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Background: Although pregnancy outcomes in women with normally functioning bioprosthetic valves (BPVs) are often good, structural valve dysfunction (SVD) may adversely affect pregnancy outcomes, but this has not been studied.Objectives: The aim of this study was to examine outcomes in pregnant women with BPVs and the association with SVD.Methods: Pregnancy outcomes in women with BPVs were prospectively collected. Adverse maternal cardiac events (CEs) included cardiac death or arrest, sustained arrhythmia, heart failure, thromboembolism, and stroke. Adverse fetal events were also studied. Determinants of adverse events were examined using logistic regression.Results: Overall, 125 pregnancies in women with BPVs were included, 27% with left-sided and 73% with right-sided BPV. SVD was present in 27% of the pregnancies (44% with left-sided BPVs vs 21% with right-sided BPVs; P = 0.009). CEs occurred in 13% of pregnancies and were more frequent in women with SVD compared with those with normally functioning BPVs (26% vs 8%; P = 0.005). CEs were more common in women with left-sided BPVs with SVD vs normally functioning BPVs (47% vs 5%; P = 0.01) but not in women with right-sided BPVs (11% in those with SVD vs 8% in those without SVD; P = 0.67). Left-sided SVD (P = 0.007), maternal age >35 years (P = 0.001), and a composite variable of "high-risk" features (P = 0.006) were predictors of CEs. Fetal events occurred in 28% of pregnancies.Conclusions: In this cohort of young women with BPVs, SVD was present in 27% at the first antenatal visit and negatively affected pregnancy outcomes. In particular, SVD of left-sided BPVs was associated with high rates of adverse outcomes. [ABSTRACT FROM AUTHOR]

Pregnancy is a period of increased cardiovascular risk in a woman's life. In the setting of an inherited arrhythmia syndrome (IAS), cardiologists and obstetricians may be unfamiliar with cardiovascular optimization and risk stratification in pregnancy. Historically, there were little data addressing the safety of pregnancy in these rare disorders. Recent advances suggest that no type of IAS represents an absolute contraindication to pregnancy. However, it is imperative that obstetric and cardiovascular clinicians understand the major forms of IAS and how they affect the risks and course of pregnancy. This includes any disease-specific proarrhythmic triggers unique to pregnancy, such as the postpartum period in long QT syndrome (especially type 2), which poses the greatest risk of arrhythmias, and the adrenergic nature of labor and delivery, which is relevant to catecholaminergic polymorphic ventricular tachycardia. Fortunately, several effective antiarrhythmic options exist that pose little fetal risk. IAS-specific optimization of implantable cardioverter-defibrillator algorithms, drug therapy, and a maternal cardiac plan addressing the antepartum, labor, and delivery and postpartum periods reduces the risk. Where evidence does not exist, there are plausible mechanistic considerations to guide clinicians. To achieve optimal outcomes, early involvement of an expert pregnancy heart team comprising obstetrics, genetics, cardiology, and anesthesiology team members and a shared decision-making approach to IAS issues in pregnancy are needed. [ABSTRACT FROM AUTHOR]

