Cardiac Catheterization in Adults with Ebstein Anomaly: Pathophysiologic Insights Regarding Surgical Repair and Prognosis.

The underlying invasive hemodynamics and physiology in Ebstein anomaly (EA) are poorly understood. Moreover, the hemodynamic impact of tricuspid valve intervention in EA has not been well studied. Retrospective cohort of 52 adults with repaired and 36 with unrepaired EA undergoing right heart catheterization at Mayo Clinic, MN between 1993 and 2021. "Repaired" EA was defined as prior tricuspid valve repair and/or replacement (83% post-tricuspid valve replacement). Repaired patients were younger than those with unrepaired EA (41.3 ± 16.0 versus 50.6 ± 15.6 years, p = 0.008) and had a lower prevalence of ≥ moderate native or prosthetic tricuspid regurgitation (67% versus 81%, p = 0.01). Right atrial (RA) pressure was higher among patients with repaired EA than in unrepaired disease [13 (11; 18) versus 10 (8; 15) mmHg; p = 0.02], but these differences were no longer present when adjusting for ≥ moderate right ventricular systolic dysfunction and ≥ moderate tricuspid regurgitation. Cardiac index (Qs) was lower among those with unrepaired EA than in repaired EA (1.9 ± 0.7 versus 2.3 ± 0.6 l/min/m2, p = 0.01), even after adjusting for similar confounders. During a follow-up of 8.6 (3.2-13.3) years, 16 (18%) patients died. Systolic pulmonary artery pressure was independently associated with all-cause mortality. In summary, higher cardiac indices were found in those with repaired EA compared to those with unrepaired disease. RA hypertension was prevalent in both groups and no differences in right filling pressures were found between groups after adjusting for potential confounders. Elevation in pulmonary pressures was independently associated with survival. The use of pulmonary vasomodulators in EA requires further investigation.

Hemodynamics in Adults with Systemic Right Ventricles: Differences Between Congenitally Corrected and Complete Transposition of the Great Arteries.

Despite their anatomical differences, congenitally corrected (ccTGA) and complete transposition of the great arteries (d-TGA) post-atrial switch are frequently studied together and managed similarly from a medical standpoint due to the shared systemic right ventricle (sRV). The aim was to assess differences in their underlying hemodynamics. The study is a retrospective review of 138 adults with ccTGA or d-TGA post-atrial switch undergoing cardiac catheterization at Mayo Clinic, MN between 2000 and 2021. ccTGA was categorized into isolated or complex ccTGA depending on concomitant ventricular septal defect and/or left ventricular outflow obstruction. There were 53 patients with d-TGA (91% post-Mustard procedure), 51 with complex and 34 with isolated ccTGA. Isolated ccTGA patients were older (51.8 ± 13.1 years) than those with d-TGA (37.5 ± 8.3 years) or complex ccTGA (40.8 ± 13.4 years). There were no differences in sRV or left ventricular size and function across groups. The ccTGA group more commonly had ≥ moderate tricuspid regurgitation than those with d-TGA; ≥ moderate mitral and ≥ moderate pulmonary regurgitation were most prevalent in complex ccTGA. There were no differences in sRV end-diastolic pressure (sRVEDP) or PAWP between groups. However, the ratio of PAWP:sRVEDP was higher in those with d-TGA compared to those with ccTGA. Cardiac index was higher in the d-TGA group than both groups of ccTGA patients with the latter showing higher indices of ventricular afterload. In conclusion, despite sharing a sRV, adults with d-TGA and ccTGA have substantial differences in hemodynamics and structural/valvular abnormalities. Further investigation regarding disease-specific responses to heart failure therapy in those with d-TGA and ccTGA is warranted.

Superiorly Based Posterior Pharyngeal Flaps: Using A Care Pathway to Optimize Speech and Airway Outcomes.

