Adverse fetal/neonatal and obstetric outcomes in pregnancies with both maternal and fetal heart disease.

OBJECTIVE: To investigate fetal/neonatal and obstetric events in pregnancies with both maternal and fetal heart disease. STUDY DESIGN: From the CARPREG database, singleton pregnancies (>24 weeks) in patients with structural heart disease that underwent fetal/neonatal echocardiograms were selected and separated in two groups: maternal heart disease only (M-HD) and maternal and fetal heart disease (MF-HD). Differences in adverse fetal/neonatal (death, preterm birth, and small for gestational age) and obstetric (preeclampsia/eclampsia) outcomes between groups were analyzed. RESULTS: From 1011 pregnancies, 93 had MF-HD. Fetal/neonatal events (38.7% vs 25.3%, p = 0.006) and spontaneous preterm birth (10.8% vs 4.9%, p = 0.021) were more frequent in MF-HD compared to M-HD, with no difference in obstetric events. MF-HD remained as a significant predictor of fetal/neonatal events after adjustment (OR:1.883; 95% CI:1.182-3.000; p = 0.008). CONCLUSIONS: Pregnancies with MF-HD are at risk of adverse fetal/neonatal events and spontaneous preterm birth. Larger studies are needed to determine their association with preeclampsia.

Development and evolution of nuclear cardiology and cardiac PET in Canada.

Gated radionuclide angiography and myocardial perfusion imaging were developed in the United States and Europe in the 1970's and soon adopted in Canadian centers. Much of the early development of nuclear cardiology in Canada was in Toronto, Ontario and was quickly followed by new programs across the country. Clinical research in Canada contributed to the further development of nuclear cardiology and cardiac PET. The Canadian Nuclear Cardiology Society (CNCS) was formed in 1995 and became the Canadian Society of Cardiovascular Nuclear and CT Imaging (CNCT) in 2014. The CNCS had a major role in education and advocacy for cardiovascular nuclear medicine testing. The CNCS established the Dr Robert Burns Lecture and CNCT named the Canadian Society of Cardiovascular Nuclear and CT Imaging Annual Achievement Award for Dr Michael Freeman in memoriam of these two outstanding Canadian leaders in nuclear cardiology. The future of nuclear cardiology in Canada is exciting with the expanding use of SPECT imaging to include Tc-99m-pyrophosphate for diagnosis of transthyretin cardiac amyloidosis and the ongoing introduction of cardiac PET imaging.

Outcomes of Pregnancy in Women With Bioprosthetic Heart Valves With or Without Valve Dysfunction.

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.

Trending Cardiac Biomarkers During Pregnancy in Women With Cardiovascular Disease.

BACKGROUND: Clinical utility of cardiac biomarker testing during pregnancy in women with preexisting cardiac disease is not well known. We studied the levels and temporal trends of NT-proBNP (N-terminal pro-B-type natriuretic peptide) and hs-cTnI (high-sensitivity cardiac troponin I) throughout pregnancy in women with preexisting cardiac disease and sought to assess the association between NT-proBNP and hs-cTnI and pregnancy outcomes. METHODS: Three hundred seven pregnant women with preexisting cardiac disease were prospectively recruited. Mixed-effects linear regression analysis was used to compare the NT-proBNP and hs-cTnI levels between time periods and subgroups. Logistic regression analysis adjusted for maternal age and CARPREG II (Cardiac Disease in Pregnancy) risk score assessed the association between NT-proBNP levels and adverse events. RESULTS: Geometric mean NT-proBNP (95% CI) was stable through pregnancy with a transient significant increase with labor and delivery (101.4 pg/mL [87.1-118.1], 90.2 pg/mL [78.5-103.6], 153.6 pg/mL [126.8-186.1], and 112.2 pg/mL [94.2-133.7] for first/second trimester, third trimester, labor/delivery and postpartum, respectively). We observed a statistically significant difference in the NT-proBNP between women with preserved versus decreased systemic ventricular function, structurally normal versus abnormal heart, modified World Health Organization class 1, 2 versus modified World Health Organization class 3, 4 and no congenital heart disease versus congenital heart disease. Compared to those without events, median (interquartile range) NT-proBNP levels were significantly higher in those who had heart failure (204 pg/mL [51-450] versus 55 pg/mL [31-97]; P=0.001) and preeclampsia (98 pg/mL [40-319] versus 55 pg/mL [31-99]; P=0.027). NT-proBNP, adjusted for age and CARPREG II risk score, was significantly associated with combined heart failure and preeclampsia (adjusted odds ratio, 2.14 [95% CI, 1.48-3.10] per log NT-proBNP increase; P<0.001). NT-proBNP <200 pg/mL had a specificity of 91% and negative predictive value of 95% in predicting combined heart failure and preeclampsia. CONCLUSIONS: NT-proBNP remains steady over the course of pregnancy with a transient increase during labor and delivery with higher levels in subgroups of stable cardiac patients. NT-proBNP level of 200 pg/mL can be used in the diagnosis of heart failure/preeclampsia in the pregnant cardiac population.

