ECG IMAGE OF THE MONTH Julia H. Indik, MD, PhD, Section Editor
Putting a Name on It: Ebstein’s Anomaly Chris Healy, MD, Tanyanan Tanawuttiwat, MD, Juan F. Viles-Gonzalez, MD Cardiovascular Division, Department of Medicine, University of Miami Leonard M Miller School of Medicine, Miami, Fla.
PRESENTATION The patient, a 36-year-old man, knew that as a child in Cuba, he had been diagnosed with a noncyanotic congenital heart defect. However, he was unsure of the details, including the type of defect he had. He reported being able to exercise regularly without symptoms, and he had never undergone any cardiac interventions. Upon referral to the cardiology department, he underwent a thorough evaluation.
ASSESSMENT A physical examination revealed a grade III/VI pansystolic murmur at the lower left sternal border. This increased with
inspiration. The remainder of the physical examination was unremarkable. An electrocardiogram (ECG) obtained in the office disclosed sinus rhythm with first-degree atrioventricular block and low voltage (Figure 1). Tall, broad P waves in relation to the amplitude of the QRS complexes and incomplete right bundle branch block were evident throughout the ECG; a multiphasic RSR’ pattern in the terminal QRS complexes was seen in the precordial leads. Echocardiography showed apical displacement of the septal leaflet of the tricuspid valve (Figure 2). Severe right atrial enlargement and normal right ventricular systolic pressure were also noted.
DIAGNOSIS Funding Sources: None. Conflict of Interest: None. Authorship: All authors had a role in writing the manuscript. Requests for reprints should be addressed to Chris Healy, MD, University of Miami, Miller School of Medicine, Clinical Research Building, Room 1107, 1120 NW 14th Street, Miami, FL, 33136. E-mail address: [email protected]
The findings on the ECG coupled with the Grade III/VI pansystolic murmur heard during examination strongly suggested Ebstein’s anomaly. Notching in the right precordial leads is also common in Ebstein’s anomaly, though it was not especially pronounced in our patient’s ECG. When apical displacement of the tricuspid valve’s septal
Figure 1 Among the abnormalities identified on an electrocardiogram were first-degree atrioventricular block, low voltage, and tall, broad P waves.
0002-9343/$ -see front matter Ó 2015 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.amjmed.2014.11.011
368
The American Journal of Medicine, Vol 128, No 4, April 2015 These pathways are thought to result from direct muscular connections that occur in conjunction with the abnormal position of the septal leaflet of the tricuspid valve and associated interruptions between the central fibrous body and septal atrioventricular ring.2 Most accessory pathways are located around the orifice of the malformed tricuspid valve. Catheter ablation has been shown to be safe and effective in these patients. It should be noted, however, that ectopic atrial tachycardia, atrial flutter, atrial fibrillation, and ventricular tachycardia can occur in patients with or without accessory pathways. In patients with Ebstein’s anomaly, atrial fibrillation and atrial flutter are most likely caused by previous surgical changes to the right atrial myocardium or from incisional sustained tachycardia.4
Figure 2 The patient underwent echocardiography. A modified apical 4-chamber view displayed apical displacement of the tricuspid valve’s septal leaflet (white arrow), a finding consistent with Ebstein’s anomaly.
leaflet was seen on echocardiography, the diagnosis was confirmed. First described by Wilhelm Ebstein in 1866, Ebstein’s anomaly is marked by malformations of the tricuspid valve and right ventricle, deformities estimated to occur in approximately 1 out of 200,000 live births.1 While the specifics and severity of the abnormalities differ greatly among patients, certain general traits have been described. The septal and posterior leaflets of the tricuspid valve can be fixed to the underlying myocardium, while the anterior leaflet can be redundant, fenestrated or tethered; the functional tricuspid annulus occupies a lower than normal position in the right ventricle so that part of the chamber is “atrialized” or directly adjoins the right atrium; the atrialized region of the right ventricle is dilated, as is the right atrioventricular junction.2 The abnormalities demonstrated on our patient’s ECG are common among patients with Ebstein’s anomaly. Tall, broad P waves reflect right atrial enlargement. Complete or incomplete right bundle-branch block is seen frequently. First-degree atrioventricular block is identified in almost half of those with the disorder.3 The multiphasic RSR’ pattern in the terminal QRS complex is evidence of infraHisian conduction disturbances and abnormal activation of the atrialized right ventricle.4 Perhaps as many as 30% of patients with Ebstein’s anomaly have an accessory atrioventricular pathway, and it is not unusual for more than 1 accessory pathway to exist.5
MANAGEMENT Surgical intervention for Ebstein’s anomaly typically involves repair or replacement of the tricuspid valve as well as concomitant closure of an atrial septal defect when present. Candidates for surgery have symptoms of deteriorating exercise capacity, cyanosis (oxygen saturation
Putting a Name on It: Ebstein’s Anomaly Chris Healy, MD, Tanyanan Tanawuttiwat, MD, Juan F. Viles-Gonzalez, MD Cardiovascular Division, Department of Medicine, University of Miami Leonard M Miller School of Medicine, Miami, Fla.
