Immediate Postpartum Breastfeeding following Pregnancy with Cardiac Disease.

OBJECTIVE: This study aimed to identify predictors of immediate postpartum breastfeeding among women with maternal cardiac disease (MCD). STUDY DESIGN: This study included all gravidas with MCD who delivered at a single institution from 2012 to 2018. Charts were abstracted for maternal demographics, obstetrical outcome, cardiac diagnoses, cardiac risk stratification scores, and prepregnancy echocardiogram findings. Kruskal-Wallis and Fisher's exact tests were used to compare the breastfeeding (BF) group versus the nonbreastfeeding (NBF) group. Logistic regression was used to obtain odds ratios (ORs) with 95% confidence intervals (CIs). RESULTS: Among 211 gravidas with MCD, 12% were not breastfeeding at the time of postpartum hospital discharge. Compared with the BF group, the NBF group had a significantly higher proportion of women with cardiomyopathy (21% NBF vs. 7% BF, OR = 3.44, 95% CI: 1.12-10.71), with modified World Health Organization (WHO) classification ≥III (33 vs. 14%, OR = 3.16, 95% CI: 1.22-8.15), and with prepregnancy ejection fraction (EF) < 50% (55 vs. 14%, OR = 7.20, 95% CI: 1.92-27.06). There were otherwise no differences between the two groups with regards to other cardiac diagnoses or cardiac risk scores. CONCLUSION: In women with MCD, cardiomyopathy, modified WHO class ≥III, and a prepregnancy EF < 50% were associated with NBF in the immediate postpartum period. These findings may guide providers in identifying a subset of women with MCD who can benefit from increased breastfeeding counseling and support. KEY POINTS: · Eighty-two percent of patients with cardiac disease are breastfeeding at the time of postpartum discharge.. · Cardiomyopathy is associated with an increased odds of not breastfeeding at postpartum discharge.. · Rationale for not breastfeeding is infrequently documented in the medical record..

Combinatorial regulation of endothelial gene expression by ets and forkhead transcription factors.

Vascular development begins when mesodermal cells differentiate into endothelial cells, which then form primitive vessels. It has been hypothesized that endothelial-specific gene expression may be regulated combinatorially, but the transcriptional mechanisms governing specificity in vascular gene expression remain incompletely understood. Here, we identify a 44 bp transcriptional enhancer that is sufficient to direct expression specifically and exclusively to the developing vascular endothelium. This enhancer is regulated by a composite cis-acting element, the FOX:ETS motif, which is bound and synergistically activated by Forkhead and Ets transcription factors. We demonstrate that coexpression of the Forkhead protein FoxC2 and the Ets protein Etv2 induces ectopic expression of vascular genes in Xenopus embryos, and that combinatorial knockdown of the orthologous genes in zebrafish embryos disrupts vascular development. Finally, we show that FOX:ETS motifs are present in many known endothelial-specific enhancers and that this motif is an efficient predictor of endothelial enhancers in the human genome.

Echocardiographic differences between D-TGA and L-TGA in adult patients.

Transposition of the great arteries (TGA) is a common cardiac malformation in which the great arteries are discordant relative to the ventricles. The two common forms of transposition include D-TGA, which presents with cyanosis early in life, and L-TGA, which on the other hand, may permit survival to adulthood without being diagnosed in childhood. There are remarkable differences between these two forms of TGA in the clinical presentation, echocardiographic findings, and long-term outcomes. Multimodality imaging in patients with TGA usually provides diagnostic and hemodynamic assessment for routine follow-up and preoperative planning prior to surgical or transcatheter intervention. In this review, we present a summary of the fundamental echocardiographic aspects of these two forms of TGA with emphasis in the adult congenital heart disease population.

A practical guide to echocardiographic evaluation of adult Fontan patients.

