Long‐term follow‐up of adult patients with congenital heart disease and an implantable cardioverter defibrillator

Objective: Sudden cardiac death is common in the adult congenital heart disease (ACHD) population. Knowledge and experience about the use of implantable cardio‐ verter defibrillators (ICD) in ACHD patients is very limited. We aimed to characterize a cohort of patients with ACHD and ICDs.
Design: Thirty consecutive ACHD patients submitted to an ICD implantation in a single tertiary center were evaluated. Data on baseline clinical features, heart defect, indication for ICD, type of device, appropriate therapies, ICD‐related complication, and mortality during follow‐up were collected.
Results: Of the 30 patients, 56.7% received appropriate therapies due to ventricular tachycardia (VT) or ventricular fibrillation (VF). The rate of inappropriate therapies and device‐related complications was 33.3%. Secondary prevention and primary pre‐ vention patients with class I indications for ICD had more appropriate therapies than complication, but this relationship was reversed for patients with class II indications. Remote monitoring played an important role in diagnosing new atrial arrhythmias be‐ fore scheduled visits in 46.2% of patients, leading to a change in medication. VT/VF episodes were associated with a composite of death, cardiac transplantation, and hos‐ pital admission (OR 13.0; 95% CI: 2.1‐81.5).
Conclusion: ICDs are not only useful in preventing SCD, but also have a major role in diagnosing atrial tachyarrhythmias ahead of scheduled visits. Although improve‐ ments in ICD technology might reduce complications and inappropriate therapies, adequate selection of candidates for primary prevention still remains difficult be‐ cause of the lack of clear indications.     

Prolonged Tpeak‐Tend interval is a risk factor for sudden cardiac death in adults with congenital heart disease

Objective: Adult congenital heart disease (ACHD) patients are at risk of sudden cardiac death (SCD). However, methods for risk stratification are not yet well‐ defined. The T_peak‐T_end (TpTe) interval, a measure of dispersion of ventricular repolari‐ zation, is a risk factor for SCD in non‐ACHD patients. We aim to evaluate whether TpTe can be used in risk stratification for SCD in ACHD patients.
Design: From an international multicenter cohort of 25 790 ACHD patients, we iden‐ tified all SCD cases. Cases were matched to controls by age, gender, congenital de‐ fect, and (surgical) intervention.
Outcome Measures: TpTe was measured on a standard 12‐lead ECG. The maximum TpTe of all ECG leads (TpTe‐max), mean (TpTe‐mean), and TpTe dispersion (maximum minus minimum) were obtained. Odds ratios (OR) for SCD cases vs controls were calculated using conditional logistic regression analysis.
Results: ECGs were available for 147 cases (median age at death 33.5 years (quartiles 26.2, 48.7), 66% male) and 267 controls. The mean TpTe‐max was 97 ± 24 ms in cases vs 84 ± 17 ms in controls (P < .001); TpTe‐mean was 70 ± 16 vs 63 ± 10 ms (P < .001); and dispersion was 51 ± 22 ms vs 41 ± 16 ms (P = .02), respectively. Assessing each ECG lead separately, TpTe in lead aVR predicted SCD most accurately. TpTe in lead aVR was 71 ± 23 ms in cases vs 61 ± 13 ms in controls (P < .001). After adjusting for impaired ventricular function, heart failure symptoms, and prolonged QRS duration, the OR of SCD of TpTe in lead aVR at an optimal cutoff of 80 ms was 5.8 (95% CI 2.7‐12.4, P < .001).
Conclusions: The TpTe interval is associated with SCD in ACHD patients. Particularly, TpTe in lead aVR can be used as an independent risk factor for SCD in ACHD patients and may, therefore, add precision to current risk prediction models.     

