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.

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.

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.

30-day patient reported outcomes can be predicted by change in left atrial pressure and not change in transmitral gradient following MitraClip.

BACKGROUND: Change in left atrial pressure (LAP) has been shown to be associated with symptom improvement post-MitraClip; however, the association between acute procedural changes in transmitral diastolic mean gradient (MG) compared to LAP and symptom improvement is not well established. METHODS: 164 consecutive patients undergoing MitraClip at Mayo Clinic between June 2014 and May 2018 were included. Preclip and postclip MG and LAP were recorded. Baseline demographics, clinical, and echocardiographic outcomes, including 30-day New York Heart Association (NYHA) functional status were obtained from patient charts. RESULTS: Median age was 81.5 years (IQR: 76.3, 87), 34% were female and 94.5% had NYHA class III and IV functional status at baseline. At baseline, median MG was 4 mmHg (IQR: 3, 5) and LAP was 19 mmHg (IQR: 16, 23.5). Following MitraClip deployment, the median MG was 4 mmHg (IQR: 3, 6) and the median LAP was 17 mmHg (IQR: 14, 21), 69.5% of patients had less than moderate MR. There was no statistically significant association between change in MG and NYHA functional class at 30 days (OR = 0.95, 95% CI: 0.76-1.20). However, a reduction in LAP following MitraClip deployment was significantly associated with improvement in NYHA functional status at 30 days following adjustments for age and sex (aOR 3.36, 95% CI: 1.34-8.65). There was no significant correlation between change in mean LAP and change in MG (p = .98). CONCLUSION: Unlike change in left atrial pressure, change in MG post-MitraClip was not associated with patient reported outcomes at 30 days and did not correlate with change in left atrial pressure. Long-term follow up is needed to evaluate the impact of LA pressure on symptoms.

Hemodynamics and Prognostic Impact of Concomitant Mitral Stenosis in Patients Undergoing Surgical or Transcatheter Aortic Valve Replacement for Aortic Stenosis.

BACKGROUND: Mitral stenosis frequently coexists in patients with severe aortic stenosis. Mitral stenosis severity evaluation is challenging in the setting of combined aortic stenosis and mitral stenosis because of hemodynamic interactions between the 2 valve lesions. The impact of aortic valve replacement (AVR) for severe aortic stenosis on mitral stenosis is unknown. This study aimed to assess the effect of AVR on mitral stenosis hemodynamics and the clinical outcomes of patients with severe aortic stenosis with and without mitral stenosis. METHODS: We retrospectively investigated patients who underwent surgical AVR or transcatheter AVR for severe aortic stenosis from 2008 to 2015. Mean transmitral gradient by Doppler echocardiography ≥4 mm Hg was identified as mitral stenosis; patients were then stratified according to mitral valve area (MVA, by continuity equation) as >2.0 cm2 or ≤2.0 cm2. MVA before and after AVR in patients with mitral stenosis were evaluated. Clinical outcomes of patients with and without mitral stenosis were compared using 1:2 matching for age, sex, left ventricular ejection fraction, method of AVR (surgical AVR versus transcatheter AVR) and year of AVR. RESULTS: Of 190 patients with severe aortic stenosis and mitral stenosis (age 76±9 years, 42% men), 184 were matched with 362 with severe aortic stenosis without mitral stenosis. Among all mitral stenosis patients, the mean MVA increased after AVR by 0.26±0.59 cm2 (from 2.00±0.50 to 2.26±0.62 cm2, P<0.01). MVA increased in 105 (55%) and remained unchanged in 34 (18%). Indexed stroke volume ≤45 mL/m2 (odds ratio [OR] 2.40; 95% CI, 1.15-5.01; P=0.020) and transcatheter AVR (OR, 2.36; 95% CI, 1.17-4.77; P=0.017) were independently associated with increase in MVA. Of 107 with significant mitral stenosis (MVA ≤2.0 cm2), MVA increased to >2.0 cm2 after AVR in 52 (49%, pseudo mitral stenosis) and remained ≤2.0 cm2 in 55 (51%, true mitral stenosis). During follow-up of median 2.9 (0.7-4.9) years, true mitral stenosis was an independent predictor of all-cause mortality (adjusted hazard ratio, 1.88; 95% CI, 1.20-2.94; P<0.01). CONCLUSIONS: MVA improved after AVR in nearly half of patients with severe aortic stenosis and mitral stenosis. MVA remained ≤2.0 cm2 (true mitral stenosis) in nearly half of patients with severe aortic stenosis and significant mitral stenosis; this was associated with worse survival among patients undergoing AVR for severe aortic stenosis.

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.

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.