Recent advances in surgical techniques and perioperative care for patients with single ventricle physiology have led to a remarkable improvement in long-term survival, such that now the majority of patients with single ventricle physiology are living to adulthood after Fontan palliation. The management of adult patients with Fontan physiology is one of the most challenging clinical dilemmas encountered in contemporary cardiology. The complex and heterogeneous anatomical and physiological abnormalities seen in Fontan patients mandate that any clinical evaluation, either for routine follow-up or preoperative evaluation prior to any transcatheter or surgical intervention, incorporates detailed information from a careful and thorough echocardiographic examination, These examinations, however, can be complex and confusing, even for experienced echocardiographers. Ideally, the interpretation of these studies is informed by an understanding of the basic anatomical lesions and of the potential long-term complications encountered in adult single ventricle patients. In this review, we present a practical and clinically oriented approach to the echocardiographic evaluation of adult patients with single ventricle physiology post-Fontan.

MEF2C regulates outflow tract alignment and transcriptional control of Tdgf1.

Congenital heart defects are the most common birth defects in humans, and those that affect the proper alignment of the outflow tracts and septation of the ventricles are a highly significant cause of morbidity and mortality in infants. A late differentiating population of cardiac progenitors, referred to as the anterior second heart field (AHF), gives rise to the outflow tract and the majority of the right ventricle and provides an embryological context for understanding cardiac outflow tract alignment and membranous ventricular septal defects. However, the transcriptional pathways controlling AHF development and their roles in congenital heart defects remain incompletely elucidated. Here, we inactivated the gene encoding the transcription factor MEF2C in the AHF in mice. Loss of Mef2c function in the AHF results in a spectrum of outflow tract alignment defects ranging from overriding aorta to double-outlet right ventricle and dextro-transposition of the great arteries. We identify Tdgf1, which encodes a Nodal co-receptor (also known as Cripto), as a direct transcriptional target of MEF2C in the outflow tract via an AHF-restricted Tdgf1 enhancer. Importantly, both the MEF2C and TDGF1 genes are associated with congenital heart defects in humans. Thus, these studies establish a direct transcriptional pathway between the core cardiac transcription factor MEF2C and the human congenital heart disease gene TDGF1. Moreover, we found a range of outflow tract alignment defects resulting from a single genetic lesion, supporting the idea that AHF-derived outflow tract alignment defects may constitute an embryological spectrum rather than distinct anomalies.

Cardiovascular events more than 6 months after pregnancy in patients with congenital heart disease.

OBJECTIVES: Patients with congenital heart disease (CHD) are increasingly pursuing pregnancy, highlighting the need for data on late cardiovascular events (more than 6 months after delivery). We aimed to determine the incidence of late cardiovascular events in postpartum patients with CHD and evaluate the accuracy of the existing risk scores in predicting these events. STUDY DESIGN: We identified patients with CHD who delivered between 2008 and 2020 at a tertiary centre and had follow-up data for greater than 6 months post partum. Late cardiovascular events were defined as heart failure, arrhythmia, thromboembolic events, endocarditis, urgent cardiovascular interventions or death. Survival analysis and Cox proportional model were used to estimate the incidence of late cardiovascular events and determine the hazard ratio of factors associated with these events. RESULTS: Of 117 patients, 19% had 36 late cardiovascular events over a median follow-up of 3.8 years. Annual incidence of any late cardiovascular event was 5.7%. Hazards of late cardiovascular events were significantly higher among those with higher Cardiac Disease in Pregnancy Study (CARPREG) II and Zwangerschap bij Aangeboren HARtAfwijking-Pregnancy in Women With Congenital Heart Disease (ZAHARA) risk scores and among patients with prepregnancy New York Heart Association class≥II. C-statistic to predict the late cardiovascular events was highest for ZAHARA (0.7823), followed by CARPREG II (0.6902) and prepregnancy New York Heart Association class≥ II (0.6677). CONCLUSIONS: Currently available risk tools designed for prognostication during the peripartum period can also be used to determine risks of late maternal cardiovascular events among those with CHD. These findings provide important new information for counselling and risk modification.

