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BACKGROUND: Pregnant patients with single ventricle (SV) physiology carry a high risk of spontaneous pregnancy loss (SPL), yet the clinical factors contributing to this risk are not well defined. METHODS: Single-centre retrospective study of pregnant patients with SV physiology seen in cardio-obstetrics clinic over the past 20 years with chart review of their obstetric history. Patients without a known pregnancy outcome were excluded. Univariable Bayesian panel-data random effects logit was used to model the risk of SPL. RESULTS: The study included 20 patients with 44 pregnancies, 20 live births, 21 SPL and 3 elective abortions. All had Fontan palliation except for two with Waterston and Glenn shunts. 10 (50%) had a single right ventricle (RV). 14 (70%) had moderate or severe atrioventricular valve regurgitation (AVVR). Atrial arrhythmias were present in 16 (80%), Fontan-associated liver disease (FALD) in 15 (75%) and FALD stage 4 in 9 (45%). 12 (60%) were on anticoagulation. Average first-trimester oxygen saturation was 93.8% for live births and 90.8% for SPL. The following factors were associated with higher odds of SPL: RV morphology (OR 1.72 (95% credible interval (CrI) 1.0008-2.70)), moderate or severe AVVR (OR 1.64 (95% CrI 1.003-2.71)) and reduced first-trimester oxygen saturation (OR 1.83 (95% CrI 1.03-2.71) for each per cent decrease in O2 saturation. CONCLUSION: Pregnant patients with SV physiology, particularly those with RV morphology, moderate or severe AVVR, and lower first-trimester oxygen saturations, have a higher risk of SPL. Identifying these clinical risk factors can guide preconception counselling by the cardio-obstetrics team.

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Background: d-Transposition of the great arteries (d-TGA) is a congenital cardiac defect that is typically fatal. Those patients who survive without surgical repair and who are rare in number, need adequate intracardiac shunting and will suffer from chronic cyanosis. Here, we present a rare case of an adult with unrepaired cyanotic congenital heart disease (CHD) who developed infective endocarditis (IE) and also our approach to the medical decision-making process in this uncommonly encountered dilemma. Case summary: A 52-year-old female with unrepaired d-TGA with tricuspid atresia, hypoplastic right ventricle, unrestricted atrial septal defect, ventricular septal defect, and sub-valvular as well as valvular pulmonic stenosis with a hypoplastic, bicuspid pulmonary valve presented with abdominal pain and hypoxia and was found to have an acute renal infarct. Transthoracic echocardiogram (TTE) revealed a large mobile mass on the mitral valve. Blood cultures grew Streptococcus mitis-oralis and she was diagnosed with streptococcal native mitral valve IE complicated by a renal embolus. Her large left-sided vegetation and embolic phenomenon favoured surgery. However, a right heart catheterization showed normal intracardiac pressures, likely a result of multi-level obstruction relating to sub-valvular and valvular pulmonary stenosis protecting the pulmonary vasculature from over-circulation and pulmonary hypertension. Cardiac surgery posed a significant risk of destabilizing her delicately balanced haemodynamics. Hence, she was treated with ceftriaxone for 4 weeks. A repeat TTE 8 weeks later showed a resolution of the vegetation. Discussion: A decision for surgery vs. medical treatment for IE in adult patients with compensated CHD should be made following a multi-disciplinary assessment of each patient's unique cardiac haemodynamics and after shared decision-making with the patient.

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BACKGROUND: Women with cardiomyopathy (CM) are often advised against pregnancy due to risk for major adverse cardiovascular events (MACE). However, the impact of CM subtype on maternal MACE is not understood, and so we sought to evaluate the influence of CM phenotype on maternal outcomes, as well as the effect on immediate and late left ventricular function. METHODS: We evaluated all pregnant women in our high-risk maternal cardiovascular programme (2009-2019). Composite maternal MACE included: death, inotrope use, left ventricular assist device, orthotopic heart transplant and/or escalation in transplant listing status, acute decompensated heart failure and sustained ventricular arrhythmia. RESULTS: Among 875 women followed, 32 had CM (29±7 years old, left ventricular ejection fraction (LVEF) 41%±12%): 3 ischaemic CM (ICM), 10 peripartum CM (PPCM) and 19 non-ICM (NICM). MACE events occurred in 6 (18%) women (PPCM: 2 (33%), NICM: 4 (67%)). There was no difference in LVEF at baseline, however, women with MACE had significantly lower LVEF both early (LVEF: 27±5% vs . 41±2%, p<0.05) and late post partum (LVEF: 28±5% vs . 44±2%, p<0.01). CONCLUSIONS: In this contemporary cohort of women with CM, maternal MACE rates were lower than previously reported, and were less common in PPCM as compared with ICM and NICM. Heart function in women with MACE was negatively impacted immediately after delivery and in late postpartum follow-up, suggesting that pregnancy itself likely has influence on future left ventricular function in women with underlying CM.