Extended cardiac ambulatory rhythm monitoring in adults with congenital heart disease: Arrhythmia detection and impact of extended monitoring

Background: Arrhythmias are a leading cause of death in adults with congenital heart disease (ACHD). While 24‐48‐hour monitors are often used to assess arrhythmia burden, extended continuous ambulatory rhythm monitors (ECAM) can record 2 weeks of data. The utility of this device and the arrhythmia burden identified beyond 48‐hour monitoring have not been evaluated in the ACHD population. Additionally, the impact of ECAM has not been studied to determine management recommendations.
Objective: To address the preliminary question, we hypothesized that clinically sig‐ nificant arrhythmias would be detected on ECAM beyond 48 hours and this would lead to clinical management changes.
Methods: A single center retrospective cohort study of ACHD patients undergoing ECAM from June 2013 to May 2016 was performed. The number and type of ar‐ rhythmias detected within and beyond the first 48 hours of monitoring were com‐ pared using Kaplan‐Meier curves and Cox proportional hazard models.
Results: Three hundred fourteen patients had monitors performed [median age 31 (IQR 25‐41) years, 61% female). Significant arrhythmias were identified in 156 pa‐ tients (50%), of which 46% were noted within 48 hours. A management change based on an arrhythmia was made in 49 patients (16%).
Conclusions: ECAM detects more clinically significant arrhythmias than standard 48‐ hour monitoring in ACHD patients. Management changes, including medication changes, further testing or imaging, and procedures, were made based on results of ECAM. Recommendations and guidelines have been made based on arrhythmias on 48‐hour monitoring; the predictive ability and clinical consequence of arrhythmias found on ECAM are not yet known.     

Body mass index in adults with congenital heart disease

Objective: To investigate the status of body mass index (BMI) in adult people with congenital heart disease (ACHD).
Methods: Five hundred thirty‐nine adults with CHD (53.8% men) were seen in the outpatient clinic from 2013 to 2015 and compared to a reference population (n = 1737). The severity of CHD was categorized as mild, moderate, and severe ac‐ cording to standard guidelines. Patients were categorized based on BMI as under‐ weight (<18.5), overweight (25‐30), or obese (>30). Echocardiography and magnetic resonance imaging were used to measure ventricular function while exercise capac‐ ity was estimated via cardiopulmonary exercise test.
Results: Adults with CHD had slightly lower BMI than the reference group (24.1 ± 4.3 vs 24.6 ± 4.3; P = .012). Men in the mild and severe group (23.9 ± 3.6; 23.3 ± 4.4 vs 25.1 ± 3.7; P = .007; P = .023) and women in the severe group (21.6 ± 3.3 vs 24.2 ± 4.7; P < .001) had lower BMI compared to the reference group. In the subgroups, men with ventricular septal defect, coarctation of aorta/ventricular septal defect and Fontan cir‐ culation and women with Fontan circulation had lower BMI than the reference group. Underweight was more prevalent in women with severe lesions compared to the refer‐ ence group (22.2% vs 3.8%; P < .001). BMI was associated with age and exercise ca‐ pacity in patients with mild and moderate lesions, while higher BMI was related to better ventricular function in women with Fontan circulation.
Conclusion: Underweight was more prevalent in ACHD patients with severe lesions. Special attention should be paid to the possible existence of underweight‐related comorbidities.     

Detection of arrhythmias in adult congenital heart disease patients with LINQTM implantable loop recorder

Background: Rhythm disorders are the leading cause of morbidity and mortality in adults with congenital heart disease (ACHD). Infrequent or asymptomatic arrhyth‐ mias may not be detected by routine monitoring. Implantable loop recorders (ILRs), such as the Reveal LINQ^TM, have been useful in long‐term monitoring for arrhythmias in adults with cryptogenic stroke.
Objective: We propose the Reveal LINQ^TM will detect arrhythmias, not documented by other monitoring modalities, resulting in change in management in ACHD patients.
Methods: This is a single center retrospective review of Reveal LINQ^TM use in ACHD patients from 2014‐2017. Medical records were reviewed to determine cardiac diag‐ nosis, indication for implant, ILR findings, and changes in management.
Results: Twenty‐two patients, median age 25 years, underwent ILR implantation. ILR findings resulted in change in management in nine (41%) patients. One‐third (3/9) of the patients with clinically relevant events were asymptomatic. Patients with Fontan palliation had the highest number of pertinent positive events (57%). ACHD physi‐ ologic class D patients were more likely to have a positive finding (P = .034) compared to other physiologic classes. Majority (75%) of patients with positive events had ar‐ rhythmias documented on ILR which were not demonstrated on prior Holter/event monitors. Pertinent negative event occurred in one patient with Fontan palliation (5%) who had syncope corresponding to sinus rhythm.
Conclusion: ILRs are a useful adjunct for arrhythmia monitoring in the ACHD popula‐ tion with clinically relevant events in 41% of patients. A special consideration for ILRs could be made for high‐risk asymptomatic patients.     