BACKGROUND: Pharyngeal flap (PF) surgery is effective at improving velopharyngeal sufficiency, but historical literature shows a concerning prevalence rate of obstructive sleep apnea (OSA), reported as high as 20%. Our institution has developed a protocol to minimize risk of postoperative obstructive complications and increase safety of PF surgery. We hypothesize that (1) preoperative staged removal of significant adenotonsillar tissue along with (2) multiview videofluoroscopy to guide patient-specific surgical approach via appropriately sized PFs can result in excellent speech outcomes while limiting occurrence of OSA. METHODS: This was a retrospective chart review of all patients with velopharyngeal insufficiency (VPI) (aged 2-20 years) seen at the University of Rochester from 2015 to 2022 undergoing PF surgery to correct VPI. Nasopharyngoscopy was used for surgical planning and airway evaluation. Patients with tonsillar and adenoid hypertrophy underwent staged adenotonsillectomy at least 2 months before PF. Multiview videofluoroscopy was used to identify anatomic causes of VPI and to determine PF width. Patients underwent polysomnography and speech evaluation before and at least 6 months after PF surgery. RESULTS: Forty-one children aged 8.5 ± 4.1 years (range, 4 to 18 years) who underwent posterior PF surgery for VPI were identified. This included 10 patients with 22q11.2 deletion and 4 patients with Pierre Robin sequence. Thirty-nine patients had both pre- and postoperative speech data and underwent both a pre- and postoperative sleep study. Polysomnography showed no significant difference in obstructive apnea-hypopnea index after posterior PF surgery (obstructive apnea-hypopnea index preop, 1.3 ± 1.2 events per hour; postop, 1.7 ± 2.1 events per hour; P = 0.111). Significant improvements in speech outcome were seen in patients who underwent PF (modified Pittsburgh score preop, 11.52 ± 1.37; postop, 1.09 ± 2.35; P < 0.05). CONCLUSIONS: Use of preoperative staged adenotonsillectomy as well as patient-specific PF dimensions results in effective resolution of VPI and a low risk of OSA.

Heart Failure Staging and Indications for Advanced Therapies in Adults with Congenital Heart Disease.

Heart failure (HF) is common in adults with congenital heart disease (CHD), and it is the leading cause of death in this population. Adults with CHD presenting with stage D HF have a poor prognosis, and early recognition of signs of advanced HF and referral for advanced therapies for HF offer the best survival as compared with other therapies. The indications for advanced therapies for HF outlined in this article should serve as a guide for clinicians to determine the optimal time for referral. Palliative care should be part of the multidisciplinary care model for HF in patients with CHD.

On-treatment blood pressure and cardiovascular mortality in adults with repaired coarctation of aorta.

BACKGROUND: Although, hypertension is common in adults with repaired coarctation of aorta (COA), there are no data about on-treatment blood pressure (BP), and its relationship to outcomes in this population. The purpose of this study was to determine the relationship between on-treatment BP and cardiovascular mortality in adults with repaired COA. METHODS: Retrospective study of adults with repaired COA on antihypertensive therapy (n = 461, age 39 ± 11). All BP measurements obtained within the first 3 years were averaged to determine the on-treatment BP, and the patients were stratified into BP quartiles using the cut-off points from the guidelines. RESULTS: Being in the upper systolic BP (SBP) quartiles (SBP 120-129, 130-139 and ≥140) was associated with higher risk of cardiovascular mortality (HR 1.05, 95%CI 1.01-1.07, HR 1.12, 95%CI 1.04 to 1.15 and HR 1.39, 95%CI 1.13 to 1.59), as compared to being the lowest SBP quartile. We observed a 7% increase in the risk of cardiovascular mortality for every 5 mmHg increase in SBP, and a 4% increase in risk of cardiovascular mortality for every 5 mmHg increase in DBP. CONCLUSIONS: Collectively, these data suggest that even s less severe form of hypertension SBP (120-129 mmHg) was not benign, and perhaps should be considered for antihypertensive therapy. A randomized controlled clinical trial is required to determine whether this group of patients (SBP 120 to 129 mmHg) would benefit from antihypertensive therapy, and to determine the optimal type and intensity of antihypertensive therapy in this population.

Temporal Changes in Clinical Characteristics and Outcomes of Adults With Congenital Heart Disease.

