Contraceptive Practices of Women With Complex Congenital Heart Disease.

Understanding the contraceptive practices of women with complex congenital heart disease (CHD) and providing them individualized contraception counseling may prevent adverse events and unplanned high-risk pregnancies. Given this, we sought to examine the contraceptive practices in women with CHD, describe adverse events associated with contraceptive use, and describe the provision of contraception counseling. Women >18 years were recruited from 2011 to 2014 from 9 adult CHD (ACHD) centers throughout North America. Subjects completed a 48-item questionnaire regarding contraceptive use and perceptions of contraception counseling, and a medical record review was performed. Of 505 subjects, median age was 33 (interquartile range 26 to 44) and 81% had CHD of moderate or great complexity. The majority (86%, 435 of 505) of the cohort had used contraception. The types included barrier methods (87%), oral contraception (OC) 84%, intrauterine device (18%), Depo-Provera (15%), vaginal ring (7%), patch (6%), hormonal implant (2%), Plan B (19%), and sterilization (16%). Overall OC use was not significantly different by CHD complexity. Women with CHD of great complexity were more likely to report a thrombotic event while taking OC than those with less complex CHD (9% vs 1%, p = 0.003). Contraception counseling by the ACHD team was noted by 43% of subjects. Unplanned pregnancy was reported by 25% with no statistical difference by CHD complexity. In conclusion, contraceptive practices of women with complex CHD are highly variable, and the prevalence of blood clots while taking OC is not insignificant while provision of contraception counseling by ACHD providers appears lacking.

Clinical efficacy of dofetilide for the treatment of atrial tachyarrhythmias in adults with congenital heart disease.

BACKGROUND: Atrial tachyarrhythmias (AT) including atrial fibrillation (AF), atrial flutter (AFL), and atrial tachycardia represent a clinical challenge in the adult with congenital heart disease (CHD). Dofetilide (D) is a rapidly activating delayed rectifier potassium channel (IKr) blocker effective in pharmacological conversion and maintenance of normal sinus rhythm in patients with AF and AFL. There is limited knowledge regarding the role of D in adults with CHD. METHODS: Safety and efficacy of D was evaluated in a consecutive group of thirteen adult patients (age 40 ± 11; six women) with CHD and refractory AT. RESULTS: Ten patients had persistent (four AFL, one AF, and five atrial tachycardia) and three paroxysmal (one AF and two atrial tachycardia) AT. All patients were symptomatic during tachycardia, 12 patients had previously failed 2 ± 1 antiarrhythmic drugs. Mean systemic ventricular ejection fraction was 55 ± 9%; baseline QRS complex duration was 129 ± 45 ms (>120 ms in six patients). Patients were followed on D for 33 ± 39 months (median 16). Among 10 patients with persistent AT, seven patients (70%) pharmacologically converted to sinus rhythm on D and three patients (30%) required direct current cardioversion. Two patients (15.4%) experienced complete arrhythmia suppression, and seven (53.8%) experienced significant clinical improvement with sporadic recurrences; average time to recurrence was 5.5 ± 3.5 months. One patient developed torsade de pointes during loading, and the drug was discontinued. D was discontinued in five (38.5%) other patients due to recurrence of AT (n = 4) and renal failure (n = 1). Corrected QT interval (QTc) increased from 452 ± 61 to 480 ± 49 ms (P = .04) and corrected JT interval (JTc) from 323 ± 39 to 341 ± 33 ms (P = .09). CONCLUSIONS: D should be considered a pharmacologic alternative when adult patients with CHD develop AT. D does not depress conduction, sinus node, or ventricular function but needs close monitoring for potential ventricular pro-arrhythmia.

Long-term results of the subclavian flap repair for coarctation of the aorta in infants.

BACKGROUND: Coarctation is a congenital narrowing of the aorta that often requires repair during infancy. The subclavian flap aortoplasty was once widely favored for its avoidance of a circumferential suture line and low incidence of recoarctation. The aim of this study is to report the long-term results of the subclavian flap repair for coarctation of the aorta in infants. METHODS: Our operative database was queried for infants with coarctation who underwent subclavian flap aortoplasty from 1966 to 1991. Medical records were reviewed for patient characteristics and outcomes. Survivors were identified for additional phone interview. RESULTS: Fifty-five patients met the inclusion criteria. There were 7 early deaths (in hospital), 11 late deaths, 5 patients lost to follow-up, and 32 known long-term survivors with a mean follow-up of 22.0 years (range 2.4-34.9). Hospital mortality was not associated with patient characteristics but was associated with earlier year of surgery (P = .015). A trend toward decreased overall survival was seen in patients with coarctation with associated cardiac defects (P = .072). Reintervention for recoarctation was required in 3 (6.6%) patients and was not related to the patient characteristics. There were no apparent complications related to subclavian artery sacrifice. CONCLUSIONS: Subclavian flap aortoplasty provides excellent long-term results for the repair of coarctation in infants. The incidence of recoarctation requiring reintervention is low and compares favorably with other techniques. Compromise of growth or function of the left arm was not appreciated. The subclavian flap technique remains a viable surgical option for the repair of coarctation in infants.

