Stephanie L. Curtis

Balloon expandable stent implantation for native and recurrent coarctation of the aorta—prospective computed tomography assessment of stent integrity, aneurysm formation and stenosis relief

Background Stenting for aortic coarctation is known to be effective in the medium term. Aneurysm formation following stent implantation is a recognised complication. However, data regarding aortic wall injury and stent integrity following stent placement are sparse. Objectives We report comprehensive clinical, echocardiographic and prospective CT follow-up data following stenting for aortic coarctation from a single centre. Methods Full data analysis of all patients undergoing balloon expandable stent implantation and follow-up procedures in a single tertiary congenital cardiac unit. Results Between October 2002 and April 2008, we performed 102 coarctation stent procedures on 88 patients. Median age was 20.6 years (range 8.5–65) and median weight 65 kg (range 34–101). 94 stents (26 covered) were implanted. 12 procedures were re-dilatations. Stenting resulted in a reduction of the gradient across the site of coarctation, from a median of 20 mm Hg to 4 mm Hg. There were no procedure-related deaths. Four patients had immediate complications (one requiring emergency surgery). During median follow-up of 34.5 months (range 4.2–72.8), two patients had late complications requiring additional stent procedures. Follow-up CT data are available in 84 patients with MRI in one patient (96.5%). Only one patient developed a procedure-related aortic aneurysm. All stent fractures (n=7) occurred with a single stent design. Conclusions Stenting for aortic coarctation and re-coarctation is effective with low immediate complication rates. CT is useful in the longer term for assessment of stent integrity and post-procedural aneurysm formation. Overall incidence of post-procedural aneurysm is rare and stent fractures were not seen with newer generation stents.

Low‐molecular‐weight heparin or warfarin for anticoagulation in pregnant women with mechanical heart valves: what are the risks? A retrospective observational study

Please cite this paper as: Basude S, Hein C, Curtis S, Clark A, Trinder J. Low‐molecular‐weight heparin or warfarin for anticoagulation in pregnant women with mechanical heart valves: what are the risks? A retrospective observational study. BJOG 2012;119:1008–1013.

Results of Screening for Intracranial Aneurysms in Patients with Coarctation of the Aorta

BACKGROUND AND PURPOSE: IAs are found in 2.3% of adults; the mean age at detection is 52 years. Prevalence is <0.5% in young adults. Early studies suggest that 10%–50% of patients with aortic coarctation have IAs. Screening recommendations are variable. We sought to examine the prevalence of IAs through screening with MRA. MATERIALS AND METHODS: Consecutive patients older than 16 years of age with coarctation undergoing brain MRA between May 1999 and October 2007 were included. MRA was performed by using a 1.5T scanner with a 3D time-of-flight protocol; simultaneous MR imaging was performed of the heart and aorta. Cerebral MRAs were double-reported by a neuroradiologist. Statistics are described as mean ± SD and median ± range. Continuous variables were compared by using Student t tests and Mann-Whitney U tests (categoric variables, by using the Fisher exact test). RESULTS: One hundred seventeen MRAs were double-reported. The median age was 29 ± 11 years (range, 16–59 years). IAs were found in 12 patients (10.3%). The mean diameter of IAs was 3.9 mm (range, 2.0–8.0 mm). Patients with aneurysms were older (median, 37 years; range, 16–50 years) than those without (median, 23 years; range, 16–59 years; Z = −2.01, P = .04). Hypertension was more common in those with IAs (IA 83% versus no IA 43%, P = .01). There was no association between ascending aortopathy, bicuspid aortic valves, and IAs. CONCLUSIONS: Patients with coarctation have a higher prevalence of IAs, occurring at an earlier age than in population studies. Whether routine screening is appropriate for this group of patients is unclear. Hypertension is likely to be an important pathophysiologic factor.

Efficacy of percutaneous closure of patent foramen ovale: comparison among three commonly used occluders

Background Percutaneous closure of patent foramen ovale (PFO) is standard treatment for patients with paradoxical embolism but studies examining the efficacy of the various occluders are lacking. Objective To evaluate short- and medium-term closure rates of three common occluders. Methods One hundred and sixty-six adults (47±12 (18–81 years)) were evaluated with transthoracic bubble echocardiography before and after PFO closure. Only patients with large PFOs were included (>30 bubbles in the left heart after Valsalva). Results Three occluders were used: Amplatzer (AGA Medical Corporation) (n=80, 48%), Gore Helex (n=48, 29%) and Premere TM (St Jude Medical) (n=38, 23%). One (0.6%) neurological event occurred during follow-up. At 6 months significant residual shunting after Valsalva was highest in the group that received the Helex (58.3%), and lower for Premere (39.5%) and Amplatzer (32.5%). At final follow-up residual shunting remained higher in patients with the Helex (33.3%) than in Premere (18.5%) and Amplatzer (11%). Amplatzer had a significantly lower residual shunt rate than Helex (p<0.05 at 6 months and final follow-up). The Premere had an intermediate residual shunt rate. Septal aneurysm also predicted residual shunting (RR=24.7, 95% CI: 8.2 to 74.4, p<0.0001). Conclusions Percutaneous PFO closure is an efficacious progressive treatment but closure rates also depend on the presence of aneurysm and differ between occluders.

