP, Kuan P, et al. Atypical myxoma. Chest 1991;100:550-1. Moorjani N, Harden S, Wells T, Sang GT. Prolapsing left atrial myxoma causing severe pulmonary hypertension: dynamic echocardiographic and magnetic resonance imaging. Heart 2006;92:1594. 5. Roudaut R, Gosse P, Dallocchio M. Rapid growth of a left atrial myxoma shown by echocardiography. Br Heart J 1987;58:413-6.
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Figure 1 Parasternal long-axis view. Systolic frames (left): top, B-mode (note mild mitral regurgitation) (arrow) and bottom, M-mode showing myxoma in body of left atrium (LA). Diastolic frames (right): top, B-mode and bottom, M-mode demonstrating mobile echoic mass prolapsed into left ventricle (LV). Note mild posterior pericardial effusion (arrow). RV, Right ventricle.
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Figure 2 Apical 4-chamber view. Top: Size (7 ⫻ 3.5 cm) of myxoma can be appreciated as occluding in diastole mitral valve orifice almost completely (left). Myxoma was adherent to limbus of fossa ovalis (arrow) with extension of insertion site of 1.7 cm. Stalk was much smaller than maximum diameter of mass (right). Bottom: Two-dimensional color Doppler (left) and continuous Doppler (right) of significant tricuspid regurgitation (TR). Systolic right ventricular (RV) pressure can be estimated [pulmonary artery systolic pressure ⫽ peak velocity of TR jet (m/s)2 ⫻ 4 ⫹ right atrial pressure (mm Hg) ⫽ 3.942 ⫻ 4 ⫹ 10 ⫽ 72.6 mm Hg]. LV, Left ventricle.
Figure 3 Photomicrograph of atrial myxoma shows myxoid stroma containing single and small groups of spindle cells and small vessels. (Hematoxylin-eosin stain; original magnification: ⫻100.)
We report a case of a huge left atrial myxoma with an unusual clinical presentation characterized by acute pulmonary edema. The possible pathophysiologic mechanism has been discussed. Keywords: Echocardiography, Cardiac tumor
CASE REPORT A 54-year-old woman with long-standing history of systemic arterial hypertension was admitted to our emergency department with a clinical picture of acute pulmonary edema. Arterial systemic blood pressure and heart rate were, respectively, 150/80 mm Hg and 120/min. On auscultation an accentuated first heart sound with a diastolic apical rumble were heard and rales were present on the midbasal pulmonary fields bilaterally. Transcutaneous oxygen saturation was 84%. Electrocardiography demonstrated sinus tachycardia with signs of left ventricular (LV) hypertrophy. After an initial treatment with digitalis, diuretics, morphine, and oxygen, the patient was transferred to the coronary care department, where a 2-dimensional transthoracic echocardiographic (TTE) color Doppler examination revealed a huge, ovoid, homogenously hyperechoic, pedunculated mass adherent to the caudal portion of the left side of interatrial septum, compatible with the diagnosis of myxoma (Figures 1 and 2; Movie). The mass was found prolapsing into the LV. During diastole it occluded the mitral valve orifice almost completely. Mild LV hypertrophy with normal global and regional systolic function was documented. LV filling flow was characterized by only a small diastolic turbulent jet. Pulmonary veins were not obstructed. Pulmonary artery systolic pressure was elevated. Mild posterior pericardial effusion was detected. It is worthy of mention that a TTE performed 20 months before showed no remarkable findings except for mild LV hypertrophy and mild dilatation of the left atrium (LA). The patient underwent preoperative coronary angiography, which revealed normal coronary artery, and was immediately transferred to the surgical department for excision of the LA mass. The operation was performed median opening of the sternum under cardiopulmonary bypass, using mild hypothermia (33°C) and hyperkalemic warm blood cardioplegia. Transeptal approach to the LA allowed resection of a 7- ⫻ 3-cm mass. Histologic analysis of the lesion
