"Treat-to-close": Non-repairable ASD-PAH in the adult: Results from the North American ASD-PAH (NAAP) Multicenter Registry.

BACKGROUND: Adults presenting with an unrepaired atrial septal defect and pulmonary arterial hypertension (ASD-PAH) are typically classified as "correctable" or "non-correctable". The use of directed PAH medical therapy in non-correctable ASD-PAH leading to favorable closure candidacy, repair status and long-term follow-up is not well studied. We therefore sought to characterize response to PAH targeted therapy in 'non-correctable' ASD-PAH. METHODS AND RESULTS: Nine North American tertiary care centers submitted retrospective data from adults with unrepaired ASD-PAH that did not meet recommendations for repair at initial presentation (1996-2017). Sixty-nine patients (women 51(74%), 40 ±â€¯15 years, mean pulmonary artery pressure (mPA) 51 ±â€¯13 mm Hg, pulmonary vascular resistance (PVR) 8.7 ±â€¯4.9 Wood units, Qp:Qs 1.6 ±â€¯0.4) were enrolled. All patients were prescribed PAH targeted therapy and late shunt repair occurred in 19(28%) (Women 15(29%) vs. Men 4(22%), p = 0.6). At late follow-up (4.4 ±â€¯2.9 years) 6-minute walk test distance (6MWTD) was significantly better in the group that underwent repair (486 ±â€¯89 m vs. 375 ±â€¯139 m, p < 0.05). Transthoracic echo showed significant improvement in right ventricular (RV) function (severe dysfunction in repaired 8(40%) vs. unrepaired groups 35(69%), p < 0.05). Divergent survival curves suggest that with larger studies and more follow-up, differences in survival between repaired and unrepaired groups may be important. (repaired: 17(94%) vs. unrepaired: 32(81%), p = 0.18). CONCLUSIONS: This is the first and largest multicenter study evaluating the "treat-to-close" approach in non-correctable ASD-PAH. Our new data supports further study of this strategy in patients who have reversibility of PAH in response to targeted therapy. We demonstrate that in the carefully selected patient with non-correctable ASD-PAH, successful shunt repair is possible if post-therapy PVR is ≤6.5 Wood units. Patients who underwent repair had improved RV function following PAH targeted therapy. Divergent survival curves suggest that with further study, defect repair may affect medium-term to late survival.

3D Printing is a Transformative Technology in Congenital Heart Disease.

Survival in congenital heart disease has steadily improved since 1938, when Dr. Robert Gross successfully ligated for the first time a patent ductus arteriosus in a 7-year-old child. To continue the gains made over the past 80 years, transformative changes with broad impact are needed in management of congenital heart disease. Three-dimensional printing is an emerging technology that is fundamentally affecting patient care, research, trainee education, and interactions among medical teams, patients, and caregivers. This paper first reviews key clinical cases where the technology has affected patient care. It then discusses 3-dimensional printing in trainee education. Thereafter, the role of this technology in communication with multidisciplinary teams, patients, and caregivers is described. Finally, the paper reviews translational technologies on the horizon that promise to take this nascent field even further.

Insurance access in adults with congenital heart disease in the Affordable Care Act era.

BACKGROUND: Adults with congenital heart disease (ACHD) have traditionally been viewed as an underinsured population. Whether this is true in the Affordable Care Act era is unknown. We determined insurance patterns in ACHD patients compared to the non-ACHD cardiology population in a contemporary cohort. METHODS: All cardiology outpatient visits between July 2016 and February 2017 to a large referral center in the United States were reviewed. The primary payer was categorized as health maintenance organization (HMO), preferred provider organization (PPO), Medicare, Medicaid, self-pay, or other. Diagnosis and lesion severity of ACHD were extracted from ICD-10 diagnostic codes and assigned according to the 2008 American College of Cardiology/American Heart Association ACHD guidelines. Age-matching was used to account for baseline age differences between ACHD and non-ACHD patients. RESULTS: E ACHD and 17 154 non-ACHD patients were identified. Without age-matching, ACHD patients were significantly younger than non-ACHD patients (mean age 38.5 vs 63.8 years). After age-matching (N = 805 in each group), mean age was 39.5 years in both groups. ACHD patients had less HMO (29.1% vs 34.7%, P = .012) and Medicaid (12.4% vs 17.3%, P = .006) coverage, but more PPO (34.4% vs 27.5%, P = .003) and Medicare (23.2% vs 18.1%, P = .005) coverage compared to non-ACHD patients. No differences were found in private insurance, public insurance, or self-pay. Lesion complexity had no effect on insurance in ACHD patients. Eligibility of parental plan coverage did not affect use of private insurance. ACHD patients in states with Medicaid expansion had higher rates of Medicaid (15.6% vs 10.6%, P = .045) but lower rates of HMO coverage (24.5% vs 31.7%, P = .036) and self-pay (0% vs 3.3%, P < .001). ACHD status, age, income, and residence in Medicaid expansion states were independent determinants of insurance types. CONCLUSIONS: In the Affordable Care Act era, ACHD patients are a well-insured population. Governmental policy has substantial effects on individual-level choice and access to insurance.

