Characteristics and outcomes of socioeconomically disadvantaged pregnant individuals with adult congenital heart disease presenting to a Cardio-Obstetrics Program.

BACKGROUND: Outcomes of individuals with adult congenital heart disease who are socioeconomically disadvantaged and cared for in cardio-obstetrical programs, are lacking. OBJECTIVE: This study aimed to describe the clinical characteristics, maternal pregnancy outcomes, and contraceptive uptake in individuals with adult congenital heart disease in an urban cardio-obstetrical program. STUDY DESIGN: Retrospective data were collected for individuals with adult congenital heart disease seen in the Maternal Fetal Medicine-Cardiology Joint Program at Montefiore Health System between 2015 and 2021 and compared using modified World Health Organization class I, II vs the modified World Health Organization class ≥II/III. RESULTS: Over 90% of individuals with adult congenital heart disease were pregnant at the time of referral. Modified World Health Organization class I, II (n=77, 62.4% Black or Hispanic/Latina) had a total of 94 pregnancies and modified World Health Organization class ≥II/III (n=49, 49.0% Black or Hispanic/Latina) had a total of 56 pregnancies. Over 25% of individuals in each group had a body mass index ≥30 (P=.78), and very low summary socioeconomic scores. Modified World Health Organization class ≥II/III were more likely to be anticoagulated in the first trimester than modified World Health Organization class I, II (10.7% vs 0.0%, P=.002) and throughout pregnancy (14.3% vs 3.2% P=.02). Modified World Health Organization class ≥II/III were more likely to require arterial monitoring during delivery than modified World Health Organization class I, II (14.3% vs 0.0%, P=.001) or delivery under general anesthesia (8.9% vs 1.1%, P=.03) but had a comparable frequency of cesarean delivery (35.8% vs 41.3%, P=.68). There were no in-hospital maternal deaths. There was no difference in the type of contraception recommended by modified World Health Organization class, however, modified World Health Organization class ≥II/III were more likely to receive long-acting types or permanent sterilization (35.6% vs 54.6%, P=.045). CONCLUSION: In a socioeconomically disadvantaged cohort with adult congenital heart disease from a historically marginalized community, those with modified World Health Organization class ≥II/III had more complex antepartum and intrapartum needs but similar maternal and obstetrical outcomes as modified World Health Organization class I, II. The multidisciplinary approach offered by a cardio-obstetrics program may contribute to successful outcomes in this high-risk cohort, and these data are hypothesis-generating.

Accuracy of Fetal Echocardiography in Defining Anatomic Details: A Single-Institution Experience over a 12-Year Period.

BACKGROUND: Fetal echocardiography has evolved over four decades, now permitting the prenatal diagnoses of most major congenital heart disease (CHD). To identify areas for targeted improvement, the authors explored the diagnostic accuracy of fetal echocardiography in defining major fetal CHD. METHODS: All fetuses with major fetal CHD (11 subtypes) at a single institution between 2007 and 2018 were identified (n = 827). Fetal echocardiography reports were compared with postnatal imaging and surgical or autopsy reports, and findings were categorized as follows: category 1, no errors; category 2, minor errors without impact on care, considered "accurate"; category 3, errors with minor impact on surgical approach; and category 4, errors with major impact on neonatal care or outcomes, considered "inaccurate." In addition, the contributions of era, gestational age at first fetal echocardiography, serial fetal echocardiography, maternal weight, and reviewer level of training were examined. RESULTS: Of 589 fetuses with autopsy or postnatal confirmation, accurate diagnoses were made in 530 (90%). The highest rates of accuracy were observed in univentricular hearts (97.6%; 95% CI, 87.4%-99.6%), tetralogy of Fallot (97.2%; 95% CI, 90.0%-99.2%), and transposition of the great arteries (96.1%; 95% CI, 89.2%-98.6%), and the lowest were observed in double-outlet right ventricle (81.1%; 95% CI, 70.4%-88.6%), truncus arteriosus (72.7%; 95% CI, 51.8%-86.8%), and heterotaxy (71.1%; 95% CI, 56.6%-82.2%). Greater accuracy was associated with later diagnostic era (2012-2018, P = .026), first fetal echocardiography at ≤25 weeks (P = .028), and formal fetal cardiology training of the reviewer (P = .001). Maternal pre-pregnancy weight did not affect accuracy. CONCLUSIONS: The diagnostic accuracy of fetal echocardiography for major CHD is high, particularly in the hands of fetal cardiology-trained practitioners. There are lesion-specific as well as general modifiable and nonmodifiable factors that affect diagnostic accuracy.