Psychological Distress in Adults With Congenital Heart Disease Over the COVID-19 Pandemic.

Background This study sought to better understand the experiences of adults with congenital heart disease throughout the pandemic. Objectives were to determine (1) psychological distress before and throughout the pandemic; (2) changes in day-to-day functioning; and (3) the percentage of adults with congenital heart disease who experienced COVID-19 related symptoms, underwent testing, and tested positive. Methods and Results This was a cross-sectional study paired with retrospective chart review. A web-based survey was distributed to patients between December 2020 and January 2021. Patients reported on psychological distress across 5 categories (Screening Tool for Psychological Distress; depression, anxiety, stress, anger, and lack of social support), whether they experienced symptoms of COVID-19 and/or sought testing, and changes to their work and social behavior. Five hundred seventy-nine survey responses were received, of which 555 were linked to clinical data. Patients were aged 45±15 years. The proportion of patients reporting above-threshold values for all Screening Tool for Psychological Distress items significantly increased during the early pandemic compared with before the pandemic. Stress returned to baseline in December 2020/January 2021, whereas all others remained elevated. Psychological distress decreased with age, and women reported persistently elevated stress and anxiety compared with men during the pandemic. A consistent trend was not observed with regard to American College of Cardiology/American Heart Association anatomic and physiologic classification. Fifty (9%) patients lost employment because of a COVID-19-related reason. COVID-19 symptoms were reported by 145 (25%) patients, 182 (31%) sought testing, and 10 (2%) tested positive. Conclusions A substantial proportion of adults with congenital heart disease reported clinically significant psychological distress during the pandemic.

Increasing age and atrial arrhythmias are associated with increased thromboembolic events in a young cohort of adults with repaired tetralogy of Fallot.

BACKGROUND: Adults with repaired Tetralogy of Fallot (rTOF) comprise one of the largest cohorts among adults with congenital heart disease (ACHD). These patients have a higher burden of atrial arrhythmias (AA), leading to increased adverse events, including stroke and transient ischemic attack (TIA). However, the data on factors associated with stroke/TIA in rTOF are limited, and classic risk factors may not apply. We studied event rates and associated factors for thromboembolism in a rTOF cohort. METHODS: Retrospective cohort study of all adult patients age >18 years with rTOF followed at a single ACHD tertiary care center. AA of interest were atrial fibrillation (AF) and atrial flutter (AFL). RESULTS: Data from 260 patients were identified, mean age 37.6 SD 13.3 years, followed over 5108 patient-years (mean 16.6 SD 8.2 years). 43 patients had AF and/or AFL, and 30 patients had thromboembolic events, of which 19 patients had stroke/TIA. The event rate for any thromboembolism was 3.39 per 100 patient-years follow-up in patients with AA, compared to 1.80 in patients without (P = .07). In univariate analysis, older age and diabetes were associated with thromboembolic events. In multivariate analysis, only older age was associated with thromboembolic events. CONCLUSIONS: In our relatively young cohort of adults with rTOF, there was a high prevalence of AA, associated with nearly double the rate of thromboembolic events compared to patients without AA. Older age alone is independently associated with thromboembolic events. Further studies into assessment of silent AA are required, and routine assessments should be considered at an earlier age.

Pregnancy related changes in Doppler gradients and left ventricular mechanics in women with sub-valvular or valvular aortic stenosis.

