Moderate intensity exercise in pregnant patients with cardiovascular disease: A pilot study.

BACKGROUND: Exercise in pregnancy has proven health benefits, yet the safety of exercise in patients with pre-existing cardiovascular disease (CVD) has not been established. Our aim was to determine the feasibility and safety profile of moderate intensity exercise during pregnancy in patients with CVD, compared with those without CVD. METHODS: This is a prospective single center pilot study of a moderate intensity exercise regimen, with data collection through wearable fitness trackers and personal exercise logs in pregnant patients with and without pre-existing CVD. The primary outcome was Doppler umbilical artery systolic to diastolic (S/D) ratio measured between 32 and 34 weeks' gestation. The secondary outcomes were adverse maternal and fetal events, trends in wearable fitness tracker data, C-reactive protein levels, and weight changes. RESULTS: At baseline, the CVD group (62% congenital heart disease) took part in more prepregnancy walking, less weightlifting, and had a higher body mass index compared to the control group, and on average walked 539 fewer steps per day during pregnancy than the control group. Resting heart rate (HR) was found to increase in both groups up to 30 weeks' gestation. The cardiovascular disease group displayed an overall lower exercise intensity, as measured by the ability to increase HR with exercise over resting heart rate 1 hour prior to exercise at study baseline (45% vs 59% P < .001). Umbilical artery S/D ratio was normal in both groups. No differences were seen in adverse events between groups. CONCLUSIONS: This pilot study of moderate intensity exercise in pregnant individuals with pre-existing CVD demonstrated that patients with CVD were not able to increase their HR during exercise throughout pregnancy compared to those in the control group. Although a small study group, this data supports the hypothesis that exercise interventions during pregnancy for patients with CVD are feasible without evidence abnormal fetal Doppler profiles. Further studies using wearable fitness trackers may provide the opportunity to understand how to safely tailor exercise programs to pregnant individuals with CVD.

Perioperative Anesthetic Management of the Pregnant Patient With Congenital Heart Disease Undergoing Cardiac Intervention: A Systematic Review.

Maternal congenital heart disease is increasingly prevalent, and has been associated with a significantly increased risk of maternal, obstetric, and neonatal complications. For patients with CHD who require cardiac interventions during pregnancy, there is little evidence-based guidance with regard to optimal perioperative management. The periprocedural management of pregnant patients with congenital heart disease requires extensive planning and a multidisciplinary teams-based approach. Anesthesia providers must not only be facile in the management of adult congenital heart disease, but cognizant of the normal, but significant, physiologic changes of pregnancy.

Intraperitoneal corticosteroids for recurrent ascites in patients with Fontan circulation: Initial clinical experience.

Individuals with single-ventricle congenital heart disease who are palliated to a Fontan circulation are at risk for heart failure and liver disease, with recurrent ascites being a potentially debilitating cause of late morbidity. Although ascites associated with heart failure or liver failure is usually characterized by a high serum-ascites albumin gradient (SAAG), we have observed multiple instances of ascites in Fontan patients with low SAAG, suggesting an inflammatory process. We present three cases in which recalcitrant ascites severely and adversely impacted the quality of life and describe our initial experience with intraperitoneal corticosteroids in this setting.

Adverse Pregnancy Conditions Among Privately Insured Women With and Without Congenital Heart Defects.

