CASE REPORT
Left Ventricular Myxoma Causing Sudden Death Patricia Lee Kitchin, DO, PhD* and Paul Benson, MD† Abstract: Sudden cardiac death due to a myxoma is rarely reported in the literature. Cardiac myxomas are benign tumors of the heart, most frequently located in the left atrium. Left ventricular myxomas are rare. Left ventricular myxomas attached to the anterior papillary muscle are especially rare, with only 1 case being reported in the literature. Myxomas have the potential to cause embolization, obstruction, or arrhythmia. We report a case of a 32-year-old man who sustained sudden death and was found to have a left ventricular myxoma attached to the anterior papillary muscle at autopsy. Although the subject also had evidence of a distant myocardial infarction and dilated cardiomyopathy, the myxoma was apparently clinically silent. Cardiac myxomas may be treated by surgical excision with excellent prognosis. However, they can remain silent and undiagnosed and, hence, have the potential for causing sudden cardiac death. In addition to the case report, we present a literature review of these issues involving cardiac myxomas. Key Words: myxoma, ventricular myxoma, sudden cardiac death (Am J Forensic Med Pathol 2015;36: 58–60)
C
ardiac myxoma is a rarely reported cause of sudden cardiac death. Myxomas are more common in women than in men (3:1) and constitute approximately half of all primary cardiac tumors. Myxomas are most commonly found in the left atrium (75%) and are rarely found in the left ventricle. In this case, we report the sudden cardiac death of a man with no known medical history. This man was found to have a left ventricular (LV) myxoma, cardiomegaly, and a distant myocardial infarction.
CASE REPORT The subject of this case report is a 32-year-old white man with a history of cocaine abuse but with no other known medical history. This subject sustained a witnessed collapse in the shower. The subject's girlfriend called 911 and began cardiopulmonary resuscitation. Emergency personnel arrived to find the subject unresponsive. The paramedics began advanced cardiac life support and transported the subject to the hospital where he was pronounced dead. The deceased was transported to the medical examiner's office where an autopsy was performed. The heart weighed 516 g (the normal weight for men is 300–350 g).1 The coronary arteries arose normally, followed a left dominant course, and showed no signs of atherosclerotic stenosis. The right ventricle showed moderate dilatation. The left ventricle showed mild to moderate dilatation. The myocardium showed patchy areas of tan-white scarring in the posterior wall of the left ventricle, near the septum, extending up to 6 cm in greatest dimension (Fig. 1). In this subject, this area of the heart was perfused by the right coronary artery (RCA), which was diffusely patent. The left Manuscript received May 19, 2014; accepted October 28, 2014. From the *LewisGale Hospital Montgomery, Blacksburg, VA; and †University of Tennessee, Health Science Center, West Tennessee Forensic Center, Memphis, TN. The authors report no conflict of interest. Reprints: Patricia Lee Kitchin, DO, PhD, LewisGale Hospital Montgomery, 1716 Honeysuckle Dr, Blacksburg, VA 24060. E-mail: [email protected]. Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved. ISSN: 0195-7910/15/3602-0058 DOI: 10.1097/PAF.0000000000000141
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ventricle free wall measured 1.5 cm in thickness (normal thickness, 1.3–1.5 cm). The interventricular septum measured 1.5 cm in thickness (normal thickness is 0.957–1.02 cm for men aged 30–39 years).2 The right ventricle free wall measured 0.4 cm in thickness (normal thickness, 0.3–0.5 cm). The pulmonic, tricuspid, and aortic valves were unremarkable and their circumferences were within normal limits and measured 9, 13, and 8 cm, respectively. The mitral valve showed a 3 3 2 cm organized tanpurple-yellow lobulated tumor on the tip of the anterior papillary muscle (Fig. 2). Histologically, specimens of the tumor showed a diffuse background of myxoid ground material with loosely organized spindle cells, with scattered cells showing multiple nuclei and granular material (Fig. 3). Areas of hemorrhage were present with pigment-laden macrophages (Fig. 4). Scattered areas of pseudoglandular formations were present. Specimens of the myocardium at the site of the scarring showed focal myocardial scarring with fibrosis and surrounding myocyte hypertrophy (Fig. 5). Lung specimens showed congestion. Kidney, brain, and liver specimens showed no abnormal histological pathology. Toxicology screen showed a therapeutic amount of hydrocodone (0.06 mg/L) in the blood. The pathological diagnosis was a 3 3 2 cm myxoma of the left ventricle of the heart, a 6-cm remote myocardial infarction of the posterior wall of the left ventricle of the heart, cardiomegaly, and a diffuse cerebral edema. The cause of death was determined to be cardiac arrhythmia due to cardiac myxoma and the manner of death was natural.