BACKGROUND: The purpose of this study was to assess differences in the clinical characteristics (defined by congenital heart disease [CHD] anatomic and physiologic classification scheme) of adults with CHD across different eras, and how these differences influence outcomes (heart failure hospitalization and all-cause mortality). METHOD: Patients were divided into depending on year of baseline encounter: cohort #1 (1991-2000, n = 1,984 [27%]), cohort #2 (2001-2010, n = 2,448 [34%]), and cohort #3 (2011-2020, n = 2,847 [39%]). Patients were classified into 3 anatomic groups (simple, moderate, and complex CHD) and 4 physiologic stages (stage A-D). RESULTS: There was a temporal increase in the proportion of patients in physiologic stage C (17% vs 21% vs 24%, P < .001), and stage D (7% vs 8% vs 10%, P = .09), with a corresponding decrease in physiologic stage A (39% vs 35% vs 28%, P < .001). No temporal change in anatomic groups. There was a temporal decrease in the incidence of all-cause mortality (12.7 vs 10.6 vs 9.5 per 1,000 patient-years, P < .001). However, there was a temporal increase in the incidence of heart failure hospitalization (6.8 vs 8.4 vs 11.2 per 1,000 patient-years, P < .001). CHD physiologic stage (but not anatomic groups) was associated with heart failure hospitalization and all-cause mortality. CONCLUSIONS: There is a need for better strategies to identify and treat heart failure, and to modify the risk factors associated with heart failure and all-cause mortality.

Right atrial dysfunction is associated with atrial arrhythmias in adults with repaired tetralogy of fallot.

BACKGROUND: Adults with repaired tetralogy of Fallot (TOF) have right atrial (RA) remodeling and dysfunction, and RA function can be measured using speckle tracking echocardiography. There are limited data about the role of RA strain imaging for risk stratification in this population. We hypothesized that RA reservoir strain can identify TOF patients at risk of developing atrial arrhythmia. To test this hypothesis, we assessed the relationship between RA reservoir strain and atrial arrhythmias in adults with repaired TOF. METHOD: Retrospective cohort study of adults with repaired TOF, and no prior history of atrial arrhythmias. Atrial arrhythmia was defined as atrial fibrillation, atrial flutter/atrial tachycardia, and categorized as new-onset versus recurrent atrial arrhythmias. RESULTS: We identified 426 patients (age 33 ± 12 years; males 208 (49%)) that met the inclusion criteria. The mean RA reservoir strain, conduit strain, and booster strain were 34 ± 11%, 20 ± 9%, and 15 ± 12%, respectively. Of 426 patients, 73 (17%) developed new-onset atrial arrhythmias (atrial flutter/tachycardia n = 42; atrial fibrillation n = 31); annual incidence 1.9%. RA reservoir strain was associated with new-onset atrial arrhythmias (adjusted HR 0.95, 95% CI 0.93-0.97) after multivariable adjustment. Of 73 patients with new-onset atrial arrhythmia, 41 (56%) had recurrent atrial arrhythmia (atrial flutter/tachycardia n = 18; atrial fibrillation n = 23); annual incidence 11.2%. Similarly, RA reservoir strain was associated with recurrent atrial arrhythmias (adjusted HR 0.92, 95% CI 0.88-0.96) after multivariable adjustment. CONCLUSIONS: RA strain indices can identify patients at risk for atrial arrhythmias, and this can in turn, be used to guide the type/intensity of therapy in such patients.

Adenotonsillectomy for Snoring and Mild Sleep Apnea in Children: A Randomized Clinical Trial.