BACKGROUND: The aim of our study was to characterize echocardiographic changes during pregnancy in women with known LVOT obstruction or AS compared to the healthy pregnancy controls, and to assess the relationship with pregnancy outcomes. METHODS: We retrospectively studied 34 pregnant patients with congenital LVOT obstruction or AS with healthy age-matched pregnant controls. Patients with other significant valvular lesions, structural heart disease (LVEF < 40%), or prior valve surgery were excluded. All LVOTO/AS patients underwent a minimum of two consecutive echocardiograms between 1 year pre-conception and 1 year postpartum, with at least two studies during the pregnancy. Comprehensive echocardiographic evaluation was performed including speckle-tracking LV global longitudinal strain. RESULTS: A total of 83 echocardiograms from the study group and 34 echocardiograms from the control group were evaluated. Over the range of LVOTO/AS, a significantly greater increase in the AV gradients and LV and LA volumes were observed as compared with the controls. In the sub-group of LVOTO/AS pregnant women with ≥ moderate (n = 8) versus < moderate LVOTO/AS (n = 26), averaged 2nd /3rd trimester LVEF was lower (51 ± 12)% versus (58 ± 4)%, (p = 0.02) and GLS was lower (-19.5 ± 2.8) versus (21.2 ± 2.4), (p = 0.06). Pregnancy was well tolerated despite these changes. CONCLUSION: Among pregnant women with even milder forms of LVOTO/AS, increases in cardiac volumes and AV gradients can be expected over the course of pregnancy. Significant decreases in LV function and mechanics were only observed in women with moderate or greater LVOTO/AS, although still remained in normal range.

Risk Associated With Valvular Regurgitation During Pregnancy.

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.

Aortic Dimensions, Biophysical Properties, and Plasma Biomarkers in Children and Adults with Marfan or Loeys-Dietz Syndrome.

BACKGROUND: Aortic dilation, stiffening, and dissection are common and potentially lethal complications of Marfan syndrome (MFS) and Loeys-Dietz syndrome (LDS), which involve abnormal transforming growth factor beta (TGF-ß) signalling. The relation of aortic dimensions, stiffness, and biomarker levels is unknown. The objective of this study was to measure aortic dimensions, stiffness, TGF-ß and matrix metalloproteinase (MMP) levels, and endothelial function in patients with MFS, and to compare TGF-ß levels in patients with MFS receiving different therapeutic regimens. METHODS: This was a cohort study of 40 MFS and 4 LDS patients and 87 control participants. Aortic dimension and stiffness indexes, including pulse wave velocity (PWV), were measured using echocardiography and Doppler. Total and free TGF-ß and MMP blood levels were measured using Quantikine (R&D Systems, Inc, Minneapolis, MN) and Quanterix (Billerica, MA) kits. Endothelial function was measured using brachial artery flow-mediated dilation. RESULTS: PWV was increased in patients with MFS. There were increased MMP-2 levels in those with MFS but no increase in free or total TGF-ß or MMP-9 levels compared with control participants. There was no difference in TGF-ß levels between MFS patients receiving no medications, angiotensin receptor blockers, and ß-blockers. PWV correlated most strongly with age. Endothelial function showed premature gradual decline in patients with MFS. CONCLUSIONS: Despite the increased PWV, monitoring aortic stiffness or TGF-ß levels would not be helpful in patients with MFS. TGF-ß levels were not increased and the increased MMP-2 levels suggest consideration of a different therapeutic target.