Impact of durable ventricular assist devices on post‐transplant outcomes in adults with congenital heart disease

Background: There are no published data on post‐transplant outcomes in durable ven‐ tricular assist device (VAD)‐supported adult congenital heart disease (ACHD) patients.
Methods: We compared post‐transplant outcomes in VAD‐supported vs non‐VAD‐ supported ACHD patients using the Scientific Registry of Transplant Recipients.
Results: At 1 year, there was no difference in post‐transplant mortality between VAD‐supported (12 patients) and non‐VAD‐supported (671 patients) ACHD patients.
Conclusions: In appropriate ACHD patients, VAD use as a bridge to transplant is a reasonable strategy.     

Metabolic syndrome in adults with congenital heart disease and increased intima‐media thickness

Aims: Age‐related cardiovascular diseases are a relevant risk in the aging population of adults with congenital heart diseases (ACHD). Risk factors such as the metabolic syndrome (MetS) impact the risk of increased carotid intima‐media thickness (cIMT) and thereby long‐term cardiovascular diseases. The aim of the study was to assess MetS in ACHD and outline a possible association to cIMT.
Methods and Results: In total, 512 ACHD (43.0 ± 9.6 years, 48.9% female) were screened for MetS by the standards of the International Diabetes Federation, and their cIMT by ultrasound from January 2017 to June 2019. MetS was prevalent in 72 (14.1%) of the ACHD population (34 female, 15.5%). Regarding severity class, pa‐ tients with simple forms of CHD had a MetS prevalence of 11.8%, moderate 16.7%, and severe 13.8%. ACHD with MetS had significantly increased cIMT compared to ACHD without MetS (ACHD with MetS: 0.587 ± 0.079 mm, ACHD without MetS: 0.560 ± 0.087 mm, mean difference: 0.028 mm, P = .013). Such a difference in vas‐ cular structure corresponds to roughly five years of normal vascular aging of the vessels.
Conclusion: ACHD with MetS have a thicker cIMT compared to ACHD without MetS. Screening for MetS and targeting risk factors in ACHD might help to prevent struc‐ tural alterations of the vessels at an early stage.     

Adverse effects of amiodarone therapy in adults with congenital heart disease

Objective: Amiodarone is a highly effective antiarrhythmic therapy, however its tox‐ icity profile often limits treatment. This is particularly relevant in adults with congeni‐ tal heart disease (CHD), who are often young and in whom other antiarrhythmic agents commonly fail or are contraindicated. We sought to determine incidence and predictors of adverse effects caused by amiodarone in adult CHD (ACHD).
Design: A retrospective review of patients with moderate to complex ACHD treated with amiodarone at our center between 2000 and 2017 was performed. Incidence and predictors of adverse effects were described. Efficacy of amiodarone therapy in controlling the clinical arrhythmia was assessed as complete, partial, or failed.
Results: Amiodarone was prescribed in 57 patients of 902 ACHD patients reviewed (6%), for a mean duration of 2.7 ± 4.3 years. Significant adverse effects occurred in 56%, most commonly thyroid dysfunction, with amiodarone‐induced thyrotoxicosis (AIT) in 30% and amiodarone‐induced hypothyroidism in 14%. AIT frequently led to arrhythmia exacerbation and occurred most in those with Fontan anatomy. Severe dermatological effects were seen in 7% and bradycardia requiring pacing in 5%. Interstitial lung disease, peripheral neuropathy and alopecia were observed in single cases. Amiodarone toxicity led to discontinuation of the drug in 42%. Amiodarone was highly effective when tolerated, however, achieving complete arrhythmia con‐ trol in 63%, partial control in 35%, with failure to control in only one patient.
Conclusions: Amiodarone therapy is effective in moderate to complex ACHD pa‐ tients, but is frequently limited by adverse effects. ACHD patients seem especially vulnerable to thyroid dysfunction, with Fontan patients in particular at increased risk of AIT.     