PRESENTATION The patient, a 36-year-old man, knew that as a child in Cuba, he had been diagnosed with a noncyanotic congenital heart defect. However, he was unsure of the details, including the type of defect he had. He reported being able to exercise regularly without symptoms, and he had never undergone any cardiac interventions. Upon referral to the cardiology department, he underwent a thorough evaluation.
ASSESSMENT A physical examination revealed a grade III/VI pansystolic murmur at the lower left sternal border. This increased with
inspiration. The remainder of the physical examination was unremarkable. An electrocardiogram (ECG) obtained in the office disclosed sinus rhythm with first-degree atrioventricular block and low voltage (Figure 1). Tall, broad P waves in relation to the amplitude of the QRS complexes and incomplete right bundle branch block were evident throughout the ECG; a multiphasic RSR’ pattern in the terminal QRS complexes was seen in the precordial leads. Echocardiography showed apical displacement of the septal leaflet of the tricuspid valve (Figure 2). Severe right atrial enlargement and normal right ventricular systolic pressure were also noted.
DIAGNOSIS Funding Sources: None. Conflict of Interest: None. Authorship: All authors had a role in writing the manuscript. Requests for reprints should be addressed to Chris Healy, MD, University of Miami, Miller School of Medicine, Clinical Research Building, Room 1107, 1120 NW 14th Street, Miami, FL, 33136. E-mail address: [email protected]
The findings on the ECG coupled with the Grade III/VI pansystolic murmur heard during examination strongly suggested Ebstein’s anomaly. Notching in the right precordial leads is also common in Ebstein’s anomaly, though it was not especially pronounced in our patient’s ECG. When apical displacement of the tricuspid valve’s septal
Figure 1 Among the abnormalities identified on an electrocardiogram were first-degree atrioventricular block, low voltage, and tall, broad P waves.
0002-9343/$ -see front matter Ó 2015 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.amjmed.2014.11.011
368
The American Journal of Medicine, Vol 128, No 4, April 2015 These pathways are thought to result from direct muscular connections that occur in conjunction with the abnormal position of the septal leaflet of the tricuspid valve and associated interruptions between the central fibrous body and septal atrioventricular ring.2 Most accessory pathways are located around the orifice of the malformed tricuspid valve. Catheter ablation has been shown to be safe and effective in these patients. It should be noted, however, that ectopic atrial tachycardia, atrial flutter, atrial fibrillation, and ventricular tachycardia can occur in patients with or without accessory pathways. In patients with Ebstein’s anomaly, atrial fibrillation and atrial flutter are most likely caused by previous surgical changes to the right atrial myocardium or from incisional sustained tachycardia.4
Figure 2 The patient underwent echocardiography. A modified apical 4-chamber view displayed apical displacement of the tricuspid valve’s septal leaflet (white arrow), a finding consistent with Ebstein’s anomaly.
leaflet was seen on echocardiography, the diagnosis was confirmed. First described by Wilhelm Ebstein in 1866, Ebstein’s anomaly is marked by malformations of the tricuspid valve and right ventricle, deformities estimated to occur in approximately 1 out of 200,000 live births.1 While the specifics and severity of the abnormalities differ greatly among patients, certain general traits have been described. The septal and posterior leaflets of the tricuspid valve can be fixed to the underlying myocardium, while the anterior leaflet can be redundant, fenestrated or tethered; the functional tricuspid annulus occupies a lower than normal position in the right ventricle so that part of the chamber is “atrialized” or directly adjoins the right atrium; the atrialized region of the right ventricle is dilated, as is the right atrioventricular junction.2 The abnormalities demonstrated on our patient’s ECG are common among patients with Ebstein’s anomaly. Tall, broad P waves reflect right atrial enlargement. Complete or incomplete right bundle-branch block is seen frequently. First-degree atrioventricular block is identified in almost half of those with the disorder.3 The multiphasic RSR’ pattern in the terminal QRS complex is evidence of infraHisian conduction disturbances and abnormal activation of the atrialized right ventricle.4 Perhaps as many as 30% of patients with Ebstein’s anomaly have an accessory atrioventricular pathway, and it is not unusual for more than 1 accessory pathway to exist.5
MANAGEMENT Surgical intervention for Ebstein’s anomaly typically involves repair or replacement of the tricuspid valve as well as concomitant closure of an atrial septal defect when present. Candidates for surgery have symptoms of deteriorating exercise capacity, cyanosis (oxygen saturation