CONTEXTE: La dilatation, la rigidification et la dissection de l'aorte sont des complications fréquentes et parfois mortelles du syndrome de Marfan (SM) et du syndrome de Loeys-Dietz (SLD), qui sont tous deux dûs à une anomalie de la voie de signalisation du facteur de croissance transformant bêta (TGF-ß). On ne connaît pas la relation entre les dimensions et la rigidité de l'aorte et la présence de biomarqueurs. Notre étude visait à mesurer les dimensions et la rigidité de l'aorte, les taux de TGF-ß et de métalloprotéases matricielles (MMP) et la fonction endothéliale chez des patients atteints du SM, et à les comparer aux taux de TGF-ß observés chez des patients également atteints de SM, mais recevant un autre traitement. MÉTHODOLOGIE: Il s'agissait d'une étude de cohorte menée auprès de 40 patients atteints du SM et de quatre patients atteints du SLD, ainsi que de 87 témoins. Les indices des dimensions et de la rigidité aortiques, y compris la vitesse d'onde de pouls (VOP), ont été mesurés par échocardiographie et par échographie Doppler. Les taux sanguins de TGF-ß et de MMP totaux et libres ont été mesurés à l'aide de trousses Quantikine (R&D Systems, Inc, Minneapolis, MN) et Quanterix (Billerica, MA). La fonction endothéliale a été mesurée par dilatation liée au flux dans l'artère brachiale. RÉSULTATS: La VOP était plus élevée chez les patients atteints du SM. On a aussi observé une hausse des taux de MMP-2 chez les patients atteints de SM, mais aucune augmentation des taux de TGF-ß ou de MMP-9 libres ou totaux comparativement aux témoins. Il n'y avait pas de différence entre les taux de TGF-ß chez les patients atteints de SM ne recevant aucun traitement, ceux qui prenaient un antagoniste des récepteurs de l'angiotensine et ceux qui prenaient un bêtabloquant. La VOP été plus fortement corrélée avec l'âge. La fonction endothéliale a affiché un déclin progressif prématuré chez les patients atteints du SM. CONCLUSIONS: Malgré l'augmentation de la VOP, il ne semble pas utile de surveiller la rigidité aortique ni les taux de TGF-ß en cas de SM. Les taux de TGF-ß n'étaient pas plus élevés chez les patients atteints du SM, et la hausse des taux de MMP-2 indique qu'il conviendrait de choisir une autre cible thérapeutique.

Cardiac Risk Score to Predict Small for Gestational Age Infants in Pregnant Women With Heart Disease.

BACKGROUND: One of the most common fetal complications in pregnant women with cardiovascular disease is a small for gestational age (SGA) neonate, which is associated with a higher risk of perinatal morbidity/mortality and poor long-term health outcomes. The objective of this study was to identify cardiac determinants and derive a risk score for clinically relevant SGA < 5th percentile (SGA-5th). METHODS: A prospective cohort of 1812 pregnancies in women with heart disease were studied. SGA-5th was the outcome of interest, defined as birth weight < 5th percentile for gestational age and sex. Multivariable logistic regression analysis was used to identify predictors for SGA-5th. Based on the regression coefficients, a weighted risk score was created. RESULTS: SGA-5th complicated 10% of pregnancies, 11 predictors of SGA-5th were identified, and each was assigned a weighted score: maternal cyanosis (8), Fontan palliation (7), smoking (3), moderate or severe valvular regurgitation (3), ß-blocker use throughout pregnancy (4) or only in the 2nd and 3rd trimesters (2), high baseline ß-blocker dose (4), body mass index < 18.5 kg/m2 (3) or 18.5-24.9 kg/m2 (1), Asian/other ethnicity (2), and significant outflow tract obstruction (1). In the absence of these identified risk factors, the risk of SGA-5th was approximately 4%. Pregnancies with risk scores of 1 had a rate of 5%; 2, 7%; 3, 9%; 4, 12%; 5, 14%; 6, 18%; 7, 23%; 8, 28%; and ≥ 9, 34%. CONCLUSIONS: There are a number of cardiac predictors that are associated with increased risk of SGA-5th. This is a prognostically important outcome, and consideration should be given to routinely predicting and modifying the risk whenever possible.

Evolving Paradigms in the Treatment of Atrial Septal Defects With Pulmonary Arterial Hypertension.

Atrial septal defects are one of the most frequently diagnosed congenital heart defects in adulthood. The presence of concurrent moderate or severe pulmonary arterial hypertension without Eisenmenger syndrome at the time of diagnosis can make for a challenging clinical scenario. There is continually evolving literature to determine the ideal approach to this subset of patients. Here we aim to review the clinical presentation, history, medical therapy, and closure options for atrial septal defects-pulmonary arterial hypertension with predominant left-to-right shunting, in the absence of Eisenmenger syndrome.

Atrial arrhythmias and thromboembolic complications in adults post Fontan surgery.