Background In women with congenital heart defects (CHD), changes in blood volume, heart rate, respiration, and edema during pregnancy may lead to increased risk of adverse outcomes and conditions. The American Heart Association recommends providers of pregnant women with CHD assess cardiac health and discuss risks and benefits of cardiac-related medications. We described receipt of American Heart Association-recommended cardiac evaluations, filled potentially teratogenic or fetotoxic (Food and Drug Administration pregnancy category D/X) cardiac-related prescriptions, and adverse conditions among pregnant women with CHD compared with those without CHD. Methods and Results Using 2007 to 2014 US healthcare claims data, we ascertained a retrospective cohort of women with and without CHD aged 15 to 44 years with private insurance covering prescriptions during pregnancy. CHD was defined as ≥1 inpatient code or ≥2 outpatient CHD diagnosis codes >30 days apart documented outside of pregnancy and categorized as severe or nonsevere. Log-linear regression, accounting for multiple pregnancies per woman, generated adjusted prevalence ratios (aPRs) for associations between the presence/severity of CHD and stillbirth, preterm birth, and adverse conditions from the last menstrual period to 90 days postpartum. We identified 2056 women with CHD (2334 pregnancies) and 1 374 982 women without (1 524 077 pregnancies). During the last menstrual period to 90 days postpartum, 56% of women with CHD had comprehensive echocardiograms and, during pregnancy, 4% filled potentially teratogenic or fetotoxic cardiac-related prescriptions. Women with CHD, compared with those without, experienced more adverse conditions overall (aPR, 1.9 [95% CI, 1.7-2.1]) and, specifically, obstetric (aPR, 1.3 [95% CI, 1.2-1.4]) and cardiac conditions (aPR, 10.2 [95% CI, 9.1-11.4]), stillbirth (aPR, 1.6 [95% CI, 1.1-2.4]), and preterm delivery (aPR, 1.6 [95% CI, 1.4-1.8]). More women with severe CHD, compared with nonsevere, experienced adverse conditions overall (aPR, 1.5 [95% CI, 1.2-1.9]). Conclusions Women with CHD have elevated prevalence of adverse cardiac and obstetric conditions during pregnancy; 4 in 100 used potentially teratogenic or fetotoxic medications, and only half received an American Heart Association-recommended comprehensive echocardiogram.

State of the Art Management of Mechanical Heart Valves During Pregnancy.

PURPOSE OF THE REVIEW: To review the management of women with mechanical heart valves during pregnancy, from preconception counseling through delivery with a summary of the latest guidelines. RECENT FINDINGS: The hypercoagulability of pregnancy combined with the imperfect choices of anticoagulant agents contribute to a high risk of complications in pregnant women with mechanical heart valves. Valve thrombosis remains a major concern, much of which occurs during the first trimester transition to heparin-based products. The safest method of anticoagulation, with the best balance of maternal and fetal risk, is use of low-dose vitamin K antagonists, but only if therapeutic anticoagulation can be achieved with warfarin doses of ≤ 5 mg/day. Management of mechanical heart valves in pregnancy remains fraught with difficult decisions involving balancing of maternal and fetal risks as well as a high risk of maternal and fetal complications. Preconception counseling and planning is imperative. A risk-benefit discussion with the patient will help guide the choice of anticoagulation and outline the plan for safe delivery options. A multidisciplinary approach to management is advisable with close follow-up and care in a tertiary center.

Haemodynamic impact of stent implantation for lateral tunnel Fontan stenosis: a patient-specific computational assessment.

BACKGROUND: The physiological importance of the lateral tunnel stenosis in the Fontan pathway for children with single ventricle physiology can be difficult to determine. The impact of the stenosis and stent implantation on total cavopulmonary connection resistance has not been characterized, and there are no clear guidelines for intervention. Methods and results A computational framework for haemodynamic assessment of stent implantation in patients with lateral tunnel stenosis was developed. Cardiac magnetic resonances images were reconstructed to obtain total cavopulmonary connection anatomies before stent implantation. Stents with 2-mm diameter increments were virtually implanted in each patient to understand the impact of stent diameter. Numerical simulations were performed in all geometries with patient-specific flow rates. Exercise conditions were simulated by doubling and tripling the lateral tunnel flow rate. The resulting total cavopulmonary connection vascular resistances were computed. A total of six patients (age: 14.4 ± 3.1 years) with lateral tunnel stenosis were included for preliminary analysis. The mean baseline resistance was 1.54 ± 1.08 WU · m(2) and dependent on the stenosis diameter. It was further exacerbated during exercise. It was observed that utilising a stent with a larger diameter lowered the resistance, but the resistance reduction diminished at larger diameters. CONCLUSIONS: Using a computational framework to assess the severity of lateral tunnel stenosis and the haemodynamic impact of stent implantation, it was observed that stenosis in the lateral tunnel pathway was associated with higher total cavopulmonary connection resistance than unobstructed pathways, which was exacerbated during exercise. Stent implantation could reduce the resistance, but the improvement was specific to the minimum diameter.

