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Guidelines recommend screening cardiovascular magnetic resonance (Sc-CMR) imaging for all patients after coarctation of the aorta repair, although there are limited data verifying its clinical utility. Therefore, we sought to assess the value of Sc-CMR in detecting aortic complications and at-risk abnormalities after coarctation of the aorta repair and to identify significant risk factors. We reviewed 76 patients (mean age 31 ± 10 years), including 40 with symptomatically indicated CMR (Sx-CMR) and 36 with Sc-CMR studies. CMR angiograms were evaluated for aortic abnormalities. Recoarctation was defined as residual narrowing/descending aorta at the diaphragm ≤0.5 (at risk ≤0.75), ascending aorta aneurysm as maximum ascending cross-sectional area/height ≥10 (at risk ≥5), and descending aorta aneurysm as maximum descending diameter/descending aorta at the diaphragm ≥1.5 (at risk ≥1.25). Aortic complications or abnormalities were found in 45 patients (59%). No patient met criteria for recoarctation (at risk 10 Sx-CMR vs 5 Sc-CMR). Significant risk factors included heart failure symptoms and female gender (p0.05). One patient (Sc-CMR) had ascending aneurysm (at risk 17 Sx-CMR vs 8 Sc-CMR). Time from repair was a significant predictor (p0.05). There were 10 patients (6 Sx-CMR vs 4 Sc-CMR) with descending aneurysm (at risk 8 Sx-CMR vs 7 Sc-CMR). Cardiovascular symptoms, hypertension, and echocardiogram were not predictive. In conclusion,50% of patients undergoing Sc-CMR had aortic abnormalities, which was not significantly different from those undergoing Sx-CMR. In particular, Sc-CMR identified descending aorta aneurysms that were not predicted by clinical parameters or echocardiogram.

Adult congenital heart disease (ACHD) presents a unique and growing patient population, but there are no data to suggest there are sufficient training programs available to train specialists to meet the needs of this population. The objective of this national survey was to determine the current status of ACHD fellowship curricula and training. An online questionnaire was provided to adult and pediatric cardiology fellowship programs to determine program demographics, status, duration, faculty teaching responsibilities, volume or patient exposure, and institutional obstacles. Of the 225 programs surveyed, 81 responded (36%). Nearly all were university affiliated (93%) with1/3 (29%) reporting an ACHD fellowship program. Most offered either a 1- (42%) or a 2-year (47%) fellowship. ACHD fellowship funding was provided by hospital budget (68%). Half reported an increase in applicants to their fellowship. Applicants were more likely to have had previous training in adult cardiovascular medicine (56%). Respondents ranked "Volume of Outpatients/Year" and "Lack of Institutional Support" as the most important aspect of an ACHD program to applicants and greatest obstacle to ACHD training, respectively. After training, ACHD fellows obtained adult cardiology/ACHD positions (47%) and were within an academic center (88%). In conclusion, the results demonstrate a deficiency in the number of currently available ACHD fellowship programs. Measures should be taken to strengthen and standardize ACHD training to meet the increasing workforce requirements of this population.

Patients with adult congenital heart disease are at increased risk of ventricular arrhythmia (VA) and sudden cardiac death, although no clear predictors have been found. Ventricular programmed stimulation has been shown to predict clinical ventricular tachycardia and sudden death events, but the role of screening electrophysiology studies (S-EPSs) in this population remains poorly defined. Therefore, we sought to determine the prevalence of inducible VA and to evaluate the clinical predictors in a heterogeneous group of patients with adult congenital heart disease (or =18 years old) undergoing S-EPSs at preoperative or interventional cardiac catheterization. Studies for the primary evaluation of clinical VA were excluded. The demographic, clinical, and diagnostic findings were compared between the patients with positive and negative findings. From 2005 to 2009, 80 patients (mean age 30 +/- 9 years) underwent S-EPSs, and 23 had inducible VA. The diagnoses for those with studies positive for VA included tetralogy of Fallot (n = 12), d-transposition of the great arteries (n = 6), pulmonary stenosis (n = 2), double outlet right ventricle (n = 1), double inlet left ventricle (n = 1), and Ebstein's anomaly (n = 1). Men were significantly more likely to have a S-EPS positive for VA (p = 0.015). Increasing QRS duration, decreasing peak oxygen uptake (percentage of predicted), and ventricular fibrosis with cardiovascular magnetic resonance imaging were significantly associated with studies positive for VA (p0.05). Combined fibrosis and a peak oxygen uptake80% of predicted had 100% sensitivity for positive VA findings. In conclusion, almost 30% of those with adult congenital heart disease undergoing S-EPSs had inducible VA. A prolonged QRS duration, diminished exercise capacity, and the presence of ventricular fibrosis were significantly associated with findings positive for VA and might improve patient selection for screening evaluations.

