Congenital left ventricular aneurysms and diverticula (LVA/Ds) are rare cardiac malformations that can be detected using echocardiography or other imaging techniques. Some of these patients present with ventricular arrhythmias. This study investigated clinical characteristics of patients with congenital LVA/D presenting with arrhythmic manifestations. Over the previous 20 years 250 patients were diagnosed to have congenital LVA/D at our institution. Diagnosis was made using echocardiography after exclusion of coronary artery disease, local cardiac inflammatory processes, traumatic causes, or cardiomyopathies. At initial presentation 32 of the 250 patients (13%, average age 45 years, range 25 to 65, 21 men and 11 women) exhibited arrhythmias. At least 2 LVA/Ds were present in 6 of these patients. LVA/Ds were localized at the posterobasal, apical, anteroseptal, and anterolateral walls in 12, 11, 4, and 5 patients, respectively. The most common complaints at presentation were syncope or presyncope in 18 patients and palpitations in 11 patients. One patient had survived sudden cardiac death. Long-term electrocardiographic recordings showed ventricular tachycardia (VT) or ventricular fibrillation in 17 patients (53%). Twelve patients underwent electrophysiologic testing. Nine patients had inducible ventricular tachyarrhythmia, whereas induced tachycardia was similar to that during spontaneous arrhythmia in 7 patients. In conclusion, patients with congenital LVA/Ds who present with arrhythmic manifestations commonly have VT. Electrophysiologic testing can reproduce clinical VT in most of these patients.
Cardiac arrhythmias are among the clinical manifestations of patients with congenital left ventricular aneurysm and diverticula (LVA/D). Cardiac embolism, wall rupture, infective endocarditis, and congestive heart failure also have been described. In this study we investigated in the setting of a single academic tertiary-care center clinical characteristics of patients with congenital LVA/D who had arrhythmic manifestations at initial presentation.
Methods
All consecutive patients undergoing transthoracic echocardiography from January 1, 1990 through December 31, 2010 at the University Hospital of Zurich were eligible for this study.
Diagnosis of congenital LVA/D was made after exclusion of coronary artery disease, local cardiac inflammatory process, traumatic causes, and cardiomyopathies. Patients with diverticula or aneurysms in chambers other than the left ventricle were excluded from our analysis.
Of patients with congenital LVA/D diagnosed by echocardiography, clinical data of patients with arrhythmic manifestations were analyzed. For this study a patient was considered to have an arrhythmic manifestation if there was electrocardiographic (ECG) documentation of atrial or ventricular ectopic beats or tachyarrhythmias or if there were symptoms suggesting an underlying arrhythmia such as palpitations or presyncope/syncope.
Clinical data were obtained by retrospective review of patient records. Apart from demographic and clinical data, findings from transthoracic echocardiograms, 12-lead surface and 24-hour Holter recordings, and invasive electrophysiologic studies were analyzed.
The protocol of standardized ventricular stimulation consisted of the delivery of up to 3 extra stimuli (with minimum cycle length 180 ms) at 3 different basic drive train cycle lengths (600, 500, and 400 ms) and ventricular burst pacing at 2 right ventricular sites (right ventricular apex and right ventricular outflow tract). Sustained ventricular tachycardia was defined as tachycardia of ventricular origin with duration >30 seconds or ventricular tachycardia leading to hemodynamic compromise.
Results
In total 250 patients were diagnosed with congenital LVA/D of 93,851 patients (0.26%) who underwent echocardiographic examination at our institution. Of these patients 32 (13%) had arrhythmic manifestations at initial presentation and were studied as the study cohort ( Table 1 ). Twenty-one patients were men (66%). The average age of the study cohort was 45 years (range 25 to 65). Two patients had a family history of sudden cardiac death in the absence of previously known heart disease.
