A Rare Report of Hypoplastic Coronary Arteries and Pulmonary Veins: A Case Report and Review of the Literature.

Congenital coronary artery anomalies are estimated to affect about 1% of the general population. Hypoplastic coronary artery disease is an uncommon subset associated with significant mortality regardless whether another major cardiac anomaly is present. In this case report, we present an extremely rare case of an infant with hypoplastic coronary artery disease and hypoplastic pulmonary veins.

Relationship of Normal Aortic Valve Cusp Dimensions: A Tool to Optimize Cusp Reconstruction Valvuloplasty.

Outcomes of surgical aortic valvuloplasty hinge primarily on optimal leaflet design. Imprecise valve reconstruction can result in insufficiency or stenosis. Predicting postrepair leaflet parameters from pre- or intraoperative readily measurable valve dimensions could result in improved reconstruction.

Prenatal Diagnosis of Uhl Anomaly with Autopsy Correlation.

Uhl anomaly is a rare form of congenital hypoplasia of the right ventricular myocardium. Here, we report, a rare finding in fetal cardiac ultrasound in a 33-year-old woman who presented at 20 weeks' of gestation. A diagnosis of Uhl anomaly was made.

Echocardiographic diagnosis and prognosis of fetal left ventricular noncompaction.

Background: Left ventricular noncompaction (LVNC) has rarely been described in the fetus.

Methods: The presence of associated congenital heart disease and rhythm disturbance was identified and the presence of heart failure was assessed using the cardiovascular profile score in all fetuses with LVNC presenting from January 1999 to July 2010. The left ventricle was divided into 12 segments-four segments each at the base, midpapillary, and apical regions-in the short-axis view to calculate the noncompaction/compaction ratio for each segment.

Successful percutaneous cardiac resynchronization despite an occlusive Thebesian valve.

We report the case of a patient with symptomatic heart failure referred after an unsuccessful attempt at cardiac resynchronization therapy. An occlusive Thebesian valve prevented entry into the coronary sinus ostium. Careful analysis of the patient's cardiovascular physiology and anatomy revealed the "fortuitous" presence of a persistent left superior vena cava.

Three-dimensional transesophageal echocardiography of atrial septal defect: a qualitative and quantitative anatomic study.

Background: Real-time three-dimensional (3D) transesophageal echocardiography (TEE) was used to analyze atrial septal defect (ASD) with 4 goals: (1) to determine feasibility, (2) to analyze the accuracy of qualitative and quantitative data, (3) to assess strengths and weaknesses of the available modes of 3D TEE, and (4) to provide 3D transesophageal echocardiographic reference images.

Methods: Sixty-five patients with ASDs (age, 5-64 years; weight, 20-114 kg; body surface area, 0.8-2.

Right coronary artery occlusion during RF ablation of typical atrial flutter.

Right coronary artery (RCA) occlusion and acute myocardial infarction are rare during radiofrequency (RF) ablation of the cavotricuspid isthmus. Ventricular fibrillation (VF) or cardiac arrest in the periprocedural period may be the initial or only clinical manifestation. Septal or lateral RF delivery may increase the risk.

Extensive left ventricular to coronary artery connections in hypoplastic left heart syndrome.

We demonstrate the echocardiographic features of a rare case of very extensive left ventricular to coronary arterial connections (VCC) to both the right and left coronary arteries in a fetus and subsequent neonate with hypoplastic left heart syndrome (HLH). The right coronary artery, after receiving multiple VCC supplied the only antegrade ascending aortic flow. The left main coronary artery was very hypoplastic and the left anterior descending coronary artery was dilated and tortuous with multiple large VCC.

Cardiomyopathy in multiple Acyl-CoA dehydrogenase deficiency: a clinico-pathological correlation and review of literature.

Multiple acyl-CoA dehydrogenase deficiency (MADD) is a rare autosomal recessive defect of the electron transfer flavoprotein or ubiquinone oxidoreductase, resulting in abnormal fatty acid, amino acid, and choline metabolism, leading to metabolic acidosis, hypoglycemia, "sweaty-feet" odor, and early neonatal deaths. This report presents a child diagnosed with this disease at birth by newborn screening using the mass spectrometer, who died suddenly at the age of 6 months. The echocardiogram revealed pericardial effusion, thickened ventricular musculature, and insufficiency of both the atrio-ventricular valves.

Morphologic study of the ascending aorta and aortic arch in hypoplastic left hearts: surgical implications.

Objectives: The ascending aorta and aortic arch in patients with hypoplasia of the left heart are hypoplastic as a result of diminished blood flow. In this study, the presence and degree of obstruction owing to areas of narrowing or infolding within the diminutive aorta are quantified, and their surgical significance is discussed.

Methods: Ninety-six specimens with hypoplasia of the left heart were studied and measurements were taken at specified sites to evaluate areas of narrowing.

Computer three-dimensional reconstruction of the atrioventricular conduction system.

The human atrioventricular conduction system (AVCS), which includes the AV node and its approaches, AV bundle (penetrating, branching, and bifurcating parts), and the bundle branches, is a curved complex structure that has not been reconstructed in three dimensions using computer technology. Microscopic slides of every 40(th) serial section (cut at 7 micron level) of the AVCS were digitized into 600 dots/inch color images. External outlines of each section were manually segmented using commercially available three-dimensional rendering software (Rhinoceros).

Atrioventricular nodal fast pathway modification: mechanism for lack of ventricular rate slowing in atrial fibrillation.

Objectives: Atrioventricular node (AVN) modification is one of the alternatives for ventricular rate control in patients with drug refractory atrial fibrillation (AF). However, the underlying mechanisms, and in particular the role of the dual pathway electrophysiology is not clear. By using a novel index, His electrogram (HE) alternans, we have previously demonstrated in rabbits that both the slow (SP) and the fast pathways (FP) are involved in AVN conduction during AF.

His electrogram alternans reveal dual atrioventricular nodal pathway conduction during atrial fibrillation: the role of slow-pathway modification.

Background: Traditional tools to study dual-pathway atrioventricular nodal (AVN) electrophysiology are not applicable in subjects with permanent atrial fibrillation (AF). The presence of fast-pathway (FP) and slow-pathway (SP) wavefronts and their possible modification remain uncertain in this condition. We demonstrated previously that His electrogram (HE) alternans can determine whether the FP or the SP reaches the His bundle on a beat-by-beat basis.