Current trends in the management of heart disease in pregnancy

BACKGROUND The management of heart disease in pregnancy is highly specialized. Guidelines are based on observational studies. This paper describes our experience of these patients, including adverse cardiac events, adherence to guidelines, and areas of suboptimal management. METHODS Patients referred to the service between 01/05/1999 and 30/06/2005 were identified using clinic lists and keyword searches in databases. A list of 40 management standards was created from European Society of Cardiology and the Confidential Enquiry into Maternal and Child Health guidelines. Adherence to these was recorded and adverse cardiac events noted. RESULTS There were 177 pregnancies in 155 women with a mean age of 28+/-6 years. Service referrals increased linearly throughout the study period. Of 131 cardiac pregnancies 101 had congenital heart disease (77.1%). Pulmonary oedema, deteriorating functional class, sustained arrhythmia or cardiac intervention occurred in 13 pregnancies (10.2%), though not always in high risk cases. Management guidelines were largely followed, though areas of suboptimal management included lack of pre-conception advice and inadequate post-partum follow-up. Controversial areas include the use of beta-blockade in coarctation of the aorta and the use of elective Caesarean section in high risk patients. CONCLUSIONS Cardiac pregnancies are increasing, mainly due to the rise in patients with congenital heart disease. Some patients will experience adverse cardiac events, including low risk patients. Pre-conception advice and post-partum follow-up should be improved. In the absence of prospective studies, management is likely to be driven by observational studies.

Cardiac outcome of pregnancy in women with a pacemaker and women with untreated atrioventricular conduction block

AIMS The natural history and outcome of pregnancy in patients with a pacemaker or those presenting with atrioventricular conduction block in pregnancy are unknown with only a limited number of case reports published. METHODS AND RESULTS This study examines the progress and outcome of 25 pregnancies in 18 women who were either paced or presented with untreated atrioventricular conduction block during pregnancy. All patients were seen in a single referral centre between 1998 and 2008 and were evaluated at regular intervals with ECG, echocardiography, and 24 h Holter. Four women (4 pregnancies) had new-onset atrioventricular block, 3 women (5 pregnancies) had previously diagnosed atrioventricular block who had not undergone pacing, and 11 women (16 pregnancies) had known atrioventricular block with a pacemaker prior to pregnancy. Of the four patients presenting for the first time in pregnancy, the frequency or severity of atrioventricular conduction block increased during pregnancy. One required pacing during and one after pregnancy. In two patients the conduction disturbance resolved postpartum. In the three patients who had known but untreated atrioventricular block before pregnancy, this progressed during each pregnancy but did not require pacing. In patients paced before pregnancy, there were no complications as a result of the pacemaker, but maternal complications were seen in patients with underlying structural heart disease. CONCLUSIONS Atrioventricular block in pregnancy is progressive; pacing is not always required but all patients should be closely monitored during and after pregnancy. In patients paced before pregnancy, pacing is well tolerated.

Retrospective UK multicentre study of the pregnancy outcomes of women with a Fontan repair

Background The population of women of childbearing age palliated with a Fontan repair is increasing. The aim of this study was to describe the progress of pregnancy and its outcome in a cohort of patients with a Fontan circulation in the UK. Methods A retrospective study of women with a Fontan circulation delivering between January 2005 and November 2016 in 10 specialist adult congenital heart disease centres in the UK. Results 50 women had 124 pregnancies, resulting in 68 (54.8%) miscarriages, 2 terminations of pregnancy, 1 intrauterine death (at 30 weeks), 53 (42.7%) live births and 4 neonatal deaths. Cardiac complications in pregnancies with a live birth included heart failure (n=7, 13.5%), arrhythmia (n=6, 11.3%) and pulmonary embolism (n=1, 1.9%). Very low baseline maternal oxygen saturations at first obstetric review were associated with miscarriage. All eight women with saturations of less than 85% miscarried, compared with 60 of 116 (51.7%) who had baseline saturations of ≥85% (p=0.008). Obstetric and neonatal complications were common: preterm delivery (n=39, 72.2%), small for gestational age (<10th percentile, n=30, 55.6%; <5th centile, n=19, 35.2%) and postpartum haemorrhage (n=23, 42.6%). There were no maternal deaths in the study period. Conclusion Women with a Fontan circulation have a high rate of miscarriage and, even if pregnancy progresses to a viable gestational age, a high rate of obstetric and neonatal complications.

Pregnancy following Mustard or Senning correction of transposition of the great arteries: a retrospective study

To determine pregnancy outcome in women with atrial switch repair for transposition of the great arteries (TGA) and to compare follow up with a control group of childless women with the same repair.

Pregnancy outcome and follow-up cardiac outcome in women with aortic valve replacement

Objectives To compare the maternal, fetal and cardiac outcomes in women who have undergone aortic valve replacement. Method Retrospective observational study of all women with aortic valve replacement, who underwent a pregnancy (1998–2012). Maternal-, fetal- and valve-related cardiac outcomes were assessed. Results Thirty-two pregnancies in 16 women with aortic valve replacement (nine bioprosthetic, six Ross and 17 mechanical) were evaluated. There were no adverse maternal events in the bioprosthetic and Ross groups but three in the mechanical group. Fetal loss rate was highest in the mechanical valve pregnancies (53%). One woman in the bioprosthetic group needed valve re-operation, and one woman in the mechanical valve group died. There was no difference in the change of Vmax over the follow-up between the valves (p = 0.25). Conclusions There was no difference in deterioration between aortic valve replacements during and after pregnancy. The highest risk of maternal and fetal complications occurred in the mechanical valve group.

Long QT syndrome and left ventricular noncompaction in 4 family members across 2 generations with KCNQ1 mutation

The association of long QT syndrome and left ventricular noncompaction is uncommon, with only a handful of previous reports, and only one reported case in association with a mutation in KCNQ1. Here we present genetic and phenotypic data for 4 family members across 2 generations who all have evidence of prolonged QT interval and left ventricular noncompaction in association with a pathogenic mutation in KCNQ1, and discuss the potential mechanisms of this association. In conclusion, we suggest that it may be helpful to consider looking for mutations in KCNQ1 in similar patients.