Impact of pregnancy on autograft dilatation and aortic valve function following the Ross procedure.

OBJECTIVE: The effects of pregnancy on autograft dilatation and neoaortic valve function in patients with a Ross procedure have not been studied. We sought to evaluate the effect of pregnancy on autograft dilatation and valve function in these patients with the goal of determining whether pregnancy is safe after the Ross procedure. DESIGN: A retrospective chart review of female patients who underwent a Ross procedure was conducted. PATIENTS: Medical records for 51 patients were reviewed. Among the 33 patients who met inclusion criteria, 11 became pregnant after surgery and 22 did not. OUTCOME MEASURES: Echocardiographic reports were used to record aortic root diameter and aortic insufficiency before, during, and after pregnancy. Patient's charts were reviewed for reinterventions and complications. Primary endpoints included reinterventions, aortic root dilation of ≥5 cm, aortic insufficiency degree ≥ moderate, and death. RESULTS: There were 18 pregnancies carried beyond 20 weeks in 11 patients. There was no significant difference in aortic root diameter between nulliparous patients and parous patients prior to their first pregnancy (3.53 ± 0.44 vs 3.57 ± 0.69 cm, P = .74). There was no significant change in aortic root diameter after first pregnancy (3.7 ± 0.4 cm, P = .056) although there was significant dilatation after the second (4.3 ± 0.7 cm, P = .009) and third (4.5 ± 0.7 cm, P = .009) pregnancies. Freedom from combined endpoints was significantly higher for patients in the pregnancy group than those in the nonpregnancy group (P = .002). CONCLUSIONS: Pregnancy was not associated with significantly increased adverse events in patients following the Ross procedure. Special care should be taken after the first pregnancy, as multiparity may lead to increased neoaortic dilatation.

Increased Body Mass Index Is Associated with Congestive Heart Failure and Mortality in Adult Fontan Patients.

OBJECTIVE: Obesity affects adults with congenital heart disease (CHD). The effect of an increased body mass index (BMI) with respect to morbidity and mortality has not been evaluated in adults with complex CHD. Our objective was to evaluate the effects of increased BMI on heart failure and mortality in univentricular patients who had undergone Fontan palliation. METHODS: A query of Fontan patients' first appointments at the Washington University Center for Adults with CHD between 2007 and 2014 yielded 79 patients. BMI status as normal (<25 kg/m(2) ), overweight (≥25, <30 kg/m(2) ), and obese (≥30 kg/m(2) ) was established at the patient's first appointment. We analyzed demographics, diuretic requirements, New York Heart Association (NYHA) class, and laboratory values using Student's two-sample t-test and Fisher's exact test. Mortality was assessed via survival curves, and hazard ratios were compiled with proportional hazard modeling. RESULTS: The recent average BMI was significantly greater in patients with NYHA classes II-IV (29.3 ± 9 kg/m(2) ) compared with asymptomatic patients (24.8 ± 5.1 kg/m(2) , P = .006). Additionally, the average BMI of patients with a high diuretic requirement (≥40 mg/day IV furosemide equivalent) was obese, at 32.15 ± 9.1 kg/m(2) , compared with 25.91 ± 7.3 kg/m(2) for those on no or lower doses of diuretics (P = .009). Eighteen of the 79 patients met an endpoint of death, hospice placement, or cardiac transplant by the study conclusion. Kaplan-Meier analysis from time of first appointment until recent follow-up revealed a significant association between time to combined endpoint and BMI class. Cox proportional hazard modeling with age adjustment yielded a hazard ratio of 3.2 (95% CI 1.096-9.379) for obesity upon first presentation to an adult CHD clinic. CONCLUSIONS: In patients with univentricular hearts and Fontan palliation, obesity is associated with symptomatic heart failure and mortality.

Dynamic Evaluation of Coronary Anomalies Originating from the Opposite Sinus of Valsalva (ACAOS).