Importance: The utility of adenotonsillectomy in children who have habitual snoring without frequent obstructive breathing events (mild sleep-disordered breathing [SDB]) is unknown. Objectives: To evaluate early adenotonsillectomy compared with watchful waiting and supportive care (watchful waiting) on neurodevelopmental, behavioral, health, and polysomnographic outcomes in children with mild SDB. Design, Setting, and Participants: Randomized clinical trial enrolling 459 children aged 3 to 12.9 years with snoring and an obstructive apnea-hypopnea index (AHI) less than 3 enrolled at 7 US academic sleep centers from June 29, 2016, to February 1, 2021, and followed up for 12 months. Intervention: Participants were randomized 1:1 to either early adenotonsillectomy (n = 231) or watchful waiting (n = 228). Main Outcomes and Measures: The 2 primary outcomes were changes from baseline to 12 months for caregiver-reported Behavior Rating Inventory of Executive Function (BRIEF) Global Executive Composite (GEC) T score, a measure of executive function; and a computerized test of attention, the Go/No-go (GNG) test d-prime signal detection score, reflecting the probability of response to target vs nontarget stimuli. Twenty-two secondary outcomes included 12-month changes in neurodevelopmental, behavioral, quality of life, sleep, and health outcomes. Results: Of the 458 participants in the analyzed sample (231 adenotonsillectomy and 237 watchful waiting; mean age, 6.1 years; 230 female [50%]; 123 Black/African American [26.9%]; 75 Hispanic [16.3%]; median AHI, 0.5 [IQR, 0.2-1.1]), 394 children (86%) completed 12-month follow-up visits. There were no statistically significant differences in change from baseline between the 2 groups in executive function (BRIEF GEC T-scores: -3.1 for adenotonsillectomy vs -1.9 for watchful waiting; difference, -0.96 [95% CI, -2.66 to 0.74]) or attention (GNG d-prime scores: 0.2 for adenotonsillectomy vs 0.1 for watchful waiting; difference, 0.05 [95% CI, -0.18 to 0.27]) at 12 months. Behavioral problems, sleepiness, symptoms, and quality of life each improved more with adenotonsillectomy than with watchful waiting. Adenotonsillectomy was associated with a greater 12-month decline in systolic and diastolic blood pressure percentile levels (difference in changes, -9.02 [97% CI, -15.49 to -2.54] and -6.52 [97% CI, -11.59 to -1.45], respectively) and less progression of the AHI to greater than 3 events/h (1.3% of children in the adenotonsillectomy group compared with 13.2% in the watchful waiting group; difference, -11.2% [97% CI, -17.5% to -4.9%]). Six children (2.7%) experienced a serious adverse event associated with adenotonsillectomy. Conclusions: In children with mild SDB, adenotonsillectomy, compared with watchful waiting, did not significantly improve executive function or attention at 12 months. However, children with adenotonsillectomy had improved secondary outcomes, including behavior, symptoms, and quality of life and decreased blood pressure, at 12-month follow-up. Trial Registration: ClinicalTrials.gov Identifier: NCT02562040.

Congenital Heart Disease in Adults with Autosomal Dominant Polycystic Kidney Disease.

INTRODUCTION: Autosomal dominant polycystic kidney disease (ADPKD) is caused mainly by pathogenic variants in PKD1 or PKD2 encoding the polycystin-1 and -2 proteins. Polycystins have shown to have an essential role in cardiac development and function in animal models. In the current study, we describe the clinical association between ADPKD and congenital heart disease (CHD). METHODS: Medical records from Mayo Clinic were queried for all patients with confirmed ADPKD and CHD between 1993 and 2020. CHD was categorized into left-to-right shunt, obstructive, and complex lesions. Patent foramen ovale, mitral valve prolapse, and bicuspid aortic valve anomalies were excluded. RESULTS: Twenty-five out of 1,359 (1.84%) ADPKD patients were identified to have CHD. Of these, 84% were Caucasians and 44% were males. The median (Q1-Q3) age (years) at CHD diagnosis was 12.0 (2.0-43.5). Fourteen patients (56%) had left-to-right shunt lesions, 6 (24%) had obstructive lesions and 5 (20%) complex lesions. Seventeen patients (68%) had their defects surgically corrected at a median age (Q1-Q3) of 5.5 (2.0-24.7). Among 13 patients with available genetic testing, 12 (92.3%) had PKD1 pathogenic variants, and none had PKD2. The median (Q1-Q3) age at last follow-up visit was 47.0 (32.0-62.0) and median (Q1-Q3) eGFR was 35.8 (11.4-79.0) mL/min/1.73 m2. Three patients (12%) died; all of them had left-to-right shunt lesions. DISCUSSION/CONCLUSION: We observed a higher CHD frequency in ADPKD than the general population (1.84 vs. 0.4%). While only PKD1 pathogenic variants were identified in this cohort, further studies are needed to confirm this novel finding and understand the role of polycystins in the development of the heart and vessels.