Transient elastography may identify Fontan patients with unfavorable hemodynamics and advanced hepatic fibrosis.

BACKGROUND: Transient elastography (TE) offers a noninvasive correlate with the degree of hepatic fibrosis. However, factors other than fibrosis affect liver stiffness. We sought to determine whether hepatic congestion related to hemodynamics in Fontan circulation influences liver stiffness measurement (LSM) assessed by TE. METHODS: We studied 45 subjects with Fontan circulation undergoing cardiac catheterization with or without simultaneous liver biopsy. Subjects underwent TE within 5 days before catheterization. Clinical history, hemodynamic and biopsy data, and hepatic biomarkers were collected. Five subjects who had previously undergone liver biopsy and TE were also included. RESULTS: Median age was 13.1 years (range 2.4-57.8); median time since Fontan was 9.9 years (range 0.1-32.5). No subject had known hepatitis C. Mean LSM for the entire cohort was 21.4 ± 10.8 kPa. Univariate regression analysis using LSM as a continuous outcome variable shows significant correlations with age (R = 0.35, P = .01), time since Fontan (R = 0.41, P = .003), Fontan pressure (R = 0.31, P = .04), cardiac index (R = 0.33, P = .03), pulmonary vascular resistance (R = 0.34, P = .03), systemic arterial oxygen saturation (R = 0.31, P = .04), and platelet count (R = 0.29, P = .05). On multiple regression analysis, Fontan pressure (ß = 0.901, P = .03) and cardiac index (ß = 2.703, P = .02) were significant predictors of LSM with overall model R(2) = 0.206. Univariate analysis shows LSM to be associated with more severe centrilobular fibrosis (P = .05). CONCLUSIONS: Higher LSM is associated with unfavorable Fontan hemodynamics and advanced centrilobular hepatic fibrosis. TE may be a useful tool for identifying Fontan patients who warrant invasive testing.

Acute dyspnea in a young man with an old sternotomy.

Described is a 42-year-old man with complex congenital heart disease with single-ventricle physiology who underwent a Fontan operation at 27 years of age. He presented with sudden-onset chest pain and dyspnea and was initially misdiagnosed with a pulmonary embolism. This case is described in order to highlight the challenges in the evaluation of common presentations in adults with congenital heart disease.

Discovery and management of diaphragmatic hernia related to abandoned epicardial pacemaker wires in a pregnant woman with {S,L,L} transposition of the great arteries.

Epicardial pacemaker leads placed during childhood are often not removed when transvenous systems are placed later in life. The risk of complications related to retained pacemaker leads and generators is not clear but is generally considered low. We report the case of a 23-year-old pregnant woman who presented with left upper quadrant pain at 20 weeks gestation. The patient was born with {S,L,L} transposition of the great arteries and had high-grade conduction disease in infancy compelling epicardial pacemaker placement. A standard transvenous pacemaker was placed at age 9 years, without removal of the epicardial system. The patient's abdominal pain was attributed to herniation of abdominal contents through a diaphragmatic defect at the site of the abandoned epicardial pacing wire. Her pain improved spontaneously but worsened later in pregnancy leading to repair of the diaphragmatic hernia via anterolateral thoracotomy at 30 weeks gestation. The procedure was well tolerated by mother and fetus. At 38 3/7 weeks gestation, the patient underwent uneventful delivery by cesarean section for breech presentation. This case illustrates the importance of multidisciplinary collaboration in the care of women with congenital heart disease.