L Hudsmith

Femoral Vascular Access Complications in Adult Congenital Heart Disease Patients: Audit from a Single Tertiary Center

OBJECTIVE To determine the rate of vascular access complications in patients with adult congenital heart disease (ACHD). BACKGROUND Complications of femoral access following coronary angiography or percutaneous coronary intervention have been studied extensively, but the complication rate following catheterization and intervention in ACHD patients is poorly documented. DESIGN, SETTING, AND OUTCOME MEASURES: We present a retrospective audit of vascular access complications in a large tertiary ACHD center over a 12-month period. Complications were defined as any clinically significant hematoma, pseudoaneurysm, arteriovenous fistula, or bleeding resulting in the need for imaging, transfusion, vascular or radiological intervention, or delayed discharge. RESULTS Of 197 procedures (102 interventions and 95 cardiac catheterizations), a complication rate of 3.6% was identified, comparable to that of coronary angiography and percutaneous coronary intervention. The main complications were femoral artery pseudoaneurysm and hematoma resulting in delayed discharge by a mean of 2(2/3) days (range 1-4 days). Predictors of risk for vascular complications include female sex, history of diabetes, and anticoagulation; larger sheath sizes and obesity were not associated with higher complication rate. CONCLUSIONS Adult congenital heart disease patients represent a unique and ever-growing population with a higher incidence of catheterization as children, surgical cut-down scars and anatomical variants. We present a low incidence of femoral access complications in interventional and diagnostic procedures in a large series of ACHD patients over a 12-month period. Patients with risk factors for vascular complications may be considered for device closure of the venous access site.

Acquired thoracic aortic interruption: percutaneous repair using graft stents.

Two adult patients with isolated, aortic interruption were successfully treated by percutaneous insertion of graft stents. Prior to the intervention, both patients were hypertensive and on medication. In both cases, an ascending aortogram demonstrated a blind ending of the thoracic aorta distal to the left subclavian artery with a large gradient across the interruption and with multiple collaterals. A graft stent was successfully deployed across the interrupted segment in both cases. We believe that this is one of the first reported cases of percutaneous stenting of aortic interruption and represents a promising new therapeutic option for these adult patients.

Treatment of dynamic subvalvar muscular obstruction in the native right ventricular outflow tract by percutaneous stenting in adults.

Percutaneous techniques are being increasingly used in adult congenital heart disease but there is limited experience in the treatment of native nonvalvar right ventricular outflow tract obstruction. We describe two cases of percutaneous stenting of the subpulmonary region where surgery was not an option.

Spontaneous left atrial thrombus during patent foramen ovale closure

A 52-year-old male smoker suffered a left-sided stroke. Bubble contrast echocardiography demonstrated an aneurysmal atrial septum and patent foramen ovale (PFO). The patient was referred for percutaneous closure of his PFO to reduce his risk of further stroke. Despite dual antiplatelet therapy and pre-procedural heparin, he developed a spontaneous thrombus during balloon sizing of the defect identified by transoesophageal echocardiography. The balloon was immediately withdrawn to the right side and removed. Periprocedural echocardiography using either transoesophageal or intracardiac echo is essential to monitor for this potential procedural complication of percutaneous PFO closure.

Contraception in women with cardiac disease

Cardiac disease is the main cause of maternal mortality in the UK; therefore, accurate and appropriate contraceptive advice for women with cardiac disease is an essential part of any consultation. All females should be counseled regarding pregnancy and contraception before they become sexually active, with information tailored to their diagnosis and risks of a pregnancy. In this review, we discuss current clinical recommendations and practice in a number of different cardiac conditions, including congenital heart disease, as well as highlighting future developments and challenges with contraception in women with cardiac disease.