Coarctation of aorta is associated with left ventricular stiffness, left atrial dysfunction and pulmonary hypertension.

BACKGROUND: Brachial systolic blood pressure (BP) is the most commonly used metric for monitoring hypertension. However, recent studies suggest that brachial systolic BP underestimates left ventricle (LV) systolic load in patients with coarctation of aorta (COA). Since brachial systolic BP is used as a surrogate of arterial afterload in clinical practice, it is important to determine how well it correlates with LV remodeling and stiffness in patients with COA as compared to patients with idiopathic hypertension. METHODS: This is cross-sectional study of COA patients with hypertension (COA group) and adults with idiopathic hypertension (control group). Both groups were matched 1:1 based on age, sex, BMI and systolic BP. We hypothesized that the COA group will have higher LV systolic and diastolic stiffness, and more advanced left atrial remodeling and pulmonary hypertension. We assessed LV systolic stiffness using end-systolic elastance, and diastolic stiffness using LV stiffness constant and chamber capacitance (LV-end-diastolic volume at an end-diastolic pressure of 20mm Hg) RESULTS: There were 112 patients in each group. Although both groups had similar systolic BP, the COA group had a higher end-systolic elastance (2.37 ± 0.74 vs 2.11 ± 0.54 mm Hg/mL, P= .008), higher LV stiffness constant (6.91 ± 0.81 vs 5.93 ± 0.79, P= .006) and lower LV-end-diastolic volume at an end-diastolic pressure of 20mm Hg (58 ± 9 vs 67 ± 11 mL/m2, P< .001). Additionally, the COA group had more advanced left atrial remodeling and higher pulmonary artery pressures which is corroborating evidence of high LV filling pressure. CONCLUSIONS: COA patients have more LV stiffness and abnormal hemodynamics compared to non-COA patients with similar systolic BP, suggesting that systolic BP may underestimate LV systolic load in this population. Further studies are required to determine whether the observed LV stiffness and dysfunction translates to more cardiovascular events during follow-up, and whether adopting a stricter systolic BP target in clinical practice or changing threshold for COA intervention will lead to less LV stiffness and better clinical outcomes.

Elevated ventricular filling pressures and long-term survival in adults post-Fontan.

OBJECTIVE: To assess the association between elevated ventricular-end diastolic pressures (VEDP) and pulmonary artery wedge pressure (PAWP) on long-term survival in adult Fontan patients. BACKGROUND: The impact of ventricular filling pressures on long-term survival in adults post-Fontan palliation is unknown. METHODS: We included 148 adult Fontan patients (age ≥ 18 years) without atrioventricular valve prosthesis or pulmonary vein stenosis undergoing arterial and venous catheterization between December 1999 and November 2017. VEDP was defined as ≥12 mmHg and PAWP as >12 mmHg based on optimal cut-offs for prediction of mortality on receiver-operator curves (AUC 0.63 and 0.66, respectively). RESULTS: Mean age was 31.3 ± 9.2 years and 48.6% of patients were females. Most common congenital defects were tricuspid atresia (36.4%) and double-inlet left ventricle (28.3%); 59.5% patients had atriopulmonary Fontan connections. Mean VEDP was 11.5 ± 4.7 mmHg and PAWP 10.6 ± 4.5 mmHg (correlation coefficient .76). During a follow-up of 6.0 ± 4.8 years (median 5.4, IQR 1.4-9.4), there were 45 deaths (30.4%). Overall survival was lower in patients with VEDP ≥ 12 compared to those with VEDP < 12 mmHg (p = .02). Similarly, survival was lower in patients with PAWP>12 compared to patients with PAWP ≤ 12 mmHg (p < .0001). In the multivariate model, PAWP was an independent predictor of death (HR 1.1 per mmHg, 95% CI 1.02-1.15, p = .009) whereas VEDP was not (HR 1.1 per mmHg, 95% CI 1.0-1.13; p = .08). CONCLUSION: PAWP but not VEDP was independently associated with long-term overall mortality in adult Fontan patients. Perhaps PAWP rather than VEDP should be used in the risk stratification of these patients.