Background: Glomerular filtration rate is a key physiologic variable with a central role in clinical decision making and a strong association with prognosis in diverse populations. Reduced estimated glomerular filtration rate (eGFR) is common among adults with congenital heart disease (ACHD).Methods: We conducted a prospective cohort study of outpatient ACHD ≥18 years old seen in 2012-2017. Creatinine and cystatin C were measured; eGFR was calculated using either the creatinine or cystatin C Chronic Kidney Disease-Epidemiology Collaboration equation (CKD-EPICr and CKD-EPICysC, respectively). Survival analysis was performed to define the relationship between eGFR and both all-cause mortality and a composite outcome of death or nonelective cardiovascular hospitalization.Results: Our cohort included 911 ACHD (39 ± 14 years old, 49% female). Mean CKD-EPICr and CKD-EPICysC were similar (101 ± 20 vs 100 ± 23 mL/min/1.73 m2), but CKD-EPICr estimates were higher for patients with a Fontan circulation (n = 131, +10 ± 19 mL/min/1.73 m2). After mean follow-up of 659 days, 128 patients (14.1%) experienced the composite outcome and 31 (3.4%) died. CKD-EPICysC more strongly predicted all-cause mortality (eGFR <60 vs >90 mL/min/1.73 m2: CKD-EPICysC unadjusted HR = 20.2 [95% CI 7.6-53.1], C-statistic = 0.797; CKD-EPICr unadjusted HR = 4.6 [1.7-12.7], C-statistic = 0.620). CKD-EPICysC independently predicted the composite outcome, whereas CKD-EPICr did not (CKD-EPICysC adjusted HR = 3.0 [1.7-5.3]; CKD-EPICr adjusted HR = 1.5 [0.8-3.1]). Patients reclassified to a lower eGFR category by CKD-EPICysC, compared with CKD-EPICr, were at increased risk for the composite outcome (HR = 2.9 [2.0-4.3], P < .0001); those reclassified to a higher eGFR class were at lower risk (HR = 0.5 [0.3-0.9], P = .03).Conclusions: Cystatin C-based eGFR more strongly predicts clinical events than creatinine-based eGFR in ACHD. Creatinine-based methods appear particularly questionable in the Fontan circulation. [ABSTRACT FROM AUTHOR]

Background: Identifying women at high risk is an important aspect of care for women with heart disease.Objectives: This study sought to: 1) examine cardiac complications during pregnancy and their temporal trends; and 2) derive a risk stratification index.Methods: We prospectively enrolled consecutive pregnant women with heart disease and determined their cardiac outcomes during pregnancy. Temporal trends in complications were examined. A multivariate analysis was performed to identify predictors of cardiac complications and these were incorporated into a new risk index.Results: In total, 1,938 pregnancies were included. Cardiac complications occurred in 16% of pregnancies and were primarily related to arrhythmias and heart failure. Although the overall rates of cardiac complications during pregnancy did not change over the years, the frequency of pulmonary edema decreased (8% from 1994 to 2001 vs. 4% from 2001 to 2014; p value = 0.012). Ten predictors of maternal cardiac complications were identified: 5 general predictors (prior cardiac events or arrhythmias, poor functional class or cyanosis, high-risk valve disease/left ventricular outflow tract obstruction, systemic ventricular dysfunction, no prior cardiac interventions); 4 lesion-specific predictors (mechanical valves, high-risk aortopathies, pulmonary hypertension, coronary artery disease); and 1 delivery of care predictor (late pregnancy assessment). These 10 predictors were incorporated into a new risk index (CARPREG II [Cardiac Disease in Pregnancy Study]).Conclusions: Pregnancy in women with heart disease continues to be associated with significant morbidity, although mortality is rare. Prediction of maternal cardiac complications in women with heart disease is enhanced by integration of general, lesion-specific, and delivery of care variables. [ABSTRACT FROM AUTHOR]

Objectives The aims of this study were to determine D-shaped mitral annulus (MA) dimensions in control subjects without significant cardiac disease and in patients with moderate to severe mitral regurgitation (MR) being considered for transcatheter mitral therapy and to determine predictors of annular size, using cardiac computed tomography. Background The recently introduced D-shaped method of MA segmentation represents a biomechanically appropriate approach for annular sizing prior to transcatheter mitral valve implantation. Methods Patients who had retrospectively gated cardiac computed tomography performed at our institution (2012 to 2014) and were free of significant cardiac disease were included as controls (n = 88; 56 ± 11 years of age; 47% female) and were compared with patients with moderate or severe MR due to functional mitral regurgitation (FMR) (n = 27) or mitral valve prolapse (MVP) (n = 32). MA dimensions (projected area, perimeter, intercommissural, and septal-to-lateral distance), maximal left atrial (LA) volumes, and phasic left ventricular volumes were measured. Results MA dimensions were larger in patients with FMR or MVP compared with controls (area index 4.7 ± 0.6 cm 2 /m 2 , 6.0 ± 1.3 cm 2 /m 2 , and 7.3 ± 1.7 cm 2 /m 2 ; perimeter index 59 ± 5 mm/m 2 , 67 ± 9 mm/m 2 , and 75 ± 10 mm/m 2 ; intercommissural distance index 20.2 ± 1.9 mm/m 2 , 21.2 ± 3.1 mm/m 2 , and 24.7 ± 3.2 mm/m 2 ; septal-to-lateral distance index 14.8 ± 1.6, 18.1 ± 3.3, and 19.5 ± 3.4 mm/m 2 in controls and patients with FMR and MVP, respectively; p < 0.05 between controls and MR subgroups). Absolute MA area was 18% larger in patients with MVP than patients with FMR (13.0 ± 2.9 cm 2 vs. 11.0 ± 2.3 cm 2 ; p = 0.006). Although LA and left ventricular volumes were both independently associated with MA area index in controls and patients with MVP, only LA volume was associated with annular size in patients with FMR. Conclusions Moderate to severe MR was associated with increased MA dimensions, especially among patients with MVP compared with control subjects without cardiac disease. Moreover, unlike in controls and patients with MVP, annular enlargement in FMR was more closely associated with LA dilation. [ABSTRACT FROM AUTHOR]