Employment characteristics of a complex adult congenital heart disease cohort

Background Due to advances in surgical techniques and subsequent management, there have been remarkable improvements in the survival of patients with congenital heart disease. In particular, larger numbers of patients with complex disease are now living into adulthood and are entering the workforce. Aims To establish the types of employment complex adult congenital heart disease (ACHD) patients are engaged in, based on the largest cohort of patients with a single-ventricle circulation in the UK. Methods Records of all patients with a univentricular (Fontan) circulation at the Queen Elizabeth Hospital were reviewed. Employment status was categorized according to the Standard Occupational Classification criteria (2010). Results A total of 210 patient records were reviewed. There was the same proportion of professionals in our cohort compared to the rest of the UK (20% versus 20%). There were greater proportions working in the caring, leisure and other service occupations (15% versus 9%), the elementary occupations (17% versus 11%), sales and customer service occupations (14% versus 8%) and administrative and secretarial occupations (12% versus 11%). The reverse trend was observed for associate professions and technical occupations (7% versus 14%), skilled trades (10% versus 11%), process, plant and machine operatives (3% versus 6%) and managers, directors and senior officials (2% versus 10%). Conclusions The data show that ACHD patients with a single ventricle are engaged in a diverse range of occupations. It is essential that early education and employment advice are given to this cohort to maximize future employment potential.

Pregnancy Outcomes in Women With Transposition of the Great Arteries After an Arterial Switch Operation

Importance A growing number of women are approaching childbearing age after arterial switch surgery for transposition of the great arteries. Prepregnancy counseling requires updated knowledge of the additional cardiovascular risks pregnancy poses for this cohort of women and the potential effect on their offspring; however, to our knowledge, this information is currently unknown. Objective To determine the pregnancy outcomes of women with transposition of the great arteries after an arterial switch operation, as well as the outcomes of their offspring. Design, Setting, and Participants This cohort study assessed women who had had arterial switch surgery from 1985 to the present and who were 16 years or older as of January 2018. All women with a previous arterial switch surgery for transposition of the great arteries with completed or ongoing pregnancy were included. Data were collected in a level 1 congenital cardiology center and joint obstetrics-cardiology clinic in Birmingham, United Kingdom. Exposures Patients were assessed before, during, and after pregnancy. Main Outcomes and Measures Adverse maternal cardiac events (arrhythmia, heart failure, aortic dissection, or acute coronary syndrome) and aortic root dilatation, aortic regurgitation, and left ventricular function before and after pregnancy were the main outcomes. Mode of delivery and fetal outcomes were considered secondary outcomes. Results A total of 25 pregnancies were identified in 15 women; 8 women had had 1 pregnancy, while 7 were multiparous. There were no adverse maternal cardiac events. Before pregnancy, 8 women (53%) had no aortic regurgitation, 1 (7%) had a trivial degree of regurgitation, 4 (26%) had mild regurgitation, and 2 (14%) had moderate regurgitation. After pregnancies, 1 woman (7%) had minor progression of aortic regurgitation. Five women (36%) had mild neoaortic root dilatation prepregnancy, but none developed progressive dilatation in the first year post-partum. A total of 24 pregnancies were completed by the end of the study, with all infants born alive and well. Nineteen modes of delivery were known; there were 7 cesarean deliveries (37%), of which 2 (11%) were recommended for aortic dilatation and 5 (26%) for obstetric indications or maternal choice. Conclusions and Relevance Pregnancy is well tolerated after arterial switch operation; no adverse maternal cardiac events or early progression of neoaortic root dilatation or aortic regurgitation were observed in this study. These results provide evidence to allow reassurance of women with previous arterial switch surgery who are planning pregnancies.

Pregnancy outcomes in patients with a fontan circulation and proposal for a risk-scoring system: single centre experience