Incidence and outcomes of prosthetic valve endocarditis in adults with tetralogy of Fallot.

BACKGROUND: The risk of endocarditis varies with CHD complexity and the presence of prosthetic valves. The purpose of the study was therefore to describe incidence and outcomes of prosthetic valve endocarditis in adults with repair tetralogy of Fallot. METHODS: Retrospective review of adult tetralogy of Fallot patients who underwent prosthetic valve implantation, 1990-2017. We defined prosthetic valve endocarditis-related complications as prosthetic valve dysfunction, perivalvular extension of infection such abscess/aneurysm/fistula, heart block, pulmonary/systemic embolic events, recurrent endocarditis, and death due to sepsis. RESULTS: A total of 338 patients (age: 37 ± 15 years) received 352 prosthetic valves (pulmonary [n = 308, 88%], tricuspid [n = 13, 4%], mitral [n = 9, 3%], and aortic position [n = 22, 6%]). The annual incidence of prosthetic valve endocarditis was 0.4%. There were 12 prosthetic valve endocarditis-related complications in six patients, and these complications were prosthetic valve dysfunction (n = 4), systemic/pulmonary embolic events (n = 2), heart block (n = 1), aortic root abscess (n = 1), recurrent endocarditis (n = 2), and death due to sepsis (n = 1). Three (50%) patients required surgery at 2 days, 6 weeks, and 23 weeks from the time of prosthetic valve endocarditis diagnosis. Altogether three of the six (50%) patients died, and one of these deaths was due to sepsis. CONCLUSIONS: The incidence, complication rate, and outcomes of prosthetic valve endocarditis in tetralogy of Fallot patients underscore some of the risks of having a prosthetic valve. It is important to educate the patients on the need for early presentation if they develop systemic symptoms, have a high index of suspicion for prosthetic valve endocarditis, and adopt a multi-disciplinary care approach in this high-risk population.

Impact of atrial arrhythmia on survival in adults with tetralogy of Fallot.

BACKGROUND: Atrial arrhythmia is a late complication after tetralogy of Fallot (TOF) repair, but arrhythmia outcomes data are limited. OBJECTIVES: The purpose of the study was to describe atrial arrhythmia presentations, outcomes of antiarrhythmic therapy, and impact of arrhythmia on transplant-free survival. METHODS: We reviewed the MACHD (Mayo Adult Congenital Heart Disease) Registry and identified 113 patients (age 49 ±â€¯13 years) with documented arrhythmia, and 302 patients without history of arrhythmia, 1990-2017. We classified arrhythmias into atrial fibrillation and atrial flutter/tachycardia based on the rhythm on the first abnormal electrocardiogram. RESULTS: At the time of first documented arrhythmia, 58(51%) had atrial fibrillation while 55(49%) had atrial flutter/tachycardia. Of the 113 patients, 14(12%) received rhythm control with class I/III antiarrhythmic drugs (AAD), 79(70%) had direct current cardioversion, 9(8%) received rate control with class II/IV AAD, and 11(10%) received only anticoagulation. Successful cardioversion occurred in 100(89%) patients, and arrhythmia recurrence rate was 16 per 100 patient-years. The multivariate risk factors for death and/or heart transplant were atrial fibrillation (HR 1.94, CI 1.10-3.15, P = .031) and older age (HR 1.63, CI 1.12-2.43, P = .019) per 5 year increment. CONCLUSIONS: Atrial fibrillation, but not atrial flutter, was associated with reduced survival in our repaired TOF cohort. Further studies are required to determine if more aggressive antiarrhythmic therapy will improve survival in patients with atrial fibrillation.

Temporary balloon occlusion of atrial septal defects in suspected or documented left ventricular diastolic dysfunction: Hemodynamic and clinical findings.