Background: Tetralogy of Fallot (TOF) is a complex congenital heart disease with clinical and genetic heterogeneity. Of the few known causes, 22q11.2 deletion syndrome (22q11DS) is the most common. We sought to define other clinical subgroups by focusing on cardiac and extracardiac features. Methods: We prospectively screened a cohort of adults with TOF using an established protocol by which subjects were categorized as “syndromic” if they had at least 2 of 3 features: dysmorphic facies, learning difficulties, or voice abnormalities. We then compared the prevalence of cardiac and extracardiac features between subjects in the syndromic group (n = 56) and 112 age- and gender-matched subjects who did not meet our syndromic criteria. Results: The syndromic group was more likely than the nonsyndromic group to have pulmonary atresia and/or major aortopulmonary collateral arteries (25% vs 13%, P = .04). There was a trend toward a higher prevalence of one or more major congenital extracardiac anomalies, primarily involving the musculoskeletal and genitourinary systems (25% vs 13%, P = .06). Later-onset conditions, including neuropsychiatric disorders (32% vs 17%, P = .03), thyroid disorders (20% vs 4%, P = .001), and hearing deficits (20% vs 0, P < .001), were more common in the syndromic group. The syndromic group tested (n = 50) had neither 22q11.2 deletions nor karyotypic anomalies. Conclusion: Similar to 22q11DS, adults with TOF meeting screening criteria for a possible genetic syndrome are enriched for more severe cardiac disease and late-onset extracardiac features. Increased awareness of this subgroup with a multisystem condition may be helpful for identifying individuals for referral to medical genetics and optimizing management. [Copyright &y& Elsevier]

Objectives: The objectives of this study were to determine adverse outcomes during pregnancy in women with dilated cardiomyopathy (DCM) and to compare their cardiac outcomes with those of nonpregnant women with DCM. Background: Women with DCM are at risk for complications during pregnancy, but few studies have examined outcomes in this specific population. Methods: This was a substudy of a larger prospective cohort study of outcomes in women with heart disease. Maternal cardiac, obstetric, and fetal outcomes in pregnancy in women with DCM were examined. For comparison, cardiac outcomes in nonpregnant women with DCM (n = 18) matched by age and left ventricular (LV) systolic function were examined. A matched-pair survival analysis was used to compare groups. Results: Thirty-six pregnancies in 32 women with DCM were included. Thirty-nine percent (14 of 36) of the pregnancies were complicated by at least 1 maternal cardiac event. In the multivariate analysis, moderate or severe LV dysfunction and/or New York Heart Association functional class III or IV (p = 0.003) were the main determinants of adverse maternal cardiac outcomes during pregnancy. In the subset of women with moderate/severe LV dysfunction, 16-month event-free survival was worse in pregnant women compared with nonpregnant women (28 ± 11% vs. 83 ± 10%, p = 0.02). The adverse neonatal event rate was highest among women with obstetric and cardiac risk factors (43%). Conclusions: In pregnant women with DCM the risk of adverse cardiac events is considerable, and pre-pregnancy characteristics can identify women at the highest risk. Pregnancy seems to have a short-term negative impact on the clinical course in women with DCM. [Copyright &y& Elsevier]

