Pregnancy in Patients with the Fontan Operation.

Improved survival rates for patients with a Fontan circulation has allowed more women with this complex cardiac physiology to contemplate pregnancy. However, pregnancy in women with a Fontan circulation is associated with a high risk of adverse maternal and fetal outcomes, high rates of miscarriage and preterm delivery. Factors associated with a successful pregnancy outcome are: younger age, normal body weight, absence of significant functional limitation, no Fontan-related complications, and well-functioning single ventricle physiology. Appropriate care with timely preconception counselling and regular, frequent clinical reviews by a multidisciplinary team based at a tertiary centre, improves the chance of a successful pregnancy. Empowerment of patients with education on their specific congenital cardiac condition and its projected trajectory, helps them make informed choices regarding their health, reproductive choices and assists them to achieve their life goals.

Right ventricular systolic to diastolic duration ratio: A novel predictor of outcome in adult idiopathic pulmonary arterial hypertension.

BACKGROUND: The systolic to diastolic (SD) duration ratio reflects global RV performance in pulmonary arterial hypertension (PAH) yet limited data exists on its application to adult non-congenital PAH. We measured SD ratios on echocardiogram in idiopathic PAH (IPAH) to establish its response to pulmonary vasodilator therapy and prognostic value at diagnosis and follow up. METHODS: Incident patients with IPAH undergoing echocardiogram, haemodynamic and exercise assessments were identified within our centre between 2005 and 2018. SD ratios were adjusted for heart rate at diagnosis and follow up. RESULTS: In 98 patients at diagnosis, the mean SD ratio was 1.03 ±â€¯0.37 decreasing to 0.85 ±â€¯0.25, p < 0.001 at follow-up echocardiogram performed at a median interval of 9.0 months. The SD ratio at diagnosis correlated weakly with RV basal diameter (r = 0.24, p = 0.04) and 6MWD (r = 0.23, p = 0.04). At follow up, the mean SD ratio was lower in those receiving combination vs monotherapy pulmonary vasodilator treatment (71 ±â€¯25 vs 92 ±â€¯22% baseline respectively, p < 0.001). After a median follow-up of 4.8 years, 3 patients were transplanted and 23 patients died. The SD ratio at diagnosis and follow up predicted an increased risk of death/transplantation (HR 2.41 (1.09-5.29), p = 0.03; HR 5.02 (1.27-19.77), p = 0.02 respectively), retaining its predictive value at diagnosis in bivariate models with 6MWD (HR 2.18 (1.06-4.08)), WHO Functional Class (HR 2.33 (1.04-5.21)) and TAPSE (HR 2.36 (1.07-5.19)), all p < 0.05. CONCLUSIONS: The SD ratio carries prognostic value at diagnosis and follow up in IPAH. Its further evaluation alongside current PAH risk stratification parameters should be considered.

The importance of left heart disease as a cause of pulmonary hypertension in COPD.

Chronic obstructive pulmonary disease (COPD) is a common respiratory condition that presents with varying degrees of severity and can be complicated by further comorbidities. Up to a third can also have pulmonary hypertension, which is an important risk factor associated with an increase in morbidity and mortality. The etiology of pulmonary hypertension contributes to diagnosis. Considering and identifying the type of pulmonary hypertension at the right time will influence management and outcome in patients with COPD.

Anomalous left coronary artery from the pulmonary artery (ALCAPA) diagnosed in adulthood: Varied clinical presentation, therapeutic approach and outcome.

INTRODUCTION: The diagnosis of ALCAPA syndrome is sporadic in adulthood, of the limited cases in the literature most are incidental or without symptoms. There is a broad spectrum of clinical manifestations of ALCAPA syndrome however, including sudden cardiac death. CASES: We present herewith a series of 12 consecutive patients with ALCAPA, all diagnosed in adulthood (between 18 and 73 years of age). Five patients developed symptoms (breathlessness) after the fourth decade of life, 3 were undiagnosed despite a history of previous mitral valve repair, one presented with heart failure, one with resuscitated cardiac arrest, whereas two patients were asymptomatic. We review in this paper, the clinical history, diagnostic approach and therapeutic choices of ALCAPA syndrome. CONCLUSION: ALCAPA syndrome is not confined to childhood, late diagnosis in adulthood has a varied clinical presentation. ALCAPA syndrome should be particularly considered as a potential, albeit uncommon cause of mitral regurgitation and/or dilated cardiomyopathy.

Pre-pregnancy counseling for women with heart disease: A prospective study.