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Ebstein anomaly is a congenital malformation involving primarily the tricuspid valve, with failure of delamination from the underlying myocardium and right ventricular myopathy. Echocardiography is diagnostic in most patients and demonstrates apical displacement of the septal leaflet and variable tethering of leaflet tissue to the right ventricular myocardium. Operative intervention is considered for exertional symptoms, progressive right ventricular enlargement, or right ventricular dysfunction. Tricuspid valve cone repair is the preferred surgical approach. Tricuspid valve replacement and bidirectional cavopulmonary shunt also are considered in patients with advanced disease. Pregnancy generally is well tolerated. Patients with Ebstein anomaly require lifelong follow-up.

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BACKGROUND: Cardiovascular disease is the leading cause of death in patients with Turner syndrome (TS), and cardiovascular surgery is frequently required for management of these patients. TS is associated with medical comorbidities than can complicate the care of this patient population. AIM: To describe the cardiovascular surgical outcomes of patients with TS. METHODS: A retrospective case series was compiled of 51 consecutive TS patients who had at least one cardiovascular surgery at Mayo Clinic Rochester from 1977-2017. The baseline clinical data of these patients were reviewed including demographics, medical comorbidities, congenital heart disease history, and medications. Echocardiographic reports were analyzed in detail. Operative reports and surgical hospital courses were reviewed. Long-term mortality was determined using medical records and the Social Security Death Index. Survival analysis was performed with the Kaplan Meier method. RESULTS: The cohort comprised 51 TS patients, average age at the time of surgery at Mayo Clinic was 28 (8-41) years, and 23 (45%) patients were under the age of 18. At the time of first Mayo Clinic surgery, 18 (35%) patients had previously undergone cardiac surgery at another institution. The most common procedures were repair of aortic coarctation in 14 (28%) patients, aortic valve replacement in 6 (12%) patients, and composite aortic root/ascending aorta replacement in 7 (14%) patients, with 7 patients undergoing repair of more than one lesion. Aortic dissection required operative intervention in 5 patients. After initial Mayo Clinic surgery, subsequent operations were required in 6 (13%) patients. Average hospital length of stay was 6 ± 2 d. There were 4 (8%) early surgical deaths. Freedom from death was 97% and 89% at 10 and 20 years, and the freedom from reoperation was 93% and 81% at 10 and 20 years. CONCLUSION: Cardiovascular surgery is associated with 8% early mortality given the medical complexity of TS patients. Those who survive to dismissal have good survival. Later cardiovascular reoperations are not rare.

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Background Turner syndrome ( TS ) is the most common sex chromosome abnormality in women and is associated with increased morbidity and mortality. We describe long-term outcomes in a large cohort of patients with TS . Methods and Results Retrospective review of patients with TS followed at Mayo Clinic Rochester from 1950 to 2017 was performed. Clinical, imaging, surgical, and genetic data were analyzed. Survival analysis was performed with the Kaplan-Meier method using age- and sex-matched Olmsted County residents as the reference group. The study cohort comprised 317 patients with TS . Average age at diagnosis was 9 (range, 2-12) years, genetic testing was performed in 202 (64%), and pure monosomy X was present in 75 (37%). Congenital heart disease occurred in 131 (41%), with the most frequent lesions being bicuspid aortic valve (n=102, 32%) and coarctation of the aorta (n=43, 14%). Ascending aortic dilation was common, with mean aortic root size index 2 cm/m2, and aortic dissection occurred in 6 (2%) patients. The average follow-up was 11 (range, 2-26) years, yielding 3898 patient-years, and during this period 46 (14%) patients died; mean age at the time of death was 53±17 years. Patients with TS had reduced survival compared with the control group (82% versus 94% at 30 years; P<0.001), and the leading causes of death were cardiovascular disease, liver disease, and malignancy. Conclusions Patients with TS have reduced survival compared with age-matched controls, and cardiovascular disease is the major cause of death. Further studies are required to determine if targeted cardiovascular risk factor modification will result in improved survival in this population.