Background: Severe mitral regurgitation (MR) leads to progressive enlargement of left ventricular dimensions and, consequently, the mitral valve (MV) annulus. Data from animal and cadaver studies suggest that the mitral annulus may dilate asymmetrically in certain conditions, which may influence the choice of valve repair technique. Although it is generally accepted that the posterior mitral annulus dilates in patients with severe MR due to leaflet prolapse, the stability of the anterior intertrigonal distance has not yet been demonstrated in humans. Methods: We obtained real-time, three-dimensional (3D) transesophageal echocardiographic images of the MV in 44 patients: 29 patients scheduled to undergo MV repair for severe MR due to leaflet prolapse (MV disease group) and 15 normal outpatients undergoing evaluation for various reasons (control group). Mitral valve repair was performed by median sternotomy or minimally invasively using thoracoscopic or robotic assistance. All patients underwent implantation of a standard-length flexible 63-mm posterior annuloplasty band at the time of mitral repair and we obtained postoperative 3D images for 11 patients after separation from bypass. Mitral annular dimensions were measured throughout the cardiac cycle using reconstructive analysis software (QLAB MVQ Version 6.0; Phillips, Bothell, WA). Results: The mean patient age was 60 years; 30 were men. The mean ejection fraction was 0.61 and was similar between the two groups (p = 0.16). In patients with MR due to leaflet prolapse, posterior annular length and total annular circumference were significantly larger than in control patients (p < 0.001). In contrast, there was no detectable difference in the anterior intertrigonal distance between patients with MR and normal controls. After mitral valve leaflet repair and posterior annuloplasty there was a significant decrease in both the total annular circumference and posterior annular length (p < 0.0001) while cyclic annular contraction was preserved. Conclusions: Although the posterior mitral annulus is enlarged in patients with significant MR due to degenerative leaflet prolapse, there is no evidence that the intertrigonal distance is abnormal in these patients. Our data support the conclusion that posterior annular reduction with a flexible device at the time of mitral valve repair is important, and that altering the anterior intertrigonal portion of the mitral annulus is unnecessary. [Copyright &y& Elsevier]

More than 40% of patients hospitalized with heart failure have preserved left ventricular ejection fraction (HF-PLVEF) and are at high risk for cardiovascular (CV) events. The purpose of this study was to determine the value of N-terminal pro–brain natriuretic peptide (NT-proBNP) and brain natriuretic peptide (BNP) in predicting CV outcomes in patients with HF-PLVEF. Participants with an ejection fraction >40% in the prospective CHARM Echocardiographic Substudy were included in this analysis. Plasma NT-proBNP levels were measured, and 2 cut-offs were selected prospectively at 300 pg/ml and 600 pg/ml. BNP cut-off was set at 100 pg/ml. Clinical characteristics were recorded, and systolic and diastolic function were evaluated by echocardiography. The primary substudy outcome was the composite of CV mortality, hospitalization for heart failure, and myocardial infarction or stroke. A total of 181 patients were included, and there were 17 primary CV events (9.4%) during a median follow-up time of 524 days. In a model including clinical characteristics, echocardiographic measures, and BNP or NT-proBNP, the composite CV event outcome was best predicted by NT-proBNP >300 pg/ml (hazard ratio 5.8, 95% confidence intervals [CI] 1.3 to 26.4, p = 0.02) and moderate or severe diastolic dysfunction on echocardiography. When NT-proBNP >600 pg/ml was used in the model, it was the sole independent predictor of primary CV events (hazard ratio 8.0, 95% CI 2.6 to 24.8, p = 0.0003) as was BNP >100 pg/ml (hazard ratio 3.1, 95% CI 1.2 to 8.2, p = 0.02) in the BNP model. In conclusion, both elevated NT-proBNP and BNP are strong independent predictors of clinical events in patients with HF-PLVEF. [Copyright &y& Elsevier]

