Novel contouring method for optimizing MRI flow quantification in patients with aortic valve disease.

Optimizing MRI aortic flow quantification is crucial for accurate assessment of valvular disease severity. In this study, we sought to evaluate the accuracy of a novel method of contouring systolic aortic forward flow in comparison to standard contouring methods at various aortic levels. The study included a cohort of patients with native aortic valve (AoV) disease and a small control group referred to cardiac MRI over a 1-year period.

Human fitting of pediatric and infant continuous-flow total artificial heart: visual and virtual assessment.

Background: This study aimed to determine the fit of two small-sized (pediatric and infant) continuous-flow total artificial heart pumps (CFTAHs) in congenital heart surgery patients.

Methods: This study was approved by Cleveland Clinic Institutional Review Board. Pediatric cardiac surgery patients ( = 40) were evaluated for anatomical and virtual device fitting (3D-printed models of pediatric [P-CFTAH] and infant [I-CFTAH] models).

Infant Cardiac Rhabdomyoma With Incessant Arrhythmia: Role of Tumor Debulking and Margin Cryoablation.

We describe the management of a 4-month-old girl (5.0 kg) who presented with atrial arrhythmias in the setting of cardiac rhabdomyomas. Despite maximal medical therapy and extracorporeal membrane oxygenation support, atrial arrhythmias persisted.

Biventricular Conversion in Unseptatable Hearts: "Ventricular Switch".

Patients with complex systemic and pulmonary venous anatomy, common atrioventricular canal defects and conotruncal anomalies have traditionally been routed to univentricular palliation and labeled as "unseptatable." This report describes our initial experience in septation/biventricular conversion ("ventricular switch"), utilizing the left ventricle (LV) as the subpulmonary ventricle, essentially recapitulating the physiology of congenitally corrected transposition of the great arteries. Five consecutive patients with challenging anatomic configuration underwent septation.

Tetralogy of Fallot with absent pulmonary valve-When the ductus is present: A case of isolated branch pulmonary artery and review of literature.

Tetralogy of Fallot/Absent Pulmonary Valve (TOF/APV) has been classically associated with the absence of a patent ductus arteriosus (PDA). We present a rare case of APV in TOF with a discontinuous left pulmonary artery (LPA) that was suspected during fetal echocardiogram. Postnatal echocardiogram confirmed the origin of a hypoplastic LPA from the PDA.

Rare case of septum primum malposition defect in dextrocardia and situs inversus totalis without heterotaxy syndrome.

Septum primum malposition defect is an extremely rare CHD and is often found in patients with visceral heterotaxy, particularly of the polysplenia type. We describe a unique patient with dextrocardia, situs inversus totalis, and bilateral superior vena caval veins without heterotaxy syndrome who was diagnosed with an interatrial defect and partial anomalous pulmonary venous drainage due to malposition of the septum primum.

Congenital and Acquired Systemic Venous Anomalies: A Potpourri of Right to Left Shunts.

Systemic venous anomalies can occur in isolation or in association with other congenital heart disease. A left superior vena cava draining to a roofed coronary sinus represents the most common venous variant and is usually inconsequential. Embryologic or acquired disruptions in systemic veins can be problematic due to shunting and/or venous obstruction.

A unique pattern of delayed enhancement of a large cardiac fibroma on magnetic resonance imaging.

MRI is a valuable noninvasive tool that helps in predicting the type of cardiac tumors and guiding management decisions. Several reports have described the appearance of cardiac fibromas on MRI, which typically show hyperenhancement on myocardial delayed enhancement (MDE) imaging, with or without a dark core. This report demonstrates the unique appearance of a large solitary ventricular septal cardiac fibroma in a 5-month-old patient on MDE imaging, with two discrete dark cores, each surrounded by a hyperenhancing pseudocapsule.

Scimitar Syndrome and H-type Tracheo-esophageal Fistula in a Newborn Infant.

Scimitar syndrome is a rare congenital anomaly characterized by partial anomalous pulmonary venous drainage of the right lung to the inferior vena cava (IVC) creating a tubular opacity paralleling the right cardiac border on chest radiography which resembles a curved Turkish sword or scimitar. Associated pulmonary and vascular anomalies have been reported in cases of Scimitar syndrome, most commonly hypoplasia of right lung, dextroposition of the heart, hypoplasia of the right pulmonary artery, and aberrant arterial supply from the descending aorta to the affected lobe of the right lung. To the best of our knowledge, this is the first case of Scimitar syndrome with an H-type tracheoesophageal fistula that has ever been reported.

Computed tomographic angiography identification of intramural segments in anomalous coronary arteries with interarterial course.

Certain coronary anomalies are associated with high risk features. We sought to determine the diagnostic accuracy of coronary computed tomographic angiography (CTA) in determining high-risk features, particularly intramural segments. Anomalous coronary arteries can be associated with adverse clinical events.

Methods to access the surgically excluded cavotricuspid isthmus for complete ablation of typical atrial flutter in patients with congenital heart defects.

Background: Cavotricuspid isthmus (CVTI)-dependent flutter in postoperative congenital heart disease patients is common and difficult to treat.

Objective: The purpose of this study was to evaluate techniques for accessing excluded portions of the CVTI after Fontan or atrial switch procedures and completely ablating flutter.

Methods: Patients who had undergone Fontan or atrial switch procedures and had CVTI-dependent flutter requiring ablation between 1990 and 2007 were identified.

Successful transcatheter closure of an aorto-left atrial fistula.

Aorto-left atrial fistula is a rare entity in which the integrity of the aortic root bordering the left atrium is disrupted. The clinical presentation is highly variable, depending predominantly on the size of the fistula and the pressure difference between the aorta and the left atrium. Surgical repair was the standard treatment.