OBJECTIVE: Patients with Fontan surgery experience late complications in adulthood. We studied the factors associated with the development and maintenance of atrial arrhythmias and thromboembolic complications in an adult population with univentricuar physiology post Fontan surgery. METHODS: Single centre retrospective cohort study of patients ≥18 years of age with Fontan circulation followed at our quaternary care centre for more than 1 year were included. Univariate and multivariate regression models were used where applicable to ascertain clinically significant associations between risk factors and complications. RESULTS: 93 patients were included (age 30.2±8.8 years, 58% men). 28 (30%) had atriopulmonary Fontan connection, 35 (37.6%) had lateral tunnel Fontan and 29 (31.1%) had extracardiac Fontan pathway. After a mean of 7.27±5.1 years, atrial arrhythmia was noted in 37 patients (39.8%), of which 13 developed had atrial fibrillation (14%). The presence of atrial arrhythmia was associated with the number of prior cardiac surgeries/procedures, increasing age and prior atriopulmonary Fontan operation. Thromboembolic events were present in 31 patients (33%); among them 14 had stroke (45%), 3 had transient ischaemic attack (9.7%), 7 had pulmonary embolism (22.6%) and 5 had atrial thrombus with imaging (16.1%). The presence of thromboembolic events was only associated with age and the presence of cirrhosis in multivariate analysis. CONCLUSIONS: Atrial arrhythmias are common in adults with Fontan circulation at an early age, and are associated with prior surgical history and increasing age. Traditional risk factors may not be associated with atrial arrhythmia or thromboembolism in this cohort.

Preventing Complications in Pregnant Women With Cardiac Disease.

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.

Tricuspid Intervention Following Pulmonary Valve Replacement in Adults With Congenital Heart Disease.

BACKGROUND: Tricuspid regurgitation (TR) is common among adults with corrected tetralogy of Fallot (TOF) or pulmonary stenosis (PS) referred for pulmonary valve replacement (PVR). Yet, combined valve surgery remains controversial. OBJECTIVES: This study sought to evaluate the impact of concomitant tricuspid valve intervention (TVI) on post-operative TR, length of hospital stay, and on a composite endpoint consisting of 7 early adverse events (death, reintervention, cardiac electronic device implantation, infection, thromboembolic event, hemodialysis, and readmission). METHODS: The national Canadian cohort enrolled 542 patients with TOF or PS and mild to severe TR who underwent isolated PVR (66.8%) or PVR+TVI (33.2%). Outcomes were abstracted from charts and compared between groups using multivariable logistic and negative binomial regression. RESULTS: Median age at reintervention was 35.3 years. Regardless of surgery type, TR decreased by at least 1 echocardiographic grade in 35.4%, 66.9%, and 92.8% of patients with pre-operative mild, moderate, and severe insufficiency. In multivariable analyses, PVR+TVI was associated with an additional 2.3-fold reduction in TR grade (odds ratio [OR]: 0.44; 95% confidence interval [CI]: 0.25 to 0.77) without an increase in early adverse events (OR: 0.85; 95% CI: 0.46 to 1.57) or hospitalization time (incidence rate ratio: 1.17; 95% CI: 0.93 to 1.46). Pre-operative TR severity and presence of transvalvular leads independently predicted post-operative TR. In contrast, early adverse events were strongly associated with atrial tachyarrhythmia, extracardiac arteriopathy, and a high body mass index. CONCLUSIONS: In patients with TOF or PS and significant TR, concomitant TVI is safe and results in better early tricuspid valve competence than isolated PVR.

A Postpartum Type A Dissection.

A 28-year-old woman with familial thoracic aortic aneurysm and dissection syndrome and a mildly dilated aorta presented 3 days postpartum with a type A aortic dissection. This case illustrates the unpredictability of this disease and the challenges with risk stratification of women with underlying aortopathy contemplating pregnancy. (Level of Difficulty: Intermediate.).

Pregnancy Outcomes in Women With Heart Disease: The CARPREG II Study.

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.

Impact of frequent premature ventricular contractions on pregnancy outcomes.

