- Email: [email protected]
Anomalous Origin of the Left Main Coronary Artery from the Pulmonary Trunk Masquerading as Peripartum Cardiomyopathy
Anomalous Origin of the Left Main Coronary Artery from the Pulmonary Trunk Masquerading as Peripartum Cardiomyopathy Curt Daniels,
MD,
James Bacon, MD, Mary E. Fontana, MD, Gregory Eaton, Daniel Cohen, MD, and Carl V. Leier, MD
MD,
eripartum cardiomyopathy is a rare cause of congestive P heart failure (CHF) with an estimated incidence between 1:1,300 and 1:15,000 live births.1 – 3 Diagnostic criteria require the clinician to consider other cardiomyopathic processes,2,4 a view supported by the following patient presentation. jjj A 16-year-old gravida one para one white female patient with no previous medical problems was referred for the management of suspected peripartum cardiomyopathy. She described a healthy childhood with no limitations to physical activity. At 15 years of age, she was followed through an uneventful pregnancy. During labor and delivery of her healthy term infant girl, she became acutely dyspneic. Clinical examination and chest radiograph were consistent with CHF. Initial echocardiogram performed on the day of delivery revealed moderate global left ventricular systolic dysfunction and an ejection fraction of 30%. She was treated with diuretics and afterload reduction therapy and discharged after a 1week hospitalization. After discharge, she had dyspnea walking 30 to 40 feet at 0% grade or 1 flight of stairs. She denied chest pain, orthopnea, paroxysmal nocturnal dyspnea, or edema. Followup echocardiogram performed at 4 months postpartum demonstrated persistent global left ventricular systolic dysfunction with an ejecFrom The Division of Cardiology, The Ohio State University Medical Center, Columbus; The Division of Pediatric Cardiology and The Division of Cardiothoracic Surgery, Columbus Children’s Hospital, Columbus, Ohio. Dr. Leier’s address: The Ohio State University Medical Center, Division of Cardiology, 1654 Upham Drive, 669 Means Hall, Columbus, Ohio, 43210. Manuscript received November 4, 1996; revised manuscript received and accepted January 2, 1997.
1307
FIGURE 1. a, anterior-posterior view; b, lateral view of the right coronary artery (RCA) angiogram, demonstrating a dilated tortuous right coronary artery feeding via a collateral network, the left anterior descending (LAD) and left circumflex (LCX) coronary arteries. The LAD and LCX empty as the left main coronary artery into the proximal posterior region of the main pulmonary artery (MPA).
tion fraction of 35% to 40%. At 6 months postpartum, the patient was referred to our institution. Her blood pressure was 128/70 mm Hg. The jugular venous pressure was normal. Her lungs were clear to auscultation. S1 was normal, S2 physiologically split, and an S4 was present. A grade 2/6 systolic ejection murmur and a 2/6 low-
pitched nearly pandiastolic murmur were heard at the lower left sternal border. Liver size was normal. There was no subcutaneous edema. An electrocardiogram disclosed normal sinus rhythm, small Q waves, and voltage criteria for left ventricular hypertrophy with repolarization abnormality. Chest radiograph showed mild cardio-
Q1997 by Excerpta Medica, Inc. All rights reserved.
