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Patients with a Fontan circulation suffer from progressive multiorgan dysfunction, yet central biochemical drivers remain poorly defined. Our recent work exploring metabolomic analyses have identified - elevated circulating bile acids (BAs) in adult Fontan patients compared with healthy controls. Elevated BAs, especially secondary and hydrophobic ones produced by the gut microbiome were found to correlate with worse exercise capacity, greater frailty, and impaired hemodynamics. Bile acid accumulation may contribute to Fontan pathophysiology. Colesevelam, a nonabsorbed bile acid sequestrant, offers a potential targeted therapy to reduce BA levels and interrupt this disease pathway. The MYSTIC trial is a prospective, randomized, double-blind, placebo-controlled cross-over pilot study in 25 adult Fontan patients (with 25 age- and sex-matched healthy controls for baseline comparisons) to evaluate the safety, tolerability, and efficacy of colesevelam in lowering plasma BA levels. Primary outcomes include safety/tolerability and change in total plasma BA levels. Secondary outcomes include changes in noninvasive hemodynamics, gut microbiome composition, fecal bile acid excretion, and biochemical profiles. Single center. 25 patients and 25 healthy subjects. September 2025 to August 2027. NCT06197763 This manuscript describes the rationale and design of the MYSTIC study, which to our knowledge is the first interventional trial targeting a Fontan-specific metabolic derangement. The results will inform the feasibility of BA sequestration therapy in Fontan patients and guide future larger studies aimed at improving outcomes in this growing high-risk population.

Background Cardiovascular disease in pregnancy is the leading cause of maternal mortality in North America. Although transthoracic echocardiography (TTE) is the most widely used imaging modality for the assessment of cardiovascular function during pregnancy, little is known on the role of cardiovascular magnetic resonance (CMR). The objective of the Cardiac Hemodynamic Imaging and Remodeling in Pregnancy (CHIRP) study was to compare TTE and CMR in the non-invasive assessment of maternal cardiac remodeling during the peripartum period. Methods Between 2010–2012, healthy pregnant women aged 18 to 35 years were prospectively enrolled. All women underwent TTE and CMR during the third trimester and at least 3 months postpartum (surrogate for non-pregnant state). Results The study population included a total of 34 women (mean age 29 ± 3 years). During the third trimester, TTE and CMR demonstrated an increase in left ventricular end-diastolic volume from 95 ± 11 mL to 115 ± 14 mL and 98 ± 6 mL to 125 ± 5 mL, respectively (p < 0.05). By TTE and CMR, there was also an increase in left ventricular (LV) mass during pregnancy from 111 ± 10 g to 163 ± 11 g and 121 ± 5 g to 179 ± 5 g, respectively (p < 0.05). Although there was good correlation between both imaging modalities for LV mass, stroke volume, and cardiac output, the values were consistently underestimated by TTE. Conclusion This CMR study provides reference values for cardiac indices during normal pregnancy and the postpartum state.

Pregnancy is hazardous with pulmonary arterial hypertension, but maternal mortality may have fallen in recent years. We sought to systematically evaluate pulmonary arterial hypertension and pregnancy-related outcomes in the last decade. We searched for articles describing outcomes in pregnancy cohorts published between 2008 and 2018. A total of 3658 titles were screened and 13 studies included for analysis. Pooled incidences and percentages of maternal and perinatal outcomes were calculated. Results showed that out of 272 pregnancies, 214 pregnancies advanced beyond 20 gestational weeks. The mean maternal age was 28 ± 2 years, mean pulmonary artery systolic pressure on echocardiogram was 76 ± 19 mmHg. Etiologies include idiopathic pulmonary arterial hypertension (22%), congenital heart disease (64%), and others (15%). Majority (74%) had good functional class I/II. Only 48% of women received pulmonary arterial hypertension-specific therapy. Premature deliveries occur in 58% of pregnancies at mean of 34 ± 1 weeks, most (76%) had Cesarean section. Maternal mortality rate was 12% overall (n = 26); even higher for idiopathic pulmonary arterial hypertension etiology alone (20%). Reported causes of death included right heart failure, cardiac arrest, pulmonary arterial hypertension crises, pre-eclampsia, and sepsis; 61% of maternal deaths occur at 0–4 days postpartum. Stillbirth rate was 3% and neonatal mortality rate was 1%. In conclusion, pulmonary arterial hypertension in pregnancy continues to be perilous with high maternal mortality rate. Continued prospective studies are needed.

Several studies have correlated elevations in cardiac biomarkers of injury post marathon with transient and reversible right ventricular (RV) systolic dysfunction as assessed by both transthoracic echocardiography (TTE) and cardiovascular magnetic resonance (CMR). Whether or not permanent myocardial injury occurs due to repeated marathon running in the aging population remains controversial. To assess the extent and severity of cardiac dysfunction after the completion of full marathon running in individuals greater than 50 years of age using cardiac biomarkers, TTE, cardiac computed tomography (CCT), and CMR. A total of 25 healthy volunteers (21 males, 55 ± 4 years old) from the 2010 and 2011 Manitoba Full Marathons (26.2 miles) were included in the study. Cardiac biomarkers and TTE were performed one week prior to the marathon, immediately after completing the race and at one-week follow-up. CMR was performed at baseline and within 24 hours of completion of the marathon, followed by CCT within 3 months of the marathon. All participants demonstrated an elevated cTnT post marathon. Right atrial and ventricular volumes increased, while RV systolic function decreased significantly immediately post marathon, returning to baseline values one week later. Of the entire study population, only two individuals demonstrated late gadolinium enhancement of the subendocardium in the anterior wall of the left ventricle, with evidence of stenosis of the left anterior descending artery on CCT. Marathon running in individuals over the age of 50 is associated with a transient, yet reversible increase in cardiac biomarkers and RV systolic dysfunction. The presence of myocardial fibrosis in older marathon athletes is infrequent, but when present, may be due to underlying occult coronary artery disease.