There has been a tremendous focus on gynecologic health with the increasing number of women with congenital heart disease (CHD). Conversely, experience in providing sexual health screening to men with CHD is lacking. The purpose of this study was to identify sexual health issues in men with CHD. An anonymous survey was distributed to men in our adult CHD clinic. Age, type of CHD, medications, detailed sexual history including cardiovascular symptoms with intercourse, erectile dysfunction (ED), and willingness to discuss ED with a physician was obtained. A logistic regression analysis was performed to analyze the relation between medications, CHD complexity, and ED; 86 men completed the survey with a mean age of 34 +/- 10 years. The prevalence of ED was 38%. Men were treated with cardiac medications including beta blockers (BB; 24%), angiotensin-converting enzyme (ACE) inhibitors (8%), calcium-channel blockers (6%), and combination BB and ACE inhibitor therapy (16%). Men on BB were 3.13 times more likely to report ED (p = 0.045). Complexity of CHD did not increase the likelihood of ED symptoms (p0.10). Although most men attributed symptoms to their underlying CHD, most were willing to discuss issues of ED with their cardiologist. In conclusion, ED is prevalent in young men with CHD. Although an emotional component may contribute, certain medications may exacerbate symptoms.

Percutaneous interventions can treat long-term complications after Mustard atrial switch operation in patients with d-transposition of the great arteries (d-TGA), but follow-up for these procedures has not been established. Four patients with d-TGA and previous Mustard operation underwent percutaneous placement of covered stents to relieve superior and inferior vena caval baffle obstructions and leaks. At 6 to 13 months, assessment with 16-slice spiral computed tomography identified stent patency as well as lead placement and visualization of additional devices.

Pulmonary autograft aortic valve replacement (Ross procedure) is increasing in popularity, particularly in children and young adults. We performed a controlled study of pediatric and adult Ross procedure patients to evaluate postoperative exercise valvular hemodynamics and cardiac rhythm. Thirty-one patients and 24 controls underwent stress echocardiography. Doppler and 2-dimensional echocardiography assessed valvular hemodynamics and right and left ventricular size at baseline and after exercise. Electrocardiography monitored cardiac rhythm. Patients and controls had neoaortic and neopulmonic valve insufficiency ranging from none to moderate that improved or did not change with exercise. Baseline and exercise mean peak transaortic gradients were not significantly different between patients (baseline 6.3 +/- 3.8 mm Hg, exercise 12.8 +/- 7.3 mm Hg) and controls (baseline 6.7 +/- 2.5 mm Hg, exercise 14.3 +/- 5.2 mm Hg). However, baseline and exercise mean peak transpulmonic gradients were significantly different between patients (baseline 20.7 +/- 9.6 mm Hg, exercise 45.2 +/- 23.5 mm Hg) and controls (baseline 3.7 +/- 1.3 mm Hg, exercise 10.6 +/- 3.7 mm Hg). Significant exercise-induced arrhythmias occurred in 8 patients (26%) and no controls. Occurrence of arrhythmias correlated with an older age at surgery and age at study. After the Ross procedure, patients have valve competence and transaortic gradients similar to controls at baseline and with exercise. However, patients have significantly increased baseline and exercise transpulmonic gradients compared with controls. Furthermore, exercise-induced arrhythmias occurred frequently in our patient group and were associated with an older age at surgery and age at study.

We present a 16-year-old girl who developed congestive heart failure during and after delivery of her first child, and who was diagnosed as having peripartum cardiomyopathy. Cardiac catheterization with coronary arteriography confirmed the correct diagnosis of anomalous origin of the left main coronary artery from the pulmonary trunk.