OPINION STATEMENT: Coronary anomalies originating from the opposite sinus of Valsalva (ACAOS) are a rare anomaly associated with sudden cardiac death. Dynamic, invasive evaluation using coronary angiography, intravascular ultrasound, and fractional flow reserve can more clearly identify important pathophysiologic variants and guide treatment. This dynamic evaluation can assist the clinician in the appropriate surgical and percutaneous treatment options and aid in patient counseling. Long-term outcomes data regarding treatment and prognosis is still lacking.

Contraceptive Use and Unintended Pregnancy in Women With Congenital Heart Disease.

OBJECTIVE: To identify patterns of contraceptive use and pregnancy in an academic adult congenital cardiology practice. METHODS: In this cross-sectional study, from October 2013 through March 2014, 100 women with congenital heart disease aged 18-45 years were recruited from an academic congenital heart disease clinic and administered a survey regarding pregnancy history, contraception use, and understanding of pregnancy-related and contraceptive-related risk. The primary outcome was current use of long-acting reversible contraception, including intrauterine devices or subdermal implants. RESULTS: Of 83 sexually active women, 63 (75.9%, 95% confidence interval [CI] 65.3-85.1) reported currently using any contraceptive method, including 30 of 83 (36.1%, 95% CI 25.9-47.4) using tier I methods (typical-use failure rates of less than 1% per year) and 20 of 83 (24.1%, 95% CI 15.4-34.7) using tier II methods (typical-use failure rates of 6-12% per year). Nine of 83 (10.8%, 95% CI 5.1-19.6) reported currently using long-acting reversible contraception. Sixty-four of 141 total pregnancies (45.4%, 95% CI 31.9-58.9) were self-reported by participants as "unexpected" rather than "planned." Only one (1.6%, 95% CI 0-4.6) of the 64 unintended pregnancies occurred when the woman was using a tier I method of contraception at the time of conception. CONCLUSION: Most women with congenital heart disease of childbearing age are sexually active. The high incidence of unintended pregnancy in this group may be related to underuse of highly effective methods of contraception. Specific counseling on tier I methods may reduce unintended pregnancies in women with congenital heart disease. LEVEL OF EVIDENCE: III.

Segmental analysis of congenital heart disease: putting the "puzzle" together with computed tomography.

Advances in surgical and medical treatment for congenital heart disease have resulted in greater life expectancy. As a result, there has been an increase in the utilization of cross-sectional imaging for diagnosis and management of complex congenital heart disease. This manuscript describes a morphological and sequential segmental approach to deciphering the code of complex congenital heart defects in cross-sectional imaging, mostly computed tomography. This manuscript will review approaches to differentiate types of transposition, the anatomic relationships of cardiac structures, and the application of these relationships in the description of complex congenital heart disease.

Cardiovascular disease in late survivors of tetralogy of fallot: a tertiary care center experience.

Patients with tetralogy of Fallot can survive to late adulthood; however, there are few data on cardiovascular outcomes in this population. We conducted a single-center retrospective analysis of cardiovascular outcomes and risk factors in 208 patients with tetralogy of Fallot to better evaluate the burden of cardiovascular disease in this group. Descriptive statistics were used to determine the prevalence of relevant cardiovascular risk factors and outcomes, including a composite analysis of cardiovascular disease. Rates and mean values from the American Heart Association 2011 Heart Disease and Stroke Statistics Update were used as population estimates for comparison. In tetralogy of Fallot patients, cardiovascular disease prevalence was not different from that found in the general population (40% vs. 36%, P=0.3). However, there was significantly more cardiovascular disease in tetralogy of Fallot men aged 20 to 39 years (30% vs. 14%, P < 0.05) and in tetralogy of Fallot men aged 40 to 59 years (63% vs. 29%, P < 0.0001). This was due to higher prevalence of coronary disease (12% vs. 7%, P < 0.05) and heart failure (16% vs. 2%, P < 0.0001). In particular, the increased prevalence of heart failure (regardless of pulmonary valve disease) accounts for the frequency of cardiovascular disease in tetralogy of Fallot men aged 20 to 59 years. These data support the need to routinely screen young adult male survivors of tetralogy of Fallot for asymptomatic heart failure. Further studies are needed to determine the incidence, severity, and long-term effects of cardiovascular disease in the adult congenital heart disease population.

Usefulness of medical therapy for pulmonary hypertension and delayed atrial septal defect closure.