Fetal double outlet right ventricle without heterotaxy syndrome: Diagnostic spectrum, associated extracardiac pathology and clinical outcomes.

OBJECTIVES: To document the clinical spectrum and outcomes of fetal double outlet right ventricle (DORV) without heterotaxy in a recent diagnostic era. METHODS: Prenatal cases of DORV consecutively diagnosed from 2007 to 2018 were retrospectively identified. Clinical records, including details regarding genetic testing and pre and postnatal imaging were reviewed. RESULTS: DORV was diagnosed in 99 fetuses without heterotaxy. The most common anatomic subtype was subaortic ventricular septal defect (VSD) and normally related great arteries with (n = 45, 45%) or without (n = 13, 13%) pulmonary stenosis. The remainder had a subpulmonic VSD with transposed great arteries (n = 15, 15%), atrioventricular valve atresia (n = 24, 24%), or remote VSD (n = 2, 2%). A genetic diagnosis was found in 32 (34%) of 93 tested. Major extracardiac anomalies were found in 40 (40%), including 17/24 (71%) with and 22/69 (32%) without an abnormal karyotype, with VACTERL association in 9. Genetic and/or extracardiac pathology was identified in 37/58 (64%) with a subaortic VSD, 5/15 (33%) with a subpulmonic VSD, 9/24 (38%) of those with AV valve atresia and 2/2 (100%) with a remote VSD. A genetic abnormality was a significant predictor of fetal demise (9/37 vs 1/62 p < 0.01) or pregnancy termination (12/35 vs 9/64 p = 0.03). CONCLUSIONS: Fetal DORV is associated with a high rate of genetic abnormalities and extracardiac pathology. The presence of genetic abnormalities impacts prenatal outcomes and parental decision-making.

Impact of Obesity on Outcomes of Pregnancy in Women With Heart Disease.

BACKGROUND: Women with heart disease are at risk for complications during pregnancy. This study sought to examine the effect of maternal obesity on pregnancy complications in women with heart disease. OBJECTIVES: The objective was to determine the incidence of adverse cardiac events (CE) in pregnant women with heart disease and obesity. METHODS: Adverse CE during pregnancy were examined in a prospective cohort of women with heart disease. CE were a composite of the following: cardiac death/arrest, arrhythmias, heart failure, myocardial infarction, stroke, aortic dissection, and thromboembolic events. Pre-eclampsia and post-partum hemorrhage were also studied. Outcomes were examined according to body mass index (BMI). To identify additional predictors of CE, a baseline risk score (CARPREG [Canadian Cardiac Disease in Pregnancy Study] II score) for predicting cardiac complications was calculated for all pregnancies and included in a multivariable logistic regression model. RESULTS: Of 790 pregnancies, 19% occurred in women with BMI ≥30 kg/m2 (obesity), 25% in women with BMI 25 to 29.9 kg/m2 (overweight), 53% in women with BMI 18.5 to 24.9 kg/m2 (normal weight), and 3% in women with BMI <18.5 kg/m2 (underweight). Women with obesity were at higher risk of CE when compared with women with normal weight (23% vs. 14%; p = 0.006). In a multivariable model, obesity (odds ratio: 1.7; 95% confidence interval: 1.0 to 2.7) and higher CARPREG II risk scores (odds ratio: 1.7; 95% confidence interval: 1.5 to 1.9) predicted CE. Pre-eclampsia was more frequent in women with obesity compared with those with normal weight (8% vs. 2%; p = 0.001). CONCLUSIONS: Obesity increases the risk of maternal cardiovascular complications in pregnant women with heart disease. This modifiable risk factor should be addressed at the time of preconception counseling.

Congenital Heart Disease and Women's Health Across the Life Span: Focus on Reproductive Issues.

From adolescence to older age, women with congenital heart disease (CHD) face unique challenges. In this review we explore the ways in which CHD affects women's sexual and reproductive health and, in turn, how their sexual and reproductive history affects the course of their CHD. In adolescence, special attention must be paid to menstrual irregularities and concerns of developing sexuality and self-image. Discussions about sexuality and reproduction are an important part of transition planning and must be done with an awareness of the adolescent's developing understanding and maturity. Pregnancy imposes a hemodynamic load on the heart which may lead to cardiac, obstetric, and fetal/neonatal complications in women with CHD. Prepregnancy counselling must include an assessment of maternal and fetal risk according to several well developed models. Counselling should also include discussions about fertility and alternatives to pregnancy when appropriate. Recommendations for contraception must be made according to the patient's cardiac lesion. In caring for women with CHD during pregnancy, a multidisciplinary cardio-obstetrics team is recommended to optimize care. More research is needed into the long-term impact of pregnancy on the prognosis of patients with CHD. As women with CHD increasingly survive into old age, more attention will need to be directed toward the treatment of menopause and acquired heart disease in this population.