Vasopressor magnitude predicts poor outcome in adults with congenital heart disease after cardiac surgery

Background: High levels of vasoactive inotrope support (VIS) after congenital heart surgery are predictive of morbidity in pediatric patients. We sought to discern if this relationship applies to adults with congenital heart disease (ACHD).
Methods: We retrospectively studied adult patients (≥18 years old) admitted to the intensive care unit after cardiac surgery for congenital heart disease from 2002 to 2013 at Mayo Clinic. Vasoactive medication dose values within 96 hours of admis‐ sion were examined to determine the relationship between VIS score and poor out‐ come of early mortality, early morbidity, or complication related morbidity.
Results: Overall, 1040 ACHD patients had cardiac surgery during the study time frame; 243 (23.4%) met study inclusion criteria. Sixty‐two patients (25%), experi‐ enced composite poor outcome [including eight deaths within 90 days of hospital discharge (3%)]. Thirty‐eight patients (15%) endured complication related early mor‐ bidity. The maximum VIS (maxVIS) score area under the curve was 0.92 (95% CI: 0.86‐0.98) for in‐hospital mortality; and 0.82 (95% CI: 0.76‐0.89) for combined poor clinical outcome. On univariate analysis, maxVIS score ≥3 was predictive of compos‐ ite adverse outcome (OR: 14.2, 95% CI: 7.2‐28.2; P < 0.001), prolonged ICU LOS ICU LOS (OR: 19.2; 95% CI: 8.7‐42.1; P < 0.0001), prolonged mechanical ventilation (OR: 13.6; 95% CI: 4.4‐41.8; P < 0.0001) and complication related morbidity (OR: 7.3; 95% CI: 3.4‐15.5; P < 0.0001).
Conclusions: MaxVIS score strongly predicted adverse outcomes and can be used as a risk prediction tool to facilitate early intervention that may improve outcome and assist with clinical decision making for ACHD patients after cardiac surgery.     

Pulsatile Glenn as long‐term palliation for single ventricle physiology patients

Objective: There are limited studies analyzing pulsatile Glenn as a long‐term pallia‐ tion strategy for single ventricle patients. This study sought to determine their out‐ comes at a single institution.
Design: A retrospective review was performed.
Setting: Study performed at a single pediatric hospital.
Patients: All single ventricle patients who underwent pulsatile Glenn from 1995 to 2016 were included.
Outcome measures: Pulsatile Glenn failure was defined as takedown, transplant, or death. Further palliation was defined as Fontan, 1.5, or biventricular repair. Risk fac‐ tors were assessed by Cox multivariable competing risk analyses.
Results: Seventy‐eight patients underwent pulsatile Glenn at age 9 months (inter‐ quartile range, 5‐14). In total, 28% had heterotaxy, 18% had a genetic syndrome, and 24% had an abnormal inferior vena cava. There were 3 (4%) perioperative mortalities. Further palliation was performed in 41 (53%) patients with a median time‐to‐pallia‐ tion of 4 years (interquartile range, 3‐5). Pulsatile Glenn failure occurred in 10 (13%) patients with 8 total mortalities. Five‐ and 10‐year transplant‐free survival were 91% and 84%, respectively. At a median follow‐up of 6 years (interquartile range, 2‐8), 27 patients (35%) remained with PG (age 7 years [interquartile range, 3‐11], oxygen sat‐ uration 83% ± 4%). Preoperative moderate‐severe atrioventricular valve regurgita‐ tion (AVVR) (hazard ratio 7.77; 95% confidence interval 1.80‐33.43; P =.005) and higher pulmonary vascular resistance (hazard ratio 2.59; 95% confidence interval 1.08‐6.15; P =.031) were predictors of pulsatile Glenn failure after adjusting for co‐ variates. Reaching further palliation was less likely in patients with preoperative moderate‐severe AVVR (hazard ratio 0.22, 95% confidence interval 0.08‐0.59; P =.002).
Conclusion: Pulsatile Glenn can be an effective tool to be used in challenging circum‐ stances, these patients can have a favorable long‐term prognosis without reducing their suitability for further palliation.     

