Impact of Genotype-Phenotype Interactions on Cardiovascular Function in Paediatric Loeys-Dietz Syndrome.

Background: The relationship between genotype and phenotypical vascular and cardiac properties in paediatric Loeys-Dietz syndrome (LDS) patients are not well characterized. This study explores the phenotypical differences in aortic properties and cardiac structural and functional parameters between paediatric LDS patients with and mutations.

Methods: We included 32 LDS patients with either (n = 17) or (n = 15) mutations.

Unusual Presentation of Congenital Pseudoaneurysm of Mitral Aortic Intervalvular Fibrosa in a Healthy Child.

• PA-MAIVF is a rare condition that is usually asymptomatic. • Symptoms related to PA-MAIVF generally represent complications. • Early identification of this entity is crucial to guide acute management strategies.

Isolated Congenital Right Atrial Aneurysm: Monitoring Parameters for Asymptomatic Patients.

Isolated congenital right atrial aneurysm is rare. Indications for surgery in asymptomatic patients with moderate-size right atria remain controversial. Evidence in support of medical management and timing of prophylactic surgery is reviewed.

Prevalence of Left Ventricular Dyssynchrony in Patients with Congenital Atrioventricular Block and Long-Term Pacing: A Three-Dimensional Echocardiographic Study.

Background: Left ventricular (LV) dysfunction is the major reason for poor outcomes in patients with congenital complete atrioventricular block (CCAVB) and pacemaker. Long-term pacing has been associated with LV mechanical dyssynchrony. However, the relationship of dyssynchrony and LV dysfunction is not clear.

Morphology of the patent ductus arteriosus does not preclude successful patent ductus arteriosus stent implantation in high-risk patients undergoing hybrid stage I palliation: recommendations to optimize ductal stent positioning.

Objective: To describe patent ductus arteriosus (PDA) stenting regardless of ductal morphology in high risk patients with hypoplast physiology undergoing hybrid stage I palliation (PDA stenting and placement of bilateral pulmonary artery bands).

Background: Hybrid palliation is an accepted alternative for patients with hypoplastic left heart syndrome. Patients weighing less than 2.

Right pulmonary artery thrombosis after hybrid stage 1 palliation: resolution after direct tissue plasminogen activator infusion.

A patient with unbalanced right ventricular dominant atrioventricular septal defect with ascending and transverse arch hypoplasia underwent hybrid stage 1 palliation. On postoperative day 7, he experienced irritability and acute cyanosis. Echocardiography demonstrated a thrombus occluding the right pulmonary artery band.

The impact of preexisting myocardial remodeling on ventricular function early after tetralogy of Fallot repair.

Background: Twenty-three patients (median age 23 months) who underwent Fallot's tetralogy repair were investigated prospectively to detect a possible association between histopathologic myocardial remodeling and echocardiographic findings of systolic or diastolic ventricular dysfunction.

Methods: Intraoperatively resected infundibular bands and subendocardial biopsy samples from the right ventricle (RV) and left ventricle were obtained for histopathologic evaluation. Tissue Doppler echocardiographic interrogation of the ventricles was performed before surgery and in the postoperative period.

Biventricular repair in unbalanced atrioventricular septal defect.

We present the case of a 2.5 year-old child with unbalanced atrioventricular septal defect due to a small left ventricle (LV) (mitral annulus of 10mm and a 0.4 ratio in relation to the tricuspid annulus, LVDD: 17 mm, LV Vd2: 15 ml/m(2) and LV/RV long-axis ratio of 0.

Prognostic value of dobutamine stress echocardiography with early injection of atropine with versus without chronic beta-blocker therapy in patients with known or suspected coronary heart disease.

Although a new protocol of dobutamine stress echocardiography with the early injection of atropine (EA-DSE) has been demonstrated to be useful in reducing adverse effects and increasing the number of effective tests and to have similar accuracy for detecting coronary artery disease (CAD) compared with conventional protocols, no data exist regarding its ability to predict long-term events. The aim of this study was to determine the prognostic value of EA-DSE and the effects of the long-term use of beta blockers on it. A retrospective evaluation of 844 patients who underwent EA-DSE for known or suspected CAD was performed; 309 (37%) were receiving beta blockers.

A novel adjustable pulmonary artery banding system for hypoplastic left heart syndrome.

Purpose: We describe the first case in which a neonate with hypoplastic left heart syndrome was initially managed using a mini adjustable banding system.

Description: Through a mid-sternotomy, a 5-day-old neonate underwent bilateral pulmonary artery banding using this new system, combined with placement of a main pulmonary artery to the innominate artery shunt.

Evaluation: The patient had an uneventful postoperative course.

A novel adjustable pulmonary artery banding system for hypoplastic left heart syndrome.

Objective: Hypoplastic left heart syndrome remains a challenge for worldwide surgeons. Initial palliation employing bilateral pulmonary artery banding along with ductal stent implantation and atrial septostomy has been proposed as an alternative approach. However, the surgically placed bands are fixed and may become inadequate after sternum closure or with somatic growth of the patient.

Gender differences in chronotropic and hemodynamic responses during dobutamine-atropine stress echocardiography.

Aim: The aim of this study was to determine the influence of gender on chronotropic and hemodynamic response during dobutamine stress echocardiography with early injection of atropine (EA-DSE).

Methods: From January 2000 to June 2003, we retrospectively evaluated patients who underwent EA-DSE for known or suspected coronary artery disease. We studied 494 patients, 243 men and 251 women, who were not under beta-blocker or calcium channel-blocker therapy (Group A) and 326 patients, 157 men and 169 women, using these medications (Group B).

Evaluation of blood flow reserve in left anterior descending coronary artery territory by quantitative myocardial contrast and Doppler echocardiography.

We sought to compare the feasibility and accuracy of myocardial blood flow reserve (MBFR) measured by quantitative real-time myocardial contrast echocardiography with those of coronary flow velocity reserve (CFVR) obtained by transthoracic Doppler echocardiography for detecting left anterior descending coronary artery (LAD) stenosis. We studied 71 patients who underwent adenosine stress contrast echocardiography, transthoracic Doppler echocardiography, and quantitative coronary angiography within 1 month. An index of myocardial blood flow (A x beta) was determined by quantification of peak plateau acoustic intensity (A) and microbubble replenishment velocity (beta) by contrast echocardiography.

Aneurysm of right atrium diagnosed by 3-dimensional real-time echocardiogram.

We describe a case of giant aneurysm of right atrium in a child with a previous diagnosis of Ebstein's anomaly of tricuspid valve. Three-dimensional real-time echocardiography provided more precise anatomic detail of the tricuspid valve, right atrium, and right ventricle than did its 2-dimensional counterpart.