Association of Body Mass Index With Clinical Features and Outcomes in Adults With Fontan Palliation.

Background With improving survival of patients with single ventricle physiology who underwent Fontan palliation, there is also an increase in the prevalence of overweight and obesity in these patients. This tertiary care single-center study aims to determine the association of body mass index (BMI) with the clinical characteristics and outcomes in adults with Fontan. Methods and Results Adult patients (aged ≥18 years) with Fontan who were managed at a single tertiary care center between January 1, 2000, and July 1, 2019, and had BMI data available were identified via retrospective review of medical records. Univariate and multivariable (after adjusting for age, sex, functional class, and type of Fontan) linear and logistic regression, as appropriate, were utilized to evaluate associations between BMI and diagnostic testing and clinical outcomes. A total of 163 adult patients with Fontan were included (mean age, 29.9±9.08 years), with a mean BMI of 24.2±5.21 kg/m2 (37.4% of patients had BMI ≥25 kg/m2). Echocardiography data were available for 95.7% of patients, exercise testing for 39.3% of patients, and catheterization for 53.7% of patients. Each SD increase in BMI was significantly associated with decreased peak oxygen consumption (P=0.010) on univariate analysis and with increased Fontan pressure (P=0.035) and pulmonary capillary wedge pressure (P=0.037) on multivariable analysis. In addition, BMI ≥25 kg/m2 was independently associated with heart failure hospitalization (adjusted odds ratio [AOR], 10.2; 95% CI, 2.79-37.1 [P<0.001]) and thromboembolic complications (AOR, 2.79; 95% CI, 1.11-6.97 [P=0.029]). Conclusions Elevated BMI is associated with poor hemodynamics and worse clinical outcomes in adult patients with Fontan. Whether elevated BMI is the cause or consequence of poor clinical outcomes needs to be further established.

Outcomes among adult survivors of total cavopulmonary Fontan palliation for single ventricle.

OBJECTIVE: This tertiary centre study aims to identify factors associated with adverse outcomes in adult survivors with total cavopulmonary connection (TCPC) Fontan palliation for single ventricle. METHODS: This retrospective review of medical records identified adult (≥18 years) survivors of TCPC Fontan palliation who were followed at a single tertiary centre between 1 January 2000 and 1 July 2019. Adverse outcomes were defined as arrhythmia, pacemaker/implantable cardioverter defibrillator placement, liver cirrhosis, protein losing enteropathy, hospitalisation for heart failure, thromboembolic complication and/or death. RESULTS: 160 adult TCPC patients met the inclusion criteria: 117 (73.1%) extracardiac and 43 (26.9%) lateral tunnel. The median (IQR) duration of follow-up since TCPC palliation was 17.5 (11.8-21.3) years. An adverse outcome occurred in 87 (54.4%) patients. Adverse outcome-free survival rates at 10, 20 and 25 years post TCPC were 89% (95% CI 82% to 93%), 60% (95% CI 50% to 69%) and 24% (95% CI 15% to 35%), respectively. On multivariate analysis, extracardiac Fontan (HR 2.21, 95% CI 1.20 to 4.08, p=0.011) was observed to be an independent risk factor for adverse outcomes after adjusting for age, race, morphology of the systemic ventricle and history of fenestration. CONCLUSIONS: In this single-centre retrospective study of adult survivors of TCPC palliation, extracardiac Fontan was associated with an increased hazard for adverse outcomes. This finding could guide clinicians in developing risk modification strategies and management decisions to improve long-term outcomes in these patients.

Association of Insurance Status With Emergent Versus Nonemergent Hospital Encounters Among Adults With Congenital Heart Disease.