The role of echocardiography for quantitative assessment of right ventricular size and function in adults with repaired tetralogy of Fallot

Background: Quantitative assessment of right ventricular (RV) systolic function by echocardiography is challenging in patients with congenital heart disease because of the complex geometry of the RV and the iatrogenic structural abnormalities resulting from prior cardiac surgeries. The purpose of this study was to determine the correla‐ tion between echocardiographic indices of RV systolic function and cardiac magnetic resonance imaging (CMRI) derived RV ejection fraction (RVEF) in adults with repaired tetralogy of Fallot (TOF).
Methods: Quantitative assessment of RV function was performed with RV tissue Doppler systolic velocity (RV s'), tricuspid annular plane systolic excursion (TAPSE), and fractional area change (FAC). These echocardiographic indices were compared to RVEF from CMRI performed on the same day as echocardiogram.
Results: Of 209 patients, the mean RV FAC was 39 ± 9%, TAPSE was 18 ± 4 mm, RV s' was 10 ± 2 cm/s, and RVEF was 40 ± 10%. There was a good correlation be‐ tween TAPSE and RVEF (r = 0.79, P < .001), good correlation between RV s' and RVEF (r = 0.71, P < .001), and modest correlation between FAC and RVEF (r = 0.66, P < .001). TAPSE < 17 mm effectively discriminated between patients with RV systolic dysfunc‐ tion defined as RVEF < 47% (sensitivity 81%, specificity 79%, area under the curve [AUC] 0.805). FAC < 40% was associated with RVEF < 47% (sensitivity 72%, specificity 63%, AUC 0.719). RV s' < 11 cm was associated with RVEF < 47% (sensitivity 83%, specificity 68%, AUC 0.798).
Conclusion: Despite the structural and functional abnormalities of the RV in patients with repaired TOF, quantitative assessment of RV systolic function by echocardiog‐ raphy is feasible and had good correlation with CMRI‐derived RVEF.     

Identifying self‐reported neurocognitive deficits in the adult with congenital heart disease using a simple screening tool

Objective: Children with congenital heart disease (CHD) and adults with acquired heart disease are at an increased risk of neurocognitive impairment. The objective of this study was to determine the prevalence of self‐reported neurocognitive impair‐ ment and its risk factors in the adult congenital heart disease (ACHD) population.
Design: The Wisconsin Adult Congenital Heart Disease Program recently began screening ACHD patients to identify those with significant self‐perceived neurocog‐ nitive impairments. Screening consists of using a validated neuro‐oncology screening instrument that has been modified for the ACHD population. Patients who answer this survey in a predetermined fashion consistent with significant self‐perceived neu‐ rocognitive deficits are referred for a formal neurocognitive evaluation. Demographic and clinical information are obtained by chart review.
Results: Three hundred ten patients (49% males) completed the screening process. The average age was 30 years (range: 17–69 years). For the cohort, 57 (18%) patients had no prior cardiac surgeries, 85 (28%) one surgery, 77 (25%) two, and 91 (29%) at least three surgeries. Of those screened, 106 (34%) met criteria for a formal neuro‐ cognitive evaluation. Patients who were referred had undergone a greater number of prior cardiac surgeries (2.2 vs 1.7, P = .008) and were more likely to have severe com‐ plexity CHD (P = .006). Of those patients who were referred, the worst perceived functioning was in math and attention.
Conclusion: There is a high prevalence of ACHD patients with significant self‐per‐ ceived neurocognitive deficits. Simple screening questionnaires may help identify those patients at high risk and allow for timely and appropriate referral for formal neurocognitive evaluation, diagnosis, and therapy.     

Ventricular force‐frequency relationships during biventricular or multisite pacing in congenital heart disease

Background: Traditional indices to evaluate biventricular (BiV) pacing are load dependent, fail to assess dynamic changes, and may not be appropriate in patients with congenital heart disease (CHD). We therefore measured the force‐frequency relationship (FFR) using tissue Doppler‐de‐ rived isovolumic acceleration (IVA) to assess the dynamic adaption of the myocardium and its vari‐ ability with different ventricular pacing strategies.
Methods: This was a prospective pilot study of pediatric and young adult CHD patients with biventricular or multisite pacing systems. Color‐coded myocardial velocities were recorded at the base of the systemic ventricular free wall. IVA was calculated at resting heart rate and with incremental pacing. FFR curves were obtained by plotting IVA against heart rate for different ventricular pacing strategies.
Results: Ten patients were included (mean: 22 ± 7 years). The FFR identified a best and worst ventricular pacing strategy for each patient, based on the AUC at baseline, submaximal, and peak heart rates (P < .001). However, there was no single best ventricular pacing strategy that was optimal for all patients. Additionally, the best ventricular pacing strategy often differed within the same patient at different heart rates.
Conclusion: This novel assessment demonstrates a wide variability in optimal ventricular pac‐ ing strategy. These inherent differences may play a role in the unpredictable clinical response to BiV pacing in CHD, and emphasizes an individualized approach. Furthermore, the optimal ventricular pacing varies with heart rate within individuals, suggesting that rate‐responsive ventricular pacing modulation may be required to optimize ventricular performance.     

