The 'Cauliflower Heart': a case report of congenital bi-atrial aneurysms causing non-controllable arrhythmia.

BACKGROUND: Congenital aneurysms of the atrium are very rare malformations. Known complications are therapy-resistant arrhythmias. Different treatments such as medical therapy, electrophysiological ablation, and surgery have been proposed. However, there are no guidelines on treatment. CASE SUMMARY: We describe the case of a neonate with bi-atrial aneurysms causing atrial arrhythmia. Arrhythmia was first observed in the 28th week of gestation. Maternal digoxin treatment did not show any effect. After birth, bi-atrial aneurysms were diagnosed and determined as the probable cause of the atrial tachycardia and later of atrial flutter. Antiarrhythmic drug treatment was initiated. However, only frequency control could be achieved. At the age of 7 months, the patient underwent surgical resection. Since surgery, sinus rhythm is present. CONCLUSION: Atrial aneurysms are rare malformations, known complications are atrial arrhythmia. If medical treatment fails, surgery correction appears to be indicated going along with low operative risk and a high probability of successful termination of arrhythmia.

Surgical Management of Congenital Bilateral Multiple Atrial Aneurysms.

Congenital atrial aneurysms are a rare malformation, often associated with supraventricular arrhythmias. Here, we present the case of a child with biatrial aneurysms and a type 2 atrioseptal defect. Directly after birth the girl became symptomatic with incessant ectopic atrial tachyarrhythmia. On echocardiography, multiple biatrial aneurysms and septations were observed. The diagnosis was confirmed with computed tomography. After 7 months of antiarrhythmic therapy, the child underwent surgical intervention by aneurysm resection, atrioseptal defect closure, and ablation. Since then the patient has been in stable sinus rhythm.

Hepatocyte Transfection in Small Pigs After Weaning by Hydrodynamic Intraportal Injection of Naked DNA/Minicircle Vectors.

Liver is an attractive organ for gene delivery in order to correct various genetic (metabolic) diseases. Hydrodynamic vein injection of naked DNA/minicircles devoid of viral or plasmid backbones was demonstrated in, for example, murine phenylketonuria to allow sustained therapeutic transduction of hepatocytes. Here we show successful hepatocyte transfusion in domestic small pigs immediately after weaning upon portal vein catheterization and hydrodynamic injection of naked DNA/minicircle vectors expressing the luciferase gene from the CMV or a liver-specific promoter. First, we established a surgical method allowing hydrodynamic portal vein pressurization up to 120 mmHg and infusion of naked DNA in pigs (n = 5) with long-term survival. No acute adverse effects such as changes in liver transaminases or signs of liver cell damage were observed. We then showed efficiency of stable hepatocyte transfection at 10 and 28 days in single experiments (n = 7) where we found that up to 60% of samples (45/75) were polymerase chain reaction (PCR)-positive for minicircle-DNA. Of these samples, 13% of the positive specimen (6/45) showed low but stable luciferase expression when driven by a liver-specific promoter, as well as appropriate copy numbers per diploid genome. In conclusion, we accomplished a safe procedure for stable transfection of liver cells upon hydrodynamic gene delivery using minicircle vectors in small pigs as a prerequisite to potentially treat infants with genetic liver diseases.