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OBJECTIVE: To describe outcomes in patients with cor triatriatum sinister (CTS). DESIGN: Retrospective review of patients with CTS followed at Mayo Clinic Rochester from 1990 to 2016. Clinical notes, operative reports, and baseline imaging studies were reviewed including echocardiogram, magnetic resonance imaging, computed tomography, and cardiac catheterization. RESULTS: Fifty-seven patients (median age 34 years; men 32 (56%)) were enrolled. Definitive or suspected CTS diagnosis was made by transthoracic echocardiogram in 41 (72%) patients, and additional multimodality imaging was required in 39 (68%) patients. Of these 57 patients, initial diagnosis was made in adulthood in 35 (61%) patients, and 33 of 57 (58%) patients had additional congenital heart disease (CHD) diagnosis. A total of 27 (47%) patients required surgical resection of CTS membrane during median follow-up of 76 months, and these patients were younger at the time of CTS diagnosis (26 vs 41, P = 0.01) and more likely to have associated CHD (55% vs 45%, P = 0.02). There was one perioperative mortality and no late mortality. There was no recurrence of CTS membrane obstruction in the patients that underwent surgery. Similarly there was no significant increase in CTS membrane gradient in the patients that were managed conservatively. CONCLUSIONS: The natural history of CTS is stability without progressive left atrial obstruction, especially in patients with isolated CTS and in those with initial CTS diagnosis made in adulthood. In patients requiring surgical membrane resection due to flow obstruction, surgery is safe and effective with very low risk of recurrence.

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Anomalous origin of the left coronary artery from the pulmonary artery is rare and typically results in mitral regurgitation, ventricular arrhythmias, heart failure, and sudden death. The condition most often manifests itself in early childhood, but some individuals are diagnosed much later. We describe the case of a 75-year-old woman with heart failure in whom stepwise multimodal imaging revealed anomalous origin of the left coronary artery from the pulmonary artery.

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OBJECTIVES: Left heart obstruction in pregnancy is associated with higher rates of morbidity/mortality. The primary aim of this study was to evaluate maternal cardiovascular, obstetric, and fetal/infant events in pregnant women with left heart obstruction. STUDY DESIGN: Pregnant women with current or repaired left heart obstruction were retrospectively analyzed (2000-2014): mitral stenosis, left ventricular outflow tract obstruction (subvalvar, valvar, supravalvar), and coarctation of the aorta. Maternal cardiovascular events were defined as: heart failure, arrhythmia, urgent/emergent cardiac surgery or percutaneous transcatheter intervention, transient ischemic attack/cerebrovascular accident, and death up to 6 months postpartum. RESULTS: There were 90 pregnancies in 67 women (29±7 years old) who had 15 maternal cardiovascular events. Isolated mitral stenosis (n=6) or >1 serial left heart obstructive lesion (n=6) were the source of the event in the majority pregnancies. Women with isolated mitral stenosis had increased cardiovascular events compared to other single left heart obstructive lesions (OR 18.6, 95% CI: 3.8-91.1). If >1 serial obstructive lesion was present, there was also an increased risk of maternal cardiovascular events (OR 6.8, 95% CI: 1.6-29.1), however isolated mitral stenosis carried similar risk to serial left heart obstructive lesions (OR 2.7, 95% CI: 0.7-11.2). Baseline characteristics associated with events included: New York Heart Association functional class >2 (27% vs. 0, p<0.001), any current left heart obstruction (73% vs. 36%, p=0.01), severe left heart obstruction (40% vs. 29%, p<0.001), and higher Cardiac Disease in Pregnancy ("CARPREG") score (1.2±0.7 vs. 0.5±0.7, p=0.01). There was no difference in rate of obstetric/fetal/infant complications in women with cardiovascular events; however, term birth weight was lower (2.7±0.5 vs. 3.1±0.6kg, p=0.01). There was no maternal mortality. CONCLUSIONS: Isolated mitral stenosis and serial (>1) left heart obstructive lesions carry the highest risk of maternal cardiovascular events. We are the first to show higher event rates in women with serial left heart obstructive lesions. The data supports the need for specialized and experienced high-risk obstetric-cardiac teams to care for women with left heart obstruction, and demonstrates excellent outcomes in a complex cohort of pregnant women with all types of left heart obstruction.

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