Background Although the number of hospital visits has exponentially increased for adults with congenital heart disease (CHD) over the past few decades, the relationship between insurance status and hospital encounter type remains unknown. The purpose of this study was to evaluate the association between insurance status and emergent versus nonemergent encounters among adults with CHD ≥18 years old. Methods and Results We used California Office of Statewide Health Planning and Development Database from January 2005 to December 2015 to determine the trends of insurance status and encounters and the association of insurance status on encounter type among adults with CHD. A total 58 359 nonpregnancy encounters were identified in 6077 patients with CHD. From 2005 to 2015, the number of uninsured encounters decreased by 38%, whereas government insured encounters increased by 124% and private by 79%. Overall, there was a significantly higher proportion of emergent than nonemergent encounters associated with uninsured status (13.0% versus 1.8%; P<0.0001), whereas the proportion of nonemergent encounters associated with private insurance was higher than emergent encounters (35.8% versus 62.4%; P<0.0001). When individual patients with CHD became uninsured, they were ≈5 times more likely to experience an emergent encounter (P<0.0001); upon changing from uninsured to insured, they were significantly less likely to have an emergent encounter (P<0.001). After multivariate adjustment, uninsured status exhibited the highest odds of an emergent rather than nonemergent encounter compared with all other covariates (adjusted odds ratio, 9.20; 95% CI, 7.83-10.8; P<0.0001). Conclusions Efforts to enhance the ability to obtain and maintain insurance throughout the lifetime of patients with CHD might result in meaningful reductions in emergent encounters and a more efficient use of resources.

Safety of transvenous lead removal in adult congenital heart disease: a national perspective.

INTRODUCTION AND OBJECTIVES: Data are scarce on outcomes of transvenous lead removal (TLR) in adult congenital heart disease (CHD). We evaluated the safety of the TLR procedure in adult CHD patients from a 10-year national database. METHODS: We used the Healthcare Cost and Utilization Project Nationwide Inpatient Sample to identify TLR procedures in adult patients with and without CHD from 2005 to 2014. Outcomes included in-hospital mortality and complications. RESULTS: Of 132 068 adult patients undergoing TLR, 1939 had simple CHD, 657 had complex CHD, and 626 had unclassified CHD. The number of TLR procedures in adult CHD slightly increased from 236 in 2005 to 445 in 2014, with fluctuations over the study period. The overall rate of any complications in the TLR procedure was 16.6% in patients with CHD vs 10.1% in patients without CHD (P <.001). In a propensity score-matched cohort, CHD was associated with a higher risk of any complication after full adjustment vs patients without CHD (adjusted odd ratio, 1.49; 95% confidence interval, 1.11-1.99; P=.007). Simple and complex CHD were associated with 1.5- and 2.1-fold increased risks of any TLR-related complication, respectively. CHD was not associated with an increased risk of in-hospital mortality (adjusted odd ratio, 0.77; 95% confidence interval, 0.42-1.39; P=.386). CONCLUSIONS: Compared with patients without CHD, adult patients with simple and complex CHD undergoing TLR are more likely to have complications but show no increase in mortality.

Determinants of myogenic specificity within MyoD are required for noncanonical E box binding.

The MyoD family of basic helix-loop-helix (bHLH) transcription factors has the remarkable ability to induce myogenesis in vitro and in vivo. This myogenic specificity has been mapped to two amino acids in the basic domain, an alanine and threonine, referred to as the myogenic code. These essential determinants of myogenic specificity are conserved in all MyoD family members from worms to humans, yet their function in myogenesis is unclear. Induction of the muscle transcriptional program requires that MyoD be able to locate and stably bind to sequences present in the promoter regions of critical muscle genes. Recent studies have shown that MyoD binds to noncanonical E boxes in the myogenin gene, a critical locus required for myogenesis, through interactions with resident heterodimers of the HOX-TALE transcription factors Pbx1A and Meis1. In the present study, we show that the myogenic code is required for MyoD to bind to noncanonical E boxes in the myogenin promoter and for the formation of a tetrameric complex with Pbx/Meis. We also show that these essential determinants of myogenesis are sufficient to confer noncanonical E box binding to the E12 basic domain. Thus, these data show that noncanonical E box binding correlates with myogenic potential, and we speculate that the myogenic code residues in MyoD function as myogenic determinants via their role in noncanonical E box binding and recognition.