Prenatal Detection, Comorbidities, and Management of Vascular Rings.

The 3-vessel and trachea view is now integrated into obstetrical screening and facilitates prenatal detection of vascular rings. We examined trends in prenatal detection, associated cardiac and extracardiac anomalies, and surgical management in this population. We reviewed a population-based cohort of pediatric vascular ring patients diagnosed prenatally and postnatally between 2002 and 2017 in Alberta, Canada. Of 106 cases, 28 (26%) had a prenatal diagnosis. Prenatal detection increased over time: 0/29 from 2002 to 2009, 4/28 (14%) from 2009 to 2011, 7/23 (30%) from 2012 to 2014, and 17/26 (65%) from 2015 to 2017 (p <0.01). The prenatal group more commonly had right aortic arch/left ductus/aberrant left subclavian artery (24/28vs 53/78, p = 0.04) and associated cardiac pathology (18/28vs 33/78, p = 0.05). The rate of genetic anomalies was overall higher than previously reported (34%) and did not differ between groups (11/28vs 25/78, p = 0.48). Those with a prenatal diagnosis were less likely to require cross-sectional imaging (9/28vs 48/78, p <0.01), modifying the vascular ring subtype diagnosis in 2 patients. Surgical intervention was common and did not differ between groups (24/28vs 66/78, p = 0.89). In conclusion, prenatal detection of vascular rings has increased. Despite differences in vascular ring subtype and associated cardiac pathology, the incidence of genetic anomalies and need for surgical intervention is not associated with timing of diagnosis. Genetic counseling should be universally offered. The diagnostic accuracy of echocardiography suggests additional imaging may not be routinely required.

Revisiting the utility of technical performance scores following tetralogy of Fallot repair.

OBJECTIVE: Although an important quality metric, current technical performance scores may not be generalizable and may omit operative factors that influence outcomes. We examined factors not included in current technical performance scores that may contribute to increased postoperative length of stay, major complications, and cost after primary repair of tetralogy of Fallot. METHODS: This is a retrospective single site study of patients younger than age 2 years with tetralogy of Fallot undergoing complete repair between 2007 and 2015. Medical record data and discharge echocardiograms were reviewed to ascertain component and composite technical performance scores. Primary outcomes included postoperative length of stay, major complications, and total hospital costs. Multivariable logistic and linear regression identified determinants of each outcome. RESULTS: Patient population (n = 115) had a median postoperative length of stay of 8 days (interquartile range, 6-10 days), and a median total cost of $71,147. Major complications occurred in 33 patients (29%) with 1 death. Technical performance scores assigned were optimum in 28 patients (25%), adequate in 59 patients (52%), and inadequate in 26 patients (23%). Neither technical performance score components nor composite scores were associated with increased postoperative length of stay. Optimum or adequate repairs versus inadequate had equal risk of a complication (P = .79), and equivalent mean total cost ($100,000 vs $187,000; P = .25). Longer cardiopulmonary bypass time per 1-minute increase (P < .01) was associated with longer postoperative length of stay and reintervention (P = .02). The need to return to bypass also increased total cost (P < .01). CONCLUSIONS: Current tetralogy of Fallot technical performance scores were not associated with selected outcomes in our postoperative population. Although returning to bypass and bypass length are not included as components in the current score, these are important factors influencing complications and resource use in our population. Revisions anticipated from a prospective trial should consider including these variables.

A case of an infant with compound heterozygous mutations for hypertrophic cardiomyopathy producing a phenotype of left ventricular noncompaction.

A male infant was born to a 38-year-old G1P0 mother with hypertrophic cardiomyopathy (HCM). Fetal echocardiography was suspicious for HCM; however, postnatal echocardiography demonstrated features consistent with left ventricular noncompaction (LVNC). The infant was initially stable but presented at 2 months of age in cardiogenic shock. On genetic analysis, both parents were heterozygous for mutations associated with HCM. The proband was a compound heterozygote. This case, in which 2 mutations for HCM produced a phenotype of LVNC, has not been demonstrated in humans and raises the question of whether HCM and LVNC represent a continuum of pathologic processes.

Pulmonary valve replacement in repaired tetralogy of Fallot: determinants of early postoperative adverse outcomes.