A subset of adult patients with an open atrial septal defect (ASD) have pulmonary arterial hypertension (PAH). We sought to identify predictors of response to PAH-specific medical therapy in this group. Invasive hemodynamic and clinical parameters from 12 patients with an open ASD and PAH (pulmonary vascular resistance [PVR], 8.8 ± 1.2 Wood units; mean pulmonary artery pressure, 55 ± 6 mm Hg; Qp:Qs ratio, 1.1 ± 0.1; and 6-minute walk test distance of 1,046 ± 116 feet) were analyzed. Responders (n = 5) underwent successful ASD closure at 1.3 ± 0.3 years after initiation of medical therapy and were characterized by >30% reduction in PVR (7.2 ± 1.5 to 4.6 ± 0.9 Wood units) versus <20% in nonresponders (n = 7; 9.9 ± 1.7 to 8.2 ± 1.5 Wood units, p <0.03), increased 6-minute walk test distance (1,087 ± 174 vs 1,405 ± 109 feet, p = 0.05), and higher Qp:Qs ratio after therapy (1.9 ± 0.2 vs 1.1 ± 0.2, p <0.02). Body mass index was a significant clinical predictor of response (23.3 ± 1.9 vs 30.0 ± 2.1 kg/m(2), p <0.05) and the change in arterial saturation with exercise correlated inversely with change in PVR (r = -0.739, p <0.01). In conclusion, medical therapy led to a significant improvement in hemodynamic and clinical parameters in a subset of patients with an open ASD and PAH, who were able to safely undergo delayed ASD closure.

Massive global right ventricular hypertrophy with both fixed and dynamic obstruction and pulmonary valve dysplasia.

Congenital pulmonary valvular dysplasia is an unusual condition typically associated with Noonan syndrome. Among its other cardiac manifestations, occasional patients with Noonan syndrome have been demonstrated to have hypertrophic nonobstructive cardiomyopathy, which may be biventricular. We report a unique case of pulmonary valvular dysplasia, dynamic right ventricular outflow tract obstruction and profound right ventricular hypertrophy with only mild left ventricular hypertrophy, in a patient without Noonan syndrome.

Inferior type sinus venosus atrial septal defect: MR findings.

Atrial septal defects can occur at various levels of the interatrial septum. Sinus venosus atrial septal defect (SVASD) results from abnormal resorption of the embryologic sinus venosus, and may be of the superior or inferior type. In this paper, we describe a 46-year-old man with inferior-type SVASD who presented with arrhythmias and dyspnea. Cardiac magnetic resonance and cardiac catheterization were useful in evaluating the anatomic/functional characteristics of inferior-type SVASD and distinguishing it from unroofed coronary sinus.

Role of protein binding in renal elimination of leptin.

OBJECTIVE: Leptin, a hormone produced by fat which signals to the brain the extent of fat stores, is known to be eliminated from circulation primarily by the kidney. The hormone circulates in both free and protein-bound forms, but there is little information concerning the inter-relationship of these forms of leptin, or which form is influenced by physiological processes such as renal elimination. We studied total, free and bound concentrations of leptin in ambulatory adults undergoing catheterization for diagnosis/management of congenital cardiac disease. DESIGN: Blood specimens were collected from both the arterial circulation and the renal vein, for determination of the fractional extraction of leptin resulting from a single pass through the kidney. PATIENTS: Thirteen subjects were studied. MEASUREMENTS: Total leptin concentrations were measured by radioimmunoassay, and free/protein-bound leptin concentrations were quantified by an high-performance liquid chromatography method. Adequacy of renal vein sampling was assessed by comparing the creatinine concentration of arterial and venous specimens. RESULTS: Mean fractional extraction of creatinine was 28 +/- 7% in the 13 subjects studied. Fractional extraction of total leptin was 18 +/- 8%, significantly less than that for creatinine. Fractional extraction of total leptin was not related to arterial leptin concentration or the fractional extraction of creatinine. Both free and bound fractions of leptin were significantly reduced by passage through the kidney, with fractional extractions of 22% and 25%, respectively. Efficiency of extraction was not influenced by the relative proportion of free or bound leptin fractions. Leptin-binding capacity (a measure of the concentrations of leptin-binding proteins) was not altered by passage through the kidney. CONCLUSIONS: Both free and bound leptin are metabolically active with regard to elimination. Protein-bound leptin equilibrates with the free leptin fraction in circulation as the result of a dynamic equilibrium. The data are consistent with either glomerular filtration or active uptake as mechanisms of elimination. Leptin-binding proteins are apparently neither eliminated or produced by the kidney.