OBJECTIVES: To determine cardiac and fetal/neonatal event rates among pregnant women with premature ventricular contractions (PVCs) and compare with control groups. METHODS: Prospective case-control cohort study: 53 consecutive pregnancies in 49 women referred to the St. Paul's Hospital between 2010 and 2016 with PVC burden >1% in women without underlying cardiac disease. Maternal cardiac and fetal/neonatal outcomes were compared with two pregnant control groups: (1) supraventricular tachycardia (SVT) group of 53 women referred for a history of SVT/SVT in the current pregnancy and (2) low-risk group of 53 women with no cardiac disease. RESULTS: The maximal PVC burden was 9.2% (range 1.1%-58.7%). Six of 53 (11%) pregnancies were complicated by a maternal cardiac event: heart failure n=1 and sustained ventricular tachycardia requiring therapy n=5 as compared with no cardiac events in both control groups. All women with an adverse event had a PVC burden >5%. Seven (13%) pregnancies were complicated by an adverse fetal and/or neonatal event and this was similar to the normal control group (5 (9%), P=0.45) and significantly less than the SVT group (16 (30%), P=0.03). The adverse fetal event was driven by small for gestational age neonates and preterm delivery. CONCLUSIONS: In our cohort of pregnant women with a structurally normal heart and 'high' PVC burden, we found an adverse maternal event rate of 11%, and all events were successfully managed with medical therapy. The rate of adverse fetal events in the PVC group was similar to the normal control group.

Anticoagulation for Thromboembolic Risk Reduction in Adults With Congenital Heart Disease.

Adults with congenital heart disease (ACHD) represent one of the fastest expanding groups of patients in contemporary practice with unique and often complex management pathways. ACHD have a higher risk of thromboembolism not only from their complex physiology and anatomy but also from substrates such as valvular heart disease, atrial arrhythmias, cyanosis, and ventricular dysfunction, resulting in significant morbidity and mortality. Thromboembolic event rates in ACHD have been shown to be related to Congestive Heart Failure, Hypertension, Age (≥75 years), Diabetes, Stroke/Transient Ischemic Attack, Vascular Disease, Age (65-74 years), Sex (Female) (CHA2DS2-VASc) scores (0.75%, 1.24%, and 2.65% per patient-year for CHA2DS2-VASc scores of 0, 1, and ≥ 2 respectively), but are likely more strongly associated with disease complexity. However, optimum anticoagulation strategies have not been studied extensively in this group, mostly because of their heterogeneity and complex clinical profiles. Individual comorbidities, disease severity, and the presence of prosthetic material must be taken into consideration when determining the ideal anticoagulation strategy in pregnant patients. With improving survival and expanding treatment options, the requirement of thromboprophylaxis is steadily increasing, however, safety, choice, and duration of anticoagulation strategies in this group has not been well studied. In this review we aim to highlight the current understanding of antithrombotic therapy in the ACHD population, with discussion regarding special considerations in unique clinical situations relevant to ACHD.

Stress Echocardiographic Evaluation for D-Transposition of the Great Arteries after Atrial Redirection: Unmasking Early Signs of Myocardial Dysfunction and Baffle Stenosis.

BACKGROUND: The authors used semisupine cycle ergometry stress echocardiography to assess cardiac function and unmask baffle stenosis in patients with d-transposition of the great arteries after atrial redirection surgery. METHODS: This was a retrospective review of semisupine cycle ergometry stress echocardiography performed in 53 patients (64% male; mean age, 24.0 years; 90% Mustard procedure) and 56 healthy control subjects. Incremental exercise to volitional fatigue was performed. Hemodynamic data, echocardiographic cardiac dimensions, area change, tissue Doppler velocities, strain, ventricular synchronization, and superior vena cava flow velocities before and immediately after exercise are reported. RESULTS: Patients had lower exercise capacity (870 vs 1,854 J/kg, P < .001) and peak heart rates (132 vs 167 beats/min, P < .001). Stroke volume index did not increase with exercise (45 vs 47 mL/m2, P = .400). Cardiac index increased in both groups with exercise (3.0 vs 6.1 and 2.9 vs 7.0 L/min/m2, P < .001) and was higher in control subjects (P = .006). Right ventricular diastolic and systolic areas decreased significantly with exercise in both the short-axis and four-chamber views. Right and left ventricular contraction time shortened with exercise (405 vs 247 and 338 vs 217 msec, P < .001) and remained synchronous (ratio of right ventricular to left ventricular contraction time = 0.080). Doppler velocities in patients with baffle obstruction were higher in the lower superior vena cava with exercise compared with nonobstructed patients (1.87 vs 1.46 m/sec, P = .020) and normalized after catheter intervention (1.49 vs 1.46 m/sec, P = .800). CONCLUSIONS: Patients with d-transposition of the great arteries have lower exercise capacity and peak heart rates. The systemic right ventricle presents a lesser but qualitatively normal systolic response and decreased diastolic filling. Semisupine cycle ergometry stress echocardiography unmasked SVC obstruction.