Development of the endocardium.

The endocardium, the endothelial lining of the heart, plays complex and critical roles in heart development, particularly in the formation of the cardiac valves and septa, the division of the truncus arteriosus into the aortic and pulmonary trunks, the development of Purkinje fibers that form the cardiac conduction system, and the formation of trabecular myocardium. Current data suggest that the endocardium is a regionally specialized endothelium that arises through a process of de novo vasculogenesis from a distinct population of mesodermal cardiogenic precursors in the cardiac crescent. In this article, we review recent developments in the understanding of the embryonic origins of the endocardium. Specifically, we summarize vasculogenesis and specification of endothelial cells from mesodermal precursors, and we review the transcriptional pathways involved in these processes. We discuss the lineage relationships between the endocardium and other endothelial populations and between the endocardium and the myocardium. Finally, we explore unresolved questions about the lineage relationships between the endocardium and the myocardium. One of the central questions involves the timing with which mesodermal cells, which arise in the primitive streak and migrate to the cardiac crescent, become committed to an endocardial fate. Two competing conceptual models of endocardial specification have been proposed. In the first, mesodermal precursor cells in the cardiac crescent are prespecified to become either endocardial or myocardial cells, while in the second, fate plasticity is retained by bipotential cardiogenic cells in the cardiac crescent. We propose a third model that reconciles these two views and suggest future experiments that might resolve this question.

Catheter-Directed Thrombolysis for Submassive Pulmonary Embolism in the Third Trimester of Pregnancy.

A 37-year-old woman presented with chest pain and shortness of breath in the third trimester of pregnancy. Diagnostic imaging demonstrated a saddle pulmonary embolism, severe impairment of right ventricular function, and an extensive deep venous thrombus. She underwent catheter-directed thrombolysis with tissue plasminogen activator and delivered a healthy infant at term. (Level of Difficulty: Intermediate.).

Contraceptive Choices in the Immediate Postpartum Period in Women With Cardiac Disease.

Maternal cardiac disease (MCD) is associated with increased maternal and neonatal morbidity and mortality. Because unplanned pregnancies are especially risky, active use of reliable contraception is critical in this population. Studies in the noncardiac population have demonstrated that the postpartum period is an ideal time to address contraceptive plans. This retrospective cohort study was designed to describe contraceptive choices in women with MCD in the immediate postpartum period and to identify factors associated with specific contraceptive plans. We included women with MCD who delivered from January 2008 to September 2017 at a tertiary care institution with a multidisciplinary obstetrics and cardiology team. Maternal demographics, specifics of MCD, obstetrical outcomes, and contraceptive plans were obtained through chart review. Contraceptive plans were categorized into highly reliable methods (sterilization or long-acting reversible contraceptive methods) or less reliable methods (nonlong-acting reversible contraceptive methods or no contraception). In the 254 women included in this study, 40% planned to use highly reliable methods, while 60% planned to use less reliable methods. Women with cardiomyopathy were more likely to choose a highly reliable method of contraception (adjusted odds ratio 2.6, 95% confidence interval 1.2 to 5.7), a reassuring finding, given the particularly high risk of poor pregnancy outcome with this diagnosis. There were no differences in other cardiac diagnoses between the 2 contraceptive groups. In conclusion, the finding that <50% of postpartum women with MCD plan to use a highly reliable method of contraception warrants further examination to identify and address barriers to reliable contraceptive plans in this high-risk population.

Pregnancy complications and premature cardiovascular events among 1.6 million California pregnancies.