OBJECTIVE: We sought to examine the frequency of early postoperative complications and preoperative predictors of prolonged hospitalization in adults with repaired tetralogy of Fallot undergoing pulmonary valve replacement. METHODS: This was a retrospective study of consecutive adult patients undergoing surgical intervention between 1995 and 2006. A multivariate logistic regression model was used to identify determinants of prolonged hospitalization. RESULTS: One hundred sixteen patients (mean age, 36 +/- 11 years) underwent 118 pulmonary valve replacements. Most (95%) operations included additional procedures, such as pulmonary artery/outflow tract reconstruction or tricuspid valve annuloplasty. The early postoperative mortality (<30 days) was 2.5%. The majority of the patients (60%) had no postoperative complications. The postoperative adverse events included postoperative arrhythmias (19%), respiratory complications (13%), reoperation during admission (13%), renal dysfunction (13%), and myocardial infarction (3%). Postoperative adverse events were associated with prolonged hospitalization (14 +/- 12 vs 7 +/- 3 days, P = .001). In the multivariate analysis age at reoperation of greater than 45 years (odds ratio, 6.1; 95% confidence interval, 1.6-23.6; P = .009), the number of previous sternotomies (odds ratio, 3.8; 95% confidence interval, 1.4-10; P = .007), and the need for urgent surgical intervention (odds ratio, 5.7; 95% confidence interval, 1.1-27.8; P = .03) were predictors of prolonged hospitalization. CONCLUSION: Pulmonary valve replacement in adults with repaired tetralogy of Fallot has a low mortality risk. The most common early postoperative complications are arrhythmias and respiratory and renal complications. Although most early postoperative complications do not result in long-term sequelae, they are associated with prolonged hospitalization. Patients undergoing urgent interventions, older patients, and those with multiple previous sternotomies are at the highest risk for prolonged hospitalization.

Transcatheter closure of atrial septal defects with multiple devices in adults: procedural and clinical outcomes.

OBJECTIVE: This study reports the procedural, short and medium term outcomes in patients receiving multiple implants for device closure of secundum atrial septal defects (ASD). DESIGN AND SETTING: From the database of the Toronto Congenital Cardiac Centre, 36 consecutive patients (mean age 46+/-15 years; 23 females) received >1 implant for closure of an interatrial communication. Short term (mean 97+/-77 days) and medium term (mean 2.5+/-1.4 years) follow-up data were analyzed. Procedures were performed under intracardiac echo (ICE) and fluoroscopic guidance. RESULTS: The Amplatzer Septal Occluder (ASO) was implanted in all except one. Thirty-one patients had 2, and 4 patients, 3 ASO devices. There were no major adverse events at implantation or on follow-up. Patients with >NYHA class I symptoms fell from 44% to 6% (p<0.05) at 3 months. Right ventricular (RV) systolic pressure fell from 39+/-7 to 32+/-4 mm Hg in the short term (p<0.05), and to 30+/-4 mm Hg in the medium term (p<0.05, compared to baseline) and RV diameters fell from 48+/-5 to 40+/-5 (p<0.05) in the short term and to 38+/-8 mm (p=ns) in the medium term. Small residual leaks were present in 22% at 3 months and 12% (p<0.05) at 1 year. CONCLUSION: Multiple ASD device implants can be safely employed with excellent outcomes. Significant reductions in RV pressure and diameter occur in the short term with a continued trend to benefit.

Improvements in cardiac form and function after transcatheter closure of secundum atrial septal defects.

OBJECTIVES: We set out to study the effect of transcatheter closure of atrial septal defect (ASD) on right ventricular (RV) and left ventricular (LV) function assessed by myocardial performance index (MPI), as well as left atrial (LA) volumes. BACKGROUND: The hemodynamic response to the closure of ASD is well-documented in surgically treated patients. However, few studies have documented echocardiographic evaluation of ventricular function in patients undergoing transcatheter closure of ASDs. METHODS: Pre- and post-ASD device closure echocardiograms of 25 consecutive patients were retrospectively reviewed. Measurements of RV and LV MPI and LA volumes were made. RESULTS: Twenty-five patients with an average age of 45.5 +/- 16.3 years underwent transcatheter closure of ASD. There was statistically significant improvement in RV MPI (0.35 to 0.28, p = 0.004), LV MPI (0.37 to 0.31, p = 0.04), and LA volume index (25.7 to 21.8 ml/m(2), p < 0.001) after closure of ASD. CONCLUSIONS: Device closure of ASDs leads to improvement of both RV and LV function as well as reduction in LA volume. These hemodynamic improvements provide insights into the symptomatic benefits gained in closure of ASDs using the transcatheter approach.