Predictors and rates of recurrence of atrial arrhythmias following catheter ablation in adults with congenital heart disease

Background: Catheter ablation is commonly performed to treat atrial arrhythmias in adult congenital heart disease (ACHD). Despite the frequency of ablations in the ACHD population, predictors of recurrence remain poorly defined.
Objective: We sought to determine predictors of arrhythmia recurrence in ACHD patients following catheter ablation for atrial arrhythmias.
Methods: We performed a retrospective study of all catheter ablations for atrial arrhythmias performed in ACHD patients between January 12, 2005 and February 11, 2015 at our institution. Prespecified exposures of interest and time from ablation to recurrence were determined via chart review.
Results: Among 124 patients (mean age: 45 years) who underwent catheter ablation, 96 (77%) were treated for macro‐reentrant atrial tachycardia, 10 (7%) for focal atrial tachycardia, 9 (7%) for atrial fibrillation, 7 (6%) for atrioventricular nodal reentrant tachycardia, and 2 (2%) for atrioventricular reentrant tachycardia. 15 (12%) required transseptal/transbaffle puncture. Fifty‐one percent of patients recurred with a median time to recurrence of 1639 days. By univariate and multivariable analysis, body mass index (BMI) and Fontan status were the only variables associated with recurrence. Dose‐dependent effect was observed with overweight (HR = 2.37, P = .012), obese (HR = 2.67, P = .009), and morbidly obese (HR = 4.23, P = .003) patients demonstrating an increasing risk for arrhythmia recurrence postablation. There was no significant different in recurrence rates by gender, age, non‐Fontan diagnosis, or need for transseptal puncture.
Conclusions: In our cohort of ACHD patients, BMI was a significant risk factor for arrhythmia recurrence postablation, independent of Fontan status. These findings may help guide treatment decisions for persistent arrhythmias in the ACHD population.     

Achieving biventricular circulation in patients with moderate hypoplastic right ventricle in pulmonary atresia intact ventricular septum after transcatheter pulmonary valve perforation

Objective: Transcatheter valve perforation for pulmonary atresia intact ventricular septum is the standard of care for patients with mild right ventricular hypoplasia. However, its role in moderate right ventricular hypoplasia has been less well defined. We sought to report the long‐term outcome of patients with moderate hypoplastic right ventricle who had undergone the procedure.
Design, Settings, and Patients: We performed a retrospective analysis on patients who had undergone transcatheter pulmonary valve perforation from January 1996 to January 2015 at our institution. The procedures would be carried out irrespective of the right ventricular size, as long as there were no absolute contraindications. Intervention and Outcome Measures: Demographic and procedural data were cor‐ related with outcome measures. Outcomes analyzed included procedural success, reintervention rates, final circulation type, and functional class. Multivariate analysis and receiver operator curve were used to identify for parameters in predicting biven‐ tricular circulation.
Results: The procedural success rate was 92% (33 out of 36) in this group with mod‐ erate right ventricular hypoplasia (tricuspid valve z score −4.2 ± 3.0, 69.4% of pa‐ tients with z score <−2.5). Early reintervention rate was 39%, mostly being insertion of modified Blalock–Taussig shunt. Overall reintervention‐free survival was 53%, 30%, and 19% at 1, 6, and 12 months postintervention. Despite no significant catch‐ up right ventricular growth, majority of survivors (84%) enjoyed a biventricular circu‐ lation with good functional status. A tricuspid to mitral valve ratio >0.79 was a good predictor of biventricular outcome. (specificity of 100%, positive predictive value 100%).
Conclusion: Encouraging long‐term results with biventricular circulation and func‐ tional status were demonstrated with transcatheter pulmonary valve perforation in patients even with moderate hypoplastic right ventricle, which is comparable to that with mild right ventricular hypertrophy. The baseline tricuspid to mitral valve ratio was identified as a potentially useful tool in predicting biventricular circulation.     