BACKGROUND: Women with cardiac disease and their infants are at a greater risk of mortality and morbidity during pregnancy. Expert groups recommend preconception counseling (PCC) for all women with cardiac disease so they are made aware of these risks. We have run a specialist maternal cardiac clinic since 1996. The aim of this study was to evaluate the experience of women who have received PCC within an established multidisciplinary tertiary clinic and to establish their views regarding the counseling they received. METHODS: Single centre prospective study using a patient questionnaire was given to women attending a specialist cardiac preconception counseling clinic from November 2015 to August 2016, with analysis of descriptive data and free text comments from the questionnaire responders. RESULTS: 40/65 returned patient questionnaires. Prior to the consultation fewer than half felt well informed regarding how their heart disease could impact upon pregnancy but a similar proportion felt nonetheless that they would be able to have a healthy pregnancy. Women reported two main areas of concerns, their own health (whether they would survive a pregnancy) and the health of their child. 15% of women reported that these concerns had prevented them from pursuing a pregnancy. Women reported high satisfaction rates with the clinic. CONCLUSIONS: There is an increasing demand for PCC services for women with cardiac disease; our study is the first attempt to determine both the acceptability and the impact of PCC from the patient perspective. Patients reported a high level of satisfaction with the service provided.

Anaesthetic management of parturients with univentricular congenital heart disease and the Fontan operation.

Women with a single ventricle circulation palliated with the Fontan operation require specialist multidisciplinary management. We report 14 such cases with successful pregnancies and detail the pathophysiology encountered. A combined obstetric and cardiac service between Chelsea and Westminster Hospital and Royal Brompton Hospital provides care for women with heart disease, and maintains a prospective database of referred women. We searched this database for women with a known Fontan circulation and reviewed the case notes and electronic patient records between January 1994 and December 2015. Eight women palliated with the Fontan operation delivered 14 live babies over the study period, with detailed peripartum management available for 11. Low-dose combined spinal-epidural or epidural labour analgesia was the intended mode of analgesia or anaesthesia for all deliveries (depending on clinical scenario and clinician preference), and was performed in 79%. Seven cases (50%) had a caesarean delivery. A neuraxial catheter technique was preferred (86%), whether or not vaginal delivery was attempted first. There were no deliveries under general anaesthesia. Fifty percent of cases were complicated by postpartum haemorrhage. Other peripartum complications included arrhythmias (29%), chest pain (14%) and intrauterine growth restriction (57%). Women with a Fontan circulation are increasingly encountered in obstetric practice. A good understanding of the underlying anatomy and its impact on physiology, coupled with meticulous care are essential to allow safe delivery for mother and baby. Multidisciplinary input into peripartum care is required, with anticipation of increased risk of complications such as haemorrhage and arrhythmias.

Physiologic determinants of exercise capacity in patients with different types of right-sided regurgitant lesions: Ebstein's malformation with tricuspid regurgitation and repaired tetralogy of Fallot with pulmonary regurgitation.

BACKGROUND: Exercise capacity relates to right ventricular (RV) volume overload in congenital heart disease and may improve after surgery. We herewith investigate the relation between exercise capacity, cardiac index, and RV volume overload due to tricuspid regurgitation (TR) in Ebstein's malformation and pulmonary regurgitation (PR) after repair of tetralogy of Fallot (rToF). METHODS: We measured cardiac index and tricuspid/pulmonary regurgitant fraction by cardiovascular magnetic resonance in patients with Ebstein's malformation (n = 40) or rTOF (n = 53) with at least moderate TR/PR and 24 healthy controls. Exercise tolerance was determined by peak oxygen consumption (peak VO2) during cardiopulmonary exercise testing. RESULTS: TR and PR fraction were similar in Ebstein and rTOF patients (43 ± 17% versus 39 ± 12%, respectively). Cardiac index was reduced in Ebstein (2.7 ± 0.6L/min/m(2) compared to controls 3.5 ± 0.9L/min/m(2), p < 0.001) but not in rToF patients (3.2 ± 0.5L/min/m(2)). Multiple regression analysis revealed a significant correlation between peak VO2 and cardiac index in Ebstein. Furthermore, peak VO2 correlated with peak heart rate in both groups but not with regurgitation fraction. CONCLUSIONS: Despite comparable amounts of regurgitation from a right sided heart valve in patients with Ebstein and rToF, reduction of cardiac index was observed only in the former group. Greater physiologic complexity and adverse ventricular interaction with chronotropic incompetence in Ebstein's malformation may account for this.

Maternal cardiac and obstetric performance in consecutive pregnancies in women with heart disease.

