Diagnosis of anomalous origin of the right subclavian artery from the right pulmonary artery in a patient with D-transposition of the great arteries utilizing transthoracic echocardiography.

Diagnosis of anomalous origin of the right subclavian artery (AORSA) from the right pulmonary artery (RPA) is usually made using CT, MRI, or invasive angiography. We report a patient diagnosed using transthoracic echocardiography (TTE). A newborn girl prenatally known to have d-TGA presented with cyanosis sparing the right hemithorax and arm. Oxygen saturations on the right hand were persistently higher than on the right ear and other extremities. Repeat TTE using a modified echocardiographic imaging plane allowed for full visualization of the entire subclavian artery course, revealing AORSA from RPA. We discuss further the approach to echocardiographic diagnosis and surgical implications.

3D Printing to Guide Ventricular Assist Device Placement in Adults With Congenital Heart Disease and Heart Failure.

As the population of adults with congenital heart disease continues to grow, so does the number of these patients with heart failure. Ventricular assist devices are underutilized in adults with congenital heart disease due to their complex anatomic arrangements and physiology. Advanced imaging techniques that may increase the utilization of mechanical circulatory support in this population must be explored. Three-dimensional printing offers individualized structural models that would enable pre-surgical planning of cannula and device placement in adults with congenital cardiac disease and heart failure who are candidates for such therapies. We present a review of relevant cardiac anomalies, cases in which such models could be utilized, and some background on the cost and procedure associated with this process.

Subpulmonary stenosis assessed in midtrimester fetuses with tetralogy of Fallot: a novel method for predicting postnatal clinical outcome.

This study aimed to determine whether quantification of subpulmonary stenosis (SPS) in tetralogy of Fallot (TOF) in the second-trimester fetus can predict postnatal clinical outcome measured by pulmonary valve size and/or timing or type of intervention. The study retrospectively identified fetuses with TOF from 1998 to 2010 diagnosed at 26 weeks gestation or earlier. The data evaluated included pre- and postnatal pulmonary valve z-scores (PVZ). To quantify fetal SPS, the authors created a novel index, the SPS/DAO ratio, a ratio of the minimum infundibular diameter to the descending aorta diameter (DAO). Multiple linear regression was used to predict postnatal PVZ from prenatally determined parameters, including SPS/DAO. Fetal parameters were analyzed by logistic regression for association with postnatal outcomes, namely, timing of surgery (<1 month), used as a surrogate for severity, and type of surgery [transannular patch (TAP) vs valve sparing surgery]. A total of 23 fetuses met the inclusion criteria. The mean gestational age was 21.8 ± 1.9 weeks (range, 16.6-25.4 weeks). There was excellent correlation between predicted and measured PVZ (r = 0.82; p < 0.0001) using the following derived equation: -3.68 + (0.91 × prenatal PVZ) - (4.44 × SPS/DAO) - 3.19 (prenatal PVZ × SPS/DAO). An SPS/DAO value lower than 0.5 had 100 % sensitivity and 56 % specificity for repair before the age of 1 month, and a value lower than 0.47 had 100 % sensitivity and 75 % specificity for TAP repair. Prenatal PVZ and the SPS/DAO ratio at 26 weeks gestation or earlier can reliably predict postnatal PVZ in fetuses with TOF. Quantification of SPS with the SPS/DAO ratio identifies patients who may require early intervention secondary to disease severity and may predict the type of repair, thereby influencing prenatal counseling.

Cardiovascular magnetic resonance as an alternate method for serial evaluation of proximal aorta: comparison with echocardiography.

Thoracic aortic disease is a known cause of aortic dilatation and poses significant risk of aortic dissection and rupture. Serial assessment of aortic root dimensions is traditionally performed using echocardiography, which is limited with older age and following surgery, due to poor acoustic windows. Although diastolic measurements are utilized as standard practice in decision making of adult aortopathy, systolic diameters are utilized in pediatric practice. Three-dimensional steady-state free precision (3D-SSFP) has shown promise as an alternate method for providing accurate and reproducible aortic measurements. The agreement between proximal aorta measurements by diastolic 3D-SSFP and echocardiography (both systole and diastole) was examined in 40 subjects. The maximum inner diameters at aortic annulus, root and sinotubular junction demonstrated excellent agreement between 3D-SSFP and echocardiography for all the 3 levels. The best agreement was observed for diastolic root dimensions with a mean difference of +0.01 cm, limits of agreement being -0.26 to +0.28 cm. Three D-SSFP can be used interchangeably with echocardiography in the serial assessment of the aortic root size. Careful attention to obtain an imaging plane utilizing 3D multiplanar reformatting is critical to maximize the agreement between the two imaging modalities.