Do we have the ACHD physician resources we need to care for the burgeoning ACHD population?

Background: Delivery of care to the adult congenital heart disease (ACHD) popula‐ tion has been limited by a shortage in the ACHD physician resources. There is limited data regarding the adequacy of the ACHD physician resources in the United States and our population estimates are extrapolated from Canadian data. Therefore, we proposed to evaluate the adequacy of ACHD physician: patient ratios in the United States at both national and regional levels.
Methods: Data from the Adult Congenital Heart Association (ACHA) website along with metropolitan area and statewide population data from 2016 US Census Bureau estimates were analyzed. Physicians listed on the ACHA website were cross‐refer‐ enced with ABIM to verify ACHD board certification status.
Results: There are 115 self‐identified ACHD programs and 418 self‐identified ACHD physicians listed in the ACHA website. There are 320 board‐certified ACHD cardiolo‐ gists in the United States today, including 161 not listed in the ACHA website. Regarding ratios of ACHD‐certified physicians to patients, the best served metro‐ politan statistical area (MSA) is Raleigh‐Cary, NC, and the worst served MSA is Riverside‐San Bernardino‐Ontario, CA. The best served State is Washington, DC, and the worst served State is Indiana.
Conclusions: The ACHD population continues to grow, and the looming national phy‐ sician shortage is likely to greatly affect the ability to meet the complex needs of this growing population. In order to bring the ACHD patient: physician ratio to 1000:1, a minimum of 170 additional ACHD board‐certified physicians are needed now.     

Perspectives on advance care planning and palliative care among adults with congenital heart disease

Background: Patients with adult congenital heart disease (ACHD) report that ad‐ vance care planning (ACP) is important, and that they want information about prog‐ nosis. However, recognizing importance and being willing to participate are different constructs, and how and when to begin ACP and palliative care discussions remains ill‐defined.
Methods: We conducted a cross‐sectional survey of 150 consecutive outpatients to assess willingness to participate in ACP, with whom, and important barriers and facili‐ tators to these discussions.
Results: The majority of participants (69%) reported being willing to participate in ACP; 79% to have a meeting to discuss goals and care preferences; and 91% to speak to a clinician who specializes in palliative care. Being married and anticipating a shorter lifespan were associated with increased reported willingness to participate in ACP. The health care provider with whom most participants preferred to have these discussions was their ACHD clinician. Participants identified important barriers and facilitators to these discussions.
Conclusion: Patients with ACHD report being willing to participate in ACP and pallia‐ tive care discussions. Patients prefer to have these discussions with their ACHD clini‐ cians, thus ACHD clinicians need to be prepared to address these issues as part of routine care.     

Atrial septal defect in adults is associated with airway hyperresponsiveness

Objective: The association between secundum atrial septal defects (ASD) and asthma‐ like dyspnea with consequent long‐term pulmonary inhalant use, is poorly understood in adult ASD patients. Airway hyperresponsiveness is suggested to be the underlying mechanism of cardiac asthma from mitral valve disease and ischemic cardiomyopathy. We hypothesized that airway hyperresponsiveness may also be found in adult ASD patients. Our aim was to study airway responsiveness in adult ASD patients before percutaneous closure and at short‐and long‐term postprocedural follow‐up.
Methods: This prospective study included 31 ASD patients (65% female, mean age 49 ± 15y) who underwent spirometry and bronchoprovocation testing pre‐and six‐ month postprocedurally, with additional bronchoprovocation at 2‐year follow‐up. Airway hyperresponsiveness was defined as ≥20% fall of forced expiratory volume in 1‐second (FEV1) following <8.0 mg/mL of inhaled methacholine.
Results: Airway hyperresponsiveness was found in 19/30 patients (63%[95%CI 45%‐81%]; post hoc statistical power = 89%). Asthma‐like symptoms wheezing, chest tightness, and cough were more frequently reported in airway hyperresponsive pa‐ tients. Airway responsiveness was not influenced by successful percutaneous ASD closure, corresponding to persistence of asthma‐like symptoms postclosure. Regardless of airway responsiveness, postprocedural right‐sided reverse remodeling significantly improved dyspnea and pulmonary function.
Conclusions: This study is the firsttoreport ahighprevalenceof airwayhyperresponsiveness in a cohort of unrepaired adult ASD patients, and confirms the association between asthma‐ like symptoms and ASD in adults. Attention to symptoms and pulmonary function should be given during clinical follow‐up of adult ASD patients, both before and long afterrepair.     