Effusive-Constrictive Pericarditis: Doppler Findings.

PURPOSE OF REVIEW: To review the echo-Doppler findings in effusive-constrictive pericarditis (ECP). ECP corresponds to the coexistence of a hemodynamically significant pericardial effusion and markedly reduced compliance of the pericardium, manifested by constrictive physiology post-pericardiocentesis. RECENT FINDINGS: We summarize herein the recent observations regarding the prevalence of ECP based on echocardiography as well as the pre- and post-pericardiocentesis echo-Doppler features of ECP. ECP diagnosed by echocardiography was seen in approximately 15% of patients with ECP pre- and post-pericardiocentesis echo-Doppler findings sharing features with both cardiac tamponade and constrictive pericarditis. ECP post-pericardiocentesis is common but its natural history in the current era might be better than previously reported. Further studies and (particularly simultaneous echocardiography-cardiac catheterization) are still critically needed to better understand the underlying hemodynamics of ECP. Moreover, it remains to be determined whether pre- and post-pericardiocentesis echo-Doppler findings can be used to prognosticate or to guide therapy of those undergoing pericardiocentesis.

Mechanism for temporal changes in exercise capacity after Fontan palliation: Role of Doppler echocardiography.

BACKGROUND: The objective was to better understand Doppler hemodynamics and exercise capacity in patients with Fontan palliation by delineating the hemodynamic mechanism for temporal changes in their peak oxygen consumption (V̇o2). METHODS: We performed a retrospective review of adult Fontan patients with systemic left ventricle (LV) who underwent serial transthoracic echocardiograms (TTE) and cardiopulmonary exercise tests (CPET) at Mayo Clinic in 2000-2015. TTE and CPET data were used (1) to determine agreement between V̇o2 and Doppler-derived LV function indices (eg, stroke volume index [SVI] and cardiac index [CI]) and (2) to determine agreement between temporal changes in peak V̇o2 and LV function indices. RESULTS: Seventy-five patients (44 men; 59%) underwent 191 pairs of TTE and CPET. At baseline, mean age was 24±3 years, peak V̇o2 was 22.9±4.1 mL/kg/min (63±11 percent predicted), SVI was 43±15 mL/m2, and CI was 2.9±0.9 L/min/m2. Peak V̇o2 correlated with SVI (r=0.30, P<.001) and with CI (r=0.45, P<.001) in the 153 pairs of TTE and CPET in patients without cirrhosis. Temporal changes in percent predicted peak V̇o2 correlated with changes in SVI (r=0.48, P=.005) and CI (r=0.49, P=.004) among the 33 patients without interventions during the study. In the 19 patients with Fontan conversion, percent predicted peak V̇o2 and chronotropic index improved. CONCLUSIONS: Overall, there was a temporal decline in peak V̇o2 that correlated with decline in Doppler SVI. In the patients who had Fontan conversion operation, there was a temporal improvement in peak V̇o2 that correlated with improvement in chronotropic index.

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.

Progressive right ventricular enlargement due to pulmonary regurgitation: Clinical characteristics of a "low-risk" group.

BACKGROUND: The optimal interval between serial cardiac magnetic resonance imaging (CMRI) scans for monitoring right ventricular (RV) enlargement in the setting of severe pulmonic valve regurgitation (PR) is unknown. The purposes of this study were to (1) determine the annual change in RV volume on serial CMRI scans and (2) identify the risk factors for rapid progression of RV enlargement. METHODS: A retrospective study of adults with postintervention native valve PR and ≥2 CMRI scans at Mayo Clinic Rochester from 2000 to 2015 was conducted. Rapid progression of RV enlargement was defined as first upper quartile of annual increase in RV end-diastolic volume index (RVEDVi) for the cohort. RESULTS: Of the 63 patients (age, 36 ±â€¯9 years) in the study, 43 (68%) had tetralogy of Fallot, whereas 20 (32%) had valvular pulmonic stenosis. Right ventricular outflow tract interventions that resulted in PR were balloon pulmonary valvuloplasty (n = 4; 7%), transannular patch repair (n = 30; 58%), and nontransannular patch repair (n = 18; 35%). Interval between baseline and second CMRI was 2 (1-4) years. In comparison to baseline CMRI, RVEDVi increased from 130 (109-141) to 135 (126-155) mL/m2 and median annual change in RVEDVi was 3.1 (1.7-5.9) mL/m2. Univariate risk factors for rapid progression of RV enlargement (annual increase in RVEDVi >6 mL/m2) were ≥moderate tricuspid regurgitation and RVEDVi >130 mL/m2. Among the 24 patients without these risk factors (low-risk subgroup), RVEDVi increased by only 3 (0-7) mL/m2 over 7 (5-9) years. CONCLUSIONS: Patients with PR without RVEDVi >130 mL/m2 and/or ≥moderate tricuspid regurgitation represent a low-risk subgroup that may be appropriate for clinical and echo follow-up but may potentially require infrequent CMRI follow-up.