Abstract: Background: Current approaches to cardiovascular (CV) risk assessment have limitations. Subclinical atherosclerosis (SCA) as determined by carotid ultrasound is an independent predictor of myocardial infarction and stroke and can refine CV risk assessment. Objectives: We aimed to determine the prevalence and predictors of SCA in a multiethnic population classified as low risk for coronary heart disease (CHD) events by the Framingham Risk Assessment Model. Methods: We conducted a cross-sectional population study in 1015 Canadian adults of Caucasian European, South Asian, Chinese and Aboriginal ancestry. CHD risk was calculated by the 10-year Framingham Risk Score (FRS). Novel and conventional CHD risk factors were measured and high-resolution carotid ultrasound was performed. SCA was defined as carotid intima media thickness (IMT) ≥75th percentile adjusted for age, sex and ethnicity. Results: Seven hundred and fifty two (74%) participants were classified as low risk by FRS. Of these, 175 (23%) had evidence of SCA. Independent predictors of SCA among low-risk subjects included female sex, systolic blood pressure, and apolipoprotein B. Conclusions: Many individuals classified at low CHD risk by the FRS have SCA and are at increased long-term risk for vascular events. Carotid IMT can identify subjects with SCA, who may benefit from early intervention. [Copyright &y& Elsevier]

The uncertain significance of hazy areas at the margins of coronary stents may lead to further, at times unnecessary, stenting. However, the risk of restenosis increases substantially when additional stents are deployed. We used intravascular ultrasound (IVUS) to identify the causes of hazy segments adjacent to stents. We identified 13 cases with hazy regions adjacent to coronary stents and 20 controls without hazy regions matched by age, gender, and vessel stented. Hazy regions were defined from the angiogram as reduced contrast density without a clearly defined intimal tear, dissection, thrombus, or stenosis (> 50%). IVUS images were obtained from the reference, stent, and hazy and control regions adjacent to the stent. Computerized planimetery was used to measure the vessel, lumen, and plaque cross-sectional areas (CSAs), the maximum arc of calcium, and the eccentricity ratio (minimum:maximum lumen diameter). There were no significant differences between hazy and control segments in the vessel, lumen, and plaque CSAs. All lumen CSAs were >4.0 mm2. Compared with control regions, the hazy regions had calcified plaque more often (69% vs 25%; odds ratio [OR] 6.75, 95% confidence intervals [CI] 1.82 to 25.0]) and more frequent intimal tears (23% vs 0%, OR 6.67, 95% CI 1.98 to 35.0). Haziness was particularly associated with calcified plaque and eccentric lumen (p = 0.037). Thus, haziness at the margins of coronary stents is often caused by calcified plaque. IVUS can differentiate calcified plaques from intimal tears and thereby obviate unnecessary stenting. [ABSTRACT FROM AUTHOR]

Background: Pregnancies in women with regurgitant valve lesions are generally considered low risk, but this has not been well studied.Objectives: This study determined the frequency of adverse cardiac events (CEs) in pregnant women with moderate or severe regurgitant valve lesions.Methods: Maternal and fetal outcomes in women with moderate or severe chronic valve regurgitation enrolled in a prospective multicenter study on pregnancy outcomes were examined. Adverse CEs included heart failure, sustained arrhythmias, cardiac arrest, or death. A multivariate logistic regression model was used to identify determinants of CEs in women at the highest risk.Results: Outcomes of 430 pregnancies in women with moderate or severe regurgitant lesions were examined: 145 with mitral regurgitation (MR), 101 with pulmonary regurgitation (PR), 71 with multivalve disease, 73 with tricuspid regurgitation (TR), and 40 with aortic regurgitation (AR). Most women had associated congenital or acquired heart disease. Adverse CEs occurred in 13% of pregnancies: 27% of pregnancies with multivalve disease; 15% with MR; 15% with TR; 5% with AR; and 3% with PR. Maternal mortality was rare. In women with MR, TR, or multivalve disease (n = 289), left ventricular systolic dysfunction (p = 0.001), pulmonary hypertension (p = 0.005), and cardiac events before pregnancy (p < 0.001) were important determinants of CEs during pregnancy.Conclusions: Women with AR and PR are at low risk for cardiac complications during pregnancy. While many women with MR, TR, and multivalve regurgitation do well during pregnancy, additional clinical variables help stratify those at highest risk. This new information will enhance the quality and precision of preconception counseling and pregnancy planning. [ABSTRACT FROM AUTHOR]