| Patient Number | Age (years)/Sex | Clinical Presentation | ECG Abnormality During Sinus Rhythm | Echocardiographic Findings | EPS | Spontaneous Arrhythmias/Inducibility on EPS, if Available |
|---|---|---|---|---|---|---|
| 1 | 65/M | palpitations | none | LVD, apical | 0 | VT (on monitor) |
| 2 | 34/M | palpitations | VES, IVCD | LVD, anterolateral | 0 | VES (LBB pattern, inf axis) |
| 3 | 40/M | syncope | none | LVD, anteroseptal | 0 | 0 |
| 4 | 49/M | syncope | signs of LVH | LVD, apical | 0 | 0 |
| 5 | 39/M | survived SCD | none | LVD, posterobasal | 0 | VF |
| 6 | 76/M | palpitations | IVCD | LVD, apical | 0 | Ns VT |
| 7 | 68/M | syncope | none | LVD, posterobasal | + | monomorphic VT/EPS negative |
| 8 | 34/F | palpitations | VES | LVD, posterobasal | + | Ns polymorphic VT/same VT on EPS |
| 9 | 22/F | palpitations | none | LVD, apical | + | none/AVNRT on EPS |
| 10 | 56/F | syncope | VES | LVD, posterobasal | + | Ns polymorphic VT/same VT on EPS |
| 11 | 20/F | syncope | none | LVD, anteroseptal | 0 | 0 |
| 12 | 30/M | syncope | none | LVD, apical | + | polymorphic VT/same VT on EPS |
| 13 | 78/F | syncope | VES | LVD, anterolateral | + | VES (RBB pattern, sup axis)/EPS negative |
| 14 | 35/M | palpitations | IVCD | LVD, posterobasal | + | VT (RBB pattern, inf axis)/same VT on EPS |
| 15 | 58/M | palpitations | AVBI°, IVCD | LVD, anteroseptal | 0 | VT (RBB pattern, inf axis) |
| 16 | 21/M | syncope | AVBI°, IVCD | LVD, anterolateral | + | VT (RBB pattern, inf axis)/VF induced on EPS |
| 17 | 22/M | syncope | IVCD | LVD, anterolateral | 0 | 0 |
| 18 | 72/M | syncope | none | LVD, posterobasal | + | none/polymorphic VT on EPS |
| 19 | 28/M | syncope | AVBI° | LVD, anteroseptal | 0 | 0 |
| 20 | 19/F | syncope | ST/T change | LVD, posterobasal | + | VT (RBB pattern, sup axis)/same VT on EPS |
| 21 | 48/F | palpitations | none | LVD, apical | 0 | Ns VT |
| 22 | 21/M | syncope | ST/T change | LVD, apical | 0 | 0 |
| 23 | 63/M | chest pain | VES, ST/T change | LVD, apical | 0 | VES on Holter monitor |
| 24 | 62/M | palpitations | VES | LVD, apical | 0 | VES (LBB pattern, inf axis) |
| 25 | 70/M | fatigue, dyspnea | IVCD, ST/T change | LVA, posterobasal | 0 | atrial flutter |
| 26 | 62/M | syncope | LAHB, ST/T change | LVD, posterobasal | + | polymorphic VT/same VT on EPS |
| 27 | 6/F | palpitations | none | LVD, apical | 0 | SVT |
| 28 | 23/F | palpitations | IVCD, VES | LVD, apical | 0 | VES (LBB pattern, inf axis) |
| 29 | 58/M | presyncope | signs of LVH | LVD, posterobasal | 0 | VT (RBB pattern, sup axis) |
| 30 | 49/F | presyncope | AVBI°, RBBB | LVA, posterobasal | 0 | polymorphic VT |
| 31 | 28/M | presyncope | LAHB | LVA, anterolateral | + | VT (RBB pattern, inf axis)/same VT on EPS |
| 32 | 69/F | syncope | IVCD | LVA, posterobasal | 0 | VT (RBB pattern, sup axis) |
The most common complaints at initial presentation were syncopal spells in 18 patients (56%) and palpitations in 11 patients (34%). Other symptoms were fatigue, chest pain, dyspnea, and tachycardia. One patient had survived sudden cardiac death.
Localization of congenital LVA/D was posterobasal in 12 patients and apical in 11 patients ( Figure 1 ) . Anteroseptal or anterolateral wall involvement was observed in 9 patients ( Figure 2 ) . Six patients had >1 LVA/D.
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