DISCUSSION This case presents several interesting and unusual findings. The most notable finding is sudden cardiac death due to an apparently asymptomatic LV myxoma. Another finding is a distant myocardial infarction thought to have occurred 2 months or more before death on the basis of the gross and histological findings involving the myocardium.1 The finding of cardiomegaly, based on increased heart size, is accompanied by dilatation of both ventricles and myocyte hypertrophy. Both the cardiomegaly and the infarction will be discussed in light of how they might be related to or caused by the cardiac myxoma. Myxomas are benign tumors but tend to cause fatal consequences such as emboli, obstruction, and cardiac arrhythmias
FIGURE 1. Gross image showing tan-white myocardial scar in the posterior wall of the left ventricle. Am J Forensic Med Pathol • Volume 36, Number 2, June 2015
Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
Am J Forensic Med Pathol • Volume 36, Number 2, June 2015
FIGURE 2. Gross image of the tumor attached to the tip of the anterior papillary muscle in the left ventricle.
Left Ventricular Myxoma Causing Sudden Death
FIGURE 4. Histopathological image of the tumor showing hemosiderin and scattered mononuclear cell infiltrate (original magnification, 2000).
leading to sudden death. Emboli originating from myxomas have been reported to cause strokes and myocardial infarctions.3,4 Historically, most LV myxomas were found at autopsy. Modern advances in imaging and surgical techniques have allowed detection and removal of these tumors with lower mortality. Grossly, myxomas can have variable shapes such as lobulated, ball-shaped, papillary, or elongated. The cut surface is often hemorrhagic or yellow and gelatinous. Microscopically, myxomas consist of a mucopolysaccharide background interspersed with stellate mesenchymal cells. Glandular formations are rarely present and inflammatory cells are common.5 Primary cardiac tumors are rare. Reynen6 reviewed 22 autopsy series and found the percent of primary cardiac tumors to be approximately 0.02%. McAllister and Fenoglio7 found in a large autopsy series that three quarters of primary cardiac tumors were benign and, of those benign tumors, nearly half were myxomas. These myxomas were located predominantly in the left atrium. Less than 5% of myxomas originate in the ventricles, more commonly in the right ventricle. Left ventricular myxomas typically arise from the posterior papillary muscle, inferior wall, or interventricular septum.8 In an older review of the literature on myxomas, Meller et al9 found that 2.5% reported to be LV. Forty-seven cases of LV myxomas were reported between 1957 and 1997.10 Recent literature since then contains isolated case reports on ventricular myxomas,11 most of these reporting on successful surgical removal of the myxoma. Only 1 case of an LV myxoma attached to the anterior papillary muscle has been reported in the literature.12 Ventricular tumors may cause conduction defects or arrhythmias. Reports of ventricular myxomas with arrhythmias are rare, and only 1 documented case of LV myxoma with arrhythmia is reported in the literature.13 Ventricular myxomas have also been documented to cause LVoutflow tract obstruction.10,14–16 Patients
rarely show symptoms more than 4 years before diagnosis and onset of symptoms is often swift. It has been suggested that this is caused by rapid growth of myxomas. 17,18 There are reports of ventricular myxomas being found incidentally in asymptomatic patients.19,20 In a literature review by Meller et al,9 there were 3 times more female patients than male patients with LV myxomas and the average age at presentation was 24 years old. In the patients with LV myxomas, 40% reported syncope. Only 10% of the patients with LV myxomas reported any constitutional symptoms, but 64% of the patients presented with systemic embolizations, mostly cerebral, which were not correlated to tumor size. Sudden death is defined as “nontraumatic death within a short period after the onset of symptoms (usually