Role of Doppler echocardiography for cardiac output assessment in Fontan patients.

BACKGROUND: To determine (1) correlation between Doppler stroke volume index (SVI) and cardiac magnetic resonance imaging (CMRI) SVI and (2) association between Doppler SVI and Fontan-associated diseases (FAD) and Fontan failure. METHODS: Review of Fontan patients who underwent same-day CMRI and transthoracic echocardiography (TTE), 2005 to 2015. We defined FAD as cardiac thrombus, protein-losing enteropathy, arrhythmia, and hospitalization for heart failure. Fontan failure was defined as Fontan conversion or revision, heart transplantation or listing, or death. RESULTS: Fifty-three patients with systemic left ventricle (LV) underwent 86 sets of TTE/CMRI. Mean (SD) age 31 (6) years. SVI (45 [16] vs 42 [13] mL/m2), CI (3.0 [1.1] vs 2.8 [0.8] L min-1 m-2), and ejection fraction (53 [4]% vs 51 [5]%) were similar for both modalities (P>.05 for all). Doppler SVI correlated with CMRI (r=0.68; P<.001). Sixteen patients had cirrhosis, and these patients had a higher CI (3.9 [0.9] vs 2.8 [1.0] L min-1 m-2; P<.01). Among the 37 patients without cirrhosis, Doppler SVI <39 mL/m2 was associated with FAD (odds ratio [OR], 2.11; 95% confidence limit, 1.26-3.14; P=.02); Fontan failure was more common in patients with CI was <2.5 L min-1 m-2 (3/9 [33%] vs 0/28 [0%], P=.01). Another 11 patients with systemic right ventricle (RV) underwent 17 sets of TTE/CMRI, mean (SD) age 17 (3) years, and CMRI SVI also correlated with Doppler SVI (r=0.75; P<.001). CONCLUSION: Doppler SVI correlated with CMRI SVI in patients with systemic LV and systemic RV. The association between output measures (SVI and CI) and FAD were seen only in single LV patients (single RV patients not assessed for this outcome due to small numbers). An association between low Doppler CI and Fontan failure was suggested in a small number of single LV patients.

Carcinoid Heart Disease without Severe Tricuspid Valve Involvement.

Carcinoid syndrome causes a rare form of acquired valvular heart disease which typically occurs in the setting of liver metastases. In carcinoid-induced valvular heart disease, the tricuspid valve is almost universally affected; left-sided valve disease occurs infrequently in affected patients. Herein, we report 2 cases of carcinoid-induced valvular heart disease; one case had no evidence of tricuspid valve involvement despite severe involvement of all other valves, while the other case was without severe tricuspid valve involvement.

PA-VSD Without MAPCA(s): Review of Long-Term Outcomes and Reinterventions.

BACKGROUND: Pulmonary atresia with ventricular septal defect without major aortopulmonary collateral arteries (MAPCAs) is an uncommon form of congenital heart disease. As more patients with congenital heart disease live to adulthood, the objective of this article was to review the long-term results of this specific population. METHODS: A review of the PubMed database was performed using pertinent key words (pulmonary atresia, tetralogy of Fallot, conduit, right ventricle-pulmonary artery) concentrating on studies from 1990-present and published in English. Most studies of pulmonary atresia-ventricular septal defect patients included those with and without MAPCAs. Analysis included examination of the entire cohort, consideration of the proportion of patients with MAPCAs, and any subgroup analysis of the patients without MAPCAs. RESULTS: Survival is approximately 80% at ten years and is improved with complete repair and larger pulmonary arteries. Some studies have found genetic syndromes and extracardiac anomalies to impact survival, while others have not. Incomplete repair has been shown to be associated with worse survival. Independent of initial management strategy, patients with pulmonary atresia and ventricular septal defects without MAPCAs require repeat intervention on the right ventricular outflow tract. Hypoplastic pulmonary arteries have been shown to be a risk factor for reintervention, and decreased conduit durability has been shown with younger age at implantation of conduit. CONCLUSIONS: Long-term outcomes have improved for patients with pulmonary atresia-ventricular septal defect without MAPCAs, with complete repair and adequate pulmonary arteries favorable for survival. Long-term outcomes include reinterventions, both catheter-based and surgical, predominantly on the right ventricular outflow tract.