Background: Cardiovascular complications of pregnancy present an opportunity to assess risk for subsequent cardiovascular disease. We sought to determine whether peripartum cardiomyopathy and hypertensive disorder of pregnancy subtypes predict future myocardial infarction, heart failure or stroke independent of one another and of other risks such as gestational diabetes, preterm birth and intrauterine growth restriction. Methods and results: The California Healthcare Cost and Utilization Project database was used to identify all hospitalised pregnancies from 2005 to 2009, with follow-up through 2011, for a retrospective cohort study. Pregnancies, exposures, covariates and outcomes were defined by International Classification of Diseases, Ninth Revision codes. Among 1.6 million pregnancies (mean age 28 years; median follow-up time to event excluding censoring 2.7 years), 558 cases of peripartum cardiomyopathy, 123 603 hypertensive disorders of pregnancy, 107 636 cases of gestational diabetes, 116 768 preterm births and 23 504 cases of intrauterine growth restriction were observed. Using multivariable Cox proportional hazards models, peripartum cardiomyopathy was independently associated with a 39.2-fold increase in heart failure (95% CI 30.0 to 51.9), resulting in ~1 additional hospitalisation per 1000 person-years. There was a 13.0-fold increase in myocardial infarction (95% CI 4.1 to 40.9) and a 7.7-fold increase in stroke (95% CI 2.4 to 24.0). Hypertensive disorders of pregnancy were associated with 1.4-fold (95% CI 1.0 to 2.0) to 7.6-fold (95% CI 5.4 to 10.7) higher risk of myocardial infarction, heart failure and stroke, resulting in a maximum of ~1 additional event per 1000 person-years. Gestational diabetes, preterm birth and intrauterine growth restriction had more modest associations. Conclusion: These findings support close monitoring of women with cardiovascular pregnancy complications for prevention of early cardiovascular events and study of mechanisms underlying their development.

Age- and Lesion-Related Comorbidity Burden Among US Adults With Congenital Heart Disease: A Population-Based Study.

Background As patients with congenital heart disease (CHD) are living longer, understanding the comorbidities they develop as they age is increasingly important. However, there are no published population-based estimates of the comorbidity burden among the US adult patients with CHD. Methods and Results Using the IBM MarketScan commercial claims database from 2010 to 2016, we identified adults aged ≥18 years with CHD and 2 full years of continuous enrollment. These were frequency matched with adults without CHD within categories jointly defined by age, sex, and dates of enrollment in the database. A total of 40 127 patients with CHD met the inclusion criteria (mean [SD] age, 36.8 [14.6] years; and 48.2% were women). Adults with CHD were nearly twice as likely to have any comorbidity than those without CHD (P<0.001). After adjusting for covariates, patients with CHD had a higher prevalence risk ratio for "previously recognized to be common in CHD" (risk ratio, 9.41; 95% CI, 7.99-11.1), "other cardiovascular" (risk ratio, 1.73; 95% CI, 1.66-1.80), and "noncardiovascular" (risk ratio, 1.47; 95% CI, 1.41-1.52) comorbidities. After adjusting for covariates and considering interaction with age, patients with severe CHD had higher risks of previously recognized to be common in CHD and lower risks of other cardiovascular comorbidities than age-stratified patients with nonsevere CHD. For noncardiovascular comorbidities, the risk was higher among patients with severe than nonsevere CHD before, but not after, the age of 40 years. Conclusions Our data underscore the unique clinical needs of adults with CHD compared with their peers. Clinicians caring for CHD may want to use a multidisciplinary approach, including building close collaborations with internists and specialists, to help provide appropriate care for the highly prevalent noncardiovascular comorbidities.

Cardiopulmonary exercise testing in the evaluation of liver disease in adults who have had the Fontan operation.