/ 2w21 0982 Mp
1307
Friday Mar 28 08:53 AM
0002-9149/97/$17.00 PII S0002-9149(97)00110-0
EL–AJC (v. 79, no. 9 ’97)
0982
megaly. The echocardiogram now demonstrated a thickened, mildly dilated, and hypokinetic left ventricle with an ejection fraction of 40%. Color flow Doppler demonstrated multiple diastolic flow disturbances within the ventricular septum consistent with coronary blood flow. The right-sided pressures were normal without evidence of a shunt by oximetry. Left ventricular pressure was 122/10 mm Hg. Left ventricular cineangiography revealed mild hypokinesia, most notably involving the anterolateral wall, with an overall ejection fraction of 45%. Coronary angiography demonstrated a single ostium in the right coronary sinus giving rise to a large tortuous right coronary artery. The right coronary artery fed an extensive collateral network connecting to both the left anterior descending and left circumflex coronary arteries,
1308
which came together as a single vessel (left main coronary artery) emptying into the posterior aspect of the pulmonary trunk just cephalad to the pulmonary valve (Figure 1). Three weeks later, the anomalous left coronary artery was reimplanted to the left coronary sinus and the pulmonary trunk was repaired with an allograft patch. jjj This is the first report of anomalous origin of the left main coronary artery from the pulmonary trunk presenting with CHF during the peripartal period and thus masquerading as peripartum cardiomyopathy. The physical examination, electrocardiogram, chest radiograph, and general echocardiographic findings were all consistent with a primary cardiomyopathic process. The only clue to the correct diagnosis in our case was the low-pitched di-
THE AMERICAN JOURNAL OF CARDIOLOGYT
/ 2w21 0982 Mp
1308
VOL. 79
Friday Mar 28 08:53 AM
astolic murmur. A detailed Doppler echocardiogram was then recorded, demonstrating abnormal diastolic flow within the ventricular septum consistent with dilated coronary collaterals. Coronary arteriography confirmed the correct diagnosis and ultimately led to the appropriate treatment. Thus, anomalous origin of the left main coronary artery from the pulmonary trunk is a treatable lesion that can present during the peripartal period, mimicking peripartum cardiomyopathy. 1. Ribner HS, Silverman RI. Peripartal cardiomyopathy. In: Elkayam U, Gleicher N, eds. Cardiac Problems in Pregnancy: Diagnosis and Management of Maternal and Fetal Disease, 2 ed. New York: Alan R. Liss, 1990:115–127. 2. Lampert MB, Lang RM. Peripartum cardiomyopathy. Am Heart J 1995;130:860–870. 3. Veille JC. Peripartum cardiomyopathies: a review. Am J Obstet Gynecol 1984;148:805–818. 4. Demakis JG, Rahimtoola SH, Sutton GC, Meadows R, Szanto PB, Tobin JR, Gunnar RM. Natural course of peripartum cardiomyopathy. Circulation 1971;44:1053–1061.
MAY 1, 1997
EL–AJC (v. 79, no. 9 ’97)
0982
MD,
James Bacon, MD, Mary E. Fontana, MD, Gregory Eaton, Daniel Cohen, MD, and Carl V. Leier, MD
MD,
eripartum cardiomyopathy is a rare cause of congestive P heart failure (CHF) with an estimated incidence between 1:1,300 and 1:15,000 live births.1 – 3 Diagnostic criteria require the clinician to consider other cardiomyopathic processes,2,4 a view supported by the following patient presentation. jjj A 16-year-old gravida one para one white female patient with no previous medical problems was referred for the management of suspected peripartum cardiomyopathy. She described a healthy childhood with no limitations to physical activity. At 15 years of age, she was followed through an uneventful pregnancy. During labor and delivery of her healthy term infant girl, she became acutely dyspneic. Clinical examination and chest radiograph were consistent with CHF. Initial echocardiogram performed on the day of delivery revealed moderate global left ventricular systolic dysfunction and an ejection fraction of 30%. She was treated with diuretics and afterload reduction therapy and discharged after a 1week hospitalization. After discharge, she had dyspnea walking 30 to 40 feet at 0% grade or 1 flight of stairs. She denied chest pain, orthopnea, paroxysmal nocturnal dyspnea, or edema. Followup echocardiogram performed at 4 months postpartum demonstrated persistent global left ventricular systolic dysfunction with an ejecFrom The Division of Cardiology, The Ohio State University Medical Center, Columbus; The Division of Pediatric Cardiology and The Division of Cardiothoracic Surgery, Columbus Children’s Hospital, Columbus, Ohio. Dr. Leier’s address: The Ohio State University Medical Center, Division of Cardiology, 1654 Upham Drive, 669 Means Hall, Columbus, Ohio, 43210. Manuscript received November 4, 1996; revised manuscript received and accepted January 2, 1997.
