Atrial Flutters in Adults with Congenital Heart Disease.

The macroreentrant atrial tachycardia is very frequent in the adults with congenital heart disease. The impact of the arrhythmias on this type of patients is related to several factors: the anatomy and physiopathology of the specific congenital heart disease (CHD), the sequelae of the corrective surgery or surgical palliation, the presence of residual lesions (shunt, regurgitation), and the age and the clinical status of the patient and the comorbidities. In turn, the mechanism of the MAT depends on the peculiar features of the conduction's system in the CHD and native and acquired (post-surgery) substrates.

Anterior and posterior leaflets augmentation to treat tricuspid valve regurgitation.

In congenital non-Ebstein anomalies of the tricuspid valve, the septal leaflet is often involved and tethered. We describe a standardized approach to address septal leaflet tethering by concomitant augmentation of the anterior and posterior leaflets.

Brachial arteriovenous fistula in patients with cavopulmonary connection and poor ventricular function: a bridge to Fontan operation.

OBJECTIVE: Augmentation of pulmonary blood flow is the only surgical treatment to reduce symptoms of cyanosis in patients with cavopulmonary connection unsuitable for Fontan operation. Brachial arteriovenous fistula is a good option to increase pulmonary blood flow. In this report, we analyze its possible consequences on ventricular function. METHODS: Six patients considered unsuitable for a Fontan completion because of poor ventricular function underwent brachial arteriovenous fistula. The fistula was performed with the interposition of a 4-mm gore-tex conduit between the brachial vein and the brachial artery. RESULTS: The mean oxygen saturation increased from 76.8 ± 1.9% to 84.1 ± 1.4% (p < 0.01); hemoglobin and hematocrit decreased from 21.13 ± 0.7 g/dL to 18.12 ± 0.5 g/dL (p < 0.01) and from 63.3 ± 2.7% to 54.2 ± 0.7% (p < 0.01), respectively. Mean ejection fraction and shortening fraction increased from 38.6 ± 1.5% to 49.5 ± 2.3% (p < 0.01) and from 23.3 ± 1.8% to 28.8 ± 1.3% (p < 0.01), respectively. A Fontan completion and takedown of the fistula was then accomplished, within six years, with no mortality or major complications. At a mean follow-up of 15 months, all six patients are clinically in good condition. CONCLUSION: In patients with a cavopulmonary connection and poor ventricular function, the brachial arteriovenous fistula may play a role in increasing oxygen saturation, optimizing ventricular preload, and reducing blood viscosity and pulmonary arteriovenous malformations. This approach might represent an intermediate step to rescue patients previously considered unsuitable or at high risk for Fontan operation.

Pericardiectomy for pleuropericardial effusion complicating bacterial pneumonia.

Severe pericardial effusion is a rare complication of bacterial pneumonia and it usually disappears under medical treatment. Herein we report a case of a girl with a congenital immunodeficient syndrome and bacterial pneumonia, who developed recurrent and life-threatening pericardial effusion refractory to medical treatment. She was finally treated with pericardiectomy.

The 212A variant of the APJ receptor gene for the endogenous inotrope apelin is associated with slower heart failure progression in idiopathic dilated cardiomyopathy.

BACKGROUND: Idiopathic dilated cardiomyopathy (IDC) has multiple genetic and acquired causes. Apelin is an endogenous peptide that increases cardiac inotropism through his APJ receptor. No data are available concerning the APJ gene mutations responsible for IDC or on the role of APJ receptor gene variants in predicting heart failure (HF) progression. METHODS AND RESULTS: We prospectively evaluated 202 consecutive patients with IDC and 202 matched controls: 90 were screened for APJ gene mutations and all 202 were genotyped for G212A and A445C APJ receptor polymorphisms. No mutations were found within the coding or untranslated regions of the APJ receptor, and no differences in allelic or genotype frequencies were observed comparing patients with a healthy control population. The correlations between APJ receptor polymorphisms and HF progression were assessed. During a median follow-up of 37 months, 35 patients experienced HF progression. Univariate analysis showed that patients carrying at least 1 copy of 212A had a significantly lower risk for HF-related events than those who were homozygous for the G212 variant, and multivariate analysis confirmed that it was significantly related to a more favorable prognosis. CONCLUSIONS: APJ is unlikely to be a gene causing IDC, but the independent correlation between the 212A allele and a better prognosis suggests that it might act as a modifier gene.