Background: Pregnancy can lead to complications in women with heart disease, and these complications can be life threatening. Understanding serious complications and how they can be prevented is important.Objectives: The primary objectives were to determine the incidence of serious cardiac events (SCEs) in pregnant women with heart disease, whether they were preventable, and their impact on fetal and neonatal outcomes. Serious obstetric events were also examined.Methods: A prospectively assembled cohort of 1,315 pregnancies in women with heart disease was studied. SCEs included cardiac death or arrest, ventricular arrhythmias, congestive heart failure or arrhythmias requiring admission to an intensive care unit, myocardial infarction, stroke, aortic dissection, valve thrombosis, endocarditis, and urgent cardiac intervention. The Harvard Medical Study criteria were used to adjudicate preventability.Results: Overall, 3.6% of pregnancies (47 of 1,315) were complicated by SCEs. The most frequent SCEs were cardiac death or arrest, heart failure, arrhythmias, and urgent interventions. Most SCEs (66%) occurred in the antepartum period. Almost one-half of SCEs (49%) were preventable; the majority of preventable SCEs (74%) were secondary to provider management factors. Adverse fetal and neonatal events were more common in pregnancies with SCEs compared with those without cardiac events (62% vs. 29%; p < 0.001). Serious obstetric events were less common (1.7%) and were primarily due to pre-eclampsia with severe features.Conclusions: Pregnant women with heart disease are at risk for serious cardiac complications, and approximately one-half of all SCEs are preventable. Strategies to prevent serious cardiac complications in this high-risk cohort of women need to be developed. [ABSTRACT FROM AUTHOR]

Comprehensive assessment of tetralogy of Fallot (TOF) outcomes extends beyond morbidity and mortality to incorporate patient-reported outcomes (PROs), including quality of life (QOL) and health status (HS). This study explored PROs in adolescents and adults with TOF and delineated variables associated with PROs. This was a cross-sectional observational study within a larger prospective registry of adolescents and adults with repaired TOF and moderate or greater pulmonary regurgitation from North America, Europe, and Asia. Participants completed PROs, including a QOL linear analogue scale (QOL-LAS) and an HS visual analogue scale (HS-VAS). Scores were classified according to age cohorts: <18, 18 to 25, 26 to 40, and >40 years. The study included 607 patients (46.3% female; median age 28.5 years). Median QOL-LAS scores (0-100) were similar across age cohorts (85, 80, 80, 80; P = 0.056). Median HS-VAS scores (0-100) were lowest for the oldest cohort (77) compared with the 3 younger cohorts (85, 80, 80) (P = 0.004). With advancing age, there were increased reports of poor mobility (P < 0.001) and pain or discomfort (P = 0.004); problems in these dimensions were reported by 19.1% and 37.2% of patients aged >40 years, respectively. Of factors associated with superior PROs on multivariable regression modeling (ie, being White, being nonsyndromic, having employment, and having better left ventricular function; P < 0.05), asymptomatic status (functional class I) was the variable associated with the greatest number of QOL and HS measures (P < 0.001). Strategies to improve TOF outcomes should consider PROs alongside conventional clinical variables. Factors associated with poorer PROs represent opportunities to intervene to improve the lives of patients with TOF. [Display omitted] [ABSTRACT FROM AUTHOR]