1307
FIGURE 1. a, anterior-posterior view; b, lateral view of the right coronary artery (RCA) angiogram, demonstrating a dilated tortuous right coronary artery feeding via a collateral network, the left anterior descending (LAD) and left circumflex (LCX) coronary arteries. The LAD and LCX empty as the left main coronary artery into the proximal posterior region of the main pulmonary artery (MPA).
tion fraction of 35% to 40%. At 6 months postpartum, the patient was referred to our institution. Her blood pressure was 128/70 mm Hg. The jugular venous pressure was normal. Her lungs were clear to auscultation. S1 was normal, S2 physiologically split, and an S4 was present. A grade 2/6 systolic ejection murmur and a 2/6 low-
pitched nearly pandiastolic murmur were heard at the lower left sternal border. Liver size was normal. There was no subcutaneous edema. An electrocardiogram disclosed normal sinus rhythm, small Q waves, and voltage criteria for left ventricular hypertrophy with repolarization abnormality. Chest radiograph showed mild cardio-
Q1997 by Excerpta Medica, Inc. All rights reserved.
/ 2w21 0982 Mp
1307
Friday Mar 28 08:53 AM
0002-9149/97/$17.00 PII S0002-9149(97)00110-0
EL–AJC (v. 79, no. 9 ’97)
0982
megaly. The echocardiogram now demonstrated a thickened, mildly dilated, and hypokinetic left ventricle with an ejection fraction of 40%. Color flow Doppler demonstrated multiple diastolic flow disturbances within the ventricular septum consistent with coronary blood flow. The right-sided pressures were normal without evidence of a shunt by oximetry. Left ventricular pressure was 122/10 mm Hg. Left ventricular cineangiography revealed mild hypokinesia, most notably involving the anterolateral wall, with an overall ejection fraction of 45%. Coronary angiography demonstrated a single ostium in the right coronary sinus giving rise to a large tortuous right coronary artery. The right coronary artery fed an extensive collateral network connecting to both the left anterior descending and left circumflex coronary arteries,
1308
which came together as a single vessel (left main coronary artery) emptying into the posterior aspect of the pulmonary trunk just cephalad to the pulmonary valve (Figure 1). Three weeks later, the anomalous left coronary artery was reimplanted to the left coronary sinus and the pulmonary trunk was repaired with an allograft patch. jjj This is the first report of anomalous origin of the left main coronary artery from the pulmonary trunk presenting with CHF during the peripartal period and thus masquerading as peripartum cardiomyopathy. The physical examination, electrocardiogram, chest radiograph, and general echocardiographic findings were all consistent with a primary cardiomyopathic process. The only clue to the correct diagnosis in our case was the low-pitched di-
THE AMERICAN JOURNAL OF CARDIOLOGYT
/ 2w21 0982 Mp
1308
VOL. 79
Friday Mar 28 08:53 AM
astolic murmur. A detailed Doppler echocardiogram was then recorded, demonstrating abnormal diastolic flow within the ventricular septum consistent with dilated coronary collaterals. Coronary arteriography confirmed the correct diagnosis and ultimately led to the appropriate treatment. Thus, anomalous origin of the left main coronary artery from the pulmonary trunk is a treatable lesion that can present during the peripartal period, mimicking peripartum cardiomyopathy. 1. Ribner HS, Silverman RI. Peripartal cardiomyopathy. In: Elkayam U, Gleicher N, eds. Cardiac Problems in Pregnancy: Diagnosis and Management of Maternal and Fetal Disease, 2 ed. New York: Alan R. Liss, 1990:115–127. 2. Lampert MB, Lang RM. Peripartum cardiomyopathy. Am Heart J 1995;130:860–870. 3. Veille JC. Peripartum cardiomyopathies: a review. Am J Obstet Gynecol 1984;148:805–818. 4. Demakis JG, Rahimtoola SH, Sutton GC, Meadows R, Szanto PB, Tobin JR, Gunnar RM. Natural course of peripartum cardiomyopathy. Circulation 1971;44:1053–1061.
MAY 1, 1997
EL–AJC (v. 79, no. 9 ’97)
0982