BackgroundPregnancy in women with Fontan physiology poses a significant management challenge and is deemed high risk. The aim of this study is to describe short and long-term pregnancy outcomes in women who have undergone Fontan palliation and propose a novel risk-stratification model specific to women with a Fontan heart.MethodsWe undertook a single-centre, retrospective cohort study of all female Fontan patients (n = 78) from 1991–2015. We recorded pregnancy outcome, maternal cardiovascular and obstetric complications and fetal outcome. We propose a risk stratification model to identify those women who might be at highest risk of adverse outcomes during pregnancy.ResultsTwenty-one women had 55 pregnancies, with 13 (24%) live births and 38 (69%) spontaneous miscarriages (p < 0.001). Eight (62%) out of 13 live birth pregnancies incurred maternal cardiovascular complications and six (46%) experienced maternal obstetric complications. Median gestational age at delivery was 32(27–39) weeks with 12 out of 13 (92%) pregnancies resulting in pre-term delivery. There were more pregnancies (OR 4.90, 95% CI 1.46–16.42, p ≤ 0.01) and a trend towards a higher proportion of live births (OR 7.60, 95% CI 1.81–31.97, p = 0.06), in the ‘lower risk’ compared to those women in the ‘very high risk’ group. There were no maternal deaths.ConclusionsWe observed a high first trimester miscarriage rate, significant maternal cardiovascular and obstetric complication rates and a high rate of pre-term births in pregnant Fontan women. Our risk stratification model requires further investigation but may identify those women at particularly high risk of a poor outcome, and inform realistic pre-pregnancy counselling.

Feasibility of performing non-contrast magnetic resonance angiography in pregnant subjects with familial aortopathies

BACKGROUND Pregnancy is associated with an increased risk of aortic pathology. We sought to assess the feasibility of performing non-contrast 3D steady-state free-precession (SSFP) magnetic resonance angiography (MRA) in pregnant subjects with inherited aortopathy. METHODS Fifteen pregnant subjects (age 27±4yr) with positive genotyping for aortopathy (Marfan, Loeys-Dietz, Ehlers-Danlos) and/or a family history of aortic dissection underwent non-contrast 3D-SSFP MRA at 1.5T (Avanto, Siemens Healthcare, Erlangen, Germany) using a modified ECG-triggered orientated in a sagittal-oblique plane with a respiratory navigator at the diaphragmatic level (mean acquisition time 4.1±1.9min). Imaging was performed during the mid-trimester (21±5weeks). Image analysis was performed off-line using Cvi42 software (Circle Cardiovascular Imaging, Calgary, Canada). An assessment of image quality (score 0-3) was made before performing inner edge to inner edge measurements of the thoracic aorta at 7 levels from the multiplanar reconstructions by two independent blinded observers. RESULTS Non-contrast 3D-MRA was successfully acquired in all 15 subjects. Image quality was deemed excellent in 87% (13/15) of cases after a mean acquisition time of 4.1±1.9min. There was a high level of agreement for aortic measurements, with low intra- and inter-observer variability (ICC ranges; 0.95-0.99 and 0.92-0.98, respectively). All pregnancies reached term (≥37/40) with a mean gestation at delivery of 38.0±0.5weeks. The mode of delivery was vaginal in 9 out of 15 subjects (60%). CONCLUSIONS Non-contrast SSFP MRA imaging provides a quick and reproducible method of assessing the thoracic aorta in pregnancy.

Suitability of cardiac resynchronisation therapy in patients with Fontan circulation and congenitally corrected transposition of the great arteries

BACKGROUND Cardiac resynchronisation therapy (CRT) is a well-recognised treatment in systolic heart failure. There is limited evidence in congenital patients with univentricular hearts or systemic right ventricles. In 2014 PACES/HRS published a consensus statement recommending CRT if ventricular ejection fraction (EF)≤35%, QRS duration≥150ms (with RBBB in systemic RV), NYHA II-IV and ventricular dilatation. The incidence of patients meeting these criteria in whom CRT is possible is not known. METHODS Retrospective analysis of 203 patients with a univentricular Fontan circulation and 55 patients with ccTGA under specialist ACHD care. RESULTS Univentricular functional data was available for 194 (96%), 10 (5%) having EF≤35%. QRS duration was available for 190 (94%) and was ≥150ms in five (3%). EF data was available for 54 (98%) ccTGA patients, and was ≤35% in 6 (11%). QRS duration was ≥150ms in 13 (26%). Only four patients fulfilled recommendations and two received CRT. CONCLUSIONS Only a small proportion of patients with single ventricles or ccTGA meet the criteria for CRT. In many of these patients there are significant anatomical barriers to CRT which limit its use in this population. The decision to implant CRT in complex ACHD requires discussion in a combined ACHD electrophysiology surgical multidisciplinary meeting and close collaboration with patients.