Left Ventricular Myxoma Causing Sudden Death Patricia Lee Kitchin, DO, PhD* and Paul Benson, MD† Abstract: Sudden cardiac death due to a myxoma is rarely reported in the literature. Cardiac myxomas are benign tumors of the heart, most frequently located in the left atrium. Left ventricular myxomas are rare. Left ventricular myxomas attached to the anterior papillary muscle are especially rare, with only 1 case being reported in the literature. Myxomas have the potential to cause embolization, obstruction, or arrhythmia. We report a case of a 32-year-old man who sustained sudden death and was found to have a left ventricular myxoma attached to the anterior papillary muscle at autopsy. Although the subject also had evidence of a distant myocardial infarction and dilated cardiomyopathy, the myxoma was apparently clinically silent. Cardiac myxomas may be treated by surgical excision with excellent prognosis. However, they can remain silent and undiagnosed and, hence, have the potential for causing sudden cardiac death. In addition to the case report, we present a literature review of these issues involving cardiac myxomas. Key Words: myxoma, ventricular myxoma, sudden cardiac death (Am J Forensic Med Pathol 2015;36: 58–60)
C
ardiac myxoma is a rarely reported cause of sudden cardiac death. Myxomas are more common in women than in men (3:1) and constitute approximately half of all primary cardiac tumors. Myxomas are most commonly found in the left atrium (75%) and are rarely found in the left ventricle. In this case, we report the sudden cardiac death of a man with no known medical history. This man was found to have a left ventricular (LV) myxoma, cardiomegaly, and a distant myocardial infarction.
CASE REPORT The subject of this case report is a 32-year-old white man with a history of cocaine abuse but with no other known medical history. This subject sustained a witnessed collapse in the shower. The subject's girlfriend called 911 and began cardiopulmonary resuscitation. Emergency personnel arrived to find the subject unresponsive. The paramedics began advanced cardiac life support and transported the subject to the hospital where he was pronounced dead. The deceased was transported to the medical examiner's office where an autopsy was performed. The heart weighed 516 g (the normal weight for men is 300–350 g).1 The coronary arteries arose normally, followed a left dominant course, and showed no signs of atherosclerotic stenosis. The right ventricle showed moderate dilatation. The left ventricle showed mild to moderate dilatation. The myocardium showed patchy areas of tan-white scarring in the posterior wall of the left ventricle, near the septum, extending up to 6 cm in greatest dimension (Fig. 1). In this subject, this area of the heart was perfused by the right coronary artery (RCA), which was diffusely patent. The left Manuscript received May 19, 2014; accepted October 28, 2014. From the *LewisGale Hospital Montgomery, Blacksburg, VA; and †University of Tennessee, Health Science Center, West Tennessee Forensic Center, Memphis, TN. The authors report no conflict of interest. Reprints: Patricia Lee Kitchin, DO, PhD, LewisGale Hospital Montgomery, 1716 Honeysuckle Dr, Blacksburg, VA 24060. E-mail: [email protected]. Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved. ISSN: 0195-7910/15/3602-0058 DOI: 10.1097/PAF.0000000000000141
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www.amjforensicmedicine.com
ventricle free wall measured 1.5 cm in thickness (normal thickness, 1.3–1.5 cm). The interventricular septum measured 1.5 cm in thickness (normal thickness is 0.957–1.02 cm for men aged 30–39 years).2 The right ventricle free wall measured 0.4 cm in thickness (normal thickness, 0.3–0.5 cm). The pulmonic, tricuspid, and aortic valves were unremarkable and their circumferences were within normal limits and measured 9, 13, and 8 cm, respectively. The mitral valve showed a 3 3 2 cm organized tanpurple-yellow lobulated tumor on the tip of the anterior papillary muscle (Fig. 2). Histologically, specimens of the tumor showed a diffuse background of myxoid ground material with loosely organized spindle cells, with scattered cells showing multiple nuclei and granular material (Fig. 3). Areas of hemorrhage were present with pigment-laden macrophages (Fig. 4). Scattered areas of pseudoglandular formations were present. Specimens of the myocardium at the site of the scarring showed focal myocardial scarring with fibrosis and surrounding myocyte hypertrophy (Fig. 5). Lung specimens showed congestion. Kidney, brain, and liver specimens showed no abnormal histological pathology. Toxicology screen showed a therapeutic amount of hydrocodone (0.06 mg/L) in the blood. The pathological diagnosis was a 3 3 2 cm myxoma of the left ventricle of the heart, a 6-cm remote myocardial infarction of the posterior wall of the left ventricle of the heart, cardiomegaly, and a diffuse cerebral edema. The cause of death was determined to be cardiac arrhythmia due to cardiac myxoma and the manner of death was natural.