Heart failure (HF) is the leading cause of death in adult patients with congenital heart disease (ACHD). No risk prediction model exists for HF hospitalization (HFH) for ACHD patients. We aimed to develop a clinically relevant one-year risk prediction system to identify ACHD patients at high risk for HFH. Data source was the Quebec CHD Database. A retrospective cohort including all ACHD patients aged 18–64 (1995–2010) was constructed for assessing the cumulative risk of HFH adjusting for competing risk of death. To identify one-year predictors of incident HFH, multivariable logistic regressions were employed to a nested case-control sample of all ACHD patients aged 18–64 in 2009. The final model was used to create a risk score system based on adjusted odds ratios. The cohort included 29,991 ACHD patients followed for 648,457 person-years. The cumulative HFH risk by age 65 was 12.58%. The case-control sample comprised 26,420 subjects, of whom 189 had HFHs. Significant one-year predictors were age ≥ 50, male sex, CHD lesion severity, recent 12-month HFH history, pulmonary arterial hypertension, chronic kidney disease, coronary artery disease, systemic arterial hypertension, and diabetes mellitus. The created risk score ranged from 0 to 19. The corresponding HFH risk rose rapidly beyond a score of 8. The risk scoring system demonstrated excellent prediction performance. One eighth of ACHD population experienced HFH before age 65. Age, sex, CHD lesion severity, recent 12-month HFH history, and comorbidities constructed a risk prediction model that successfully identified patients at high risk for HFH. • Heart failure (HF) is the leading cause of death and hospitalization in ACHD patients. • No risk prediction model exists for HF hospitalizations (HFH) in ACHD patients. • Age, sex, CHD severity, recent HFH history, and comorbidities are predictors of one-year HFH in ACHD patients. • Weighting these independent predictors, we created a risk score that accurately predicts HFH in ACHD patients. [ABSTRACT FROM AUTHOR]

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

The short lifespan of right ventricleetoepulmonary artery (RV-PA) conduits used in repairs of complex congenital heart defects makes future surgical replacement inevitable. Percutaneous pulmonary valve implantation (PPVI) now offers an attractive alternative to surgery in some patients. The objectives of this study were to examine the pattern of conduit reoperations, the factors affecting conduit longevity, and to discuss the role of PPVI in these patients. Forty-nine patients (mean age 27±8 years) with pulmonary atresia or pulmonary stenosis with tetralogy of Fallot who underwent surgery for RV-PA conduits from September 1974 to October 2011 were reviewed. A total of 106 RV-PA conduits were implanted, 57 of which were replacements. Second, third, and fourth conduits were required during the follow-up period in 39, 16, and 2 patients, respectively. Freedom from reoperation at 10 years for the first, second, and third conduits was 50%, 74%, and 86%, respectively. Significant independent predictors of shorter conduit longevity included smaller conduit and conduit type (homograft and other vs Dacron). Furthermore, a significant proportion (32 of 57 [56%]) of conduit replacements took place from ages 9 to 18 years. There were 37 adults whose current existing conduits had not yet failed, with 73% (27 of 37) potentially suitable in the future for PPVI on the basis of conduit size of 16 to 27 mm. In conclusion, multiple RVPA conduit revisions were required in patients who survived to adulthood, with many replacements taking place during adolescence. Most conduits in this adult cohort met size criteria for PPVI, thereby offering these patients a potential alternative to surgical intervention for conduit failure. [ABSTRACT FROM AUTHOR]

Objectives: The aim of this study was to evaluate the impact of mitral regurgitation (MR) on outcomes after transcatheter aortic valve replacement (TAVR) and the impact of TAVR on MR. Background: Little is known of the influence of MR on outcomes after TAVR. Methods: The outcomes of patients with mild or less (n = 319), moderate (n = 89), and severe (n = 43) MR were evaluated after TAVR at 2 Canadian centers. Results: Patients with moderate or severe MR had a higher mortality rate than those with mild or less MR during the 30 days after TAVR (adjusted hazard ratio: 2.10; 95% confidence interval: 1.12 to 3.94; p = 0.02). However, the mortality rates after 30 days were similar (adjusted hazard ratio: 0.82; 95% confidence interval: 0.50 to 1.34; p = 0.42). One year after TAVR, moderate MR had improved in 58%, remained moderate in 17%, and worsened to severe in 1%, and 24% of patients had died. Severe MR had improved in 49% and remained severe in 16%, and 35% of patients had died. Multivariate predictors of improved MR at 1 year (vs. unchanged MR, worse MR, or death) were a mean transaortic gradient ≥40 mm Hg, functional (as opposed to structural) MR, the absence of pulmonary hypertension, and the absence of atrial fibrillation. Conclusions: Moderate or severe MR in patients undergoing TAVR is associated with a higher early, but not late, mortality rate. At 1-year follow-up, MR was improved in 55% of patients with moderate or severe MR at baseline. Improvement was more likely in patients with high transaortic gradients, with functional MR, without pulmonary hypertension and without atrial fibrillation. [ABSTRACT FROM AUTHOR]