Right ventricular and pulmonary vascular function indices for risk stratification of patients with pulmonary regurgitation

Background: We hypothesized that echocardiographic indices of right ventricular to pulmonary artery (RV‐PA) coupling were comparable to cardiac magnetic resonance imaging (CMRI)‐derived RV volumetric indices in predicting disease severity in chronic pulmonary regurgitation (PR).
Methods: Patients with ≥ moderate PR (2003‐2015) with and without prior CMRI scans were enrolled into the study cohort and validation cohort, respectively. Endpoint was to determine the association between noninvasive RV‐PA coupling in‐ dices (tricuspid annular plane systolic excursion/right ventricular systolic pressure [TAPSE/RVSP] and fractional area change [FAC]/RVSP ratio) and markers of disease severity, and compared this association to that of CMRI‐derived RV volumetric indi‐ ces and markers of disease severity (peak oxygen consumption [VO2], NT‐proBNP and atrial and/or ventricular arrhythmias).
Results: Of the 256 patients in the study cohort (age 33 ± 6 years), 187 (73%) had tetralogy of Fallot (TOF) while 69 (27%) had valvular pulmonic stenosis (VPS). TAPSE/ RVSP (r = 0.73, P < .001) and FAC/RVSP (r = 0.78, P < .001) correlated with peak VO2. Among the CMRI‐derived RV volumetric indices analyzed, only right ventricular end‐ systolic volume index correlated with peak VO2 (r = −0.54, P < .001) and NT‐proBNP (r = 0.51, P < .001). These RV‐PA coupling indices were tested in the validation cohort of 218 patients (age 37 ± 9 years). Similar to the study cohort, TAPSE/RVSP (r = 0.59, P < .001) and FAC/RVSP (r = 0.70, P < .001) correlated with peak VO2. TAPSE/RVSP (but not FAC/RVSP) was also associated with arrhythmia occurrence in both the study cohort and validation cohorts.
Conclusion: Noninvasive RV‐PA coupling may provide complementary prognostic data in the management of chronic PR. Further studies are required to explore this clinical tool.     

Contributors to disease‐specific health knowledge in adults with congenital heart disease: A correlational study

Objective: Growth in the adults with congenital heart disease (ACHD) population represents a challenge to the health care infrastructure. As patients with chronic disease are increasingly held accountable for their own care, contributors to disease‐ specific health knowledge, which are known to correlate with patients’ participation in care, merit investigation to design patient‐focused interventions.
Design: We conducted a single‐site, cross‐sectional study of ACHD patients. Investigators retrospectively gathered clinical data as well as psychometric and health status assessments completed at the time of enrollment.
Outcome Measures: We investigated the impact of clinical and psychological varia‐ bles on Leuven Knowledge Questionnaire for Congenital Heart Diseases health knowledge composite scores (HKCS). Variables with significant associations were considered in a stepwise multivariable regression model to determine which combi‐ nation of variables jointly explained variability in HKCS.
Results: Overall HKCS was associated with the number of prior cardiac surgeries (r = 0.273; 95% CI: 0.050‐0.467; P = .016), perceived stress (r = 0.260; 95% CI: 0.033‐0.458; P = .024), SF‐36 emotional well‐being (r = −0.251; 95% CI: −0.451, −0.024; P = .030), history of noncardiac surgery (P = .037), cirrhosis (P = .048), and presence of implantable cardioverter‐defibrillator (P = .028). On multivariable mod‐ eling, only the number of cardiac surgeries was found to correlate with HKCS.
Conclusions: While univariate correlations were found between HCKS and several other clinical and psychological variables, only number of prior cardiac surgeries inde‐ pendently correlated with disease‐specific health knowledge in ACHD patients. These results suggest that clinical and psychological variables are not impediments to disease‐specific health knowledge.