DISCUSSION This case presents several interesting and unusual findings. The most notable finding is sudden cardiac death due to an apparently asymptomatic LV myxoma. Another finding is a distant myocardial infarction thought to have occurred 2 months or more before death on the basis of the gross and histological findings involving the myocardium.1 The finding of cardiomegaly, based on increased heart size, is accompanied by dilatation of both ventricles and myocyte hypertrophy. Both the cardiomegaly and the infarction will be discussed in light of how they might be related to or caused by the cardiac myxoma. Myxomas are benign tumors but tend to cause fatal consequences such as emboli, obstruction, and cardiac arrhythmias
FIGURE 1. Gross image showing tan-white myocardial scar in the posterior wall of the left ventricle. Am J Forensic Med Pathol • Volume 36, Number 2, June 2015
Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
Am J Forensic Med Pathol • Volume 36, Number 2, June 2015
FIGURE 2. Gross image of the tumor attached to the tip of the anterior papillary muscle in the left ventricle.
Left Ventricular Myxoma Causing Sudden Death
FIGURE 4. Histopathological image of the tumor showing hemosiderin and scattered mononuclear cell infiltrate (original magnification, 2000).
leading to sudden death. Emboli originating from myxomas have been reported to cause strokes and myocardial infarctions.3,4 Historically, most LV myxomas were found at autopsy. Modern advances in imaging and surgical techniques have allowed detection and removal of these tumors with lower mortality. Grossly, myxomas can have variable shapes such as lobulated, ball-shaped, papillary, or elongated. The cut surface is often hemorrhagic or yellow and gelatinous. Microscopically, myxomas consist of a mucopolysaccharide background interspersed with stellate mesenchymal cells. Glandular formations are rarely present and inflammatory cells are common.5 Primary cardiac tumors are rare. Reynen6 reviewed 22 autopsy series and found the percent of primary cardiac tumors to be approximately 0.02%. McAllister and Fenoglio7 found in a large autopsy series that three quarters of primary cardiac tumors were benign and, of those benign tumors, nearly half were myxomas. These myxomas were located predominantly in the left atrium. Less than 5% of myxomas originate in the ventricles, more commonly in the right ventricle. Left ventricular myxomas typically arise from the posterior papillary muscle, inferior wall, or interventricular septum.8 In an older review of the literature on myxomas, Meller et al9 found that 2.5% reported to be LV. Forty-seven cases of LV myxomas were reported between 1957 and 1997.10 Recent literature since then contains isolated case reports on ventricular myxomas,11 most of these reporting on successful surgical removal of the myxoma. Only 1 case of an LV myxoma attached to the anterior papillary muscle has been reported in the literature.12 Ventricular tumors may cause conduction defects or arrhythmias. Reports of ventricular myxomas with arrhythmias are rare, and only 1 documented case of LV myxoma with arrhythmia is reported in the literature.13 Ventricular myxomas have also been documented to cause LVoutflow tract obstruction.10,14–16 Patients
rarely show symptoms more than 4 years before diagnosis and onset of symptoms is often swift. It has been suggested that this is caused by rapid growth of myxomas. 17,18 There are reports of ventricular myxomas being found incidentally in asymptomatic patients.19,20 In a literature review by Meller et al,9 there were 3 times more female patients than male patients with LV myxomas and the average age at presentation was 24 years old. In the patients with LV myxomas, 40% reported syncope. Only 10% of the patients with LV myxomas reported any constitutional symptoms, but 64% of the patients presented with systemic embolizations, mostly cerebral, which were not correlated to tumor size. Sudden death is defined as “nontraumatic death within a short period after the onset of symptoms (usually