BACKGROUND: Liver disease (LD) is a long-term complication in patients with a single ventricle who have had the Fontan operation. A decline in cardiopulmonary exercise testing (CPET) variables is associated with increased risk of hospitalization, but its association with LD is unknown. AIM: To determine the association between CPET variables and LD in adults who have had the Fontan operation. METHODS: We retrospectively reviewed the medical records from two tertiary institutions. RESULTS: We identified 114 adults (≥18 years; mean 30.9±7.4 years) who had undergone the Fontan operation: 56% were women; 63% had total cavopulmonary connection; 66% had New York Heart Association (NYHA) class I status; 42% had arrhythmias; 22% had systemic right ventricle; and 35% had ventricular dysfunction. Of 81 patients with liver-imaging data, 41% had LD (i.e. imaging evidence of cirrhosis, with or without portal hypertension, splenomegaly or varices). There were no differences in clinical or echocardiographic variables between those with and without LD. Among the 58 patients with CPET data, mean peak oxygen consumption (VO2) was 18.6±5.7mL/kg/min, per-cent-predicted peak VO2 was 53.9±15.5%, peak oxygen pulse was 9.3±2.9mL/beat and per-cent-predicted peak oxygen pulse was 82.6±21.5%. Of the 44 patients with liver and CPET data, each standard deviation decrease in per-cent-predicted peak VO2 (16%) and per-cent-predicted peak oxygen pulse (22%) was associated with a 2.3-fold increase in the odds of LD, after adjusting for NYHA, institution and Fontan type (P=0.04). Similarly, each standard deviation decrease in per-cent-predicted peak VO2 and oxygen pulse was associated with an estimated 5.9-year and 4.9-year earlier onset of LD, respectively (P>0.05). CONCLUSIONS: Decline in per-cent-predicted peak VO2 and oxygen pulse was associated with increased odds of LD in adults who had undergone the Fontan operation. Our study supports more rapid hepatic evaluation among patients with abnormal or worsening CPET variables.

Phonocardiographic timing of third and fourth heart sounds during acute myocardial infarction.

A 47-year-old diabetic woman was referred for diagnostic coronary angiography as part of an evaluation before renal transplantation. During attempts to cannulate the left coronary artery, the patient developed severe dyspnea and new ST-segment elevations in the anterior leads. Left coronary angiography showed acute occlusion of the proximal left anterior descending (LAD) coronary artery. She then underwent emergent percutaneous intervention of the occluded vessel. Continuous electrocardiographic and phonocardiographic recordings were obtained during the procedure that documented the timing and appearance of ST-segment elevations and the onset of third and fourth heart sounds. In this report, the sequence of these events is discussed, and the literature is reviewed.

Coexistence of abnormal systolic motion of mitral valve in a consecutive group of 324 adult Tetralogy of Fallot patients assessed with echocardiography.

BACKGROUND: The presence of mitral valve prolapse (MVP) in congenital heart disease (CHD) patients is not well described. Tetralogy of Fallot (TOF) is the most common cyanotic CHD associated with overall good long-term survival after palliation. Since MVP is more often identified in adults and TOF patients are now surviving longer, we thus sought to perform this cohort study with a case-control design to (1) determine the prevalence of MVP and systolic displacement of mitral leaflets (SDML) in adult TOF patients, and (2) describe their clinical and imaging characteristics. METHODS: Retrospective interrogation of our echocardiography database identified 328 consecutive TOF patients ≥18 years from 1 January 2000 to 31 December 2014. All images were reviewed to identify patients with concomitant MVP (prolapse >2 mm beyond the long-axis annular plane) or SDML (<2 mm beyond the annular plane). RESULTS: 26 (8%) TOF patients fulfilled criteria for systolic mitral valve abnormality (SMVA) (15 MVP; 11 SDML). 2 had moderate to severe mitral regurgitation requiring repair. When compared with 52 TOF patients without SMVA, those with SMVA were more likely to be females (60.7% vs 33.9%, p=0.03), less likely to have transannular patch (52% vs 97.4%, p<0.0001), had lower right ventricular ejection fraction (36.5% vs 43.8%, p=0.03) and a trend towards increased risk of atrial (44% vs 30.4%, p=0.5) and ventricular arrhythmias (32% vs 25.5%, p=0.6). On multivariate logistic regression, SMVA was independently associated with the absence of transannular patch (p=0.002) and atrial arrhythmias (p=0.04). CONCLUSIONS: In this series of adult TOF patients, we describe a novel finding of a high prevalence of systolic mitral valve abnormalities.