Repeatability of cardiac-MRI-measured right ventricular size and function in congenital heart disease.

BACKGROUND: The measurement error for right ventricular (RV) size and function assessed by cardiac MRI (CMRI) in congenital heart disease has not been fully characterized. As CMRI parameters are being increasingly utilized to make clinical decisions, defining error in the clinical setting is critical. OBJECTIVE: This investigation examines the repeatability of CMRI for RV size and function. MATERIALS AND METHODS: Forty consecutive people with congenital heart disease involving the RV were retrospectively identified. Contouring of RV volumes was performed by two expert CMRI clinicians. The coefficient of variability and repeatability coefficients were calculated. Repeatability coefficients were multiplied by the mean value for each group studied to define a threshold beyond which measurement error was unlikely to be responsible. RESULTS: The variability for indexed RV end-diastolic volume = 3.2% and 3.3% for intra- and interobserver comparisons, respectively. The repeatability coefficients were 13.2% and 14.9% for intra- and interobserver comparisons, which yielded threshold values of 15.1 ml/m^2 and 20.2 ml/m^2, respectively. For RV ejection fraction (EF), the repeatability coefficients for intra- and interobserver comparisons were 5.0% and 6.0%, which resulted in threshold values of 2.6 EF% and 3.0 EF%. CONCLUSION: The threshold values generated can be used during serial assessment of RV size and function.

Echocardiographic diagnosis of clinically silent congenital coronary artery anomalies.

BACKGROUND: The incidence of congenital coronary anomalies is mainly derived from autopsy series and pre-participation exams in athletes. Limited data exist regarding the spectrum of coronary anomalies that can be detected in asymptomatic patients. We sought to describe echocardiographically detected congenital coronary artery anomalies in asymptomatic children after implementing a screening protocol mandating identification of coronary artery origin and proximal course in all initial studies. METHODS: Our database was searched from 1/1/1993 to 3/31/2006 and all echocardiograms coded for coronary anomalies were identified. Clinically "silent" congenital coronary anomalies were culled from that group. RESULTS: Of the 168 "silent" coronary anomalies detected, 111 were anomalies of aortic origin, including 59 patients with "high coronary takeoff" and 30 patients with "wrong sinus" origin of either the left or right coronary artery. Small coronary fistulas were seen in 57. Associated congenital heart defects were found in 53% of individuals with coronary anomalies. CONCLUSIONS: This study comprises the largest group of echocardiographically detected, "silent" but potentially clinically significant, congenital coronary anomalies in children. Prospective echocardiographic diagnosis of "high coronary takeoff", a risk factor for injury during cardio-pulmonary bypass, and asymptomatic intraseptal coronary stenosis are described for the first time.

Cardiac MR imaging assessment following tetralogy of fallot repair.

Survivors of tetralogy of Fallot (TOF) repair constitute a large and growing population of patients. Although postsurgical outcome is generally favorable, as these patients move into adulthood, late morbidity is becoming more prevalent and the notion that TOF has been "definitively repaired" is increasingly being challenged. Recent evidence suggests that adverse long-term postsurgical outcome is related to chronic pulmonary regurgitation, right ventricular dilatation, and deteriorating ventricular function. Cardiac magnetic resonance (MR) imaging has been established as an accurate technique for quantifying ventricular size, ejection fraction, and valvular regurgitation. Cardiac MR imaging does not expose the patient to ionizing radiation and is therefore ideal for serial postsurgical follow-up. Familiarity with the anatomic basis of TOF, the surgical approaches to repair, and postrepair sequelae is essential for performing and interpreting cardiac MR imaging examinations. For example, awareness of the complications and sequelae that can occur will assist in determining when to intervene to preserve ventricular function and will improve long-term outcome. Technical facility is necessary to tailor the examination to the individual patient (eg, familiarity with non-breath-hold modifications that allow evaluation of young and less compliant patients). The radiologist can play an essential role in the treatment of patients with repaired TOF by providing noninvasive anatomic and physiologic cardiac MR imaging data. Further technologic advances in cardiac MR imaging are likely to bring about new applications, better normative data, and more examinations that are operator independent.