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. Despite an aneurysmal (right pulmonary artery) (RPA), axial imaging demonstrated widely patent tracheobronchial system with no evidence of bronchial compression. Clinically, the child required only minimal respiratory support. Genetic testing was positive for 22 q11deletion, commonly associated with this lesion. Surgery consisted of unifocalization of the discontinuous LPA with placement of a valved pulmonary homograft during complete repair of this lesion. Our case highlights the importance of prenatal detection, to aid in the prompt initiation of prostaglandins so as to ensure early rehabilitation of the left lung. Inability to visualize one of the branch pulmonary arteries (PA's) and a PDA on fetal echocardiogram in TOF/APV must raise suspicion for an eccentric branch PA with ductal origin.

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.

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.

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. Inclusion criteria included aortic flow quantification at aortic valve and one additional level, and no or trace mitral regurgitation (MR) documented both by the MRI AND an echocardiogram done within a year. In addition to flow quantification with standard contouring (SC), a novel Selective Systolic Contouring (SSC) method was performed at aortic valve level, contouring the area demarcated by the AoV leaflets in systole. The bias in each technique's estimate of aortic forward flow was calculated as the mean difference between aortic forward flow and left ventricular stroke volume (LV SV). 98 patients (mean age 56, 71% male) were included: 33 with tricuspid and 65 with congenitally abnormal (bicuspid or unicuspid) AoV. All methods tended to underestimate aortic forward flow, but the bias was smallest with the SSC method (p < 0.001). Therefore, SSC yielded the lowest estimates of mitral regurgitant volume (4.8 ml) and regurgitant fraction (3.9%) (p < 0.05). SSC at AoV level better approximates LV SV in our cohort, and may provide more accurate quantitative assessment of both aortic and mitral valve function.

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 (n = 40) were evaluated for anatomical and virtual device fitting (3D-printed models of pediatric [P-CFTAH] and infant [I-CFTAH] models). The virtual sub-study consisted of analysis of preoperative thoracic radiographs and computed tomography (n = 3; 4.2, 5.3, and 10.2 kg) imaging data. Results: P-CFTAH pump fit in 21 out of 40 patients (fit group, 52.5%) but did not fit in 19 patients (non-fit group, 47.5%). I-CFTAH pump fit all of the 33 patients evaluated. There were critical differences due to dimensional variation (p < 0.0001) for the P-CFTAH, such as body weight (BW), height (Ht), and body surface area (BSA). The cutoff values were: BW: 5.71 kg, Ht: 59.0 cm, BSA: 0.31 m2. These cutoff values were additionally confirmed to be optimal by CT imaging. Conclusions: This study demonstrated the range of proper fit for the P-CFTAH and I-CFTAH in congenital heart disease patients. These data suggest the feasibility of both devices for fit in the small-patient population.

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. All patients were severely cyanotic and had important functional limitations. All patients required complex atrial septation. Ventricular septation was precluded by fixed pulmonary vascular resistance in 2 patients. Systemic venous return was diverted to the morphologic LV as part of physiological 2V (n = 4) or 1.5 V repair (n = 1). Median conversion age was 9 years (range 11 months-46 years). Four patients had 12 previous cardiac surgical procedures in preparation for univentricular repair elsewhere. Three dimensional-printed heart models evaluated feasibility of septation. All patients are alive at a median follow-up of 0.6 years (range 0.08-2.7 years). Median hospital stay was 13 (range 10-60) days. LV recruitment improved functional status and significantly increased systemic oxygen saturation in all patients (79 ± 7% vs 95 ± 5%, P = 0.003). We report a novel paradigm for successfully utilizing both ventricles with the morphologic LV as the subpulmonary ventricle, in a complex population thought to be unseptatable. This approach is versatile and can likely be extrapolated to other complex anatomic configurations. Although we utilized this strategy in patients of variable age, earlier ventricular switch may yield the best results.

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. In this case series, we report four systemic venous anomalies in patients with atrial and ventricular situs solitus. Each case demonstrates how clinically significant right to left shunts can occur from a systemic vein to the left atrium. Diagnosis in each of these patients was delayed due to suboptimal diagnostic testing. Accurate diagnosis can be made by thorough anatomic evaluation of the presence, patency, and drainage of all systemic veins and supported with proper planning and interpretation of bubble studies.

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. Anomalous coronaries that course between the great vessels (interarterial) have been associated with sudden death. High-risk features of interarterial vessels described in the literature include; a slit-like orifice, acute angle of origin, and intramural segments (within the wall of the aorta). Although computed tomography (CT) findings of acute angle and slit like orifice have been described previously no prior evaluations regarding CT identification of an intramural segment have been reported. An intramural segment has distinct surgical management implications. All interarterial anomalous coronary arteries do not have an intramural segment. Since October 2004, 15 patients were diagnosed by CTA as having an anomalous coronary artery with an interarterial course, which were then confirmed by intraoperative examination of their coronary origins and course during aortic root/coronary artery surgery. The CTA images were retrospectively analyzed for the presence of high-risk features by a radiologist blinded to the surgical findings. Comparison of these findings was made to the findings at surgery. The anomalous coronary was the right coronary artery in 10 patients and the left coronary artery in 5. Eleven patients had an intramural segment identified at surgery. Pre-operative coronary CTA showed that all patients with an intramural course of the anomalous artery, had slit-like orifice, an acute angle of origin (mean 18.4 ± 3.4°), and an elliptical shaped cross-section throughout the intramural segment of the anomalous vessel. The average vessel height/width ratio for anomalous coronary vessels without an intramural segment was 1.03; compared to a ratio of 2.19 for anomalous vessels with an intramural segment (P = 0.003). Coronary CTA can identify an intramural segment of an anomalous interarterial coronary artery by its elliptical shape. Identifying an intramural segment has important clinical and surgical implications.

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. Flutters induced, methods for accessing the CVTI, use of intracardiac echocardiography, complications, and success rates were noted. RESULTS: Sixteen patients (44% males, mean age at ablation 28 years) were identified: 14 prior Fontan and 2 Mustard repair, with a total of 19 ablation procedures. In 13 (81%) of 16 patients, access to the entire CVTI could not be achieved via a systemic venous route. The excluded CVTI was accessed by retrograde transaortic approach in 6 and by anterograde transconduit puncture in 1 patient, with termination and lack of reinducibility of CVTI-dependent flutter achieved in all cases. One patient developed high-grade AV block requiring pacemaker therapy. Follow-up data (range 1-89 months, mean 29 months) were available for 18 of 19 procedures. CVTI atrial flutter recurred in 1 of 7 patients involving access to the pulmonary venous side. CONCLUSION: Even when surgical procedures exclude a portion of the CVTI, complete ablation of "typical" atrial flutter, including documentation of bidirectional block, can be achieved by novel approaches targeting the surgically excluded arrhythmogenic atrial tissue.

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. Recently, however, there have been reports of successful transcatheter closure. We report a 32-year-old male with Shone's syndrome who had multiple prior surgical procedures including aortic and mitral valve replacements. He presented with an aorto-left atrial fistula that was successfully closed percutaneously using an Amplatzer atrial septal defect device.