Aortic root dilatation in adults with surgically repaired tetralogy of fallot: a multicenter cross-sectional study.

BACKGROUND: Although aortic root pathology has been described in patients with tetralogy of Fallot, the scope of the problem remains poorly defined. We sought to determine the prevalence and predictors of aortic root dilatation in adults with repaired tetralogy of Fallot. METHODS AND RESULTS: A multicenter cross-sectional study was conducted with standardized reassessment of echocardiographic parameters in 474 adults (≥18 years) with surgically repaired tetralogy of Fallot or pulmonary atresia with ventricular septal defect. The aortic root was measured in a parasternal long-axis view, in diastole, at the level of the sinus of Valsalva. Prevalence and predictors of an absolute diameter ≥40 mm and of an observed-to-expected ratio >1.5 were assessed. The aortic root dimension was ≥40 mm in 28.9% (95% confidence interval, 26.9%-30.9%). In multivariate analyses, the only independently associated factor was male sex (odds ratio, 4.48; 95% confidence interval, 1.55-12.89; P=0.006). The prevalence of an observed-to-expected aortic root dimension ratio >1.5 was 6.6% (95% confidence interval, 5.3%-7.9%). It was associated with pulmonary atresia and moderate or severe aortic regurgitation in univariate analyses, but no independent predictor was identified. The side of the aortic arch was not associated with aortic root dimension. The prevalence of moderate or severe aortic regurgitation was 3.5% (95% confidence interval, 2.7%-4.2%). CONCLUSIONS: Although nearly one third of adults with repaired tetralogy of Fallot have an aortic root diameter ≥40 mm, the prevalence of a dilated aortic root, when defined by an indexed ratio of observed-to-expected values, is low. Similarly, moderate or severe aortic regurgitation is uncommon.

Heart rate response during exercise and pregnancy outcome in women with congenital heart disease.

BACKGROUND: Cardiopulmonary exercise testing is often used to evaluate exercise capacity in adults with congenital heart disease including women who are considering pregnancy. The relationship between cardiopulmonary exercise testing parameters and pregnancy outcome has not been defined. METHODS AND RESULTS: We conducted a multicenter retrospective observational study of women with congenital heart disease who had undergone cardiopulmonary exercise testing within 2 years of pregnancy or during the first trimester. Cardiopulmonary exercise testing variables included peak oxygen consumption and measures of chronotropic response: peak heart rate, percentage of maximum age predicted heart rate, heart rate reserve (peak heart rate-resting heart rate), and chronotropic index [(peak heart rate-resting heart rate)/(220-age-resting heart rate)]. We identified 89 pregnancies in 83 women. There were 4 spontaneous abortions and 1 termination. One or more adverse cardiac events occurred in 18%; congestive heart failure in 14%, and sustained arrhythmia in 7%. Peak heart rate (odds ratio [OR] 0.71; 95% confidence interval [CI] [0.53, 0.94]; P=0.02), percentage of maximum age predicted heart rate (OR 0.93; 95% CI [0.88, 0.98]; P=0.01), and chronotropic index (OR 0.65; 95% CI [0.47, 0.90]; P=0.01) were associated with a cardiac event. Neonatal events occurred in 20%. Peak heart rate (OR 0.75; 95% CI [0.58, 0.98]; P=0.04), percentage of maximum age predicted heart rate (OR 0.94; 95% CI [0.89, 0.99]; P=0.02), heart rate reserve (OR 0.8; 95% CI [0.64, 0.99]; P=0.04), and chronotropic index (OR 0.73; 95% CI [0.54, 0.98]; P=0.04) correlated with a neonatal event. Peak oxygen consumption was not associated with an adverse pregnancy outcome. CONCLUSIONS: Abnormal chronotropic response correlates with adverse pregnancy outcomes in women with congenital heart disease and should be considered in refining risk stratification schemes.

Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study.

BACKGROUND: The arrhythmia burden in tetralogy of Fallot, types of arrhythmias encountered, and risk profile may change as the population ages. METHODS AND RESULTS: The Alliance for Adult Research in Congenital Cardiology (AARCC) conducted a multicenter cross-sectional study to quantify the arrhythmia burden in tetralogy of Fallot, to characterize age-related trends, and to identify associated factors. A total of 556 patients, 54.0% female, 36.8+/-12.0 years of age were recruited from 11 centers. Overall, 43.3% had a sustained arrhythmia or arrhythmia intervention. Prevalence of atrial tachyarrhythmias was 20.1%. Factors associated with intraatrial reentrant tachycardia in multivariable analyses were right atrial enlargement (odds ratio [OR], 6.2; 95% confidence interval [CI], 2.8 to 13.6), hypertension (OR, 2.3; 95% CI, 1.1 to 4.6), and number of cardiac surgeries (OR, 1.4; 95% CI, 1.2 to 1.6). Older age (OR, 1.09 per year; 95% CI, 1.05 to 1.12), lower left ventricular ejection fraction (OR, 0.93 per unit; 95% CI, 0.89 to 0.96), left atrial dilation (OR, 3.2; 95% CI, 1.5 to 6.8), and number of cardiac surgeries (OR, 1.5; 95% CI, 1.2 to 1.9) were jointly associated with atrial fibrillation. Ventricular arrhythmias were prevalent in 14.6% and jointly associated with number of cardiac surgeries (OR, 1.3; 95% CI, 1.1 to 1.6), QRS duration (OR, 1.02 per 1 ms; 95% CI, 1.01 to 1.03), and left ventricular diastolic dysfunction (OR, 3.3; 95% CI, 1.5 to 7.1). Prevalence of atrial fibrillation and ventricular arrhythmias markedly increased after 45 years of age. CONCLUSIONS: The arrhythmia burden in adults with tetralogy of Fallot is considerable, with various subtypes characterized by different profiles. Atrial fibrillation and ventricular arrhythmias appear to be influenced more by left- than right-sided heart disease.

Sterile inflammation associated with transradial catheterization and hydrophilic sheaths.

In 1999, we noted the development of inflammation and/or abscesses at the site of radial access in a group of patients. Over a 3-year period, we noted this inflammation in 33 patients out of 2,038 (1.6%) who had catheterization via the radial approach. The radial abscesses occurred in 30 patients out of 1,063 (2.8%) in whom we could confirm the use of a hydrophilic-coated sheath, but in no patient for whom we can document that an uncoated sheath was used. No infectious agent could be implicated, and the time course for the development of the abscess, typically 2 to 3 weeks, seemed long for a bacterial infection. Later patients had biopsies, and granulomatous reactions were seen in most. Additionally, a few of the biopsies showed an amorphous extravascular substance consistent with the catheter coating. All patients had good long-term outcomes.

Same-day transradial outpatient stenting with a 6-hr course of glycoprotein IIb/IIIa receptor blockade: a feasibility study.

The aim of the study was to explore the feasibility of same-day outpatient stent placement using a short course of intravenous antiplatelet therapy. Patients (n = 26) had stent placement and 6 hr of eptifibatide therapy. Demographics, procedural information, CPK data, and length of stay were recorded along with postdischarge outcomes. Twenty-one men and five women with median age of 60 years (49, 69) underwent transradial stenting. Baseline characteristics included diabetes 62%, hyperlipidemia 77%, prior coronary bypass surgery 19%, and unstable angina 35%. There were no CPK elevations (> 2 x normal) or ECG changes. Discharge occurred after 6.5 hr (5.8, 7.0). Neither vascular site complications nor readmission for procedure-related problems occurred. One patient later expressed concerns about discharge education. Outpatient stent placement with 6-hr infusion of GP IIb/IIIa inhibitor appears feasible and efficient in select patients. There may be challenges to meet with regard to patient education. Further studies with larger populations are needed to evaluate and optimize this approach.

Radial approach to right heart catheterization: early experience with a promising technique.

Transradial cardiac catheterization has been historically limited to arterial catheterization. Using small-diameter introducers and catheters, a technique of combined right and left heart catheterization is described using both the radial artery and its neighboring vein. Evaluation of 55 sequential right and left heart procedures showed that it could be accomplished in > 50% using venous access in the forearm. This approach has many potential advantages, although the ability to access routinely both venous and arterial vessels at the level of the wrist is unclear.