From the U.O. UTIC-Cardiologia, San Luca Hospital, Vallo della Lucania, Italy. Reprint requests: Rodolfo Citro, MD, U.O. UTIC-Cardiologia, San Luca Hospital, Via F., Cammarota Vallo della Lucania (SA), Italy (E-mail: [email protected]). 0894-7317/$34.00 Copyright by the American Society of Echocardiography. doi:10.1016/j.echo.2007.10.016
demonstrated typical microscopic features of an atrial myxoma (Figure 3). The postoperative recovery was uncomplicated and the patient was discharged home on the seventh postoperative day. Predischarge TTE showed no residual mass in the LA, complete resolution of the pulmonary hypertension (pulmonary artery systolic pressure ⫽ 25 mm Hg), and only a mild pericardial effusion. At 6-month follow-up the patient remained asymptomatic and TTE result was unchanged. DISCUSSION Primary cardiac tumors may either be benign (approximately 75%) or malignant.1 Myxomas are the most common type of benign cardiac primary tumors occurring predominantly in women between the third and sixth decades of life.2 They are usually located in the LA (80%-90%) and frequently attached to the interatrial septum.2 Although benign, they may cause cardiac obstruction, systemic emboli, and life-threatening complications such as heart failure, syncope, or sudden death.2 Furthermore, they may also trigger, depending on the size and site, secondary pulmonary hypertension. In our case the size of the myxoma was higher than the range (2-6 mm) generally reported in the literature.3 The giant mass obstructed the LV filling, mimicking the pathophysiologic mechanism of the “mitral stenosis.” On these grounds, the unusual clinical presentation might be explained with postcapillary pulmonary hypertension.4 Furthermore, the decreased LV diastolic filling time, because of tachycardia, may have contributed to the onset of acute pulmonary edema. Remarkably, an echocardiogram performed 2 years earlier did not reveal any major abnormalities; this observation implies, in accordance with previous examinations, a growth rate of 0.38 cm/month.5 Thus, the rapid growth rate and the possible recurrence should be kept in mind to plan elective surgery and serial postoperative clinical and imaging follow-up. REFERENCES 1. 2. 3. 4.
Reynen K. Frequency of primary tumors of the heart. Am J Cardiol 1996;77:107. Reynen K. Cardiac myxomas. N Engl J Med 1995;333:1610-7. Hwang JJ, Lien WP, Kuan P, et al. Atypical myxoma. Chest 1991;100:550-1. Moorjani N, Harden S, Wells T, Sang GT. Prolapsing left atrial myxoma causing severe pulmonary hypertension: dynamic echocardiographic and magnetic resonance imaging. Heart 2006;92:1594. 5. Roudaut R, Gosse P, Dallocchio M. Rapid growth of a left atrial myxoma shown by echocardiography. Br Heart J 1987;58:413-6.
978.e1
978.e2
Citro et al
Journal of the American Society of Echocardiography
Figure 1 Parasternal long-axis view. Systolic frames (left): top, B-mode (note mild mitral regurgitation) (arrow) and bottom, M-mode showing myxoma in body of left atrium (LA). Diastolic frames (right): top, B-mode and bottom, M-mode demonstrating mobile echoic mass prolapsed into left ventricle (LV). Note mild posterior pericardial effusion (arrow). RV, Right ventricle.
Journal of the American Society of Echocardiography Volume Number
Citro et al
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Figure 2 Apical 4-chamber view. Top: Size (7 ⫻ 3.5 cm) of myxoma can be appreciated as occluding in diastole mitral valve orifice almost completely (left). Myxoma was adherent to limbus of fossa ovalis (arrow) with extension of insertion site of 1.7 cm. Stalk was much smaller than maximum diameter of mass (right). Bottom: Two-dimensional color Doppler (left) and continuous Doppler (right) of significant tricuspid regurgitation (TR). Systolic right ventricular (RV) pressure can be estimated [pulmonary artery systolic pressure ⫽ peak velocity of TR jet (m/s)2 ⫻ 4 ⫹ right atrial pressure (mm Hg) ⫽ 3.942 ⫻ 4 ⫹ 10 ⫽ 72.6 mm Hg]. LV, Left ventricle.
Figure 3 Photomicrograph of atrial myxoma shows myxoid stroma containing single and small groups of spindle cells and small vessels. (Hematoxylin-eosin stain; original magnification: ⫻100.)