OBJECTIVE: Second pregnancies are usually less complicated than first pregnancies, and have a better outcome in terms of fetal growth. We studied a group of women with heart disease to assess whether their second pregnancy was less complicated and resulted in a larger baby. DESIGN: Retrospective case control study. SETTING: Tertiary referral academic obstetric unit. POPULATION: First and second pregnancies in 77 women with congenital and acquired heart disease and in 154 control women were identified. METHODS: Data were collected from medical and obstetric records. MAIN OUTCOME MEASURES: Cardiac complications, obstetric complications, intra-partum events, birthweight and perinatal complications. RESULTS: The rate of obstetric complication was greater in first pregnancies in both the heart disease and the control groups (38% versus 26%, cf. 20% versus 17%). In the heart disease group, the rate of cardiac complications was similar in first and second pregnancies (9% versus 6%). Overall, significantly more perinatal complications were seen in the heart disease group, with no significant difference between first and second pregnancies (36% versus 27%, cf. 14% versus 12%). Median birthweight was significantly higher in second pregnancies in the control group (3308 versus 3519 g P < 0.001), but not significantly different between pregnancies in the heart disease group (3014 versus 3133 g, P = 0.19). CONCLUSIONS: This case control study demonstrates that women with mild to moderate heart disease have similar pregnancy outcomes in consecutive pregnancies. However, while the median birthweight was higher in the control second pregnancies, it was not increased in the women with heart disease. TWEETABLE ABSTRACT: A study of women with heart disease to assess whether their second pregnancy was less complicated.

Prevalence and prognostic implication of restenosis or dilatation at the aortic coarctation repair site assessed by cardiovascular MRI in adult patients late after coarctation repair.

BACKGROUND: Cardiovascular magnetic resonance (CMR) is ideal for assessing patients with repaired aortic coarctation (CoA). Little is known on the relation between long-term complications of CoA repair as assessed by CMR and clinical outcome. We examined the prevalence of restenosis and dilatation at the repair site and the long-term outcome in patients with repaired CoA. METHODS AND RESULTS: CMR imaging and clinical data for adult CoA patients (247 patients aged 33.0 ± 12.8 years, 60% male), were analyzed. The diameter of the aorta at the repair site was measured on CMR and its ratio to the aortic diameter at the diaphragm (repair site-diaphragm ratio, RDR) was calculated. Restenosis (RDR≤70%) was present in 31% of patients (and significant in 9% [RDR<50%]), and dilatation (RDR>150%) in 13.0%. A discrete aneurysm at the repair site was observed in 9%. Restenosis was more likely after resection and end-end anastomosis, whereas dilatation after patch repair. Systemic hypertension was present in 69% of patients. Of the hypertensive patients, blood pressure (133 ± 20/73 ± 10 mm Hg) was well controlled in 93% with antihypertensive therapy. Mortality rate over a median length of 5.9 years was low (0.69% per year, 95% CI: 0.33-1.26), but significantly higher than age-matched healthy controls (standardised mortality ratio 2.86, CI 1.43-5.72, p<0.001). CONCLUSION: Restenosis or dilatation at the CoA repair site as assessed by CMR is not uncommon. Medium term survival remains good, however, albeit lower than in the general population. Life-long follow-up and optimal blood pressure control are likely to secure a good longer term outlook in these patients.

Marfan syndrome and pregnancy: maternal and neonatal outcomes.

OBJECTIVE: To report outcomes in a recent series of pregnancies in women with Marfan syndrome (MFS). DESIGN: Retrospective case note review. SETTING: Tertiary referral unit (Chelsea and Westminster and Royal Brompton Hospitals). SAMPLE: Twenty-nine pregnancies in 21 women with MFS between 1995 and 2010. METHODS: Multidisciplinary review of case records. MAIN OUTCOME MEASURES: Maternal and neonatal mortality and morbidity of patients with MFS and healthy controls. RESULTS: There were no maternal deaths. Significant cardiac complications occurred in five pregnancies (17%): one woman experienced a type-A aortic dissection; two women required cardiac surgery within 6 months of delivery; and a further two women developed impaired left ventricular function during the pregnancy. Women with MFS were also more likely to have obstetric complications (OR 3.29, 95% CI 1.30-8.34), the most frequent of which was postpartum haemorrhage (OR 8.46, 95% CI 2.52-28.38). There were no perinatal deaths, although babies born to mothers with MFS were delivered significantly earlier than those born to the control group (median 39 versus 40 weeks of gestation, Mann-Whitney U-test, P = 0.04). These babies were also significantly more likely to be small for gestational age (24% in the MFS group versus 6% in the controls; OR 4.95, 95% CI 1.58-15.55). CONCLUSIONS: Pregnancy in women with MFS continues to be associated with significant rates of maternal, fetal, and neonatal complications. Effective pre-pregnancy counselling and meticulous surveillance during pregnancy, delivery, and the puerperium by an experienced multidisciplinary team are warranted for women with MFS.