OBJECTIVE: To review our experience with balloon testing prior to atrial septal defect (ASD) closure in adults with left ventricular (LV) diastolic dysfunction. BACKGROUND: ASD closure in patients with LV diastolic dysfunction may precipitate LV failure. Temporary ASD occlusion has been used in this scenario but data are limited. METHODS: Retrospective review of 27 patients age ≥ 50 years undergoing temporary ASD balloon occlusion between 2000 and 2018 for suspected LV diastolic dysfunction or elevated LV end-diastolic pressure (LVEDP). RESULTS: Median age was 72 years (IQR 66.7; 75.2). Atrial fibrillation was seen in 58% of patients, hypertension in 58%, and coronary artery disease in 26%; 52% were females. Median ASD size was 13 mm (10; 18) and Qp/Qs 1.8 (1.6; 2.2). Median LVEDP was 14 mmHg (12; 22); pulmonary artery wedge pressure (PAWP) 12 mmHg (9; 16.5) and left atrial pressure (LAP) 13.5 mmHg (8; 16.3). After a median of 5 min (3; 10) of balloon occlusion, patients with baseline LVEDP ≥15 mmHg had more significant increases in LVEDP (9 [6; 12] vs. 2 mmHg [0.5; 5]; p = 0.03) and LAP/PAWP (10.5 [8.3; 16.3] vs. 1.5 mmHg [-1.5; 3]; p = 0.0003) than those with baseline LVEDP <15 mmHg. None of those with a baseline LVEDP <15 mmHg had a LAP/PAWP >15 mmHg during balloon testing compared to 92% of patients with a baseline LVEDP ≥15 mmHg. CONCLUSION: LVEDP might be used to predict LAP post-ASD closure. Comorbidities typically associated with LV diastolic dysfunction are common in these patients and should be considered in their management.

Management of the Adult Patient With Congenitally Corrected Transposition: Challenges and Uncertainties.

Congenitally corrected transposition (ccTGA) is a rare form of congenital heart disease characterized by atrioventricular and ventriculoarterial discordance. Patients with ccTGA usually have associated congenital cardiovascular conditions; less than 1% have no associated lesions. Generally, ccTGA is identified during infancy or childhood with features of heart failure or cyanosis when there are associated lesions such as ventricular septal defect and/or pulmonic stenosis. Presentation later in life generally occurs when there are either mild or no associated lesions. Presentation during adulthood may be prompted by symptoms or signs of cardiovascular disease or due to abnormal findings on cardiac testing. Management of patients with ccTGA depends on presentation, symptoms, and associated defects. In this review, we will focus on the management of adult patients with ccTGA.

Sudden death in patients with Ebstein anomaly.

Aims: Ventricular dysfunction or structural alteration of either ventricle is a well-established risk factor for sudden death (SD). Ebstein anomaly (EA) can present with both right and left heart abnormalities; however, predictors of SD have not been described. We therefore sought to characterize the incidence and risk factors of SD among a large cohort of patients with EA. Methods and results: All EA patients who underwent evaluation at a high-volume institution over a 4-decade period were retrospectively reviewed. Clinical variables, cardiovascular surgical procedure(s), and cause of death were recorded. Sudden death incidence from birth and following tricuspid valve (TV) surgery were estimated using the Kaplan-Meier method. Cox regression analysis was used to identify clinical and surgical predictors of SD. The cohort comprised of 968 patients [mean age 25.3 years, 41.5% male; 79.8% severe EA, 18.6% accessory pathway, 0.74% implantable cardioverter-defibrillator (ICD) placement]. The 10-, 50-, and 70-year cumulative incidences of SD from birth were 0.8%, 8.3%, and 14.6%, respectively. Prior ventricular tachycardia [hazard ratio (HR) 6.37, P < 0.001)], heart failure (HR 5.64, P < 0.001), TV surgery (HR 5.94, P < 0.001), syncope (HR 2.03, P = 0.019), pulmonic stenosis (HR 3.42, P = 0.001), and haemoglobin > 15 g/dL (HR 2.05, P = 0.026) were multivariable predictors of SD. In a similar subgroup analysis of patients who underwent TV surgery, all of the above factors except syncope were significantly associated with post-operative SD on multivariable analysis. Conclusion: Patients with EA are at significant risk for SD. Key clinical SD predictors identified can aid in risk stratification and potentially guide primary prevention ICD implantation.

Management of Pregnancy in Patients With Complex Congenital Heart Disease: A Scientific Statement for Healthcare Professionals From the American Heart Association.

