Congenital left ventricular diverticula are rare cardiac malformations that usually remain asymptomatic. However, life-threatening complications as ventricular arrhythmias, systemic embolism, spontaneous rupture and development of valvular regurgitation, are described. Diagnosis is based on excluding coronary artery disease, traumatic or inflammatory causes, and other underlying cardiomyopathies. Treatment is directed towards the potential complications, yielding mainly therapy of ventricular arrhythmia. Surgical resection is required for larger-sized congenital aneurysms with adverse hemodynamic effects. We present two cases of a left ventricular diverticulum causing cardiac arrhythmia which led to further surgical treatment.

BACKGROUND: The coexistence of congenital left ventricular aneurysm and abnormal cardiac trabeculation with gene mutation has not been reported previously. Here, we report a case of coexisting congenital left ventricular aneurysm and prominent left ventricular trabeculation in a patient with LIM domain binding 3 gene mutation.
METHODS: A 30-year-old Asian man showed paroxysmal sinus tachycardia and Q waves in an electrocardiogram health check. There were no specific findings in physical examinations and serological tests. A coronary-computed tomography angiography check showed normal coronary artery and no coronary stenosis. Both left ventricle contrast echocardiography and cardiac magnetic resonance showed rare patterns of a combination of an apical aneurysm-like out-pouching structure with a wide connection to the left ventricle and prominent left ventricular trabecular meshwork. High-throughput sequencing examinations showed a novel mutation in the LDB3 gene (c.C793>T; p.Arg265Cys).
CONCLUSIONS: Our finding indicates that the phenotypic expression of two heart conditions, congenital left ventricular aneurysm and prominent left ventricular trabeculation, although rare, can occur simultaneously with LDB3 gene mutation. Congenital left ventricular aneurysm and prominent left ventricular trabeculation may share the same genetic background.

The authors present the case of a 34-year-old male patient seen in our department due to palpitations. On the electrocardiogram monomorphic ventricular tachycardia (VT) was documented, treated successfully with amiodarone. The subsequent study revealed a normal echocardiogram and an apical aneurysm of the left ventricle on magnetic resonance imaging, confirmed by computed tomography coronary angiography that also excluded coronary disease. He underwent an electrophysiological study to determine the origin of the VT and to perform catheter ablation using electroanatomical mapping. VT was induced and radiofrequency applications were performed in the left ventricular aneurysm area. VT was no longer inducible, with acute success. Despite this it was decided to implant a subcutaneous implantable cardioverter-defibrillator (ICD). Eight months after the ablation the patient was admitted again due to VT, treated by the ICD.

We report a case of left ventricular aneurysm evaluated using the velocity vector imaging (VVI) technique, an angle-independent method used to calculate the tissue velocity strain and strain rate on routine gray scale two-dimensional images and display the velocities of endocardial points as vectors overlaid on B-mode images. It uses a combination of speckle tracking and complex geometric analysis, allowing tracing of myocardial activity throughout the cardiac cycle. In this case, evaluation of the myocardial dynamics in the fetus by VVI was started at 27 weeks of gestation, and both the systolic and diastolic velocities and the ejection fraction in the aneurysmal segment were less than in the other segments. VVI imaging at 35 weeks of gestation showed this difference even more clearly. Postnatal VVI examination showed no significant difference between results for the systolic and diastolic velocities or ejection fraction and those obtained at the prenatal VVI examination. VVI seemed to be as useful at this time, especially for assessing ventricular systolic function, as it was for postnatal examination.

Described herein is the case of a rare combination of congenital left ventricular (LV) aneurysm and left ventricular non-compaction (LVNC) in a newborn. The patient developed refractory heart failure soon after birth and died at 5 months of age. The etiology of both congenital LV aneurysm and LVNC seems to be maldevelopment of the ventricular myocardium during early fetal life. Treatment should be individually tailored depending on clinical severity, and treatment options are limited. Given that this combination of congenital LV aneurysm and LVNC is significantly associated with poor prognosis, it appears that patients with congenital LV aneurysm and LVNC are candidates for early, aggressive intervention, including surgical aneurysmectomy and evaluation for transplantation. It is important to be aware of this combination of congenital LV aneurysm and LVNC, and to make earlier decisions on therapeutic strategy.

Aortico-left ventricular tunnel (ALVT) is a rare congenital anomaly presenting abnormal connection between the ascending aorta and the left ventricle. In most reported cases, the aortic end of the tunnel is above the right coronary sinus. Cases of ALVT related to the left aortic sinus are extremely rare. We herein report a case diagnosed preoperatively as ALVT arising from the left aortic sinus. The actual diagnosis observed at surgery was aortic valve insufficiency with a left ventricular outflow tract aneurysm. We successfully performed aortic valve repair and plication of the left ventricular aneurysm.