Characteristics and outcome of primary congenital left ventricular aneurysm and diverticulum: analysis of cases from the literature

Abstract

Twenty-six fetuses with prenatal diagnosis of LVA (50% male subjects) and 16 with LVD (38% male subjects) were identified. Fetal age at diagnosis was 23.8 ± 6.1 weeks. Mean maternal age was 30.6 ± 6.1 years old. LVA was larger (375 ± 237 vs 60 ± 71 mm(2); p = 0.002), and LVA/LVD was frequently observed in an apical location (62%). Associated cardiac/extracardiac defects were reported in 13/42 (31%). Symptomatic patients presented with arrhythmias (LVA 16% vs LVD 0%), hydrops fetalis (LVA 27% vs LVD 18.8%), or rupture (LVA 4% vs LVD 38%; p = 0.02). Size changes of the LVA/LVD during pregnancy were reported in 17%. Fetal death occurred in nine cases (21.4%); termination of pregnancy was performed in six cases (14.3%). All other babies were delivered at term. During follow-up (mean 29.1 ± 38.2 months), the rate of adverse events was significantly increased in patients with LVA (40 vs 0%; p = 0.04) CONCLUSION: The rate of fetal complications including fetal death is high. Long-term outcome among the survivors is significantly worse in patients with LVA.

Prenatal diagnosis of left ventricular diverticulum and coarctation of the aorta

“…Traditionally, an aneurysm has a broader inlet, shows evidence of fibrosis and is dyskinetic or akinetic. In contrast, a diverticulum has a narrower inlet and histological assessment shows differentiating layers of myocardium, which contract during the cardiac cycle. The precise pathophysiology is unclear but suggested hypotheses include interruption of the development of the embryonic ventricle in the 4 th embryonic week or myocardial injury resulting in the formation of an epicardial hematoma.…”

“…In contrast, a diverticulum has a narrower inlet and histological assessment shows differentiating layers of myocardium, which contract during the cardiac cycle. The precise pathophysiology is unclear but suggested hypotheses include interruption of the development of the embryonic ventricle in the 4 th embryonic week or myocardial injury resulting in the formation of an epicardial hematoma. Exposure of the organized hematoma to the left ventricular pressure may result in the aneurysm/diverticulum.…”

“…Fetal presentation includes an abnormal four‐chamber view of the heart, arrhythmia, isolated pericardial effusion or fetal hydrops with associated heart failure. Ohlow et al identified 42 cases of fetal LVA/LVD. In the majority of cases, lesions showed no enlargement, however progressive dilatation was described in five cases, increasing the risk of fetal hydrops and intrauterine death (IUD).…”

Fetal Right Ventricular Diverticulum Detected by Prenatal Ultrasound Screening

“…As aforementioned, for distinction between ventricular diverticulum and aneurysm, we recommend evaluation of the wall thickness by B-mode ultrasound and the wall contractility by M-mode ultrasound and color Doppler. However, because Ohlow et al [20] reported that 8% of ventricular aneurysms had normal contractility and that 10% of ventricular diverticula were akinetic, clinicians must make an overall judgment based on other criteria. Indeed, in this study, we diagnosed the ventricular diverticulum by reliance on both the thickness and contractility of the abnormal heart chamber, as previously supported.…”

In Utero Evolution of an Apical Left Ventricular Aneurysm

“…1 In utero stability, after the development of severe LV enlargement and dysfunction, is unexpected. Serial images illustrate an apical LV aneurysm, which evolves into a dilated cardiomyopathy indistinguishable from other underlying etiologies.…”