Today, most female children born with congenital heart disease will reach childbearing age. For many women with complex congenital heart disease, carrying a pregnancy carries a moderate to high risk for both the mother and her fetus. Many such women, however, do not have access to adult congenital heart disease tertiary centers with experienced reproductive programs. Therefore, it is important that all practitioners who will be managing these women have current information not only on preconception counseling and diagnostic evaluation to determine maternal and fetal risk but also on how to manage them once they are pregnant and when to refer them to a regional center with expertise in pregnancy management.

Outcomes of hospitalization in adults with Fontan palliation: The Mayo Clinic experience.

The outcomes of hospitalization in the Fontan population have not been specifically studied. The purpose of this study was to describe outcomes of hospitalization (frequency and indications for hospitalization, and in-hospital mortality) in this population and to determine how these outcomes differ from those of other adults with congenital heart disease (CHD). METHODS: This was a retrospective study of adult Fontan patients hospitalized at Mayo Clinic Rochester in 1990-2015. We selected age- and gender-matched control group of patients with repaired CHD and biventricular circulation hospitalized within the study period. RESULTS: A total of 367 Fontan patients (age 31±7 years and 259 [71%] with atriopulmonary Fontan) had 853 hospital admissions in 4 years (58 hospitalizations per 100 patient-years). The most common indications were arrhythmia (n=188, 22%), heart failure (n=169, 20%), and cardiac surgery (n=133, 16%). Overall in-hospital mortality was 4% (n=38), and the highest in-hospital mortality occurred in patients hospitalized for cardiac surgery (n=15, 11%) and heart failure (n=13, 8%). In comparison to the repaired CHD and biventricular circulation group, the Fontan group had more frequent hospitalizations (22 vs 58 per 100 patient-years, P<.001) and higher overall in-hospital mortality (1% vs 5%, P<.001), mortality after cardiac surgery (2% vs 11%, P=.01), and mortality for heart failure-related hospitalizations (2% vs 8%, P=.04). CONCLUSIONS: Adults with Fontan palliation had more frequent hospitalization and in-hospital mortality compared to the rest of the CHD population. Arrhythmia and heart failure were the most common indications for hospitalization. Perhaps optimal management of heart failure and arrhythmia may improve outcomes in this population.

Risk stratification and clinical outcomes after surgical pulmonary valve replacement.

BACKGROUND: To determine if RV volume was predictive of survival and cardiovascular adverse event (CAE) after pulmonary valve replacement (PVR). METHODS: We reviewed the MACHD (Mayo Adult Congenital Heart Disease) database for patients with tetralogy of Fallot (TOF) undergoing PVR, 2000-2015. The patients were divided into quartiles based on RV end-diastolic volume index (RVEDVI); those in the lowest quartile (Group A, n = 46) and the top quartile (Group B, n = 42) were selected as the study cohort. RESULTS: In comparison to Group A, Group B patients were older at time of PVR (28 ±â€¯4 vs 33 ±â€¯5 years, P = .011) and had larger RV volumes (RVEDVI 127 [117-138] mL/m2 vs 1 91 [179-208], P < .001; RVESVI 64 [57-73] mL/m2 vs 122 [103-136], P < .001). A total of 28 CAE occurred in 23 patients during 69 (33-94) months follow-up: death (n = 4), heart transplant listing (n = 1), initiation of palliative care (n = 1), heart failure hospitalization (n = 11), stroke (n = 2) and sustained ventricular tachycardia/aborted sudden cardiac death (n = 9). Survival was similar between Groups A and B (95% vs 91% at 10 years, P = .273) but freedom from CAE was significantly lower in Group B (67% vs 36% at 10 years, P = .002). Combination of RVESVI: >95 mL/m2 and tricuspid annular plane systolic excursion/RV systolic pressure (TAPSE/RVSP) <0.4 predicted CAE with sensitivity of 67% and specificity of 92%. CONCLUSION: Patients undergoing PVR at larger RV volumes had similar survival but more overall CAE. A larger study population with a longer follow-up will be required to determine if early PVR provides survival benefit in the long-term.