Congenital aneurysm of the left atrium associated with double-outlet right ventricle in a trisomy-18 fetus: Case report and literature review.

Congenital aneurysm of the left atrium is a rare cardiac anomaly, most commonly detected between the 2nd and 4th decades of life in a symptomatic patient. We report a congenital aneurysm of the left atrium diagnosed at 24 weeks of gestational age, associated with other congenital heart diseases and 47XY, +18 karyotype. The literature of the left atrial aneurysm diagnosed by fetal echocardiography is also reviewed in this report.

Maternal hyperoxygenation improves left heart filling in fetuses with atrial septal aneurysm causing impediment to left ventricular inflow.

OBJECTIVES: Aneurysm of the atrial septum (AAS) with excessive excursion of septum primum into the left atrium is an uncommon and relatively benign fetal condition associated with impediment to left ventricular (LV) filling and the appearance of a slender, but apex-forming, LV on fetal echocardiography. Impediment to filling can be severe, creating the image of LV hypoplasia with retrograde aortic flow. We hypothesize that maternal hyperoxygenation alters atrial septal position, improves LV filling, and normalizes aortic flow in fetuses with AAS by increasing fetal pulmonary venous return. METHODS: Fetal echocardiography was performed prior to, and at 10 min of, maternal hyperoxygenation in 12 fetuses with AAS who were referred to our center because of LV hypoplasia. Atrial septal excursion (ASE), LV and right ventricular (RV) sphericity index (SI) and direction of flow in the aortic isthmus, as determined by Doppler, were measured. RESULTS: With maternal hyperoxygenation, mean ± SD ASE decreased (0.76 ± 0.17 before maternal hyperoxygenation vs 0.53 ± 0.23 after maternal hyperoxygenation; P < 0.01), consistent with increased pulmonary venous return, LV-SI increased (0.29 ± 0.06 vs 0.42 ± 0.06; P < 0.001), indicating increased LV filling, and the direction of aortic isthmus flow changed from retrograde in all cases prior to maternal hyperoxygenation to antegrade in 10 and to bidirectional in two. RV-SI remained unchanged (0.53 ± 0.13 vs 0.52 ± 0.10; P = 0.7). CONCLUSIONS: In cases of AAS, short-term maternal hyperoxygenation increases fetal pulmonary venous return, substantially alters LV geometry and promotes antegrade flow in the aortic isthmus. This demonstrates proof-of-concept that maternal hyperoxygenation can improve filling of the left side of the fetal heart in AAS.

Pediatric giant right atrial aneurysm: a case series and review of the literature.

Giant right atrial aneurysm is a rare form of congenital heart disease with a wide spectrum of clinical presentation varying from asymptomatic patients to those with refractory atrial arrhythmias or severe airway obstruction. Diagnosis is often confused with other causes of right atrial dilation such as Ebstein disease. Because of its rare occurrence and variable clinical presentation, inconsistencies in medical and surgical management strategies exist between centers. We present five cases of giant right atrial aneurysm managed at our institution and discuss the clinical presentation, diagnostic challenges, and medical and surgical management.

Prenatal diagnosis of left ventricular aneurysm in association with interruption of the aortic arch.

Left ventricular aneurysms in the foetus are a rare abnormality that can occur in isolation or associated with pentalogy of Cantrell. Here, we report a case of a foetus with a left ventricular aneurysm in association with interruption of the aortic arch, and no features of pentalogy of Cantrell. To our knowledge, this is the first report of such an association.

Fetal diagnosis of left ventricular aneurysm of the free wall and the interventricular septum: report of two cases and review of the literature.

Two cases of aneurysmatic bulgings of the free left ventricular wall and the interventricular septum, respectively diagnosed in late third trimester pregnancies are described. The diagnosis was confirmed by meticulous echocardiographic examination of the fetuses, who were referred for dysrhythmia. Based on sonographic findings and analysis of the clinical outcome, our previous cases emphasise management options both antenatally and postnatally. We further reviewed the current literature and discussed differences between aneurysmatic lesions with respect to their specific localisation to enhance our understanding of these fetal cardiac anomalies.

Congenital left atrial appendage aneurysm diagnosed by fetal echocardiography.

A newborn infant was evaluated because of a large cystic mass adjacent to the left atrium on prenatal ultrasound. The diagnosis was an aneurysm of the left atrial appendage. Most cases of this rare lesion are recognized between the 2nd and 4th decades of life. The present case, however, was detected on fetal echocardiography. The patient showed significant mitral regurgitation caused by the compression of the left atrioventricular groove. Simple aneurysmectomy without mitral valvuloplasty was sufficient to relieve significant mitral regurgitation in this case.

Prenatally diagnosed right ventricular outpouchings: a case series and review of the literature.

Congenital right ventricular aneurysms and right ventricular diverticula are rare forms of congenital heart disease for which little information exists regarding optimal management, natural history, or prognosis. With advancements in prenatal cardiac evaluation and ultrasound, the reported frequency of these lesions, as well as a better understanding of the natural history of this form of congenital heart disease, is likely to evolve. We present three cases of fetal right ventricular aneurysms/diverticula diagnosed at our institution, along with a review of the current literature. We describe the pre- and postnatal courses as well as suggest counseling and management strategies.