Pulmonary hypertension and pregnancy--a review of 12 pregnancies in nine women.

OBJECTIVE: To report outcomes in a recent series of pregnancies in women with pulmonary hypertension (PH). DESIGN: Retrospective case note review. SETTING: Tertiary referral unit (Chelsea and Westminster and Royal Brompton Hospitals). SAMPLE: Twelve pregnancies in nine women with PH between 1995 and 2010. METHODS: Multidisciplinary review of case records. MAIN OUTCOME MEASURES: Maternal and neonatal mortality and morbidity. RESULTS: There were two maternal deaths (1995 and 1998), one related to pre-eclampsia and one to arrhythmia. Maternal morbidity included postpartum haemorrhage (five cases), and one post-caesarean evacuation of a wound haematoma. There were no perinatal deaths, nine live births and three first-trimester miscarriages. Mean birthweight was 2197 g, mean gestational age was 34 weeks (range 26-39), and mean birthweight centile was 36 (range 5-60). Five babies required admission to the neonatal intensive care unit, but were all eventually discharged home. All women were delivered by caesarean section (seven elective and two emergency deliveries), under general anaesthetic except for one emergency and one elective caesarean performed under regional block. CONCLUSIONS: Maternal and fetal outcomes for women with PH may be improving. However, the risk of maternal mortality remains significant, so that early and effective counselling about contraceptive options and pregnancy risks should continue to play a major role in the management of such women when they reach reproductive maturity.

Sinus venosus atrial septal defect in a 31-year-old female patient: a case for surgical repair.

A 31-yr-old female patient previously diagnosed with idiopathic pulmonary arterial hypertension (PAH) was referred to our centre for further evaluation. Cardiac magnetic resonance imaging (MRI) and echocardiography revealed a superior sinus venosus atrial septal defect (ASD) with partial anomalous pulmonary venous drainage. Following re-diagnosis, surgical repair was considered. Despite a disproportionately high mean pulmonary artery pressure (P(pa)) of 47 mmHg relative to the patient's age and defect, the decision to operate was based on the absence of oxygen desaturation (either at rest or during exercise), cyanosis or abnormally elevated haemoglobin. Other operability criteria included normal sinus rhythm at rest, vasoreactivity and a pulmonary to systemic blood flow ratio of 1.9 at rest. Surgical repair and continued advanced therapy with bosentan 125 mg b.i.d. and aspirin 75 mg o.d. proved successful, with post-operative improvements in exercise capacity and dyspnoea, and a reduction in P(pa) to 25 mmHg. Keen to start a family, the risks of pregnancy were discussed. This case illustrates the importance of secondary PAH in sinus venosus ASD and the need to exclude a sinus venosus ASD in unexplained right ventricular dilatation. Access to expertise in congenital heart disease and use of cardiac MRI can improve diagnosis and, in turn, treatment decisions.

Cerebrovascular accidents in adult patients with congenital heart disease.

OBJECTIVE: To investigate the prevalence and characteristics of cerebrovascular accidents (CVA) in a large population of adults with congenital heart disease (CHD). METHODS AND RESULTS: In a retrospective analysis of aggregated European and Canadian databases a total population of 23 153 patients with CHD was followed up to the age of 16-91 years (mean 36.4 years). Among them, 458 patients (2.0%) had one or more CVA, with an estimated event rate of 0.05% per patient-year. Permanent neurological sequelae were noted in 116 patients (25.3%). The prevalence of CVA in selected diagnostic categories was as follows: open atrial septal defect 93/2351 (4.0%); closed atrial or ventricular septal defect 57/4035 (1.4%); corrected tetralogy of Fallot 52/2196 (2.4%); Eisenmenger physiology 24/467 (5.1%); other cyanotic 50/215 (23.3%); mechanical prostheses (29/882 (3.3%). Associated conditions in patients with CVA were absence of sinus rhythm (25%), transvenous pacemakers (7%), endocarditis (2%), cardiac surgery (11%) and catheter intervention (2%), but with the exception of absent sinus rhythm these were not significantly more prevalent in patients with CVA. CONCLUSION: CVA are a major contributor to morbidity in this young population despite absence of classical cardiovascular risk factors. Although the prevalence of CVA in patients with CHD appears low, it is 10-100 times higher than expected in control populations of comparable age. Residua occur in a strong minority of patients. The subjects at highest risk are those patients with CHD with cyanotic lesions, in whom the prevalence is over 10-fold above the average.