Gas chromatography-mass spectrometry showed that the stones were composed of uric acid. Intermittent alkalinisation of urine dissolves crystals that have formed and promotes urinary stone resorption, although patients with hyperuricosuria do not form uric acid stones in the presence of alkaline urine.

ObjectiveTo perform a systematic review and meta-analysis of maternal/fetal outcomes in pregnant women with moderate/severe native valvular heart disease (VHD) from medium/higher Human Development Index (HDI) countries.MethodsOvidSP platform databases were searched (1985-January 2019) to identify studies reporting pregnancy outcomes in women with moderate/severe VHD. The primary maternal outcome was maternal mortality. The primary fetal/neonatal outcome was stillbirth and neonatal death. Pooled incidences and 95% confidence intervals (CI) of maternal/fetal outcomes could only be calculated from studies involving mitral stenosis (MS) or aortic stenosis (AS).ResultsTwelve studies on 646 pregnancies were included. Pregnant women with severe MS had mortality rates of 3% (95% CI, 0% to 6%), pulmonary oedema 37% (23%–51%) and new/recurrent arrhythmias 16% (1%–25%). Their stillbirth, neonatal death and preterm birth rates were 4% (1%–7%), 2% (0%–4%), and 18% (7%–29%), respectively. Women with moderate MS had mortality rates of 1%(0%–2%), pulmonary oedema 18% (2%–33%), new/recurrent arrhythmias 5% (1%–9%), stillbirth 2% (1%–4%) and preterm birth 10%(2%–17%).Pregnant women with severe AS had a risk of mortality of 2% (0%–5%), pulmonary oedema 9% (2%–15%), and new/recurrent arrhythmias 4% (0%–7%). Their stillbirth, neonatal death and preterm birth rates were 2% (0%–5%), 3% (0%–6%) and 14%(4%–24%), respectively. No maternal/neonatal deaths were reported in moderate AS, however women experienced pulmonary oedema (8%; 0%–20%), new/recurrent arrhythmias (2%; 0%–5%), and preterm birth (13%; 6%–20%).ConclusionsWomen with moderate/severe MS and AS are at risk for adverse maternal and fetal/neonatal outcomes. They should receive preconception counseling and pregnancy care by teams with pregnancy and heart disease experience.

Background Ventricular septal rupture (VSR), a mechanical complication following an acute myocardial infarction (MI), is thought to result from coagulation necrosis due to lack of collateral reperfusion. Although the gold standard test to confirm left-to-right shunting between ventricular cavities remains invasive ventriculography, two-dimensional transthoracic echocardiography (TTE) with color flow Doppler and cardiac MRI (CMR) are reliable tests for the non-invasive diagnosis of VSR. Case presentation A 62-year-old Caucasian female presented with a late case of a VSR post inferior MI diagnosed by multimodality cardiac imaging including TTE, CMR and ventriculography. Conclusion We review the presentation, diagnosis and management of VSR post MI.

Indications for the closure of pressure restrictive perimembranous ventricular septal defects (pmVSD) are not well established in the pediatric population. We sought to assess practice variability among pediatric cardiologists in the United States (US), Canada, Australia, and New Zealand. A survey ascertaining practice patterns, including case vignettes with incremental progression of disease severity, was designed and administered through representative professional cardiac organizations and email listservs in the designated countries. Among the 299 respondents, 209 (70.0%) were from the US, 65 (21.7%) were from Canada and 25 (8.3%) were from Australia and New Zealand. Indications for pressure restrictive pmVSD closure included the presence of left ventricular (LV) dilation for 81.6% (244/299) (defined as z -score ≥ 2 for 59.0% (144/244) and ≥ 3 for 40.2% (98/244)) and significant pulmonary-systemic flow ratio (QP:QS) for 71.2% (213/299) [defined as ≥ 1.5:1 for 36.2% (77/213) and ≥ 2 for 62% (132/213)]. US pediatric cardiologists elected to close restrictive pmVSD at lower LV z -score and QP:QS ratio cut-offs ( p -value 0.0002 and 0.013, respectively). In a case vignette, 63.6% (173/272) chose to intervene if there was right coronary cusp prolapse with stable mild aortic regurgitation. Of the remaining cardiologists, 93% (92/99) intervened if the aortic regurgitation was progressive (from trivial to mild). Commonly identified indications with variable thresholds for closure of pressure restrictive pmVSDs included the presence or progression of LV dilation, significant volume loading, and aortic valve prolapse with regurgitation. US pediatric cardiologists may have a lower threshold for pmVSD closure.